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SECONDARY SHORT ESOPHAGUS DUE TO SECONDARY SHORT ESOPHAGUS DUE TO PROLONGED REFLUX: STILL A PROBLEM?PROLONGED REFLUX: STILL A PROBLEM?

Ognyan BrankovOgnyan BrankovUniversity Department of Pediatric Surgery University Department of Pediatric Surgery Hospital “Pirogov” – Sofia, BulgariaHospital “Pirogov” – Sofia, Bulgaria

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BackgroundBackground

The The problem problem of the short esophagus in antireflux of the short esophagus in antireflux

surgery is surgery is widely widely discussed for discussed for manymany years. The years. The

association between severe esophagitis, strictureassociation between severe esophagitis, stricture

formation, and esophageal shortening is supportedformation, and esophageal shortening is supported by by

many many previously publishedpreviously published studies. studies.

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BackgroundBackground

Acquired esophageal Acquired esophageal shortening most commonly occurs shortening 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 include include giant hiatalgiant hiatal hernias hernias in the newborn in the newborn, Barrett’s , Barrett’s esophagusesophagus, caustic ingestion, which can result in a , caustic ingestion, which can result in a profoundprofound inflammatory reaction inflammatory reaction and and subsequentsubsequent fibrosisfibrosis with significant cephalad displacementwith significant cephalad displacement of the of the gastroesophageal junction.gastroesophageal junction.

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The purpose of The purpose of our presentationour presentation is to discuss the is to discuss the pathogenesis pathogenesis of the shortof the short esophagus, to review the history esophagus, to review the history of treatment, and to of treatment, and to present the challenges which might present 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 we In our initial experience with laparoscopic surgery we faced the problem and have been forced twice to convert to faced the problem and have been forced twice to convert to open surgery because of refractory shortening and fibrotic open surgery because of refractory shortening and fibrotic periesophageal adhesions into the mediastinum. periesophageal adhesions into the mediastinum.

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J.Alvin MerendinoDisplacement 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 the 2. Normal esophagus; the stomach herniates about the esophagus into theesophagus into the hernial sac. However, the esophagus hernial sac. However, the esophagus remains in its normal situation andremains in its normal situation and does not occupy a does not occupy a position in the sac (para-esophageal). position in the sac (para-esophageal).

3. Normal esophagus; the stomach herniates through the 3. Normal esophagus; the stomach herniates through the esophageal hiatusesophageal hiatus pushing the esophagus ahead of it into pushing the esophagus ahead of it into the sac. the sac.

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K.Alvin Merendino I

Displacement of the esophagus into a new diaphragmaticorifice 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

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K.Alvin Merendino II

Abdominal ApproachAbdominal Approach

The ventral esophageal displacement provides an extension of the intraabdominal portion.

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K. Alvin Merendino, D.H. DillardThe Concept of Sphincter Substitution by an Interposed JejunalSegment for Anatomic and Physiologic Abnormalities atthe Esophagogastric Junction Ann Surg, 1955,Vol 142, 3, 486-507

1955 Merendino 1955 Merendino developed developed experimentally and clinically texperimentally and clinically the he concept of cardiac sphincter concept of cardiac sphincter substitutionsubstitution with interposition of with interposition of pedicled jejunal patch pedicled jejunal patch for certain for certain clinical conditions.clinical conditions.A tentative working classificationA tentative working classification has has evolved where this procedureevolved where this procedure might might have merithave merit::

Physiological DisordersPhysiological DisordersI. Cardiac sphincter relaxation.I. Cardiac sphincter relaxation. A. Reflux esophagitis with complicationsA. Reflux esophagitis with complications B. Congenitally short esophagusB. Congenitally short esophagus

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J. Leigh Collis from Queen Elizabeth Hospital, BirminghanAn 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 short short oesophagus presents a problemoesophagus presents a problem,, for which for which there is there 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 problem oesophagus makes the problem unsuitable forunsuitable for treatment by the standard operations for hiatustreatment by the standard operations for hiatushernia, hernia, while some of the suggested treatments,such as oesophago-jejunostomy, are so formidable, are so formidablethat they are unsuitable for the frail and oftenthat they are unsuitable for the frail and oftenaged subjects.aged subjects.

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““Collis 1” procedureCollis 1” procedure

Downward positioning of the GE junction in order to restore Downward positioning of the GE junction in order to restore the the acute acute angle of Hisangle of His..

Thorax (1957), 12, 181

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J. Leigh Collis Gastroplasty Thorax (1961), 16, 197

““Collis II” procedureCollis II” procedure

The classic Collis procedure – The classic Collis procedure – achieving a sufficient abdominal achieving a sufficient abdominal esophageal length.esophageal length.

The The goalgoal is is a a surgical reflux surgical reflux control.control. T The main part of he main part of the the stomach will bestomach will be s seen to enter een to enter well below the diaphragm andwell below the diaphragm and a at t an acute anglean acute angle..

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But But Collis did not perform aCollis did not perform a fundoplication because it fundoplication because it was believed at that time thatwas believed at that time that intraabdominal reduction intraabdominal reduction of the GEJ and recreation of theof the GEJ and recreation of the acute angle of His was acute angle of His was effective as an antireflux barrier.effective as an antireflux barrier.

TThe Collis gastroplasty alone, without ahe Collis gastroplasty alone, without awrap, did not control refluxwrap, did not control reflux (Adler RH, 1990) (Adler RH, 1990)..

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In order to ensure a antireflux barier after performing the In order to ensure a antireflux barier after performing the

esophageal lengthening procedure a Nissen fundoplication esophageal lengthening procedure a Nissen fundoplication wrap is made – the so called cwrap is made – the so called combined Collis-Nissen ombined Collis-Nissen reconstruction of the esophagogastric junction. reconstruction of the esophagogastric junction.

Orringer MB, Sloan H, Ann Thorac Surg. 1978;25(1):16-21.

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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 fundoplicationJ Pediatr Surg 1997; 32:887– 891.

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

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Laparoscopic Collis techniqueLaparoscopic Collis technique

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Our clinical data (1990 – 2009)Our clinical data (1990 – 2009)

For a period of 20 years a total 171 children For a period of 20 years a total 171 children were operated on for different pathological were operated on for different pathological condition: 138 for GERD, 12 for congenital condition: 138 for GERD, 12 for congenital hiatus hernia and 21 children for secondary hiatus hernia and 21 children for secondary reflux following the prolonged dilatation reflux following the prolonged dilatation treatment for lye stricturetreatment for lye stricture..

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Clinical dataClinical data

In 27 of all the children we diagnosed a In 27 of all the children we diagnosed a secondary short esophagus which was treated secondary 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 yearsMale - 16 Female - 11 Male - 16 Female - 11

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Clinical dataClinical data

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

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Clinical investigationClinical investigation

The diagnosis was carried out by barium The diagnosis was carried out by barium esophagogram, endoscopy, 24-h pH esophagogram, endoscopy, 24-h pH monitoring and radionuclide studies. monitoring and radionuclide studies.

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ResultsResults

All endoscopic examinations showed mucosal All endoscopic examinations showed mucosal erosion at the level of the fibrous stricture erosion at the level of the fibrous stricture impassable for the scope.impassable for the scope.

During barium study a wide open cardia is During barium study a wide open cardia is demonstrated, with an obtuse angle of Hiss. A demonstrated, with an obtuse angle of Hiss. A marked hiatal hernia is present and regurgitation marked hiatal hernia is present and regurgitation of the contrast material is demonstrated. of the contrast material is demonstrated.

The 24-hour pH-monitoring showed prolonged The 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

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According to the classification of K.Horvath we divide According to the classification of K.Horvath we divide our cases as follow:our cases as follow:

TTrue, nonreduciblerue, nonreducible short short esophagusesophagus (GERD - 3) (GERD - 3)

Transthoracic fundoplication (2), Transthoracic fundoplication (2), uncut Collis gastroplasty (1)uncut Collis gastroplasty (1)

TTrue but reducible short rue 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 stricture and 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

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Surgical procedures in 27 childrenSurgical procedures in 27 children

Abdominal Nissen fundoplication n = 21Abdominal Nissen fundoplication n = 21 LLaparoaparo--thoracothoracotomy with intrathoracic esophago–gastro tomy with intrathoracic esophago–gastro anastomosis anastomosis n = 3 n = 3 Transthoracic esophago–gastro anastomosisTransthoracic esophago–gastro anastomosis n = 2 n = 2 Uncut Collis gastroplasty n = 1Uncut Collis gastroplasty n = 1

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Transabdominal Merendino procedureTransabdominal Merendino procedure

Standart Nissen fundoplication was performed in 21 cases. In Standart Nissen fundoplication was performed in 21 cases. In order to ensure a longer intraabdominal esophageal portion we order to ensure a longer intraabdominal esophageal portion we adapt the transthoracic procedure of Merendino. After deep adapt the transthoracic procedure of Merendino. After deep mediastinal dissection of the esophagus we incise the hiatus mediastinal 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.

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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

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Right thoracotomy a. intrathoracic anastomosisRight thoracotomy a. intrathoracic anastomosis

Right thoracotomy, stricture resection, fundoplication. After resection of Right 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.

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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, relaparotomy Right thoracotomy, reposition, relaparotomy

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Secondary brachiesophagus due to lye corrosionSecondary brachiesophagus due to lye corrosion

Persistent gastro-esophageal reflux was found in 9 Persistent gastro-esophageal reflux was found in 9 children. They showed symptoms of progressive children. They showed symptoms of progressive dysphagia related to increasing stenosis of the dysphagia related to increasing stenosis of the esophageal lumen. During prolonged dilatation esophageal lumen. During prolonged dilatation treatment of severe corrosive esophagitis in children, treatment of severe corrosive esophagitis in children, an inflammatory shortening of the esophagus may an inflammatory shortening of the esophagus may lead to secondary GER. This shortening of the muscle lead to secondary GER. This shortening of the muscle wall may cause incompetence of the cardia and a wall may cause incompetence of the cardia and a hiatal hernia.hiatal hernia.

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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 refluxII dgr – small fixed hiatal hernia, moderate refluxII dgr – small fixed hiatal hernia, moderate refluxIII 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

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Postoperative complications – 9 (33 %)Postoperative complications – 9 (33 %)

Recurrence - 6 Recurrence - 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 Esophagocoloplasty Esophagocoloplasty – 4 children– 4 children

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Additionally four children with lye stricture Additionally four children with lye stricture required a second required a second stage stage esophagealesophageal replacement replacement due to irreversible changes and due to irreversible changes and persistent stricture despite antireflux procedure. persistent stricture despite antireflux procedure.

Coloplasty n = 3Coloplasty n = 3

Gastroplasty n = 1Gastroplasty n = 1

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ConclusionsConclusions

Esophageal shortening as a complication of Esophageal shortening as a complication of advanced gastroesophageal reflux disease is advanced gastroesophageal reflux disease is seen in 2seen in 2 -- 44 % of patients with GERD. For % of patients with GERD. For such patients undergoing laparoscopic such patients undergoing laparoscopic antireflux surgery, the procedure antireflux surgery, the procedure hold the risk hold the risk of recurrence due toof recurrence due to excessive tension excessive tension..

Kleinmann E, Halbfass HJKleinmann E, Halbfass HJ Zur Problematik des „short Zur Problematik des „short esophagus“ in der laparoskopischen Antirefluxchirurgie esophagus“ in der laparoskopischen Antirefluxchirurgie Der Der Chirurg. 2001 Apr;Chirurg. 2001 Apr; 7272 (4):408-13(4):408-13

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Most of cases with Most of cases with short esophagusshort esophagus can be can be appropriatelyappropriately managed with extensive mediastinal managed with extensive mediastinal mobilization of themobilization of the esophagus to achieve the esophagus to achieve the required intraabdominal esophageal length to required intraabdominal esophageal length to perform a wrap. perform a wrap.

TheThe remaining require remaining require different different aggressive aggressive surgical surgical approaches approaches to create an adequateto create an adequate antireflux valve mechanism at the gastro-antireflux valve mechanism at the gastro-esophageal junction. esophageal junction.

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Because Because a a short esophagus is uncommon, short esophagus is uncommon, a a laparoscopic laparoscopic surgeons surgeons should beshould be familiar with familiar with its diagnosis andits diagnosis and management. A complete management. A complete understanding of thunderstanding of this entity is entity and methods for and methods for surgical correction are surgical correction are needed needed to avoidto avoid typical typical postoperative complicationspostoperative complications..