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
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.
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
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.
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.
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.
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
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
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
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.
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.
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
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.
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.
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
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