Presented on 10th DANUBE SYMPOSIUM PEDIATRIC SURGERY November 26–28, 2010 VIENNA, AUSTRIA Background Acquired shortening of the esophagus may occurs in children with mistreated gastroesophageal reflux which results in a profound inflammatory reaction with subsequent fibrosis and significant cephalad displacement of the cardia. Our purpose is to discuss the pathogenesis of the short esophagus, to review the history of treatment and to present our own experience in open surgical procedures. Methods For a period of 20 years (1990 - 2009) 171 children were operated on: 138 for GERD, 12 for congenital hiatus hernia and 21 for secondary reflux following the prolonged dilatation treatment for lye stricture. In 27 children we diagnosed a secondary short esophagus. According to K.Horvath we divide our cases as follow: 1.True, nonreducible short esophagus (3) 2. True but reducible short esophagus (mistreated GERD – 11, lye stricture – 7) 3.Apparent short esophagus (6). Results Standart Nissen fundoplication was performed in 21 cases. In order to ensure a longer intraabdominal esophageal portion we adapt the transthoracic procedure of Merendino. After deep mediastinal dissection of the esophagus we incise the hiatus arch about 3 cm and positioned the esophagus anteriorly. In three cases the standard laparotomy was converted in a thoraco-laparotomy because of severe changes in the distal esophagus and an intrathoracic Nissen procedure was accomplished. In two cases we performed a transthoracic fundoplication. In one case the uncut Collis gastroplasty was applied. There was no postoperative death. Additionally four children with lye stricture required a second stage colon replacement due to irreversible changes. Conclusions In our series the incidence of the short esophagus represent 15,8 % of patients undergoing antireflux surgery. Most of them can be appropriately managed with extensive mediastinal mobilization of the esophagus to achieve the required intraabdominal esophageal length to perform a wrap. The remaining require different aggressive surgical approaches to create an adequate antireflux valve mechanism at the gastro-esophageal junction.

1. 1
SECONDARY SHORT ESOPHAGUS DUE TOSECONDARY SHORT ESOPHAGUS DUE TO
PROLONGED REFLUX: STILLA PROBLEM?PROLONGED REFLUX: STILLA PROBLEM?
Ognyan BrankovOgnyan Brankov
University Department of Pediatric SurgeryUniversity Department of Pediatric Surgery
Hospital “Pirogov” – Sofia, BulgariaHospital “Pirogov” – Sofia, Bulgaria

2. 2
BackgroundBackground
TheThe problemproblem of the short esophagus in antirefluxof the short esophagus in antireflux
surgery issurgery is widelywidely discussed fordiscussed for manymany years. Theyears. The
association between severe esophagitis, strictureassociation between severe esophagitis, stricture
formation, and esophageal shortening is supportedformation, and esophageal shortening is supported byby
manymany previously publishedpreviously published studies.studies.

3. 3
BackgroundBackground
Acquired esophagealAcquired esophageal shortening most commonly occursshortening most commonly occurs
in patientsin patients with chronic gastroesophageal refluxwith chronic gastroesophageal reflux disease.disease.
Other entities associated with esophageal shorteningOther entities associated with esophageal shortening
includeinclude giant hiatalgiant hiatal herniashernias in the newbornin the newborn, Barrett’s, Barrett’s
esophagusesophagus, caustic ingestion, which can result in a, caustic ingestion, which can result in a
profoundprofound inflammatory reactioninflammatory reaction andand subsequentsubsequent fibrosisfibrosis
with significant cephalad displacementwith significant cephalad displacement of theof the
gastroesophageal junction.gastroesophageal junction.

4. 4
The purpose ofThe purpose of our presentationour presentation is to discuss theis to discuss the
pathogenesispathogenesis of the shortof the short esophagus, to review the historyesophagus, to review the history
of treatment, and toof treatment, and to present the challenges which mightpresent the challenges which might
arise while performing a laparoscopic surgical procedures.arise while performing a laparoscopic surgical procedures.
In our initial experience with laparoscopic surgery weIn our initial experience with laparoscopic surgery we
faced the problem and have been forced twice to convert tofaced the problem and have been forced twice to convert to
open surgery because of refractory shortening and fibroticopen surgery because of refractory shortening and fibrotic
periesophageal adhesions into the mediastinum.periesophageal adhesions into the mediastinum.

5. 5
J.Alvin Merendino
Displacement of the esophagus into a new diaphragmatic orifice in the repair of para-
esophageal and esophageal hiatus hernia.
Annals of Surgery, 129,2,1949,185-198
There are three main types of esophageal hiatus hernia:There are three main types of esophageal hiatus hernia:
11. Short esophagus with "thoracic" stomach (rare). Short esophagus with "thoracic" stomach (rare)
2. Normal esophagus; the stomach herniates about the2. Normal esophagus; the stomach herniates about the
esophagus into theesophagus into the hernial sac. However, the esophagushernial sac. However, the esophagus
remains in its normal situation andremains in its normal situation and does not occupy adoes not occupy a
position in the sac (para-esophageal).position in the sac (para-esophageal).
3. Normal esophagus; the stomach herniates through the3. Normal esophagus; the stomach herniates through the
esophageal hiatusesophageal hiatus pushing the esophagus ahead of it intopushing the esophagus ahead of it into
the sac.the sac.

6. 6
K.Alvin Merendino I
Displacement of the esophagus into a new diaphragmatic
orifice in the repair of para-esophageal and esophageal
hiatus hernia. Ann Surgery, 129,2,1949,185-198
Transthoracic displacement of the esophagusTransthoracic displacement of the esophagus

7. 7
K.Alvin Merendino II
Abdominal ApproachAbdominal Approach
The ventral esophageal
displacement provides an
extension of the intraabdominal
portion.

8. 8
K. Alvin Merendino, D.H. Dillard
The Concept of Sphincter Substitution by an Interposed Jejunal
Segment for Anatomic and Physiologic Abnormalities at
the Esophagogastric Junction Ann Surg, 1955,Vol 142, 3, 486-507
1955 Merendino1955 Merendino developeddeveloped
experimentally and clinically texperimentally and clinically thehe
concept of cardiac sphincterconcept of cardiac sphincter
substitutionsubstitution with interposition ofwith interposition of
pedicled jejunal patchpedicled jejunal patch for certainfor certain
clinical conditions.clinical conditions.
A tentative working classificationA tentative working classification hashas
evolved where this procedureevolved where this procedure mightmight
have merithave merit::
Physiological DisordersPhysiological Disorders
I. Cardiac sphincter relaxation.I. Cardiac sphincter relaxation.
A. Reflux esophagitis with complicationsA. Reflux esophagitis with complications
B. Congenitally short esophagusB. Congenitally short esophagus

9. 9
J. Leigh Collis from Queen Elizabeth Hospital, Birminghan
An Operation for Hiatus Hernia with short Oesophagus Thorax (1957), 12, 181
The patient with a hiatus hernia and a markedlyThe patient with a hiatus hernia and a markedly shortshort
oesophagus presents a problemoesophagus presents a problem,, for whichfor which there isthere is
not at present a generally accepted line ofnot at present a generally accepted line of treatment.treatment.
The shortening of theThe shortening of the oesophagus makes the problemoesophagus makes the problem
unsuitable forunsuitable for treatment by the standard operations for hiatustreatment by the standard operations for hiatus
hernia,hernia, while some of the suggested treatments,
such as oesophago-jejunostomy, are so formidable, are so formidable
that they are unsuitable for the frail and oftenthat they are unsuitable for the frail and often
aged subjects.aged subjects.

10. 10
““Collis 1” procedureCollis 1” procedure
Downward positioning of the GE junction in order to restoreDownward positioning of the GE junction in order to restore thethe acuteacute
angle of Hisangle of His..
Thorax (1957), 12, 181

11. 11
J. Leigh Collis Gastroplasty Thorax (1961), 16, 197
““Collis II” procedureCollis II” procedure
The classic Collis procedure –The classic Collis procedure –
achieving a sufficient abdominalachieving a sufficient abdominal
esophageal length.esophageal length.
TheThe goalgoal isis aa surgical refluxsurgical reflux
control.control. TThe main part ofhe main part of thethe
stomach will bestomach will be sseen to entereen to enter
well below the diaphragm andwell below the diaphragm and aatt
an acute anglean acute angle..

12. 12
ButBut Collis did not perform aCollis did not perform a fundoplication because itfundoplication because it
was believed at that time thatwas believed at that time that intraabdominal reductionintraabdominal reduction
of the GEJ and recreation of theof the GEJ and recreation of the acute angle of His wasacute angle of His was
effective as an antireflux barrier.effective as an antireflux barrier.
TThe Collis gastroplasty alone, without ahe Collis gastroplasty alone, without a
wrap, did not control refluxwrap, did not control reflux (Adler RH, 1990)(Adler RH, 1990)..

13. 13
In order to ensure a antireflux barier after performing theIn order to ensure a antireflux barier after performing the
esophageal lengthening procedure a Nissen fundoplicationesophageal lengthening procedure a Nissen fundoplication
wrap is made – the so called cwrap is made – the so called combined Collis-Nissenombined Collis-Nissen
reconstruction of the esophagogastric junction.reconstruction of the esophagogastric junction.
Orringer MB, Sloan H, Ann Thorac Surg.
1978;25(1):16-21.

14. 14
Next stepp – the “Stapler” Collis – cut and uncutNext stepp – the “Stapler” Collis – cut and uncut
Cameron BH, Cochran WJ, McGill CW.
The uncut Collis-Nissen fundoplication
J Pediatr Surg 1997; 32:887– 891.

15. 15
Steichen FM. Abdominal approach to the Collis gastroplasty and
Nissen fundoplication. Surg Gynecol Obstet 1986; 162:372–374

16. 16
Laparoscopic Collis techniqueLaparoscopic Collis technique

17. 17
Our clinical data (1990 – 2009)Our clinical data (1990 – 2009)
For a period of 20 years a total 171 childrenFor a period of 20 years a total 171 children
were operated on for different pathologicalwere operated on for different pathological
condition: 138 for GERD, 12 for congenitalcondition: 138 for GERD, 12 for congenital
hiatus hernia and 21 children for secondaryhiatus hernia and 21 children for secondary
reflux following the prolonged dilatationreflux following the prolonged dilatation
treatment for lye stricturetreatment for lye stricture..

18. 18
Clinical dataClinical data
In 27 of all the children we diagnosed aIn 27 of all the children we diagnosed a
secondary short esophagus which was treatedsecondary short esophagus which was treated
by means of different surgical procedures.by means of different surgical procedures.
Age – between 4 and 9 yearsAge – between 4 and 9 years
Male - 16 Female - 11Male - 16 Female - 11

19. 19
Clinical dataClinical data
GERD with fibrous esophageal stricture n = 17GERD with fibrous esophageal stricture n = 17
Secondary brachiesophagus due to lye corrosion n = 7Secondary brachiesophagus due to lye corrosion n = 7
Neonatal thoracic stomach (Thoraxmagen) n = 3Neonatal thoracic stomach (Thoraxmagen) n = 3

20. 20
Clinical investigationClinical investigation
The diagnosis was carried out by bariumThe diagnosis was carried out by barium
esophagogram, endoscopy, 24-h pHesophagogram, endoscopy, 24-h pH
monitoring and radionuclide studies.monitoring and radionuclide studies.

21. 21
ResultsResults
All endoscopic examinations showed mucosalAll endoscopic examinations showed mucosal
erosion at the level of the fibrous strictureerosion at the level of the fibrous stricture
impassable for the scope.impassable for the scope.
During barium study a wide open cardia isDuring barium study a wide open cardia is
demonstrated, with an obtuse angle of Hiss. Ademonstrated, with an obtuse angle of Hiss. A
marked hiatal hernia is present and regurgitationmarked hiatal hernia is present and regurgitation
of the contrast material is demonstrated.of the contrast material is demonstrated.
The 24-hour pH-monitoring showed prolongedThe 24-hour pH-monitoring showed prolonged
acid reflux with a reflux-index of 18 to 67 %acid reflux with a reflux-index of 18 to 67 %
The reflux scintigraphy confirmed the diagnosisThe reflux scintigraphy confirmed the diagnosis

22. 22
According to the classification of K.Horvath we divideAccording to the classification of K.Horvath we divide
our cases as follow:our cases as follow:
TTrue, nonreduciblerue, nonreducible shortshort
esophagusesophagus (GERD - 3)(GERD - 3)
Transthoracic fundoplication (2),Transthoracic fundoplication (2),
uncut Collis gastroplasty (1)uncut Collis gastroplasty (1)
TTrue but reducible shortrue but reducible short
esophagusesophagus (mistreated GERD –(mistreated GERD –
11, lye stricture – 7)11, lye stricture – 7)
DDeep mediastinal dissectioneep mediastinal dissection
““MerendinoMerendino” (18)” (18)
AApparent short esophaguspparent short esophagus
(GERD – 3, HH - 3)(GERD – 3, HH - 3)..
TThoraco-laparotomyhoraco-laparotomy and strictureand stricture
resection (3), “resection (3), “MerendinoMerendino” (3)” (3) K. D. Horvath, Lee L. Swanstrom, B. A. Jobe,
The Short Esophagus Ann Surg (2000) Vol.
232, No. 5, 630–640

23. 23
Surgical procedures in 27 childrenSurgical procedures in 27 children
Abdominal Nissen fundoplication n = 21Abdominal Nissen fundoplication n = 21
LLaparoaparo--thoracothoracotomy with intrathoracic esophago–gastrotomy with intrathoracic esophago–gastro
anastomosisanastomosis n = 3n = 3
Transthoracic esophago–gastro anastomosisTransthoracic esophago–gastro anastomosis n = 2n = 2
Uncut Collis gastroplasty n = 1Uncut Collis gastroplasty n = 1

24. 24
Transabdominal Merendino procedureTransabdominal Merendino procedure
Standart Nissen fundoplication was performed in 21 cases. InStandart Nissen fundoplication was performed in 21 cases. In
order to ensure a longer intraabdominal esophageal portion weorder to ensure a longer intraabdominal esophageal portion we
adapt the transthoracic procedure of Merendino. After deepadapt the transthoracic procedure of Merendino. After deep
mediastinal dissection of the esophagus we incise the hiatusmediastinal dissection of the esophagus we incise the hiatus
arch about 3 cm and positioned the esophagus anteriorly.arch about 3 cm and positioned the esophagus anteriorly.

25. 25
LLaparoaparo--thoracothoracotomy a. intrathoracic anastomosistomy a. intrathoracic anastomosis
Three children with refractory stricturesThree children with refractory strictures
Underestimate periesophageal changesUnderestimate periesophageal changes
Extremely shortening of the esophagusExtremely shortening of the esophagus

26. 26
Right thoracotomy a. intrathoracic anastomosisRight thoracotomy a. intrathoracic anastomosis
Right thoracotomy, stricture resection, fundoplication. After resection ofRight thoracotomy, stricture resection, fundoplication. After resection of
the stricture we perform a partial fundoplasty, similar to the “the stricture we perform a partial fundoplasty, similar to the “InkwellInkwell
esophagogastrostomyesophagogastrostomy” procedure in the meaning of P.” procedure in the meaning of P. OttosenOttosen..
Ottosen, P.a.al: Acta Chir.
Scand., 117: l, 1959.

27. 27
Neonatal thoracic stomach (Thoraxmagen)Neonatal thoracic stomach (Thoraxmagen)
Total herniation – 2 childrenTotal herniation – 2 children
Partial herniation – 1 childPartial herniation – 1 child
Surgery – Laparotomy, Nissen n = 2Surgery – Laparotomy, Nissen n = 2
Right thoracotomy, reposition, relaparotomyRight thoracotomy, reposition, relaparotomy

28. 28
Secondary brachiesophagus due to lye corrosionSecondary brachiesophagus due to lye corrosion
Persistent gastro-esophageal reflux was found in 9Persistent gastro-esophageal reflux was found in 9
children. They showed symptoms of progressivechildren. They showed symptoms of progressive
dysphagia related to increasing stenosis of thedysphagia related to increasing stenosis of the
esophageal lumen. During prolonged dilatationesophageal lumen. During prolonged dilatation
treatment of severe corrosive esophagitis in children,treatment of severe corrosive esophagitis in children,
an inflammatory shortening of the esophagus mayan inflammatory shortening of the esophagus may
lead to secondary GER. This shortening of the musclelead to secondary GER. This shortening of the muscle
wall may cause incompetence of the cardia and awall may cause incompetence of the cardia and a
hiatal hernia.hiatal hernia.

29. 29
We have defined three grades of secondary brachiesophagus:We have defined three grades of secondary brachiesophagus:
I dgr – obtuse angle of Hiss, insignificant refluxI dgr – obtuse angle of Hiss, insignificant reflux
II dgr – small fixed hiatal hernia, moderate refluxII dgr – small fixed hiatal hernia, moderate reflux
III dgr – marked HH, deteriorated esophagus, expressive refluxIII dgr – marked HH, deteriorated esophagus, expressive reflux
Grade II and III are indication for antireflux plastyGrade II and III are indication for antireflux plasty

30. 30
Postoperative complications – 9 (33 %)Postoperative complications – 9 (33 %)
Recurrence - 6Recurrence - 6 (1 HH, 1 GERD, 4 lye stricture)(1 HH, 1 GERD, 4 lye stricture)
Paraesophageal hernia – 2Paraesophageal hernia – 2
Slipped Nissen – 1Slipped Nissen – 1
Mediastinal abscess after extensive mediastinal mobilisation 1Mediastinal abscess after extensive mediastinal mobilisation 1
Redo SurgeryRedo Surgery – 4 children– 4 children EsophagocoloplastyEsophagocoloplasty – 4 children– 4 children

31. 31
Additionally four children with lye strictureAdditionally four children with lye stricture required a secondrequired a second
stagestage esophagealesophageal replacementreplacement due to irreversible changes anddue to irreversible changes and
persistent stricture despite antireflux procedure.persistent stricture despite antireflux procedure.
Coloplasty n = 3Coloplasty n = 3
Gastroplasty n = 1Gastroplasty n = 1

32. 32
ConclusionsConclusions
Esophageal shortening as a complication ofEsophageal shortening as a complication of
advanced gastroesophageal reflux disease isadvanced gastroesophageal reflux disease is
seen in 2seen in 2 -- 44 % of patients with GERD. For% of patients with GERD. For
such patients undergoing laparoscopicsuch patients undergoing laparoscopic
antireflux surgery, the procedureantireflux surgery, the procedure hold the riskhold the risk
of recurrence due toof recurrence due to excessive tensionexcessive tension..
Kleinmann E, Halbfass HJKleinmann E, Halbfass HJ Zur Problematik des „shortZur Problematik des „short
esophagus“ in der laparoskopischen Antirefluxchirurgieesophagus“ in der laparoskopischen Antirefluxchirurgie
DerDer Chirurg. 2001 Apr;Chirurg. 2001 Apr; 7272 (4):408-13(4):408-13

33. 33
Most of cases withMost of cases with short esophagusshort esophagus can becan be
appropriatelyappropriately managed with extensive mediastinalmanaged with extensive mediastinal
mobilization of themobilization of the esophagus to achieve theesophagus to achieve the
required intraabdominal esophageal length torequired intraabdominal esophageal length to
perform a wrap.perform a wrap.
TheThe remaining requireremaining require differentdifferent aggressiveaggressive
surgicalsurgical approachesapproaches to create an adequateto create an adequate
antireflux valve mechanism at the gastro-antireflux valve mechanism at the gastro-
esophageal junction.esophageal junction.

34. 34
BecauseBecause aa short esophagus is uncommon,short esophagus is uncommon, aa
laparoscopiclaparoscopic surgeonssurgeons should beshould be familiar withfamiliar with
its diagnosis andits diagnosis and management. A completemanagement. A complete
understanding of thunderstanding of this entityis entity and methods forand methods for
surgical correction aresurgical correction are neededneeded to avoidto avoid typicaltypical
postoperative complicationspostoperative complications..