• Share
  • Email
  • Embed
  • Like
  • Save
  • Private Content
Pediatric surgery atlas
 

Pediatric surgery atlas

on

  • 9,498 views

 

Statistics

Views

Total Views
9,498
Views on SlideShare
9,498
Embed Views
0

Actions

Likes
3
Downloads
915
Comments
2

0 Embeds 0

No embeds

Accessibility

Categories

Upload Details

Uploaded via as Adobe PDF

Usage Rights

© All Rights Reserved

Report content

Flagged as inappropriate Flag as inappropriate
Flag as inappropriate

Select your reason for flagging this presentation as inappropriate.

Cancel

12 of 2 previous next

  • Full Name Full Name Comment goes here.
    Are you sure you want to
    Your message goes here
    Processing…
Post Comment
Edit your comment

    Pediatric surgery atlas Pediatric surgery atlas Document Transcript

    • SPRINGER SURGERY ATLAS SERIES Series Editors: J. S. P. Lumley · J. R. Siewert
    • SPRINGER SURGERY ATLAS SERIES Series Editors: J. S. P. Lumley · J. R. Siewert
    • P. Puri · M. E. Höllwarth (Eds.)Pediatric Surgery With 589 Color Figures, in 666 separate Illustrations
    • Prem Puri MS, FRCS, FRCS (Ed), FACSNewman Clinical Research Professor,University College, DublinConsultant Paediatric Surgeon andDirector of Research Children’s Research CentreOur Lady’s Hospital for Sick ChildrenCrumlinDublin 12, IrelandMichael E. Höllwarth MDProfessor & HeadDepartment of Paediatric SurgeryMedical University of GrazAuenbruggerplatz8036 GrazAustria ISBN-10 3-540-40738-3 Springer-Verlag Berlin Heidelberg New York ISBN-13 978-3-540-40738-6 Springer-Verlag Berlin Heidelberg New York Library of Congress Control Number: 2004104708 This work is subject to copyright. All rights are reserved, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilm or in any other way, and storage in data banks. Duplication of this publication or parts thereof is permitted only under the provisions of the German Copyright Law of September 9, 1965, in its current ver- sion, and permission for use must always be obtained from Springer. Violations are liable to prosecution under the German Copyright Law. Springer is a part of Springer Science+Business Media springeronline.com © Springer-Verlag Berlin Heidelberg 2006 Printed in Germany The use of general descriptive names, registered names, trade- marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. Product liability: the publishers cannot guarantee the accuracy of any information about dosage and application contained in this book. In every individual case the user must check such information by consulting the relevant literature. Editor: Gabriele Schröder, Heidelberg, Germany Desk Editor: Stephanie Benko, Heidelberg, Germany Wissenschaftliche Zeichnungen: Reinhold Henkel, Heidelberg Production: ProEdit GmbH, 69126 Heidelberg, Germany Cover: Frido-Steinen-Broo, EStudio, Calamar, Spain Typesetting: K. Detzner, 67346 Speyer, Germany Printed on acid-free paper 21/3151 ML 543210
    • PrefaceDuring the past two decades major advances in prenatal quired conditions in infants and children. The book isdiagnosis, imaging, resuscitation, intensive care, mini- intended for trainees in paediatric surgery, establishedmally invasive surgery and operative techniques have paediatric surgeons, paediatric urologists and generalradically altered the management of infants and chil- surgeons with an interest in paediatric surgery. It is ourdren with surgical conditions. There are now several ex- sincere hope that the readers will find this volume a use-cellent paediatric surgery texts available which focus on ful reference in the operative management of childhoodthe historical background, embryogenesis, pathophy- surgical disorders.siology, diagnosis and management of childhood surgi- We wish to thank all the contributors most sincerelycal disorders. The main aim of this new textbook on for their outstanding work in producing this innovativepaediatric surgery was to provide a comprehensive de- textbook. We are indebted to Reinhold Henkel for hisscription of operative techniques for various conditions excellent artwork. We wish to express our gratitude toin children. The book contains contributions by out- Karen Alfred, Louise McCrossan (Dublin) and Gudrunstanding and well-known paediatric surgeons and Raber (Graz) for their skilful secretarial help. Finally wepaediatric urologists from five continents. Each contrib- wish to thank the editorial staff of Springer, particular-utor was selected to provide an authoritative, compre- ly Gabriele Schroeder, who has been behind each step ofhensive and complete account of their respective topic. this book, from its original concept to its delivery.The text is organised in a systematic manner, providingstep-by-step, detailed practical advice on the operative Prem Puriapproach in the management of congenital and ac- Michael Höllwarth
    • SPRINGER SURGERY ATLAS SERIES Series Editors: J. S. P. Lumley · J. R. Siewert
    • Contents PART I HEAD and NECK Chapter 14 Extracorporeal Membrane Oxygenation . . . . . . . . . . . 125 Jason S. Frischer, Charles J.H. StolarChapter 1 Thyroglossal Duct Cyst . . . . . 3 Michael E. HöllwarthChapter 2 Branchial Cysts and Sinus . . . . 7 PART IV ABDOMEN Michael E. HöllwarthChapter 3 Cystic Hygroma . . . . . . . . . . 13 Chapter 15 Hernias – Inguinal, Umbilical, Baird M. Smith, Craig T. Albanese Epigastric, FemoralChapter 4 Tracheostomy . . . . . . . . . . 19 and Hydrocele . . . . . . . . . . 139 Thom E. Lobe Juan A. Tovar Chapter 16 Omphalocele . . . . . . . . . . . 153 Stig Somme, Jacob C. Langer PART II OESOPHAGUS Chapter 17 Gastroschisis . . . . . . . . . . . 161 Marshall Z. SchwartzChapter 5 Oesophageal Atresia . . . . . . 29 Chapter 18 Hypertrophic Pyloric Stenosis . 171 Michael E. Höllwarth, Paola Zaupa Takao FujimotoChapter 6 Gastro-oesophageal Reflux Chapter 19 Gastrostomy . . . . . . . . . . . 181 and Hiatus Hernia . . . . . . . . 49 Michael W. L. Gauderer Keith E. Georgeson Chapter 20 Malrotation . . . . . . . . . . . . 197Chapter 7 Achalasia . . . . . . . . . . . . . 61 Agostino Pierro, Evelyn GP Ong Paul K. H. Tam Chapter 21 Duodenal Obstruction . . . . . 203Chapter 8 Colonic Replacement Yechiel Sweed of the Oesophagus . . . . . . . . 67 Alaa Hamza Chapter 22 Jejuno-ileal Atresia . . . . . . . 213 Heinz Rode, Alastair J. W. MillarChapter 9 Gastric Transposition for Oesophageal Replacement . 77 Chapter 23 Meconium Ileus . . . . . . . . . 229 Lewis Spitz Massimo Rivosecchi Chapter 24 Gastrointestinal Duplications . 239 Mark D. Stringer PART III CHEST Chapter 25 Short Bowel Syndrome . . . . . 257 Michael E. HöllwarthChapter 10 Thoracoscopy . . . . . . . . . . . 89 Klaas Bax Chapter 26 Hirschsprung’s Disease . . . . . 275 Prem PuriChapter 11 Repair of Pectus Excavatum . . 97 Robert C. Shamberger Chapter 27 Anorectal Anomalies . . . . . . 289 Alberto Peña, Marc A. LevittChapter 12 Pulmonary Malformations . . . 107 Brian T. Sweeney, Keith T. Oldham Chapter 28 Intussusception . . . . . . . . . 313 Karl-Ludwig WaagChapter 13 Congenital Diaphragmatic Hernia and Eventration . . . . . . . . . 115 Chapter 29 Appendectomy . . . . . . . . . . 321 Prem Puri Vincenzo Jasonni
    • ContentsVIII Chapter 30 Omphalomesenteric Chapter 44 Liver Tumours   .  .  .  .  .  .  .  .  .  . 459 Duct Remnants . . . . . . . . . . 327 Wendy T. Su, Michael P. La Quaglia David Lloyd Chapter 45 Testicular Tumours   .  .  .  .  .  .  . 477 Chapter 31 Ulcerative Colitis . . . . . . . . . 333 Jonathan Ross Risto J. Rintala Chapter 32 Crohn’s Disease . . . . . . . . . . 347 Risto J. Rintala PART VIII UROLOGY Chapter 46 Pyeloplasty   .  .  .  .  .  .  .  .  .  .  . 485 PART V LIVER, PANCREAS AND SPLEEN Boris Chertin, Prem Puri Chapter 47 Endoscopic Treatment Chapter 33 Biliary Atresia . . . . . . . . . . . 357 of Vesicoureteral Reflux   .  .  .  . 493 Ryoji Ohi, Masaki Nio Prem Puri Chapter 34 Choledochal Cyst . . . . . . . . . 371 Chapter 48 Vesicoureteral Reflux – Takeshi Miyano, Masahiko Urao, Surgical Treatment   .  .  .  .  .  .  . 499 Atsuyuki Yamataka Jack S. Elder Chapter 35 Cholecystectomy . . . . . . . . . 387 Chapter 49 Ureteric Duplication   .  .  .  .  .  . 515 Thom E. Lobe Claude C. Schulman Chapter 36 Surgery for Persistent Chapter 50 Posterior Urethral Valves   .  .  .  . 523 Hyperinsulinaemic Chester J. Koh, David A. Diamond Hypoglycaemia of Infancy . . . 395 Chapter 51 Hypospadias   .  .  .  .  .  .  .  .  .  . 529 Lewis Spitz Pierre Mouriquand, Chapter 37 Splenectomy . . . . . . . . . . . 403 Pierre-Yves Mure Peter Borzi Chapter 52 Phimosis and Buried Penis   .  .  . 543 Peter Cuckow Chapter 53 Orchidopexy   .  .  .  .  .  .  .  .  .  . 555 PART VI SPINA BIFIDA John M. Hutson AND HYDROCEPHALUS Chapter 54 Variocele   .  .  .  .  .  .  .  .  .  .  .  . 569 Michael E. Höllwarth Chapter 38 Spina Bifida . . . . . . . . . . . . 413 Martin T. Corbally Chapter 55 Genitoplasty for Congenital Adrenal Hyperplasia   .  .  .  .  .  . 577 Chapter 39 Hydrocephalus . . . . . . . . . . 419 Amicur Farkas Kai Arnell, Leif Olsen, Tomas Wester Chapter 56 Bladder Exstrophy and Epispadias 589 Dominic Frimberger, Chapter 40 Dermal Sinus . . . . . . . . . . . 427 John P. Gearhart Andrew B. Pinter Chapter 57 Cloacal Exstrophy  .  .  .  .  .  .  .  . 607 Duncan Wilcox, Manoj Shenoy PART VII TUMOURS Chapter 58 Augmentation Cystoplasty and Appendicovesicostomy Chapter 41 Sacrococcygeal Teratoma . . . . 435 (Mitrofanoff Principle)   .  .  .  .  . 613 Kevin C. Pringle Boris Chertin Chapter 42 Neuroblastoma . . . . . . . . . . 443 Chapter 59 The ACE (Antegrade Continence Edward Kiely Enema) Procedure   .  .  .  .  .  .  . 623 Chapter 43 Wilms Tumour . . . . . . . . . . 451 Robert Carachi
    • List of ContributorsCraig T Albanese MD Martin T Corbally MCh, FRCSI, FRCSProfessor of Surgery Consultant Paediatric SurgeonChief, Division of Pediatric Surgery Our Lady’s Hospital for Sick ChildrenStanford University Medical Center CrumlinPalo Alto, Calfornia Dublin 12USA IrelandKai Arnell MD Peter M Cuckow FRCSDepartment of Paediatric Surgery Consultant Paediatric UrologistUniversity Children’s Hospital Great Ormond Street Hospital for Sick ChildrenSE-751 85 Uppsala 30 Guilford StreetSweden London WC1N 1EH UKKlass MA Bax MD, PhD, FRCS (Ed)Professor of Pediatric Surgery David A Diamond MDWilhelmina Children’s Hospital Associate Professor of Surgery (Urology)University Medical Center Utrecht Children’s Hospital BostonPO Box 85090, 3508 AB Utrecht and Harvard Medical SchoolThe Netherlands 300 Longwood Avenue, Hunnewell 3 Boston, MA 02115Peter Borzi MB, BS, FRACS, FRCS USAPaediatric Surgery & Paediatric UrologyTaylor Medical Centre Jack S Elder MD40 Annerley Road DirectorWoolloongabba 4102 Division of Pediatric UrologyAustralia Rainbow Babies & Children’s Hospital 11100 Euclid AvenueRobert Carachi MD, FRCS Cleveland, OH 44106Professor of Paediatric Surgery USAHead of DepartmentDepartment of Surgical Paediatrics Amicur Farkus MDRoyal Hospital for Sick Children Professor and HeadYorkhill, Glasgow G2 8SJ Department of UrologyUK Shaare Zedek Medical Center Jerusalem, Israel 91031Boris Chertin MDConsultant Pediatric Urologist Dominic Frimberger MDDepartment of Urology Johns Hopkins HospitalShaare Zedek Medical Center Urology, Marburg 149Jerusalem, Israel, 91031 600N Wolfe St Baltimore, MD 21287 USA
    • List of ContributorsX Takao Fujimoto MD, PhD Vincenzo Jasonni MD Director of Pediatric Surgery Professor and Director Imperial Gift Foundation Universita degli Studi di Genova The Aiiku Maternal & Children’s Medical Centre Largo Gerolamo Gaslini 5 5-6-8 Minami-Azabu, Minato-Ku 16147 Genova Tokyo 106-8580 Italy Japan Edward Kiely FRCSI, FRCS, FRCPCH Michael W L Gauderer MD, FACS, FAAP Consultant Paediatric Surgeon Professor, Department of Pediatric Surgery 234 Great Portland Street Children’s Hospital London W1W 5QT Memorial Medical Office Building, Suite 440 UK 890 West Fans Road Greenville, South Carolina 29605-4253 Chester J Koh MD USA Fellow in Pediatric Urology Children’s Hospital Boston John P Gearhart MD and Harvard Medical School Professor & Director 300 Longwood Avenue, Hunnewell 3 Division of Pediatric Urology Boston, MA 02115 James Buchanan Brady Urological Institute USA Johns Hopkins Hospital Baltimore, Maryland Jacob C Langer MD USA Professor, Chief of Paediatric General Surgery Hospital for Sick Children Keith E Georgeson MD Rm 1526, 555 University Ave Professor and Director Toronto, ON M5G 1X8 Division of Pediatric Surgery Canada Children’s Hospital and Alabama 1600 Seventh Avenue South Michael P La Quaglia MD Birmingham, Alabama 35233 Department of Surgery USA Memorial Sloan-Kettering Cancer Center 1275 York Ave. Alaa F Hamza MD, FRCS New York, NY 10021 Consultant Paediatric Surgeon USA 45 Ramsis Street 11341 Heliopolis Marc A Levitt MD Cairo Assistant Professor of Surgery and Pediatrics Egypt Schneider Children’s Hospital North Shore-Long Island Jewish Health System Michael E Höllwarth MD 269-01 76th Avenue Professor & Head New Hyde Park, NY 11040 Department of Paediatric Surgery USA Medical University of Graz Auenbruggerplatz David A Lloyd Mchir, FRCS, FCS(SA) A-8036 Graz Professor of Paediatric Surgery Austria 15 Eshe Road North Blundellsands John M Hutson BS, MD(Monash), MD(Melb), FRACS Liverpool L23 8UE Professor & Director UK General Surgery Royal Children’s Hospital Thom E Lobe MD Parkville, Victoria 3052 Chairman, Section of Pediatric Surgery Australia Blank Childrens Hospital Des Moines Iowa USA
    • List of Contributors XIPadraig S J Malone MCh, FRCSI, FRCS Keith Oldham MDConsultant Paediatric Urologist Division of Pediatric SurgeryDepartment of Paediatric Urology Medical College of WisconsinSouthampton University Hospitals NHS Trust Children’s Hospital Office BuildingTremona Road 9000 West Wisconsin AvSouthampton S016 6YD Milwaukee, Wisconsin 53201Hampshire, UK USAAlastair J W Millar FRCS(Eng) (Edin), FRACS, DCH Leif Olsen MD, PhDConsultant Paediatric Surgeon Department of Paediatric SurgeryDepartment of Paediatric Surgery University Children’s HospitalBirmingham Childres Hospital SE-751 85 UppsalaBirmingham SwedenUK Evelyn G P Ong MBBS, BSc, FRCS (Eng)Takeshi Miyano MD, PhD, FAAP(Hon), FACS, Clinical Research FellowFAPSA (Hon) Paediatric Surgery UnitProfessor and Head Institute of Child Health & Great Ormond StreetDepartment of Pediatric Surgery Hospital for ChildrenJuntendo University School of Medicine 30 Guilford Street2-1-1 Hongo, Bunkyo-ku London WC1N 1EHTokyo 113-8421 UKJapan Alberto Pena MDPierre Mouriquand MD, FRCS(Eng), FEBU Cincinnati Children’s Hospital Medical CenterProfessor, Service d’Urologie Pediatrique CincinnatiHopital Debrousse USA29, rue Soeur Bouvier69322 Lyon Cedex 05 Agostino Pierro MD, FRCS (Eng), FRCS (Ed), FAAPFrance Professor, Department of Paediatric Surgery Institute of Child Health & Great Ormond StreetPierre-Yves Mure Hospital for ChildrenService d’Urologie Pediatrique 30 Guilford StreetHopital Debrousse London WC1N 1EH29, rue Soeur Bouvier UK69322 Lyon Cedes 05France Andrew B Pinter Professor of Paediatric SurgeryMasaki Nio MD Department of Paediatrics/Surgical UnitDepartment of Pediatric Surgery Jozsef A. u. 7., 7623Tohoku University School of Medicine PecsSendai, 980 HungaryJapan Kevin C Pringle MB, ChB, FRACSRyoji Ohi MD O&G Health of DepartmentProfessor, Department of Pediatric Surgery Capital Coast HealthTohoku University School of Medicine Private Bag 8902Sendai, 980 Riddiford StreetJapan Wellington South, New Zealand
    • List of ContributorsXII Prem Puri MS, FRCS, FRCS (Ed), FACS Robert C Shamberger MD Consultant Paediatric Surgeon Department of Surgery Professor & Director of Research Children’s Hospital Boston Children’s Research Centre 300 Longwood Avenue Our Lady’s Hospital for Sick Children Boston, Massachusetts 02115 Crumlin USA Dublin 12, Ireland Manoj Shenoy FRCS Risto J Rintala MD Consultant Paediatric Urologist Professor, Department of Paediatric Surgery City Hospital Hospital for Children and Adolescents Nottingham University of Helskinki UK PO Box 281 Fin-00029 Hus Baird M Smith MD Finland Assistant Professor of Surgery Division of Pediatric Surgery Massimo Rivosecchi MD Stanford University Professor, Department of Pediatric Surgery Palo Alto, California Bambino Gesu’ Children’s Hospital USA Palidoro Rome Stig Somme MD Italy Research Fellow Department of Surgery Heinz Rode Mmed(Chir), FC(SA), FRCSEd Hospital for Sick Children Professor of Paediatric Surgery 555 University Avenue Red Cross Children’s Hospital Toronto, ON M5G 1X8 Rondebosch 7700 Canada South Africa Lewis Spitz MB, ChB, PhD, MD(Hon), FRCS(Edin), Jonathan Ross MD FRCS(Eng) Head, Section of Pediatric Urology Nuffield Professor of Paediatric Surgery Glickman Urological Institute Institute of Child Health Cleveland Clinic Children’s Hospital 30 Guilford Street 9500 Euclid Avenue London WC1N 1EH Cleveland, OH 44195 UK USA Charles J H Stolar MD Claude C Schulman MD, PhD Children’s Hospital of New York Professor 3959 Broadway, 202N Hospital Erasure New York, NY 10032 Route de Lennik 808 USA 1070 Bruxelles Belgium Mark D Stringer BSc, MS, FRCS FRCS(Paed), FRCP, FRCPCH Marshall Z Schwartz MD Consultant Paediatric Surgeon St. Christopher’s Hospital for Children Children’s Liver & GI Unit Department of Surgery Gledhow Wing Erie Avenue at Front Street St James’s University Hospital Philadelphia, PA 19134 Leeds LS9 7TF USA UK
    • List of Contributors XIIIWendy T Su MD Karl-Ludwig Waag MDDepartment of Surgery Professor, Department of Paediatric SurgeryMemorial Sloan-Kettering Cancer Center Mannheim/Heidelberg1275 York Av. Im Neuenheimer Feld 110New York, NY 10021 D-69120 HeidelbergUSA GermanyYechiel Sweed MD Tomas Wester MD, PhDHead, Paediatric Surgery Department of Paediatric SurgeryWestern Galilee Hospital University Children’s HospitalNahariya SE-751 85 UppsalaIsrael 21/22100 SwedenBrian T Sweeney MD Duncan Wilcox MD, FRCS (Paed)Pediatric Surgery Fellow Associate ProfessorDivision of Pediatric Surgery Department of UrologyMedical College of Wisconsin University of Texas9000 W. Wisconsin Ave. South Western Medical CenterMilwaukee, WI 53226 Dallas, TexasUSA USAPaul Tam MD FRCS Atsuyuki Yamataka MDProfessor, Division of Paediatric Surgery Department of Pediatric SurgeryUniversity of Hong Kong Juntendo University School of MedicineMedical Centre 2-1-1 Hongo, Bunkyo-kuQueen Mary’s Hospital Tokyo 113-8421Pokfulam Road JapanHong Kong Paola Zaupa MDJuan A Tovar MD Department of Paediatric SurgeryProfessor, Department of Pediatric Medical University GrazSurgery Hospital Universitario “La Paz” Auenbruggerplatz 34Paseo de la Castellana 261 A-8036 Graz28046 Madrid AustriaSpainMasahiko Urao MD, PhDDepartment of Pediatric SurgeryJuntendo University, School of Medicine2-1-1 Hongo, Gunkyo-kuTokyo 113-8421Japan
    • Part I Head and Neck
    • SPRINGER SURGERY ATLAS SERIES Series Editors: J. S. P. Lumley · J. R. Siewert
    • CHAPTER 1 Thyroglossal Duct Cyst Michael E. Höllwarth INTRODUCTIONThe median cervical cyst is a remnant of the thyro- Thyroglossal cysts are the most common tumoursglossus duct, which runs from the pyramidal lobe of of the anterior cervical region. They are usually lo-the thyroid gland to the foramen caecum in the dor- cated in the midline at the level of or somewhat be-sal part of the tongue. Embryologically, the thyroid low the hyoid bone. Due to the connection with thediverticulum develops in a caudal direction from the foramen caecum of the tongue, the lesion typicallyforamen caecum after formation of the tongue. The moves upwards with swallowing like the thyroidthyroid gland descends to the neck in the same peri- gland, and, different from the latter, also with tongueod of gestation as the hyoid bone develops from the protrusion. In contrast, dermoid cysts or lymphsecond branchial arch. The thyroglossal duct may nodes do not change their position with either act.pass in front, behind or through the body of the Ultrasound examination may be helpful, in the firsthyoid bone in the middle of the neck, and islands of instance to ascertain the presence of a normally situ-thyroid tissue may be found scattered along the tract. ated normally sized thyroid gland as well as to con-At no time during embryogenesis does the thyroglos- firm the cystic nature of the mass under considera-sal duct contact the body surface; the original cysts tion. In cases of a suppurative infection, incision andthus never open to the skin. A fistula can only devel- drainage in combination with antibiotics is the ap-op secondarily, e.g., following spontaneous perfora- propriate treatment followed by excision once thetion or surgical incision of an infected cyst. acute inflammation has settled.
    • Michael E. Höllwarth 4 Figure 1.1 Figure 1.21 Following induction of general anaesthesia with en- The duct is attached to the cyst running in a cephalad dotracheal intubation, the neck is hyperextended by direction between the sternohyoid muscles to the placing a sandbag or towel roll beneath the shoul- body of the hyoid bone. It is usually not possible to ders. A horizontal skin incision is made over the cyst. recognize whether the duct perforates the hyoid In case of a fistula, the cutaneous orifice is circum- body or passes across its anterior or posterior sur- cised in a horizontally oriented elliptical fashion. face. The central part of the hyoid bone is freed from Subcutaneous tissue, platysma and cervical fascia are the muscles attached to its upper and lower margin. divided exposing the capsule of the cyst. In cases The thyrohyoid membrane is carefully dissected off with previous history of inflammation, these layers the posterior aspect with scissors. may be fibrosed and lack a clear demarcation against each other as well as against the cyst wall. The cyst is carefully separated from the surrounding tissue by blunt and sharp dissection. Figure 1.3 Figure 1.4 The exposed hyoid bone is then stabilized with If the duct is extending beyond on the posterior as- strong Kocher forceps on one side, clearly lateral to pect of the hyoid bone, it is followed cephalad and the median line, and the central segment is excised divided close to the base of the tongue with a 5/0 ab- with strong Mayo scissors. sorbable transfixation ligature. If the floor of the mouth is entered accidentally, the mucosa of the tongue is closed with interrupted plain absorbable sutures. Often, however, no duct structures are found behind the hyoid bone, in which case some of the midline connective tissue is excised in the cranial di- rection to make sure that no duct epithelium is left behind. The lateral segments of the hyoid bone are left separated, but the anterior neck muscles are approx- imated in the midline with absorbable 4/0 sutures. Platysma and subcutaneous fat are closed with ab- sorbable 5/0 sutures, and the skin is closed either with interrupted subcuticular absorbable 6/0 stitch- es or with a continuous subcuticular nonabsorbable 4/0 suture, which can be removed 3–4 days later. A drain is usually not necessary, except in cases requir- ing extensive dissection as may occur after a previ- ously infected cyst or a recurrent cyst.
    • Chapter 1 Thyroglossal Duct Cyst 5 Figure 1.1 Figure 1.2 Figure 1.3 Figure 1.4
    • Michael E. Höllwarth 6 CONCLUSION1 Complete excision of the thyroglossal cyst consists of in native tissue, dissection is much more difficult in a removal of the cyst, the entire tract and the midpor- previously infected cyst. Therefore, postponement of tion of the hyoid bone through which the tract pass- the surgical procedure is not to be recommended es. If this principle is followed, recurrence is extreme- once the diagnosis has been made. ly unlikely. While the procedure is easily performed SELECTED BIBLIOGRAPHY Horisawa M, Niiomi N, Ito T (1991) Anatomical reconstruction Telander RL, Deane S (1977) Thyroglossal and branchial cleft of the thyroglossal duct. J Pediatr Surg 26 : 766–769 cysts and sinuses. Surg Clin North Am 57 : 779–791 Smith CD (1998) Cysts and sinuses of the neck. In: O’Neill JA, Waldhausen JHT, Tapper D (2000) Head and neck sinuses and Rowe MI, Grosfeld JL, Fonkalsrud EW, Coran AG (eds) Pe- masses. In: Ashcraft KW (ed) Pediatric surgery. WB diatric surgery. Mosby, St Louis, pp 757–772 Saunders, Philadelphia, pp 987–999
    • CHAPTER 2 Branchial Cysts and Sinus Michael E. Höllwarth INTRODUCTIONDuring the fourth to eighth week of gestation, four sillar fossa. A complete sinus may discharge clearpairs of branchial arches and their intervening clefts saliva. A cyst, as a remnant of the second branchialand pouches are formed. Congenital branchial cysts pouch, presents as a soft mass deep to the upper-and sinus are remnants of these embryonic struc- third of the sternocleidomastoid muscle. The depthtures that have failed to regress completely. Treat- distinguishes it from cystic hygromas, which are lo-ment of branchial remnants requires knowledge of cated in the subcutaneous plane.the related embryology. The first arch, cleft and The third arch forms the inferior parathyroidpouch form the mandible, the maxillary process of glands and the thymus, while the fourth arch mi-the upper jaw, the external ear, parts of the Eusta- grates less far down and develops into the superiorchian tube, and the tympanic cavity. Anomalies of parathyroid glands. Sinuses of the third arch openthe first branchial pouch are rare. Sinuses typically externally in the same region as those of the secondhave their external orifice inferior to the ramus of the one, but run upwards behind the carotid artery to themandible. They may traverse the parotid gland, and piriform fossa. Cystic remnants may compress therun in close vicinity to the facial nerve in the external trachea and cause stridor. Sinuses and cysts of theauditory canal. Cysts are located anterior or posteri- fourth branchial arch and cleft are extremely rare.or to the ear or in the submandibular region. They Both, third and fourth arch remnants most common-have to be distinguished from the preauricular cysts ly present as inflammatory lateral neck masses, moreand sinuses, which are ectodermal remnants from an often on the left side. The cyst may evoke a false im-aberrant development of the auditory tubercles, tend pression of acute thyroiditis. Computed tomographyto be bilateral, and are localized anterior to the tragus (CT) of the neck helps to identify the origin of suchof the ear. Sinuses are blind, ending in close vicinity lesions. In an acute suppurative phase, external pres-of the external auditory meatus. sure onto the mass may result in laryngoscopically The most common branchial cysts and sinus de- visible evacuation of pus into the piriform fossa.rive from the second branchial pouch, which forms Cystic remnants present commonly in adoles-the tonsillar fossa and the palatine tonsils. The exter- cence and adulthood, whereas sinuses and fistulasnal orifice of the sinus can be located anywhere along are usually seen in infancy and early childhood. Inthe middle- to lower-third of the anterior border of principle, clinical manifestation – no matter at whatthe sternocleidomastoid muscle. The sinus pene- age – should be taken as an indication for electivetrates the platysma and runs parallel to the common excision before complications – mainly of an inflam-carotid artery, crosses through its bifurcation and matory nature – supervene.most commonly exits internally in the posterior ton-
    • Michael E. Höllwarth 8 Figure 2.1 The patient is placed in a supine position. Following fected side. Instillation of Methylene blue into the or-2 induction of general anaesthesia with endotracheal ifice aids identification of the sinus during dissec- intubation, the head is turned to the side. A sandbag tion. Some surgeons introduce a lacrymal duct probe is placed underneath the shoulders to expose the af- into the orifice to guide dissection of the tract. Figure 2.2 In case of branchial cyst the incision is made over the to it just underneath the skin for manipulation dur- cyst along the Langer’s lines. An elliptical incision is ing further dissection. made around the sinus. A traction suture is applied
    • Chapter 2 Branchial Cysts and Sinus 9 Figure 2.1 Figure 2.2 Hypoglossal nerve Carotid bifurcation
    • Michael E. Höllwarth 10 Figure 2.3 Figure 2.4 Subcutaneous tissue and platysma are divided until In adolescents a second transverse (stepladder) inci-2 the sinus tract is reached, which is easily palpable sion, made approximately 4–5 cm above the first, may when the traction suture is gently tensed. Mobiliza- be necessary to completely excise the sinus tract. tion of the sinus continues in cephalad direction as Both incisions are closed with absorbable interrupt- far as possible with gentle traction. The operation ed fine subcutaneous (5/0) and subcuticular (6/0) su- can usually be done through a single elliptical inci- tures. sion by keeping traction on the sinus tract and by the anaesthetist placing a gloved finger to push the ton- sillar fossa downwards. Dissection then continues through the carotid bifurcation to the tonsillar fossa. Close contact with the sinus is obligatory to avoid any injury to the arteries or the hypoglossal nerve. Close to the tonsillar fossa, the sinus is ligated with a 5/0 absorbable transfixation suture and divided. Figure 2.5 For the first branchial pouch remnants, the opening of the fascial nerve, dissection must stay close to the of the fistula is circumcised with an elliptical skin in- tract, and – exclusively bipolar – electrocoagulation cision. Careful dissection liberates the subcutaneous must be used sparingly. A neurosurgical nerve stim- segment of the embryological remnant, which is now ulator may be employed to identify and preserve fine transfixed with a stay suture. This is used for traction nerve fibres. The sinus is transected and ligated with on the duct, which facilitates its identification on an absorbable 5/0 stitch close to the auditory canal. subsequent dissection into the depth towards the au- The subcutaneous tissue is approximated using 5/0 ditory canal. Because of intimate contact with the pa- absorbable sutures, followed by interrupted subcut- rotid gland and potentially in the immediate vicinity icular absorbable 6/0 sutures.
    • Chapter 2 Branchial Cysts and Sinus 11 Figure 2.3 Figure 2.4 Figure 2.5 Facial nerve
    • Michael E. Höllwarth 12 CONCLUSION Recurrences are most likely due to proliferation of tion of the tissue layers much more difficult. Surgery2 residual epithelium from cysts or sinuses. The surgi- after infections of remnants of the first branchial cal procedure should thus be performed electively pouch carries an increased risk of facial nerve injury. soon after diagnosis. Infected cysts and sinuses are In order to avoid damage to vital vascular and nerve treated with antibiotics until the inflammatory signs structures it is important to confine dissection close subside, unless abscess formation mandates incision to the sinus tract. and drainage. Repeated infections render identifica- SELECTED BIBLIOGRAPHY Deane SA, Telander RL (1978) Surgery for thyroglossal duct Waldhausen JH, Tapper D (2000) Head and neck sinuses and and branchial cleft anomalies. Am J Surg 136 : 348–353 masses. In: Ashcraft (ed) Pediatric surgery. WB Saunders, Smith CD (1998) Cysts and sinuses of the neck. In: O’Neill JA, Philadelphia, pp 787–799 Rowe MI, Grosfeld JL, Fonkalsrud EW, Coran AG (eds) Pe- diatric surgery. Mosby, St Louis, pp 757–772
    • CHAPTER 3 Cystic Hygroma Baird M. Smith, Craig T. Albanese INTRODUCTIONLymphangiomas are benign masses with multinodu- and investigation. Some regions tend to have typicallar cysts of different sizes and contents. Microcysts lesions: for example, reddish lesions in the base of theare less than 1 cm in diameter; macrocysts are great- tongue are typically microcystic with a significanter than 1 cm in diameter and tend to be less invasive, vascular component; soft boggy masses in the super-less numerous, and less difficult to remove. Both mi- ficial neck or axilla – sometimes with a bluish hue –crocysts and macrocysts may contain blood and/or are often macrocysts with lymph. The best investi-lymph, a consequence of similar lymphatic and vas- gations to determine cyst contents is either a T2-cular embryology. In general, microcysts are more weighted gadolinium-enhanced magnetic resonancelikely to contain blood and macrocysts more likely to imaging (MRI) or needle aspiration of the dominantcontain lymph. Macrocysts that contain lymph are al- cyst. Lymph is straw-coloured; thin bloody fluid mayso called cystic hygromas and they are subsumed in occur when a lymphatic cyst is enlarged by a rup-the general category of lymphatic malformations. tured blood vessel. Abundant dark or red blood indi- The risks of expectant management include infec- cates a significant vascular component. Viscid yel-tion, progressive growth and disfigurement, exten- low-clear fluid from an intra-oral lesion may signal asion into previously uninvolved areas, dysphagia, air- ranula, deriving from salivary tissue. Depth of inva-way compromise, and erosion into vascular struc- sion and an estimate of the structures involved is besttures. Asymptomatic cysts in the premature or small- determined by MRI scanning. Rarely, a neck lesionfor-dates child may await growth and development of may extend to the anterior mediastinum and com-the infant. For the majority of patients there is no press the trachea. Spontaneous enlargement may oc-need to defer excision. cur following an upper respiratory tract infection; The determination of a lymphangioma’s size and spontaneous regression is rare although sometimescharacter is based on location, clinical examination follows local infection
    • Baird M. Smith, Craig T. Albanese 14 Figure 3.1 Figure 3.2 General anaesthesia is used and blood made avail- If the lymphangioma demonstrates dermal infiltra- able if the lesion appears vascular on pre-operative tion, an ellipse of skin is removed. Otherwise, gener- screening. If lesions are close to important motor ous sub-platysmal skin flaps are raised. The external nerves, one may use a nerve stimulator and interdict jugular vein and ansa cervicalis are not considered3 use of musculoskeletal blocking agents. essential and may be sacrificed. Pre-operative planning will usually demonstrate a safe plane of attack and may set expectations with re- gard to a complete excision or a debulking operation. Loupe magnification is often helpful, as is a bipolar cautery when working close to nerves or vital struc- tures. Microvascular lesions tend to infiltrate tissue planes, are more likely to bleed and have a high rate of recurrence. Macrocystic lesions tend to spread along fascial planes and around neurovascular struc- tures. Intra-operative rupture decreases the likeli- hood of complete resection, which averages 50%.Any residual cystic tissue will increase the likelihood of recurrence. Because this is not a malignant lesion, it is seldom necessary to sacrifice essential local struc- tures. It is commonly necessary to place a closed suc- tion drain, particularly when the lesion is incom- pletely excised. For the most common (cervical) le- sions, a transverse skin crease incision extending the length of the mass is placed in Langer’s lines. A first- generation cephalosporin is used peri-operatively. Figure 3.3 Figure 3.4 Dissection of cervical lesions begins at the superior The dissection proceeds medially, lifting the cyst margin of the mass, near the ramus of the mandible. from the surrounding alveolar tissue. Upward reflection of the facial artery and vein allow It may be necessary to divide the middle thyroid the precise visualization necessary to preserve the vein and artery as the carotid sheath is approached. marginal branch of the facial nerve. Bipolar cautery Deep dissection frequently involves the contents of may be used and optical magnification is often help- the carotid sheath and sometimes the following ful. nerves: vagus, spinal accessory, hypoglossal, sympa- thetic trunk, phrenic and the brachial plexus.
    • Chapter 3 Cystic Hygroma 15 Figure 3.1 Figure 3.2 Figure 3.3 Figure 3.4 Mandibular branch of the facial nerve Facial artery and vein Vagus nerve
    • Baird M. Smith, Craig T. Albanese 16 Figure 3.5 Care is taken to preserve the hypoglossal nerve as it terior triangle or the spinal accessory nerve as it passes through the bifurcation of the carotid artery. courses through the posterior triangle. Extension of The mass must then be freed from the hyoid bone the lymphangioma under the clavicle may lead to and submandibular gland. It is rarely necessary to re- axillary or mediastinal involvement (requiring ster-3 move the submandibular gland en bloc with the notomy if the lesion proceeds deeply). Combined mass, sacrificing the facial artery. The mass may be masses may be delivered either above or below the adherent to the brachial plexus in the floor of the an- clavicle. Figure 3.6 The platysma is re-approximated with fine absorb- sutures of similar material. Closed suction drainage able sutures and the skin closed with subcuticular is used for most lesions.
    • Chapter 3 Cystic Hygroma 17 Figure 3.5 Figure 3.6
    • Baird M. Smith, Craig T. Albanese 18 CONCLUSION Feeding resumes when the infant is awake and alert. Excision is the current gold-standard therapy. Extensive intra-oral dissection may temporarily im- There are several reports of successful use of scleros- pair swallowing and delay the onset of oral feeds. ing agents such as OK-432 or bleomycin in lymph- Drain removal may take days or weeks and is dictat- angiomas. This appears to be effective mainly in3 ed by the daily drainage volume. Antibiotics are ad- macrocystic lesions. ministered daily from 1 to 3 days. An exciting advance in the management of fetuses In cases of partial resection, recurrence typically with a high probability of upper-airway obstruction occurs within a year of surgery. Lymph leaks and at birth due to a giant cervical lymphangioma, is the nerve injuries are minimized by the use of bipolar di- development of the ex utero intrapartum treatment athermy. Rarely, lymph leaks may require re-explora- (EXIT). tion when drains are inadequate or removed early. SELECTED BIBLIOGRAPHY Banieghbal B, Davies MR (2003) Guidelines for the successful Hirose S, Farmer DL, Lee H, Nobuhara KK, Harrison MR treatment of lymphangioma with OK-432. Eur J Paediatr (2004) The exutero intrapartum treatment procedure: Surg 13 : 103–107 Looking back at the EXIT. J Pediatr Surg 39: 375–380 Bouchard S, Johnson MP, Flake AW, Howell LJ, Myers LB, Ad- Schuster T, Grantzow R, Nicolai T (2003) Lymphangioma coli: zick NS, Crombleholme TM (2002) The EXIT procedure: a new classification contributing to prognosis. Eur J Paedi- experience and outcome in 31 cases. J Pediatr Surg atr Surg 13 : 97–102 37 : 418–426 Charabi B, Bretlau P, Bille M, Holmelund M(2000) Cystic hy- groma of the head and neck – long-term follow up of 44 cases. Acta Otolaryngol Suppl 543 : 248–250
    • CHAPTER 4 Tracheostomy Thom E. Lobe INTRODUCTIONThe indications for tracheostomy in infants and chil- There are several acquired conditions that requiredren fall into five main categories: airway immatur- tracheostomy. Among them are infection, neuromus-ity, obstructing congenital anomalies, acquired ob- cular failure, chronic aspiration and subglottic steno-structions, tumours and trauma. sis. Chronic respiratory failure, sleep apnea or neuro- The immature airway manifests itself as laryn- motor problems resulting in poor airway mainte-gomalacia, tracheomalacia or a combination of the nance also require tracheostomy. Long-term respira-two conditions. These infants present with inspirato- tory support after major surgery, repair of laryngo-ry stridor, and some degree of nasal flaring and chest tracheo-oesophageal cleft or major trauma may ne-retractions. Other related conditions are congenital cessitate tracheostomy.vocal chord paralysis, which is usually due to a cen- Occasionally the management of a tumour such astral nervous system deficit, phrenic nerve injury, a cervical teratoma or sarcoma in infancy will man-which may be associated with a difficult delivery, and date a tracheostomy. More likely, a hemangioma orrecurrent laryngeal nerve injury, which may occur lymphangioma will compromise the airway to theafter ligation of a patent ductus arteriosus. extent that a more stable airway is needed. Some patients with choanal atresia and Pierre Tracheostomy in infants and children routinely isRobin syndrome or other craniofacial abnormalities performed under general anaesthesia with the pa-may be candidates for tracheostomy. tient intubated unless the patient’s condition is so Patients with a congenitally stenotic airway or tra- unstable that the patient cannot tolerate the neces-cheal agenesis are special cases. In the case of agene- sary drugs.sis, an emergency tracheostomy may be necessarywhere the trachea reestablishes distally.
    • Thom E. Lobe 20 Figure 4.1 The patient is placed supine on the operating table The endotracheal tube should be secured so that the toward the head of the table so that the surgeon can anaesthesiologist can easily remove the tube at the access the patient’s neck easily, but not so far down appropriate time. This means that any tape should be on the table that the anaesthesiologist cannot reach loosened before hand. If there is a feeding tube in the patient to manipulate the endotracheal tube place, it should be removed so that it does not inter- when required. The anaesthesiologist or anaesthetist fere with endotracheal tube manipulation. When the4 must be able to maintain control of the airway while infant is properly positioned and monitored, the en- the surgeon is exposing and manipulating the tra- tire neck from the lower lip to below the nipples chea. The neck should be extended sufficiently to al- should be prepped with a suitable surgical prep and low complete access to the neck. Sometimes, on chub- draped. The superior most surgical drape should al- by infants, it is still difficult to see the entire neck, de- low easy access to the patient by the anaesthesiolo- spite the best attempts. A roll should be placed under gist. the infant’s shoulders to facilitate proper positioning. Figure 4.2 Incision is made in the lower neck crease, about the will end up too low in the trachea. We first score the width of one finger above the jugular notch. A trans- skin with a scalpel, then use a needle-point electro- verse incision is preferable. If the incision is too low cautery device to deepen the incision, taking care not you will end up in the mediastinum and the cannula to burn the skin.
    • Chapter 4 Tracheostomy 21 Figure 4.1 Figure 4.2
    • Thom E. Lobe 22 Figure 4.3 This incision is extended through the subcutaneous ter a few small vessels that cross the midline. These fascia and platysma muscle, which is quite thin in the should be cauterized and divided as they are encoun- small infant. It is helpful to insert two right-angled tered. retractors in the corners of this incision to better ex- Once these muscles are separated, we place the pose the operative site. two retractors deep to the muscle edges and gently Next, we use two atraumatic forceps to grasp the retract laterally to better expose the trachea below.4 cervical fascia on either side of the midline and open Sometimes it is necessary to free the muscle edges it vertically in the midline.We extend this incision in- sufficiently to allow room for the blade of the retrac- feriorly to the jugular notch and superiorly to the tor to gain a secure purchase. thyroid gland. The trachea should be visualized easily. If not, The strap muscles, immediately beneath the ante- then palpation in the wound with manipulation of rior cervical fascia similarly are separated in the the endotracheal tube by the anaesthesiologist will midline. Usually, there are few to no blood vessels in help locate the trachea. the dissection thus far. Occasionally, you will encoun- Figure 4.4 The proposed tracheostomy cannula should be se- midline scored anterior trachea. Each suture incor- lected, opened and its outer diameter visually porates one or two tracheal rings. These sutures are checked against the exposed trachea to judge the cor- not tied onto the tracheal wall, but can be tied at their rectness of its size. If it seems that the initial selection ends and should be left 6–8 cm in length. At the end was incorrect, then a tracheostomy cannula of a more of the case, these sutures will be taped securely to the appropriate size should be selected. anterior chest wall and will be used to locate the tra- The pre-tracheal fascia should be scored with the cheal incision in the event of a post-operative emer- cautery to coagulate any tiny vessels on the surface of gency in which the newly placed tracheostomy can- the trachea in the midline.Again, the blades of the re- nula dislodges. These sutures also can be used to hold tractors should be deep in the wound on either side open the edges of the tracheal incision for ease of of the trachea for optimal exposure. placement of the tracheostomy cannula at operation. A suture of 4/0 monofilament nonabsorbable su- ture or its equivalent is placed on either side of the
    • Chapter 4 Tracheostomy 23 Figure 4.3 Figure 4.4
    • Thom E. Lobe 24 Figure 4.5 The surgeon should request that the endotracheal clear the lumen so that the tracheostomy cannula can tube be loosened and prepared for removal. Using a be inserted and directed caudally toward the carina. number 11 blade, a vertical incision is made through One way to avoid misplacement is to insert a suc- the tracheal wall along the score mark. Two or three tion catheter through the lumen, beyond the tip of tracheal rings should be divided. Usually these are the cannula. The suction catheter then can be insert- rings 2, 3 and 4. Rarely, it is necessary to divide the ed into the tracheal lumen first and serve as a guide4 isthmus of the thyroid gland for proper tracheosto- over which the cannula can be passed. This technique my positioning. A transverse tracheal incision or re- also is useful should the cannula become dislodged moval of a tracheal ring is likely to result in a tracheal after the procedure. deformity and thus should be avoided. If, for any reason, the tracheostomy cannula does Suction should be available in case blood or secre- not fit easily into the trachea, it should be removed tions interfere with the surgeon’s view of the tracheal and the endotracheal tube should be advanced be- lumen. The tip of the cannula to be inserted should yond the tracheal incision so that ventilation will not be lubricated with a water-soluble surgical lubricant be compromised. This might occur if the diameter of and positioned over the incision, poised for insertion the tracheal lumen has been over estimated and the when the endotracheal tube is withdrawn. previously selected tracheostomy cannula is too The surgeon then requests the anaesthesiologist large to fit into the trachea. In that case, a smaller to withdraw the endotracheal tube sufficiently to cannula should be selected. Figure 4.6 As soon as the cannula is in place, the obturator or the upper edge of the wing of the cannula (midway suction catheter should be removed and the an- between the midline and the end of the wing), aesthesiologist should disconnect the ventilator hose through the lower edge of the wing, then again from the endotracheal tube and connect it to the through the skin. When this suture is tied, the skin tracheostomy cannula. Once that is done, the an- will be drawn over the wing and usually will cover it. aesthesiologist should administer several deep After you have placed these sutures, both wings will breaths to the patient to confirm that the cannula is be securely fixed to the skin of the neck. in the proper place and that the infant can be venti- The two ties that were placed in the anterior tra- lated satisfactorily. If it appears that, although the cheal wall are now taped securely to the anterior cannula width is appropriate, the cannula is too long chest wall in such a fashion that their ends are easily and its tip rests on the carina, then several pieces of accessible in case they are needed in an emergency to gauze can be used to build up the gap between the reinsert the cannula. neck and the tracheostomy collar, thus backing the Finally, the umbilical tape or tie that usually comes tip of the cannula away from the carina. Once ade- with the cannula is passed through the holes in the quate ventilation is confirmed, then the endotracheal end of the wings and tied around the neck to further tube can be removed completely. secure the cannula. This should be tied in back of the Once the cannula is connected to the ventilator, neck. A simple gauze dressing with some antibiotic the cervical wings of the body of the cannula need to ointment is placed underneath the wings of the can- be secured to the patient.We don’t rely on a tie placed nula over the cervical incision to complete the proce- around the neck, but accomplish this with the aid of dure. sutures. We send our infants to the intensive care unit after For each wing, a suture of 3/0 silk or its equivalent a fresh tracheostomy in case of emergency. is passed through the skin of the neck, then through
    • Chapter 4 Tracheostomy 25 Figure 4.5 Figure 4.6
    • Thom E. Lobe 26 CONCLUSION Tracheostomy is a simple technical procedure to per- We change the cannula 10 days after the surgery, form, but it can be one of the more difficult proce- before the patient is discharged from the hospital, to dures in paediatrics. The cannula should be selected make certain that the cannula can be changed easily carefully to make certain that it is not too long after and to minimize the risk of cannula-related prob- the roll (used to extend the neck) is removed and the lems after discharge. patient is repositioned. Occasionally, it is necessary These patients need to be followed closely as they4 to order a special tracheostomy cannula. Such is the grow to assure the optimal cannula size and to deter- case for a short, wide trachea. mine whether the tracheostomy still is necessary. The most common problems occur post-opera- Decannulation, when possible, is done in the hos- tively when the cannula becomes occluded or, worse pital, usually after flexible or rigid bronchoscopy to yet, dislodged. This is why we secure the sutures to assess the adequacy of the tracheal lumen and the the chest wall, to make certain that if the cannula be- presence of obstructing granulation tissue or mala- comes dislodged it will be as easy to re-insert it or a cia. new cannula into the tracheal lumen. SELECTED BIBLIOGRAPHY Bach JR, Zhitnikov S (1998) The management of neuromuscu- Kenigsberg K (1994) Tracheostomy in infants. Semin Thorac lar ventilatory failure. Semin Pediatr Neurol 5 : 92–105 Cardiovasc Surg 6 : 196–199 Carr MM, Poje CP, Kingston L, Kielma D, Heard C (2001) Com- Kremer B, Botos-Kremer AI, Eckel HE, Schlondorff G (2002) plications in pediatric tracheostomies. Laryngoscope 111 : Indications, complications, and surgical techniques for 1925–1928 pediatric tracheostomies – an update. J Pediatr Surg 37 : Estournet-Mathiaud B (2001) Tracheostomy in chronic lung 1556–1562 disease: care and follow-up. Pediatr Pulmonol 23 : 135–136
    • Part II Oesophagus
    • SPRINGER SURGERY ATLAS SERIES Series Editors: J. S. P. Lumley · J. R. Siewert
    • CHAPTER 5 Oesophageal Atresia Michael E. Höllwarth, Paola Zaupa INTRODUCTIONOesophageal atresia is defined as an interruption in Prior to surgery, the type of atresia should be de-the continuity of the oesophagus with or without fis- termined. Air below the diaphragm on a plain X-raytula to the trachea. The anomaly results from an in- film including neck, chest and abdomen provides ev-sult occurring within the fourth week of gestation, idence of a commonly seen lower tracheo-oesopha-during which separation of trachea and oesophagus geal fistula. In most of these cases (type 3b/C orby folding of the primitive foregut normally takes 3c/D), a primary anastomosis between the oesopha-place. Familial cases affecting siblings or offspring geal segments is possible. In contrast, a gasless abdo-suggest genetic factors. Most cases, however, occur men indicates that a pure oesophageal atresia with-sporadically without evidence for either hereditary out lower fistula is present, and a long distanceor specific environmental teratogenic causes. The in- between the segments is to be expected (type 1/–, 2/Acidence approximates to 1:4,500 live births with a or 3a/B). A Replogle tube maximally advanced intoslight male preponderance (59%). Associated malfor- the upper pouch helps to estimate its approximatemations are obvious or easily detected in 40–60% of length.cases, and may be found in up to 80% by meticulous Additional malformations are looked for. Everysearch for structural and numerical anomalies in the neonate is checked for visible anomalies such as analskeletal system. At least 18 different syndromes have atresia or limb malformations. The thoraco-abdomi-been reported in association with oesophageal atre- nal radiography may reveal duodenal or lower intes-sia. The best known is probably the VATER or tinal atresia, a diaphragmatic hernia and/or skeletalVACTERL association of anomalies (Vertebral-Anal- anomalies. Ribs and vertebrae must be counted andCardiac-Tracheal-Esophageal-Renal-Limb). carefully examined for deformations. Usage of con- The earliest symptom of oesophageal atresia is a trast medium is rarely indicated. Cardiologic assess-polyhydramnios in the second half of pregnancy. ment, including echocardiography, forms part of rou-Polyhydramnios is an unspecific manifestation of tine pre-operative workup in order to recognize asso-swallowing disorders or of disturbance of fluid pas- ciated congenital cardiac abnormalities, which maysage through the uppermost part of the intestinal influence anaesthetic management, and the presencetract of the fetus. Prenatal ultrasound may further re- of right-sided aortic arch, which is of importance forveal forward and backward shifting of fluid in the the surgeon. Abdominal ultrasound searching for uri-upper pouch, and in cases without a lower fistula, a nary tract anomalies is performed routinely.paucity of fluid in the stomach and small intestine. The baby is nursed in the intensive care unitPostnatal presentation is characterized by drooling (ICU). Immediate surgery is rarely required, so thatof saliva and cyanotic attacks. If passage of 12 F feed- all above-mentioned investigations can be per-ing tube into the stomach is not possible, oesopha- formed step by step. Intubation and ventilation is on-geal atresia is almost certain. Immediate oro- or na- ly necessary in cases of respiratory distress, severeso-oesophageal insertion of a Replogle tube as soon pneumonia or severe associated malformations de-as the diagnosis is established is mandatory for con- manding respirator therapy. The endotracheal tubetinuous or intermittent aspiration of saliva in order should be positioned beyond a distal tracheo-oe-to prevent aspiration. The baby should be nursed sophageal fistula to avoid insufflation of gas into thepropped up in order to prevent aspiration of gastric stomach inducing a risk of rupture, especially if acontents in to the tracheobronchial tree. high gastrointestinal atresia is associated.
    • Michael E. Höllwarth, Paola Zaupa 30 Figure 5.1a–e Classifications usually take their orientation on con- corresponds to pure atresia without a fistula. The dis- currence and type of tracheo-oesophageal fistula. tance between the two segments is usually too long – The commonly used systems are those described by the same as in type 3a/B (2%) – with a fistula to the Vogt (numbers ± lower case letters) and Gross (capi- upper oesophageal pouch. The patients with type tal letters). The most frequent type of oesophageal 3c/D oesophageal atresia (3%) have an upper and a atresia (3b according to Vogt, C by Gross) affects over lower pouch fistula. Some authors classify an isolated 85% of the patients and consists of a blind-ending tracheo-oesophageal fistula without atresia – H-type upper pouch with a fistula between trachea and low- fistula – as type 4/E (3%), although it belongs to a dif- er oesophagus. Vogt’s extremely rare type 1, charac- ferent spectrum because the oesophagus is patent. In5 terized by a more or less total lack of the oesophagus Gross’ classification, congenital oesophageal stenosis is not included in Gross’ classification. Type 2/A (7%) constitutes type F.
    • Chapter 5 Oesophageal Atresia 31 Figure 5.1a–e 2/A 3a/B 3b/C 3c/D 4/E
    • Michael E. Höllwarth, Paola Zaupa 32 Figure 5.2 Surgical repair is performed under general anaesthe- ed, and the fistula to the oesophagus is localized, sia with endotracheal intubation. The endotracheal which is usually approximately 5–7 mm above the ca- tube is advanced close to the tracheal bifurcation, rina. Exceptionally, it may be found at the carina or and the infant is ventilated manually with rather low even in the right main bronchus, indicating a short inspiration pressures and small tidal volumes. These lower segment, and most likely with a long oesopha- measures serve to avoid overinflation of the stomach geal gap. The next step is to look for an upper fistula. as well as to stabilize the trachea throughout the The dorsal – membranous – region of the tracheal intervention. The Replogle tube is initially kept in wall is inspected carefully up to the cricoid cartilage. place to easily identify the upper pouch intra-opera- Small upper fistulas are easily missed. To avoid this5 tively. Broad-spectrum antibiotic prophylaxis is ad- pitfall, irregularities of the dorsal wall are gently ministered on induction. We routinely start with a probed with the tip of a 3F ureteric catheter passed tracheo-bronchoscopy using a rigid 3.5 mm endo- through the bronchoscope. If a fistula is present, the scope. Trachea and main bronchi are briefly inspect- ureteric catheter will glide into it. Figure 5.3 The standard approach for repair of an oesophageal towel under the left side of the chest improves expo- atresia is a right latero-dorsal thoracotomy. If a right sure and facilitates access in particular to the deeper aortic arch is diagnosed pre-operatively, a left-sided structures. thoracotomy is recommended. However, if an unsus- A slightly curved skin incision is placed 1 cm be- pected right descending aorta is encountered during low the tip of the scapula from the midaxillary line to surgery, the procedure can be continued in most cas- the angle of the scapula. Some surgeons prefer a ver- es, establishing the anastomosis on the right of the tical skin incision in the midaxillary line for cosmet- aortic arch. ic reasons. A major advantage in neonates is the pos- The baby is positioned on the left side, stabilized sibility of employing a muscle sparing technique – with sandbags and fixed to the table with adhesive due to their soft and mobile tissue layers. Only small bands. The right arm is abducted without undue ten- flaps of skin and subcutaneous tissue are raised sion. Mild anteversion helps to reduce the risk of around the incision. The latissimus dorsi muscle is traction injury to the brachial plexus. The elbow is mobilized by cutting through the anterior fascial at- flexed to 90°, and the forearm is best tied to a trans- tachment. It is then lifted off the thoracic wall and re- verse bar mounted over the head of the child with tracted posteriorly together with the thoracodorsal soft slings. Care must be taken that no part of the nerve, which runs on its deep surface following the body is submitted to pressure during the procedure. posterior axillary line. When the latissimus muscle is Exposed sites must be well padded. Soft pillars may rectracted, the border of the serratus anterior muscle be placed between the knees and underneath the is mobilized along its origin from the tip of the scap- feet, or the limbs wrapped with cotton wool, which ula to the sixth rib and retracted up and forwards si- protects against heat loss at the same time. A folded multaneously with the scapula.
    • Chapter 5 Oesophageal Atresia 33 Figure 5.2 Figure 5.3 Serratus anterior Trapezius Scapula Latissimus dorsi
    • Michael E. Höllwarth, Paola Zaupa 34 Figure 5.4–5.6 The intercostals muscles are divided along the upper towards the dorsal mediastinum, the use of two soft border of the fifth rib. When the parietal pleura is ex- pledgets is recommended, one to hold the already posed in one spot, a tiny moist cotton swab mounted reflected pleura under mild tension by pressing it to- on an artery forceps is used to sweep it off the thorac- wards the dorsal mediastinum, the other to proceed ic wall for an extrapleural approach. As soon as pos- with the dissection. An inadvertent tear in the pleura sible, a rib spreader is inserted and opened stepwise can be closed with a fine (6/0) monofilament absorb- with care. For continuation of the pleural stripping able suture.5
    • Chapter 5 Oesophageal Atresia 35 Figure 5.4 Figure 5.5 Figure 5.6
    • Michael E. Höllwarth, Paola Zaupa 36 Figure 5.7 The azygos vein is mobilized with right-angled for- be taken to avoid any trauma to the delicate tissue. ceps and divided in between two ligatures (4/0 Vi- Handling and squeezing the oesophageal wall with cryl). The right vagus nerve is identified, which runs forceps should be restricted to an absolute mini- along the lateral border to the upper pouch and ac- mum. Preservation of all vagal fibres supplying the companies the tracheo-oesophageal fistula towards lower oesophagus is also aimed for. Denudation in- the lower oesophagus. The lower oesophagus is usu- variably entails a significant motility disorder and ally rather thin and hypoplastic. Extreme care must may cause severe gastro-oesophageal reflux.5 Figure 5.8, 5.9 Right-angled forceps are passed behind the distal oe- Traction sutures are then placed at the tracheal sophagus and a vascular sling is placed around it in and oesophageal ends of the fistula, and one addi- order to pull it away from the trachea. This facilitates tional stay suture nearby holds the lower oesopha- identification of tracheo-esophageal fistula, which is gus. now freed from surrounding tissue.
    • Chapter 5 Oesophageal Atresia 37 Figure 5.7 Vagus nerve Trachea Distal esophagus with tracheoesophageal fistula Azygos vein Figure 5.8 Figure 5.9
    • Michael E. Höllwarth, Paola Zaupa 38 Figure 5.10 At this stage, the fistula is divided and closed with a remains in the trachea. The fistula closure is tested continuous absorbable monofilament 6/0 suture. for an air leak by watching out for air bubbles during Some authors prefer interrupted stitches, others ap- forceful ventilation after filling warm saline solution ply transfixation stitches. The level of division must into the chest. At this stage it is advisable to tempo- be as close to the trachea as possible without risking rarily relieve the lung from the continuous retraction a narrowing of the airway. Since most fistulas run and achieve through careful ventilation cycles a full obliquely upwards, a small residual pouch frequently expansion of all collapsed areas.5 Figure 5.11 The upper pouch is often retracted into the neck. tered, it is transected close to the oesophagus and Asking the anaesthetist to push on the Replogle tube closed on both sides with interrupted monofilament serves to advance the upper pouch into the operative absorbable 6/0 sutures. Contrary to the lower oe- field. Traction sutures are placed on either side of the sophagus, the upper pouch has an excellent blood pouch to assist mobilization. Dissection of the supply and can be dissected up to the thoracic inlet if oesophagus from the trachea is most challenging necessary. Thus, if a large gap exists, further dissec- because they are adherent to each other by an inter- tion of the upper oesophagus is preferable to exten- vening firm connective tissue layer. Sharp scissor dis- sive mobilization of the lower segment which in- section is required taking extreme care to avoid any volves the risks of ischaemia and subsequent dysmo- accidental penetration into either organ. Anterior tility. After the upper oesophageal pouch is mobi- and lateral aspects of the upper pouch are easily lized, both segments are approximated to see wheth- freed using pledgets. If an upper fistula is encoun- er an end-to-end anastomosis is possible. Figure 5.12 Opening of the upper pouch for the anastomosis tentially leading to lateral pre-anastomotic out- should be well centred at its lowermost point. This is pouching. The upper pouch is opened by a horizontal best achieved by incising the pouch exactly over the incision, which results in a fish-mouth-shaped aper- tip of the fully advanced Replogle tube. An asymmet- ture, adapted to the diameter of the lower oesopha- ric opening results in an uncentred anastomosis, po- gus.
    • Chapter 5 Oesophageal Atresia 39 Figure 5.10 Figure 5.11 Figure 5.12 Replogle tube
    • Michael E. Höllwarth, Paola Zaupa 40 Figure 5.13, 5.14 The end-to-end anastomosis is fashioned with inter- with the cut end to the tip of the Replogle tube, which rupted absorbable 6/0 sutures. The first two stitches is then withdrawn by the anaesthetist until the feed- are placed on either side. The posterior wall needs ing tube appears outside the mouth. The distal end of two or three additional sutures. Meticulous care must the feeding tube is passed into the stomach. The tube be given to take sufficiently large “bites” of muscular serves for postoperative gastrointestinal decompres- tissue together with the mucosal layer. The latter sion and early feeding, and also functions as trans- tends to retract upwards in the upper pouch as soon anastomotic splint for drainage of saliva. as it is opened. Once all posterior wall sutures are The anterior aspect of the anastomosis is complet- placed, the oesophageal segments are gently pulled ed in a similar way as described above with three or5 together, and the sutures are tied on the mucosal sur- four stitches, this time tying the knots on the outside face. Thereafter, a 5F silastic feeding tube – the con- of the oesophageal wall. nection hub of which has been cut off – is sutured Figure 5.15, 5.16 The goal of a tension-free end-to-end anastomosis balloon approximately 1 cm cranial to the future can be achieved with this technique in most cases of anastomotic line, either in a circular or in a spiral oesophageal atresia with a distal fistula. If the tension fashion. The mucosal layer of the upper pouch is appears to be too much despite mobilization of the rather thick so that mucosal tears can usually be upper pouch up to the thoracic inlet, further length avoided with careful dissection. The upper pouch can may be gained with a circular myotomy in the upper be lengthened by 5–10 mm by this method, which pouch according to Livaditis. This is achieved by in- may suffice to create an anastomosis without undue troduction of a 8F balloon catheter into the upper tension. Development of a pseudodiverticulum (out- pouch transorally, which is transfixed at the lower pouching of the mucosa through the established gap end of the pouch with a 4/0 monofilament traction in the muscle layer) after circular myotomy has been suture and the balloon is blown up until it fills the described. pouch. The muscle layer is then divided above the
    • Chapter 5 Oesophageal Atresia 41 Figure 5.13 Figure 5.14 Muscle Mucosa Upper esophagus Lower esophagus Figure 5.15 Mucosa Figure 5.16
    • Michael E. Höllwarth, Paola Zaupa 42 Figure 5.17, 5.18 Another way to reduce inappropriate tension on the forced ventilation until all collapsed regions are well anastomosis is to fashion a mucosal-muscular flap aerated again. from a larger upper oesophagus. A right-angled inci- The ribs are approximated with two or three peri- sion is made in one half of the upper pouch. The flap costal sutures. Latissimus dorsi and serratus anterior thus created is turned by 90° so that the vertical cut muscles are allowed to fall back into their original surface faces downwards. It is then rolled into a tube. positions and are sutured to their fascial insertion However, the gain in length results in a reduction in sites with one or two 3/0 absorbable sutures each. diameter. The subcutaneous fat is readapted with 5/0 absorb- If a satisfactory dorsal wall anastomosis can be es- able sutures including the corium. This technique ap-5 tablished, but undue tension arises in the anterior proximates the skin perfectly in most cases so that half, a right-angled flap in the corresponding part of separate skin sutures are not necessary. The incision the upper pouch without tubularization may bridge is simply approximated with adhesive strips. In those the gap and result in a safe anastomosis. cases in whom wound margin adaptation remains The thoracic cavity is irrigated with normal saline. unsatisfactory, a continuous subcuticular monofila- A soft drain is introduced via a separate intercostal ment 5/0 suture is applied, which is pulled after a few stab incision and the tip placed near the anastomo- days. sis. Before closure, the lungs are fully expanded by
    • Chapter 5 Oesophageal Atresia 43 Figure 5.17 Figure 5.18
    • Michael E. Höllwarth, Paola Zaupa 44 Figure 5.19 An airless abdomen on thoraco-abdominal X-ray also used for estimation of the length of the gap as leads to suspicions of oesophageal atresia without a well as for the distal elongation manoeuvre. lower fistula (10%).A primary end-to-end anastomo- A transverse incision is made in the left epigastric sis is not possible in these cases due to the long dis- area at a level midway between umbilicus and costal tance between the oesophageal pouches. angle. We favour a Stamm gastrostomy with two cir- Two basic surgical strategies are available in cases cular 3/0 absorbable purse-string sutures close to the of long-gap oesophageal atresia: either preservation gastric angle on the lesser curve. The stomach wall is of the patient’s own oesophagus or oesophageal incised in the centre of the purse-string sutures. If replacement. Three opinions exist concerning pres- stretching of the lower pouch is not desired, a proper5 ervation and delayed repair of the native oesophagus gastrostomy tube is introduced, the purse-string su- in the absence of a lower fistula. The first is to await tures are tied and fixed to the parietal peritoneum spontaneous growth, which is more pronounced in within the incision. If, however, a longitudinal bou- the upper stump. As experience tells us, it takes 8–12 ginage from above and below is planned, a jejunosto- weeks on the average until a safe anastomosis is fea- my for feeding is fashioned in the first jejunal loop sible. Second, one can attempt to promote elongation with a separate exit below the abdominal incision of the upper oesophageal segment by regular longi- and with a single 3/0 purse-string suture that is an- tudinal stretching. Third, approximation may be fur- chored on the internal aspect of the abdominal wall. ther accelerated by additional bouginage of the lower The feeding tube is advanced deep into the jejunum. pouch. The latter is our preferred method, permitting Enteral feeding may be started after 24 h. one to anastomose the two segments after 3–5 weeks. A primary gastrostomy is essential for enteral feeding in all long-gap oesophageal atresia cases. It is Figure 5.20 If mechanical elongation of the lower pouch is kept in the stomach for the stretching procedures, planned, the gap is assessed in the following way: a and longitudinal stretching of both oesophageal 8F–10F feeding tube is cut approximately 10–13 cm stumps is performed twice daily for 3–5 min under from its distal end, and a 70° curved metal sound is mild sedation. Gentle pressure is used in the lower, introduced into the feeding tube up to its tip. This as- more forceful pressure in the upper pouch. Leaving sembly is passed into the lower oesophageal pouch the manoeuvre in the same experienced hands via the stomach. At the same time, the anaesthetist throughout has saved us from ever causing a perfora- introduces a radio-opaque device into the upper tion. Progress of elongation is evaluated by weekly pouch. Both probes are pushed towards each other fluoroscopic calibration and radiographic documen- under fluoroscopic control, and the distance between tation. Distinct overlapping of the segments, which is the maximally approximated oesophageal stumps is necessary for end-to-end anastomosis without ten- gauged. Usually it corresponds to four or more verte- sion, is achieved within 3–5 weeks. bral bodies. The feeding tube with the metal probe is
    • Chapter 5 Oesophageal Atresia 45 Figure 5.19 Charriere tube n.10 Metal sound Figure 5.20
    • Michael E. Höllwarth, Paola Zaupa 46 Figure 5.21 H-type fistulas without atresia account for about 3% pands and exposes the right cervical area. The inci- of the tracheo-oesophageal anomalies. Presentation sion follows a suitable skin crease, approximately is usually more protracted and sometimes delayed 1 cm above the medial third of the right clavicle.After beyond the first year of life. Typical symptoms are dividing the platysma, the medial border of the ster- choking episodes during feeding together with cya- nomastoid muscle is retracted posteriorly. notic spells. Diagnosis is made either by contrast The dissection proceeds medially to the carotid oesophagogram or tracheobronchoscopy. If an H- artery, and it may be necessary to divide the middle type fistula is confirmed, a 3F ureteric catheter is thyroid vein and the inferior thyroid artery to reach passed across the fistula during bronchoscopy. Most trachea and oesophagus which are situated medial5 H-type fistulas can be approached from the neck be- and posterior to thyroid lobes and isthmus. Palpation cause they are usually situated at or above the level of of the tracheal cartilages and the feeding tube in the the second thoracic vertebra. For the cervical repair, oesophagus facilitates anatomical orientation. The the child is placed supine on the operating table. The recurrent laryngeal nerve runs upwards in the head is turned to the left and a folded towel or a groove between trachea and oesophagus close to the sandbag is placed underneath the shoulders to hy- fistula. It must be clearly identified and protected perextend the neck. This position maximally ex- from any injury. Figure 5.22–5.24 The plane between oesophagus and trachea is care- monofilament absorbable 6/0 suture is employed to fully developed. The ureteric catheter in the fistula close the tracheal side of the fistula and the oesopha- aids its identification. Right-angled forceps are used gus in interrupted technique. The wound is closed in to dissect the fistula and a small vascular sling is layers with absorbable suture material finishing with passed around it. Two stay sutures are placed on the interrupted subcuticular absorbable 6/0 sutures. At oesophageal side of the fistula, which is divided after the end of the operation, the motility of the vocal withdrawal of the ureteric catheter. A transfixation cords should be reassessed.
    • Chapter 5 Oesophageal Atresia 47 Figure 5.21 Figure 5.22 Figure 5.23 Figure 5.24
    • Michael E. Höllwarth, Paola Zaupa 48 CONCLUSION The first successful primary repair of an oesophageal distal oesophageal pouch. However, severe swallow- atresia was achieved by Cameron Haight in 1941. ing problems with dysphagia are rare, but impaction Mortality remained, however, high in the following of foreign bodies, most often bread, meat or fruit decades. The outcome was influenced by birth pieces, may be partially attributable to the motility weight, severity of additional malformations, and de- disorder. velopment of aspiration pneumonia due to delayed An anastomotic stricture can be either the result diagnosis. Nowadays, the diagnosis is, in most cases, of an anastomosis fashioned under high tension, im- established immediately after birth, and pneumonia paired perfusion and/or an anastomotic leak, or it can be prevented by continuous suction of the upper may be caused by continuous acid exposure due to5 pouch. Survival of premature infants has significant- gastro-oesophageal reflux. Clinically, delayed clear- ly improved with progress in neonatal intensive care. ance of acid reflux is probably of greater importance Thus, severe associated anomalies have become the due to the high incidence of gastro-oesophageal re- main factor determining outcome. While basic surgi- flux disease that exceeds 40% in patients with oe- cal management has become uniform for the most sophageal atresia. common type of oesophageal atresia with a lower fis- Atypically shaped cartilaginous C-rings and a tula, the best strategy for babies with long-gap oe- wide intercartilagineous membrane within the re- sophageal atresia has remained controversial. Some gion of the former fistula may be underlying causes authors – including our team – prefer to restore the of another common complication: tracheomalacia native oesophagus whenever possible, even at the with an incidence around 20%. The anterior-posteri- price of severe gastro-oesophageal reflux, whereas or diameter of the trachea is reduced and may col- others propagate more generous indications for oe- lapse completely with strained inspiration and expi- sophageal substitution, either with colon or stomach. ration. The anomaly rarely causes serious problems The overall prognosis of patients with oesopha- and usually resolves with age and growth. Some- geal atresia is good, but recurrent dysphagia, secon- times, however, severe respiratory distress with near- dary problems of gastro-oesophageal reflux, and an miss events may occur. Continuous monitoring and increased incidence of recurrent respiratory tract in- urgent treatment are then indicated. Aortopexy fection – possibly due to repeated minimal aspira- under bronchoscopic control is currently the most tions during sleep – are common sequel. The distal commonly used surgical method. It resolves the oesophagus frequently suffers from delayed clear- problem in many cases, unless the weak tracheal seg- ance due to disturbed motility. The impairment of ment is too long. Recently, tracheoscopic stabiliza- propulsive peristalsis may be part of the malforma- tion with a self-expanding or balloon-expandable tion pattern, but may be iatrogenically worsened by stent has been advocated. The ideal stent has, howev- damage of vagal nerve fibres during dissection of the er, yet to be found and long-term results are awaited. SELECTED BIBLIOGRAPHY Deurloo JA, Ekkelkamp S, Schoorl M, Heij HA, Aronson DC Little DC, Rescorla FJ, Grosfeld JL, West KW, Scherer LR, (2002) Esophageal atresia: historical evolution of manage- Engum SA (2003) Long-term analysis of children with ment and results in 371 patients. Ann Thorac Surg 73 : esophageal atresia and tracheooesophageal fistula. J Pedi- 267–272 atr Surg 38 : 737–739 Kluth D, Steding G, Seidl W (1987) The embryology of foregut Livaditis A, Rafberg L, Odensjo G (1972) Esophageal end-to- malformations. J Pediatr Surg 18 : 217–219 end anastomosis. Reduction of anastomotic tension by Lemmer JH, Mark NG, Symreng T, Ross AF, Rossi NP (1990) circular myotomy. Scand J Thorac Cardiovasc Surg 6 : Limited lateral thoracotomy. Arch Surg 125:873–877 206–211
    • CHAPTER 6 Gastro-oesophageal Reflux and Hiatus Hernia Keith E. Georgeson INTRODUCTIONMost infants spit up milk after feedings, sometimes id reflux in paediatric patients. Upper gastrointesti-in a spectacular fashion. This post-prandial regurgi- nal endoscopy is occasionally useful in defining thetation is rarely associated with any serious conse- presence of oesophagitis secondary to GER. Gastricquences to the baby and is usually outgrown by 1 year emptying studies are not usually useful in the work-of age. up for GER in children. Pathologic gastro-oesophageal reflux (GER) in in- Proton pump inhibitors and promotility agentsfants is associated with potentially serious complica- are useful therapeutic modalities for most childrentions including failure to thrive, recurrent apnea and with pathologic GER. Even those patients who do notaspiration of gastric contents. Older children can al- fully respond to medical management should beso present with reactive airways disease, chronic si- treated for 8 weeks before surgical therapy is consid-nusitis and peptic oesophagitis. ered, unless the patient is experiencing life-threaten- A careful history is invaluable in eliciting the ing symptoms.symptoms of GER in children. Frequent regurgita- Antireflux surgery is indicated in patients with in-tion, asthma associated with recumbency and ex- adequate response to medical management or intreme irritability are all potential signals of GER. children who cannot be weaned from medical man-Further workup should include an upper gastrointes- agement. Antireflux surgery is also appropriate intinal study to rule out other anatomic causes of re- children with the complication of peptic oesopha-gurgitation and to detect the presence of a hiatus gitis presenting with a stricture or Barrett’s oesopha-hernia. A 24-h pH probe study is considered the gold gus. Those infants with life-threatening events de-standard for detecting pathological GER in children. spite optimal medical management are also candi-A negative pH probe study does not rule out sympto- dates for immediate surgery.matic GER due to the common occurrence of non-ac-
    • Keith E. Georgeson 50 Figure 6.1 The intubated patient is positioned at the end of the stomach. The dilator should be large enough to fully operating table. The knees are flexed and the feet distend the distal oesophagus for safer peri-oesopha- cushioned. The patient is taped to the table so he/she geal dissection. The patient’s head should be posi- will not slide when placed in the reversed Trendelen- tioned so that the anaesthetist has access to withdraw burg’s position. The operator stands at the end of the and advance the dilator as needed throughout the table, which is positioned low enough for easy ma- course of the operative procedure. The patient is nipulation of the laparoscopic instruments. A large prepped from nipples to groin. Maloney bougie is passed through the mouth into the Figure 6.26 Five trocars are inserted in the abdominal wall. Un- structures. The Veress needle inside the expandable like adult patients, who have a precisely defined posi- sheath is then removed and the trocar cannula in- tion for each trocar, children have more variation in serted through the plastic sheath expanding the tro- body habitus and liver position so the placement of car and fixing it to the abdominal wall due to its snug the trocars must be tailored to the individual patient. fit. Suture fixation is sometimes necessary in smaller The initial trocar placement is through the centre of infants with a thin abdominal wall.A 30º 4-mm scope the umbilicus in the midline. Each trocar site should is advanced through the umbilical trocar after a be infiltrated prior to the placement of the trocar pneumoperitoneum has been instilled. This scope is with a local anaesthetic. The incision in the umbili- then used for surveillance during the placement of cus should be the same size as the trocar. The umbil- the other four trocars. The second trocar is placed in ical scar does not stretch well.An extremely tight tro- the right upper quadrant. This trocar should be posi- car will cause ischaemic injury to the skin of the um- tioned at the inferior margin of the liver border in bilicus if the skin incision is not large enough to ac- the right anterior axillary line. The articulated re- commodate the trocar. The incision should be made tractor is passed toward the left upper quadrant and through the central portion of the umbilicus. The tightened to form its working position. It is then se- peritoneal cavity is usually easily entered through the cured to the frame of the operating table by way of a umbilicus because the layers of the abdomen are retractor holder. The third, fourth and fifth trocars scarred together at this point. Once the peritoneal are then placed under laparoscopic surveillance. All cavity is opened with a no. 11 blade, a curved mosqui- but the umbilical trocars are reusable 3- or 4-mm tro- to clamp is introduced into the peritoneal cavity with cars except in patients over 20 kg when a larger liver the tip upward pointing away from the abdominal retractor is passed through a 5-mm trocar. viscera. The clamp is pushed inward to dilate the um- Trocar site 3 is used for the endoscope and is also bilical opening allowing easier access to the perito- the prospective gastrostomy site during gastrostomy neal cavity. A radially expanding disposable 5-mm button placement. Trocar sites 2 and 4 are the work- trocar with a fitted Veress needle is then passed into ing ports for the operating surgeon. Trocar site 5 is the peritoneal cavity while pulling upward on the the initial entry point and is also used for surveil- umbilical skin. The trocar should be advanced at a lance during placement of the other 4 ports. When 30º angle and its tip kept as close to the parietal peri- the endoscope is moved to port 3 to perform the op- toneum of the anterior abdominal wall as possible to eration, port 5 is used for intraperitoneal access by avoid injury to intra-abdominal or retroperitoneal the surgeon’s assistant.
    • Chapter 6 Gastro-oesophageal Reflux and Hiatus Hernia 51 Figure 6.1 Figure 6.2 4 2 1 3 5
    • Keith E. Georgeson 52 Figure 6.3 The intraperitoneal dissection is begun by opening and to facilitate a faster operation. The dissection is the upper part of the hepatogastric ligament. The continued in this plane bluntly and sharply over the dissection is performed sharply. The hepatic branch- top of the oesophagus and down the left side. The an- es of the vagus nerve are divided. The small vessels to terior vagus nerve is usually tightly adherent to the the liver are also divided using electrocautery. Care is muscle of the oesophageal wall. However, occasional- taken to avoid transecting the left hepatic artery, ly the nerve falls away and is only loosely associated which can be found in this ligament in a very small with the oesophageal wall. Linear structures along number of patients. The dissection is carried up to the anterior oesophageal wall should be carefully the hiatus in an avascular plane. The phreno-oesoph- evaluated before dividing them. The cleavage ageal ligament is opened between the oesophagus between the oesophagus and left crus should be car- and the right crus. The peri-oesophageal plane ried posteriorly until the fundus of the stomach is6 should be entered cleanly to avoid excessive bleeding encountered. Figure 6.4 The short gastric vessels are divided routinely. Divid- in dividing the short gastric vessels. The gastrosplen- ing these vessels allows for much better visualization ic ligament is opened at the mid portion of the of the left crus and also contributes to a better geom- spleen. The dissection is carried cephalad from this etry of the fundoplication wrap. In most patients, the point. Most patients have both an anterior and poste- vessels are divided with a hook electrocautery. In rior leaflet of the gastrosplenic ligament. Vessels run large or obese patients an ultrasonic scalpel is useful in both leaflets.
    • Chapter 6 Gastro-oesophageal Reflux and Hiatus Hernia 53 Figure 6.3 Figure 6.4
    • Keith E. Georgeson 54 Figure 6.5 Figure 6.6 The left crus should be followed as it courses toward The crura are closed in every case by approximating the right side behind the oesophagus. Dissection is them behind the oesophagus with non-absorbable performed from both sides. The fundus is pulled sutures. Generous bites of crus are taken on both the down using a grasper through the umbilical port site, left and right sides and are tied snugly. The aorta is which allows excellent visualization of the left crus. located behind the posterior aspect of the left crus For visualization of the right crus, the grasper is and should not be incorporated in the suture closing pulled downward and toward the splenic flexure of the crura. The author prefers to close the crura with the colon. By dissecting both sides alternately using the dilator withdrawn into the oesophagus. Great the left crus as a guide, a window is safely formed be- care should be taken to avoid closing the hiatus too hind the oesophagus. The tissues tethering the oe- tightly. If a large dilator is left in the intra-abdominal sophagus to the crura are further divided circumfe- oesophagus, suture placement is more difficult and6 rentially to lengthen the intra-abdominal portion of the hiatus is often left larger than it should be. With the oesophagus. The posterior vagus is vulnerable large hiatal defects, the hiatus may require both pos- and should be identified and preserved. An instru- terior and anterior closure. Once the hiatus is closed ment passed through the umbilical trocar is used to around the oesophagus, at least 2 cm or more of oe- retract downward on the gastro-oesophageal junc- sophagus is fixed in the abdomen utilizing three or tion while lengthening the intra-abdominal oesoph- four collar sutures. These sutures are usually placed agus. Circumferential dissection around the oesoph- at the 11, 7 and 3 o’clock positions on the oesophagus agus is continued as far into the mediastinum as nec- incorporating a portion of the oesophageal wall and essary to provide at least 2.5 cm of oesophagus in the coapting it to the associated crus. For large hiatus abdomen with no downward tension on the oesoph- hernias it may take four or more collar sutures to ad- agus. If less than 2.5 cm of oesophagus remains in the equately close the oesophageal hiatus. abdomen after releasing the downward tension on the oesophagus, consideration should be given to lengthening the abdominal oesophagus by tubulariz- ing the upper stomach. As much as possible, the fas- cia covering the crura should be left intact. Care should also be taken to avoid entry into the plural cavity on either side. If a hole is made in the pleura it should be enlarged to avoid the development of a tension pneumothorax caused by a one-way ball- valve effect. The pneumothorax can be evacuated by needle thoracentesis at the end of the operation. Figure 6.7 The mobilized fundus is pushed up beside the left The fundus is fluffed until a geometric symmetry is side of the oesophagus. A grasper via the umbilical achieved. A “shoe shine” manoeuvre is used to con- port is used to lift the oesophagus exposing the fun- firm the fundal wrap and to avoid attaching the fun- dus behind the oesophagus. The fundus is grasped dus to the mid portion of the stomach. and pulled through the retro-oesophageal window.
    • Chapter 6 Gastro-oesophageal Reflux and Hiatus Hernia 55 Figure 6.5 Figure 6.6 Figure 6.7
    • Keith E. Georgeson 56 Figure 6.8 The dilator should be repositioned into the stomach left and right sides of the fundal wrap and securing at this time. The left side of the fundus is then basted them to the oesophagus. The wrap should be no to the right side of the fundus with a single stitch that more than 1.5–2 cm in length and lie loosely around does not incorporate oesophageal tissue. The wrap the oesophagus. A figure-of-eight suture is then should be loose and should encircle the oesophagus. placed near the bottom of the wrap as a second layer Two or three non-absorbable sutures are placed to secure the fundoplication and prevent wrap break- above and/or below the first stitch incorporating the down.6 Figure 6.9 Gastrostomy placement is performed in conjunction was not performed, the stomach should be grasped with fundoplication only in those patients who have close to the lesser curvature. Using a large curved swallowing disorders or severe failure to thrive. It is needle with a monofilament suture swaged to the not used as a routine procedure to decompress the needle, a U-suture is passed through the abdominal stomach after a fundoplication. The laparoscope is wall through the stomach taking a 1–0.5 cm bite of moved back to the umbilical port. A locking grasper stomach and back through the abdominal wall. Pass- is passed through the medial left upper quadrant tro- ing the suture into the gastric lumen does not seem car site. This trocar is initially positioned with the in- to lead to complications. A second U-suture is passed tention of using this site as the gastrostomy site. The parallel to the first 1.5 cm lateral to the first suture. stomach is grasped near the greater curvature at the The grasper is then removed along with the trocar. junction of the body and antrum. If a fundoplication
    • Chapter 6 Gastro-oesophageal Reflux and Hiatus Hernia 57 Figure 6.8 Figure 6.9
    • Keith E. Georgeson 58 Figure 6.10 The anaesthetist passes a single lumen orogastric stomach and the needle removed over the J-wire. The tube into the stomach and inflates the stomach with tract is dilated with vascular dilators from a size 8- 60–120 cc air. A hollow needle is passed through the French up to a size 20-French. The 20-French dilator medial left upper quadrant trocar site into the inflat- should be passed through the abdominal wall only ed stomach. The passage of the needle into the lumen and not into the stomach. The U-suture should be al- of the stomach should be visualized completely and lowed to slacken during passage of the 20-French di- should not occur on the blind side of the stomach. A lators to avoid passing of the dilator into the stom- J-wire is then passed through the needle into the ach.6 Figure 6.11 The gastrostomy button is stiffened by passing the 8- to close the umbilical fascia. Once closure of the um- French dilator through it. The dilator and balloon bilical fascia has been achieved, the pneumoperiton- button are passed over the guide wire. Gentle twist- eum is reinstated and the umbilical closure visual- ing of the balloon button while holding countertrac- ized from a lateral port site to confirm that the omen- tion on the U-suture allows the balloon button to slip tum has not been incorporated in the umbilical clo- into the stomach under laparoscopic surveillance. sure. The other trocars are then removed. The fascia The balloon should be inflated under direct visual- in these other trocar sites does not usually require ization. The U-sutures are slackened at this point to closure. The skin is closed with subcuticular sutures make certain that the stomach is independently held and skin strips. The umbilical skin should be closed against the abdominal wall by the inflated balloon carefully with rapidly absorbable braided suture. button. The U-sutures are then tied over the wings of Careless closure of the umbilicus can result in granu- the balloon button. The laparoscope is passed loma formation post-operatively. through the lateral left upper quadrant trocar site to The patients are fed clear liquids on the day of sur- look at the gastrostomy button from a different angle gery. Pureed foods are useful for 3–4 weeks to avoid to assure that it is properly positioned and remains the dysphagia associated with oedema of the fun- inflated. doplication wrap. Discharge is 1–3 days after surgery. The liver retractor is removed using laparoscopic Post-operative pain is controlled with intravenous surveillance. The umbilical trocar is the first trocar to ketorolac, scheduled acetaminophen and ibuprofen. be removed after the pneumoperitoneum is evacuat- Narcotic agents are only used when necessary. The ed. The fascia of the umbilicus is closed using a U-sutures in the gastrostomy are removed on the groove director to protect the underlying bowel and second post-operative day. omentum. A simple or figure-of-eight suture is used
    • Chapter 6 Gastro-oesophageal Reflux and Hiatus Hernia 59 Figure 6.10 Figure 6.11
    • Keith E. Georgeson 60 CONCLUSION Reflux control is excellent after fundoplication. Poor and can be ameliorated by dividing the feeding bolus results are obtained in children whose pre-operative into two parts given 30 min apart or by continuous symptoms were unrelated to GER. Dysphagia can be drip feeding. Retching after the third or fourth bolus symptomatic in up to 40% of children if they are feeding of the day is often due to a low satiety set allowed solid foods during the first few weeks of point and can be temporarily improved by anabolic surgery. Long-term dysphagia occurs in less than 2% steroids, which increases the child’s appetite. Gag- of patients. Post-operative oesophageal dilatation ging unrelated to feeding is often induced by a varie- should be avoided, as it is associated with breakdown ty of stimuli and is very difficult to treat. This global of the fundoplication wrap and/or herniation of the type of retching often results in breakdown of the stomach into the chest. Recurrent reflux at 2 years is fundoplication because of the chronic and forceful less than 5% with little further recurrence after 2 nature of the gagging.6 years. Retching and choking are most commonly Fundoplication is a highly reliable therapy in chil- seen in neurologically impaired children. Those chil- dren with persistent or life-threatening GER. Lapar- dren with new onset retching after fundoplication oscopic fundoplication is superior to open fundopli- are usually responding to overzealous feeding lead- cation and should be in the repertoire of all paediat- ing to hypersatiety. Retching immediately after bolus ric surgeons who operate on children with GER. feeding is often due to gastric distension or dumping SELECTED BIBLIOGRAPHY Fonkalsrud EW, Ashcraft KW, Coran AG et al (1998) Surgical Sampson LK, Georgeson KE, Winters DC (1996) Laparoscopic treatment of gastroesophageal reflux in children: a com- gastrostomy as an adjunctive procedure to laparoscopic bined hospital study of 7,467 patients. Pediatrics 101 : fundoplication in children. Surg Endosc 10 : 1106–1110 419–422 Wulkan ML, Owings E,Georgeson KE (1998) Safety and effica- Georgeson KE (1998) Laparoscopic fundoplication and gas- cy of the 2 U-stitch gastrostomy tube. Surg Endosc 12 : 643 trostomy. Semin Laparosc Surg 5 : 25–30 Rothenberg SS (1998) Experience with 220 consecutive lapar- oscopic Nissen fundoplication in infants and children. J Pe- diatr Surg 33 : 274–278
    • CHAPTER 7 Achalasia Paul K. H. Tam INTRODUCTIONAchalasia (a Greek term meaning “does not relax”) is supports an autoimmune aetiology. Similaritya rare motility disorder characterized by an absence between achalasia and Chagas’ disease caused byof normal oesophageal peristalsis, and an increased Trypanosoma cruzi suggests that a neurotropic infec-basal resting pressure and failure of complete relaxa- tious agent may be responsible. Rarely, familial casestion of the lower oesophageal sphincter (LOS). Less and association with microcephaly and the congeni-than 5% of all cases present before the age of 15 years, tal anomalies have been observed.giving an estimated incidence of 0.1 per 100,000 chil- Patients usually present one or more of the follow-dren. Males and females are equally affected. ing symptoms: vomiting/regurgitation of undigested The condition was first described in 1674 by Willis food, progressive dysphagia, weight loss/failure towho successfully treated a patient by repeated oe- thrive, choking, retrosternal discomfort, and pulmo-sophageal dilatation using a sponge-tipped whale nary problems such as recurrent coughing or chestbone rod. In the early 1900s, based on observations in infections.Vomiting and dysphagia are the common-100 reported cases, von Mikulicz suggested cardios- est initial symptoms. Vomiting occurs more fre-pasm as the aetiology. In 1914 Heller described cardi- quently in infants and young children whereas dys-omyotomy, a procedure that carries his name and phagia is commoner in older children.forms the basis of all surgical approaches to this Chest X-ray may show an air-fluid level in the oe-problem up to this date. The original operation con- sophagus; there may be a soft tissue shadow in thesisted of two myotomies anteriorly and posteriorly mediastinum on the left hemithorax correspondingon the lower oesophagus performed through a lapa- to a dilated lower oesophagus, and sometimes pneu-rotomy. A single anterior cardiomyotomy was subse- monic changes. The characteristic radiological fea-quently found to be adequate for symptomatic relief. tures of achalasia in a contrast swallow are a proxi-The operation has been performed through a thora- mal dilated oesophagus with a smooth tapering ofcotomy, as well as thoracoscopically and laparoscop- the gastro-oesophageal junction (bird’s-beak sign orically with or without an additional antireflux proce- rat-tail deformity). There is an absence of coordinat-dure. ed peristaltic waves in the proximal oesophagus and The pathogenesis of primary achalasia is not well a persistent failure of relaxation of the LOS on swal-understood. The most consistent histologic finding is lowing.a decrease or loss of myenteric ganglion cells, and Endoscopy confirms a dilated oesophagus whichthis is more pronounced in advanced cases. The de- funnels smoothly towards a narrowed LOS. Retainedgenerative process especially involves neurones pro- food or yeast oesophagitis may be noted in the oe-ducing neuropeptides and nitric oxide, the latter be- sophagus. Although the LOS is closed, it provides lit-ing identified as inhibitory neurotransmitters. Loss tle resistance to the advancing endoscope.of inhibitory innervation causes increased tonic con- Oesophageal manometry is the “gold standard” fortraction and interference with normal relaxation of the diagnosis of achalasia. Diagnostic features in-LOS, as well as aperistalsis of oesophageal body. clude a failure of relaxation of LOS on swallowingThere are no specific histologic changes in the oe- and absence of peristalsis in the body of the oesoph-sophageal muscles. The cause of the neuronal dam- agus. Features that are characteristic but not requiredage remains unknown. Various mechanisms, includ- for the diagnosis include elevated resting LOS pres-ing autoimmune, infectious, genetic, toxic and pri- sure (>45 mmHg), and resting pressure in the oe-mary, have been proposed. The finding of myenteric sophageal body exceeding that in the stomach.inflammation, which is predominantly lymphocytic, Symptomatic relief can be achieved by loweringthe presence of serum auto-antibodies to myenteric LOS pressure with nitrate or calcium channel blockerplexus, and the increased frequency of class II histo- (nifedipine) medication or with intrasphincteric in-compatibility antigens in patients with achalasia jection of botulinum toxin. The need for life-long
    • Paul K. H. Tam 62 medication (with its associated side-effects) or re- be achieved using either rigid or balloon dilators, the peated injections, respectively, limits the role of med- latter being the preferred choice in children. Pneu- ical therapy to those children who are not suitable for matic dilatation can be used for primary treatment treatment by dilatation or surgery. or as a secondary procedure when symptoms recur Definitive treatment of achalasia consists of dila- after surgery. Our experience suggests that dilatation tation or oesophago-cardiomyotomy. Dilatation is less effective than surgery for long-term sympto- should be guided by endoscopy/fluoroscopy and can matic relief. Figure 7.1 Figure 7.2 Heller’s oesophago-cardiomyotomy remains the The phreno-oesophageal ligament is incised. The an- mainstay of treatment for achalasia and can be per- terior vagus is seen on the anterior wall of the oe- formed via the abdomen or thorax, either as an open sophagus and should be preserved. The hiatal win- procedure or by the minimally invasive approach, dow is identified adjacent to the caudate lobe and the7 and with or without a concomitant fundoplication. tissues between the oesophagus and the crura are di- Preoperatively, any yeast oesophagitis should have vided. The abdominal oesophagus is further freed by been eradicated with antifungal medication. The pa- blunt dissection into the posterior mediastinum, tak- tient is kept on clear fluids a day prior to surgery to ing care not to penetrate the pleura proximally. The minimize the risk of aspiration of retained food on posterior vagus is preserved. A cotton tape encircling anaesthesia induction. Preoperative endoscopy en- the cardio-oesophageal junction is used to retract the sures complete emptying of dilated oesophagus. A abdominal oesophagus caudally. The site of myoto- large feeding tube or a balloon catheter is introduced my is marked by electrocautery to the left of the an- into the stomach. terior vagus. Depending on the surgeon’s preference, surgical access can be achieved via the abdomen or left tho- rax. The abdominal approach is more popular, and allows concomitant fundoplication to be performed more easily. With the patient supine, an upper mid- line is made for laparotomy. For laparoscopic access, the patient is placed in a lithotomy position with the surgeon at the end of the operating table; four to five ports are placed as shown. The telescope is placed in the supra-umbilical port (1). To expose the oesophagus in the open procedure, the left lobe of the liver is retracted superiorly and medially; the left triangular ligament may be divided to enhance the exposure. For the laparoscopic ap- proach, a cotton-tipped rod is inserted into the epi- gastric port (3) to retract the caudate lobe of the liver cephalad. Instrumentation is carried out via the re- maining ports (2, 4, 5).
    • Chapter 7 Achalasia 63 Figure 7.1 Figure 7.2 3 2 4 5 1
    • Paul K. H. Tam 64 Figure 7.3 Figure 7.4 Myotomy extends for 4–6 cm above, and 0.5–1 cm be- To avoid the long-term complication of gastro-oe- low the cardio-oesophgeal junction. A superficial in- sophageal reflux after myotomy, many surgeons rec- cision is made with the diathermy tip. The thickened ommend a concomitant fundoplication. Details of oesophageal muscle is then divided with scissors and the procedure are separately described (see Chap. 6). separated by blunt dissection with grasping/prepara- The fundoplication should be loose to avoid dyspha- tion forceps until the submucosal plane is reached. gia. A posterior 180° (Toupet) fundoplication can be Great care is taken to avoid mucosal perforation. performed over the distal 1–1.5 cm of the oesophagus. Myotomy is continued proximally and distally with The fundus is sutured separately to the cut edge of diathermy hook and blunt dissection until all con- the oesophageal muscle on either side using three stricting muscles have been separated and the muco- non-absorbable interrupted sutures. This procedure sa is seen bulging outwards; the muscular edges holds the myotomy edges apart in addition to pro- should be undermined for 50% of the oesophageal viding an antireflux mechanism. circumference. The gastro-oesophageal junction is recognized by the “collar-like” configuration of the7 circular muscles. The gastric muscles are usually more adherent to the mucosa. Mucosal perforation is tested by insufflation of the oesophagus; if present, this should be repaired by fine suture.A widened hia- tus should be narrowed by one or two non-absorb- able deep sutures placed through the crura. The wound is closed in the usual manner. Figure 7.5 Alternatively, an anterior 180° (Dor or Thal) fundop- A nasogastric tube is left overnight. Fluid diet is lication is performed. The anterior fundus is commenced after a contrast study confirms the ab- “draped” over the anterior oesophagus, covering the sence of a leak and when gastric stasis has resolved. myotomy. This procedure may be more appropriate Return to normal feeds is usually faster for laparos- for patients with mega-oesophagus as the posterior copic procedures. fundoplication is more prone to result in outflow ob- struction. It may also provide additional cover after repair of a mucosal perforation.
    • Chapter 7 Achalasia 65 Figure 7.3 Figure 7.4 Figure 7.5
    • Paul K. H. Tam 66 CONCLUSION Oral medication with nifedipine or nitrates can result omyotomy can be due to mega-oesophagus, incom- in a 50% decrease in LOS but is commonly associated plete myotomy or gastro-oesophageal reflux. Incom- with side-effects such as headache. Experience of plete myotomy usually responds to secondary pneu- intrasphincteric injection of botulinum toxin is lim- matic dilatation. Gastro-oesophageal reflux is pre- ited in children. Recent studies suggest a mean dura- ventable by a concomitant fundoplication during tion of effect of 4 months; more than half of the pa- myotomy; attempts to perform a fundoplication as a tients are expected to require a repeat injection with- second operation after an initial myotomy without in 6 months. fundoplication is technically more difficult. Laparos- Pneumatic dilatation has been reported to be ef- copic myotomy is gaining popularity. Transthoracic fective in 50–90% of cases in selected small series. video-assisted Heller’s myotomy has also been per- Most authors, including ourselves, have been unim- formed successfully. An additional fundoplication is pressed with its efficacy as primary treatment in chil- easier to perform laparoscopically than thoracoscop- dren with achalasia. Multiple dilatations are often re- ically. Compared to open procedures, the minimally quired. Complications include oesophageal perfora- invasive approach results in superior cosmesis, less7 tion and symptomatic gastro-oeosophageal reflux. post-operative pain, earlier return to resumption of Heller’s oesophago-cardiomyotomy is the method feeding (means: 2.7 days for laparoscopic procedure, of choice for the treatment of achalasia in children. 9.0 days for open) and shorter hospital stay. Conver- Long-term symptomatic relief is obtained in 86% of sion to open myotomy is necessitated in 10% of la- children after surgery. Most series report zero mor- paroscopic procedures, usually as a result of intra- tality. Complications include oesophageal perfora- operative oesophageal perforation. With increasing tion (10%), atelectasis and post-operative fever experience, even oesophageal perforations can be re- (42%), dysphagia (14%) and gastro-oesophageal re- paired laparoscopically. flux (20%). A poor result following oesophago-cardi- SELECTED BIBLIOGRAPHY Babu R, Grier D, Cusick E et al (2001) Pneumatic dilatation for Mehra M, Bahar RJ, Ament ME et al (2001) Laparoscopic and childhood achalasia. Pediatr Surg Int 17 : 505–507 thoracoscopic esophagomyotomy for children with achala- Esposito C, Medoza-Sagaon M, Roblot-Maigret B et al (2000) sia. J Pediatr Gastoenterol Nutr 33 : 466–471 Complications of laparoscopic treatment of esophageal Vane DW, Cosby K, West K et al (1988) Late results following achalasia in children. J Pediatr Surg 35 : 680–683 esophagomyotomy in children with achalasia. J Pediatr Hurwitz M, Bahar RJ, Ament ME et al (2000) Evaluation of the Surg 23 : 515–591 use of botulinum toxin in children with achalasia. J Pediatr Gastroenterol Nutr 30 : 509–514
    • CHAPTER 8 Colonic Replacement of the Oesophagus Alaa Hamza INTRODUCTIONTo date, there is no better substitute for the native oe- tions. Extensive infection with candida, epidermoly-sophagus because the ideal graft does not exist. Many sis bullosa or, very rarely, massive varices due to por-studies have been done and different organs are tal hypertension and strictures after injection areused: the jejunum, the stomach as a tube or as the rare causes for replacement.whole organ, and the colon. There is no agreement on Over the last 30 years more than 850 oesophageala single organ or a single route. The colon is the most replacements have been performed in the Pediatriccommonly used organ, and experienced centres con- Surgery Department of Ain-Shams University. Thesider it as a good substitute in most of the cases. technique has evolved from gastric pull-up to colon Indications include oesophageal atresia failed af- replacement, initially subcutaneously, then retroster-ter repair or a wide gap. Full-thickness injury to a nally. In the last 13 years we started transhiatal oe-long segment of the oesophagus after caustic inges- sophagectomy with posterior mediastinal colon re-tion invariably results in an intractable stricture that placement. The left colon based on the left colic ar-fails to respond to repeated dilatation and requires tery as a graft in all cases of oesophageal replacementsubstitution. Other indications include multiple ex- or bypass has been used since 1972. The graft is usu-tensive strictures, marked irregularity or pocketing ally isoperistaltic.of the oesophagus, and the need for frequent dilata-
    • Alaa Hamza 68 Figure 8.1 Figure 8.2 All patients are given intestinal antiseptics 3 days be- After incising the skin, subcutaneous tissue and pla- fore surgery (metronidazole and colimycin). Colonic tysma the cervical fascia is opened along the anterior washouts are done three times per day 48 h prior to border of the sternomastoid. Dissection continues surgery. Patients with a gastrostomy have saline infu- with the strap muscles either divided (easier for dis- sions through the tube at 20 ml/kg body weight over section) or retracted. Internal jugular vein and the 30 min. This is repeated three times every 2 h. Intra- common carotid artery all retract laterally. The oe- venous cephalosporin and metronidazole are given sophagus is identified and the dissection distal to the with premedication. stricture is started to avoid proximal devasculariza- The patient is placed in the supine position with a tion. Isolation of the oesophagus is done after visual- small sandbag under the shoulder with the neck ex- ising the recurrent laryngeal nerve and retracting it tended and turned to the right side. A tube is placed medially. If the oesophagus is severely adherent to through the nose into the oesophagus to allow easy the trachea, distal dissection and identification of the dissection. Skin preparation includes neck, chest and nerve at its entry to the neck is important to avoid abdomen. nerve injury. Now the oesophagus is encircled with a Left transverse supraclavicular incision is made, tape and mobilized proximal to the strictured seg- which can be extended upwards in a hockey stick ment for only 2–3 cm to prevent devascularization in-8 manner over the anterior border of the sternomas- jury of the blood supply. Distal dissection, around the toid. If oesophagostomy is present stay sutures are oesophagus is usually done bluntly through the pos- placed around the oesophagus and an elliptical inci- terior mediastinum. sion around the oesophagostomy is made. Dissection of the oesophagus should not extend proximally more than 4–5 cm to avoid ischaemic injury to the wall. Figure 8.3 The abdomen is entered through a midline incision. site (insert). Usually division of the middle colic ves- Mobilization of the colon is done carefully and it sels is needed and before that the blood supply is should be freed from the ascending to the descend- clamped by bulldog clips and the colon is left inside ing colon and exteriorized for examination of the the abdomen to verify adequate circulation. If there vascular supply. The graft is chosen on the territory are any vascular anomalies, the right or even the supplied by the upper left colic artery with the length middle colic artery are utilized. equal to the distance from the antrum to the stricture
    • Chapter 8 Colonic Replacement of the Oesophagus 69 Figure 8.1 Figure 8.2 Esophagus Strap muscle Sternocleidomastoid Recurrent laryngeal nerve muscle Figure 8.3 Medial colic nerve Resection line
    • Alaa Hamza 70 Figure 8.4 The dissection starts by incising the left triangular kept very close to the oesophageal wall to avoid inju- ligament of the liver followed by dissection of the oe- ry to the surrounding structures sophagus at the hiatus and incising the phreno-oe- With blunt and sharp dissection the oesophagus is sophageal ligament. The vagi are evident at this stage freed as high as possible. Care is taken to avoid enter- and both are divided. Sometimes the posterior vagus ing the pleura or an intercostal tube drain has to be can be saved with meticulous intra-thoracic dissec- inserted. The blunt dissection is continued from tion. The oesophagus is encircled with a tape to facil- above and below until the oesophagus is freed com- itate mobilization. The hiatus is explored utilizing pletely. It is essential to avoid aggressive dissection in two malleable retractors. Under direct vision all oe- the region of the aortic arch and to stay close to the sophageal vessels are diathermized. Traction is ob- oesophageal wall. tained with the help of the tape and the dissection is8 Figure 8.5, 8.6 The two tapes encircling the oesophagus are both used for the passage of the colon through the hiatus moved up and down to be sure of having freed the later on. oesophagus from all attachments. Oesophagectomy The colon is re-evaluated and the pulsation of the is then done by dividing the oesophagus at the cardia marginal artery is carefully examined. The exact with occlusion of the gastric end with an intestinal measurement of the colon is examined after oesoph- clamp. The oesophagus is then passed upward by ageal resection; extra length leads to redundancy lat- traction with a long silk suture to the gastric end of er on. The graft is washed with diluted povidone io- the oesophagus. The silk suture is left in place to be dine solution and left open with no clamps.
    • Chapter 8 Colonic Replacement of the Oesophagus 71 Figure 8.4 Esophagus Stomach Liver Anterior vagal nerve Figure 8.5 Figure 8.6
    • Alaa Hamza 72 Figure 8.7 Then, the colon is resected and passed behind the the cervical and gastric end, avoiding injury to the stomach in an isoperistaltic manner taking care to pedicle. avoid either stretch or torsion of the pedicle. To facil- If the oesophagus has not been resected and a co- itate passage through the chest, the silk suture previ- lon bypass procedure is planned, then a retrosternal ously present is sutured to the proximal end of the tunnel is made by blunt dissection, dividing the en- colon and pulled through the cervical incision until dothoracic fascia very close to the sternum, at the the colon is in place in the posterior mediastinum. upper end from the neck incision after division of the Care should be taken regarding the position of the muscles at the supra sternal notch and at the lower pedicle and repositioning should be done immedi- end by incising the posterior aspect of the lower end ately in case of torsion or traction of the vessels. Vi- of sternum. The tunnel is enlarged using fingers, tak- ability of the graft is confirmed by noting bleeding ing care not to injure the pleura, and a long silk su- from its cervical end. Redundant parts are resected at ture is passed through the tunnel.8 Figure 8.8 The oesophageal end is examined to rule out any Suturing the strap muscles is important to avoid proximal strictures. According to the oesophageal blowing of the neck during swallowing. Closure of size and disparity to the colonic end, the type of the wound is done in layers, leaving a drain in place. anastomosis is chosen. If both sizes are equal or In cases of caustic pharyngeal strictures the pha- without marked disparity, an end-to-end single layer ryngo-colic anastomosis is made as an end-to-side to anastomosis is made using 4/0 absorbable sutures. If the wall of the pharynx. First, the incision should ex- the colonic end is slightly bigger, a posterior incision tend to the angle of the mandible. Then, the dissec- of the oesophagus to accommodate a larger size of tion should reach the wall of the pharynx, opened on colon can facilitate the anastomosis. A single layer, stay sutures, and healthy mucous membrane should end-to-side, oesophago-colic anastomosis is made, be available for anastomosis. The colonic graft with closure of the colonic stump if the oesophagus should be long enough to reach the pharynx. A wide is much smaller in diameter than the colon. Fixation single-layer end-to-side anastomosis is made with no of the colon to the neck muscles is done to avoid trac- tension. Sometimes a wide bore tube is left as a splint tion. for 1 week and endoscopy is done before discharge to check the anastomosis.
    • Chapter 8 Colonic Replacement of the Oesophagus 73 Figure 8.7 Figure 8.8
    • Alaa Hamza 74 Figure 8.9 After passage of the colonic graft to the neck the gas- be taken to close the window after the colonic resec- tro-colic anastomosis is performed in two layers. It is tion and this could be achieved by fixing the colon to done at the cardia with a 270º anti-reflux wrap of the the edge of duodenum. In patients with no gastrosto- stomach to avoid injury to the pedicle. In cases of ret- my, a Stamm-type gastrostomy is preformed. The ab- rosternal colon the anastomosis is made to the ante- domen is closed in layers with a mediastinal drain. rior wall of the stomach near the antrum and the co- Patients usually stay in the Intensive Care Unit for lon should be positioned correctly since it could be 2–4 days. The drains are removed after 48 h, and the hinged by the liver edge. The colon should be fixed to patients are fed by the gastrostomy for 7–10 days. A the edges of the tunnel in cases of retrosternal colon contrast study is performed and, if there is no leak- and to the edge of the hiatus in cases of posterior age, feeding is started The gastrostomy tube is mediastinal colonic replacement. clamped and removed 3 months after surgery unless Pyloroplasty is done in all cases with posterior there is dysphagia. In cases with proximal anasto- mediastinal replacement. It is performed as a Heinz- motic strictures, if dilatation is unsuccessful, surgical Mickulikz type with single-layer anastomosis. The revision of the colo-oesophageal anastomosis is colo-colic anastomosis is performed and care should undertaken.8
    • Chapter 8 Colonic Replacement of the Oesophagus 75 Figure 8.9
    • Alaa Hamza 76 CONCLUSION We share the view of many authors, that an isoperi- right colon and fewer problems are associated with staltic left colon segment based on the left colic ves- its removal. The colon has proved to be relatively ac- sels is the best method of oesophageal replacement id-resistant, and significant ulceration in the inter- for benign caustic oesophageal strictures in children. posed segment is unusual. A sufficient length is available to replace the whole In a survey of the last 475 cases, we had five deaths oesophagus and even the lower pharynx if needed. related to respiratory problems. No instance of graft The blood supply from the left colic vessels is robust necrosis occurred in this series; however, three pa- and rarely prone to anatomic variation. The close re- tients developed late graft stenosis, two of which lationship between the marginal vessels and the bor- were at the distal part. Both patients required surgi- der of the viscus results in a straight conduit with lit- cal revision, and the third patient developed an un- tle redundancy or tendency to kinking. The left colon usual proximal stenosis that was corrected by gastric seems to transmit solid food more easily than the pull-up. SELECTED BIBLIOGRAPHY8 Bahnassy AF, Bassiouny IE (1993) Esophagocoloplasty for Hamza AF, Abdelhay S, Sherif H et al (2003) Caustic esopha- caustic strictures of the esophagus: changing concepts. Pe- geal strictures in children: 30 years experience. J Pediatr diatr Surg Int 8 : 103 Surg 38 : 828–833 Bassiouny IE, Bahnassy AF (1992) Transhiatal esphagectomy Spitz L (1988) Esophageal replacement in children. In: Coran and colonic interposition for caustic strictures. J Pediatr A, Fonkalsrud E, O’Neil J, Grosfeld J (eds) Pediatric surgery, Surg 27 : 1091–1096 6th edn, Mosby Year Book, St Louis Freeman NV, Cass DT (1982) Colon interposition: a modifica- tion of the Waterstone technique using the normal esopha- geal route. J Pediatr Surg 17 : 17–21
    • CHAPTER 9 Gastric Transposition for Oesophageal Replacement Lewis Spitz INTRODUCTIONWhile every attempt should be made to retain the Gastric transposition has been my procedure ofchild’s own oesophagus there are circumstances in choice for oesophageal replacement for over 20 years.which this aim cannot be achieved. These include: It has the following advantages:í Oesophageal atresia: in particular very long gap í The stomach has an excellent blood supply. pure atresia where delayed primary anastomosis í Adequate length to reach the cervical region can has failed, and, in addition, complicated oesopha- usually be achieved. geal atresia when the primary repair has disrupt- í The procedure involves a single anastomosis. ed and a cervical oesophagostomy established í The leak and stricture rates are relatively low.í Caustic oesophageal damage that fails to respond í The procedure itself is simple to perform. to dilatationí Injuries to the oesophagus by prolonged foreign It is recommended that bowel preparation is carried body impaction out to ensure an empty colon in the event that theí Tumours of the oesophagus, e.g., diffuse leiomat- stomach is unavailable for the transposition proce- osis, inflammatory pseudo-tumour dure. The surgeon should be capable of performingí Motility disorders the various alternative methods of oesophageal re- placement.There are four recognized methods of oesophagealsubstitution, which include:í Colon interpositioní Gastric tube oesophagoplastyí Jejunal interpositioní Gastric transposition
    • 78 Lewis Spitz Figure 9.1 A midline upper abdominal incision is made. An el- encounters any difficulty in mobilizing the thoracic liptical incision around the cervical oesophagostomy oesophagus, which may have been damaged by caus- or, alternatively, a right or left low transverse cervical tic oesophagitis or repeated attempts at retaining the incision is made to expose the cervical oesophagus.A child’s own oesophagus. lateral thoracotomy may be required if the surgeon9 Figure 9.2 The stomach is exposed, the gastrostomy site is taken the stomach continues proximally by dividing the down and the defect in the stomach closed. The short gastric vessels between the fundus of the stom- greater and lesser curvatures of the stomach are mo- ach and the spleen and by ligating and dividing the bilized, preserving the integrity of the right gastroep- left gastric artery and vein. iploic and right gastric arcades. The mobilization of
    • Chapter 9 Gastric Transposition for Oesophageal Replacement 79 Figure 9.1 Figure 9.2
    • 80 Lewis Spitz Figure 9.3 The stump of the distal oesophagus (in the case of anterior and posterior vagal nerves are divided. The long gap atresia) is mobilized from the posterior oesophagus is divided at the oesophago-gastric junc- mediastinum by dividing the phreno-oesophageal tion and the defect in the stomach repaired. membrane and dissecting out the oesophagus. The9 Figure 9.4 A pyloroplasty is performed. The sutured gastrosto- terials are placed in the fundus. The orientation of my site and the closed-off gastro-oesophageal junc- these sutures is used to ensure that rotation of the tion are shown. The highest point on the stomach stomach does not occur while it is being pulled up and the place for the oesophago-gastric anastomosis into the neck. is the top of the fundus. Two sutures of different ma-
    • Chapter 9 Gastric Transposition for Oesophageal Replacement 81 Figure 9.3 Figure 9.4
    • 82 Lewis Spitz Figure 9.5 Via the cervical incision, full thickness of the oesoph- avoided as the vascularity of the oesophagus will be agus is mobilized. It is easy to enter into the submu- impaired. The recurrent laryngeal nerves must be cosal plane during the dissection but this should be preserved during the mobilization procedure.9 Figure 9.6 The plane of dissection for the mediastinal tunnel is If any problems are encountered in creating the directly anterior to the prevertebral fascia. From posterior mediastinum tunnel by blunt finger dissec- above the dissection proceed immediately posterior tion, it is advisable to perform a lateral transpleural to the trachea posteriorly and caudally into the pos- thoracotomy and complete the dissection under di- terior mediastinum. From below, through a widened rect view. This approach is also essential to remove a hiatus, dissection is carried out under vision in the scarred oesophagus or a tumour of the oesophagus. prevertebral space behind the heart. The tunnel is completed from above and from below by gentle dig- ital dissection in the posterior mediastinum.
    • Chapter 9 Gastric Transposition for Oesophageal Replacement 83 Figure 9.5 Figure 9.6
    • 84 Lewis Spitz Figure 9.7 Using the “stay-sutures” as guides, the stomach is sition should be smooth and under no tension, and pulled up through the hiatus in the diaphragm, the stay-sutures should be correctly orientated to through the posterior mediastinal tunnel until the avoid twisting of the stomach in the posterior medi- fundus appears at the cervical incision. The transpo- astinum.9 Figure 9.8 The anastomosis between the end of the cervical oe- at the cervical incision. A feeding-tunnelled jejunos- sophagus and the top of the fundus of the stomach is tomy is highly recommended for infants with oe- fashioned using a single layer of 5/0 or 6/0 sutures, sophageal atresia who have not previously been es- taking the full thickness of the walls of the oesopha- tablished on oral feeding. In addition to the usual gus and the stomach. Before completing the anterior post-operative management following any major wall of the anastomosis a size 10F–12F nasogastric procedure, it has been our practice to electively para- tube is passed with the tip in the intrathoracic stom- lyse and mechanically ventilate our patients for a ach. The wounds are closed with a soft rubber drain minimum of 48–72 h post-operatively.
    • Chapter 9 Gastric Transposition for Oesophageal Replacement 85 Figure 9.7 Figure 9.8
    • 86 Lewis Spitz CONCLUSION Mortality of this procedure is in the region of 5% Most of the children prefer to take small frequent while the morbidity is significant and includes: meals, although in the older children a normal eating í Anastomotic leak rate 12% pattern is generally established. Many of the patients í Anastomotic stricture rate 19.6% grow at a slower rate than normal and are in the low- í Swallowing problems 30% er half of the growth charts for both weight and í Delayed gastric emptying 8.7% height. This applies particularly to children who are í Complications with the jejunal feeding tube 4% born with oesophageal atresia. í Dumping syndrome 3% SELECTED BIBLIOGRAPHY Ludman L, Spitz L (2003) Quality of life after gastric transposi- Spitz L (1998) Esophageal replacement. In: O’Neill JA, Rowe tion for oesophageal atresia. J Pediatr Surg 38 : 53–57 MI, Grosfeld JL, Fonkalsrud EW, Coran AG (eds) Pediatric Spitz L (1984) Gastric transposition via the mediastinal route Surgery, 5th edn. Mosby Year Book, St Louis, pp 981–995 for infants with long-gap esophageal atresia. J Pediatr Surg Spitz L, Kiely EM, Pierro A (2004) Gastric transposition in chil- 19 : 149–154 dren – a 21-year experience. J Pediatr Surg 39 : 276–281 Spitz L (1995) Gastric transposition of the esophagus. In: Spitz L, Coran AG (eds) Pediatric surgery, 5th edn. Chapman and9 Hall, London, pp 152–158
    • Part III Chest
    • SPRINGER SURGERY ATLAS SERIES Series Editors: J. S. P. Lumley · J. R. Siewert
    • CHAPTER 10 Thoracoscopy Klaas Bax INTRODUCTIONPediatric surgeons have been involved in thoracosco- í Sequestrationpy for a long time. Until the late 1980s most thoracos- í Lobectomycopies were purely diagnostic. The explosion of en- í Metastasesdoscopic surgical techniques came shortly after the í Mediastinumintroduction of the chip camera and video technolo- í Thymusgy into surgery. Since that time most of the opera- – Thymectomytions that have been classically performed through a í Heart and great vesselsformal thoracotomy can now be performed in a vid- – Closure of the ductus arteriosuseo-assisted way using a number of small access holes. – Pericardial cystsThe term VATS is often used and stands for video-as- – Vascular accesssisted thoracoscopic surgery. This technique pro- í Esophagusvides excellent view of the internal thoracic anatomy. – AtresiaAdditionally, it avoids trauma to the thoracic wall not – Achalasiaonly as a result of the transection of the various tis- – Duplicationsues but also because of the spreading of the ribs. – Esophagectomy for caustic burn The following are indications for VATS: í Sympathetic chainí Diagnostic procedures – Neurogenic tumours í Interstitial lung disease – Sympathectomy for hyperhydrosis í Metastatic lung disease í Thoracic duct í Mediastinal lesions – Ligationí Therapeutic procedures í Spine í Chest wall í Anterior spinal fusion í Empyema í Diaphragm í Pectus excavatum correction according í Diaphragmatic hernia, eventration, to the Nuss method relaxation í Trachea and lungs í Diaphragmatic pacing í Tracheomalacia í Pneumothorax Thoracoscopy has also gained a definitive position in í Bronchogenic cysts the treatment of childhood and adolescent cancer.
    • Klaas Bax 90 Figure 10.1 A good working space is of paramount importance Such a technique, however, is not simple and de- for good quality VATS. In children the working space mands a high level of expertise as well as time. is very limited, a greater reason to make all available Instead of one-lung ventilation, the lung can be space available. The organ that limits the working pushed out of the way by inflating the ipsilateral tho- space during VATS is most usually the lung. Tech- rax with CO2. Pressures of up to 5 mmHg at a flow of niques have been developed to keep the lung out of 2 l/min are well tolerated, even in the neonate. After a the way. short while, the ipsilateral lung collapses, and once By allowing air to enter the pleural cavity, the lung that stage is reached an even lower inflation pressure will collapse. However, VATS is usually done under usually suffices. In order to maintain the CO2 pneu- general anaesthesia and positive pressure ventila- mothorax, valved cannulae have to be used. The res- tion. As a result, the lungs expand during each insuf- piratory pressure will be increased by the same flation. One-lung ventilation by selective intubation amount as the CO2 inflation pressure and hypercap- of the main bronchus with a cuffed tube (Fig. 1) or by nia will occur as a result of CO2 absorption. Both can the use of a double lumen tube is a good alternative be managed by adjustment of the ventilator settings, but only applicable in larger children, e.g., children e.g., by increased rate and minute volume. Close col- over 10 years of age. In children below 10 years of age, laboration between surgeon and anaesthesiologist is one lung ventilation is theoretically possible by ven- mandatory. It is very important that the surgeon is tilating the child endotracheally while the ipsilateral patient enough to allow the body to seek a new equi- main bronchus is blocked with a Fogarty catheter. librium.10 Figure 10.2 The surgeon, the operative target area and the screen mediastinal surgery. The cameraman, when right- should be inline. This means that the surgeon stands handed, usually stays to the left of the surgeon. The behind the back of the patient for anterior mediasti- scrub nurse usually stands on the opposite side. nal surgery and in front of the patient for posterior
    • Chapter 10 Thoracoscopy 91 Figure 10.1 Figure 10.2 Scrub nurse Anesthesiologist Surgeon Assistant
    • Klaas Bax 92 Figure 10.3, 10.4 Gravitational forces should also be used to get the opened and air is sucked into the chest. Next a cannu- lung out of the way. This means that the position of la with blunt trocar is inserted. The hole in the thorax the child on the table has to be adjusted to take max- wall for the cannula should be as small as possible so imal advantage of these forces. that the tissues fit snugly around the cannula in order For anterior mediastinal surgery a three-quarters to avoid CO2 leakage. All secondary cannulae are in- posterolateral decubitus position should be chosen; serted in the same way but under concomitant tele- for posterior mediastinal surgery rather a three- scopic control. quarters anterolateral decubitus position should be Especially in small children, who have a rather used. Moreover, for VATS in the upper part of the thin body wall, cannulae have a tendency to glide fur- chest, the table should be put in a reversed Trenden- ther into the body cavity, thereby further limiting the burg position, whereas for VATS in the lower part of working space, or to glide out. Using radially expand- the chest the table should be put in a Trendelenburg able cannulae may lessen this. Cannulae with a screw position. When all above measures are taken, usually on the outer side should not be used, as these will be no retractors are needed. If they are needed, they pulled out resulting in a rather large hole. The best should be used with care as they can easily damage way to prevent this gliding in and out is to put a snug- the organ that has to be retracted. ly fitting sleeve of silastic tubing around the cannula. The cannulae can be inserted in a closed or open The sleeve can then be sutured to the skin. Alterna- way. When the closed way is chosen, a radially ex- tively the stopcock of the cannula is sutured to the pandable cannula is usually used. A Veress needle skin and circular tape is applied around the cannula with radially expandable sheet is punctured through and tied at the base. the intercostal space at the desired place. Air is al- The most ergonomic position of the cannulae is10 lowed to enter the chest through the needle so that triangular or V-shaped. The tip of the V is directed the pleurae detach. The Veress needle is then re- towards the surgeon and the open side of the V to- moved and the sheath left behind for dilatation with wards the patient. The telescope cannula is inserted the cannula and blunt trocar. at the tip of the V while the cannulae for the working In the open way, a small incision is made through instruments are positioned at the top of the limbs of the skin. Next, the wound is deepened just over the the V. Ideally the angle of the V should be around 60°. upper border of the rib until the pleural cavity is
    • Chapter 10 Thoracoscopy 93 Figure 10.3 Figure 10.4
    • Klaas Bax 94 Figure 10.5 The smaller the diameter of the telescope, the less Stapling devices require an 11-mm diameter can- good the quality of the picture is and the less light nula, which is enormous for small children. Such a that can be transmitted. Telescopes with a diameter cannula will damage the intercostal space in small of 5 mm are of sufficient quality to be used for all en- children and should therefore be avoided. Moreover, doscopic operations in children. The optical axis can these staplers need a deep working space to allow the vary with the axis of the physical axis of the telescope stapling beak to be opened and closed. from 0° to 75°. The most commonly used scopes have Tying and especially suturing of structures are con- an angle of 30°. In contrast to 0° scopes, angled sidered to be the most difficult endoscopic surgical scopes allow one to look around structures, which tasks and are the Achilles heel of endoscopic surgery. has great advantages. Most telescopes have a length Tying of blood vessels structures has been largely of 33 cm. For use in small children, the 24-cm length eliminated by the availability of new energy-applying is advantageous. systems, which allow even large blood vessels to be For most endoscopic surgical operations in chil- well sealed. Pre-tied loops can be used for, for exam- dren, instruments with a diameter of 3.5 mm, to be ple, tying-off leaking lung tissue. There are also dis- used in conjunction with cannulae with a diameter of posable suturing devices on the market but these 3.8 mm, are appropriate. In neonates and infants, 20- have a diameter of 10 mm. cm long instruments instead of 30-cm long ones The problem of suturing is certainly not solved at should be used. These 3.5 mm instruments can be the present. A major problem in endoscopic suturing used with monopolar high-frequency electrocautery is the introduction of the needle. Most needles just (HFE), which suffices for most operations in smaller don’t fit 3.8 mm cannulae. In small children, the nee- children. dle can be put directly through the body wall. Once10 Ligating loops can be used to seal leaking lung or the suturing has been finished, the needle has to be the take a lung biopsy. directed back through the wall. This process is time For the application of bipolar HFE or of ultrason- consuming especially when many sutures have to be ic energy, the minimal diameter of the instrument is applied as, for example, in oesophageal anastomosis. 5 mm. This also applies for the endoscopic Ligasure Another possibility is to straighten the curved needle instrument, which is a sophisticated bipolar HFE in- so that it will fit together with the needle holder strument allowing one to seal vessels with a diameter through the cannula. The tying of the knot can be up to 7 mm in diameter. These 5-mm instruments are done extracorporeally or intracorporeally. rather long to be used in small children. Clipping devices also have a minimal diameter of 5 mm and are quite long.
    • Chapter 10 Thoracoscopy 95 Figure 10.5
    • Klaas Bax 96 CONCLUSION VATS has revolutionized surgery not only in adults omy can now be performed using VATS. VATS gives a but also in infants and children. Almost all opera- perfect view of the anatomy and dissection is not tions were classically performed through a thoracot- particularly difficult. The difficulty is suturing. SELECTED BIBLIOGRAPHY Bax KM, van der Zee DC (2002) Feasibility of thoracoscopic re- Rothenberg SS (2000) Thoracoscopic lung resection in chil- pair of esophageal atresia with distal fistula. J Pediatr Surg dren. J Pediatr Surg 35 : 271–274 37 : 192–196 Roviaro GC,Varoli F,Vergani C, Maciocco M (2002) State of the Cury EK, Schraibman V, De Vasconcelos Macedo AL, Eche- art in thoracoscopic surgery: a personal experience of 2000 nique LS (2001) Thoracoscopic esophagectomy in children. videothoracoscopic procedures and an overview of the lit- J Pediatr Surg 36 : E17 erature. Surg Endosc 16 : 881–892 Maher JW, Conklin J, Heitshusen DS (2001) Thoracoscopic es- Smith TJ, Rothenberg SS, Brooks M, Bealer J, Chang J, Cook BA, ophagomytomy for achalasia: preoperative patterns of acid Cullen JW (2000) Thoracoscopic surgery in childhood can- reflux and long-term follow-up. Surgery 130 : 570–576 cer. J Pediatr Hematol Oncol 24 : 429–43510
    • CHAPTER 11 Repair of Pectus Excavatum Robert C. Shamberger INTRODUCTIONPectus excavatum is a congenital deformity of the an- Determination of the subject’s appropriateness forterior chest wall. It consists of two primary elements. repair is dependent upon multiple considerations.The first component is posterior depression of the These include the degree of psychologic distressbody of the sternum generally beginning at the level created by the deformity, the extent of impairment ofof the insertion of the second or third costal cartilag- physical activity by cardiopulmonary symptoms, andes. The second component is posterior depression of results of the pulmonary function and physiologicthe attached costal cartilages. This depression gener- exercise studies.ally involves ribs 3–7 and sometimes will extend to Techniques for repair of pectus excavatum havethe level of the second costal cartilage. In older teen- evolved significantly since it was first repaired inagers the posterior depression of the ribs will involve 1911. Modern approaches date to 1949 when Ravitchpart of the osseous as well as the cartilage compo- first reported a technique that involved excision of allnent. This is a congenital deformity and in greater deformed costal cartilages with the perichondrium,than 90% of children it will be apparent within the and division of the xiphoid and the intercostal bun-first year of life. It has an increased frequency of oc- dles from the sternum. A sternal osteotomy wascurrence in families with a history of chest wall de- created and the sternum was secured anteriorly withformity, and has been estimated to have an incidence Kirschner wire fixation. This approach was modifiedof 1 in 300 to 1 in 400 births. by Baronofsky (1957) and Welch (1958) when they The physiologic implications of pectus excavatum stressed the need for preservation of the perichon-have been evaluated for the last four decades. It has drial sheaths to allow optimal cartilage regenerationbeen demonstrated that a “restrictive” defect occurs for durability of the repair. Fixation with metallicin individuals with pectus excavatum. The total lung struts anterior to the sternum was the next modifica-capacity and the vital capacity are decreased relative tion developed by Rehbein and Wernicke in 1957. Ret-to normative values. The values for an individual of- rosternal strut fixation was described by Adkins andten do not fall out of the “normal range” but, taken as Blades in 1971. While recent innovations for strut fix-a group, individuals with pectus excavatum do have ation have included the use of such materials as bio-decreased pulmonary volume compared with nor- absorbable struts, Marlex mesh or Dacron vascularmals. The extent of this impairment is variable and it graft, no evidence demonstrates that these are betterdepends upon the severity of the depression and the than traditional metallic struts.depth of the chest. The second physiologic impair- In 1998 Donald Nuss first described a method forment which has been demonstrated is a decrease in repair of pectus excavatum utilizing a heavy metalthe filling capacity of the heart, in particular the right strut to displace anteriorly the sternum and de-ventricle. This is produced by anterior compression pressed costal cartilages. It did not require resectionfrom the depressed sternum. Studies dating back to or remodelling of any of the costal cartilages. In thisthose of Beiser have shown a decreased stroke vol- chapter, I will present both the current open tech-ume, particularly in the upright position, associated nique with its modifications that I utilize, as well aswith significant chest wall deformity. While subse- the innovative Nuss technique, which is also knownquent studies have shown variable results when using as the minimally invasive repair of pectus excavatumradioisotope techniques, this impairment is clearly (MIRPE). The latter technique is still awaiting out-one of the components of decreased cardiopulmo- come analysis. The first report by Nuss of 42 patientsnary function in patients with severe pectus excava- utilized a fairly young cohort in which the mediantum. Workload studies have demonstrated that indi- age was 5 years. A subsequent report by Croitoru inviduals with pectus excavatum develop symptoms of 2002 utilizing this method included a larger and old-fatigue earlier in gaited exercise protocols than do er cohort of 303 patients. In that group only 23.4% ofnormal probands. Two studies by Cahill in 1984 and the patients had the bars removed.Peterson in 1985 have also demonstrated that follow-ing repair of the chest wall deformity, the level of theexercise tolerance has increased.
    • Robert C. Shamberger 98 Figure 11.1a,b A transverse skin crease incision is placed below and deformity and inferiorly to the tip of the xiphoid (b). within the nipple lines (a). In females, it is of particu- The flaps are developed just anterior to the pectoral lar importance to see that this is placed in the future fascia to keep them well vascularized. The pectoral inframammary crease to avoid unsightly tethering of muscles are then elevated off the sternum being cau- a scar between the two breasts. The skin flaps are tious to preserve all of the muscle and overlying fas- then elevated superiorly to the level of the apex of the cia intact.11 Figure 11.2 Figure 11.3 To facilitate identification of the appropriate plane of Incisions are then placed through the perichondrial dissection, the muscle is first elevated just anterior to sheaths parallel with the axis of the cartilage. It is one of the costal cartilages. When this plane is de- helpful to keep the incision on the flat anterior aspect fined, an empty knife handle is then inserted anteri- of the rib. The perichondrial sheaths are dissected off or to the costal cartilage and passed laterally. It is the costal cartilage utilizing perichondrial elevators. then replaced with a right angle retractor to elevate Freeing the edge of the perichondrium from the me- the muscle anteriorly. This step is then repeated ante- dial aspect of the rib provides better visualization of rior to the next costal cartilage just above or below the posterior aspect of the cartilage facilitating this the first rib defined. Elevation of the muscle flap in process. The cross-sectional shape of the ribs must between the two right angle retractors facilitates be remembered. Ribs 2 and 3 are fairly flat. Ribs 4 and identification of the correct plane of dissection. The 5 are round, and ribs 6 and 7 have a narrow width and origin of the salmon-coloured pectoral muscles are greater depth. divided with electrocautery making certain to stay out of the intercostal bundles, which are covered with a glistening white fascia. Injury of the intercostal bundles can result in significant bleeding. The mus- cle flaps are mobilized laterally to the costochondral junction or to the lateral extent of the deformity. Generally cartilages 3–7 are involved, but sometimes the second cartilage is as well.
    • Chapter 11 Repair of Pectus Excavatum 99 Figure 11.1a,b a b Figure 11.2 Figure 11.3
    • Robert C. Shamberger 100 Figure 11.4 Figure 11.5 The medial aspect of the cartilage is then incised The wedge osteotomy is then created on the anterior from the sternum (see insert) with the posterior as- surface of the sternum at the apex of the deformity. pect protected by the perichondrial elevator. Incising The segment of bone is then mobilized using one of the cartilage directly adjacent to the sternum will al- the wings of the perichondrial elevators, but without so minimize the risk of injury to the internal mam- entirely dislodging it from the sternum. Leaving it mary vessels, which are generally 1 to 1.5 cm lateral to partially in place will facilitate more rapid healing of the margin of the sternum. To minimize any impair- the fracture. ment of subsequent growth of the ribs, 1 to 1.5 cm of the costal cartilage is preserved with the costochon- dral junction.11 Figure 11.6 Figure 11.7 The sternum is then elevated with a towel clip and A retrosternal strut is tunnelled posterior to the ster- posterior pressure is applied to the upper portion of num. This retrosternal tunnel is made by partially di- the sternum to fracture the posterior sternal plate. viding one of the perichondrial sheaths directly adja- While in the past the xiphoid was divided along with cent to the sternum. A tunnel is then created posteri- the rectus muscle from the tip of the sternum, I cur- or to the sternum with a Schnidt clamp, which is rently avoid this step. This minimizes the occurrence brought out directly adjacent to the sternum to avoid of an unsightly depression at the base of the sternum. injury to the internal mammary vessels on the con- Using a posterior sternal strut it is also unnecessary tralateral side. Prior to passing the strut behind the to divide the lower perichondrial sheaths as was done sternum, it is preformed so that there is a slight in- in the past. This division of the lower perichondrial dentation in which the sternum will sit and the strut sheaths also contributed to the depression below the is curved somewhat posteriorly on each end to allow sternum. If the xiphoid produces an unsightly pro- it to conform to the shape of the ribs and avoid any trusion when the sternum is in its corrected position, unsightly protrusions into the skin and the muscle. it can be divided from the sternum using a lateral ap- The Schnidt clamp is then used to draw the strut be- proach with cautery. This avoids taking down the hind the sternum with the concave portion of the rectus attachment. strut anterior. Once it is behind the sternum and in an appropriate position just anterior to the ribs on each side, it is rotated 180°. It is important in this step to make certain that the strut is deep to the pectoral muscle flap to provide adequate soft tissue coverage over the strut. The strut is then secured to the perios- teum laterally with two heavy no. 0 absorbable su- tures. This will secure the strut in position.
    • Chapter 11 Repair of Pectus Excavatum 101 Figure 11.4 Figure 11.5 Figure 11.6 Figure 11.7
    • Robert C. Shamberger 102 Figure 11.8 Figure 11.9 This depicts the position of the retrosternal strut For the Nuss procedure two incisions are made at the from an anterior perspective with it secured to the mid-axillary line at the level of maximal sternal de- ribs on each side. The pectoralis major muscle flaps pression. A Lorenz tunneller or long clamp is then are then approximated over the sternum. The flaps passed through one lateral incision along the chest are advanced inferiorly to compensate for the fairly wall, and enters into the pleural cavity at the inner as- bare lower portion of the sternum. This allows it to pect of the pectus ridge. It is tunnelled behind the be covered with soft tissue. At the inferior aspect the sternum and anterior to the pericardium and it is flap is attached to the rectus muscle with interrupted brought out the contralateral side. The point of exit absorbable sutures. from the thorax is also aimed at the inner aspect of the pectus ridge. Thereafter, it is passed along the outside of the chest wall and out through the skin at the anterior axillary line. An umbilical tape is then grasped by the clamp or Lorenz tunneller and brought through the tunnel. Two tapes are often used in case one breaks. Several adaptations have been uti- lized to minimize the risk of cardiac injury from this manoeuvre. The first adaptation now widely utilized involves a thoracoscope to monitor the passage of the tunneller behind the sternum. A second adaptation less frequently used is to make a small incision at the tip of the sternum through which a bone hook can be inserted. The sternum is elevated anteriorly as the clamp is passed across the chest to broaden the retro- sternal space.11 Figure 11.10 The preformed strut which has been pre-measured patient’s chest is then brought through the chest and and bent to make certain that it fits the breadth of the passed so that the concave surface is anterior.
    • Chapter 11 Repair of Pectus Excavatum 103 Figure 11.8 Figure 11.9 Figure 11.10
    • Robert C. Shamberger 104 Figure 11.11 Figure 11.12 Once the bar is in position, it is rotated 180° with a The most frequent complication of this procedure special “Lorenz flipper” to elevate the sternum and when it was initially performed was rotation of the costal cartilages. During this manoeuvre the skin and Lorenz strut. To reduce this risk, a “stabilizer” may be muscle flaps are elevated over the end of the bar so attached to both sides of the strut with heavy no. 3 that the bar sits directly along the chest wall. wire or suture. Once attached to the strut, it is then sutured to the soft tissues of the chest to provide se- cure fixation and prevent rotation of the bar and loss of correction of the deformity.11 Figure 11.13, 11.14 This diagram shows the Lorenz strut in position costal cartilages to correct the pectus excavatum de- prior and after rotation. The bar in the final position formity. The bar is electively removed in 2 to 3 years. is displacing the sternum anteriorly along with the
    • Chapter 11 Repair of Pectus Excavatum 105 Figure 11.11 Figure 11.12 Figure 11.13 Figure 11.14
    • Robert C. Shamberger 106 CONCLUSION The overall results of repair of pectus excavatum the minimal access procedure which have not oc- should be excellent. The peri-operative risks must be curred with the standard open technique include limited. The most significant complication is a major thoracic outlet syndrome and the rare occurrence of recurrence, which has been described in large series a carinate deformity after repair. Occurrence of an al- as occurring in 5 to 10% of patients. A limited pneu- lergic reaction to the metal Lorenz struts has also oc- mothorax requiring aspiration is infrequent and curred in 1% of patients who present with rashes rarely requires a thoracostomy tube. Wound infec- along the area of the bar requiring replacement with tion should be rare with the use of peri-operative bars composed of other alloys. Older patients seem to antibiotic coverage and protective coverage of the encounter significant pain with the minimally inva- skin during the operative procedure to minimize any sive procedure, but quantitative comparisons to the contamination by skin flora. standard open operation have not yet been reported. Long-term outcome of the Nuss procedure in Both techniques appear to achieve excellent cor- teenagers is not well documented at this time as it rection of the deformity. Comparison of complica- has been used for less than a decade in older patients. tion rates of each technique has not yet been accom- The most frequent complication described in early plished, but hopefully a multi-institutional prospec- use of the minimally invasive procedure was rotation tive study of these surgical techniques will define of the strut. Lateral stabilizers have significantly de- their relative benefits and risks. Repair of pectus ex- creased the incidence of this complication. Other cavatum is important for children who are either complications described include pneumothorax, per- psychologically distressed or physiologically im- icarditis, and hemothorax. Complications unique to paired by their deformity. SELECTED BIBLIOGRAPHY11 Croitoru DP, Kelly RE Jr, Goretsky MJ et al (2002) Experience Shamberger RC (2003) Congenital thoracic deformities. In: and modification update for the minimally invasive Nuss Puri P (ed) Newborn surgery. Arnold, London, pp 239–246 technique for pectus excavatum repair in 303 patients. J Pe- Sidden CR, Katz ME, Swoveland BC, Nuss D (2001) Radiologic diatr Surg 37 : 437–445 considerations in patients undergoing the Nuss procedure Hebra A, Swoveland B, Egbert M et al (2000) Outcome analysis for correction of pectus excavatum. Pediatr Radiol 31 : of minimally invasive repair of pectus excavatioum: review 429–434 of 251 cases. J Pediatr Surg 35 : 252–258 Nuss D, Kelly RE Jr, Croitura DP et al (1998) A 10-year review of a minimally invasive technique for the correction of pectus excavatum. J Pediatr Surg 33 : 545–552
    • CHAPTER 12 Pulmonary Malformations Brian T. Sweeney, Keith T. Oldham INTRODUCTIONCongenital lung abnormalities are uncommon and the plain chest radiograph is the best initial diagnos-diverse in their presentations. Congenital lobar over- tic test in the neonate.inflation (CLO), otherwise known as congenital lobar Pulmonary sequestrations make up 10–30% of theemphysema, is among the most common of the con- cystic bronchopulmonary foregut malformations.genital lung anomalies. It is characterized by air trap- They are classified according to whether the seques-ping and overdistension of one or more lobes which tration resides within the visceral pleura of the nor-are otherwise anatomically normal. This distension mal lung (intralobar sequestration) or is invested bycauses compression of adjacent normal lung paren- its own visceral pleura (extralobar sequestration). Inchyma and can result in mediastinal shift and cardi- both types of pulmonary sequestration, however,orespiratory compromise. CLO is believed to result there is no bronchial communication between the se-most commonly from structural deficiency or ab- questrum and the normal tracheobronchial tree. Insence of supportive cartilage in the affected lobar addition, the malformation receives its blood supplybronchus, thereby causing expiratory collapse of the from aberrant systemic arterial vessels.conducting airway with impedance to expiratory Intralobar sequestrations make up about 50–70%flow. CLO is most often seen in the Caucasian popu- of the pulmonary sequestrations and most common-lation with a male preponderance of two or three to ly involve the posterior and basal segments of the leftone. It is most common in the left upper lobe lower lobe. The arterial supply is usually derived(40–50%), with other sites affected less frequently: from aberrant branches of the descending thoracicright middle lobe 30–40%, right upper lobe 20%, and aorta, although occasionally intercostal, brachioce-lower lobes 1%. Approximately half of the patients phalic, or abdominal aortic aberrant vessels are en-develop respiratory distress within the newborn pe- countered. Venous drainage is usually via the asso-riod while the remainder present up to 4 to 6 months ciated pulmonary vein. Extralobar sequestrations areof age or later. Presenting signs are those of respira- completely separated from the normal lung and in-tory embarrassment, including dyspnea, tachypnea, vested by an individual pleura. They are completelyagitation and wheezing. separate from the functional airways. They are found Congenital cystic adenomatoid malformations in the left lower chest most commonly, but may occur(CCAM) are a rare group of cystic lobar hamartoma- anywhere. Rarely, subdiaphragmatic locations are re-tous lesions, represent up to 50–70% of the broncho- ported. A 3:1 male predominance is reported in mostpulmonary foregut malformations in some reports. series for extralobar sequestrations. These sequestra-The lesions are generally large, firm, multicystic tions also derive arterial blood supply from the de-masses that are composed of terminal respiratory scending aorta, with up to 20% having an aberrantstructures, usually bronchiolar in origin. vessel traversing the diaphragm. Since the advent of routine ultrasound in obstetric Patients with intralobar sequestration will typical-practice, the majority of cystic lung lesions are now ly present with pulmonary infections due to abnor-discovered prenatally in many institutions. Serial mal air-space connections with inadequate drainage,ultrasonographic examinations may demonstrate or from compressive atelectasis of adjacent paren-shrinkage or even spontaneous resolution in up to chyma. Extralobar sequestrations, on the other hand,40% of fetal CCAMs. After birth, some neonates are frequently seen on prenatal ultrasound.demonstrate tachypnea, dyspnea, cyanosis or im- Congenital lung cysts comprise up to one-third ofpending respiratory failure. Of the remainder, most bronchopulmonary foregut malformations in somewill present within the first years of life with recur- reports. The most common of these lesions are bron-rent or persistent respiratory infections, pulmonary chogenic cysts. Bronchogenic cysts arise from theabscesses, reactive airway disease or failure to thrive. trachea, bronchus or other conducting airways butAs for all bronchopulmonary foregut malformations, have usually lost their connection with the parent
    • Brian T. Sweeney, Keith T. Oldham 108 structure. They are usually simple, and contain mu- Figure 12.1–12.2 cus; however, air-fluid levels and infection may be seen if there is continuity with the tracheobronchial Lung surgery in children is generally similar to that tree. In contrast to sequestrations, bronchogenic in adults except that the diminutive size, the associat- cysts have a normal bronchial blood supply. Al- ed lesions, and the unique pathologic entities require though bronchogenic cysts may reside anywhere in certain special considerations. Lobectomy can be the respiratory tract, including paravertebral, para- performed by conventional thoracotomy or video- oesophageal, subcarinal and cervical areas, the ma- assisted thoracosocpy. jority are found in the lung parenchyma or mediasti- Lobectomy is the procedure of choice for the treat- num. ment of congenital lobar emphysema, CCAM, intra- Some patients with bronchogenic cysts are lobar sequestrations and some parenchymal lung asymptomatic. Of those with symptoms, the most cysts. The patient is positioned in the lateral decubit- common presentations are wheezing, tachypnea or us position, with the upper arm extended and placed dyspnea, all related to compression of the adjacent over the head. Rolled towels and other positioning conducting airway with partial obstruction. devices may be placed in order to optimize stabiliza- Plain radiograph of the chest will usually demon- tion and exposure of the operative field. strate the pathology of the congenital malformation Optimal exposure is gained by transverse or of the lung. In most infants and children with con- oblique incision over the fourth or fifth intercostal genital malformations of the lung additional imaging space, below and lateral to the nipple to avoid cos- is required. Ultrasound with Doppler, computed to- metic and functional damage to the breast tissue. mography scan with contrast, or magnetic resonance There should be some space between the tip of the imaging provide good anatomical details and dem- scapula and the posterior extent of the incision. This onstrate relationship to the neighbouring structures. becomes important during closure of the muscle Treatment of congenital malformations of the layers, especially if the incision must be extended lung is usually by lobectomy of the affected lobe, posterolaterally. Underlying muscle and subcutane- which is very well tolerated in the infant population. ous tissue is divided along the line of incision by elec- trocautery. To limit postoperative morbidity, it is de- sirable and usually possible to employ a muscle spar-12 ing approach. This affords adequate exposure yet avoids division of the serratus anterior and chest wall musculature other than the latissimus dorsi. The scapula is elevated off the chest wall by retractor to gain exposure, and palpation is used to count the ribs to the correct interspace. In most situations in in- fants, the highest palpable rib is the second. General- ly, the fourth interspace is used for a lobectomy al- though the fifth can be used effectively as well.
    • Chapter 12 Pulmonary Malformations 109 Figure 12.1 Figure 12.2
    • Brian T. Sweeney, Keith T. Oldham 110 Figure 12.3, 12.4 The incision is then continued with electrocautery injury to the lung parenchyma beneath. A rib spread- just superior to the lower rib of the selected intercos- er is then placed to facilitate retraction. The incision tal space to avoid damage to the neurovascular bun- may then be continued anteriorly or posteriorly from dle that runs along the inferior border of each rib. inside the chest if further exposure is needed. Care must be taken when entering the pleura to avoid12 Figure 12.5 The following technique and illustrations are de- al and inferior traction on the lobe exposes the hi- scribed for left upper lobectomy, however, the princi- lum. The visceral pleura is carefully incised circumfe- ples are the same for any lobe resection. Gentle later- rentially, exposing the hilar structures.
    • Chapter 12 Pulmonary Malformations 111 Figure 12.3 Figure 12.4 Figure 12.5 Lt. main pulmonary vein Lt. main pulmonary artery Phrenic nerve Aorta Lt. lung
    • Brian T. Sweeney, Keith T. Oldham 112 Figure 12.6, 12.7 Meticulous dissection reveals the left main pulmo- These are individually encircled, ligated and divided. nary artery as it courses under the aortic arch and This is typically done with heavy silk and using dou- crosses the left upper lobe bronchus. Nearby struc- ble proximal ligatures. The bronchial blood supply tures to be noted are the left phrenic nerve anterio- travelling with the left upper lobe bronchus is like- medially along the mediastinum, and the recurrent wise identified and ligated. Attention is then directed laryngeal nerve branching from the vagus under the to the left upper lobe venous drainage. Again, indi- aortic arch. A review of segmental anatomy of the vidual branches are circumferentially dissected and lung describes four main arterial branches supplying ligated using the same approach as for the arterial the left upper lobe, however this can be variable. circulation.12 Figure 12.8 The bronchus is then clamped and divided. Closure lobe, or it may be done early in the dissection to facil- of the bronchial stump with commercial surgical sta- itate exposure. The superior and inferior pulmonary pling devices is appropriate in older children; howev- vein sometimes have a common stem outside the er, size and other technical limitations make this un- pericardium, which if unrecognized, may necessitate desirable in infants where a simple sewn closure is total pneumonectomy. A chest tube is placed within best. Air leaks may be identified for suture repair by the pleura for drainage, and the wound is closed in filling the chest with warm saline coincident with in- anatomical layers using absorbable suture. Post-op- flation of the residual lobe by the anaesthesiologist. eratively, drains can be removed early, provided no The inferior pulmonary ligament should be divided air leak is demonstrable. at this time to facilitate expansion of the left lower
    • Chapter 12 Pulmonary Malformations 113 Figure 12.6 Figure 12.7 Left upper lobe bronchus Left pulmonary artery Artery branches of the left upper lobe Left vein branches of the left upper lobe Figure 12.8
    • Brian T. Sweeney, Keith T. Oldham 114 CONCLUSION Lung surgery in neonates and infants is generally lung surgery, technical problems may result in seri- similar to that in adults except that the diminutive ous and irreversible consequences. Collaboration size, the associated lesions and the unique patholog- with paediatric anaesthesiologists familiar with the ic entities require certain special considerations. Of unique circumstances of paediatric chest surgery is course, the smaller the child, the more care must be essential. taken in order to avoid technical injury. As with all SELECTED BIBLIOGRAPHY Black TL (2003) Pulmonary sequestration and congenital cys- Lo, HP, Oldham KT (2003) Congenital malformations of the tic adenomatoid malformation. In: Ziegler MM, Azizkhan lung. In: Puri P (ed) Newborn surgery. Arnold, London, pp RG, Weber TR (eds) Operative pediatric surgery. McGraw- 295–307 Hill, New York, pp 445–454 Oldham KT (1997) Lung. In: Oldham KT (ed) Surgery of in- Adzick NS, Harrison MR, Crombleholme TM et al (1998) Fetal fants and children: scientific principles and practice. Lip- lung lesions: management and outcome.Am J Obstet Gyne- pincott-Raven, Philadelphia col 179 : 884–889 Albanese CT, Sydorak RM, Tsau K (2003) Thoracoscopic lobec- tomy for prenatally diagnosed lung lesions. J Pediatr Surg 38 : 553–55512
    • CHAPTER 13 Congenital Diaphragmatic Hernia and Eventration Prem Puri INTRODUCTION Congenital Diaphragmatic Hernia Diagnosis of CDH is made postnatally by plain ra- diography of the chest and abdomen by demonstra- tion of air-filled loops of the bowel in the chest and aCongenital diaphragmatic hernia (CDH) is a malfor- paucity of gas in the abdomen. The diaphragmaticmation characterized by a defect in the posterolater- margin is absent, there is a mediastinal shift to theal diaphragm, the foramen of Bochdalek, through opposite side and only a small portion of the lungwhich the abdominal viscera migrate into the chest may be seen on the ipsilateral side.during fetal life. The reported incidence of CDH var- The mortality rate of infants born with CDH re-ies from 1 in 2200 to 1 in 5000 births. Polyhydramni- mains high, despite optimal perinatal care. The highos is present in 20% of pregnancies involving an in- mortality rate in CDH has been attributed to pulmo-fant with CDH and in 50% of pregnancies associated nary hypoplasia and associated persistent pulmo-with infants with CDH who are stillborn. In most se- nary hypertension. In recent years, newer manage-ries, 80% of posterolateral diaphragmatic hernias ment strategies such as permissive hypercapnia, highhave been reported to occur on the left side and 20% frequency ventilation, extracorporeal membraneon the right side. Bilateral CDH are rare. The size of oxygenation and delayed surgical repair havethe defect varies from small (2 or 3 cm) to very large, emerged in the care of high-risk CDH patients, whichinvolving most of the hemidiaphragm. A rim of mus- offer some hope of improving overall survival.cle is usually present around the defect which is oftencovered posteromedially with peritoneum. A hernialsac, composed of pleura and peritoneum, has beenreported in about 20% of patients. Congenital Eventration of the Diaphragm Widespread use of obstetric sonography has led toan increase in the frequency of antenatal diagnosis of Eventration of the diaphragm has been described asCDH, which is established by demonstration of the an abnormally high or deviated position of all or partabdominal viscera in the chest. Three easily detect- of the hemidiaphragm. Eventration may be congeni-able features – polyhydramnios, mediastinal shift tal or acquired as a result of phrenic nerve palsy.and the absence of an intra-abdominal stomach bub- Congenital eventration is a developmental abnor-ble – should prompt a more careful search for herni- mality which results in muscular aplasia of the dia-ated abdominal organs in the chest. Polyhydramnios phragm. In acquired eventration, the diaphragm,is present in about 80% of the pregnancies with fe- which initially had fully developed musculature, be-tuses who have CDH and has also been associated comes atrophic secondary to phrenic nerve damagewith poor outcome. and disuse. Although this section deals with congen- Postnatally, the most severely affected babies ital eventration, the clinical features and principles ofpresent with respiratory distress (cyanosis, tachyp- management are similar in congenital and acquirednoea and sternal recession) at birth. Other infants de- forms of eventration.velop cyanosis, tachypnoea and grunting respira- Clinical features range from being asymptomatictions within minutes or hours after birth. Physical to severe respiratory distress. Patients may presentexamination reveals a scaphoid abdomen, an in- later in infancy with repeated attacks of pneumonia,creased anteroposterior diameter of the thorax and bronchitis or bronchiectasis. Occasionally, patientsmediastinal shift. Breath sounds are absent on the present later in childhood with gastrointestinalaffected side. Associated congenital anomalies may symptoms of vomiting or epigastric discomfort. Inalso be seen or revealed on further examination. patients with phrenic nerve palsy, there may be a his-CHD presents beyond the first hours of life in tory of difficult delivery. They may present with ta-10–20% cases. chypnoea, respiratory distress or cyanosis. Physical
    • Prem Puri 116 examination reveals decreased breath sounds on the Figure 13.1 affected side, mediastinal shift during inspiration and a scaphoid abdomen. General anaesthesia with muscle relaxation is used. The diagnosis of eventration is usually made on a The baby is positioned supine on a warm blanket. chest X-ray. Frontal and lateral chest X-rays will show The most commonly preferred approach is abdomi- an elevated diaphragm with a smooth, unbroken out- nal. This offers good exposure, easy reduction of the line. Fluoroscopy is a useful investigation for differ- abdominal viscera and recognition and correction of entiating a complete eventration from a hernia. Para- associated gastrointestinal anomalies. A subcostal doxical movement of the diaphragm is seen if com- transverse muscle cutting incision is made on the plete eventration is present. Ultrasonography is the side of the hernia. most useful study in the diagnosis of eventration of the diaphragm and for identification of abdominal organs underneath the eventration. Other investiga- tion modalities include pneumoperitonography, con- trast peritonography, radioisotope scanning and computed tomography scans but these are rarely re- quired. Symptomatic patients, especially those with res- piratory distress, need prompt supportive care with endotracheal intubation and ventilation with humid- ified oxygen to minimize the diaphragmatic excur- sions. A nasogastric tube is passed to decompress the stomach and intravenous fluids are commenced. Sur- gery is undertaken once the patient’s condition is sta- bilized.13 Figure 13.2 The contents of the hernia are gently reduced in the abdomen. On the right side, the small intestine and colon are first reduced and the liver is withdrawn last. After the hernia is reduced, an attempt is made to visualize the ipsilateral lung. This is usually done by retracting the anterior rim of the diaphragm. Of- ten, a hypoplastic lung can be observed at the apex. A hernial sac, composed of pleura and peritone- um, is present in about 20% of patients. The sac, if present, is excised to avoid leaving a loculated space- occupying lesion in the chest.
    • Chapter 13 Congenital Diaphragmatic Hernia and Eventration 117 Figure 13.1 Figure 13.2 Liver Stomach Colon
    • Prem Puri 118 Figure 13.3 Most diaphragmatic defects can be sutured by direct and may require dissection for delineation. The pos- sutures of the edges of the defect. Usually the anteri- terior rim of the diaphragm is mobilized by incising or rim of the diaphragm is quite evident. However, the overlying peritoneum. the posterior rim may not be immediately apparent13 Figure 13.4, 13.5 The defect is closed by interrupted non-absorbable phragm is sutured to the lower ribs with either peri- sutures. Occasionally, the posterior rim is absent al- ostial or pericostal sutures. together, in which case the anterior rim of the dia-
    • Chapter 13 Congenital Diaphragmatic Hernia and Eventration 119 Figure 13.3 Figure 13.4 Figure 13.5
    • Prem Puri 120 Figure 13.6 If the defect is large, it may not be possible to repair it commonly used prosthetic material presently is Sur- by direct suture. Various techniques have been de- gisis soft tissue graft, which is incorporated into ad- scribed and include the use of prerenal fascia, rib jacent tissue, and this tends to lessen the risk, exten- structures, the latissimus dorsi muscle, rotational sion or displacement, with a decreased risk of infec- muscle flaps from the thoraco-abdominal wall and tion. prosthetic patches. The operations involving muscle Abdomen is closed in layers. If the abdominal cav- flaps are too long and complex for critically ill pa- ity is small, gentle stretching of the abdominal wall tients and can lead to unsightly chest deformities. will enable safe closure in most of the patients. Chest Prosthetic materials, including Marlex mesh, rein- drain should be avoided. The argument against the forced silicone elastomer, preserved pericardial het- use of a chest drain is in avoidance of barotraumas as erografts, preserved dura and the polytetrafluoroe- it increases the transpulmonary pressure gradient. thylene patch (PTFE), have been advocated. The most Figure 13.7 Plication of the diaphragm has been used for many proach through a posterolateral incision via the sixth years to treat eventration. Plication increases both ti- space may be used for right-sided lesions. The trans- dal volume and maximal breathing capacity and has abdominal approach allows good visualization of the been successful in many clinical series. An abdomi- entire diaphragm from front to back and easier mo- nal approach through a subcostal incision is pre- bilization of abdominal contents. ferred for left-sided eventration but a thoracic ap-13
    • Chapter 13 Congenital Diaphragmatic Hernia and Eventration 121 Figure 13.6 Figure 13.7
    • Prem Puri 122 Figure 13.8, 13.9 Plication of the diaphragm is carried out using non- absorbable sutures and avoiding injury to the phren- ic nerve. In cases of complete eventration, the dia- phragm may be strengthened by a muscle flap or prosthetic patch.13
    • Chapter 13 Congenital Diaphragmatic Hernia and Eventration 123 Figure 13.8 Figure 13.9 Right phrenic nerve
    • Prem Puri 124 CONCLUSION After transfer to the intensive care unit, the infant is lowest possible pressures and low tidal volumes. The kept warm, given maintenance requirements of intra- intrathoracic air pocket will usually reabsorb but ev- venous fluids and has vital signs monitored closely idence of increasing air and fluid with mediastinal with regular blood gas analyses and monitoring of shift requires insertion of a chest drain. Weaning preductal and postductal oxygenation. Ventilatory from ventilation should be meticulous and slow as support is continued postoperatively with the aim of small variations in pH, PO2 and PCO2 will lead to per- maintaining preductal PO2 around 80–100 mmHg, sistent pulmonary hypertension. Weaning should PCO2 up to 60 mmHg, and pH greater than 7.25 with commence with lowering of FiO2, then peak pres- hyperventilation (rates up to 150 per min) and the sures and finally respiratory rate. SELECTED BIBLIOGRAPHY Bohn D (2002) Congenital diaphragmatic hernia. Am J Respir Puri P (1994) Congenital diaphragmatic hernia. In: Freeman Crit Care Med 166 : 911–915 NV, Burge DM, Griffiths DM, Malone PSJ (eds) Surgery of Downard CD, Jaksic T, Garza JJ et al (2003) Analysis of an im- the newborn. Churchill Livingstone, London, pp 331–325 proved survival rate for congenital diaphragmatic hernia. Sydorak RM, Harrison MR (2003) Congenital diaphragmatic J Pediatr Surg 38 : 729–732 hernia: advances in prenatal therapy. Clin Perinatol 30 : Granrholm T, Albanese CT, Harrison MR (2003) Congenital 465–479 diaphragmatic hernia. In: Puri P (ed) Newborn surgery. Arnold, London, pp 309–31413
    • CHAPTER 14 Extracorporeal Membrane Oxygenation Jason S. Frischer, Charles J.H. Stolar INTRODUCTIONExtracoporeal membrane oxygenation (ECMO) is a than 12 h. Older infants and children do not have aslife-saving technology that affords partial heart/lung well defined criteria for high mortality risk. Thebypass for extended periods. ECMO is a supportive combination of a ventilation index (respiratory raterather than a therapeutic modality as it provides suf- PaCO2 peak inspiratory pressure/1000) greater thanficient gas exchange and perfusion in patients with 40 and an OI>40 correlates with a 77% mortality,acute, reversible cardiac or respiratory failure. It pro- whereas a mortality of 81% is associated with anvides a finite period to “rest” the cardiopulmonary A-aDO2>580 mmHg and a peak inspiratory pressuresystems at which time they are spared insults from of 40 cmH2O. Indications for support in patients withtraumatic mechanical ventilation and perfusion im- cardiac pathology are based on clinical signs such aspairment. ECMO was first implemented in newborns hypotension despite the administration of inotropesin 1974. Since then, the Extracorporeal Life Support or volume resuscitation, oliguria (urine output <0.5 cc/Organization (ELSO) has recorded approximately kg per h), and decreased peripheral perfusion.24,000 neonatal and paediatric patients treated with In addition, the gestational age should be at leastECMO for a wide range of cardiorespiratory disor- 34–35 weeks due to the increased risk for intracranialders. In the neonatal period the most common disor- haemorrhage (ICH), and the birth weight at least 2 kgders treated with ECMO are meconium aspiration secondary to cannula size limitations. The length ofsyndrome (MAS), congenital diaphragmatic hernia mechanical ventilation, and its associated toxicity(CDH), sepsis, persistent pulmonary hypertension of from prolonged exposure to high concentration oxy-the neonate (PPHN), and cardiac support. For the gen and positive pressure ventilation, prior to ECMOpaediatric population, viral and bacterial pneumo- should be no longer than 10–14 days due to the devel-nia, acute respiratory failure (non-ARDS), acute res- opment of bronchopulmonary dysplasia. Babies withpiratory distress syndrome (ARDS), and cardiac dis- lethal congenital anomalies should not be consideredease are the most common pathophysiologic pro- for ECMO support. Treatable conditions such as totalcesses requiring ECMO intervention. anomalous pulmonary venous return and transposi- Candidates for ECMO are expected to have a re- tion of the great vessels, which may masquerade in-versible cardiopulmonary disease process, with a itially as pulmonary failure can be corrected withpredictive mortality greater than 80–90%, and ex- surgery, but may require ECMO resuscitation initial-haustion of ventilatory and pharmaceutical thera- ly. Therefore, echocardiogram should be rapidly ob-pies. Obviously, these criteria are subjective and vary tained to determine cardiac anatomy. There shouldbetween institutions. Subjective criteria for mortality be no evidence of significant neurologic injury suchrisk in neonatal respiratory failure have been sug- as seizures. Suggestion of a small ICH (grades I–II)gested to identify infants with a >80% mortality. should be considered on an individual basis andThese include (a) the oxygenation index (OI), calculat- monitored very closely for worsening bleeding. Ined as FiO2 mean airway pressure 100/PaO2 (OI>40 fact, all patients with gross active bleeding or majorequates with 80% mortality), and (b) an alveolar-ar- coagulopathy should be corrected prior to initiatingterial oxygen gradient (A-aDO2) >625 mmHg for ECMO.more than 4 h, or an A-aDO2>600 mmHg for more
    • Jason S. Frischer, Charles J.H. Stolar 126 Figure 14.1a–c The goal of ECMO support is to provide gas ex- turn. VV and DLVV avoid these disadvantages and change and oxygen delivery. Three different extra- provide pulmonary support but do not assist with corporeal configurations; venoarterial (VA), venove- circulation. VV bypass is accomplished with drain- nous (VV), and double-lumen single cannula venov- age from the RA via the IJ with reinfusion into the fe- enous (DLVV) bypass are available. VA bypass allows moral vein. DLVV is carried out by means of the IJ. A for support of both the pulmonary and cardiac sys- major disadvantage of VV and DLVV ECMO is that a tems. Venous blood is drained from the right atrium fraction of freshly infused blood recirculates back (RA) through the internal jugular vein (IJ), and oxy- into the circuit and requires approximately a 20% in- genated blood is returned via the carotid artery (CA) crease in flow rate. A limitation in DLVV catheter size to the aorta. Potential disadvantages of this arrange- confines use of this method of support since it can- ment include the sacrifice of a major artery, risk of not accommodate very small infants or larger pa- gaseous or particulate emboli into systemic circula- tients. In summary, it is recommended that patients tion, reduced pulmonary perfusion, decreased pre- who require only respiratory support use VV or load and increased afterload, which may reduce car- DLVV bypass and those that necessitate cardiac sup- diac output, non-pulsatile flow, and the coronaries port use VA ECMO and, if necessary, one can convert are perfused by left ventricular blood which is rela- VV or DLVV to a VA circuit. tively hypoxic and is compensated by arterialized re-14
    • Chapter 14 Extracorporeal Membrane Oxygenation 127 Figure 14.1a–c IVC IVC SVC SVC Gravity Gravity a Lung b Lung IVC SVC Gravity c Lung
    • Jason S. Frischer, Charles J.H. Stolar 128 Figure 14.2 The circuit is comprised of three main components; a is a two-compartment chamber composed of a spiral roller pump, a membrane oxygenator, and a heat ex- wound silicone membrane and a polycarbonate core, changer. Right atrial blood is drained by gravity si- with blood flow in one direction and oxygen flow in phon into a venous servo-mechanism, which acts as a the opposite direction. The size of the oxygenator is safety valve. The servo detects diminished venous re- chosen based on the patient’s size. The oxygenated turn, slows or shuts off the pump and sounds an blood flows through a heat exchanger and is then re- alarm, hence stopping blood flow and relieving the turned to the patient. A bridge is constructed to con- risk of introducing air into the circuit and a cavita- nect the venous line shortly after exiting the patient tion. Next, a roller pump, with continuous servoregu- and the arterial line just prior to entering the patient lation and pressure monitoring, perfuses the blood so that during weaning the patient and the circuit can through the membrane oxygenator. The oxygenator easily form two separate circuits.14
    • Chapter 14 Extracorporeal Membrane Oxygenation 129 Figure 14.2 Temp Probe Connector Arterial Line Heat Exchanger Membrane Bridge Oxygenator Venous Line Oxymetrics Probe Bladder Pump
    • Jason S. Frischer, Charles J.H. Stolar 130 Figure 14.3, 14.4 Cannulation can be performed in the neonatal or the sternomastoid muscle retracted to expose the ca- paediatric intensive care units under adequate seda- rotid sheath. Using sharp dissection and meticulous tion, with proper monitoring. The patient is posi- haemostasis, the sheath is opened and the internal tioned with the head at the foot of the bed, supine, jugular vein, common carotid artery, and vagus and the head and neck hyperextended over a shoul- nerve are identified. All vessels must be handled with der roll and turned to the left. Local anaesthesia is extreme care as to avoid spasm. The vein is dissected administered over the proposed incision site. A free first and mobilized over proximal and distal lig- transverse cervical incision is made along the anteri- atures. Occasionally it is necessary to ligate the infe- or border of the sternomastoid muscle, one finger’s rior thyroid vein. The common carotid artery lies breadth above the right clavicle. The platysma mus- medial and posterior, contains no branches, and is cle is divided with electrocautery. Self-retaining re- mobilized in a similar fashion. The vagus nerve tractors are placed and dissection is carried out with should be identified and protected from injury.14
    • Chapter 14 Extracorporeal Membrane Oxygenation 131 Figure 14.3 Figure 14.4 Common carotid a. Vagus n. Internal jugular v.
    • Jason S. Frischer, Charles J.H. Stolar 132 Figure 14.5 The patient is then systemically heparinized with and a transverse arteriotomy is made near the liga- 50–100 U/kg heparin, which is allowed to circulate ture. Stay sutures, using 5/0 or 6/0 prolene, are placed for 3 min so that an activating clotting time (ACT) of through the full thickness of the medial, lateral, and >300 s is attained. The arterial cannula (usually 10F proximal edges of the arteriotomy. To help prevent for newborns) is measured so that the tip will lie at subintimal dissection, the sutures are gently retract- the junction of the brachiocephalic artery and the ed and the clamp slowly released as the arterial cath- aorta (2.5–3 cm, one-third the distance between the eter is inserted into the carotid artery to its proper sternal notch and the xiphoid). The venous cannula position. The cannula is then fastened into place with (12–14F for neonates) is measured to have its tip in two silk ligatures (2/0 or 3/0), with a small piece of the distal RA (6–8 cm, one-half the distance between vessel loop, on the anterior aspect, inside the liga- the suprasternal notch and the xiphoid process). For tures to protect the vessel from injury during decan- VA bypass, the carotid artery is ligated cranially. nulation. Proximal control is obtained with an angled clamp, Figure 14.6, 14.7 In preparation for the venous cannulation, the pa- For VV and DLVV bypass the procedure is exactly tient is given succinylcholine to prevent spontaneous as described above including dissection of the artery, respiration. The vein is then ligated cranially. Gentle which is marked with a vessel loop, so that a future traction is placed on the lower ligature to help de- switch from VV to VA ECMO can be accomplished, if crease back bleeding, and a venotomy is made close necessary, with as little complication as possible. The to the proximal ligation. The drainage catheter is catheter tip should be in the mid-right atrium (5 cm passed to the level of the RA and secured in a manner in the neonate) with the arterial portion of the cathe- similar to that used for the arterial catheter. The can- ter pointed toward the ear. This directs the oxygenat- nulas are then debubbled with back bleeding and ed blood flow towards the tricuspid valve. heparinized saline. Then they are connected to the Cannula position is confirmed by chest X-ray and ECMO circuit and bypass is initiated. Both cannulas transthoracic echocardiogram. The venous catheter14 are then secured to the mastoid process using mono- should be located in the inferior aspect of the right filament. The wound is irrigated, meticulous hae- atrium, and the arterial catheter at the ostium of the mostasis obtained, and closed in layers with a run- inominate artery and the aorta. With a double-lumen ning nylon for skin closure. The site is covered with a venous catheter, the tip should be in the mid-right sterile dressing and the cannulas are fixed securely to atrium with return oxygenated blood flow towards the bed. the tricuspid valve.
    • Chapter 14 Extracorporeal Membrane Oxygenation 133 Figure 14.5 Figure 14.6 Figure 14.7
    • Jason S. Frischer, Charles J.H. Stolar 134 PATIENT MANAGEMENT IN ECMO Once the cannulas are connected to the circuit, by- fluid extravasation into the soft tissues. The patient pass is initiated and flow is slowly increased to becomes oedematous and may require volume re- 100–150 ml/kg per min so that the patient is stabi- placement (crystalloid, colloid, or blood products) in lized. Continuous inline monitoring of the venous order to maintain adequate intravascular and bypass (prepump) SvO2 and arterial (postpump) PaO2 as flows, haemodynamics, and urine output greater well as pulse oximetry is vital. The goal of VA ECMO than 1 cc/kg per h. By the third day of bypass, diuresis is to maintain a mixed venous PO2 (SvO2) of 37–40 - of the excess extracellular fluid begins, and can be fa- mmHg and saturation of 65–70%. VV ECMO is more cilitated with the use of furosemide if necessary. difficult to monitor due to recirculation, which may Surgical procedures, such as CDH repair, may be produce a falsely elevated SvO2. Inadequate oxygena- performed while the child remains on bypass. Haem- tion and perfusion are indicated by metabolic acido- orrhagic complications are a frequent morbidity as- sis, oliguria, hypotension, elevated liver function sociated with this situation, and increases mortality. tests, and seizures. Arterial blood gasses should be To avoid these complications, prior to the procedure monitored hourly with PaO2 and PaCO2 maintained the platelet count should be greater than 150,000/ at as close to normal level as possible. As soon as mm3, a fibrinogen level above 150 mg/dl, an ACT re- these parameters are met, all vasoactive drugs are duced to 180–200 s, ECMO flow increased to full sup- weaned, and ventilator levels are adjusted to “rest” port, and it is imperative meticulous haemostasis be settings. Gastrointestinal prophylaxis is initiated and obtained throughout the surgery. Fibrinolysis inhibi- mild sedation and analgesia is provided usually with tor aminocaproic acid (100 mg/kg) just prior to inci- fentanyl and midazolam, but the use of a paralyzing sion followed by a continuous infusion (30 mg/kg per agent is avoided. Ampicillin and either gentamicin or h) until all evidence of bleeding ceases is a useful ad- cefotaxime are administered for prophylaxis. Routine junct. blood, urine, and tracheal cultures should be taken. As the patient improves, the flow of the circuit may Heparin is administered (30–60 mg/kg per h) be weaned at a rate of 10–20 ml/h as long as the pa- throughout the ECMO course in order to preserve a tient maintains good oxygenation and perfusion. circuit free of thrombus. ACTs should be monitored Flows should be decreased to 30–50 ml/kg per min hourly and maintained at 180–220 s. A complete blood and the ACT should be at a higher level (200–220 s) count should be obtained every 6 h and coagulation to prevent thrombosis. Moderate conventional venti- profiles daily. In order to prevent a coagulopathy, lator settings are used, but higher settings can be platelets are transfused to maintain a platelet count used if the patient needs to be weaned from ECMO above 100,000/mm3 and some authors sustain fi- urgently. If the child tolerates the low flow, all medi-14 brinogen levels above 150 mg/dl. The haematocrit cations and fluids should be switched to vascular ac- should remain above 40% using red blood cell trans- cess on the patient, and the cannulas may be clamped fusions so that oxygen delivery is maximized. with the circuit bypassing the patient via the bridge. Volume management of patients on ECMO is ex- The patient is then observed for 2–4 h, and if this is tremely important and very difficult. It is imperative tolerated, then decannulation should be performed. that all inputs and outputs be diligently recorded and This should be executed under sterile conditions in electrolytes monitored every 6 h. All fluid losses the Trendelenberg position with muscle relaxant on should be repleted and electrolyte abnormalities cor- board to prevent air aspiration into the vein. The rected.All patients should receive maintenance fluids catheters are removed and the vessels are ligated. The as well as adequate nutrition using hyperalimenta- wound should be irrigated and closed over a small tion. The first 48 to 72 h of ECMO typically involve drain, which is removed 24 h later. COMPLICATIONS Extracranial bleeding is a common complication of as Gelfoam, Surgicel, or topical thrombin. For all sites the heparinized ECMO patient either at the site of of bleeding, the platelet count should be increased to cannulation or at other sites. Bleeding is noted in 21% >150,000 mm3 and the ACT lowered to 180–200 s. of neonatal cases, 44% of paediatric respiratory cas- Sometimes the temporary discontinuation of hepar- es, and 40% of all cardiac cases. Bleeding at the site of in and normalization of the coagulation status is war- cannulation can often be treated with local pressure ranted to help stop the haemorrhage. Aggressive sur- or the placement of topical haemostatic agents such gical intervention is warranted if bleeding persists.
    • Chapter 14 Extracorporeal Membrane Oxygenation 135 Neurological sequelae are a serious morbidity of Acute tubular necrosis (ATN), marked by oliguriathe ECMO population and include learning disor- and increasing blood urea nitrogen and creatinineders, motor dysfunction, and cerebral palsy. These levels, is often seen in the ECMO patient during theoutcomes appear to be due to hypoxia and acidosis initial 48 h, at which time renal function is expectedprior to the ECMO course. ICH is the most devastat- to improve. If this does not occur, consideration musting complication of ECMO and occurs in 5.9% of pa- be towards poor tissue perfusion. This may be due totients and carries with it a 54% mortality. Frequent low cardiac output, insufficient intravascular volume,comprehensive neurologic exams should be per- or inadequate pump flow, all of which should be cor-formed and cranial ultrasounds obtained daily for rected. In the event of continued renal failure, hae-the first 3 days of ECMO and then every other day. mofiltration or haemodialysis can be attached to theBlood pressure should be carefully monitored and circuit to maintain proper fluid balance and electro-maintained within normal parameters to help de- lyte levels and are reported to be required in 14% ofcrease the risk of ICH. If necessary, electroencephalo- cases.grams may be helpful in the neurologic evaluation. CONCLUSIONAs of January 2003, more than 19,000 neonates (74% fects have a 38% survival, bridge to transplant 43%,survival) and 4,800 paediatric patients (48% survi- cardiomyopathy 49%, and the highest survival rate isval) have been treated with ECMO. In the neonatal for myocarditis, 58%.population, MAS is the most common indication for Recent medical advances, such as permissive hy-ECMO and carries with it a survival rate of 94%. Oth- percapnea and the use of oscillatory ventilation haveer frequent diagnosis (with survival rates in paren- spared numerous babies from ECMO, yet many chil-theses) include PPHN (79%), sepsis (75%), and CDH dren still benefit from this modality. In summary, any(54%).Viral pneumonia is the most common aetiolo- patient with reversible cardiopulmonary disease,gy amongst the paediatric population requiring EC- who meets criteria, should be considered an ECMOMO and has a 62% survival. Aspiration carries the candidate. ECMO provides an excellent opportunitygreatest survival at 65%, where as non-ARDS respira- to provide “rest” to the cardiopulmonary systems andtory failure has a 47% survival, ARDS 55%, and bacte- allows the patient to recover using pharmacologicrial pneumonia 52% survival. Cardiac patients have and surgical therapies.an overall survival of 39%. Specifically, congenital de- SELECTED BIBLIOGRAPHYCampbell BT, Braun TM, Schumacher RE et al (2003) Impact of Kim ES, Stolar CJ (2000) ECMO in the newborn. Am J Perinat- ECMO on neonatal mortality in Michigan (1980–1999). J ol 17 : 345–356 Pediatr Surg 38 : 290–295 Kim ES, Stolar CJH (2003) Extracorporeal membrane oxygen-Extracorporeal Life Support Organization (2003) Internation- ation for neonatal respiratory failure. In: Puri P (ed) New- al Registry Report of the Extracorporeal Life Support Or- born surgery. Arnold, London, pp 317–327 ganization. January 2003. University of Michigan Medical Center, Ann ArborHirschl RB, Bartlett RH (1998) Extracorporeal life support in cardiopulmonary failure. In: O’Neill JA Jr, Rowe MI, Gros- feld JL, Fonkalsrud EW, Coran AG (eds) Pediatric surgery, 5th edn. Mosby, New York, pp 89–102
    • SPRINGER SURGERY ATLAS SERIES Series Editors: J. S. P. Lumley · J. R. Siewert
    • Part IV Abdomen
    • SPRINGER SURGERY ATLAS SERIES Series Editors: J. S. P. Lumley · J. R. Siewert
    • CHAPTER 15 Hernias – Inguinal, Umbilical, Epigastric, Femoral and Hydrocele Juan A. Tovar INTRODUCTIONInguinal hernia is one of the most common surgical A hydrocele of the tunica vaginalis usuallyconditions in infancy, with a peak incidence during presents as a soft, nontender fluid filled sac that maythe first 3 months of life. The diagnosis of inguinal transilluminate. Most hydroceles usually involutehernia is made with increasing frequency in new- spontaneously during the first 12 months of life.borns; this period carried a particularly high risk of Those that persist beyond 1 year of age are associatedincarceration. On the other hand, the incidence of with a patent processus vaginalis and require opera-hernia is much higher in premature infants who sur- tive intervention, the same as for an inguinal hernia.vive in growing numbers after sophisticated inten- Femoral hernias are rare in children. The diagno-sive care management. Direct hernia is exceedingly sis is based on the observation of a groin swelling lo-rare at this age and practically all congenital indirect cated underneath the external inguinal orifice, al-inguinal hernias develop because the processus va- though this location is easily missed because, unlessginalis remains patent after birth. The most common the bulge is visible upon examination, relatives andpresentation of inguinal hernia in a child is a groin doctors will first interpret its appearance as the ex-bulge, extending towards the top of the scrotum. The pression of an inguinal hernia. This explains whytreatment of inguinal hernia is always surgical. In in- 50% of these patient are mistakenly operated uponfants and toddlers, herniotomy can be performed for inguinal hernia and why, only when the sac is notthrough the external inguinal orifice without any at- found, exploration of the femoral area allows diagno-tempt at parietal reinforcement. In older children, sis and repair. The femoral orifice, located below thehowever, the length of the canal makes it advisable to inguinal ligament, allows passage of the femoral vein,open the external oblique aponeurosis in order to artery and nerve from the pelvis to the thigh. Theachieve a high ligation of the sac. The incidence of hernial orifice is always medial and the sac is there-congenital indirect inguinal hernia in full-term neo- fore in close contact with the femoral vein.nates is 3.5–5%. The incidence of inguinal hernia in Umbilical hernia is as a result of failure of closurepreterm infants is considerably higher and ranges of the umbilical ring. The hernial sac protrudesfrom 9–11%. The incidence approaches 60% as birth through the defect. Most umbilical hernias have aweight decreases from 500 to 750 g. Inguinal hernia is tendency to resolve spontaneously. In view of the fa-more common in males than in females. Most series vourable natural history of umbilical hernias, surgi-report a male preponderance over females ranging cal indications are limited to those hernias locatedfrom 5:1 to 10:1. Of all inguinal hernias, 60% occur on above the umbilicus, to those that persist beyond thethe right side, 25–30% on the left, and 10–15% are bi- age of 4 years and to those occurring in children withlateral. connective tissue disorders. The anatomy of the inguinal canal varies slightly Epigastric hernia (fatty hernia of linea alba) usu-with age. In adults and children, the internal and ex- ally occurs in the midline of the anterior abdominalternal inguinal orifices are widely separated, whereas wall. It is usually a small defect through which pre-in young infants they practically overlap. In girls, the peritoneal fat protrudes and may cause pain.anatomy is similar except for the absence of spermat-ic elements which are replaced by the round liga-ment.
    • Juan A. Tovar 140 Figure 15.1 General anaesthesia with endotracheal intubation is hernia repair is associated with a lower incidence of preferred in small infants. Premature infants under- post-operative apnea. The infant is placed in the su- going surgery have an increased risk of life-threaten- pine position on a heating blanket. A 1.5-cm trans- ing post-operative apnea. The use of spinal anaesthe- verse inguinal skin crease incision is placed above sia in low birth-weight infants undergoing inguinal and lateral to the pubic tubercle.15 Figure 15.2, 15.3 The subcutaneous fat and the fascia of Scarpa (which and external ring are exposed. The external inguinal is surprisingly dense in infants) are opened, grasping ring is not opened except in older children and ado- them with small-toothed Adson forceps. Using blunt lescents. scissors or cautery, the external oblique aponeurosis
    • Chapter 15 Hernias – Inguinal, Umbilical, Epigastric, Femoral and Hydrocele 141 Figure 15.1 Figure 15.2 Figure 15.3
    • Juan A. Tovar 142 Figure 15.4 The external spermatic fascia and cremaster are sep- from the vas and vessels. A haemostat is placed on arated along the length of the cord by blunt dissec- the fundus of the sac. tion. The hernial sac is seen and gently separated Figure 15.5 The sac is divided between the clamps and twisted so the sac beyond the stitch is usually excised. In the as to reduce its content into the abdominal cavity. case of hydrocele, the distal part of the sac is widely The spoon can be used to keep vas and vessels away slit allowing adequate drainage of fluid. In girls, the from the neck of the sac. The sac is transfixed with a operation is even more straightforward since there is 4/0 stitch at the level of internal ring, which is no risk for the vas or the vessels and the external ori- marked by an extraperitoneal pad of fat. The part of fice can be closed after excising the sac.15 Figure 15.6, 15.7 Subcutaneous tissues are approximated using two or the wound if necessary. At the end of the operation, three 4/0 absorbable interrupted stitches and the the testis, always tractioned upwards during opera- skin is closed with a 5/0 absorbable continuous sub- tive manoeuvres, must be routinely pulled back into cuticular suture.A small dressing can be applied over the scrotum to avoid iatrogenic ascent.
    • Chapter 15 Hernias – Inguinal, Umbilical, Epigastric, Femoral and Hydrocele 143 Figure 15.4 Figure 15.5 Figure 15.6 Figure 15.7
    • Juan A. Tovar 144 Figure 15.8 (Femoral Hernia) Figure 15.9 The operative approach for femoral hernia is initially The spermatic cord is retracted in order to obtain ac- identical to the more commonly used approach for cess to the femoral region. The sac is identified and inguinal hernia. An inguinal skin crease incision is delivered into the wound avoiding damage to the fe- made and the subcutaneous layers and Scarpa’s fas- moral vein which is in close contact with the sac lat- cia are opened in order to expose the external erally. It may be convenient to ligate and divide the oblique aponeurosis at the level of the external ingui- inferior epigastric vessels in order to better expose nal ring. The aponeurosis is incised longitudinally the femoral area from behind. taking care to preserve the ilio-inguinal nerve. The inguinal canal is open dorsally sectioning with caut- ery the conjoined tendon and the fascia transversalis. Figure 15.10 The sac is then opened to ensure that it has no con- taking care of not compressing the femoral vessels. tents and it is subsequently suture-ligated with a fine The inguinal canal is reconstructed and the superfi- stitch flush with the peritoneum. The femoral defect cial layers and the skin are closed like those in ingui- is then narrowed by approximating the internal in- nal hernias. Femoral hernia repair can also be ac- sertions of the Cooper ligament and the inguinal lig- complished by an infra-inguinal approach. ament with two or three fine non-absorbable stitches15
    • Chapter 15 Hernias – Inguinal, Umbilical, Epigastric, Femoral and Hydrocele 145 Figure 15.8 Figure 15.9 External oblique aponeurosis Ilioinguinal n. External inguinal ring Inferior epigastric Femoral artery, vein artery and vein Femoral sac Figure 15.10
    • Juan A. Tovar 146 Figure 15.11, 15.12 (Umbilical Hernia) Umbilical hernia repair is carried out under general the hernial sac. By blunt dissection with a mosquito anaesthesia. A semicircular incision is made in the clamp, a plane is developed on both sides of the sac skin crease immediately below the umbilicus. The and the sac is encircled with a haemostat and is di- subcutaneous layers are dissected in order to expose vided.15
    • Chapter 15 Hernias – Inguinal, Umbilical, Epigastric, Femoral and Hydrocele 147 Figure 15.11 Figure 15.12
    • Juan A. Tovar 148 Figure 15.13–15.15 A clamp is placed on either side of the umbilical de- ing it to the subcutaneous layer in the midline. The fect for traction. The defect is closed by interrupted wound is closed with several interrupted sutures 2/0 absorbable sutures. A stitch is used to invaginate placed in the subcuticular plane. A slightly compres- the umbilical scar, tractioning it downwards and fix- sive dressing is maintained for 24 h.15
    • Chapter 15 Hernias – Inguinal, Umbilical, Epigastric, Femoral and Hydrocele 149 Figure 15.13 Figure 15.14 Figure 15.15
    • Juan A. Tovar 150 Figure 15.16–15.18 (Epigastric Hernia) Epigastric hernias are repaired when they are prom- ously marked location of the hernia. The fatty mass inent or when they are symptomatic. It is important protruding through the linea alba defect is excised to mark the location of the defect before anaesthesia, after a transfixation stitch. The defect in the linea al- because in the recumbent position they are often im- ba is closed with interrupted 3/0 absorbable sutures. possible to palpate along the widened linea alba. A The skin is approximated using subcuticular sutures. transverse incision is made directly over the previ-15
    • Chapter 15 Hernias – Inguinal, Umbilical, Epigastric, Femoral and Hydrocele 151 Figure 15.16 Figure 15.17 Figure 15.18
    • Juan A. Tovar 152 CONCLUSION The overall complication rates after elective hernia í Iatrogenic ascent of the testes. This event is rela- repair are low at about 2%, whereas these are in- tively rare since slightly more than 1% of patients creased to 8–33% for the incarcerated hernias requir- operated upon for inguinal hernia during infancy ing emergency operations. Complications of inguinal required subsequently orchidopexy. This compli- hernia repair include: cation is probably due to entrapment of the testis í Haematoma – can be avoided with meticulous at- in the scar tissue or failure to pull it down into the tention to haemostasis. It is rarely necessary to scrotum at the end of the operation and to main- evacuate wound, cord or scrotal hematoma. tain it there. í Wound infection – low risk and should not exceed í Recurrence. The acceptable recurrence rate for in- 1%. guinal hernia repair is less than 1% but when op- í Gonadal complications – occur due to compres- eration is performed in the neonatal period this sion of the vessels by incarcerated viscera. Though complication can occur in up to 8%. The factors large numbers of testes look nonviable in patients that predispose to recurrence are ventriculoperit- with incarcerated hernia, the actual incidence of oneal shunts, sliding hernia, incarceration and testicular atrophy is low and therefore, unless the connective tissue disorders. Recurrence may be testis is frankly necrotic, it should not be re- indirect or direct. Indirect recurrence is due to ei- moved. ther failure to ligate the sac at high level, tearing of í Intestinal resection. This is necessary in about a friable sac, a slipped ligature at the neck of the 3–7% of patients in whom the hernia is not re- sac, missed sac, or wound infection. Direct hernia duced and it may cause some additional morbid- may be due to inherent muscle weakness or to in- ity corresponding to resection itself and contami- jury to the posterior wall of the inguinal canal. nation of the field. í Mortality. In the present-day situation, the mor- tality rate of inguinal hernia operation should be zero. SELECTED BIBLIOGRAPHY Coats RD, Helikson MA, Burd RS (2000) Presentation and technique: a survey of North America pediatric surgeons. J management of epigastric hernias in children. J Pediatr Pediatr Surg 37 : 439–449 Surg 35 : 1754–1756 Skinner MA, Grosfeld JL (1993) Inguinal and umbilical hernia De Caluwe D, Chertin B, Puri P (2003) Childhood femoral her- repair in infants and children. Surg Clin North Am nia: a commonly misdiagnosed condition. Pediatr Surg Int 73 : 439–449 19 : 608–609 Tovar JA (2003) Inguinal hernia. In: Puri P (ed) Newborn sur-15 Levitt MA, Ferraraccio D, Arbesman MC, Brisseau GF, Caty gery. Arnold, London, pp 561–568 MG, Glick PL (2002) Variability of inguinal hernia surgical
    • CHAPTER 16 Omphalocele Stig Somme, Jacob C. Langer INTRODUCTIONExomphalus (also known as omphalocele) is a condi- sarean delivery for fetuses with a large exomphalustion that is seen in newborn infants, and is thought to to avoid liver injury and rupture of the sac.result from failure of the intestines to return to theabdomen after the migration into the umbilical cord í Postnatal Management. Immediate postnatalthat occurs between the sixth and tenth week after management consists of:conception. The incidence of exomphalus has not í Nasogastric tube placement to decompress thechanged over the last several decades. There is no stomachknown environmental, racial or geographic predilec- í Intubation, if the child is in respiratory distresstion, although in rare cases there may be a familial í Coverage of the sac with moist gauze and plasticpredisposition. Exomphalus is also associated with a foillower than normal birth weight and gestational age. í Intravenous fluids Exomphalus is characterized by a central defect at í Routine neonatal bloodworkthe umbilical ring; a membrane composed of viscer- í Temperature control with a heating lampal peritoneum,Wharton’s jelly and amnion covers the í Vitamin K administrationeviscerated abdominal contents. The umbilical cord í Antibiotics, if the sac is rupturedinserts onto the exomphalus sac. The sac usually con-tains loops of small and large intestine, stomach and, In addition, a thorough assessment for other abnor-in approximately 50% of cases, liver. The abdominal malities must be performed, which will directly affectmuscles are normally developed. Rupture of the sac decisions related to the care of the child. Detailedis reported in 10–18% of cases. This can happen in physical examination, radiological studies, echocardi-utero, at time of delivery or after delivery. ography and abdominal ultrasound are important to Exomphalus is frequently associated with other identify any associated anomalies. Since some largeanomalies, the most common of which are cardiac and defects are associated with pulmonary hypoplasia,gastrointestinal tract abnormalities. Chromosomal careful assessment of oxygenation and ventilationabnormalities are often seen, particularly in children should be done and respiratory support using intuba-with small defects that do not contain liver. Exomphal- tion and mechanical ventilation should be institutedus is also associated with Beckwith-Wiedeman syn- if necessary. The exomphalus itself should be evaluat-drome, cloacal exstrophy, and pentalogy of Cantrell. ed to determine its size, contents, and integrity. Newborns with abdominal wall defects requireí Prenatal Diagnosis and Management. Exomphal- more intravenous fluids in the first few days of lifeus is often suspected because of an elevated level of than a normal infant, due to evaporative loss andmaternal serum α-fetoprotein. Prenatal diagnosis third spacing. The daily intravenous fluid require-can be accurately made using prenatal sonography. ment must be adjusted based on the hourly urineExomphalus can be differentiated from gastroschisis output and other parameters for end-organ perfu-because of the location of the defect and the presence sion. Infants with a silo are at a particularly high riskof a sac, although this may be more difficult if the sac of fluid, protein and temperature loss.has ruptured. If exomphalus is detected or suspected Based on the clinical status of the patient and theit is important to search for other abnormalities. In characteristics of the exomphalus, there are threeaddition to a thorough ultrasound examination, am- broad categories of options for the surgical manage-niocentesis for karyotype analysis should be recom- ment of this condition:mended and fetal echocardiography should be done 1. Primary closureto look for major cardiac abnormalities. The mother 2. Staged closureshould be transferred to a perinatal centre with ex- a. Skinperienced neonatal and surgical support. Although b. Silothere is no clear evidence to support routine caesar- c. Sequential sac ligationean section, most practitioners will recommend cae- 3. Nonoperative management with late closure
    • Stig Somme, Jacob C. Langer 154 Figure 16.1, 16.2 Small to moderate defects, particularly those in whom the liver is not in the sac, may be closed pri- marily. The sac is removed. Figure 16.3 The skin is undermined enough that a secure fascial closure can be accomplished. Absorbable or nonab-16 sorbable sutures may be used. The skin is then closed. For very small defects, the umbilical cord can be left behind to give better cosmetic results.
    • Chapter 16 Omphalocele 155 Figure 16.1 Figure 16.2 Figure 16.3
    • Stig Somme, Jacob C. Langer 156 Figure 16.4–16.6 For larger defects, it may not be possible to close the The skin is undermined as far out as possible, to per- fascia, but there may be enough skin to achieve skin mit skin closure with minimal tension. At this point, closure over the viscera. This technique was original- some surgeons opt to insert a patch into the fascia. ly described by Gross in 1948. The sac is usually re- The skin is then closed over the patch. In most cases moved, although some surgeons prefer to leave the of skin closure, the patient is left with a ventral her- sac intact and dissect between the edge of the sac and nia, which must be closed at a later time. the skin to the level of the abdominal wall muscle. Figure 16.7 The use of a silo was first described by Schuster in edge of the sac and the skin to the level of the abdom- 1967. The concept of a silo is to use a sheet of silastic inal wall muscle. In some infants, the neck of the sac reinforced with Dacron to gradually reduce the visce- at the abdominal wall is relatively small, and the fas- ra over several days to a week, and then to definitive- cial opening must be enlarged to allow gradual re- ly close the fascia and skin. This technique is useful duction of the viscera. for children with a large or ruptured exomphalus. Monofilament nonabsorbable sutures are then The silastic sheeting is sutured to the edges of the placed around the edges to avoid any gaps through16 musculae fascial layer, after as much of the intestine which intestine can herniate. The silo is then closed and liver as possible have been returned to the abdo- over the top, by suturing it to itself. It should be per- men. Some surgeons also include the skin in these pendicular and suspended or supported to avoid any sutures. Although most surgeons remove the sac, kinks in the intestine. some prefer to leave it intact and dissect between the
    • Chapter 16 Omphalocele 157 Figure 16.4 Figure 16.5 Figure 16.6 Figure 16.7
    • Stig Somme, Jacob C. Langer 158 Figure 16.8 The sac is then gradually reduced at least once daily tapes, and roller devices. Once the viscera are com- until all of the viscera have been reduced. Various pletely reduced, the child is brought back to the oper- techniques have been used to close the top of the silo, ating room usually after a week and the fascia and including sutures, umbilical cord clamps, umbilical skin are closed.everal years in some cases. Figure 16.9, 16.10 This recently described technique uses the exom- heart disease or pulmonary hypophasia. For these phalus sac as a silo. It requires a sac which is relative- children it is best to cover the sac with a material ly strong, and it is relatively difficult if the liver is ad- which allows it to form granulation tissue and even- herent to a large part of the sac. However, it can be tually epithelialize. Early on mercurochrome or io- performed at the bedside in the nursery, with only dine solution were used for this purpose, but there minimal sedation. The technique involves gently were problems with toxicity; this resulted in the16 kneading the sac to release minor adhesions between abandonment of this practice. The use of plastic the sac and the intestine or liver. Traction is applied sheeting (“Op-site”) has been described. We current- to the sac to slowly reduce the contents, and the sac is ly recommend silver sulfadiazine, which prevents in- then twisted and ligated with umbilical ties. Once the fection and results in a good bed of granulation tis- viscera are reduced as much as possible, the child is sue. It takes several months for this to occur, and an- taken to the operating room for definitive closure. other several months for the granulation tissue to Some infants with exomphalus are very poor can- epithelialize. The resulting huge ventral hernia can didates for any kind of surgical intervention. This in- be repaired electively whenever the child’s underly- cludes premature infants, those with chromosomal ing cardiac, pulmonary, or other conditions have im- abnormalities, and those with significant congenital proved. This may take several years in some cases.
    • Chapter 16 Omphalocele 159 Figure 16.8 Figure 16.9 Figure 16.10
    • Stig Somme, Jacob C. Langer 160 CONCLUSION Care of the infant following definitive closure re- an important adjunct to prevent abdominal com- quires a neonatal intensive care unit for all but the partment syndrome, which may result in high airway smallest defects. Infants that have undergone repair pressures, oliguria and intestinal ischemia due to de- of larger defects usually require postoperative me- creased organ perfusion. Intra-abdominal pressures chanical ventilation for days to weeks, depending on above 15 to 20 mmHg, or an increase in central ve- their pulmonary status. It is important to carefully nous pressure of more than 4 mmHg are associated observe the child for signs of abdominal compart- with visceral ischaemia in both animal and human ment syndrome, such as oliguria, acidosis, intestinal studies, and should stimulate consideration of con- ischemia, and liver dysfunction. Infants with other version to a staged closure technique. congenital malformations require continued investi- The outcome for infants with exomphalus is de- gation and management as needed. pendent on gestational age, the presence of associat- After closure of the abdomen, infants with exom- ed chromosomal and structural anomalies, the pres- phalus often develop an ileus, although intestinal ence or absence of pulmonary hypoplasia, and the function usually returns more quickly than seen in size of the defect. Long-term problems that are com- infants with gastroschisis. A nasogastric tube is monly seen in these infants include gastro-oesopha- therefore necessary initially. Total parenteral nutri- geal reflux, feeding disorders, and adhesive bowel ob- tion should be initiated early. Many surgeons place a struction. However, most of these issues can be cor- central venous catheter at the time of the initial oper- rected or improve on their own with time, and most ation. infants with exomphalus who do not have severe ad- Intra-abdominal pressure monitoring using intra- ditional anomalies or pulmonary hypoplasia do very gastric or intravesical catheters during closure can be well, and grow up to be normal individuals. SELECTED BIBLIOGRAPHY Bruch SW, Langer JC (2003) Omphalocele and gastroschisis. In: Hong AR, Sigalet DL, Guttman FM, Laberge JM, Croitoru DP Puri P (ed) Newborn surgery. Arnold, London, pp 605–613 (1994) Sequential sac ligation for giant omphalocele. J Pedi- Grosfeld JL, Weber TR (1982) Congenital abdominal wall de- atr Surg 29 : 413–415 fects: gastroschisis and omphalocele. Curr Probl Surg Langer JC (2003) Abdominal wall defects. World J Surg 27: 19 : 157–213 117–124 Hendrickson RJ, Patrick RJ, Janik JS (2003) Management of Schuster SR (1967) A new method for staged repair of large giant omphalocele in a premature low birth weight neonate omphaloceles. Surg Gynecol Obstet 125 : 837–850 utilizing a bedside sequential clamping technique without prosthesis. J Pediatr Surg 38 : E14–E1616
    • CHAPTER 17 Gastroschisis Marshall Z. Schwartz INTRODUCTIONGastroschisis is one of several congenital abdominal the diameter of the abdominal wall defect, may be in-wall defects that evolves in the first four post-concep- dications for earlier delivery to avoid bowel infarc-tion weeks. It is generally accepted that this congeni- tion. It is important to provide an opportunity for thetal abdominal wall defect is embryologically different family to meet with a fetal management team includ-from omphalocele. The anomaly is thought to be the ing perinatology, paediatric surgery, and neonatolo-result of a defect at the site where the second umbili- gy to review the problem and likely course followingcal vein involutes. Nonrotation of the bowel always delivery. The recommended mode of delivery overaccompanies this anomaly and there is an increase in the past several decades has been somewhat contro-intestinal abnormalities including atresia (mostly in- versial. It is generally believed that caesarian sectionvolving the small intestine) perforation, and infarc- is not necessary unless for obstetric reasons. Electivetion resulting from in utero midgut volvulus or vas- premature delivery is also unnecessary.cular thrombosis. However, unlike omphalocele, Management immediately following delivery andthere is no increase in anomalies of other organs. The prior to surgical correction requires prompt attentionincidence of gastroschisis is approximately 1 in and is critical to the outcome. The two most impor-4,000–6,000 live births. Infants with gastroschisis tant goals are to provide a mechanism of maintainingtypically are slightly premature (35–37 weeks of ges- normal thermogenesis and establishing intravenoustation) and frequently have growth retardation with access to provide appropriate fluid resuscitation. In-birth weights from approximately 2000–2500 g. fants with gastroschisis are usually hypovolemic and Most abdominal wall defects can be diagnosed in require at least 125–150% of maintenance intravenousutero after 14 weeks gestation when the fetal midgut fluid to establish and maintain adequate hydration.has returned to the peritoneal cavity. If gastroschisis Establishing intravenous access initially can be doneis noted on fetal ultrasonography it is strongly rec- through a peripheral intravenous site. Infants withommended that serial examinations be performed gastroschisis require central venous access. Oncelooking for changes in the size and thickness of the intravenous access is established, it is optimal to insti-bowel as well as the diameter of the abdominal wall tute broad spectrum antibiotic coverage. To avoiddefect. Significant bowel wall thickening and bowel having the bowel get more distended, a nasogastricdilatation, especially associated with a decrease in tube should be inserted and placed on suction.
    • Marshall Z. Schwartz 162 Figure 17.1 Figure 17.2 The specific features of gastroschisis include an ab- Because the abdominal wall defect in gastroschisis is dominal wall defect measuring 2–4 cm in diameter, relatively small (2–3 cm) it may be difficult to reduce which is almost always to the right of a normal um- the herniated mid-gut through this small opening. bilical cord. There is no sac covering the herniated Thus, it may be necessary to enlarge the abdominal contents. The herniated contents typically include wall opening. The optimum way to do this is extend- the entire intestinal mid-gut. There is shortening of ing the gastroschisis defect superiorly by incising the the mesentery and thickening of the bowel wall. The fascia along the midline with a finger placed below bowel surface may be covered with a fibrin “peel”. the fascia to avoid an injury to the bowel. Depending on the size of the abdominal wall defect, Extending the defect superiorly is safer than a cau- it is possible that the stomach, and/or the urinary dal incision because the bladder is very close to the bladder, and the fallopian tubes and ovaries in a fe- inferior aspect of the abdominal wall defect and this male may be herniated through the abdominal wall limits the ability to extend the opening inferiorly. defect. General anaesthesia including muscle relaxation is required for the appropriate intra-operative man- agement of gastroschisis. The bowel and anterior ab- dominal wall should be prepped. It is my preference to use a warm, dilute 50/50 mixture of povodine iodine and saline. The umbilical cord should be clamped and tied 2–3 cm above the abdominal wall and the excess umbilical cord then removed. At this point, appropriate draping is indicated.17
    • Chapter 17 Gastroschisis 163 Figure 17.1 Figure 17.2
    • Marshall Z. Schwartz 164 Figure 17.3 Figure 17.4 After enlarging the abdominal wall defect, the bowel If it is possible to reduce all of the herniated intesti- can be reduced into the peritoneal cavity. The degree nal contents into the peritoneal cavity, primary clo- of thickening and fibrin peel determines how malle- sure should be undertaken. It is important to identify able the herniated bowel is and how easily it is to good fascial edges for the closure. The choice of su- place it within the abdominal cavity. If the initial as- ture material and the technique for placement of su- sessment suggests that primary closure may not be tures, whether they are interrupted, figure-of-eight obtainable, two techniques have been described to sutures, or a running suture is personal preference. It increase the chances of a primary abdominal wall has been my approach to use 3/0 or 2/0 absorbable closure. The first approach is to attempt to empty the braided suture if there is mild to moderate tension intestinal contents either retrograde into the stomach and 3/0 or 2/0 monofilament sutures if there is mod- which then can be aspirated through the nasogastric erate-to-significant tension. These sutures are placed tube or antegrade into the colon and out the rectum. in a figure-of-eight fashion. A second technique is manual stretching of the ante- It is preferable to place all of the sutures prior to rior abdominal wall to increase the size of the perito- tying them. An important point in patients with gas- neal cavity. Although gentle stretching is potentially troschisis is the placement of sutures at the level of advantageous, vigorous stretching can result in hae- the umbilicus. The incidence of an umbilical defect morrhage and swelling of the rectus muscles in the following gastroschisis closure is high. To avoid this, rectus sheath. the fascia lateral to the umbilical ring should be clearly identified and used for placement of the su- ture. If the sutures are placed medial to the umbilical ring, then it is highly likely that an umbilical defect will result in requiring subsequent repair. In tying the sutures in sequence a thin ribbon retractor placed in the peritoneal cavity underneath the fascia is advantageous to avoid trapping the bowel during tying of the sutures. Prior to closing the skin, any compromised or ischaemic skin should trimmed. The degree of tension on the skin sutures can dictate the type of closure.17
    • Chapter 17 Gastroschisis 165 Figure 17.3 Figure 17.4
    • Marshall Z. Schwartz 166 Figure 17.5 Figure 17.6 A significant percentage of infants with gastroschisis After the sheets are attached to the fascia on either can undergo reduction of the herniated intestinal side of the defect, they are then sewn around the her- contents and primary abdominal wall closure. The niated contents with a running suture. As much bow- reported percentage ranges from 60% to nearly el as will be tolerated is reduced into the peritoneal 100%. cavity and then a running suture line is placed across If it is determined at the time of the initial opera- the top of the silastic sac. On successive days the sac tive procedure that primary closure is not possible, is squeezed as much as possible to reduce the herni- then an abdominal wall “silo” can be created. This ated contents. A row of running suture in the silo is technique, initially described by Schuster and col- placed to maintain the reduction. Once the bowel has leagues, has undergone several modifications since been reduced into the peritoneal cavity, the fascial its initial description in 1967. However, the concept edges approximate enough to allow removal of the si- remains the same. Creation of a sac, which is sewn to lo, then primary fascial and skin closure is per- the abdominal fascia circumferentially and then formed in the operating theatre. around the herniated contents, allows for staged re- duction of the sac with resulting reduction of the herniated contents into the abdominal cavity. This approach produces progressive stretching of the ab- dominal cavity with simultaneous reduction of the swelling and rigidity of bowel. Shown in this illustra- tion is placement of reinforced silastic sheeting which is sutured to the fascial edges with horizontal mattress sutures of interrupted 3/0 silk suture.17
    • Chapter 17 Gastroschisis 167 Figure 17.5 Figure 17.6
    • Marshall Z. Schwartz 168 Figure 17.7, 17.8 Recently, the use of a preformed spring-loaded silo function and fewer complications. Also recently, re- bag in infants with gastroschisis has been shown to duction of gastroschisis bowel has been successfully be associated with improved fascial closure rates, performed with and without anaesthesia, and with- fewer ventilator days, more rapid return of bowel out enlarging the abdominal wall defect.17
    • Chapter 17 Gastroschisis 169 Figure 17.7 Figure 17.8
    • Marshall Z. Schwartz 170 CONCLUSION The outcome for patients with gastroschisis has dra- Once there is evidence of bowel function it is ap- matically improved. Whereas the mortality was 80 to propriate to begin enteral feeding. An elemental for- 90% three or four decades ago, the survival is now mula may be better tolerated. When the infant has more than 90%. The improvement in outcome is re- reached adequate caloric intake enterally then dis- lated to the availability of intravenous nutrition and charge is appropriate. the use of staged closure when indicated. Late com- In the absence of complications during the recov- plications and mortality are related to sepsis either ery from surgery and resolution of the bowel oede- from an intra-abdominal or wound complication or ma, infants with gastroschisis usually reach goal from a central venous catheter placed for parental feedings within 3–4 weeks. Long-term complications nutrition. are unusual once the infants are discharged from the hospital. SELECTED BIBLIOGRAPHY Baerg J, Kaban G, Tonita J et al (2003) Gastroschisis: a sixteen Schuster SR (1967) A new method for the staged repair of large year review. J Pediatr Surg 38 : 771–774 omphaloceles. Surg Gynecol Obstet 125 : 837–850 Driver CP, Bruce J, Bianchi A et al (2000) The contemporary Schwartz MZ, Tyson KR, Milliorn K et al (1983) Staged reduc- outcome of gastrochisis. J Pediatr Surg 35 : 1719–1723 tion using a silastic sac is the treatment of choice for large Schlatter M, Norris K, Uitvlugt N et al (2003) Improved out- congenital abdominal wall defects. J Pediatr Surg 18 : 713– comes in the treatment of gastroschisis using a preformed 719 silo and delayed repair approach. J Pediatr Surg 38 : 459– 46417
    • CHAPTER 18 Hypertrophic Pyloric Stenosis Takao Fujimoto INTRODUCTIONInfantile hypertrophic pyloric stenosis (IHPS) is a veals typical hypoechoic ring with echogenic centrecommon surgical condition encountered in early in- of increased muscle thickness. A contrast meal mayfancy, occurring in 2~3 per 1,000 live births. It is be required in difficult and/or complicated presenta-characterized by hypertrophy of the circular muscle, tion and shows characteristic narrowed elongatedcausing pyloric narrowing and elongation. The inci- pyloric canal.dence of disease varies widely with geographic loca- Persistent non-bilious vomiting in these patientstion, season and ethnic origin. Boys are affected four results in chloride depletion, metabolic alkalosis andtimes more than girls. dehydration. Haematological and biochemical analy- There is evidence of a genetic predisposition to sis should be undertaken. Any fluid and electrolytethe development of this condition. Siblings of pa- and acid base imbalance should be corrected prior totients with IHPS are 15 times more likely to suffer the surgery. Oral feeding should be discontinued and acondition than children who have no family history nasogastric tube inserted prior to surgery to keep theof IHPS. The cause of hypertrophic circular muscle of stomach empty. The operation for pyloric stenosis ispylorus is still obscure and various hypotheses have not an emergency and should never be undertakenbeen advocated including abnormal peptidergic in- until serum electrolytes have returned to normal.nervation, abnormality of nitrergic innervation, ab- Ramstedt’s pyloromyotomy is the universally ac-normalities of extracellular matrix proteins, abnor- cepted operation for pyloric stenosis. A 3-cm trans-malities of smooth-muscle cells and abnormalities of verse right upper quadrant, muscle-splitting incisionintestinal hormones. provides excellent exposure and direct access to the Typical clinical presentation of infants with IHPS pylorus with minimal retraction. Another incisionis non-bilious vomiting usually occurring at 2–8 that is commonly used is a supra-umbilical fold inci-weeks of age. Initially there is only regurgitation of sion.Although supra-umbilical skin-fold incision hasfeeds, but over several days vomiting progresses to be a better cosmetic result, it has been argued that deliv-characteristically projectile. It occasionally contains ery of the pyloric tumour can be difficult and timealtered blood in emesis appearing as brownish dis- consuming and may damage the serosa of the stom-colouration or coffee-grounds as a result of gastritis ach or duodenum by tearing. Recently, laparoscopicand/or oesophagitis. pyloromyotomy has been advocated. The main ad- The diagnosis is usually based on the clinical his- vantage of the laparoscopic pyloromyotomy is thetory and physical examination of a “palpable pyloric superior cosmetic result.tumour”. Ultrasonographic scanning of abdomen re-
    • Takao Fujimoto 172 Figure 18.1 A nasogastric tube must be placed before the induc- underlying external oblique, internal oblique and tion of anaesthesia if the tube was not placed pre-op- transverse muscles are split. The peritoneum is eratively. And if the barium meal study has been car- opened transversely in the line of the incision. ried out prior to surgery, it may be necessary to re- When supra-umbilical skin fold incision is em- move the residual barium meal by gastric aspiration ployed, a circumumbilical incision is made through and irrigation. The patient is placed in the supine po- about two-thirds of the circumference of the umbili- sition. After the induction of anaesthesia and endo- cus. The skin is undermined in a cephalad direction tracheal intubation, careful abdominal palpation will above the umbilical ring and the linea alba is ex- usually identify the site of the pyloric tumour. A 2.5- posed. The linea alba is divided longitudinally in the to 3-cm long transverse incision is made lateral to the midline from the umbilical ring to as far cephalad as lateral border of the rectus muscle. The incision is necessary to allow easy delivery of the pyloric tu- deepened through the subcutaneous tissue and the mour. Figure 18.2 The stomach is identified and is grasped proximal to be delivered through the wound. Grasping the duod- the pylorus with non-crushing clamp and brought enum or pyloric tumour directly by forceps often re- through the wound. Then, the greater curvature of sults in serosal laceration, bleeding or perforation, the stomach can be held in a moist gauze swab, and therefore should be avoided. with traction inferiorly and laterally, the pylorus can18
    • Chapter 18 Hypertrophic Pyloric Stenosis 173 Figure 18.1 Figure 18.2
    • Takao Fujimoto 174 Figure 18.3 The pylorus is held with surgeon’s thumb and fore- along the gastric antrum. The incision should go far finger to stabilize and assess the extent of hypertro- enough onto the gastric antrum at least 0.5~1.0 cm phied muscle. A seromuscular incision is made over from the antropyloric junction where the muscle is the avascular area of pylorus with a scalpel, com- thin. mencing 1~2 mm proximal to the pre-pyloric vein Figure 18.4, 18.5 The scalpel handle is used to further split the hyper- Loose prolapsing of intact mucosa is evidence of a trophied muscle down to the submucosal layer. Then satisfactory myotomy. To test the mucosal injury, the pyloric muscle is spread widely. Spreader is placed at stomach is inflated through the nasogastric tube, and the midpoint of incision line and muscle is spread passage of air through the pylorus to duodenum is perpendicularly and spreading must be continued confirmed. Then the pylorus is dropped back into the proximally and distally. Gentle spreading is required abdomen. Bleeding from the myotomy edge or sub- to obtain a complete myotomy. Mucosal tears are mucosal surface is frequently seen; however, it is gen- most common at the pyloroduodenal junction be- erally venous and always stops after returning the py- cause of the attempt to split all remaining muscle fi- lorus to the abdominal cavity. Posterior rectus fascia bres. In order to reduce the risk of mucosal tear, care and peritoneum is approximated with a running 4/0 should be taken when spreading pyloric muscle fi- absorbable suture material and anterior fascia is bres at the duodenal end. closed with 5/0 absorbable suture material.18
    • Chapter 18 Hypertrophic Pyloric Stenosis 175 Figure 18.3 Figure 18.4 Figure 18.5
    • Takao Fujimoto 176 Figure 18.6 For the laparoscopic procedure the patient is placed ity after laparoscopic pyloromyotomy is completed. in the supine position at the end of the operating ta- Intra-abdominal pressure is maintained at 8 mmHg, ble (or 90º to the anaesthesiologist). The video mon- and insufflation rate is set at 0.5 l/min. In the right itor is placed at the head of the table, and the surgeon mid-clavicular line just below the costal margin (just stands at the end of the table with the assistant to the above the liver edge), a no. 11 scalpel blade is used to patient’s right. The abdomen is scrubbed and draped make a 2- to 3-mm stab incision under direct vision. in a sterile fashion. Attention must be paid to ensure Also using the no. 11 scalpel blade, a second stab inci- the appropriate preparation of the umbilicus. sion is made under direct vision, just below the costal The access sites are injected with local anaesthetic margin in the left mid-clavicular line. (0.25% bupivacaine) with epinephrine, which is used An atraumatic grasper is placed directly through the to reduce the post-operative pain and reduce the risk right upper quadrant stab wound and is used to re- of bleeding from the stab wound. The author prefers tract the inferior border of the liver superiorly and an open procedure for insertion of the primary port. expose the hypertrophic pylorus. A retractable myo- A 4.0- to 5.0-mm curvilinear supra-umbilical inci- tomy knife (retractable arthrotomy knife or Endo- sion is made and carried down to the peritoneal cav- tome) is inserted directly through the left stab ity. At the level of umbilical fascia, 4/0 absorbable su- wound. Working ports are usually not necessary and ture material is placed circumferentially to anchor instruments are directly introduced through these the port and to use for closure of the peritoneal cav- stab wounds.18
    • Chapter 18 Hypertrophic Pyloric Stenosis 177 Figure 18.6 Video monitor Surgeon Anesthesiologist Scrub nurse Assistant
    • Takao Fujimoto 178 Figure 18.7 The working instruments, retractable myotomy es the proximal margin of hypertrophied muscle that knife, atraumatic laparoscopic grasper are used to as- is seen as a deep fold in the wall of stomach. sess the extent of the hypertrophied pylorus by pal- A seromuscular incision is made over the hyper- pating the margins of the pylorus as one would use trophic pylorus with retractable myotomy knife com- with thumb and forefinger in the open procedure. mencing at 1–2 mm proximal to the pyloroduodenal The duodenum is then grasped just distal to the junction extending to the gastric antrum. The inci- pyloric vein (pyloroduodenal junction) and retract- sion should go far enough onto antrum at least ed using the atraumatic grasper to expose the avas- 0.5~1.0 cm proximal to antropyloric junction. Care cular surface of hypertrophic pylorus. The tips of po- must be taken at this stage that this incision is deep sitioning the pylorus for myotomy is that lateral and enough to allow the insertion of the pyloric spreader slightly anterocephalad retraction of the distal pylor- blades and must penetrate the pyloric muscle some- us achieve excellent exposure of the avascular surface what deeper than is usual with the conventional open of hypertrophic pylorus. This manoeuvre also expos- procedure. Figure 18.8 After the muscle is incised, the blade is then retract- To test for the mucosal injury, the stomach is in- ed and the sheath of the knife is used to further split flated through the nasogastric tube (160–180 ml) as is the hypertrophied muscle fibre, as the scalpel handle usually done in open techniques. Bulging of the mu- is used in open procedure, until mucosa is visualized. cosal layer with no evidence of defect should be con- The retractable myotomy knife is removed and a la- firmed. Greenish or yellowish fluid at the myotomy paroscopic pyloromyotomy spreader is introduced area is a sign of mucosal tear. into abdominal cavity directly through the left stab After the successful myotomy, the instruments are wound to complete the pyloromyotomy. The spread- withdrawn under direct vision and the pneumoperi- er is placed in the midpoint of the seromuscular inci- toneum is evacuated. The nasogastric tube is also re- sion line and the muscle is spread perpendicularly. moved after completing the surgery. The umbilical Once the initial spread reaches the mucosa, spread- fascia is reapproximated with 4/0 absorbable suture ing must be continued proximally and distally. Push- material, which is already in place, and the skin of all ing the spreader towards the mucosa or rapid spread- the wound is reapproximated with skin adhesive ing can result in mucosal tear. In order to avoid the tapes.18 mucosal tear, the spreader should not be placed at the proximal and distal edges of the incisional (myoto- my) line.
    • Chapter 18 Hypertrophic Pyloric Stenosis 179 Figure 18.7 Pyloric vein Figure 18.8
    • Takao Fujimoto 180 CONCLUSION Pyloromyotomy is the standard therapy for IHPS. of and tension on the pylorus to deliver it through Mortality associated with this operation is very un- the wound can induce oedema in muscle layer, muco- common today. Early diagnosis and proper peri-op- sal swelling and, occasionally serosal laceration. A la- erative management reduces complications. In spite paroscopic pyloromyotomy (LP) is a less traumatic of these advances, there remains about an 8–10% in- operation. The tolerance of an early feeding regimen cidence of associated peri-operative morbidity such in the LP confirms that there is lack of trauma to the as perforation, wound infection and wound dehis- pylorus during the procedure. We feel this is the most cence. considerable benefit of LP. Use of 3.0-mm instru- In an open procedure, essentially right umbilical ments allow us to improve the cosmesis. incision and circumumbilical incision, manipulation SELECTED BIBLIOGRAPHY Fujimoto T, Lane GJ, Segawa O et al (1999) Laparoscopic ex- Puri P, Lakshmanadaas G (2003) Hypertrophic pyloric stenosis tramucosal pyloromyotomy versus open pyloromyotomy In: Puri P (ed) Newborn surgery. Arnold, London, pp 389– for infantile hypertrophic pyloric stenosis: which is better? 398 J Pediatr Surg 34 : 370–372 Tan KC, Bianchi A (1986) Circumumbilical incision for pylo- Leinwand MJ, Shaul DB, Anderson KD (1999) The umbilical romyotomy. Br J Surg 73:399 fold approach to pyloromyotomy: is it a safe alternative to right upper quadrant approach. J Am Coll Surg 189 : 362– 36718
    • CHAPTER 19 Gastrostomy Michael W. L. Gauderer INTRODUCTIONIn infants and children, gastrostomies are indicated trostomy only. If, on the other hand, they are not, anprimarily for long-term enteral feedings and less fre- anti-reflux operation is done in conjunction with thequently for decompression or a combination of both. gastrostomy. If the need to control reflux surgicallyIn the last three decades, advances in peri-operative arises at a later date, an anti-reflux operation may bemanagement have led to a more selective use of gas- added, usually without taking down the gastrostomy.trostomies in patients with various typical paediatric There are three basic methods of constructing asurgical conditions such as congenital anomalies of gastrostomy.the gastrointestinal tract and the abdominal wall. On First is the formation of a serosa-lined channelthe other hand, there has been a markedly increased from the anterior gastric wall around a catheter. Thisutilization of gastrostomies in infants and children catheter is placed in the stomach and made to exit ei-without surgical pathology. The main indication for ther parallel to the serosa as in the Witzel technique,direct gastric access in these patients is an inability to or vertically as in the Stamm or Kader methods. Theswallow, usually secondary to central nervous system anterior gastric serosa is apposed to the peritonealimpairment.An additional indication for a gastrosto- surface of the anterior abdominal wall with sutures.my is the need to provide feeding supplementation in The Stamm technique is the most widely employedchildren unable or unwilling to consume adequate gastrostomy with celiotomy. It can be used in chil-calories orally. Other uses of gastric stomas include dren of any size and even on the smallest stomachaccess for oesophageal bougienage and the long- (e.g., in newborns with oesophageal atresia withoutterm administration of unpalatable diet or medica- fistula).tions. Second is the formation of a tube from a full- When feeding is the main indication, two impor- thickness gastric flap, leading to the skin surfacetant questions must be addressed. where it is anchored with sutures. A catheter is then First, nasogastric tube or gastrostomy? Nasogastric introduced intermittently for feeding. The construc-tubes should be preferred if the expected duration of tion of a gastric wall tube is seldom used in children.enteral access is less than 1 or 2 months, because the The technique is more time consuming, difficult tonewer small feeding tubes are highly biocompatible perform in small children, not suited for the passageand remain smooth and soft for prolonged periods of of dilators and is more prone to leakage at the skintime. Gastrostomies should be considered when gas- level unless an anti-reflux manoeuvre is added, fur-tric access is expected to last more than several ther complicating this approach. This method alsomonths. interferes with reoperations on the stomach, because Second, gastrostomy only or gastrostomy plus anti- part of the gastric wall has been used for the conduit.reflux operation? Neurologically impaired children, The third method consists of percutaneous tech-the main candidates for a gastrostomy, frequently niques in which the introduced catheter holds thehave foregut dysmotility and associated gastro-oe- gastric and abdominal walls in apposition. Thesesophageal reflux. Because gastrostomies can unmask procedures are based on the principle of suturelessreflux, these children should be evaluated prior to approximation of a hollow viscus to the abdominalplacing a stoma, usually with an upper gastrointesti- wall. In addition to the original method, in whichnal contrast series and a pH probe study. Endoscopy gastroscopy was employed, the catheter may also bewith biopsy, manometry and gastric emptying stud- placed with radiological or, more recently, laparos-ies may be added, if deemed necessary. Unfortunate- copic assistance. Laparoscopic control can also bely, these studies are not particularly helpful in pre- employed to enhance the safety of percutaneous gas-dicting post-gastrostomy reflux. For this reason, we trostomy placement in select patients with abnormalemploy a trial of nasogastric tube feedings for 1 to 2 upper abdominal anatomy in whom injury to adja-weeks. If these are well tolerated, we place the gas- cent organs, such as the colon, is a concern.
    • Michael W. L. Gauderer 182 Percutaneous endoscopic gastrostomy (PEG) was us, potential for gastric bleeding or wound disrup- initially developed for high-risk paediatric patients tion. There is only minimal interference with subse- to allow precise tube placement with endoscopic as- quent operations on the stomach. The likelihood of sistance, without celiotomy. Depending on how the an infection is very small and similar to that of the catheter is inserted, the three main variations are: Stamm procedure. PEG is not generally suited for the pull (Gauderer-Ponsky), push (Sachs-Vine) and in- passage of dilators. troducer or “poke” (Russell) methods. The first gas- With certain modifications, either one of these trostomy without celiotomy, the Gauderer pull PEG basic interventions can be performed by minimally remains the most widely employed gastrostomy, both invasive techniques or in conjunction with laparos- in adult and paediatric patients. The procedure time copy. is short and there is practically no postoperative ile- Figure 19.1 The Stamm gastrostomy operation is performed us- cised transversely and the muscle retracted or tran- ing general endotracheal anaesthesia. A single dose sected. The catheter exit site should be approximate- intravenous antibiotic is administered. A nasogastric ly at the junction of the lower two-thirds and the tube may be inserted to evacuate the contents and upper one-third of a line drawn from the umbilicus help identify the stomach in children with abnormal to the mid-portion of the left rib cage, over the mid- upper abdominal anatomy. The child is positioned rectus muscle. A vertical incision may be useful in with a small roll behind the back to elevate the epi- children with a high-lying stomach or a narrow co- gastrium, then prepared and draped. In infants, a stal angle. The catheter exit site should not be too thin plastic, small-aperture drape is used to help with close to the rib cage because, with the child’s growth, temperature maintenance. We prefer to use silicone this distance tends to become shorter. A gastric ac- rubber de Pezzer-type catheters ranging in size from cess device that is too close to the ribs will cause dis- 12F (full-term neonates) to 20F for adolescents, or comfort and interfere with care. Additionally, the ex- PEG-type catheters in which the “dome” has been cessive pivoting motion resulting from breathing and modified to allow insertion with a stylet. For pre- moving will lead to stoma enlargement and leakage. term infants or neonates with a very small stomach, a Catheters should not be brought out through the in- 10F T-tube or Malecot catheter is employed. The pro- cision because this approach predisposes the site to cedure may be modified slightly to accommodate the wound complications and leakage. The linea alba initial placement of a skin-level device or “button”. tends to be broad and very thin in small children and (See Fig. 7). should also be avoided as an exit site. The stomach is approached through a short trans- verse supra-umbilical incision. Fascial layers are in-19
    • Chapter 19 Gastrostomy 183 Figure 19.1
    • Michael W. L. Gauderer 184 Figure 19.2 Figure 19.3 The selection of the gastrotomy site on the anterior A lower guy suture may be added to pull the stomach gastric wall is very important. The mid body is best caudally, enhancing the exposure and allowing better suited for the catheter insertion. The opening should gastric access. The gastrotomy is performed with fine be away from the gastric pacemaker at the level of the scissors or cautery while the upper guy sutures are splenic hilum; away from the greater curvature be- lifted to prevent injury to the posterior gastric wall. cause that site may be needed for construction of a The de Pezzer catheter is introduced using a simple gastric tube and because proximity to the transverse stylet while the sutures are elevated. colon could eventually lead to a gastro-colic fistula; away from the fundus to allow for a possible future fundoplication. It is critical to avoid the antrum to prevent pyloric obstruction by the catheter tip and interference with gastric emptying. A stoma in this position is also more likely to leak. If the catheter is to be placed cranially and close to the lesser curvature of the stomach with the intent of creating an anti- reflux mechanism, care must be taken to avoid the vagus nerve. Traction guy sutures lifting the gastrotomy site, and a purse-string suture of age appropriate synthet- ic absorbable material are placed. The diameter of the purse-string should be adequate for invagination of the gastric wall upon insertion of the catheter, but not excessive to avoid narrowing of the stomach. Figure 19.4, 19.5 A continuous synthetic absorbable monofilament su- through. The catheter end is grasped and the tube ture (polydioxanone) is used to anchor the stomach brought out through the counter incision. to the anterior abdominal wall. A clamp is placed Placement of the continuous monofilament suture through the counter-incision and the abdominal wall is then completed. When tied, this suture provides a layers pushed inward. After the posterior 180° of the 360° fixation of the stomach to the abdominal wall “anastomosis” are completed, the peritoneum and and a watertight seal. In most cases, this manoeuvre fascia are incised and the tip of the clamp pushed obviates the need for a second purse-string suture.19
    • Chapter 19 Gastrostomy 185 Figure 19.2 Figure 19.3 Figure 19.4 Figure 19.5
    • Michael W. L. Gauderer 186 Figure 19.6 The abdominal wall layers are closed with synthetic monofilament sutures (polypropylene or nylon). absorbable sutures and the skin is approximated These are removed 1–2 weeks after the operation. If with subcuticular stitches and adhesive strips. The the tube is to remain long, a small immobilizing wound is infiltrated with a long-lasting local an- crossbar is added to prevent distal catheter migra- aesthetic. The catheter is secured with synthetic tion. Figure 19.7 The standard procedure shown may be modified to ditional few millimetres of “play” to allow for postop- allow for the primary insertion of a skin-level gas- erative oedema, ease of care and subsequent growth trostomy device. The gastrostomy balloon-tipped and weight gain. Following insertion, the stomach is “buttons” are available in different shaft sizes and di- vented through the skin-level device or by means of a ameters. The shaft’s length should encompass the in- nasogastric tube. vaginated gastric wall, the abdominal wall and an ad-19
    • Chapter 19 Gastrostomy 187 Figure 19.6 Figure 19.7
    • Michael W. L. Gauderer 188 Figure 19.8 Percutaneous endoscopic gastrostomy (PEG) is best children and adolescents. Hybrid catheters leading to performed in the operating room. In older children the primary implantation of skin-level devices are al- and those able to tolerate endoscopy without com- so available. Immediate conversion to a skin-level de- promising the upper airway, the procedure may be vice is our favoured approach. undertaken using local anaesthesia with sedation as Contraindications to PEG are inability to perform needed. Younger children require general endotra- upper tract endoscopy safely or to identify transab- cheal anaesthesia primarily because of anticipated dominal illumination and clearly recognize an ante- difficulties with the airway management. Two indi- rior gastric wall indentation. Anatomical abnormal- viduals are required – one for the endoscopy and one ities such as intestinal malrotation or marked scolio- for the insertion of the guide wire and pulling back sis, ascitis, coagulopathy, and intra-abdominal infec- the catheter.A single dose of a broad-spectrum intra- tion, if severe, may render the procedure inadvisable. venous antibiotic is given shortly before the proce- One must also be cautious in the presence of intra- dure. For the endoscopy, the smallest available flex- peritoneal shunts. ible paediatric gastroscope is used. The catheter with The site for catheter placement is similar to the its retaining internal crossbar, “cup”, “dome” or disk one described for the Stamm procedure. The stoma must be soft and collapsible enough to glide atrau- should be away from the rib cage for the reasons matically through the oropharynx and oesophagus. mentioned above and to allow placement of an inci- A 14F to 16F silicone rubber catheter is well suited for sion if a fundoplication becomes necessary in the fu- younger children and a 20F tube is used for older ture. Figure 19.9 The abdomen is prepared and draped. The catheter Once the stomach is appropriately distended, dig- site is tentatively selected. The gastroscope is insert- ital pressure is applied to the proposed gastrostomy ed but the stomach is not immediately insufflated. site, which usually corresponds to the area where The snare is advanced into the operating channel of transillumination is brightest. Transillumination and the scope. Once all necessary equipment is available clear visualization of an anterior gastric wall inden- and ready for use, the room lights are dimmed and tation are key points. Without these, laparoscopic the stomach insufflated. Under-insufflation or, more control or an open gastrostomy should be employed. importantly, over-insufflation should be avoided to If additional confirmation of proper relation of the minimize the possibility of accidentally piercing the structures is desired, a fine spinal needle may be in- colon. Excessive insufflation of the small intestine troduced through abdominal and gastric walls. Be- tends to push the transverse colon in front of the cause the length of the needle is known, one can esti- stomach and into harm’s way. It also distorts the mate the distance between the gastric mucosa and stomach, which may interfere with correct catheter the skin. Excessive length warrants caution. placement.19
    • Chapter 19 Gastrostomy 189 Figure 19.8 Figure 19.9
    • Michael W. L. Gauderer 190 Figure 19.10, 19.11 The stoma site is infiltrated with a long acting local nula, which will allow the placement of the guide- anaesthetic. An incision of 8–10 mm is made trans- wire, is placed in the incision between the slightly versely in the skin and a curved haemostat applied to spread prongs of the haemostat. The needle-cannula maintain the intragastric indentation. The gastro- is then firmly thrust through the abdominal and gas- scope is moved gently in small increments. The en- tric walls, exiting through the tip of the mound into doscopist then places the polypectomy snare around the loop of the polypectomy snare. The snare is par- or over this “mound”. The intravenous needle-can- tially closed, but not tightened around the cannula.19
    • Chapter 19 Gastrostomy 191 Figure 19.10 Figure 19.11
    • Michael W. L. Gauderer 192 Figure 19.12, 19.13 The needle is removed and the plastic-coated looped tor or biopsy forceps.) The guide-wire is then pulled steel guide-wire inserted through the cannula. The back with the endoscope from the stomach, through polypectomy snare is allowed to slide away from the the oesophagus, exiting from the patient’s mouth. cannula and is tightened around the guide-wire. (An The guiding tract is thus established. alternative method is to retrieve the wire with alliga-19
    • Chapter 19 Gastrostomy 193 Figure 19.12 Figure 19.13
    • Michael W. L. Gauderer 194 Figure 19.14, 19.15 The catheter is attached to the guide-wire by inter- markings on the catheter shaft help in judging the locking the two steel wire loops and lubricated. Trac- correct distance from mucosa to skin. The external tion is applied to the abdominal end of the guide- crossbar is slipped over the catheter and guided to wire, guiding the catheter through oesophagus, the skin level. The cross bar is advanced only enough stomach and across gastric and abdominal walls. to produce a good approximation of the gastric sero- With the age appropriate catheter, the gastric retain- sa to the abdominal peritoneum. Excessive approxi- er collapses enough to slide through the oesophagus mation can lead to ischemia with tissue necrosis and without producing injury (for diagrammatic purpos- embedding of the retainers. The catheter is cut to the es, a shortened catheter is shown). The commercially desired length and the feeding adaptor is attached. available catheters are long enough to permit the ta- No sutures are used and the catheter is connected to pered end of the tube to exit through the abdominal a small clear plastic trap. A dry gauze pad and tape wall before the gastric retainer enters the patient’s are applied without kinking the tube. Alternatively, mouth, allowing complete control of the catheter the catheter can be converted to a skin-level device during placement. Traction is continued until the with the changeable port-valve. gastric and abdominal walls are in loose contact. The19
    • Chapter 19 Gastrostomy 195 Figure 19.14 Figure 19.15
    • Michael W. L. Gauderer 196 CONCLUSION Enteral feedings are begun following an open gas- a supplement in the introducer-type PEG technique), trostomy once the ileus has resolved, and on the day it is acceptable to insert a balloon-type catheter in after the operation following a PEG or a laparoscopic the stomach and obtain an immediate water-soluble gastrostomy. Although it has been shown that enteral contrast study to ascertain the correct position. feedings following PEG can be started as early as a However, if no fixation was used, as in the case of the few hours post-procedure, we have maintained the described PEG, a more aggressive approach is need- original, more conservative approach. The dressing is ed and a celiotomy usually indicated. Laparoscopy removed after 24–36 h, the wound is examined and may be used as a safe alternative. A re-do PEG alter- the external retaining crossbar loosened if necessary. native has also been described. Fortunately, acciden- Thereafter, the wound is cleaned with mild soap and tal PEG catheter removal is very rare in children. water only. Granulation tissue tends to form after a Most separations stem from a catheter change. Pneu- few weeks and is controlled with silver nitrate sticks. moperitoneum following a percutaneous gastrosto- If excessive, the area is anaesthetized locally, the my is common but, fortunately, without sequelae. granulation tissue excised and the tract cauterized. If One of the most troublesome long-term problems the problem is recurrent, we have had good results is severe leakage from the gastrostomy tract. Initially, with a cream containing a mixture of topical steroid this should be managed using conservative meas- and an anti-fungal. The abnormal growth ceases ures, such as using smaller catheters to allow the wid- once the skin rim grows inward creating a lined gas- ened fistula to contract. If these fail, the stoma may be tro-cutaneous tract. If a long catheter needs to be re- relocated using a simple non-endoscopic variation of moved in order to be replaced by another catheter or the percutaneous endoscopic gastrostomy. A new a skin-level device other than the port-valve, this ma- stoma site is selected and a small incision made. A noeuvre should be done with great care. This is par- large curved needle is placed through the leaking ticularly true for the initial change following a PEG. stoma, exiting through the new site. The suture is The time required to form a firm adherence between pulled through, establishing a tract. The catheter fol- the gastric serosa and the abdominal wall peritone- lows the tract, entering through the malfunctioning um following percutaneous techniques, varies. We stoma and exiting through the new one. Once the have used 3 months as a guideline, but shorter peri- catheter is in place – as following the described pull ods may be adequate, provided appropriate safe- PEG here – the leaking stoma is closed extraperito- guards are used. Patients on steroids or children with neally. cyanotic heart disease are at particular risk of separ- If a long-standing gastrostomy is no longer need- ation during early catheter change. ed, the gastric access device is simply removed. If the tract is roughly less than 1 year old, it will usually í Complications. Although generally considered a close fully spontaneously. However, well-established, basic procedure, gastrostomy is associated with a skin- and mucosa-lined older gastro-cutaneous fis- long list of complications related to technique, care tulae will continue to drain. Simple, extraperitoneal and catheter use. Serious technique-related problems excision of the tract with a few sutures in the fascia, include separation of the stomach from the abdomi- subcutaneous layer and skin suffice to close the com- nal wall, wound separation, haemorrhage, infection, munication. injury to the posterior gastric wall or other organs, and placement of the tube in an inadequate site. The í Follow-up. All children with gastrostomies must most common and potentially lethal complication is be carefully followed to prevent long-term gastric ac- early complete or partial gastric separation. If the cess device-related complications and monitored for stomach was initially secured to the abdominal wall adequate nutritional management as well as mani- as in the Stamm procedure or secured with T-fasten- festations of foregut dysmotility, particularly gastro- ers (advocated by some surgeons managing adults as oesophageal reflux.19 Figure 22.1 Gauderer MWL (2002) Percutaneous endoscopic gastrostomy Gauderer MWL, Abrams RS, Hammond JH (1998) Initial expe- and the evolution of contemporary long-term enteral ac- rience with the changeable skin-level port-valve: a new cess [Review]. Clin Nutr 21 : 103–110 concept for long-term gastrointestinal access. J Pediatr Gauderer MWL, Stellato TA (1986) Gastrostomies: evolution, Surg 33 : 73–75 techniques, indications, and complications [Monograph]. Sampson LK, Georgeson KE, Winters DC (1996) Laparoscopic Curr Probl Surg 23 : 658–719 gastrostomy as an adjunctive procedure to laparoscopic Gauderer MWL, Ponsky JL, Izant RJ Jr (1980) Gastrostomy fundoplication in children. Surg Endosc 10 : 1106–1110 without laparotomy: a percutaneous endoscopic tech- Vanek VW (2003) Ins and outs of enteral access. Part 2 – long- nique. J Pediatr Surg 15 : 872–875 term access – esophagostomy and gastrostomy [Review]. Nutr Clin Pract 18 : 50–74
    • CHAPTER 20 Malrotation Agostino Pierro, Evelyn GP Ong INTRODUCTIONMalrotation is congenital abnormal positioning of ciated with situs inversus, asplenia and polysplenicthe midgut. Intestinal development is traditionally syndromes.described as a process of elongation, rotation and fix- Acute bowel obstruction due to Ladd’s bands oration. The process begins in the fifth week of gesta- intermittent midgut volvulus can present with vom-tion. Elongation of the bowel exceeds abdominal cav- iting, typically bilious, as the commonest presentingity expansion and the bowel herniates from the ab- feature accompanied by colicky abdominal pain anddomen. As the bowel returns to the abdomen, it ro- abdominal distention. An infant with abdominal ten-tates 270° anticlockwise around the superior mesen- derness and blood per rectum is suggestive of bowelteric artery (SMA). Rotation is completed by week 10 ischaemia due to midgut volvulus. Older childrenof gestation, with the SMA contained within a broad without acute volvulus more often present withmesenteric base attachment. The distal duodenum chronic episodic obstructive symptoms, failure tocomes to lie across the midline towards the left upper thrive, malabsorption, diarrhoea and non-specificquadrant, attached by the ligament of Treitz at the colicky abdominal pain. Up to 10% of diagnoses ofduodeno-jejunal (D-J) flexure to the posterior ab- malrotation are made as an incidental finding.dominal wall. The caecum passes to the right and Plain abdominal radiograph is often normal butdownwards and becomes fixed to the posterior ab- features suggestive of malrotation with or withoutdominal wall. This latter process may be incomplete midgut volvulus are a distended stomach and proxi-at birth giving rise to a “high” caecum, a variant of mal duodenum with a paucity of gas distally, eithernormal in the neonate. throughout or unilaterally. An upper gastrointestinal The commonest features of malrotation are: (1) contrast study is the investigation of choice for anythe D-J flexure lies right of midline, (2) the dorsal child presenting with bilious vomiting and should bemesenteric attachment is narrow, and (3) peritoneal performed urgently. Findings in malrotation are: (1)folds cross from colon and caecum to duodenum, liv- D-J flexure right of left vertebral pedicle and/or infe-er and gallbladder (Ladd’s bands), thus possibly ob- rior to pylorus, (2) the duodenum passes caudallystructing the duodenum. Whether Ladd’s bands are and anteriorly, and (3) contrast tapering or a “cork-substantial enough to cause mechanical obstruction screw” appearance suggests obstruction and/or vol-is debatable. The narrowed mesenteric base can lead vulus.to midgut volvulus, bowel obstruction and mesenter- In a recent series, sensitivity and specificity of thisic vessel occlusion. Antenatal volvulus can result in test were 92% and 20%, respectively. Caecal positionbowel atresia. is highly variable and may be normal in up to 15% of Malrotation is estimated from autopsy studies to cases of malrotation. Contrast enema is therefore notoccur in 0.5–1% of the population, although only 1 in always helpful.6000 live births will present with clinical symptoms. Abdominal ultrasound may show reversal in theIncidence is slightly higher in males than females. relationship of SMA to superior mesenteric veinFifty to 75% of patients become symptomatic in the (SMV). In a normal situation the SMV is located tofirst month of life and 90% will present before 1 year the right of the SMA, while SMV to the left of the ar-of age but presentation can occur at any age. Malrota- tery is suggestive of malrotation.tion is present in patients with gastroschisis, exom- All symptomatic patients with positive investiga-phalos and congenital diaphragmatic hernia. Coexis- tive findings should undergo urgent laparotomy.tent congenital anomalies (cardiac anomalies, bowel Management of the asymptomatic patient is moreatresia, duodenal web, anorectal anomalies, ortho- controversial. The risk of bowel ischaemia due topaedic anomalies) are common and affect 50% of midgut volvulus is invariably present and the major-children with malrotation. Malrotation is also asso- ity of surgeons would proceed to prompt operation.
    • Agostino Pierro, Evelyn GP Ong 198 Figure 20.1 The principles of the procedure have remained al- peritoneal fluid is examined. Frequently it is clear; most unchanged since originally described by Ladd bloodstained fluid implies bowel ischaemia and vol- in 1936. The patient is positioned supine, legs extend- vulus; faecal staining indicates bowel perforation and ed. A right upper quadrant transverse incision is should be cultured. made. The umbilical vein is divided and ligated. The Figure 20.2, 20.3 The midgut is delivered from the wound and the base primary anastamosis formed. If extensive ischaemic examined. Any volvulus should be derotated anti- bowel of doubtful viability is present, a second-look clockwise, noting the number of turns. The bowel is laparotomy is performed after 24 h with the aim of examined for viability and any ischaemic bowel minimizing the extent of bowel resection required. should be wrapped in a damp swab and re-examined Ladd’s bands are divided. after 5–10 min. Non-viable bowel is resected and a20
    • Chapter 20 Malrotation 199 Figure 20.1 Figure 20.2 Figure 20.3
    • Agostino Pierro, Evelyn GP Ong 200 Figure 20.4 The SMA is identified and mesenteric base broad- closed. The nasogastric (NG) tube is aspirated hour- ened as much as possible by division of the perito- ly for the first 24 h. Intravenous fluids are continued neal folds. Care must be taken not to injure the super- postoperatively and NG tube fluid loss is replaced, ior mesenteric vessels. The abnormal position of the millilitre for millilitre, with normal saline and potas- appendix may cause diagnostic problems in future sium chloride (20 mmol/l saline). Enteral feeds are and, therefore, removal is advocated. The bowel is re- restarted when aspirates are clear and reducing in placed with the duodenum to the right and the cae- volume, usually after 24 h. cum in the left upper quadrant. The abdomen is Figure 20.5 Figure 20.6 Laparoscopy may be used in non-acute cases of mal- The anatomy is defined and Ladd’s bands identified. rotation without volvulus, e.g., in incidentally diag- Care must be taken to correctly identify landmarks nosed malrotation. The patient is positioned supine such as the duodenum and ascending colon. To gain with the legs abducted. The surgeon stands between access to the duodenum, it is useful to raise the head the patient’s feet with the assistant to the left of the of the operating table and elevate the right flank. The patient. The umbilical port is placed first. A perium- ascending colon falls towards the left side of the ab- bilical incision is made. The midline fascia is held in domen. The duodenum is exposed and Ladd’s bands two arterial clips, one on either side of the midline. are divided using either an ultrasonic blade or a com- The linea alba is divided and a 5- or 10-mm port bination of sharp dissection and electrocautery.After placed into the abdominal cavity under direct vision. division, the bowel is examined along its length for The port is secured with a purse-string and the ends any further causes of obstruction. The root of the of the sutures attached to an anchor on the port. Car- mesentery is broadened by dividing the peritoneal bon dioxide is insufflated via the port until a final folds. Care must be taken in not injuring the superior intra-abdominal pressure of 8–10 mmHg is reached mesenteric vein. Appendicectomy is carried out ei- in an infant, or 10–12 mmHg in an older child. During ther using an endoloop for intracorporeal ligation or insufflation the abdomen is palpated and percussed by delivering the appendix through a trocar site and to ensure adequate pneumoperitoneum is achieved. excising it extra-abdominally in smaller patients. The flow rate of carbon dioxide is set between 0.5 and Trocar sites are closed. 1.5 l/min. The laparoscope is then inserted into this port. Two further 5-mm ports are placed under direct camera vision – left lower quadrant and right lower20 quadrant. Non-traumatic grasping forceps are insert- ed into these ports to manipulate the bowel.
    • Chapter 20 Malrotation 201 Figure 20.4 Superior mesenteric vessels Figure 20.5 Figure 20.6 Liver Duodenum 1 2 3 Ladd’s bands Caecum
    • Agostino Pierro, Evelyn GP Ong 202 CONCLUSION The outcome of patients undergoing Ladd’s proce- rate of 7–15%; necrosis of more than 75% of the mid- dure for isolated malrotation is very good and the gut is associated with short bowel syndrome. Up to majority make a full recovery. The commonest post- 18% of children with short bowel syndrome on long operative complication is adhesional obstruction term total parenteral nutrition have an original diag- (3–5%). Midgut volvulus occurs in 45–65% of chil- nosis of midgut volvulus. dren with malrotation and still carries a mortality SELECTED BIBLIOGRAPHY Bass KD, Rothenberg SS, Chang JH (1988) Laparoscopic Ladd’s Prasil P, Flageole H, Shaw KS, Nguyen LT,Youssef S, Laberge JM procedure in infants with malrotation. J Pediatr Surg 33 : (2000) Should malrotation in children be treated different- 279–281 ly according to age? J Pediatr Surg 35 : 756–758 Clark LA, Oldham KT (2002) Malrotation. In: Ashcraft KW, Spitz L (2003) Malrotation. In: Puri P (ed) Newborn surgery. Murphy JP, Sharp RJ, Sigalet DL, Snyder CL (eds) Pediatric Arnold, London, pp 435–439 surgery, 3rd edn. WB Saunders, Philadelphia, pp 425–434 Kluth D, Fiegel H (2003) The embryology of foregut. Semin Pe- diatr Surg 12 : 3–920
    • CHAPTER 21 Duodenal Obstruction Yechiel Sweed INTRODUCTIONCongenital duodenal obstruction is the result of sev- so indicated because of the high incidence of asso-eral embryologic defects in foregut development, ca- ciated malformations in other organ systems.nalization or rotation. In addition, abnormal em- Diagnosis is achieved in most cases by plain ab-bryologic relationships between the duodenum and dominal radiographs, which demonstrate dilatedother structures in close anatomic proximity such as stomach and duodenum, giving the characteristicpancreas and portal vein may also lead to congenital appearance of a “double-bubble” sign. No gas is ob-duodenal obstruction. served beyond the second bubble in instances of Ladd classified these lesions as either intrinsic or complete obstruction. In this setting, the plain film isextrinsic. Intrinsic lesions include duodenal atresia, sufficiently diagnostic so that no further imaging ofduodenal stenosis or duodenal web, whereas annular the gastrointestinal tract is necessary. In partial du-pancreas, malrotation, peritoneal bands and anterior odenal obstruction a plain film of the abdomen willportal vein are classified as extrinsic. show a double-bubble appearance but there is usual- The incidence of duodenal obstruction is reported ly some air in the more distal intestine. Early upperto be 1 in 5,000 to 10,000 births. There is a high inci- gastrointestinal contrast radiography is indicated indence of associated anomalies in patients with in- these patients in order to establish the cause of in-trinsic duodenal obstruction, especially Down’s syn- complete duodenal obstruction.drome that occurs in about 30% of these patients. Although duodenal atresia is a relative emergency,Other associated anomalies include: congenital heart the infant should not be rushed to the operatingdisease, malrotation, annular pancreas, oesophageal room until his haemodynamic and fluid and electro-atresia, urinary tract malformations, anorectal lyte status is stable. If the clinical history and findingsanomalies and other bowel atresias. on physical examination indicate that the baby is in The diagnosis of duodenal obstruction may be no distress, and the radiograph is consistent with thesuspected prior to the child’s birth by prenatal ultra- usual presentation of duodenal atresia with no airsonography. It may identify maternal polyhydramni- beyond the second bubble, surgery should be per-os and demonstrate distension of the stomach and formed within the first 2 days of life. However, inthe first portion of the duodenum with swallowed patients with duodenal obstruction caused by malro-amniotic fluid. tation resulting in extrinsic compression related to Although prenatal ultrasonography is an accurate Ladd’s bands across the duodenum, or to acute vol-predictor of duodenal obstruction and allows prep- vulus of the midgut, an immediate surgical explora-aration of parents, physicians, and institutions for tion should be performed.the anticipated arrival of the patient needing prompt Incomplete duodenal obstruction may lead to de-care at birth, it has neither influenced the incidence layed onset of symptoms, and the diagnosis of duod-of associated life-threatening anomalies nor changed enal diaphragm with a central aperture is sometimesthe survival rate. delayed for months or years. The clinical presentation of duodenal obstruction Pre-operative management consists of nasogastricis usually characterized by feeding intolerance and decompression and fluid and electrolyte replace-by onset of vomiting in the first 24 to 48 h of life. ment. Care is taken to preserve body heat and avoidSince 80% of obstructions are located in the postam- hypoglycaemia, since most of these newborn pa-pullary region of the duodenum, vomitus in the ma- tients are premature or small for date. Pre-operativejority of cases is bile stained. A careful physical eval- systemic antibiotics are administered at least 30 minuation for associated anomalies is performed. Car- prior to the start of the operation.diac and renal ultrasonographic examinations are al-
    • Yechiel Sweed 204 Figure 21.1 The baby is placed supine on the table with a small midline and extending laterally into the right upper roll under his upper abdomen and on a warming quadrant. A small incision is made in the posterior blanket. Endotracheal anaesthesia is used. A naso- fascia and peritoneum after these are drawn up with gastric tube is passed to decompress the stomach. An forceps. To enlarge this initial incision, two fingers intravenous infusion is set up. The abdominal skin is are inserted and the fascia and peritoneum are cut prepared by cleaning with prewarmed povidone- along the length of the wound. The underlying struc- iodine. tures are retracted. A transverse supra-umbilical abdominal incision is made 2 cm above the umbilicus starting in the Figure 21.2 After exposing the peritoneal cavity, the surgeon in- then passed distally into the dilated duodenum and spects the entire bowel for the presence of other helps to locate the point of obstruction and deter- anomalies. There may be an associated annular pan- mine if a “windsock” abnormality is present. creas or malrotation in one-third of the patients. If The type of atresia as well as any pancreatic ab- the colon is in a normal position, malrotation is normality (annular pancreas) or the presence of a probably not a coexisting factor. rare preduodenal portal vein are noted. In patients The stomach and first portion of the duodenum with an annular pancreas, the pancreatic tissue are usually thickened and dilated. The liver is careful- should never be divided and should be bypassed. The ly retracted superiorly. The ascending colon and the duodenum distal to the site of obstruction is small hepatic flexure of the colon are mobilized medially and decompressed. The requirements for distal mo- and downwards to expose the dilated duodenum. bilization vary according to the location of the atresia The duodenum is then adequately mobilized and and to the gap between the two segments. If neces- freed from its retroperitoneal attachments – Kocher sary, the ligament of Treitz is divided, and mobiliza- manoeuvre. Great care must be exercised not to dis- tion and displacement of the distal duodenum is per- sect or manipulate either segment of the duodenum formed behind the superior mesenteric vessels, thus medially, to avoid injury to the ampulla of Vater or allowing a satisfactory anastomosis to be performed21 the common bile duct. The tube in the stomach is without any tension.
    • Chapter 21 Duodenal Obstruction 205 Figure 21.1 Figure 21.2
    • Yechiel Sweed 206 Figure 21.3, 21.4 Duodenoduodenostomy is the procedure of choice distal to the occlusion. These are made in such a po- for patients with duodenal atresia, stenosis and an- sition as to allow good approximation of the open- nular pancreas. The two surgical techniques, either ings without tension. The papilla of Vater is located side-to-side duodenoduodenostomy or proximal by observing bile flow. This is performed by gentle transverse to distal longitudinal – “diamond-shape” compression of the gall bladder. anastomosis – may be performed. Diamond-shaped The orientation of the sutures in the diamond- duodenoduodenostomy has been reported to allow shape anastomosis and the overlapping between the earlier feeding, earlier discharge and good long-term proximal transverse incision and the distal longitudi- results. With two traction sutures, the redundant wall nal incision are shown. At this stage a small Nelaton of the proximal duodenum is pulled downward to catheter is passed distally through the opening made overlie the proximal portion of the distal duodenal in the distal segment. 20–30 ml of warm saline is segment. A transverse incision is made in the distal injected to rule atresias distally. The catheter is then end of the proximal duodenum and a longitudinal removed. incision is made in the smaller limb of the duodenum21
    • Chapter 21 Duodenal Obstruction 207 Figure 21.3 C’ A’ B’ D’ D A B C Figure 21.4 C’ B’ A’ B A C
    • Yechiel Sweed 208 Figure 21.5 A single layer anastomosis using interrupted 5/0 or rior wall. Before completion of the anterior part of 6/0 Vicryl sutures with posterior knots tied inside the anastomosis, a transanastomotic feeding tube (5F the posterior wall of the anastomosis and interrupt- silicone) may be passed down into the upper jejunum ed sutures with anterior knots tied outside the ante- for an early post-operative enteral feeding. Figure 21.6 After abdominal exploration and the diagnosis of odenal wall. A longitudinal incision of 2.5–3 cm is duodenal web (identified by the advancement of the performed above the “transitional zone” between the gastric tube into the proximal dilated duodenum) wide and the narrow segments of the duodenum, and two stay sutures are placed at the anterior dilated du- the duodenum is opened.21
    • Chapter 21 Duodenal Obstruction 209 Figure 21.5 Figure 21.6
    • Yechiel Sweed 210 Figure 21.7, 21.8 Two other stay sutures are placed at the margins of Thus, the close relationship of the membrane to the duodenal incision. The windsock duodenal web the papilla of Vater makes its identification mandato- must be clearly identified because the visible transi- ry, before excision of the web. tion from the distended proximal duodenum to the A single 4/0 Vicryl stay suture is placed at the cen- small downstream duodenum may be several centi- tre of the membrane. The web is opened along the metres distal to the base of the web. Traction applied lateral side of the membrane and excision from the at the apex of the web deforms the duodenum at its duodenal wall takes place, leaving a rim of tissue of point of attachment and allows excision at the base. 2–3 mm. The medial portion of the membrane The duodenal membrane is usually localized in should remain intact, thus avoiding damage to the the second part of the duodenum and occasionally in ampulla of Vater. An intermittent bile flow is usually the third portion. It can be complete or with a central seen via the papilla of Vater indicating to the surgeon hole.Anatomically, the ampulla of Vater may open di- the exact line of excision. rectly into the medial portion of the web itself – ante- riorly, posteriorly, or with dual openings into the membrane – or it may open close to it. Figure 21.9 Then the resection line is over sewn using interrupt- A nasogastric tube is left in place for post-opera- ed 5-0 absorbable sutures. The duodenum is then tive gastric drainage. A gastrostomy may be per- closed transversely with interrupted sutures. Because formed if the need is anticipated. Intravenous thera- of the pitfalls in cases of lax membrane that may py and antibiotics are continued post-operatively. bulge downwards distally into the distended duode- The patient is kept without oral intake until stool is num (the so-called windsock phenomenon), and in passed and limited clear or pale-green gastric drain- order to avoid missing the anomaly, the patency of age is noted (<1 ml/kg per h). The commencement of the distal duodenum must be identified by inserting oral feeding may be delayed for several days and oc- a catheter through the duodenotomy before its clo- casionally for 2 weeks or more. Post-operatively, pa- sure. Following completion of the web resection and tients may have a prolonged period of bile-stained closure of the duodenum, the abdominal cavity is ir- aspirate, which is mainly due to the inability of the rigated with 50 ml sterile warm saline. The wound is markedly dilated duodenum to produce effective closed in layers: the peritoneum and the posterior peristalsis. Many surgeons therefore use transanasto- fascia and the anterior fascia by two layers using con- motic tubes for feeding in the early post-operative21 tinuous 4/0 Dexon or Vicryl sutures. The skin is period. closed with a running intracuticular suture using 5/0 Vicryl or Dexon suture.
    • Chapter 21 Duodenal Obstruction 211 Figure 21.7 Figure 21.8 Figure 21.9
    • Yechiel Sweed 212 CONCLUSION Long-term outcome after repair of congenital duod- Early post-operative complications include anas- enal obstruction is excellent with contemporary op- tomotic obstruction, continuing obstruction due to erative survival exceeding 95% and with the majority missed anomalies, leakage at the anastomosis and of patients reported as asymptomatic and with nor- prolonged adynamic ileus. Upper intestinal radio- mal growth. graphic studies are necessary to reveal the source of The main factors contributing to the mortality in the problem. patients with duodenal obstruction are a high inci- The late gastrointestinal complications include dence of associated anomalies, prematurity and low megaduodenum, duodenogastric reflux, gastritis, birth weight. The associated complex cardiac defects peptic ulcer and gastro-oesophageal reflux. Mega- continue to be the leading cause of death in particu- duodenum is a particularly troublesome problem, lar in infants with trisomy 21. However, recent re- which may result either from anastomotic obstruc- views document that advances in both paediatric tion or from an inherent motility disorder of the cardiology, and cardiac surgery in neonates and in- proximal duodenum. fants have reduced this mortality significantly. SELECTED BIBLIOGRAPHY Arnbjornsson E, Larsson M, Finkel Y (2002) Transanastomotic McCollum MO, Jamiesson DH, Webber EM (2002) Annular feeding tube after an operation for duodenal atresia. Eur J pancreas and duodenal stenosis. J Pediatr Surg 37 : 1776– Pediatr Surg 12 : 159–162 1777 Bailey PV, Tracy TFJ, Connors RH et al (1993) Congenital duod- Sweed Y (2003) Duodenal obstruction. In: Puri P (ed) New- enal obstruction: a 32-year review. J Pediatr Surg 28 : 92–95 born surgery. Arnold, London, pp 423–433 Kimura K, Mukohara N, Nighijima E et al (1990) Diamond- shaped anastomosis for duodenal atresia: an experience with 44 patients over 15 years. J Pediatr Surg 25 : 977–97921
    • CHAPTER 22 Jejuno-ileal Atresia Heinz Rode, Alastair J.W. Millar INTRODUCTIONDefects in the continuity of the small bowel can mor- more distal the obstruction the more generalized thephologically be divided into either stenosis or atresia abdominal distension. Proximal jejunal atresia oftenand represent one of the most common causes of presents with gastric distension and one or two loopsneonatal intestinal obstruction. Jejuno-ileal atresia of visible bowel in the upper abdomen relieved byhas a prevalence rate of approximately 1:1000 live nasogastric tube aspiration. With delay in presenta-births, with a third of the infants being either born tion increasing intraluminal pressure and/or secon-prematurely or small-for-date. The anomaly is rarely dary torsion of the proximal atretic distended bowelgenetically determined and <1% of babies will have may lead to ischaemia, perforation and peritonitis.chromosomal or other associated anomalies. The differential diagnosis includes midgut volvu- Most jejuno-ileal atresias or stenoses result from a lus, intestinal stenosis, meconium ileus, duplicationlocalized intra-uterine vascular insult to the develop- cyst, internal hernia, strangulated inguinal hernia,ing bowel with ischaemic necrosis and subsequent Hirschsprung’s disease and ileus due to sepsis, birthreabsorption of the affected segment(s). Additional trauma, maternal medications, prematurity or hypo-pathology in the form of intra-uterine fetal intussus- thyroidism.ception, malrotation and midgut volvulus, throm- The diagnosis of jejuno-ileal atresia can be estab-boembolic occlusions, transmesenteric internal her- lished in most cases by an abdominal roentgenogramnias and incarceration or snaring of fetal bowel in a with air as contrast medium. Erect and supine ab-gastroschisis or exomphalos further supports the dominal radiographs show distended air and fluidischaemic hypothesis. The ischaemic insult also ad- filled loops of bowel.versely influences the structure and subsequent The lower the obstruction the greater the numberfunction of the remaining bowel. Histological and of distended loops of bowel and fluid levels that willhistochemical abnormalities can be observed up to be observed. Occasionally intraperitoneal calcifica-20 cm cephalad from the end of the atretic proximal tion may be seen on the plain radiographs signifyingsegment. The distal bowel is unused and potentially intrauterine bowel perforation, meconium spill andnormal in function. dystrophic calcification. In the presence of complete Prompt recognition of intestinal atresia is essen- obstruction a contrast enema is usually performed totial for the early institution of management. A prena- confirm the level of obstruction and document thetal history of polyhydramnios and ultrasonography calibre of the colon, exclude colonic atresia, and to lo-showing dilated and obstructed fetal intestine are cate the position of the caecum as an indication ofstrong indicators of congenital intestinal atresia. A malrotation. With incomplete upper small bowel ob-positive family history will help to identify heredi- struction an upper gastrointestinal contrast study istary forms. Postnatally, intestinal atresia or stenosis indicated to demonstrate the site and nature of themay present initially with large intragastric volumes obstruction and to exclude midgut volvulus.at birth (>20 ml gastric aspirate) followed by persis- The clinical and radiological presentation of jeju-tent bile-stained vomiting. In 20% of children symp- no-ileal stenosis will be determined by the level andtoms may be delayed for more than 24 h. Abdominal degree of stenosis. The diagnosis is often delayed fordistension is frequently present from birth and the years because of subclinical symptoms and findings.
    • Heinz Rode, Alastair J.W. Millar 214 Figure 22.1–22.3 The morphological classification of jejuno-ileal atre- veloped distal bowel by a fibrous cord along the sia into types I–IV has significant prognostic and edge of the mesentery. The proximal bowel is dis- therapeutic implications. The most proximal atresia tended and hypertrophied for several centimetres. determines whether it is classified as jejunal or ileal. There is no mesenteric defect and the bowel Although single atresias are most commonly en- length is not foreshortened. countered, 6–12% of infants will have multiple atretic í Atresia type III(a) (16%) is similar to type II ex- segments and up to 5% may have a second colonic cept that the fibrous connecting cord is absent and atresia. The appearance of the atretic segment is de- there is a V-shaped mesenteric defect. The bowel termined by the type of occlusion, but in all cases the length may be foreshortened. maximum dilatation of the proximal bowel occurs at í Atresia type III(b) (apple peel) (19%) consists of a the site of the obstruction and is often hypoperistal- proximal jejunal atresia often with associated tic and of questionable viability when presentation is malrotation, absence of most of the superior mes- delayed. enteric artery and a large mesenteric defect. The í Stenosis (12%) is characterised by a short local- distal bowel is coiled in a helical configuration ized narrowing of the bowel without discontinuity around a single perfusing artery arising from the or a mesenteric defect. The bowel is of normal right colic arcades. There is always a significant length. reduction in intestinal length. The infants are usu- í Atresia type I (23%) is represented by a translumi- ally of low birth weight and may have associated nal membrane or short atretic segment causing anomalies. complete intestinal obstruction. The bowel re- í Atresia type IV (20%) represents multiple seg- mains in continuity, has no mesenteric defect, and ment atresias like a string of sausages or a combi- is of normal length. nation of types I–III. Bowel length is always re- í Atresia type II (10%) has the blind-ending proxi- duced. mal bowel attached to the collapsed and underde-22
    • Chapter 22 Jejuno-ileal Atresia 215 Figure 22.1 Stenosis Type I Figure 22.2 Type II Type III(a) Figure 22.3 Type IV Type III(b)
    • Heinz Rode, Alastair J.W. Millar 216 Figure 22.4 The infant is placed supine on a warming blanket and í Single-layer end-to-end or end-to-back anastom- the exposed abdomen cleaned and sealed with plas- osis. tic adherent drapes. Access to the abdominal cavity is í Bowel lengthening procedures have no place dur- obtained through an adequate transverse supra-um- ing the initial surgery. bilical incision transecting the rectus abdominis í Gastric decompression post-operatively is best muscles. The ligamentum teres is divided between achieved with a Replogle tube on low continuous ligatures. suctioning. Neither decompression Stamm gas- Exploration and basic surgical considerations: the trostomy nor transanastomotic feeding tubes are small intestine can easily be exteriorised from the ab- recommended. dominal cavity by gentle pressure on the wound edg- í The fashioning of proximal or distal stomas are es and manual delivery of the intestine. Anatomic- only indicated in the presence of established peri- pathological findings will determine the operative tonitis, or with compromised vascularity of the re- procedure. maining intestine. Steps in the operative procedures are: í Additional steps may include derotation of a prox- í Identification of pathological type and possible imal jejunal atresia, back resection to the distal aetiology. second part of the duodenum and excision or in- í Confirmation of patency of distal small and large version tapering of the duodenum if very dilated. bowel with saline injection (patency of the colon Where total bowel length is significantly reduced could have been demonstrated by contrast enema (type III and type IV), the bulbous dilated seg- prior to surgery). ment proximal to the atresia is conserved. As pro- í Resection of the proximal bulbous atretic segment. grade peristalsis of this bowel is deficient the lu- í Volvulated bowel must be untwisted carefully, es- men calibre should be reduced. Maximum muco- pecially in type III(b) atresia. sal conservation is achieved by inversion plication í Limited distal bowel resection. prior to anastomosis to the distal bowel. í Accurate measurement of residual bowel length proximal and distal to the anastomosis. Figure 22.5 í Detection of Distal Atretic Areas. It is imperative í Bowel Length Measurement. The total length of to exclude distal atresia, which have a prevalence rate the small bowel is measured along the antimesenter- of 6–21%. This is best achieved with a preoperative ic border. Once bowel resection has been completed, barium enema to exclude an associated colonic atre- residual bowel length is of prognostic significance sia and by injecting saline into the distal collapsed and may determine the method of reconstruction es- small bowel and following the fluid column distally pecially in types III and IV atresia. The normal bow- until it reaches the caecum. el length at full term is approximately 250 cm and in the premature infant it is 115–170 cm.22
    • Chapter 22 Jejuno-ileal Atresia 217 Figure 22.4 Ligament of Treitz Figure 22.5
    • Heinz Rode, Alastair J.W. Millar 218 Figure 22.6 The atretic region and the adjacent distended proxi- and distal bowels are of approximately equal size to mal and collapsed distal bowel are isolated with ster- facilitate easy axial or end-to-back (Denis-Browne) ile moist swabs. The intestinal content is milked single-layer anastomosis. However, the discrepancy backwards into the stomach from where it is aspirat- in luminal width of the proximal and distal bowel ed and a bacteriology swab is sent for culture and may vary from 2–5 cm depending on the distance sensitivity. Alternatively, proximal bowel contents are from the stomach. milked into the bulbous blind end if it is to be resect- With type III(b) or high jejunal atresia the proxi- ed. An atraumatic bowel clamp is then placed across mal bowel should be derotated and resection of the the bowel a few centimetres proximal to the elected bulbous portion may be extended into the third or site for transection. second part of the duodenum without jeopardizing If total bowel length is deemed of adequate length the ampulla of Vater. The distal “apple peel” compo- (>80 cm + ileocaecal valve) the bulbous hypertro- nent of Type III(b) atresia may require release of re- phied proximal bowel is resected (5–15 cm) alongside stricting bands along the free edge of the distally the mesenteric bowel border in order to preserve coiled and narrow mesentery to avoid kinking and maximal mesentery for later use, until normal diam- interference with the blood supply. The large mesen- eter bowel has been reached. The bowel should then teric defect is usually left open but with proximal be divided at right angles leaving an opening of ap- bowel resection the residual mesentery can be used proximately 0.5–1.5 cm in width. The blood supply to obliterate the defect. Furthermore, to prevent should be adequate to ensure a safe anastomosis. kinking of the marginal artery after completion of This is followed by very limited distal small bowel re- the anastomosis, the bowel needs to be replaced very section over a length of 2–3 cm. The resection line carefully into the peritoneal cavity in a position of should be slightly oblique towards the antimesenter- non-rotation. ic border to ensure that the openings of the proximal Figure 22.7, 22.8 The anastomosis is either end-to-end or end-to-back anti-mesenteric borders of the divided ends. The “an- (Denis-Browne method); 5/0 or 6/0 absorbable sutu- terior” edges of the bowel are then united with inter- res stitches are used. The mesenteric border of the di- rupted through-and-through extramucosal stitches, vided ends is united with a stay suture and a match- which are tied on the serosal surface. ing stitch is placed at corresponding points of the22
    • Chapter 22 Jejuno-ileal Atresia 219 Figure 22.6 Figure 22.7 Figure 22.8
    • Heinz Rode, Alastair J.W. Millar 220 Figure 22.9 After completion of one-half of the anastomosis the Multiple type IV atresias, present in 18% of in- bowel is rotated through 180° and the “posterior” fants, are often localized necessitating en-bloc resec- anastomosis completed. Alternatively the posterior tion and a single anastomosis, rather than multiple edge of the bowel is anastomosed with the stitches anastomosis. It is important, however, to maintain tied on the mucosal surface followed by anastomosis maximum bowel length to avoid the short bowel syn- of the “anterior” edges with interrupted stitches tied drome. on the serosal surface. The suture lines are inspected Similar techniques are used for intestinal stenosis for anastomotic integrity or tested with saline injec- and type I atresias. Procedures such as simple trans- tion on completion. verse enteroplasties, excision of membranes, bypass- Although isolated type I atresia is best dealt with ing techniques or side-to-side anastomosis are no by primary resection and anastomosis, multiple dia- longer utilized. They fail to remove the abnormal phragms have been successfully perforated with dysfunctional segments of intestine, thus increasing transluminal bougies being passed along the entire the risk of the blind loop syndrome. length of the affected small bowel. Figure 22.10 The defect in the mesentery is repaired by approxi- í Wound Closure. The peritoneal cavity is thorough- mating or overlapping the divided edges with inter- ly irrigated with warm saline to remove all macro- rupted sutures taking great care not to incorporate scopic debris and the bowel then returned to the ab- blood vessels or kinking the anastomosis. Closure of dominal cavity. Care is taken not to kink the anas- the large mesenteric defect can be facilitated by using tomosis. The abdomen is closed by approximating en the preserved mesentery of the resected proximal mass all the layers of the abdominal wall, excluding bowel. Scarpa’s fascia, with a single continuous 4/0 monofil- ament absorbable suture, followed by subcutaneous and subcuticular absorbable stitches. No drains or trans-anastomotic tubes are used.22
    • Chapter 22 Jejuno-ileal Atresia 221 Figure 22.9Figure 22.10
    • Heinz Rode, Alastair J.W. Millar 222 Figure 22.11, 22.12 Alterative surgical techniques may be required if the í Tapering duodeno-jejunoplasty. This method is ischaemic insult has resulted in an atresia with mark- primarily indicated to conserve bowel length (high edly reduced intestinal length, where large resections jejunal atresia, type III(b) atresia) and to reduce dis- of abnormal or multiple atretic segments are re- parity in anastomotic diameter size. The atretic jeju- quired or if the measured residual small intestinal noduodenum is derotated and the antimesenteric length is <80 cm. segment of the dilated proximal segment is resected Indications for tapering are: over a 22–24F catheter. The resection may extend í As part of bowel length preservation where the cephalad to the second part of the duodenum. An in- proximal atretric segment is grossly dilated and testinal auto-stapling device may be used to facilitate hypertrophied over an extended distance – typical the resection and anastomosis. The longitudinal in type III(b) atresia and high jejunal atresia anastomotic line is reinforced with an absorbable 5/0 í To equalize disparity in anastomotic lumen size or 6/0 Lambert suture. Tapering can safely be done í For the correction of a failed inversion plication over a length of 20–35 cm. The tapered bowel is then procedure primarily anastomosed to the distal bowel (with or í To improve function in a persistently dilated non- without equal bowel diameters) and left in a depen- functioning mega-duodenum following surgery dant position as for corrected malrotation with the for upper jejunal atresia caecum in the left hypochondrium.22
    • Chapter 22 Jejuno-ileal Atresia 223 Figure 22.11 Figure 22.12
    • Heinz Rode, Alastair J.W. Millar 224 Figure 22.13, 22.14 í Plication and Folding. The basic derotation and obstruction with care being taken not to narrow the back resection methods are used as previously de- lumen excessively. The “keel” must be trimmed and scribed for tapering procedures. The plication meth- closed with interrupted sutures. The main drawback od has the advantage of reducing the risk of leaking of this method is unravelling of the suture line with- from the antimesenteric suture line, conserves mu- in a few months, necessitating revision. The bowel is cosal surface area and may even facilitate return of left in a position of derotation with the duodenojej- bowel peristalsis. More than half of the antimesen- unum dependant, the mesentery broad based and the teric bowel circumference may be infolded into the caecum in the left hypochondrium. lumen over an extended length without causing an22
    • Chapter 22 Jejuno-ileal Atresia 225 Figure 22.13 Figure 22.14
    • Heinz Rode, Alastair J.W. Millar 226 Figure 22.15–22.17 í Antimesenteric Seromuscular Stripping and In- excised taking care not to damage the mucosa. The version Plication. This technique prevents unravell- two denuded mucosal strips are then approximated ing of the plication method and preserves maximal with a running monofilament suture. The keel of the mucosal surface for absorption. A single or two anti- inverted bowel should be trimmed and bowel edges mesenteric converging seromuscular strips ±2 mm approximated with interrupted sutures prior to in width are resected. This is easily performed by sta- anastomosis to the distal bowel. Proximal and distal bilizing the proposed line of resection with straight luminal size can be approximated to facilitate the non-toothed forceps. A seromuscular strip is then anastomosis.22
    • Chapter 22 Jejuno-ileal Atresia 227 Figure 22.15 Figure 22.16 Figure 22.17
    • Heinz Rode, Alastair J.W. Millar 228 CONCLUSION The overall survival amongst newborn babies with intestinal dysfunction, blind loop syndrome and the intestinal atresia has increased from a dismal 10% in short bowel syndrome can be minimized by careful 1952 to 90% at present. This came about primarily attention to the presenting anatomical detail, meticu- from a change in the surgical procedure from pri- lous surgical technique and maximal bowel length mary anastomosis without resection to liberal resec- conservation methods. Because of the high incidence tion of the blind proximal and distal ends followed by of unravelling, the plication technique is rarely used. end-to-end anastomosis. The short bowel syndrome is a major factor influ- Understanding the pathogenesis of atresia and encing outcome. It may be due to predisposing fac- adapting surgical procedures to minimize loss and tors such as extensive intra-uterine bowel loss, oper- conserve bowel length ensured that most infants will ative factors, i.e., over-zealous bowel resection and is- have sufficient bowel length for normal alimentary chaemic injury to the bowel or post-operative com- tract function and overall growth and development. plications. Under ideal circumstances a survival rate Despite improvement in surgery, anaesthesia and of 46–70% can be expected in most infants with less peri-intra- and post-operative care, type III(b) atre- than 25 cm jejuno-ielum. sia still carries a mortality of 19% predominantly due Several surgical procedures have been identified to gangrene of the proximal end of the distal segment to improve the outcome of the short bowel syndrome (7%), anastomotic leak (15%) and stricture formation including reversal of segments of bowel, interposi- (15%). The prognosis of intestinal atresia is further tion of colonic segments and methods to increase determined by genetic factors, prematurity (30%), mucosal surface area for absorption purposes. Most delay in presentation, associated diseases, i.e., cystic are of experimental value only except for bowel- fibrosis, malrotation (45%), exomphalos, gastroschi- lengthening procedures. The latter should not be sis and Hirschsprung’s disease, together with other performed initially until conservative methods to gastro-intestinal atresias, infarction of the proximal stimulate and allow maximum bowel adaptation to atresia with peritonitis, sepsis, pneumonia and the occur, have failed. Full bowel adaptation may require complications of prolonged parenteral nutrition. 6–18 months to become achieved. The incidence of post-surgical complications such as anastomotic leaks, stricture formation, transient SELECTED BIBLIOGRAPHY Kling K, Applebaum H, Dunn J, Buchmiller T, Atkinson J Rode H, Millar AJW (2003) Intestinal atresia and stenosis: In: (2000) A novel technique for correction of intestinal atresia Puri P (ed) Newborn surgery. Arnold, London, pp 445–456 at the ligament of Treitz. J Pediatr Surg 35 : 353–356 Weber TR, Wane DW, Grosfeld JL (1982) Tapering enteroplasty Louw JH, Barnard CM (1955) Congenital intestinal atresia: ob- in infants with bowel atresia and short bowel syndrome. servations on its origin. Lancet 2 : 1065–1067 Arch Surg 117 : 684–688 Malcynski J T, Shorter N A, Mooney D P (1994) The proximal mesenteric flap: a method for closing large mesenteric de- fects in jejunal atresia. J Pediatr Surg 29 : 1607–160822
    • CHAPTER 23 Meconium Ileus Massimo Rivosecchi INTRODUCTIONMeconium ileus is the earliest clinical manifestation thick, dark green and putty-like meconium, firmlyof cystic fibrosis (CF) and occurs in 8–10% of pa- adherent to the walls. The intestinal obstructiontients with CF at birth. The clinical features are main- causing a hyper peristalsis is responsible for the con-ly due to the presence of abnormal, inspissated and gestion and hypertrophy of the walls. The distal ile-viscid mucus of intestinal origin. In neonates affect- um is full of concretions called “rabbit pellets”, greyed by this condition, the impacted meconium pro- stained and with a typical beaded appearance. Thisduces an intraluminal obstruction occurring in the small bowel condition is responsible for a narrow,midileum, leading to a progressive distension. About empty and small colon, never used, which is called40% of patients with meconium ileus are complicat- microcolon.ed by intestinal volvolus, atresia, gangrene and ne- Main symptoms include abdominal distensioncrosis, perforation, peritonitis with abdominal cal- (96%), bilious vomiting (50%) and delayed passagecifications and, finally, meconial pseudocyst. of meconium (36%). From a clinical point of view, it More recently relief of the intestinal obstruction is possible to recognize two different conditions: ahas been obtained by irrigating the intestine with simple, uncomplicated and non-surgical type, and avarious solutions such as normal saline, 1% N-acetyl- complicated, severe type, with a mortality of at leastcysteine, hyperosmolar Gastrografin enema, surfac- 25% of all cases. In the first type (58%), signs andtant, or DNase. With respect to different types of sur- symptoms of a distal ileal obstruction are seen notgical and medical efforts, the survival rate at 1 year later than 48 h after birth: generalized abdominalincreased from 10 to 90%, and the operative mortal- distension with dilated and visible as well as palpableity is drastically decreased to 15–23% of the treated loops of bowel, bilious vomiting, no stools and nar-newborn. rowing of the anus and rectum, with only a dense and Meconium ileus accounts for 9–33% of all neonatal rubber-like grey meconium sticking to the anal wall.intestinal obstructions (300 new cases in Italy each In the second type (42%), the neonate represents ayear), with an incidence of 1:2500 newborns, repre- surgical emergency, which must be treated withinsenting the third most common cause of neonatal 24 h of birth, when the signs of a hypovolemic shocksmall bowel obstruction after ileal and duodeno-jej- or sepsis are not well established. A fetus with com-unal atresia and malrotation. Polyhydramnios is the plex meconium ileus is at increased risk of postnatalmost frequent feature observed in prenatal diagnosis bowel obstruction and perforation.A usual X-ray im-of complicated forms of meconium ileus. The pres- age of fine, granular soap-bubble (“Singleton’s sign”)ence of fetal hyperechogenic bowel on the ultra- or ground-glass appearance (“Neuhauser’s sign”) issound, associated with dilated bowel and/or ascites due to a dense meconium mixed with air, typical ofcould be indicative of an intestinal obstruction. A the distal ileum; this picture is usually located in thefamily history of CF is clearly evident in almost 25% mid-abdomen or in the right iliac fossa. When theof these patients. Meconium ileus is uncommon in meconium ileus is complicated, the abdominal radio-premature infants (5–12%), and associated congenital graph may show calcification as a result of meconi-anomalies are rare. um peritonitis due to a fetal perforation of the intes- In the meconium ileus, the intestine shows differ- tine. A double-bubble image or air-fluid levels can beent aspects if we consider the proximal, the middle seen when a secondary ileal atresia (single or double)and the distal ileum. In the first portion, nearly nor- is the final bowel remodelling after a complete volvu-mal evidence is present, with a progressive dilatation lus associated with a severe ischaemic damage. If theat the mid-portion borderline. In the proximal ileum, intestinal perforation occurs early in the antenatalthe content has a semi-liquid consistency and is not period, the X-ray appearance of a round rim of cal-yet viscous. A marked and severe dilatation of the cification underlines a meconium pseudocyst.middle ileum is always seen: the intestine contains
    • Massimo Rivosecchi 230 Figure 23.1, 23.223 The first step of the treatment includes a nasogastric cedure prevents leakage of the contrast medium by tube decompression, antibiotic prophylaxis, and cor- taping the buttocks as well as the catheter disloca- rection of dehydration, electrolytes and hypother- tion. If the contrast medium fails to progress into di- mia. lated small bowel loops, the presence of an acquired A contrast enema with water-soluble and hyper- atresia is definite and the radiologist must stop the or iso-osmolar contrast is the medical treatment of examination because of a high risk of perforation. Of choice and mucosal safe, for uncomplicated cases. A those submitted to this procedure, 50% of neonates recent study that used various enema solutions ad- benefit from enema alone over the next 48 h, without ministered in a mouse model showed that surfactant any additional treatment; in some cases, a second en- and Gastrografin were the most efficacious for the in ema may be used with a complete evacuation of the vivo relief of constipation in comparison with perflu- meconium filling the ileal loops. Acetylcysteine ad- bron, Tween-80, Golytely, DNase, N-acetylcysteine ministered by mouth is useful and helps to relieve the and Viokase. Intestinal mucosal damage was absent obstruction. Radiographs are taken at 3, 6, 12, 24 and and viscosity had been significantly reduced in vitro. 48 h with the aim of evaluating progression and pos- The enema evacuation should be obtained under sible complications. At this time feeding is begun. fluoroscopic control, with a gentle and progressive Hypovolemic shock and early perforation are around increasing of the intraluminal pressure, thus avoid- the corner, but an appropriate and meticulous proce- ing unexpected fractures of the colon. A correct pro- dure can avoid these complications.
    • Chapter 23 Meconium Ileus 231 Figure 23.1 Figure 23.2
    • Massimo Rivosecchi 232 Figure 23.323 Only 6% to 10% of uncomplicated forms fail the non- using an enterotomy placed in the dilated hyper- operative management using a water-soluble con- trophic ileum. The catheter is two-way directed with trast enema. If no significant difference in intestinal care, clearing the small as well as the large bowel. Af- diameters and no microcolon is present, a limited en- ter exclusion of an atretic intestinal segment, the en- terotomy and repeated warm saline irrigations terostomy is closed by single interrupted seromuscu- through a smooth catheter provide for the best result. lar stitches. Meconium discharge may be manually supported, Figure 23.4, 23.5 In contrast, approximately half of neonates with me- Once all intestinal contents are evacuated, a 10 Ch conium ileus cannot be treated adequately with irri- feeding tube with larger cut openings is inserted gations and/or present additionally an intestinal ob- through the enterotomy in the proximal non-dilated struction complicated by neonatal intestinal perfora- ileum, and a second 5 Ch tube is inserted into the dis- tion or ileal atresia secondary after intrauterine per- tal narrow ileum or microcolon in a T-tube fashion. foration. They always require a surgical procedure The enterotomy is closed around the tubes and is such as resection of the dilated meconium-filled ile- tightly fixed to the appropriate part of the ventral ab- um and ileal anastomosis (as shown in Chap. 22). Ad- dominal wall – in a similar fashion as in a gastrosto- ditionally, complicated cases of meconium ileus are my. Both tubes are carefully fixed with nonabsorb- seen in newborns with an extreme difference in di- able sutures to the skin. If a bowel segment had to be ameters of the proximal and distal ileum, and a sig- resected, the stoma for the tubes is best situated ap- nificant microcolon. In those cases, the double-tube proximately 5 cm in front of the anastomosis, and the ileostomy technique according to Rehbein has small tube is passing the anastomosis far into the proved to be an effective treatment and avoids the narrow intestine. Post-operatively the large tube secondary laparotomy for stoma closure. serves for suctioning and evacuation of the intestinal A horizontal predominantly right-sided laparoto- contents; the small distal tube serves for constant ir- my, approximately 2 cm below the umbilicus, is per- rigation with increasing amounts of fluid (first sa- formed. A small transverse incision into the enlarged line, later pre-digested milk), thereby promoting a ileum is performed, approximately 5–7 cm in front of rapid enlargement of the ileum and the microcolon. the narrow part filled with stool pellets. Four stay su- As soon as the evacuations through the large tube be- tures are inserted into the margins of the incision. If come less and normal bowel movements occur, it is an atresia exists, the atretic part is resected and the indicative that most of the intestinal contents are thickened meconium from the proximal part and the passing by distally. The double tubes can then be grey stool pellets from the distal part are evacuated simply removed. We have treated our patients since by numerous irrigations with warm saline through a the 1980s by this double-tube method successfully, 5–8 Ch feeding tube supporting the manoeuvre by and cutaneous enterostomy is no longer performed. gentle manual forward and backward manipulation.
    • Chapter 23 Meconium Ileus 233 Figure 23.3 Enterotomy irrigation Enema Figure 23.4 Figure 23.5
    • Massimo Rivosecchi 234 Figure 23.6–23.923 Different surgical techniques have been developed in as an ileostomy. A variation of this technique has the past consisting of a resection of the enlarged been described, using an angulating proximal seg- bowel segment and temporary decompression by ment, which is obliquely anastomosed with the distal means of a distal or proximal enterostomy. The most stump. Proximal chimney enterostomy, the so-called simple form is a double-barrelled ileostomy accord- Santulli procedure, consists of a proximal ileostomy ing to Mikulicz, with the two loops brought out side- with end-to-side ileal anastomosis. The end of the to-side. This solution is quick and avoids an intra-ab- distal limb is anastomosed to the side of the proximal dominal anastomosis. More technical alternatives limb, the end of which is used as the enterostomy. have been described thereafter: a distal ileostomy This technique should facilitate irrigation as well as with end-to-side ileal anastomosis (Bishop-Koop) decompression of the proximal small bowel. has been called “distal chimney enterostomy”. This The enterostomies can be closed by an end-to-end procedure consists of a Roux-en-Y anastomosis anastomosis when uninhibited passage of intestinal between the end of the proximal segment and the contents is established, mostly between 7 and 12 days side of the distal segment, at least 3 to 5 cm from the after. open end. The open limb of the distal segment is used
    • Chapter 23 Meconium Ileus 235 Figure 23.6 Figure 23.7 Mikulicz Bishop-Koop Figure 23.8 Figure 23.9 modified Bishop-Koop Santulli
    • Massimo Rivosecchi 236 Figure 23.1023 When a terminal ileostomy or colostomy is desired in supply. Seromuscular wall is sutured to the fascia in neonates and/or children, the preferred method used four quadrants. Next the stitch is taken through the is the nipple-valve system formation. This is an easy skin seromuscular wall and edge of the stoma, there- technical procedure and avoids any kind of infiltra- by creating the nipple. Four to six sutures are nor- tion or stricture or retraction of the neostoma. mally needed depending upon the size of the stoma. A 2- to 3-cm-long intestinal segment is used with a serosal surface free of fat and with a good vascular
    • Chapter 23 Meconium Ileus 237 Figure 23.10
    • Massimo Rivosecchi 238 CONCLUSION23 Generally, the lesser the extend of to bowel resection origin) and blind loop syndromes (30% of the cases). the earlier is the recovery of peristalsis. These long-term problems are more commonly as- Actually, bowel resection with primary anastomo- cribed to a higher incidence of peritonitis and small sis has been proved to be as well effective and safe as bowel ischaemia. Nearly all patients undergoing en- stoma formation, but is associated with a reduced terotomy and irrigation do not manifest long-term length of initial hospital stay. Complications include surgical morbidity. There is general agreement that pulmonary infections, which is the most important resection and stomas could be avoided with a more one with an incidence of at least 8 to 10%. Anasto- meticulous attention to care and clearing the intesti- motic leakage occurs for different reasons: a techni- nal walls from the more dense concretions. cal mistake, an insufficient blood supply, or an unrec- Long-term complications in neonates affected by ognized distal obstruction. Delayed recovery of per- uncomplicated meconium ileus who were not opera- istalsis is another frequently observed complication tively treated are rarely seen, and only mild and tran- and is due to an abnormal stretching of the intestinal sient complications have been observed in newborns walls during the fetal life. Total parenteral nutrition is treated with minor surgical procedures, such as ente- the support of choice and a central venous catheter is rotomies and irrigations. However, meconium ileus mandatory in these situations. did not directly affect long-term functional or social Meconium ileus may be an early indication of a status in children with CF, and the quality of life is more severe phenotype of CF. This was suggested by mainly correlated with the extension of lung lesions, the significantly lower pulmonary function found in which is the most limiting factor with respect to the children with a history of meconium ileus compared school, social life and physical activity. to age- and sex-matched children who did not have Survival of neonates with meconium ileus has im- meconium ileus. In this view, patients without CF proved over the last two decades because of neonatal demonstrated better growth and functional status, intensive care, improved surgical technique and and had a lower incidence of lung diseases as well as medical treatment. In general, an overall immediate gastrointestinal problems. It is also possible that pa- survival of 90% is achieved using the modern proto- tients affected by meconium ileus have a more severe cols and nearly all deaths are pertinent to the adoles- form of CF that, from a pathological point of view, cents. Only few children die because liver and or sep- consists of a more severe compromise of mucous tic complications. Deaths are mainly due to sepsis, glands primary or secondary to pulmonary interstitial em- The complicated forms are susceptible to a higher physema, or due to aspiration pneumonia. In a large number of long-term surgical complications, includ- series reported and analysed by Fuchs, only one child ing especially small bowel obstructions (of adhesive died because of the meconium ileus itself. SELECTED BIBLIOGRAPHY Burke MS, Ragi JM, Karamanoukian HL, Kotter M, Brisseau Irish MS, Ragi JM, Karamanoukian HL, Borowitz DS, Schmidt GF, Borowitz DS, Ryan ME, Irish MS, Glick PL (2002) New D, Glick PL (1997) Prenatal diagnosis of the fetus with cys- strategies in nonoperative management of meconium ileus. tic fibrosis and meconium ileus. Pediatr Surg Int 12 : J Pediatr Surg 37 : 760–764 434–436 Fanconi G, Uehlinger E, Knauer C (1936) Das Coeliakiesyn- Rehbein F (1976) Dünndarmatresie. In: Rehbein F (ed) Kinder- drom bei angeborener zystischer Pancreasfibromatose und chirurgische Operationen. Hippokrates Verlag, Stuttgart, Bronchiektasien. Wien Med Wochenschr 28 : 753–766 pp 273–293 Fuchs JR, Langer JC (1998) Long-term outcome after neonatal meconium obstruction. Pediatrics 101:4–7
    • CHAPTER 24 Gastrointestinal Duplications Mark D. Stringer INTRODUCTIONAlimentary tract duplications are rare congenital sion of caudal twinning and some intestinal lesionsmalformations that may occur anywhere from may result from an intrauterine mesenteric vascularmouth to anus. They are usually single, vary widely in accident.Alimentary tract duplications are best treat-size, are more often cystic than tubular, and are lined ed by early complete excision to avoid future compli-by alimentary tract mucosa. Intestinal duplications cations. In addition to the problems described above,are typically located on the mesenteric aspect of the there is a risk of late malignant degeneration, partic-intestine and usually share a smooth muscle wall and ularly with rectal and gastric duplications. Early re-common blood supply with the adjacent bowel. Oc- section avoids the additional operative difficultiescasionally, they are found separate from the alimen- arising from inflammation or perforation of the du-tary tract. They may communicate with the lumen of plication cyst. The surgeon must be familiar with thethe gut and can contain heterotopic gastric mucosa; local anatomy and the range of available operativeboth features are more likely with tubular lesions. techniques. Rarely, complete resection is excessivelySome duplications are associated with vertebral hazardous and alternative techniques such as muco-anomalies. Foregut duplications may be associated sal stripping or fenestration are necessary. Incom-with intraspinal pathology when they are sometimes plete excision may lead to late, potentially fatal, com-termed neurenteric cysts. plications such as cyst recurrence, infection, meningi- There is no satisfactory single explanation to ac- tis (neurenteric cysts), gastrointestinal bleeding andcount for the development of gastrointestinal dupli- perforation. At operation, a careful search is made forcations. The pathogenesis of those with associated additional duplications and other congenital gas-vertebral anomalies can be explained by the abnor- trointestinal malformations.Asymptomatic appendi-mal adherence of the endoderm of the roof of the ceal duplications found in association with cloacal orprimitive gut to the notochord (the split notochord bladder exstrophy may be preserved for use in subse-theory). Some hindgut duplications are an expres- quent reconstructive surgery.
    • Mark D. Stringer 240 Figure 24.1 Nearly half of all duplications occur in the midgut pre-operative investigations – plain and contrast ra-24 while a third is localized in the foregut. Most duplica- diography, abdominal sonography and occasionally tions cause symptoms in infancy or childhood. Some a radioisotope technetium scan to detect heterotopic are found incidentally in infants undergoing evalua- gastric mucosa. tion of another congenital malformation or in chil- In contrast, thoracoabdominal lesions demand dren with unrelated symptoms investigated by ultra- detailed radiologic imaging of mediastinal, abdomi- sound or other imaging modalities. In recent years, nal and spinal components. Magnetic resonance im- an increasing number of gastrointestinal duplica- aging (MRI) and computerised tomography (CT) en- tions have been detected during prenatal ultrasound able evaluation of the cranial and caudal extent of the scanning. The diverse presenting features of duplica- cyst. Upper gastrointestinal contrast studies and en- tions reflects their wide distribution and variety. doscopy may be necessary in some cases. The pos- Symptoms and signs arise from obstruction (e.g., sibility of a separate intestinal duplication must be respiratory, oesophageal, intestinal), haemorrhage, considered with all foregut duplications. infection/inflammation, perforation, or intussuscep- In older children, endoscopic retrograde cholan- tion. Some are discovered as an asymptomatic mass. giopancreatography or magnetic resonance cholan- Vertebral anomalies such as bifid or hemi-verte- giography are helpful in the assessment of pancreat- brae are important diagnostic pointers, particularly ic and selected duodenal duplications and magnetic with foregut duplications. Other congenital malfor- resonance angiography may help to plan surgery for mations are found in about half of all patients. For large retroperitoneal lesions. Pelvic duplications are example, intestinal malrotation or atresia may be as- best imaged by CT or MRI in conjunction with a con- sociated with midgut duplications and genitourinary trast enema, fistulogram, endoscopy, and urinary anomalies or bladder exstrophy with hindgut lesions. tract sonography. Imaging is a critical aspect of preoperative prepar- ation. Isolated small bowel duplications require few
    • Chapter 24 Gastrointestinal Duplications 241 Figure 24.1 Foregut 36% Oesophageal 19% Thoracoabdominal 4% Gastric 9% Duodenal 4% Midgut 50% Jejunal 10% Ileal 35% Hindgut 14% Colonic 7% Appendiceal 2% Rectal 5% Cecal 3%
    • Mark D. Stringer 242 Figure 24.2–24.4 í Oesophageal Duplications. These are usually supialization alone is inadequate. Mucosal integrity24 intramural non-communicating cystic lesions, more can be checked before closing the muscle layer by in- often related to the right side of the oesophagus. A sufflating air through a nasogastric tube. Occasional- few are separate from the oesophagus and do not ly, an adjacent oesophageal stricture or ulcer will re- share a common muscular wall. Intrathoracic oe- quire segmental oesophageal resection. The neck (or sophageal duplication cysts can be approached chest in the case of intrathoracic oesophageal dupli- transpleurally through a posterolateral thoracotomy cations) is closed and pleural drainage is not usually (typically right sided). necessary. Cervical oesophageal duplications can be re- Video-assisted thoracoscopic resection of a tho- moved via a supraclavicular approach. The neck racic oesophageal duplication is feasible in some cas- must be extended with a sandbag between the shoul- es. Single lung ventilation with collapse and retrac- ders. The cyst is fully mobilized by dissecting close to tion of the ipsilateral lung facilitates surgical expo- its wall. Excision of the cyst is most easily achieved by sure. A 10-mm 0° end-viewing telescope is intro- transecting the lesion close to the oesophagus and duced under direct vision after gentle finger dissec- removing the residual mucosa. The proximity of the tion of the port site. Two or more 5-mm instrument vagus and phrenic nerves and the right lymphatic ports are subsequently sited. After dissection, if the duct/thoracic duct should be noted. Any communi- cyst remains intact, it is aspirated to enable removal cation with the oesophagus should be closed and the through a port site. Prior to removing the viewing residual muscular defect can be repaired using the telescope, the lung is re-expanded and checked for air muscular fringe from the duplication. Complete mu- leaks. A thoracostomy drain is left in situ. cosal excision is essential to avoid recurrence – mar-
    • Chapter 24 Gastrointestinal Duplications 243 Figure 24.2 Figure 24.3 Figure 24.4
    • Mark D. Stringer 244 Figure 24.5 í Thoracoabdominal Duplications. Thoracoab- Complete excision of the duplication may involve24 dominal duplication usually descends to the right of dissecting its upper extremity free from any bony the oesophagus, but often separate from it, in the vertebral attachment. This sometimes requires a posterior mediastinum. It communicates through gouge or chisel. Some thoracoabdominal duplica- the diaphragm with the stomach, duodenum, jeju- tions are complicated by peptic ulceration which may num or ileum. These lesions are best approached by erode into the lung parenchyma causing haemopty- separate thoracic (posterolateral thoracotomy) and sis; in these cases, excision may require oversewing abdominal incisions. Occasionally, a laminectomy is the fistula or rarely a lobectomy. The duplication is also necessary to deal with an intraspinal component traced distally where it usually passes behind the di- of the cyst but this should be anticipated by prior im- aphragm and may become tenuous at this point. The aging. The connection with the spinal cord may be lesion is pulled up into the chest and divided between tenuous and easily overlooked at operation, under- ligatures and the chest is closed with pleural drain- lining the importance of adequate detailed preoper- age before proceeding to the laparotomy.Alternative- ative imaging. Multidisciplinary planning involving ly, the thoracic component of the cyst is passed neurosurgical expertise is necessary in such cases. A through the diaphragmatic defect into the abdomen staged approach, excising each component of the where it is subsequently retrieved. The abdominal thoracoabdominal duplication cyst sequentially, portion most often appears as a tubular lesion com- should be avoided if possible. Although an asympto- municating with the jejunum but may occasionally matic abdominal component can be temporarily left end blindly along the greater curve of the stomach. It in situ, an undrained thoracic segment is hazardous. can usually be excised without difficulty.
    • Chapter 24 Gastrointestinal Duplications 245 Figure 24.5
    • Mark D. Stringer 246 Figure 24.6, 24.7 í Gastric Duplications. Cystic gastric duplications closure. Extensive duplications of the greater curve of24 rarely communicate with the stomach lumen. Very the stomach are best treated by partial resection, rarely, they may communicate with the pancreas. stripping of the residual mucosal lining, and repair. A Most greater curve or pyloric duplication cysts can less optimum approach is to divide the septum sep- be completely excised by dissecting the cyst off the arating the tubular duplication from the gastric lu- gastric submucosa and repairing the residual sero- men with linear staplers introduced via proximal and muscular defect with inverting absorbable sutures. distal gastrotomies. This division should be as com- Gastric mucosal integrity can be checked prior to re- plete as possible but since the mucosal lining of the pair of the stomach wall by insufflating air via a nas- duplication is not removed, there remains some risk ogastric tube. of long-term complications. Small gastric duplications are sometimes more Laparoscopic staple excision of smaller gastric simply excised by a wedge resection of the cyst and a duplication cysts is an alternative technique. margin of stomach followed by a two layer gastric Figure 24.8 í Duodenal Duplications. Some of these are simple dine and an oblique incision is made in the lateral du- cystic lesions which are easily excised. Others related odenum to expose the medially situated duplication to the medial aspect of the second part of the duod- cyst. If needle aspiration of the cyst yields bile it enum may be complex and communicate with the should be cautiously deroofed, the ampulla identified pancreatic and/or common bile duct. Previous pan- and protected. Most of the cyst can be excised and the creatitis or peptic ulceration secondary to heterotop- edges of a residual medial disc of mucosa (centred on ic gastric mucosa may compound the surgical diffi- the ampulla) should cautiously be oversewn with fine culties. Facilities for intra-operative cholangiopan- absorbable sutures to achieve haemostasis. creatography should be available with prior posi- If the opening of the common bile duct or pan- tioning of the child on a radiolucent operating table. creatic duct is uncertain, intra-operative cholangio- Surgical alternatives are dictated by the anatomy graphy may be helpful. Alternatively, a cholecystecto- and include complete excision of the cyst with division my can be performed and a fine probe passed distal- of any ductal communication, partial excision and mu- ly through the cystic duct into the duodenum. cosectomy of the remaining part of the cyst or, when Rarely, a duodenal duplication cyst is located ex- the duplication is adjacent to the ampulla of Vater, it clusively within the head of the pancreas and causes can be fenestrated into the duodenal lumen. If the latter recurrent pancreatitis. Therapeutic alternatives are is performed, the presence of gastric mucosa should be complete local excision with Roux loop drainage of excluded by intraoperative biopsy and the window the residual cavity or a Whipple-type pancreatodu- must be of sufficient size to allow free dependent drain- odenectomy. Roux loop drainage of the cyst alone is age without the tendency to form a cul de sac. inadequate – the mucosal lining of the cyst must be The duodenum is approached via a right upper completely excised if pancreatitis is to be prevented. quadrant incision and “Kocherised” to elevate it into Where the duplication lies in the tail of the pancreas, the wound. The adjacent peritoneal cavity is packed distal pancreatectomy with splenic preservation (la- off with swabs soaked in dilute aqueous povidone io- paroscopic or open) can be performed.
    • Chapter 24 Gastrointestinal Duplications 247 Figure 24.6 Figure 24.7 Figure 24.8
    • Mark D. Stringer 248 Figure 24.9–24.11 í Small Bowel Duplications. Cystic lesions of the il- placed across the intestine (but not the mesentery)24 eum or jejunum are the commonest duplications and will help prevent contamination. Before dividing the complete excision is relatively straightforward. The bowel with needle cautery or scissors, the surgical continuous muscle coat of the normal bowel and du- field should be protected by surrounding gauze plication cyst usually make excision of the cyst alone swabs soaked in dilute aqueous povidone iodine. impractical. For localised lesions, the duplication End-to-end anastomosis is performed using a single should be resected with the adjacent intestine after layer of interrupted extramucosal absorbable sutures ligation and division of associated mesenteric ves- such as 5/0. Laparoscopic techniques are applicable sels. If the bowel is obstructed, atraumatic clamps in selected cases.
    • Chapter 24 Gastrointestinal Duplications 249 Figure 24.9 Figure 24.10 Figure 24.11
    • Mark D. Stringer 250 Figure 24.12, 24.13 Short tubular duplications may also be excised in tion may be safely left in situ provided haemostasis24 continuity with adjacent bowel. Care is required to has been achieved. For tubular duplications within obtain a complete excision at the proximal and distal the mesentery but separate from the intestine, careful margins of tubular lesions since the exact point of separation of the two leaves of the mesentery and di- termination of the duplicated bowel can sometimes vision of vessels on one side only may enable enu- be difficult to determine. cleation of the duplication without jeopardizing the Very extensive tubular duplications, which if re- blood supply of the adjacent bowel. Whichever tech- sected would risk a short bowel syndrome, pose a nique is used, it is essential to check the viability of much more difficult problem. In such cases, submu- the remaining intestine and to resect the junction of cosal resection is an alternative option. The mucosal duplicated and normal bowel since heterotopic gas- lining is stripped out using a series of longitudinal tric mucosa is frequently present in tubular intestinal seromuscular incisions in the duplication. Bipolar di- duplications. Associated intestinal malrotation will athermy and gentle blunt pledget dissection are use- require a Ladd’s procedure. ful. The residual seromuscular sleeve of the duplica-
    • Chapter 24 Gastrointestinal Duplications 251 Figure 24.12 Figure 24.13a Figure 24.13b
    • Mark D. Stringer 252 Figure 24.14, 24.15 í Colonic (and Appendiceal) Duplications. All cys- through an enterotomy near the distal margin of the24 tic and most tubular duplications can be excised by duplication. To avoid leaving a problematic spur, the segmental colonic resection. The bowel is reanasto- distal end of the septum must be divided completely. mosed with an end-to-end, single layer, extramuco- If there is complete hindgut duplication with two sal suture technique. With rare total colonic duplica- perineal openings, then a preliminary double de- tions, the duplicated bowel may lie lateral or medial functioning colostomy is recommended. The dupli- to the normal colon and often has a proximal con- cated bowel can later be transected at the level of the nection. Heterotopic gastric mucosa is rarely found rectum and anastomosed to the normal rectum. The and thus distal fenestration is possible. This can be mucosa of the redundant distal segment is excised. done with a linear stapling device introduced
    • Chapter 24 Gastrointestinal Duplications 253 Figure 24.14 Figure 24.15a Figure 24.15b
    • Mark D. Stringer 254 Figure 24.16, 24.17 í Rectal Duplications. Rectal duplications often into the retro rectal space and do not communicate24 present in the neonatal period as a perineal mucosal with the urinary tract. Rarely, they first manifest as swelling and/or a fistula extending to the perianal ar- rectal prolapse in an infant. They may be excised by ea or anorectum. Some are cystic lesions with no one of several approaches. external or internal communication. They extend Figure 24.18, 24.19 Small submucosal rectal lesions can be excised endo- of larger or more complex duplications, some of rectally. As the anus is dilated with an anal retractor, which have a lateral or cranial diverticulum, the pos- the cyst bulges forward. The rectal mucosa over the terior sagittal approach provides excellent exposure cyst is incised and, working within the submucosal of the retrorectal space. With attention to bowel plane of the rectum, the cyst is gradually dissected preparation and antibiotic prophylaxis, a covering free. Local injection of 1:200,000 adrenaline and bi- colostomy is rarely necessary. Infected rectal duplica- polar diathermy are helpful in the dissection. The tion cysts are best treated by preliminary drainage cyst should not be decompressed until near the end and then resected once the acute inflammation has of the dissection as the wall tension can facilitate the settled. Rectal duplications should be completely ex- dissection. The incision in the rectal mucosa is re- cised if possible because numerous examples of late paired with interrupted absorbable sutures. malignant degeneration of these cysts have been de- A limited perineal excision is all that is required scribed. for a localised small mucosal duplication. In the case
    • Chapter 24 Gastrointestinal Duplications 255 Figure 24.16 Figure 24.17 Figure 24.18 Figure 24.19
    • Mark D. Stringer 256 CONCLUSION The histology of all excised duplications should be 30% of duplications (more common in foregut and24 examined to assess the completeness of the excision, tubular duplications). Twelve patients had incidental, to document the presence of heterotopic mucosa and asymptomatic duplications associated with other to exclude neoplasia. The specific features of postop- major congenital anomalies. In 60 patients, with a erative clinical management are determined by the median age of 3 months, the duplication was the anatomy of the duplication cyst and the complexity principal pathology. In this group, thoracoabdominal of the surgical procedure. Surgical complications are lesions were responsible for much of the overall mor- related to the size and location of the cyst, the pres- bidity and mortality; five patients (9%) died from ence of a communication with the gastrointestinal post-operative complications. However, adherence to tract or spinal canal, the existence of heterotopic gas- the principles outlined can minimize the hazards as- tric mucosa and potential involvement of mesenteric sociated with large or complex gastrointestinal du- vessels. Incomplete excision is a particular risk with plication cysts. Mortality should now be exceptional- thoracoabdominal duplications because of their size ly rare. and complexity; the consequences of incomplete ex- The key steps in the successful surgical manage- cision include meningitis, gastrointestinal bleeding ment of gastrointestinal duplications include: a and perforation and respiratory complications. thorough understanding of the spectrum of these le- In an analysis of 72 children with alimentary tract sions, careful preoperative assessment of the cyst and duplications referred to the Hospital for Sick Chil- its potential associated pathology, appropriate opera- dren, London, between 1973 and 1992, vertebral tive planning, complete excision where possible, and anomalies were found in 20% of patients (especially a full understanding of the alternative techniques if foregut lesions) and heterotopic gastric mucosa in excision is deemed too hazardous. SELECTED BIBLIOGRAPHY LaQuaglia MP, Feins N, Eraklis A, Hendren WH (1990) Rectal Puri P (2003) Duplications of the alimentary tract. In: Puri P duplications. J Pediatr Surg 25 : 980–984 (ed) Newborn surgery. Arnold, London, pp 479–488 Norris RW, Brereton RJ, Wright VM, Cudmore RE (1986) A new Stringer MD, Spitz L, Abel R, Kiely E, Drake DP, Agrawal M, surgical approach to duplications of the intestine. J Pediatr Stark Y, Brereton RJ (1995) Management of alimentary tract Surg 21 : 167–170 duplication in children. Br J Surg 82 : 74–78 Pulligandla PS, Nguyen LT, St-Vil D et al (2003) Gastrointesti- nal duplications. J Pediatr Surg 38 : 740–744
    • CHAPTER 25 Short Bowel Syndrome Michael E. Höllwarth INTRODUCTIONThe term “short bowel” has been defined by Rickham The pathophysiological process that follows an ex-in 1967 as a small intestinal remnant of 75 cm or less tensive loss of small bowel is called intestinal adapta-in the newborn, which equals 30% of normal small tion. It includes, firstly, morphological changes lead-bowel length in that age group.A more functional de- ing to an increase of absorptive surface area, second-scription is preferred by most authors defining a ly, functional changes resulting in an augmentation“short bowel syndrome” (SBS) as a state of signifi- of the absorptive capacity of the remaining enterocy-cant maldigestion and malabsorption due to an ex- tes, and thirdly, an increased intestinal diameter withtensive loss of functional absorptive intestinal sur- a concomitant reduction of the motility therebyface area. slowing down the intestinal transit time of chyme. The prevalence of SBS has been increasing over The presence of intraluminal food is the most im-the last two decades due to the enormous progress in portant driving force for intestinal adaptation. Enter-intensive care of babies with either severe acquired al nutrients stimulate gastrointestinal secretions andintestinal diseases such as necrotizing enterocolitis hormones that are known to exert trophic effects onand volvulus, or congenital malformations leading to the mucosa. Recent evidence suggests that glucagon-the SBS, such as multiple intestinal atresia. Rarely like peptide 2, human growth hormone, epidermalSBS is caused by a genetically determined disease growth factor, and insulin-like growth factor-I maysuch as a congenital short bowel or a total intestinal play an important role in the process of intestinal ad-aganglionosis. aptation. Following extensive loss of small bowel, the symp- Surgery is indicated in selected patients only, first-toms of an individual baby depend on the absorptive ly when the absorptive surface area is definitely toocapacities of the intestinal remnants. Resection of the small to allow enteral feeding, secondly, when severejejunum is well tolerated due to an enormous adap- dysmotility in grossly dilated loops entails stagnationtive capacity of the ileum, the intact enterohepatic of chyme, and thirdly, when intestinal transit time iscirculation of bile salts and the preserved absorption too fast to allow sufficient absorption of nutrients.of vitamin B12. In contrast, if the entire ileum is lack- Whereas in the first group of patients intestinal trans-ing, absorption of nutrients is significantly more dif- plantation is the mainstay of surgical therapy, peris-ficult due to the limited intestinal adaptation capac- talsis can be improved in the second group by intesti-ities of the jejunum. Non-absorbed intestinal con- nal tapering or tapering and lengthening. In the thirdtents, including bile acids, spill over into the colon group, antiperistaltic segments, colonic interposition,and may cause significant diarrhoea. Furthermore, intestinal valves, and/or artificial invagination haveloss of the ileum leads to a reduction of the bile salt been used in selected patients. These surgical tech-pool, malabsorption of fat and fat soluble vitamins, niques are described in detail on the following pages.as well as to a vitamin B12 deficiency.
    • Michael E. Höllwarth 258 Figure 25.1 í Tapering. In patients with enough absorptive sur- al margins of the planned triangular resection. One face area – at least 50 cm with ileocecal valve – the ta- stay suture is located exactly at the antimesenteric pering can be performed by resection of a long trian- line and indicates the end of resection which is at the gular (or elliptical) antimesenteric segment. The tip of the triangle. The resection can be performed25 bowel segment is isolated from surrounding adhe- by means of a GIA stapler in very large dilated loops sions and the chosen length for the tapering is (see Chap. 22) marked by 5/0 stay sutures, which indicate the later- Figure 25.2, 25.3 We prefer to resect in small babies the antimesenter- lation, which inevitably would result if we use caut- ic redundant part with sharp scissors, thus allowing ery. The antimesenteric anastomosis can be accom- some bleeding from the resected margins, and we try plished either by a continuous running 6/0 absorb- to avoid carefully any disturbance of the local circu- able suture or by 6/0 interrupted sutures.
    • Chapter 25 Short Bowel Syndrome 259 Figure 25.1 Figure 25.2 Figure 25.3
    • Michael E. Höllwarth 260 Figure 25.4–25.7 The important part in performing any kind of intes- If, additionally, an anastomosis to the distal or tinal anastomosis lies in the technique that the stitch- proximal part of the intestinal tract has to be per- es should include only the seromuscular layer, there- formed, the same surgical technique is useful. First, by leaving out the mucosal layer. If the stitches take the two intestinal ends are brought together with two25 equal amounts of tissue on either side, both segments to four 5/0 stay sutures. Thereafter, the anastomosis of the bowel are perfectly adapted and the mucosal with interrupted 6/0 stitches begins at anterior wall. edges are lying side by side. Another important part The needle takes a good part of the seromuscular of this technique is to avoid too much tension when layer on either side. The suture is tied carefully, tying knots, thus not compromising circulation. This avoiding any strangulation. The single interrupted technique was originally described by Halsted in 1912 sutures are continued in the same way until the and has the advantage of preventing mucosal necro- whole anterior wall is anastomosed. sis just under the stitch, thereby supporting a rapid The posterior wall is sutured in a similar way, after and perfect healing of the anastomosis. turning around.
    • Chapter 25 Short Bowel Syndrome 261 Figure 25.4 Figure 25.5 Figure 25.6 Figure 25.7
    • Michael E. Höllwarth 262 Figure 25.8–25.11 The abdominal wall is closed using 3/0 or 4/0 inter- rupted single-layer figure-of-eight absorbable su- tures. The knot must be tied rather loosely without strangulating the tissue.25
    • Chapter 25 Short Bowel Syndrome 263 Figure 25.8 Figure 25.9 Figure 25.10 Figure 25.11
    • Michael E. Höllwarth 264 Figure 25.12 í Infolding. This method has the same effect as ta- on both ends. The lateral margins are approximated pering but saves all existing absorptive surface area. with 5/0 or 4/0 nonabsorbable seromuscular stitches, Therefore, it is indicated in cases with a rather short thereby enfolding the tissue in-between. It is report- intestinal tract when it seems advisable not to sacri- ed that the plicated segments are prone to break-25 fice any mucosal surface. The intestinal tract can re- down with time and some authors suggest resecting main closed in most of the patients. The intestinal on each side a serosal strip or a triangular segment loops that are selected for the infolding method are in order to support the development of dense adhe- marked with 5/0 stay sutures on the lateral side and sions (see Chap. 22) Figure 25.13, 25.14 í Tapering and Lengthening. Bianchi first reported with an intact blood supply for each side. The first an experimental procedure combining the method of step of the procedure consists of careful separation tapering with the use of the redundant tissue for of the vessel branching in the right and left group lengthening the bowel. Indications for this method and supplying either part of the selected intestinal are patients with very short bowel segments but tract. The space between the vessel layers can be largely dilated loops characterised by inefficient to- opened by introducing a haemostat and spreading and-fro peristalsis, stasis of chyme and bacterial the two layers. The small vessel groups on each side overgrowth. The technique is based on the fact that are secured by fine vessel loops of different colours the vessels coming from the mesentery are divided for the right and left parts. extramurally in branches supplying either side of the If the space between the branches is large enough bowel separately. Therefore, careful longitudinal di- that a GIA stapler can be introduced, the intestinal vision of a dilated intestinal segment between the loop can be divided in the midline and anastomosed branching vessels results in two intestinal halves longitudinally, step by step.
    • Chapter 25 Short Bowel Syndrome 265 Figure 25.12 Figure 25.13 Figure 25.14
    • Michael E. Höllwarth 266 Figure 25.15, 25.16 However, if the space is too small to insert a stapler – avoid any cautery or to use it very cautiously. Sero- which is the case when the bowel is not extremely di- muscular 6/0 absorbable sutures are used for the lon- lated – then a sharp longitudinal division is our pre- gitudinal anastomosis of the divided segments of the ferred method.As already mentioned above, we try to bowel.25 Figure 25.17, 25.18 Since both sections of the bowel hang on the same helix technique avoids traction on the nutrient ves- mesenteric segment, a helix-like isoperistaltic anas- sels, which is critical because necrosis of the divided tomosis is easier to perform than an anastomosis segments has been reported. with the two segments sliding one on the other. The
    • Chapter 25 Short Bowel Syndrome 267 Figure 25.15 Figure 25.16 Figure 25.17 Figure 25.18 A A B B A’ A’ B’ B’
    • Michael E. Höllwarth 268 Figure 25.19–25.21 Antiperistaltic segments are only indicated in cases In an infant, a 3-cm segment of small bowel is first with good propulsion of the luminal chyme, while it identified in regard to its blood supply, which should would be contraindicated in cases with a disturbed come from one major branch of the mesenteric ves- motility. A number of different intestinal interposi- sels. Care must be taken that neither the blood supply25 tions have been used in the past, both experimental- to the remainder small intestine nor to the colon, if ly and in some selected cases clinically. In the follow- present, is disturbed. The segment is isolated and the ing paragraph the method of antiperistaltic small or appropriate mesenteric base is isolated in a way that large bowel segment, as well as the interposition of a the segment can be reversed by 180º without impair- isoperistaltic colonic segment will be briefly demon- ing the blood flow. Finally the proximal and distal in- strated. testinal anastomoses are performed as described Reversal of distal small bowel loops has been stud- above. ied experimentally for years. The ideal length of the In the same way as described above a 3 cm to 5 cm reversed segment appears to be 10 cm in adults and antiperistaltic segment of the colon can be used. If no 3 cm in infants. The antiperistaltic segment acts as a ileocecal valve is present, the best way is to reverse physiological valve by causing either a retrograde the first part of the colon immediately after the small peristalsis or by functioning as an effective brake for intestine. However, if the ileocecal valve has been pre- the passage of chyme. Since the ideal length is diffi- served the antiperistaltic colonic segment can be cult to estimate for a given patient, this method has interposed between the distal small bowel and the not consistently resulted in clinical improvement. valve. The method has rarely been used in humans The distal ileum – if available – is best used as anti- and long term results are not available. peristaltic segment shortly before the ileocecal valve.
    • Chapter 25 Short Bowel Syndrome 269 Figure 25.19 Figure 25.20 A B Figure 25.21 A B
    • Michael E. Höllwarth 270 Figure 25.22, 25.23 Isoperistaltic interposition of colon has the advan- some absorptive qualities. Favourable but highly var- tage of using none of the small bowel remnants. Iso- iable results have been reported from the use of this peristaltic colonic interposition is best done with a method in children, and some groups have reported 10- to 15-cm segment into the proximal part of the improved nutrient absorption and weaning from pa-25 small intestine. Experimental evidence exists that the renteral nutrition. isoperistaltic colon prolongs transit time and gains
    • Chapter 25 Short Bowel Syndrome 271 Figure 25.22 Figure 25.23
    • Michael E. Höllwarth 272 Figure 25.24–25.26 The benefits of the ileocecal junction on long-term interrupted sutures onto the underlying seromuscu- outcome of babies with SBS has been questioned, lar bowel wall. The distal intestinal segment is then there exists a large body of evidence concerning its pulled over the everted bowel and finally anasto- powerful impact on intestinal transit time by slowing mosed to the everted segment and to the proximal25 the passage of intraluminal nutrients into the colon. intestine by seromuscular interrupted stitches. The Therefore, a variety of experimental surgical proce- result corresponds to a typical prograde intussucep- dures have been devised to slow down the intestinal tion and may act as a valve similar to the ileocecal transit time by creation of artificial valves. The valve valve. A valve less than 3 cm can also be constructed must be placed at the distal end of the small bowel. in a reversed manner by everting the distal end of The intestine is transected at an appropriate level and small bowel, thereby creating a retrograde intussu- the last 2–4 cm of the end of the proximal bowel are ception. This method may be more efficient, but def- everted and firmly fixed by 4/0 or 5/0 seromuscular inite clinical experience is very scarce.
    • Chapter 25 Short Bowel Syndrome 273 Figure 25.24 Figure 25.25 Figure 25.26
    • Michael E. Höllwarth 274 CONCLUSION The mainstay of the treatment of a newborn or child short bowel remnants. However, many of these chil- with an SBS consists of a sophisticated enteral stimu- dren died later on a total parenteral nutrition (TPN)- lation with an individually balanced nutritional associated liver failure, predominantly those with se- equilibrium among carbohydrates, proteins and fatty vere motility disorders. Therefore, today, the method25 acids. The enteral nutrition is the best stimulus for is recommended only in carefully selected patients, intestinal adaptation. The use of additional hormo- that means in SBS children around 1 year of age and nal therapies has not yet proved sufficiently effective not suffering from a life-threatening TPN-associated by controlled studies. Weaning from parenteral nu- liver failure. Furthermore, the method is indicated trition should be possible in more than 80% of the only in patients with a small intestinal length of a patients. Crucial for a successful weaning is the pres- maximum of 30 to 40 cm. In patients with longer ence of a good propulsive intestinal motility – with bowel remnants tapering alone is more appropriate or without adjunct surgical measures. Therefore, the and technically easier. above described methods are indicated only in some Basically, all surgical measures with the intention individuals as a helpful adjunct therapy to the enter- to prolong intestinal transit time must be located at al nutritional program and must be planned very the distal end of the small bowel in order to allow as carefully. much digestion and absorption as possible in the Tapering is indicated in patients with sufficient proximal band. One exception is the isoperistaltic amount of intestinal length but severely dilated small interposition of a colonic segment that must be bowel with impaired propulsive peristalsis. The in- placed in the proximal part of the small bowel be- testinal content in the enlarged intestinal loops caus- cause not only it prolongs the passage time, but it es bacterial overgrowth, inflammation, and bacterial seems to adapt by time some resorptive capacities translocation with recurrent sepsis. Grossly dilated thereby increasing additionally the absorptive sur- loops exist either primarily due to an intentionally face area. A difficulty of all these surgical methods is limited resection of small bowel in patients with in- to find the ideal balance between the desired prolon- testinal atresia, or they are the result of the process of gation of the transit time without producing an ileus. intestinal adaptation with a subsequent dilatation of None of these techniques have been used extensively the intestinal tract. in patients. Therefore, prospective studies are lack- The method of intestinal tapering and lengthen- ing, mostly due to the small number of relevant cases ing has attracted large attention in the past and has who may profit from such a particular procedure. been used even in newborns with atresia and very SELECTED BIBLIOGRAPHY Höllwarth ME (1999) Short bowel syndrome: pathophysiology Sukhotnik I, Siplovich L, Shiloni E, Mor-Vaknin N, Harmon and clinical aspects. Pathophysiology 6 : 1–19 CM, Coran AG (2002) Intestinal adaptation in short-bowel Höllwarth ME (2003) Short bowel syndrome and surgical tech- syndrome in infants and children: a collective review. Pedi- niques for the baby with short intestines. In: Puri P (ed) atr Surg Int 18 : 258–263 Newborn surgery. Arnold, London, pp 569–576 Thompson JS, Pinch LW,Young R,Vanderhoof JA (2000) Long- Mayr J, Schober PH, Weissensteiner U, Höllwarth ME (1999) term outcome of intestinal lengthening. Transplant Proc Morbidity and mortality of the short bowel syndrome. Eur 32 : 1242–1243 J Paediatr Surg 9 : 231–235
    • CHAPTER 26 Hirschsprung’s Disease Prem Puri INTRODUCTIONHirschsprung’s disease (HD) is characterised by an transitional zone into dilated colon. In the presenceabsence of ganglion cells in the distal bowel and ex- of enterocolitis complicating HD, barium enema maytending proximally for varying distances. The ab- demonstrate spasm, mucosal edema and ulceration.sence of ganglion cells has been attributed to failure The diagnosis of HD is confirmed on examinationof migration of neural crest cells. The earlier the ar- of rectal biopsy specimens. The introduction of his-rest of migration, the longer the aganglionic seg- tochemical staining technique for the detection ofment. The pathophysiology of Hirschsprung’s dis- acetylcholinestrase activity in suction rectal biopsyease is not fully understood. There is no clear expla- has resulted in a reliable and simple method for thenation for the occurrence of spastic or tonically con- diagnosis of HD. Full thickness rectal biopsy is rarelytracted aganglionic segment of bowel. indicated for the diagnosis of HD. Once the diagnosis The aganglionosis is confined to rectosigmoid in of HD has been confirmed by rectal biopsy examina-75% of patients, sigmoid, splenic flexure or trans- tion, the infant should be prepared for surgery. Biop-verse colon in 17% and total colon along with a short sies for frozen sections are taken to determine the ex-segment of terminal ileum in 8%. The incidence of tent of aganglionosis and level of transition zone.HD is estimated to be 1 in 5000 live births. The dis- In recent years, the vast majority of cases of HDease is more common in boys with a male-to-female are diagnosed in the neonatal period. Many centresratio of 4 : 1. The male preponderance is less evident are now performing one-stage pull-through opera-in long-segment HD, where the male-to-female ratio tions in the newborn with minimal morbidity ratesis 1.5–2 to 1. and encouraging results. The advantages of operat- Of all cases of HD, 80–90% produce clinical symp- ing on the newborn are that the colonic dilatationtoms and are diagnosed during the neonatal period. can be quickly controlled by washouts and at opera-Delayed passage of meconium is the cardinal symp- tion the calibre of the pull-through bowel is near nor-tom in neonates with HD. Over 90% of affected pa- mal, allowing for an accurate anastomosis that mini-tients fail to pass meconium in the first 24 h of life. mizes leakage and cuff infection. Recently, a numberThe usual presentation of HD in the neonatal period of investigators have described and advocated a va-is with constipation, abdominal distension and vom- riety of one-stage pull-through procedures in theiting during the first few days of life. About one-third newborn using minimally invasive laparoscopicof the babies with HD present with diarrhoea. Diar- techniques. More recently, a transanal endorectalrhoea in HD is always a symptom of enterocolitis, pull-through operation performed without openingwhich remains the commonest cause of death. the abdomen has been used with excellent results in The diagnosis of HD is usually based on clinical rectosigmoid HD.history, radiological studies, anorectal manometry A number of different operations have been de-and in particular on histological examination of the scribed for the treatment of HD. The four most com-rectal wall biopsy specimens. Barium enema per- monly used operations are the rectosigmoidectomyformed by an experienced radiologist, using careful developed by Swenson and Bill, the retrorectal ap-technique, should achieve a high degree of reliability proach developed by Duhamel, the endorectal proce-in diagnosing HD in the newborn. It is important dure developed by Soave and deep anterior colorectalthat the infant should not have rectal washouts or anastomosis developed by Rehbein. The basic princi-even digital examinations prior to barium enema, as ple in all these procedures is to bring the ganglionicsuch interference may distort the transitional zone bowel down to the anus. The long-term results of anyappearance and give a false-negative diagnosis. A of these operations are very satisfactory if they aretypical case of HD will demonstrate flow of barium performed correctly.from the undilated rectum through a cone-shaped
    • Prem Puri 276 Figure 26.1 The abdomen is opened via the Pfannenstiel inci- narrowed segment of bowel, a second biopsy from sion. The biopsy site is selected by observing the ap- the transition zone and a third biopsy from the dilat- parent transitional zone. In the usual case of recto- ed portion above the transition zone. Biopsies are as- sigmoid aganglionosis, three seromuscular biopsies sessed intra-operatively by frozen section, to deter- are taken along the antimesenteric surface without mine the level of ganglionic bowel. entering the lumen. One biopsy is taken from the26 Figure 26.2 Many surgeons prefer right transverse colostomy; V-shaped incision is made in the right upper quad- others advocate performing colostomy just above the rant. The V-skin-flap is reflected upwards. The exter- transition to ganglionic bowel. Ileostomy is indicated nal oblique is split and the internal oblique and in patients who have total colonic aganglionosis. A transverse abdominis muscles are divided with dia- right transverse colostomy is convenient in usual cas- thermy. The peritoneum is opened. es.We perform a loop colostomy over a skin bridge.A
    • Chapter 26 Hirschsprung’s Disease 277 Figure 26.1 Biopsy sites Figure 26.2
    • Prem Puri 278 Figure 26.3–26.5 An opening is made in the mesocolon of the selected toneum, the muscle layers of abdominal wall and the segment of transverse colon. The skin flap is pulled seromuscular layer of colon. The colon is opened through the opening in the mesocolon and sutured to longitudinally along the antimesenteric border using the opposite skin margin. A few interrupted absorb- diathermy. The bowel is sutured to the skin using able sutures of 4/0 or 5/0 are placed between the peri- interrupted 4/0 absorbable sutures.26
    • Chapter 26 Hirschsprung’s Disease 279 Figure 26.3 Figure 26.4 Figure 26.5
    • Prem Puri 280 Figure 26.6 Many surgeons have reported good results with the tions are carried out twice a day for 3 days before sur- primary neonatal pull-through operation for HD. gery. Intravenous gentamicin and metronidazole are The author prefers the one-stage transanal endorec- started on the morning of operation. tal pull-through operation for classical recto-sig- The patient is positioned on the operating table in moid HD and Swenson’s pull-through operation for the lithotomy position. The legs are strapped over long-segment HD because of their simplicity and sandbags. A Foley catheter is inserted into the blad-26 lack of complications. We have not used diversionary der. A Denis-Browne retractor or anal retractor is colostomy for usual cases. placed to retract perianal skin. The rectal mucosa is Of patients with HD, 75–80% have rectosigmoid circumferentially incised using the cautery, approxi- aganglionosis. A one-stage pull-through operation mately 5 mm above the dentate line, and the submu- can be successfully performed in these patients using cosal plane is developed. The proximal cut edge of a transanal endorectal approach without opening the the mucosal cuff is held with multiple 4/0 silk su- abdomen. This procedure is associated with excellent tures, which are used for traction. The endorectal dis- clinical results and permits early postoperative feed- section is then carried proximally, staying in the sub- ing, early hospital discharge and no visible scars. mucosal plane. Once the diagnosis of HD is confirmed, rectal irriga- Figure 26.7, 26.8 When the submucosal dissection has extended prox- bilized out through the anus. This requires division imally to a point above the peritoneal reflection, the of rectal and sigmoid vessels, which can be done rectal muscle is divided circumferentially, and the under direct vision using cautery or ligatures. full thickness of the rectum and sigmoid colon is mo-
    • Chapter 26 Hirschsprung’s Disease 281 Figure 26.6 Figure 26.7 Figure 26.8
    • Prem Puri 282 Figure 26.9 When the transition zone is encountered, full-thick- muscular cuff is split longitudinally either anteriorly ness biopsy sections are taken and frozen section or posteriorly. The colon is then divided several cen- confirmation of ganglion cells is obtained. The rectal timetres above the most proximal normal biopsy site.26 Figure 26.10 A standard Soave-Boley anastomosis is performed. formed 2 weeks after the operation. Routine rectal di- No drains are placed. The patient is started on oral lation is not performed unless there is evidence of a feeds after 24 h and discharged home on the third stricture. post-operative day. Digital rectal examination is per-
    • Chapter 26 Hirschsprung’s Disease 283 Figure 26.9 Figure 26.10
    • Prem Puri 284 Figure 26.11 For Swenson’s pull-through operation the patient is vessels are electro-coagulated under direct vision. positioned on the operating table to provide simulta- Sufficient tension-free length is obtained by dividing neous exposure of the perineum and abdomen. The the inferior mesenteric pedicle, carefully preserving pelvis is allowed to drop back over the lower end of the marginal vessels. Dissection is carried down to the table and the legs are strapped over sandbags. A the level of the external sphincter posteriorly and Foley catheter is inserted into the bladder. The abdo- laterally, but does not extend as deeply anteriorly,26 men is opened via a paramedian incision. Some sur- leaving around 1.5 cm of intact rectal wall abutting geons prefer a Pfannenstiel incision when perform- against the vagina or urethra. ing a Swenson’s pull-through operation in the neo- The mobilized rectum is intussuscepted through nate. Extramucosal biopsies are taken at intervals the anus by passing a curved clamp or a Babcock for- along the antimesenteric border and assessed by fro- ceps through the anal canal; an assistant places the zen section to determine the level of ganglionated closed rectal stump within the jaws of the clamp. bowel. The sigmoid colon is mobilized by dividing When the dissection has been completed, it should the sigmoid vessels and retaining the marginal ves- be possible to evert the anal canal completely when sels. It may be necessary to mobilize the splenic flex- traction is applied to the rectum. An incision is made ure to obtain adequate length. The proximal level of anteriorly through the rectal wall about 1 cm from resection above the ganglionated level, previously the dentate line, extending halfway through the rectal determined by frozen section, is selected and the circumference. A clamp is inserted through this inci- bowel is divided between intestinal clamps or sta- sion to grasp multiple sutures placed through the cut ples. end of the proximal colon. An outer layer of inter- The peritoneum is divided around its lateral and rupted 4-0 absorbable sutures is placed through the anterior reflection from the rectum, exposing the cut muscular edge of the rectum and the muscular muscle coat of the rectum. At this point, the bowel is wall of the pull-through colon. When the outer layer divided at the rectosigmoid junction and removed. has been completed, the proximal bowel is opened Dissection extends around the rectum, keeping very and an inner layer of interrupted 4-0 absorbable su- close to the bowel wall. It is essential to maintain the tures is placed. When anastomosis is completed, the dissection close to the muscular wall in order to pre- sutures are cut, allowing the anastomosis to retract vent damage to the pelvic splanchnic innervation. All within the anus. Figure 26.12 The advantage of the Duhamel pull-through is that terior wall of the aganglionic rectum and remainder very little manipulation of the rectum is performed of the colo-rectal anastomosis completed by approx- anteriorly thus avoiding injury to the genitourinary imating the aganglionic rectum to the posterior wall innervation. The rectum is divided and closed just of the pulled-through ganglionic bowel. Finally an above the peritoneal reflection. The redundant agan- extra long automatic stapling device is used to com- glionic bowel is resected. The retrorectal space is plete the side to side anastomosis between the agan- created by blunt dissection down to the pelvic floor. glionic rectum and the ganglionic pulled-through The posterior rectal wall is incised 1.5 to 2 cm above bowel. Some surgeons complete the side to side anas- the dentate line and sponge holding forceps is insert- tomosis prior to closing the rectal stump, thereby ed into the retrorectal space and ganglionic bowel preventing any residual septum. pulled through. The anterior half of the pulled- through ganglionic bowel is anastomosed to the pos-
    • Chapter 26 Hirschsprung’s Disease 285 Figure 26.11 Figure 26.12
    • Prem Puri 286 Figure 26.13 In Soave or endorectal pull-through the first steps of cosal tube is freed distally. The mucosal dissection is the operation are similar to those described for continued distally to the level of the dentate line. The Swenson’s or Duhamel operation. The colon is mobi- mucosa is incised circumferentially 1 cm above the lized and resected about 4 cm above the peritoneal dentate line. A Kelly clamp is inserted from below reflection. The endorectal dissection begins 2 cm be- and the ganglionic bowel is pulled through. Coloanal low the peritoneal reflection. The seromuscular layer anastomosis is completed using 4/0 absorbable su-26 is incised circumferentially and the mucosal-submu- tures. Figure 26.14 Rehbein’s technique differs from the Swenson’s pro- the terminal aganglionic rectum is left behind, which cedure, in that the anastomosis is a low, anterior is anastomosed to the ganglionic bowel. colorectal anastomosis. In this procedure, 3 to 5 cm of
    • Chapter 26 Hirschsprung’s Disease 287 Figure 26.13 Figure 26.14
    • Prem Puri 288 CONCLUSION The vast majority of patients treated with any one of intensity of bowel training, social background and the standard pull-through procedures achieve satis- respective intelligence of patients. Mental handicap, factory continence and function with time. The at- including Down syndrome, is invariably associated tainment of normal continence is dependent on the with long-term incontinence.26 SELECTED BIBLIOGRAPHY Georgeson KE (2002) Laparoscopic-assisted pull-through for Puri P (2003) Hirschsprung’s disease. In: Puri P (ed) Newborn Hirschsprung’s disease. Semin Pediatr Surg 11 : 205–210 surgery. Arnold, London, pp 513–533 Langer JC, Durrant AC, de la Torre L, Teitelbaum DH, Minkes Rolle U, Nemeth L, Puri P (2002) Nitrergic innervation of the RK, Caty MG, Wildhaber BE, Ortega SJ, Hirose S, Albanese normal gut and in motility disorders of childhood. J Pedi- CT (2003) One-stage transanal Soave pullthrough for atr Surg 37 : 551–567 Hirschsprung’s disease: a multicenter experience with 141 children. Ann Surg 238 : 569–583
    • CHAPTER 27 Anorectal Anomalies Alberto Peña, Marc A. Levitt INTRODUCTIONAnorectal malformations, represent a wide spectrum Colostomy is still a widely accepted procedure forof defects. Surgical techniques useful to repair the children born with rather high and complex malfor-most common types of anorectal malformations mations. The circumstances in which this procedureseen by a general pediatric surgeon are presented fol- is performed vary from one institution to anotherlowing an order of complexity from the simplest to and from one country to another. Some surgeons feelthe most complex. confident approaching newborns without a protec- Malformations considered “low” have traditional- tive colostomy. This is feasible and safe, provided thely been approached perineally, without a protective surgeon has experience in the management of thesecolostomy, whereas malformations that are consid- defects. Some surgeons do not feel confident withered “high” were treated by colostomy in the new- this approach and prefer a safer path in which theyborn period, subsequent definitive repair of the mal- open a protective colostomy, particularly when theformation, and eventual closure of the colostomy. baby is premature or has severe associated anoma-Surgical management has been evolving towards lies.fewer operations and minimally invasive procedures.Anorectal malformations have been included in thistrend.
    • Alberto Peña, Marc A. Levitt 290 Figure 27.1 The colostomy that we recommend in the manage- When creating the colostomy in the newborn, the ment of anorectal malformations is a descending co- surgeons should look for the descending colon and lostomy. The surgeon must understand that all colos- select the first portion of mobile sigmoid to open the tomies performed in a mobile portion of the colon colostomy. That part of the colon is usually very dis- have a tendency to prolapse. The mobile portion, in tended and full of meconium. The surgeon can place the type of colostomy that we propose, is the distal a purse-string suture, make an orifice in the centre, stoma (mucous fistula) and therefore the surgeon and pass a catheter to irrigate the sigmoid until all must either to fix this distal sigmoid colon to the an- the meconium has been removed. This simple ma- terior abdominal wall or make the mucous fistula neuver facilitates the manipulation of the colon and27 very small (4 mm in diameter) since it will be used helps to create a better colostomy. only for colonic irrigations or distal colostogram im- Loop colostomies are contraindicated in children aging procedures. The incision is created in the left with anorectal malformations. They have a tendency lower quadrant, long enough as to be sure that both to prolapse, and with them stool can pass into the the stomas are sufficiently separated as to be able to distal stoma, which may provoke faecal impaction in accommodate the stoma bag over the proximal sto- the distal rectum and also contaminate the urinary ma. The mucosa fistula should not be included under tract with faeces. the stoma bag. MALE DEFECTS Figure 27.2 í Perineal Fistula. This malformation represents ter until the posterior rectal wall that has a character- the simplest of the spectrum. In this defect, the rec- istic whitish appearance is identified. The surgeon tum opens immediately anterior to the centre of the continues dissecting in this plane, first the lateral sphincter, yet, the anterior rectal wall is intimately at- walls of the rectum and eventually the anterior rectal tached to the posterior urethra. The anal orifice is wall. While dissecting the anterior rectal wall, the frequently strictured. These patients will have bowel surgeon must put special emphasis in trying to avoid control with and without an operation. Some sur- urethral injury since there is no plane of separation geons decide not to operate on these patients. In such between rectum and urethra. Once the rectum has a case, the anus should be dilated to allow the easy been mobilized enough as to be moved back to be passing of stool, and the orifice should be sequential- placed within the limits of the sphincter. The limits of ly dilating up to no. 12 Hegar in a newborn baby. We the sphincter are determined with an electrical stim- prefer to operate on these babies to achieve a better ulator. The perineal body is then reconstructed with cosmetic effect. We also believe that the operation long-term fine absorbable sutures and the rectum is must be done as soon as the diagnosis is made. We anchored to the posterior edge of the muscle com- perform these operations without a colostomy in the plex. An anoplasty is performed within the limits of newborn baby. the sphincter with 16 circumferential 6/0 long-term The baby is placed in prone position with the pel- absorbable sutures. The baby does not have any diet vis elevated. It is mandatory to place a catheter in the limitations and receives intravenous antibiotics for urethra of the patient. The most common intra-oper- 24–48 h. ative complication is a urethral injury. The incision Dilatations are performed twice per day begin- usually extends mid-sagittally approximately two cm ning 2 weeks after surgery. The parents learn to ad- posterior to the anal orifice, dividing the entire vance one size (1 mm) every week until they reach the sphincter mechanism. Multiple very fine nonabsorb- size adequate for the patient’s age, which is no. 12 for able sutures are placed in the anal orifice in order to a newborn baby, no. 13 for a 4-month-old, no. 14 for exert traction to facilitate the dissection of the rec- an 8-month-old, no. 15 for a 1-year-old, and no. 16 for tum. The posterior incision divides the entire sphinc- older patients.
    • Chapter 27 Anorectal Anomalies 291 Figure 27.1 Figure 27.2
    • Alberto Peña, Marc A. Levitt 292 Figure 27.3, 27.4 í Rectourethral Fistula. This group of patients in- haps the kind of patient for this management would clude two specific categories: (a) rectourethral bul- be the one who has a rectal pouch located below the bar fistula (Fig 3), and (b) rectoprostatic fistula coccyx. In that way, the surgeon knows for sure that, (Fig 4). These two variants represent the majority of when he or she opens posterior sagittally, the rectum male patients with anorectal malformations. We be- is going to be found. The dissection of this distal rec- lieve that it is important to recognize the difference tum must be meticulous as it is intimately attached to between these types because of the prognostic and the urethra. therapeutic implications. Rectourethral bulbar fistu- If the surgeon does not have a specific and reliable la patients, in our experience have an 80% chance of image that shows the rectum is located below the27 having bowel control by the age of 3, whereas the rec- coccyx, he or she should never approach a patient toprostatic fistula patients only have a 60% chance. posterior sagittally without a colostomy and without Patients with a rectoprostatic fistula have a higher a distal colostogram. The distal colostogram is by far incidence of associated defects (60%) compared to the most valuable study in defining the anorectal patients with rectourethral bulbar fistula for whom anatomy. That study is done in patients with anorec- the incidence is 30%. The rectoprostatic patients re- tal malformations and can only be done when the pa- quire a more demanding perirectal dissection to mo- tient already has a colostomy. We have seen catas- bilize the rectum that is located higher in the pelvis. trophic complications during the performance of These operations are performed at our institution posterior sagittal operations in male patients, that when the babies are 4 weeks old. If the neonatal ap- did not have a distal colostogram. proach with no colostomy is to be attempted, per- Figure 27.5a–c We like to perform a cystoscopy in all patients, which muscle. In cases of the rectoprostatic fistula, (Fig. 5) helps the surgeon confirm the location of the fistula. the rectum is much smaller, it may not bulge through A Foley catheter is inserted and the patient is placed the incision, and the surgeon expects to find the rec- in prone position with the pelvis elevated. The inci- tum immediately below the coccyx. The surgeon sion is a posterior sagittal one, in between both but- should not look for the rectum in the lower part of tocks, running from the middle portion of the sa- the incision in patients with rectoprosatic fistula. crum down to the anal dimple, which is electrically Looking for a rectum without evidence that the rec- determined. The incision goes through skin, subcuta- tum is there is the main source of complications in neous tissue, parasagittal fibres, muscle complex and this approach. The surgeon will find the urethra, vas levator muscle. When the surgeon is dealing with a deferens, prostate, and seminal vesicles and will rectourethral bulbar fistula (Fig. 4), he expects to see damage the nerves important for urinary control and a bulging rectum as soon as he opens the levator sexual potency.
    • Chapter 27 Anorectal Anomalies 293 Figure 27.3 Figure 27.4 Figure 27.5a–c a b c
    • Alberto Peña, Marc A. Levitt 294 Figure 27.6, 27.7 The posterior rectal wall is easily identified by its stitches. The incision is continued distally, staying in characteristic whitish appearance. The surgeon must the midline while applying stitches in the edges of keep in mind that there is a fascia that covers the rec- the rectum. The traction on these stitches will allow tum posterior and laterally that must be removed. the surgeon to see the lumen of the rectum.When the The dissection of the rectum must be performed incision continues, extending distally in the midline, staying as close as possible to the rectal wall without it ends directly into the rectourethral fistula that is injuring the rectal wall itself. The posterior rectal identified usually as a 1–2 mm orifice. wall is opened in the midline, in between two 5/0 silk27
    • Chapter 27 Anorectal Anomalies 295 Figure 27.6 Figure 27.7
    • Alberto Peña, Marc A. Levitt 296 Figure 27.8 Multiple 6/0 silk sutures are placed in a semi-circum- from the urinary tract. The fascia that covers the rec- ferential fashion surrounding the fistula site above tum is removed, creating the lateral planes of the rec- and lateral to the fistula. These multiple sutures serve tum. The mucosa of the anterior rectal wall distal to the purpose of exerting uniform traction on the rec- the multiple 6/0 silk sutures is divided about 1 mm tal mucosa to facilitate the separation of the rectum deep.27 Figure 27.9, 27.10 The dissection continues between the rectum and tract. The separation of the urethra from the rectum urinary tract in a submucosal plane for approximate- is the most delicate part of the operation. Most of the ly 5–10 mm and then gradually becomes a full-thick- serious complications occur during this part of the ness dissection, looking at the lateral planes until the procedure. The fistula site is closed with three or four rectum is completely separated from the urinary 6/0 long-term absorbable sutures.
    • Chapter 27 Anorectal Anomalies 297 Figure 27.8 Figure 27.9 Figure 27.10
    • Alberto Peña, Marc A. Levitt 298 Figure 27.11 Figure 27.12 The surgeon should then evaluate the size of the rec- The limits of the sphincter are then electrically deter- tum and compare it to the available space to decide if mined and marked with temporary silk sutures. The the rectum has to be tapered to be accommodated perineal body is reconstructed when the incision ex- within the limits of the sphincters. In the past, we tends anterior to the centre of the sphincter. The an- performed many of these tapering procedures and terior limits of the sphincter must be reconstructed. we believe that is because patients came later in life, had inadequate colostomies, and therefore suffered from severe megarectum. Currently, babies are re- ferred to us earlier, surgeons are opening better, to-27 tally diverting colostomies, and we therefore do not see megarectums as often. Consequently, tapering is rarely necessary. When a tapering procedure is required, we recom- mend to remove an adequate portion of the posteri- or rectal wall, closing this wall into two layers of interrupted long-term absorbable sutures. Tapering on the anterior wall is absolutely contraindicated as it would leave a rectal suture line against the urethral fistula repair and a recurrent fistula may develop. Figure 27.13 The posterior edge of the levator muscle is electrical- The posterior edge of this muscle structure is su- ly determined, and the rectum is placed in front of tured together in the midline with interrupted 5/0 the levator. The posterior edges of the levator muscle long-term absorbable sutures. These sutures also are sutured together with interrupted 5/0 long-term take a bite of the posterior rectal wall in order to an- absorbable sutures. The distal continuation of the le- chor the rectum in a good position to avoid retrac- vator muscle is arbitrarily called the muscle complex. tion and/or prolapse.
    • Chapter 27 Anorectal Anomalies 299 Figure 27.11 Figure 27.12 Figure 27.13
    • Alberto Peña, Marc A. Levitt 300 Figure 27.14, 27.15 The rest of the incision is closed meticulously reap- 6/0 long-term absorbable sutures, trimming off that proximating all the layers of the wound. An anoplas- part of the rectum that has been damaged or does ty is performed with 16 circumferential stitches of not have adequate blood supply.27 Figure 27.16 í Recto-Bladder Neck Fistula. This malformation is sagittal approach alone. However, in a case of recto- the highest of all defects seen in male patients. For- bladder neck fistula, the rectum can be separated tunately, only 10% of male patients suffer from this from the urinary tract laparoscopically avoiding a particular defect. Associated defects occur in 90% of laparotomy. These patients, unfortunately, do not these patients. Usually, the sacrum is hypodeveloped. have a good functional prognosis. In our experience, This particular group of malformations is the only only 15% of them have voluntary bowel movements one that, in order to be repaired, requires not only a by the age of 3. These patients require a posterior sag- posterior sagittal approach, but also an abdominal ittal approach to create the space through which the one, either by laparotomy or laparoscopy. rectum will be pulled down. During the laparotomy The advent of minimally invasive procedures has or laparoscopy, the surgeon must separate the rectum been extended to anorectal malformations and we from the urinary tract. Fortunately, in these very high believe that it has specific indications in those pa- malformations, the common wall between the rec- tients that formerly required a laparotomy. We still tum and the urinary tract is very short. In other do not believe that the laparoscopic repair of a rec- words, the rectum connects to the bladder neck in a tourethral fistula is less invasive than the posterior “T” fashion.
    • Chapter 27 Anorectal Anomalies 301 Figure 27.14 Figure 27.15 Figure 27.16
    • Alberto Peña, Marc A. Levitt 302 Figure 27.17, 27.18 When we operate on these patients, we perform what the pelvic dissection necessary to mobilize it is mini- we call a total body preparation. The purpose is to mal. Ureters and vas deferens run very close to the have access to the perineum and to the abdomen, rectum to approach the trigone of the bladder. This when necessary. The operation is started via a poste- should be kept in mind to avoid any damage. The dis- rior sagittal approach. All the sphincter mechanisms section of the rectosigmoid must be performed while are divided in the midline. No attempt should be staying in direct contact with the bowel wall itself. made to find the rectum through this approach. A The rectum opens into the bladderneck in a T fash- rubber tube is placed in the presacral space behind ion. This means that there is no common wall above the urethra and located, following the tract the rec- the fistula as described in lower malformations. The27 tum will subsequently occupy. The perineal body, the fistula is divided and the bladder end is sutured with posterior edge of the levator muscle as well as the interrupted long-term absorbable sutures. The rub- muscle complex are reconstructed around the rubber ber tube is easily found in the presacral space. The tube that represent the rectum. The patient is then decision is made to taper the rectum when necessary turned onto a supine position and a laparotomy is and then to anchor the rectum to the rubber tube. performed. The bladder is pulled anteriorly and the The legs are lifted up and the rubber tube is pulled rectosigmoid is found. In these very high defects, the down, pulling together the rectum that will be placed rectobladderneck fistula is usually located within 1 to in the desired location. The anoplasty is performed 2 cm below the peritoneal reflection and, therefore, as previously described and the abdomen is closed. Figure 27.19 í Imperforate Anus Without Fistula. This particu- defect without Down’s syndrome have bowel control lar malformation is unique. When we say imperforat- and 80% of our Down’s syndrome patients have bow- ed anus without fistula, we do not have to refer to the el control. The technique to repair this malformation height of the defect because in all cases the rectum is is not necessarily simpler than the one for rectou- located approximately 1–2 cm above the perineal rethral bulbar fistula since the rectum is intimately skin, at the level of bulbar urethra. This malforma- attached to the posterior urethra. The surgeon has to tion only happens in 5% of all cases and half of these open the posterior rectal wall and still has to create a have Down’s syndrome. The patients with these de- plane of dissection between the anterior rectal wall fects have good prognosis, good sacrum and good and the urethra, a manoeuvre that requires a metic- sphincters. Ninety percent of our patients with this ulous dissection.
    • Chapter 27 Anorectal Anomalies 303 Figure 27.17 Figure 27.18 Figure 27.19
    • Alberto Peña, Marc A. Levitt 304 FEMALE DEFECTS Figure 27.20 Figure 27.21 Female defects are represented by a similar spec- í Rectovestibular Fistula. This defect is perhaps the trum. However, over the last 22 years, we have been most important anorectal malformation in females. learning a great deal about these defects, particularly This is because it is by far the most common defect with regard to cloacas. We have learned, for instance, seen in the females. It is also a malformation that has that the so-called rectovaginal fistula is an almost an excellent functional prognosis when managed nonexistent defect. Most of the patients that were re- correctly. Also, paradoxically and unfortunately, girls27 ferred to us after having an operation to repair a “rec- with these defects are the ones that we have seen suf- tovaginal fistula”, actually never suffered from that fer from more complications after a failed attempt to particular defect. Most of them suffered originally repair. For many years, it has been very controversial from a cloaca that was misdiagnosed; the surgeon re- whether this malformation should be treated with a paired the rectal component of the defect and left the previous protective colostomy or should be operated patient with a persistent urogenital sinus. In other primarily at birth. Again, we believe that this de- more unfortunate cases, the babies were born with pends very much on the experience of the surgeon. rectovestibular fistulas, the surgeons mislabelled and When a baby is born with this malformation at our misdiagnosed it as a rectovaginal fistula and per- institution and the baby is otherwise healthy, we re- formed an abdominal perineal procedure for a mal- pair this malformation during the newborn period. formation that could otherwise have been repaired If the baby is premature or has associated defects, it posterior sagittally with good results, leaving the pa- is always safer to open a protective colostomy. The tient totally incontinent for faeces. surgeon must keep in mind that dehiscence and in- fection in patients with anorectal malformations not í Rectoperineal Fistula. This defect is equivalent to only represent a few more days in the hospital and an the recto-perineal fistula in males already described. ugly scar but also represents the possibility of chang- Bowel control exists in 100% of our patients and less ing the prognosis for bowel control. than 10% of them have associated defects. The pa- Patients with a vestibular fistula have an excellent tients are faecally continent with and without an op- prognosis. Bowel control exists in 93% of our pa- eration. Constipation is a constant sequela and tients; 70% of them have constipation that is incur- should be treated energetically. This is true also for able but manageable and it should be treated aggres- the male patients with perineal fistulas. We have sively. learned that the lower the defect the more there is a The most important anatomical feature that chance of constipation. We have also learned that should be recognized by the surgeon is that the rec- constipation is a self-perpetuating and self-aggravat- tum and vagina share a long common wall that must ing condition that eventually produces severe mega- be separated, creating a plane of dissection where it colon, chronic faecal impaction and overflow pseu- does not exist, in order to mobilize the rectum and do-incontinence, and must vigilantly be avoided. put it in the right place. At our institution, the operation to repair this de- We believe that most of the complications that we fect is performed at birth. We offer this operation to have seen from treatment of this malformation orig- our patients mainly to avoid cosmetic, psychological inate from the lack of separation of these two struc- and potential obstetric sequelae in the future. We tures, or the defective separation of these two struc- perform this operation before the baby leaves the tures. hospital during the newborn period. The patient is placed in prone position with the pelvis elevated. Multiple 6/0 silk stitches are placed around the fistula site. The incision is about 1.5–2 cm long and divides the entire sphincter mechanism in the posterior midline. We dissect the rectum as previ- ously described in the case of perineal fistulas in male patients. The perineal body is reconstructed as shown and the rectum is anchored to the posterior edge of the muscle complex. An anoplasty is performed. These patients can eat on the same day of the sur- gery, and since they are only passing non-colonized meconium, we give antibiotics for 48 h.
    • Chapter 27 Anorectal Anomalies 305 Figure 27.20 Figure 27.21
    • Alberto Peña, Marc A. Levitt 306 Figure 27.22, 27.23 These patients are also placed in prone position with The rectum is then placed within the limits of the the pelvis elevated. The incision runs usually from sphincter as well as the muscle complex. We can see the coccyx down to the fistula site. Again, multiple only the lower part of the levator because the incision 6/0 sutures are placed in a circumferential manner in is rather limited in this operation. The rectum is an- the fistula opening. Traction always facilitates the chored to the posterior edge of the muscle complex dissections of these delicate structures. The entire and the anoplasty is performed like we discussed in sphincter mechanism is divided posteriorly until we the previous cases. identify the posterior rectal wall, and then the plane When the patient is a newborn, we keep the baby 2 of dissection is established removing the fascia that or 3 days with nothing by mouth post-operatively,27 covers the rectum. The dissection then continues lat- while receiving intravenous antibiotics. Occasionally, erally. The surgeon must put a special emphasis on a we see patients that come later in life without a colos- very meticulous separation of the rectum from the tomy; in those cases, we clean the bowel meticulous- vagina. The purpose of this dissection is to make two ly the day before surgery with a balanced electrolyte walls out of one. He or she should try at all costs to solution, insert a central line in the operating room avoid making perforations in the rectal wall or the and keep the patients 10 days with nothing by mouth, vaginal wall. Once the rectum has been completely while receiving parenteral nutrition. Following this separated from the vagina, the surgeon determines routine, we have never had a case of a wound infec- the limits of the sphincter electrically and recon- tion. structs the perineal body, bringing together the ante- rior limits of the sphincter.
    • Chapter 27 Anorectal Anomalies 307 Figure 27.22 Figure 27.23
    • Alberto Peña, Marc A. Levitt 308 Figure 27.24 í Cloaca. It was 1982 when we operated on the first All babies with cloaca should have a complete uro- patient with a cloaca using the posterior sagittal ap- logical evaluation at birth, as well as an ultrasound of proach. Since that time, until the writing of this the kidneys and ultrasound of the pelvis. The baby chapter, we have operated on 342 patients with cloa- should not be taken to the operating room without ca. Cloacas represent a spectrum of malformations. this evaluation. If the baby suffers from hydrocolpos, We have been learning many important lessons it is mandatory that the surgeon drain the hydrocol- about the treatment of these malformations. Based pos at the same time that he or she opens a colostomy. on that experience, we now believe that there are two Not draining a hydrocolpos may produce persistent distant groups of cloaca. This separation, we think is hydronephrosis and induce an inexperienced pediat-27 important for the pediatric surgeon and pediatric ric urologist to perform ureterostomies or nephrosto- urologist to recognize. In the first group are patients mies when they are not indicated. The drainage of the with a common channel shorter than 3 cm, and the vagina most frequently takes care of the problem of second group comprises patients with a common hydronephrosis. Also, the lack of drainage of a tense channel longer than 3 cm. hydrocolpos may produce infection of the vagina A cloaca is defined as a malformation in which the (pyocolpos), perforation and sepsis. rectum, vagina and urethra are congenitally fused, The colostomies must be completely diverting forming a common channel and opening in a single (separated stomas) to avoid contamination of the perineal orifice at the same location where the nor- urinary tract. We now repair cloacas when the babies mal female urethra is located. These three structures are 1-month-old, provided they are growing and de- share common walls that are very difficult to separ- veloping normally. ate. We have learned that the length of the common About 30% of the patients also have duplicated channel has important therapeutic and prognostic Mullerian structures. In other words, they have dif- implications. ferent degrees of septation and separation of the The group of cloacas with a short common chan- hemi-vaginas and two hemi-uterii. These have signif- nel (<3 cm) represents 68% of all our cloaca patients icant potential obstetric implications. and, therefore, we believe that perhaps there are Before we begin this operation, the patients are enough cloacas of this type in the world for the gen- placed in lithotomy position and a cystoscopy is per- eral pediatric surgeons to learn to repair them suc- formed. If the surgeon finds that the patient has a cessfully. On the other hand, 32% of all cloaca have a cloaca with a common channel shorter than 3 cm, it common channel longer than 3 cm, and we believe may be assumed that he or she will be able to repair that because of their complexity and rarity, they the malformation posterior sagittally only. Sugeons should be repaired by individuals with special train- may assume that they do not have to open the abdo- ing that includes a special dedication to pediatric men and that the final functional prognosis, both for urology. urinary and faecal function, is going to be reasonably Patients suffering from cloaca with a short com- good. On the other hand, if the patient has a longer mon channel (<3 cm), have a relatively low incidence common channel, she must be operated on by a high- of associated urologic, spinal, and vertebral defects, ly experienced and skilled surgeon or team of sur- whereas patients with long cloacas have a very high geons. frequency of association with complex pelvic anom- In cases of a short common channel, the patient is alies, mainly urologic, as well as vertebral. That is placed in a prone position with the pelvis elevated what makes the repair of this group very challenging. and the incision runs from the middle portion of the Patients with a short common channel can be re- sacrum all the way down to the single perineal ori- paired posterior sagittally only, without opening of fice. The entire sphincter mechanism is divided pos- the abdomen. teriorly and then the visceral structure (usually the When a baby is born with cloaca, the surgeon rectum) is exposed. For many years, the way we re- must keep in mind that approximately 50% of these paired these cloacas consisted of separating the rec- patients suffer from a very giant vagina full of fluid tum from the vagina and then separating the vagina called “hydrocolpos”. The hydrocolpos may com- from the urinary tract, reconstructing what used to press the trigone interfering with the drainage of the be the common channel as a neourethra and then ureters and therefore provoke bilateral megaureters placing the vagina behind the neourethra and the and hydronephrosis. It is imperative that the surgeon rectum within the limits of the sphincter mecha- to keep this in mind. nism.
    • Chapter 27 Anorectal Anomalies 309 Figure 27.24
    • Alberto Peña, Marc A. Levitt 310 Figure 27.25, 27.26 In the last 125 patients, we introduced a technical var- rience in the management of these cases. For that iant called total urogenital mobilization. The rectum kind of patient, the surgeon should have experience is separated from the vagina in the same way that in the management of the urinary structures includ- they were separated in the cases of rectovestibular ing bladder reconstructions, bladderneck recon- fistula. Once we expose the malformation, multiple structions, ureteral reimplantations, bladder aug- 6/0 silk stitches are placed taking the edges of the mentation and Mitrofanoff procedures. common channel and the edges of the vaginal walls. In cases with long common channels, if the pediat- We use those stitches to exert a uniform traction. ric surgeon and/or pediatric urologist both have ex- Then urethra and vagina together are mobilized in perience with these malformations they continue27 what we call total urogenital mobilization. Another with the repair, performing first what we call a total set of sutures is placed in a transverse fashion taking body preparation in order to have access both to the the mucosa of the common channel 5 mm proximal perineum and the abdomen. to the clitoris and then the entire common channel is If the common channel is longer than 5 cm, we rec- divided, full thickness, creating a plane of dissection ommend opening the abdomen directly in the mid- between the pubis and the common channel. This is a line because, in that particular case, rectum and vagi- natural plane of dissection and therefore it is very nas are not accessible posterior sagittally and are easy to dissect. We reach the upper part of the pubis more easily accessed through the abdomen. and there we can identify avascular fibrous struc- If the common channel is between 3 and 5 cm, the tures that fix the genitourinary structures to the pel- surgeon can open posterior sagittally, still will find vis. These are divided and that allows an immediate the vagina or vaginas and can try to repair them by gain of length of the urogenital structures of approx- performing the total urogenital mobilization. If the imately 2–4 cm, which is enough to repair the malfor- total urogenital mobilization proves not be enough mations with short common channels. to repair the malformation then the operation must The goal of this mobilization is to move what used be completed through a laparotomy. The separation to be a urethral meatus all the way down, to be placed of the rectum from the vagina is not difficult in very immediately behind the clitoris to be visible and ac- high malformations since it is similar to what we de- cessible for future urethral catheterizations when in- scribed in the separation of the rectum from the dicated. The vagina comes down together with the bladder neck in male patients. On the other hand, the urethra and the edges of the vagina are sutured to the separation of the vagina from the urinary tract in a skin of the perineum forming the new labia. What case of a cloaca with a long common channel is a very used to be the common channel is divided in the delicate manoeuvre that requires expertise and fi- midline creating two flaps that are preserved and su- nesse. Once the vagina has been completely separat- tured to the new labia to enrich that tissue.All this re- ed, which is a manoeuvre that may take several construction is performed with interrupted 6/0 long- hours, the surgeon then has to make important deci- term, absorbable sutures . With these kinds of tech- sions considering the way to repair the vagina. The niques, in patients with a good sacrum, 80% of them separation of the vagina from the urinary tract have bowel control and approximately 80% of them should be performed with the bladder open in the can empty the bladder voluntarily without intermit- midline and with ureteral catheters. The ureters run tent catheterization, and remain dry of urine; 20% of through the common wall that separates the vagina them require intermittent catheterization to empty from the bladder and therefore, the surgeon should the bladder. Fortunately, they have a good bladder be ready to deal with the fact that the ureters may be neck and they remain dry between catheterizations. right in the plane of separation. Once the vagina has When the sacrum is very abnormal, the results are been separated, the surgeon should evaluate the size not as good. of the mobilized vaginas and the length that he needs The repair of patients suffering from cloacas with to bring the vagina down. It may be that the patient longer common channels (>3 cm) represents a real has a very large vagina that reaches the perineum. technical challenge and requires a great deal of expe-
    • Chapter 27 Anorectal Anomalies 311 Figure 27.25 Figure 27.26
    • Alberto Peña, Marc A. Levitt 312 CONCLUSION Clinical results are different for each type of the mal- ry effort should be made to try to keep the rectosig- formations. A recent review of the authors’ series moid empty and decompressed from day 1 in these showed that 100% of patients with rectal atresia and patients. perineal fistula had voluntary bowel movements, Urinary control can be expected in the over- those with vestibular fistula 93.8%, bulbar urethral whelming majority of male patients after repair of fistula 87.5%, imperforate anus without fistula 85%, imperforate anus provided a good surgical technique cloaca 83.3%, rectourethral prostatic fistula 76.5%, was performed. Urinary incontinence is a concern and bladderneck fistula 28.6%. Soiling in patients only in male patients with absent sacrum or in some who enjoy voluntary bowel movements usually rep- female patients with cloaca. In this last defect, when27 resents a manifestation of faecal impaction and when the common channel is shorter than 3 cm, approxi- the constipation is treated properly, the soiling usual- mately 20% of patients require intermittent catheter- ly disappears. Patients who enjoy voluntary bowel ization to empty the bladder. The remaining 80% en- movements and never soil are considered “totally joy urinary control. When the common channel is continent”. longer than 3 cm it was observed that 68% of the pa- Constipation is a common sequela seen after the tients require intermittent catheterization to empty repair of an anorectal malformation. Interestingly, the bladder. Fortunately, after the repair of a cloaca, patients with lower defects, and therefore with better patients have a good bladderneck; they have the ca- prognosis for bowel control, suffer a higher incidence pacity to empty the bladder. Thus, when the bladder of constipation and vice versa. Constipation corre- becomes completely full, the patients start suffering lates directly with the degree of rectosigmoid dila- from overflow urinary incontinence. Intermittent tion at the time of colostomy closure. Therefore, eve- catheterization keeps these patients completely dry. SELECTED BIBLIOGRAPHY Hong AR, Rosen N, Acuña MF, Peña A, Chaves L, Rodriguez G Peña A (1997) Total urogenital mobilization-an easier way to (2002) Urological injuries associated with the repair of an- repair cloacas. J Pediatr Surg 32 : 263–268 orectal malformations in male patients. J Pediatr Surg Peña A (2003) Anorectal malformations. In: Ziegler M, Azizk- 37 : 339–344 han R, Gauderer M, Weber TR (eds) Operative pediatric Levitt MA, Stein DM, Peña A (1998) Gynecological concerns in surgery. Appleton & Lange, Norwalk CT the treatment of teenagers with cloaca. J Pediatr Surg Peña, A (2003) Anorectal anomalies. In: Puri P (ed) Newborn 33 : 188–193 surgery. Arnold, London, pp 535–552 Peña A (1995) Anorectal malformations. Semin Pediatr Surg 4 : 35–47
    • CHAPTER 28 Intussusception Karl-Ludwig Waag INTRODUCTIONIntussusception means by definition that one por- dominal pain with pulling legs up to the abdomention of the gut is telescoping in another distal part begins acutely at the rate of regular peristaltic activ-with further motion of the intussusception into the ities – initially around every 20 min – but with in-intussuscipiens by ongoing peristalsis. The most creasing frequency. Typically these attacks cease ascommon form is ileo-colic in 80–90% of cases, less promptly as they started and the child is quiet inoften ileo-ileal occurs in up to 15% and rarely caeco- between these regular recurrent attacks.caceo-colic, jejuno-jejunal or even ileo-ileo-colic oc- Examining the child, the right lower quadrantcur in a double or three-fold manner. Colo-colic is seems empty but a tender mass – mostly in the rightfound more often in parasitic diseases. upper quadrant – can be felt in about 85% of cases. In As far as aetiology is concerned, a mobile ascend- a later stage of intussusception, on rectal examina-ing colon, malrotation or non-rotation facilitates in- tion, mucous clots, currant jelly -like and occult, ortussusception. A leading point serves as head of in- even gross, blood may be seen. As soon as oedematussusception, which is drawn into the distal gut by and venous compression has developed, clinical ob-peristaltic activity, often in hyperperistalsis, as found struction of the bowel becomes obvious, leading toconcomitantly in episodes of gastro-enteritis or bilious vomiting, abdominal distension, dehydration,upper respiratory infections. The vast majority of tachycardia, fever and shock.cases are idiopathic (80–90%). Typical patients affected are usually 3–12 months Swollen Payer’s plaques in adenovirus or rotavirus old, well nourished and healthy infants. Incidenceinfection protrude into the lumen of the bowel, in- overall is 1–4%. Less than 1% of intussusceptions areitiating intussusception; so, lymphoid hypertrophy found in neonates and up to 95% occur within themay be primary but is secondary as well, since swol- first 2 years (van der Laan et al. 2001). With increas-len lymph nodes are found routinely. Mesentery is ing age a pathological lead point should be expected,drawn into the intussuscipiens and is more and more especially when the child is outside the usual agecompressed with lymphoid and vascular compro- range. In children older than 5 years a pathologicalmise, resulting in lymphatic and venous obstruction. lesion is found in 75–90%. Leading points oftenProgressive oedema of the bowel wall finally leads to found are Meckel’s diverticulum, polyps, appendix ormucosal bleeding and possibly to arterial insufficien- lymph nodes; rarely found but well known are neo-cy and bowel necrosis. plastic lesions like carcinoid tumour or Hodgkin Typical symptoms are explained by this patholog- lymphoma, foreign bodies, ectopic tissue of pancreasical pattern. In an early state initial vomiting – found or gastric mucosa, duplication cyst or, in newborns,in 80% – and lethargy are caused by tearing of the atresia. Intramural haematoma may induce intussus-mesentery; so far there is no obstruction, as well as ception especially in Schönlein-Hennoch disease.no abdominal distension. Colicky, intermittent ab-
    • Karl-Ludwig Waag 314 Figure 28.1 í Diagnostics. Plain abdominal radiograph is only perforation. In retrograde barium enema formerly indicated in unclear diagnosis, showing only late used, the contrast medium is outlining the apex of specific signs of abdominal gas distribution or faecal the intussusceptum. contents and, finally, signs of bowel obstruction or28 Figure 28.2 Figure 28.3 Ultrasonography as well as fluoroscopy with contrast í Non-surgical Treatment. Before preparing for enema is well established; ultrasonography is pre- conservative treatment radiologists should inform ferred mostly because there is no irradiation and the pediatric surgeon and the operating room. In re- diagnostic sensitivity of ultrasound (US) is 98.5% cent literature there is no contraindication for a con- and the specificity is 100%. Typical patterns of US are servative trial of reduction in respect to hours of case target – or doughnut or pseudo-kidney sign. This history or position of the apex. But non-surgical means “ring in the ring” in the transverse section or a treatment should not be undertaken in a patient with kidney-like formation in longitudinal section of the clinical signs of shock, peritonitis or heavy obstruc- intussusceptum. Absence of colour flow in colour tion. Hydrostatic reduction was formerly done by a Doppler suggests compromise of the mesenteric vas- barium or soluble isotonic contrast enema. Nowa- culature, indicating a difficult reduction and a lower days, saline (Hartmann’s solution) or air is preferred reduction rate. needing less fluoroscopy or even no irradiation at all, since hydrostatic reduction can be visualized and controlled easily by ultrasound. In comparison to barium enema, air reduction is reported to be quick- er, less messy, decreases time of irradiation and shows a higher reduction rate (75 vs. 90%). In air re- duction there is no risk of barium peritonitis. Hydro- static reduction under ultrasound monitoring is, in the meantime at 89%, as effective as air reduction in experienced hands. Ultrasound during hydrostatic reduction reveals the head of the intussuceptum in transverse and longitudinal sections.
    • Chapter 28 Intussusception 315 Figure 28.1 Figure 28.2 Figure 28.3
    • Karl-Ludwig Waag 316 Figure 28.4 The technique of reduction is uniformly well accept- improvement and lethargy should disappear in the ed. The infant lies prone and should be on sedation following routine observation for 24–48 h. Prognosis and analgesics. A Foley catheter as big as possible is of non-surgical reduction can be predicted according inserted into the ampulla of the rectum. The buttocks to sonographic pattern. If it is target-like, a reduction are squeezed together and taped to prevent leakage. is to be expected in 100% of cases. In doughnut sign, Pressure used for reduction should not exceed 3 ft, patient’s reduction rate is related to thickness, being i.e., 90–100 cmH2O or 80–120 mmHg using air. Pres- 100% effective for a thickness less than 7.2 mm. sure can be maintained as long as reduction is occur- Whenever it is more than 14 mm a resection is very ring. In absence of progress for more than 3–4 min, likely required. Fluid within the head of intussuscep- hydrostatic pressure is reduced before reduction is tion means unsuccessful reduction is expected to be started again for a second or a third time. Reduction very likely. In unsuccessful or incomplete reduction28 may be continued for even 45–60 min as long as the manoeuvre may be repeated after 2–3 h when the steady progress is being made. Often a delay in the infant is still in good condition. The rate of recurrent caecum region is observed before free flow of air, sa- intussusception after non-operative reduction is 13% line or contrast medium into the distal small bowel is in the typical age group. Complications of the reduc- seen as well as the disappearance of the mass. Return tion manoeuvre are perforation, marked by free air, of flatus or faeces indicates the complete reduction. and fluid or contrast medium intraperitoneally. Clinically the infant must show an overall complete Figure 28.5 í Indication for Surgery. A residual intraluminal rameters. Antibiotics may be started even pre-opera- filling defect even with terminal reflux into the ileum tively when there are signs of peritonitis or sepsis. has to be considered as incomplete reduction. Early On the operating table, the child lies on his or her or multiple recurrences mean a likely leading point back with a roll under his middle abdomen and is exists. Whenever a pathological leading point is sug- under general anaesthesia including full relaxation. gested a straight-forward surgery is recommended. Mostly, a right transverse incision supra-umbilical at With evidence of a seriously ill patient with perito- the umbilicus or lower is recommended, adapted to nitis by bacterial translocation, by perforation or by the position of the apex of intussusception.A midline necrosis, as well as in a septic status, the treatment of longitudinal incision is an easier and quicker ap- choice is primarily surgical. Whenever no intussus- proach and may be used as well. Pararectal incision ception in the colon is found in obvious bowel ob- has been abandoned. Extensions of the incisions struction by ultrasound or enema fluoroscopy, the mentioned are readily possible if required. In right operation should be started at once because intus- transverse line the skin is cut. Fat, anterior rectus susception is likely to be located in the small gut. sheet, rectus muscle, posterior rectus sheet and later- Preparation of the patient for surgery in cases of in- al abdominal muscles, i.e., m. obliquus externus, tussusception should include decompression of gas- internus and transversus, are incised, mostly using trointestinal tract by open nasogastric tube, and diathermy. As soon as abdominal cavity is entered, monitoring of body temperature and oxygen. Labor- free peritoneal fluid is aspirated and a swab is taken. atory studies show dehydration, electrolyte deficien- Cloudy or sanguineous fluid raises the suspicion that cies, base excess abnormalities and inflammatory pa- a perforation or necrosis is going to be found.
    • Chapter 28 Intussusception 317 Figure 28.4 Figure 28.5
    • Karl-Ludwig Waag 318 Figure 28.6 Figure 28.7 If the intussusception can be reduced easily and rap- A check for a leading point is very important. It is idly there is no need to deliver the intussusception. regularly found after a full reduction that the circum- Thus, the incision may remain smaller, not bringing ference of the bowel formerly intussuscepted is the whole intussusception outside the abdomen, as thickened and oedematous and a typical dimple is well as when a partial reduction could be managed recognised at the site of the former leading point. intra-abdominally. But whenever the manoeuvre of This dimple, a Payer’s patch or an oedematous ileoc- reduction is difficult and an inspection of viability is ecal valve can mimic an intraluminal pathological necessary, it is easier to be done with the intussuscep- mass as lead point, but careful palpation and the tion outside. In order to deliver the intussusception it knowledge of this likely condition of dimple forma- may be wise to mobilize the bands of right ascending tion should prevent excising local tissue as a probe or colon to lateral abdominal wall by division. Often this even an unnecessary resection, particularly in the28 is not necessary because of a mobile caecum and typical infant age group. ascending colon. Fluid contaminated by bacterial Appendectomy is usually perfomed. Additional translocation may escape from the space between fixation of terminal ileum is hardly mentioned in the intussusception. Therefore, positioning of packs literature. To preventing a second intussusception, it should avoid further contamination during reposi- does make sense to fix that part where the disease is tion. Manual reduction has to be performed very starting most often, i.e., the terminal ileum. Three se- carefully and slowly. Taking the apex of intussuscep- ro-serosal stitches, 5/0 or 4/0, from terminal ileum to tion between fingers and cup of the surgeon’s hand at ascending colon are performed quickly and not the distal end, the intussusception is squeezed gently interfering with mesenteric vessels. Since the rate of in a retrograde direction distally to proximally. A recurrence is not high, it remains a personal decision layer of gauze between bowel and fingers may facili- regarding whether to do a fixation at initial intussus- tate this procedure. This gentle manipulation should ception. In recurrent intussusception it is of definite be more pushing at the apex rather than pulling benefit, since the ileocecal valve is widened. proximally at the intussusception. Time is an impor- For closure the abdominal cavity is irrigated with tant factor since oedema must be allowed to dissipate warm saline. No drainage is used. The peritoneum in order to avoid serosal or even seromuscular tear- and posterior rectus sheet is closed at the same time ing. Should this happen, serosal defects are left alone, by running 3/0 sutures. Continuous suturing is also seromuscular flaps are repositioned and fixed by 5/0 sufficient for anterior rectus sheet.A subcuticular 4/0 stitches. Attempts to perform reduction with instru- running suture provides a good skin adaptation and ments are followed by heavy laceration due to oede- a cosmetically good scar. Skin closure by intracuticu- ma and fragility. The reduced bowel wall is always oe- lar suture line, by fibrin sealant or metal clips is op- dematous with a non-shiny serosa, but may be dis- tional. coloured or even blue or black. As a test of viability, Indications for resection include irreducible in- administering moist and warm wraps may serve in tussusception, gangrenous bowel or perforation of order to check whether there is a regular colour com- the bowel. After resection of the bowel, end-to-end ing back after some minutes of waiting. intestinal anastomosis is completed in a similar way as shown in Chap. 22.
    • Chapter 28 Intussusception 319 Figure 28.6 Figure 28.7
    • Karl-Ludwig Waag 320 CONCLUSION í Postoperative Care. In a regular case without re- likely after surgical reduction, especially when a fix- section nasogastric tube delivers decompression of ation of terminal ileum was performed. A secondary the upper gastrointestinal tract for 1 or 2 days and intussusception may be reducible at the same rate as patients are kept on intravenous lines for substitu- in the initial episode. It may be expected in around tion of fluid and glucose. As soon as oedema of the 30% of cases within the first post-operative day. Clin- bowel wall is dissipated, the passage and peristalsis ically irritability and discomfort are the first signs of recovers early. Satisfactory bowel function is indicat- an early recurrence. Therefore, it makes sense that ed by an absence of spontaneous bile-stained flow these patients, even with a definite non-surgical re- out of nasogastric tube. The abdomen is soft early duction approach, are observed for this period of on, is not distended and during gentle palpation it 24–48 h in the hospital. Indication for surgery in re- does not hurt. Post-operatively there might be a currences is given in patients with more than one28 short period of elevated body temperature but pa- episode of recurrence, because success rate dimin- tients should clinically recover rapidly. Antibiotics ishes with multiple recurrences. Here, additional sus- are administered according to the intra-operative sit- picion of an anatomical leading point arises, espe- us. Single shot is sufficient in most cases with normal cially when children are older than 2 years of age. reduction manoeuvres. In cases of resection and Post-operative intussusception after abdominal opening the gut, the post-operative course is pro- and thoracic procedures do not occur often but are longed. Patients may be discharged as soon as taking well known in the literature. Especially prone to this sufficient fluid and having bowel motion. Parents condition are those procedures due to gastro-oe- should be informed whether an appendectomy has sophageal reflux, Hirschsprung’s disease and neuro- been performed or not. blastoma. Intussusception is found in up to 10% of all post-operative intestinal obstructions. These are í The Role of Laparoscopy. In a suspected jejunal or mostly ileo-ileal. Prolonged bilious nasogastric re- ileal intussusception or in the case of post-operative flux, bile-stained vomiting, diffuse pain and abdomi- or chronic intussusception, laparoscopy might be a nal distension are the clinical signs but are hardly to diagnostic tool or the treatment might even be lapar- be distinguished from other usual post-operative oscopically. A routine diagnosis of a primary intus- problems like adhesions, the beginning of anasto- susception by laparoscopy should be exceptional motic leakage or abscess formation. since ultrasound is perfect. A primary attempt to re- Chronic intussuception is a rare condition. This duce an ileo-colic intussusception by laparoscopic means a non-strangulating, long-lasting intussus- instruments does not correspond to the treatment of ception of a minimum of 14 days, mostly due to ana- gentle squeezing the apex instead of pulling. In the tomical leading points. The clinical picture is domi- literature a warning is given because laparoscopical nated by chronic diarrhoea, which is resistant to any instruments may easily damage the vulnerable therapy. Patients are evacuating bloody or mucous bowel. stools and suffer from colicky pain including epi- sodes of vomiting all the time, leading finally to í Recurrence of Intussusception. Recurrent intus- weight loss. Diagnosis can be achieved by ultra- susception is seen after hydrostatic or air reduction sound, contrast studies or even by laparoscopy. over all in around 13% of cases. Recurrence is less SELECTED BIBLIOGRAPHY van der Laan M, Bax NM, van der Zee DC, Ure BM (2001) The Shehata S, Kholi N El, Sultan A, Sahwi E El (2000) Hydrostatic role of laparoscopy in the management of childhood intus- reduction of intussusception: barium, air, or saline. Pediatr susception. Surg Endosc 15 : 373–376 Surg Int 16 : 380–382 Linke F, Eble F, Berger S (1998) Postoperative intussusception Stringer MD, Pablot SM, Brereton RJ (1992) Paediatric intus- in childhood. Pediatr Surg Int 14 : 175–177 susception. Br J Surg 79 : 867–876 Littlewood Teele R, Vogel SA (1998) Intussusception: the pedi- atric radiologist’s perspective. Pediatr Surg Int 14 : 158–162
    • CHAPTER 29 Appendectomy Vincenzo Jasonni INTRODUCTIONAcute appendicitis is the most common surgical velops. Laboratory investigations and plain radio-emergency in childhood.Appendicitis may present at graphs are neither sensitive nor specific in the diag-any age, although it is uncommon in preschool chil- nosis of appendicitis. In recent years, graded com-dren. Approximately one-third of children with acute pression ultrasonography of the right lower quad-appendicitis have perforation by the time of opera- rant has been shown to be a useful tool in the evalua-tion. Despite advances in improved fluid resuscita- tion of patients with clinical findings that are sugges-tion and better antibiotics, appendicitis in children, tive but not diagnostic of appendicitis, having a sen-especially in preschool children, is still associated sitivity of 80–94%, a specificity of 90% and an overallwith significant morbidity. accuracy of 90%. Computed tomography (CT) may The diagnosis of acute appendicitis in childhood be helpful in selected cases but is rarely needed.can sometimes be difficult. Definite diagnosis is In patients with an uncertain diagnosis of acutemade in only 50–70% of patients at the time of initial abdominal pain, a policy of active observation inassessment. The rate of negative pediatric appendec- hospital is usually practised. A repeated structuredtomy is in the range 10–50% in various reports. The clinical examination is simple and non-invasive.patient’s history and clinical examination are the Children with perforated appendicitis must bemost important tools for the diagnosis of appendi- treated pre-operatively to prevent dehydration andcitis. Peri-umbilical pain is often the first symptom generalized sepsis. Antibiotics against aerobic andfollowed by vomiting and fever. When the inflamma- anaerobic bacteria are essential to reduce complica-tion progresses, the pain localizes to the right lower tions and to prepare the patient for surgical proce-quadrant, and right lower quadrant tenderness de- dure.
    • Vincenzo Jasonni 322 Figure 29.1, 29.2 A transverse right lower quadrant skin crease inci- sion across McBurney’s point is recommended. The muscular layers are split in the direction of their fi- bres. The peritoneum is opened and fluid sent for culture.29
    • Chapter 29 Appendectomy 323 Figure 29.1 Figure 29.2
    • Vincenzo Jasonni 324 Figure 29.3, 29.4 The mesoappendix is divided and the appendiceal domen should be irrigated with saline. Drains are not base clamped and ligated. Stump inversion is option- necessary. The abdominal wall is closed in layers. The al. Several studies have reported no difference as re- skin is usually closed by subcuticular absorbable su- gards wound infection and post-operative fever tures, even in the case of perforation. Primary wound between one group in which the appendix was ligat- closure after perforated appendicitis is safe, econom- ed and doubly invaginated and another group in ical and advantageous in pediatric practice. which it was simply ligated. If pus is present, the ab-29 Figure 29.5 Figure 29.6 Laparoscopy-assisted removal of the appendix can Skeletonizing of the appendix is performed using a be performed using two different methods: first, the bipolar instrument or a monopolar hook; large ves- complete laparoscopic approach and, second, the sels are ligated using clips or regular sutures. Gener- transumbilical laparoscopic appendectomy (TU- ally, in the case of inflamed tissues and small-sized LAP). The complete laparoscopic procedure is per- vessels, clips or sutures are not required. The base of formed using three ports. The first is inserted the appendix is closed using staplers or is ligated through the navel, initially for the telescope and af- with two preformed loops. terwards for the operating instruments and the sta- TULAP is performed using a 10-mm telescope pler and to extract the appendix. The second port is with an operating channel through the umbilicus. positioned in the left iliac region for the telescope The appendix is grabbed and pulled through the um- and for the operating instruments. The third port is bilicus. The procedure is thereafter completed from positioned in the right iliac region to grab the appen- outside using a conventional approach through the dix. A direct transparietal suture is inserted in the umbilicus. The advantages of TULAP are the limited right iliac flank to keep the appendix in tension dur- dimension of the scars and good cosmetic results. ing the dissection. Before resecting the appendix, an exhaustive evaluation of the entire abdominal cavity must be done.
    • Chapter 29 Appendectomy 325 Figure 29.3 Figure 29.4 Figure 29.5 Figure 29.6 10 mm port 5 mm port 3 mm port
    • Vincenzo Jasonni 326 CONCLUSION Advances in peri-operative care and antibiotics have dectomy in childhood is very good. A small number resulted in a zero mortality rate and low morbidity in of patients may develop late adhesive intestinal ob- children with appendicitis. The long-term outcome struction. of the vast majority of patients who undergo appen- SELECTED BIBLIOGRAPHY Emil S, Mikhail P, Laberge JM et al (2001) Clinical versus sono- Moir CR (1992) Appendectomy: open and laparoscopic ap- graphic evaluation of acute appendicitis in children: a com- proaches. In: Spitz I, Coran AG (eds) Rob and Smith’s oper- parison of patient characteristics and outcomes. J Pediatr ative surgery. Chapman & Hall, London, pp 402–410 Surg 36 : 780–783 Puri P (1998) Appendicitis. In: Stringer MD, Oldham KT, Mou- Gauderer MWL, Crane MM, Green JA et al (2001) Acute appen- riquand PDE, Howard ER (eds) Pediatric surgery and urol- dectomy in children: the importance of family history. J Pe- ogy: long-term Outcomes. WB Saunders, London, pp 321–29 diatr Surg 36 : 1214–1217 328 Meguerditchian AN, Prasil P, Cloutier R (2002) Laparoscopic appendectomy in children: a favourable alternative in sim- ple and complicated appendicitis. J Pediatr Surg 37 : 695–698
    • CHAPTER 30 Omphalomesenteric Duct Remnants David Lloyd INTRODUCTIONThe embryonic yolk sac communicates with the ali- Diverticulitis: inflammation of a Meckel’s diver-mentary canal via the viteline duct, which normally ticulum produces symptoms identical to those ofregressed by the sixth or seventh week of gestation. acute appendicitis, which is the usual pre-operativeIncomplete regression results in various abnormal- diagnosis. The true diagnosis is seldom made priorities which may be apparent in the newborn infant at to laparotomy. Meckel’s diverticulitis may lead to for-birth. These include patency of the duct (omphalo- mation of an inflammatory mass and occasionally ismesenteric or vitello-intestinal duct), persistence of complicated by perforation.the patent intestinal end of the duct as a Meckel’s di- Intussusception: a Meckel’s diverticulum may in-verticulum, persistence of the omphalo-mesenteric vert into the lumen of the ileum and be draggedduct as a fibrous cord (Meckel’s band), the presence along by peristaltic contractions. This leads to intus-of a cyst within a Meckel’s band, or an isolated rem- susception, which will present with acute abdominalnant of intestinal mucosa at the umbilicus (umbilical pain and evidence of intestinal obstruction. At oper-polyp). Each of these may give rise to problems re- ation the intussusception is reduced as far as possiblequiring operation to remove the anomaly in the new- prior to resection of the diverticulum and adjacent il-born period or beyond. eum. The indications for resection of a Meckel’s diver- Meckel’s band obstruction: loops of small intes-ticulum include bleeding, diverticulitis, intussuscep- tine may become trapped around a Meckel’s bandtion, or Meckel’s band obstruction. The Meckel’s di- leading to intestinal obstruction with a high risk ofverticulum is lined by ileal mucosa. Islands of ectop- strangulation. Division of the band and excision ofic gastric tissue may also be present in a Meckel’s di- the diverticulum are required.verticulum and secrete gastric acid, which may lead Asymptomatic Meckel’s diverticulum: there is noto ulceration of the adjacent ileal mucosa and blee- sound evidence to support routine resection of anding which at times is severe. This is a common cause asymptomatic diverticulum encountered incidental-of rectal bleeding in infancy. Ectopic gastric tissue ly at operation.may be identified by Technetium99 scanning.
    • David Lloyd 328 Figure 30.1 Figure 30.2 General anaesthesia is used. No pre-operative prep- In most cases the diverticulum is excised with the aration is required other than routine withholding of adjacent segment of ileum. This ensures a straight feeds. A single dose of broad spectrum antibiotics is anastomosis of the ileum and ensures removal of any given on induction of anaesthesia to reduce the risk ectopic gastric tissue. The lines of resection are illus- of wound infection. trated. Note that the blood supply to the Meckel’s When the diagnosis is known, a bleeding or in- diverticulum runs from the small bowel mesentery flamed Meckel’s diverticulum may be approached across the ileum to the diverticulum; this vessel must through a right transverse abdominal incision situat- be separately divided. ed just below the level of umbilicus. For intestinal ob- struction, particularly when the cause is not known, a right transverse incision situated above the umbili- cus will provide good exposure and is easier to ex- tend across the abdomen should this be necessary. The Meckel’s diverticulum may be folded over and adherent to the adjacent small bowel mesentery. It is mobilized easily by dividing these peritoneal adhe-30 sions. Figure 30.3, 30.4 A narrow-based Meckel’s diverticulum may be ex- The open ileum is inspected for islands of ectopic cised using a transverse elliptical incision as illustrat- gastric tissue, which must be excised if present, and ed. A stay suture is inserted on either side of the ile- then is closed with interrupted inverting absorbable um at the corners of the proposed excision. The di- sutures. The abdomen is closed in a routine manner. verticulum is excised with a cuff of adjacent ileum.
    • Chapter 30 Omphalomesenteric Duct Remnants 329 Figure 30.1 Figure 30.2 Figure 30.3 Figure 30.4
    • David Lloyd 330 Figure 30.5, 30.6 A patent omphalomesenteric duct presents with a fis- fascia is opened transversely on either side of the fis- tula at the umbilicus through which small bowel con- tula, or a midline incision may be used. Both umbili- tent is discharged. There may or may not be an asso- cal arteries, the single vein and the urachal remnant ciated mucosal remnant at the umbilicus. Surgical are ligated and divided. The umbilical end of the fis- management is excision of the entire fistula, the in- tula is brought out through the sub-umbilical inci- testinal end of which usually widens into a Meckel’s sion. The omphalomesenteric duct is dissected into diverticulum. the peritoneal cavity and its insertion into the termi- The umbilical end of the fistula is excised circum- nal ileum is identified, exteriorized through the sub- ferentially. The omphalomesenteric duct is then ap- umbilical incision, and resected as described for exci- proached through a sub-umbilical incision. After in- sion of a Meckel’s diverticulum. The ileum is repaired cising the skin and subcutaneous tissues, these are by end-to-end anastomosis. The umbilical incision is retracted, and the patent omphalomesenteric duct is closed using a purse-string suture and the abdominal identified by blunt dissection. The abdominal wall incision is closed in layers using absorbable sutures.30
    • Chapter 30 Omphalomesenteric Duct Remnants 331 Figure 30.5 Figure 30.6
    • David Lloyd 332 CONCLUSION Post-operative care includes nasogastric tube drain- treat complications. The risk of post-operative com- age of the stomach and intravenous fluids are re- plications, notably wound infection, is low and the quired until normal gastrointestinal function is re- outcome of surgery is excellent. Successful use of a la- established. Post-operative antibiotics may be given paroscopic approach for these anomalies has been for prophylaxis against wound infection if indicated. reported. Failure of the viteline duct to regress results in anomalies that may require resection to prevent or SELECTED BIBLIOGRAPHY Moore C (1996) Omphalomesenteric duct malformations. Teitlebaum DH, Polley TZ, Obeid F (1994) Laparoscopic diag- Semin Pediatr Surg 5 : 116–123 nosis and excision of Meckel’s diverticulum. J Pediatr Surg Swaniker F, Soldes O, Hirschl RB (1999) The utility of techne- 29 : 495–497 tium 99m pertechnetate scintigraphy in the evaluation of St Vil D, Brandt ML, Panic S (1991) Meckel’s diverticulum in patients with Meckel’s diverticulum. J Pediatr Surg 34 : children: a 20 year review. J Pediatr Surg 26 : 1289–129230 760–764
    • CHAPTER 31 Ulcerative Colitis Risto J. Rintala INTRODUCTIONApproximately 10% of all cases of ulcerative colitis taken Crohn’s disease should be ruled out by everyhave their onset during childhood. In the Western possible measures. Crohn’s disease patients shouldWorld the incidence of pediatric ulcerative colitis in- not undergo restorative proctocolectomycreased until the 1970s and 1980s but has remained at The gold standard of surgery for ulcerative colitisthe same level thereafter. A typical age at the onset of had been proctocolectomy and permanent ileosto-symptoms is prepuberty or puberty. A trend towards my. Limited colonic resections, and colectomy andearlier appearance of symptoms has been noted re- ileorectal anastomosis have been abandoned becausecently; some patients develop symptoms as early as these have been associated with a high incidence oftheir primary school ages. complications and recurrence of the disease. Proc- The aetiology of ulcerative colitis is still unclear; tocolectomy and permanent ileostomy gives excel-therefore, there is no curative treatment. The medical lent control of ulcerative colitis and related symp-therapy of ulcerative colitis is based on systemic or toms, but is not very well tolerated by children andlocal suppression of the immune response of the adolescents because of significant social restrictionslarge bowel. This is best achieved by using acetylsali- and permanently altered body image that are relatedcylic acid derivatives, and systemic or locally acting to this operation. Since the late 1970’s restorativecorticosteroids. proctocolectomy with ileoanal anastomosis has In children, ulcerative colitis is more aggressive gained overall acceptance as the standard operativethan in adults. Children present more often with procedure for adult and also pediatric ulcerative col-widespread disease and develop pancolitis more of- itis. Many pediatric surgeons advocate use of an ilealten than adults. Therefore, children require more ag- reservoir; the most popular and easiest to constructgressive medical treatment than adults; corticoster- is the J-pouch. Some pediatric surgeons still useoids are usually needed to control the initial disease. straight ileoanal anastomosis without a reservoir.Systemic corticosteroids are a major concern; the Restorative proctocolectomy is a major operationside-effects of high-dose corticosteroid treatment on with significant incidence of post-operative compli-a growing and developing body are significant and cations. Septic complications are common as mostare often an indication for surgical treatment. patients with refractory ulcerative colitis are immu- Between 40–70% of children with ulcerative col- nosuppressed because of high-dose corticosteroiditis undergo surgical treatment. As most patients can treatment. The nutritional status of many patients isbe stabilized by medical treatment, emergency oper- often not very good due to long-term diarrhoea andations for toxic megacolon, unremitting bleeding or poor nutrient intake. To avoid septic complications itrefractory fulminant colitis are not common today. is imperative that systemic corticosteroids are ta-The typical indications for surgery of ulcerative col- pered to as low a level as possible, or preferablyitis are poor response to optimal medical treatment, changed to locally acting budesonide that has lessdependence on high-dose corticosteroids with sig- systemic immunosuppressive effects. The nutritionalnificant side-effects, delay in growth and maturation status should also be improved. It is usually possibleand severe extra-intestinal manifestations of the dis- to do this by dietary measures. Parenteral nutrition isease. Surgery should not be considered as a primary sometimes but rarely required to restore proper nu-or early treatment of ulcerative colitis. A significant tritional status.proportion of patients achieve long-term symptom If the patient has chronic diarrhoea, as many ofrelief with conservative treatment and may remain in them have, the bowel may be emptied by a simple co-remission with minimal or no medication. Moreover, lonic washout. If the patient does not have diarrhoeathe functional outcome following restorative proc- a whole-bowel washout with polyethyleneglycoltocolectomy is not comparable with normal bowel (PEG) solution is advisable. The site of the coveringfunction. When patients go through several exacer- loop ileostomy should be marked with a water-resist-bation phases of the disease they gradually learn to ant marker pen before the operation. A stoma site inaccept that their bowel will function between a few to the right lower abdominal quadrant is best deter-several times a day. Before proctocolectomy is under- mined when the patient is sitting.
    • Risto J. Rintala 334 Figure 31.1 Figure 31.2 Prophylactic antibiotic treatment (cefotaxime and It is important that the surgeon assesses that the ter- metronidazole) is started at the induction of the an- minal ileum reaches down to perineum before colec- aesthesia. The operation is undertaken under gener- tomy is started. If the rotation of the bowel is normal al anaesthesia; the use of nitrous oxide as an an- and the terminal ileum reaches the pubic bone, it is aesthetic is best avoided as it may distend the bowel. very likely that an ileoanal anastomosis can be per- Insertion of an epidural catheter for local anaesthet- formed without undue tension. After mobilization of ic infusion to control post-operative pain is advis- ileocecal region, the ileum is transected by a GIA sta- able. Additional pain control can be achieved by ad- pler, flush to the ileocecal junction. ministrating opioids by a PCA (patient controlled analgesia) system. A bladder catheter is inserted and left in place until pain control with opioids and epi- dural catheter can be discontinued. The patient is positioned in a lithotomy position with a 10–15º Trendelenburg tilt. The abdomen is prepped from lower chest to perineum. A midline in- cision starting from the midpoint between the xi- phoid process and umbilicus and extending through the umbilicus down to suprapubic region is used to get free access to the whole length of colon. Usually31 there is no need to use self-retaining wound retrac- tors; they may cause wound edge ischaemia and in- crease postoperative wound pain. The whole length of the bowel is inspected to rule out Crohn’s disease. Figure 31.3 The peritoneal reflections of the ascending colon and peritoneal reflections of the descending and sigmoid hepatic flexure are mobilized. The splenocolic liga- are transected. The vessels in the colonic mesenteri- ment is severed and splenic flexure mobilized. The um are ligated or cauterized near the bowel wall; usu- greater omentum can be preserved provided it does ally only the main arteries to the colon, right, middle not tear much during its dissection off from the and left colic arteries need ligatures. The colon is transverse colon. The dissection of the omentum is transected at the junction of the sigmoid and rectum best performed by bipolar cautery or scissors; the at- with a GIA stapler. The whole colon can be now re- tachments between transverse colon and omentum moved from the operative field. are transected flush to the colonic wall. The lateral
    • Chapter 31 Ulcerative Colitis 335 Figure 31.1 Figure 31.2 Figure 31.3
    • Risto J. Rintala 336 Figure 31.4, 31.5 Stay sutures or a right-angled large clamp on the long-bladed retractors and cranial pulling from the proximal rectal stump facilitate the dissection of the rectal stump facilitate the dissection. rectum. These allow the surgeon to pull and move the The dissection is continued down to the level of bowel on either side freely. The mesentery of the rec- pelvic floor. Rectal finger-examination is useful to as- tum in patients with severe ulcerative colitis is often sess the adequacy of abdominal dissection. If the inflamed and very thick. Dissection within the me- lowest level of abdominal mobilization is within sentery is time consuming and bloody. The easiest 3–4 cm from the anal verge, no problems are expect- way to proceed is to keep the plane of dissection right ed in the transanal mucosectomy and rectal pull- on the rectal wall. The small vessels entering the bow- through. el are cauterised flush to the bowel wall. Broad and31
    • Chapter 31 Ulcerative Colitis 337 Figure 31.4 Figure 31.5
    • Risto J. Rintala 338 Figure 31.6 Figure 31.7 The next stage in the operation is the mobilization of The length of the J-pouch is between 7–10 cm. The the ileum to reach to anal canal. The ileocolic artery terminal ileum is folded and the antimesenteric tip of is ligated and transected. The mesentery of the ileum the future pouch longitudinally opened with a caut- is mobilized up to the level of proximal superior mes- ery needle. The opening should be kept short, from enteric artery. This may require mobilization of the 1.5 to 2 cm, as it widens significantly when pulled root of the mesentery from duodenum and lower rim through to the anus. The GIA-stapler blades are in- of pancreas. The mesenteric arteries to the distal two serted into each arm of the pouch; the stapler is or three vascular arcades of the terminal ileum are li- closed and fired. One firing of a 75-mm stapler, or gated and transected proximally. In order to ensure two of a 50-mm stapler, is usually sufficient to con- tension-free ileoanal anastomosis, the distal end of struct a pouch. The stapled suture line can be rein- the ileum or the tip of the J-pouch should reach in forced with 4/0 or 5/0 absorbable sutures. The pouch front of the pelvic rim to the base of penis in males or and terminal ileum are wrapped in warm and moist anterior vestibulum in females. swabs and returned the abdomen. The abdominal in- cision is loosely packed with warm and moist swabs.31
    • Chapter 31 Ulcerative Colitis 339 Figure 31.6 Figure 31.7
    • Risto J. Rintala 340 Figure 31.8 The perineal phase of the operation starts by inser- lent access to the anal canal. Adrenaline in saline tion of holding stitches between the mucocutaneous (1:100,000) is injected under the mucosa to lift it up junction of the anal canal and a colostomy ring. and decrease bleeding during the initial phases of These keep the anus open and dilated, and give excel- transanal mucosectomy.31 Figure 31.9, 31.10 The transanal mucosectomy is started at the dentate my. The author uses small triangular clamps to grasp line. A small rim (5–6 mm) of anal transitional epi- the edge of the mucosal cuff. The mucosectomy is thelium should be left in situ, otherwise the sensibil- performed by a combination of sharp and blunt dis- ity of the anus is significantly decreased and the anal section with scissors. In colitis ulcerosa, the muco- sampling reflex may be lost. Disease recurrence in sectomy is much more difficult than in non-inflam- the transitional anal epithelium is unlikely. The matory conditions; blood loss is often also signifi- whole circumference of the anal canal mucosa is in- cant. Pre-operative treatment with locally acting cor- cised and the mucosectomy started. The red line in tisone foam or suppositories may decrease blood loss Fig. 9 depicts the line of dissection between the mu- and make the dissection easier. The mucosectomy is cosa and rectal muscle. Some surgeons prefer to use continued for 5–8 cm until the level above pelvic multiple stay sutures in the mucosa just above the floor is reached. level of the mucosal incision to facilitate mucosecto-
    • Chapter 31 Ulcerative Colitis 341 Figure 31.8 Figure 31.9 Figure 31.10
    • Risto J. Rintala 342 Figure 31.11, 31.12 The cuff consisting of muscular lining of the anal ca- in case of straight pull-through) is grasped with the nal and distal rectum can be transected transanally clamp and pulled through to the anus. The assistant when the pelvic cavity is entered at the upper end of confirms through the laparotomy incision that the the mucosectomy. Pulling from the mucosal tube mesentery of the pulled-through pouch is not twist- tents the proximal end of muscular cuff inside the ed. The mesentery of the pulled-through J-pouch (or more distal cuff; thus, the cuff can be safely transect- distal ileum) is the tightest component of the pulled- ed without damaging urethra and prostate. Another through segment and requires the shortest route. option is to evert the rectum through anus and sever Therefore, it is natural that in the pelvis the mesent- the muscular cuff outside the anus at the upper end ery is positioned anteriorly to the bowel; this does of mucosectomy. Bleeding from small vessels in the not mean that the pull-through segment would be cuff can be controlled by cautery. kinked. A long soft clamp is inserted through the anal muscular cuff to pelvis. The J-pouch (or distal ileum31
    • Chapter 31 Ulcerative Colitis 343 Figure 31.11 Figure 31.12
    • Risto J. Rintala 344 Figure 31.13 Figure 31.14 As there is often significant tension when the first The space between pulled-through bowel and poste- anastomotic stitches are inserted, it is advisable to rior peritoneum is closed with running 4/0 absorb- use four-quadrant holding stitches to initiate the su- able sutures. The pelvic cavity is inspected for bleed- turing of the anastomosis. The anastomosis is per- ing sites. A round skin disk is cut off the stoma site. A formed in one layer with interrupted sutures cruciate incision is made to the abdominal muscle between full-thickness ileum and anal canal. A suit- fascia at the site of the stoma. The opening in the fas- able material is absorbable 4/0 sutures. The tension cia and abdominal muscles are bluntly enlarged to in the suture line is usually relieved when the sutures accommodate two fingers. An opening is made in the between the anus and the stoma ring are cut. This al- anterior peritoneum and a loop of ileum as near as lows the suture line to retract within the anal canal. possible to the ileoanal anastomosis is pulled out for stoma formation. The abdominal incision is closed in layers and the stoma is matured over a stoma rod. Generally there is no need to drain the pelvic cavity. Post-operative gastric decompression by nasogas- tric tube is usually not required. The bladder catheter can be removed when epidural anaesthesia is discon- tinued. Antibiotic prophylaxis is continued for 72 h post-operatively. If the patient has been on high-dose corticosteroids pre-operatively, post-operative pa-31 renteral corticosteroid therapy is required until oral intake of medication is possible. Post-operative corti- costeroids can be discontinued when sufficient func- tion of patient’s own adrenal glands is confirmed by an ACTH-stimulation test. Enteral feeding is encouraged and most patients tolerate full enteral feeding within the first 5 post-op- erative days. Stoma output is often excessive and should be replaced according to output and electro- lyte content of the stoma fluid. In most cases Ringer’s lactate is sufficient for replacement. Oral sodium supplementation to decrease stoma output is started as soon as the patient tolerates intake of salt tablets. Post-operative dietary management consists of lactose-free low residue diet. The amount of sodium supplementation can be monitored by spot urinary sodium measurements. The urinary sodium concen- tration should be kept higher than 20 mmol/l. Insuf- ficient salt intake leads to increased and watery sto- ma output. The covering stoma can be taken down when the healing of the pouch and the ileoanal anastomosis is confirmed. A distal loopogram 3–6 weeks after the operation is used to assess the integrity of the ileo- anal anastomosis and J-pouch. The immediate post- operative phase is characterized by loose and fre- quent bowel movements up to 10–12 times per 24 h. Antipropulsive medication (loperamide) is useful in slowing down gut motility. The bowel frequency gradually decreases to 2–7 bowel movements per 24 h within 3–6 months. Low residue diet and salt supple- mentation are helpful during the adaptation phase.
    • Chapter 31 Ulcerative Colitis 345 Figure 31.13 Figure 31.14
    • Risto J. Rintala 346 CONCLUSION Ileoanal anastomosis has revolutionized the treat- chronic inflammation in the pouch (pouchitis) is an ment of ulcerative colitis also in children. Long-term innate problem related to ileoanal pull-through for patient satisfaction following the operation is excel- ulcerative colitis. Its incidence varies between 20% lent despite a high incidence of post-operative com- and 50% of the patients. Most acute bouts of pou- plications. In children with a pouch ileoanal anas- chitis respond rapidly to a short course of oral antibi- tomosis, the functional outcome in terms of fecal otics, for example metronidazole. Chronic pouchitis continence is good. Usually all patients resume full is much less common and occurs in less than 10% of daytime faecal continence within 6 months following the patients. The treatment of chronic pouchitis con- closure of the stoma. A few patients suffer from slight sists of long courses of low-dose antibiotics and in re- night-time staining that may require protective pads. calcitrant cases oral corticosteroids, preferably bu- In the absence of major post-operative complica- desonide. Chronic pouchitis may be a presentation of tions, gross faecal soiling is practically nonexistent. Crohn’s disease; eventually 5–15% of patients who The frequency of bowel movements 6–12 months have undergone ileoanal anastomosis for ulcerative post-operatively is between two and seven times per colitis are discovered to have Crohn’s disease. An- 24 h. In the series of Children’s Hospital, University of other symptom that should raise suspicion of Helsinki, the median bowel frequency 6 months Crohn’s disease is pouch fistulization, especially re- post-operatively is four, ranging from two to seven current fistulas. per 24 h. Despite the multitude of potential post-operative Early and late complications occur in 20–50% of problems, a great majority of patients who have had31 patients; most common are wound infections in pa- restorative proctocolectomy for ulcerative colitis re- tients who have been using high-dose corticosteroids sume highly satisfactory lifestyle with complete fae- prior to the operation, and bowel obstructions. Pelvic cal continence and acceptable frequency of daily septic complications or separation of the ileoanal an- bowel movements. atomosis occur in less than 10% of the cases. Acute or SELECTED BIBLIOGRAPHY Coran AG (1990) A personal experience with 100 consecutive Sawczenko A, Sandhu BK (2003) Presenting features of inflam- total colectomies and straight ileoanal endorectal pull- matory bowel disease in Great Britain and Ireland.Arch Dis throughs for benign disease of the colon and rectum in Child 88 : 995–1000 children and adults. Ann Surg 212 : 242–248 Stavlo PL, Libsch KD, Rodeberg DA et al (2003) Pediatric ileal Durno C, Sherman P, Harris K et al (1998) Outcome after ileoa- pouch – anal anastomosis: functional outcomes and qual- nal anastomosis in pediatric with ulcerative colitis. J Pedi- ity of life. J Pediatr Surg 38 : 935–939 atr Gastroenterol Nutr 27 : 501–507 Rintala RJ, Lindahl H (1996) Restorative proctocolectomy for ulcerative colitis in children – is the J-pouch better than straight pull-through. J Pediatr Surg 31 : 530–533
    • CHAPTER 32 Crohn’s Disease Risto J. Rintala INTRODUCTIONThe incidence of Crohn’s disease in children has crosis factor-α antibodies (infliximab), have beenclearly been increasing since 1970s. The age at onset of adopted for management of fistulizing and refractorythe disease is similar to that of ulcerative colitis; how- Crohn’s disease. Internal and severe rectoperineal fis-ever, patients with Crohn’s disease tend to be younger tulas respond well to infliximab therapy and most pa-than those with ulcerative colitis. The diagnostic de- tients resume a longer period of remission. Similar re-lay in Crohn’s disease is usually significantly longer sults have been reported in patients who have diseasethan in ulcerative colitis, usually longer than 1 year refractory to conventional medication. However,from the onset of symptoms. The symptoms are usu- long-term efficacy and safety of biological treatmentally relatively vague at the early stages of disease. The of Crohn’s disease still remains unclear.most common symptom is non-specific abdominal It is evident that the development of drug therapiespain. General symptoms are more common in pa- has reduced the need for surgical therapy for Crohn’stients with Crohn’s disease. Growth failure and delay disease. The indications for surgery are limited to cas-of sexual maturation are significantly more common es that are refractory to medical therapy or medicalin Crohn’s disease patients than in those with ulcera- therapy that is poorly tolerated. Acute indications in-tive colitis. The presentation of Crohn’s disease in clude medically unmanageable toxic megacolon orchildren is usually less localized than in adults. acute bleeding, both of which are rare. Subacute or As in ulcerative colitis the aetiology of Crohn’s dis- chronic conditions that may require surgery includeease is unclear. It is likely that a genetic preponder- refractory strictures, internal or external fistula andance exists for Crohn’s disease since Crohn’s patients intra-abdominal abscesses.have family history of the disease more commonly The main principle in the surgical treatment ofthan those with ulcerative colitis. There is no defini- Crohn’s disease is to save bowel length. Resectiontive curative treatment for Crohn’s disease. As Crohn’s should be limited to the segment of bowel that isdisease is an affliction of the whole gastrointestinal causing symptoms. Isolated skip lesions are left alonetract surgery does not offer any permanent cure. Un- if they do not cause obstruction. In case of internallike in ulcerative colitis, surgical treatment is pallia- fistulization, the fistula usually originates from thetive in nature and aimed at treating the complications diseased proximal segment; distal target bowel isof the disease. usually healthy except for the fistula site. The strate- The medical treatment of Crohn’s disease has gy in these cases is to resect the diseased proximalevolved significantly during the last decade. The bowel and close the distal fistula site. In adults stric-mainstay of medical management is immunosup- tureplasty has been shown to be an effective bowelpression. The modality of medical management is dif- saving surgical method for multiple fibrotic stenoses.ferent in different localisations of the disease. Gener- The long-term outcome in terms of disease activity,ally speaking, the more widespread and more distal in risk of recurrence and quality of life has been verythe bowel the disease activity is, the more an aggres- similar than following resectional surgery. There aresive medical management is required. Predominantly, only a few reports of strictureplasty for Crohn’s dis-ileal disease can be managed with corticosteroids, ease in children but the preliminary results are simi-preferably with locally acting budesonide, and ami- lar as in adults.nosalicylates. Colonic involvement requires often Major bowel resections are sometimes required inantibiotics such as metronidazole or ciprofloxacillin association with severe and widespread colonic dis-in addition to the regimen used for ileal disease. Exac- ease. In these cases the rectum should be spared.erbations, relapses or refractory Crohn’s disease re- Ileoanal anastomosis with or without a pouch is notquire more effective medication; azathioprine or cy- indicated for Crohn’s disease; long-term outcome isclosporin is often helpful in inducing remission. Nov- very unpredictable and often poor and, moreover,el biological immunomodulatory drugs, tumour ne- pouch complications are very common.
    • Risto J. Rintala 348 Figure 32.1, 32.2 The most often needed surgical procedure is the re- few centimetres. Anastomosis is performed with an section of a localised stricture. The resection margin absorbable suture material and one-layer interrupt- to healthy bowel does not need to be longer than a ed stitches.32
    • Chapter 32 Crohn’s Disease 349 Figure 32.1 Figure 32.2
    • Risto J. Rintala 350 Figure 32.3, 32.4 In children, an internal fistula is often between ileum site. Proximal disease segment is resected with pri- and sigmoid bowel. The ileum is usually affected by mary anastomosis and the distal fistula opening ex- the disease and sigmoid healthy except the fistula cised and closed with interrupted sutures.32
    • Chapter 32 Crohn’s Disease 351 Figure 32.3 Figure 32.4
    • Risto J. Rintala 352 Figure 32.5, 32.6 Strictureplasty for multiple short jejunoileal stric- the macroscopically healthy bowel. The longitudinal tures. Strictureplasty can be combined with resection opening is closed transversely with interrupted su- of longer stricture segments. The stricture site is tures. opened longitudinally so that the incision extends to32
    • Chapter 32 Crohn’s Disease 353 Figure 32.5 Figure 32.6
    • Risto J. Rintala 354 CONCLUSION Operative treatment of Crohn’s disease is aimed at conservative approach is warranted and surgical management of complications of the disease that are treatment should be considered only in severely refractory to medical treatment. Although perma- symptomatic high rectoperineal or rectovaginal fis- nent cure cannot be expected following surgery, tulas that do not respond to infliximab or increased many patients resume longer periods of full remis- immunosuppressive therapy. If surgery is required sion with a normal lifestyle. Proper surgical treat- for a high perianal fistula resection of the mostly dis- ment in selected patients can also decrease or even eased usually left colonic segment and temporary abolish the need for immunosupressive and poten- bowel diversion may increase the success rate of the tially harmful medication for months and years. fistula repair. In very severe perianal disease, espe- Perianal manifestations of Crohn’s disease are cially if it is associated with severe rectal manifesta- very common in children. These include skin tags, tion, proctectomy may be the only possibility to fissures and fistulas. In most cases perianal manifes- guarantee a reasonable quality of life. tations cause mild symptoms or are asymptomatic. A SELECTED BIBLIOGRAPHY Baldassano RN, Han PD, Jeshion WC, Berlin JA, Piccoli DA, Escher JC, Taminiau JA, Nieuwenhuis EE et al (2003) Treatment Lautenbach E, Mick R, Lichtenstein GR (2001) Pediatric of inflammatory bowel disease in childhood: best available Crohn’s disease: risk factors for postoperative recurrence. evidence. Inflamm Bowel Dis 9 : 35–58 Am J Gastroenterol 96 : 2169–2176 Kim S, Ferry G (2002) Inflammatory bowel diseases in chil-32 Besnard M, Jaby O, Mougenot JF, Ferkdadji L, Debrun A, Faure dren. Curr Probl Pediatr Adolesc Health Care 32 : 108–132 C, Delagausie P, Peuchmaur M, Aigrain Y, Navarro J, Cezard Patel HI, Leichtner AM, Colodny AH, Shamberger RC (1997) JP (1998) Postoperative outcome of Crohn’s disease in 30 Surgery for Crohn’s disease in infants and children. J Pedi- children. Gut 43 : 634–638 atr Surg 32 : 1063–1067
    • Part V Liver, Pancreas and Spleen
    • SPRINGER SURGERY ATLAS SERIES Series Editors: J. S. P. Lumley · J. R. Siewert
    • CHAPTER 33 Biliary Atresia Ryoji Ohi, Masaki Nio INTRODUCTIONBiliary atresia still remains one of the most intract- between the intestinal and ductal epithelial elementsable gastrointestinal surgical diseases in infancy. The occurs.introduction of liver transplantation has revolution- Successful hepatic portoenterostomy depends onized the protocols for the treatment of this condition, early diagnosis and operation (preferably before 60but hepatic portoenterostomy, e.g., the Kasai proce- days of age), adequate operative technique, preven-dure, is still the first-choice surgical treatment. tion of post-operative cholangitis, and precise post- The main clinical manifestations of biliary atresia operative management.are persistent jaundice, clay-coloured faeces and hep- Post-operative cholangitis is the most frequentatomegaly. Although symptoms do not show a great and serious post-operative complication, resulting indifference, anatomical findings in the biliary tract fatal septicemia or re-obliteration of hepatic por-vary from case to case. Only about 10 to 20% of the toenterostomy. Although various modified recon-patients with biliary atresia have an extrahepatic bile struction procedures for prevention of cholangitisduct large enough to perform a mucosa-to-mucosa exist, the authors now perform a double-valved (spuranastomosis to the intestine. These correctable-type valve and intussuscepted valve) hepatic portojeju-cases can undergo hepatico-enterostomy. The re- nostomy. The incidence of cholangitis, however, re-maining patients, about 85 to 90%, do not have any mains almost the same as the original Kasai proce-bile duct amenable to a conventional anastomosis. dure using a long Roux-en-Y anastomosis with aFor these non-correctable types of patient, hepatic length of the limb from 50 to 70 cm. As late complica-portoenterostomy should be performed. tions, liver cirrhosis and portal hypertension and/or Hepatic porto-enterostomy was first devised in hypersplenism have been documented. It has been1957 as a corrective operation for patients with a non- shown that both a late operation and complication ofcorrectable type of biliary atresia. The basis of this cholangitis aggravate hepatic fibrosis and induceprocedure is that the intrahepatic bile ducts are pat- portal hypertension.ent in early infancy and minute intrahepatic bile As to pre-operative management, in addition toducts are present in the cone-shaped fibrous tissue, the routine pre-operative care for abdominal surgery,replacing extrahepatic biliary radicles. In hepatic vitamin K, 1~2 mg/kg per day, is usually given for sev-portojejunostomy, the extrahepatic bile ducts, in- eral days before surgery. The bowel is prepared withcluding fibrous remnants at the porta hepatis, are to- tobramycin sulfate and metronidazole orally, at atally removed and bile drainage established by anas- dose of 10 mg/kg per day, each starting 36 h beforetomosis of an intestinal conduit to the transected operation. Early discontinuation of oral feeding andsurface at the porta hepatis. Microscopic biliary enema are properly enforced. Blood is cross-structures at the liver hilus drain bile into the intesti- matched, and pre-operative broad-spectrum antibio-nal conduit and, in time, an autoapproximation tics are administered.
    • Ryoji Ohi, Masaki Nio 358 Figure 33.1 The operation is undertaken under general anaesthe- costal margin to the medial border of the left rectus sia with tracheal intubation. The patient is placed in muscle. Some pediatric surgeons recommend mobi- the supine position. A pillow is put under the back of lizing and completely exposing the liver to allow ade- the patient for better exposure. A laparotomy is per- quate visualization of the porta hepatis. formed through an right subcostal incision from the33 Figure 33.2 A liver biopsy is taken. A catheter is inserted into the A superficial peritoneum on the hepatoduodenal small gallbladder to take a cholangiogram. After the ligament is opened and the anatomy of the involved occlusion of the extrahepatic bile duct is confirmed bile duct and the hepatic arteries is assessed. Dilated by cholangiography, the gallbladder is freed from the lymphatic channels around the hepatoduodenal liga- liver and dissection is advanced along with the cystic ment should be carefully ligated and divided to avoid duct toward the common hepatic duct. The dissec- excessive lymphatic fluid loss. The common bile duct tion is helped by the use of binocular loupes. remnant is carefully dissected, since it is often adher- ent to the surrounding tissues.
    • Chapter 33 Biliary Atresia 359 Figure 33.1 Figure 33.2
    • Ryoji Ohi, Masaki Nio 360 Figure 33.3, 33.4 The common bile duct is clamped and divided adja- The separation of the fibrous remnants from the cent to the duodenum. After the common bile duct is right and left portal veins is carefully advanced pos- severed, it is pulled up and the hepatic duct remnant teriorly. The bifurcation of the right and left portal is freed from underlying hepatic arteries and the veins must be retracted to obtain proper exposure of portal vein. The hepatic duct usually transforms into the porta hepatis. Several small branches bridging a cone-shaped fibrous tissue, which is situated crani- from the portal vein to the fibrous remnants are al to the bifurcation of the portal vein. It continues to identified and divided between ligatures, facilitating the portal tracts in the liver. Even if a cyst-like struc- downward displacement of the portal vein. The pos- ture is present, it should be removed and should not terior aspect of the fibrous remnants is exposed deep be used for anastomosis to the intestine. and wide enough behind the bifurcation of the portal vein.33
    • Chapter 33 Biliary Atresia 361 Figure 33.3 Figure 33.4 Common bile duct Cystic duct Portal vein Stump of common bile duct
    • Ryoji Ohi, Masaki Nio 362 Figure 33.5 The dissection between the anterior aspect of the fi- as widely as possible, i.e., to 5 to 6 mm proximal of brous remnants and the quadrate lobe of the liver is the anterior branch of the right hepatic artery at the also sharply and sufficiently advanced. The dissec- right side and to the umbilical point of the left portal tion of the fibrous remnants must be accomplished vein at the left side.33 Figure 33.6 The transection of the fibrous remnants is carefully duct remnants at the same level. Haemorrhage from carried out by the use of small-sized round-shaped the cut surface of the porta hepatis is occasionally scissors or a sharp knife at the level of the posterior considerable. Irrigation with warm saline stops the surface of the portal vein. Although some surgeons bleeding, usually within 10 min. Ligation or cautery confirm the presence of microscopically patent ducts should not be applied because of the possibility of at the level of anastomosis by using frozen sections accidental obliteration of small bile ducts that may be during surgery, the authors do not use frozen-section opening on the transected surface. guidance because they always transect the portal bile
    • Chapter 33 Biliary Atresia 363 Figure 33.5 Figure 33.6
    • Ryoji Ohi, Masaki Nio 364 Figure 33.7 The next step is the construction of a Roux-en-Y loop rently do not use a cutaneous stoma because of the of the jejunum. A Roux-en-Y anastomosis with the frequent bleeding that occurs when the patient devel- ascending limb, approximately 50 cm in length, is ops portal hypertension, and the technical difficul- made. The end of the gastric limb of the intestine is ties that arise when subsequent liver transplantation anastomosed not to the back, but to the lateral side of may become necessary. Further, there was no reduc- the hepatic limb of the intestine. Most surgeons cur- tion in incidence of cholangitis.33 Figure 33.8, 33.9 A spur valve (A) and an intussuscepted valve (B) are the biliary limb proximal to the spur valve. The vasa made, which may prevent cholangitis. Three centi- recti of the conduit are divided by the width equal to metres of the antimesenteric half of the seromuscu- the intestinal diameter. The seromuscular layer of the lar coat is removed from the biliary limb proximal to devascularized intestine is then removed. The de- the anastomosis. The gastric and biliary limbs are nuded segment and an equivalent length of its proxi- then coapted over the denuded mucosa with sutures mal portion of the intestine is intussuscepted into the along the edges of the incised seromuscular layer. distal segment. This valve with fixed with eight to ten Subsequently, the intussuscepted valve is created in interrupted sutures.
    • Chapter 33 Biliary Atresia 365 Figure 33.7 Figure 33.8 Figure 33.9
    • Ryoji Ohi, Masaki Nio 366 Figure 33.10 After completion of valves, the hepatic limb is Stitches must not be placed in the transected surface brought up retrocolically. The end of the intestine is of the fibrous remnants in which minute bile ducts anastomosed around the transected end of the fi- are present. After all interrupted sutures of the poste- brous remnants at the porta hepatis by full-thickness rior row are placed in position, they are tied. interrupted sutures using 5/0 absorbable sutures.33
    • Chapter 33 Biliary Atresia 367 Figure 33.10
    • Ryoji Ohi, Masaki Nio 368 Figure 33.11, 33.12 The anterior row of suture is made as same as the Oral feeding is usually started on the fifth or sixth posterior row of the anastomosis. Again, it should be post-operative day when bowel activity resumes. Pre- emphasized that stitches must not be placed in the vention of post-operative cholangitis is a main goal transected surface of the fibrous remnants, but of the post-operative management. For this purpose, around the transected area. we routinely use antibiotics, choleretics and steroids Interrupted seromuscular sutures are made using as post-operative medication. For antibiotics, amika- 5/0 silk between the anterior wall of the jejunum and cin sulfate in a dose of 8 mg/kg per day, q8h, is ad- the quadrate lobe of the liver to fix the intestine at the ministered for 7 days after the operation and then porta hepatis. Several stitches are placed between the cephalosporin in a dose of 50–80 mg/kg per day is posterior wall of the jejunum and the hepatoduoden- given intravenously for a few months until the serum al ligament. Irrigation of the abdominal cavity with bilirubin level falls below 2 mg/dl. As choleretics, saline should be done sufficiently. Intestine, especial- intravenous injection of 3 ml 10% dehydrocholic acid ly the jejunum including the biliary limb is carefully diluted with 7 ml 5% glucose is started on the day of placed in order in the abdominal cavity to prevent il- operation and is given every 12 h. Ursodeoxycholic eus. A Penrose drain is placed in the foramen of Win- acid at a dose of 0.5 g/day is also given orally. Pred- slow. After the intestine in the upper abdomen, in- nisolone, 10 mg twice a day intravenously, is started cluding hepatic limb, is covered by Seprafilm, the ab- after the seventh post-operative day. Steroid therapy dominal cavity is closed in layers. is continued intravenously for 4 days and it is Patients are placed in an oxygen tent and intrave- switched to oral administration of 20 mg bid pred- nous fluid is given. nisolone every other day for several months. Decompression of the gastrointestinal tract should be performed by nasogastric aspiration and enema.33
    • Chapter 33 Biliary Atresia 369 Figure 33.11 Figure 33.12
    • Ryoji Ohi, Masaki Nio 370 CONCLUSION Between 1953 and 2001, a total of 321 patients with bil- The authors’ current strategy for surgical treat- iary atresia underwent surgery in our hospital. ment for patients with this disease include (1) early Among them, 43 patients underwent double-valved diagnosis, including prenatal diagnosis, (2) hepatic hepatic portoenterostomy (the valved procedure). portoenterostomy without stoma formation, (3) close Bile drainage after surgery was obtained in 95% of postoperative care, especially for prevention of post- the cases. The incidence of post-operative cholangitis operative cholangitis, (4) revision of hepatic por- was 56% in the valved procedure. Of the cases treated toenterosotmy only in selected cases who showed by this procedure, 49% are still alive without jaundice good bile drainage after the first procedure, (5) early after hepatic portoenterostomy alone, and 30% are liver transplantation in patients with absolutely alive after subsequent liver transplantation. failed hepatic portoenterostomy, (6) avoidance of The combination of hepatic portoenterostomy laparotomy for the treatment of oesophageal varices with subsequent liver transplantation is the treat- and hypersplenism (injection sclerotherpy and ment of choice for patients with biliary atresia. It is splenic artery embolization), and (7) consideration important, however, to attempt to keep the patient’s of exploratory laparotomy or primary liver trans- own organ by continuing efforts to achieve the best plantation for patients with advanced liver disease at possible results with hepatic portoenterostomy. the time of referral. SELECTED BIBLIOGRAPHY Kasai M (1974) Treatment of biliary atresia with special refer- Ohi R (2000) Biliary atresia; a surgical perspective. Clin Liver ence to hepatic porto-enterostomy and its modifications. Dis 4 : 779–804 Prog Pediatr Surg 6 : 5–52 Ohi R, Nio M (1998) The jaundiced infant: biliary a