Application of the principles of myotomy and strictureplasty for treatment of esophageal strictures

Background/Purpose: Strictures of the esophagus in the pediatric population may be congenital or acquired. Regardless of the etiology, the treatment goal is to relieve the symptoms and allow patients to eat normally. The cornerstone of nonoperative management is repeated esophageal dilatations. However, when nonoperative management fails, operative intervention becomes necessary. In this report the authors present 4 cases of severe upper esophageal strictures managed by applying the principles of myotomy and strictureplasty. Methods: Retrospective review of 4 patients undergoing esophageal stricturotomy from January 1, 1993 to January 1, 2000 was conducted at Childrens Hospital Los Angeles, with a mean follow-up period of 5 years. Results: Three of the 4 patients reported in this report are doing well and have not required any further surgical intervention. One patient in whom additional strictures developed has had a microvascularized free jejunal graft and also is doing well. Conclusions: Currently accepted surgical management of strictures includes resection of the affected segment with end-to-end anastomosis or esophageal replacement. The authors propose that before embarking on a long and technically hazardous operation, consideration should be given to stricturotomy. In cases of isolated strictures, this may be the preferred approach.     

Strictureplasty: An alternative approach in long segment bowel stenosis Crohn's disease

Background/Purpose: Intestinal resection is the most frequent surgical procedure for bowel stenoses in Crohn[apos ]s disease (CD). Recurrence of strictures, particularly with ileocolonic disease, often requires resection of lengthy segments of bowel, potentially resulting in short bowel syndrome. Different techniques of strictureplasty, such as those described by Mikulicz, Finney and Michelassi, are used in adults. However, these procedures are uncommon in pediatric surgery. The authors report their experience with different techniques of strictureplasty and with their modified Michelassi technique for the surgical treatment of long intestinal strictures caused by CD. Methods: Five adolescents (2 boys; 3 girls; mean age, 16 age; range, 14 to 20 years) with severe ileocolonic stenoses and intestinal obstruction, not responsive to medical and nutritional therapy, were treated with different strictureplasty techniques. In 3 of them the modified side-to-side Michelassi technique was used. Results: No postoperative complications occurred. After a mean follow-up of 20.5 months (range, 6 to 28 months), patients are free of symptoms with good nutritional status and off steroid therapy. Conclusions: Strictureplasty is a good and effective surgical option for sparing bowel length in CD patients with extensive intestinal strictures. J Pediatr Surg 38:814-818. [copy ] 2003 Elsevier Inc. All rights reserved.     

The application of vascular technology to esophageal and airway strictures

Background/Purpose: Strictures of the esophagus and airway tract can be dilated if the strictures can be traversed and dilators passed. Unfortunately, using standard methods, not all strictures can be traversed. The authors set out to find a safe, expeditious, and reproducible way to traverse otherwise impassable strictures of the esophagus and airway. Methods: Eight patients (n = 8), over a 2-year period, with strictures were entered prospectively into the study. One patient (n = 1) had a main stem bronchial stricture, and 7 patients (n = 7) had esophageal strictures from the following etiologies: esophageal atresia/tracheoesophageal fistula (EA/TEF) repair, Lye ingestion (n = 2), EA/TEF with gastroesophageal reflux, esophageal atresia without fistula, lye ingestion with colon interposition (n = 2), and iron pill inhalation lodged in left main bronchus. None of the strictures could be passed with conventional maneuvers or instrumentation including endoscopy, guide wires, Fogarty catheters, and filliform and followers. Results of barium studies showed no flow into the stomach. In the bronchial case, no lumen could be identified at bronchoscopy. Results: Utilizing the [ldquo ]Vascular Surgery Glidewire/Berenstein Catheter System[rdquo ] under fluoroscopy and utilizing the [ldquo ]spinning top[rdquo ] dynamic maneuver intrinsic to this system, all of the strictures were traversed easily. The passage of the wire/catheter system thus allowed sequential dilation of the previously impassable strictures. The mean time to cross the strictures with the wire/catheter system was 1 minute, 10 seconds. (t = 70 seconds). All of the procedures were done in the operating room under general endotracheal anesthesia by the same 2 attending pediatric surgeons. Conclusions: The use of vascular surgical technology in difficult, otherwise impassable strictures of the esophagus and upper airway proved to be an extremely effective, easy-to-perform, and reproducible method of therapy. This procedure may obviate the need for resectional surgery in this setting.     

Lengthening technique for long gap esophageal atresia and early anastomosis

Background/Purpose: The treatment of long gap esophageal atresia remains a major surgical challenge. Whereas many approaches have been used for this problem, none are ideal. The authors used a technique originally described by Dr John E. Foker and accomplished early repair in 3 infants with long gap atresia. Methods: Three infants with esophageal atresia underwent thoracotomy shortly after birth and had a long gap preventing primary anastomosis. External traction sutures were placed on each esophageal pouch and exteriorized through the thoracic wall. The esophageal ends were approximated 1 to 2 mm daily by traction on the sutures. Anastomosis was performed when the 2 ends came together. Results: Three infants were included (31, 34, 37 weeks gestation, weights 1.38 kg, 1.9 kg, and 2.3 kg, respectively). The esophageal gaps were 3, 5, and 4.5 cm, respectively. Definitive anastomosis was performed at 14, 17, and 10 days, respectively. Two patients had anastomotic leaks that were treated conservatively. One patient had an esophageal stenosis that required dilatation. Conclusions: This technique allowed rapid esophageal lengthening in these 3 cases and led to early repair of long gap esophageal atresia, avoiding the need for a prolonged hospitalization or eventual replacement as well as long-term swallowing difficulties. J Pediatr Surg 38:737-739. [copy ] 2003 Elsevier Inc. All rights reserved.     

Balloon catheter dilatation in children with congenital and acquired esophageal anomalies

Background/Purpose: Esophageal stricture requiring dilatation often is the sequelae in children with a history of congenital or acquired esophageal anomalies. Balloon catheter dilatation (BCD) for esophageal stricture is well established in adults, yet its role in children is less well described. The authors report their initial experience using BCD in children with esophageal stricture, assessing outcome. Methods: Between January 1994, and December 1998, 20 children (age range, 17 days to 12 years) underwent BCD for esophageal strictures and were divided into 2 etiology groups (A and B). (A, n = 10) included congenital anomalies (esophageal atresia, 10), and (B, n = 10) included acquired anomalies (caustic ingestion, 7; tight fundoplication, 2; peptic stricture, 1). BCD was performed in all cases under conscious sedation with a balloon catheter (6 to 25 mm) with fluoroscopy. Outcome parameters included number of dilatations; complications, ie, perforation; and success or failure of procedure. Success was defined as increasing intervals of age-appropriate food tolerance between dilatations, and failure was defined as abandonment of dilatation in favor of surgical intervention. Follow-up for A and B ranged from 6 to 42 months. Results: Seventeen of 20 (85.0%) underwent successful BCD. In A, 10 of 10 (100%) had complete resolution of symptoms with follow-up ranging from 6 to 42 months versus 7 of 10 (70.0%) in B with follow-up of 6.5 to 36 months. In A, number of dilatations ranged from 1 to 4 over an average period of 2 months. In B, number of dilatations ranged from 3 to 40 over periods ranging from 2 to 30 months. All 3 failures occurred in children with caustic ingestion, with 1 child suffering perforation requiring urgent surgical intervention. Conclusions: Balloon catheter dilatation can be applied safely and effectively to children in whom esophageal strictures develop after repair of esophageal atresia. However, its use in children with acquired esophageal anomalies, notably caustic injury, is associated with higher complication and failure rates, necessitating greater caution and lower expectations.     

Can 'long-gap' esophageal atresia be safely managed at home while awaiting anastomosis?

Background: Neonates with [ldquo ]long gap[rdquo ] esophageal atresia (EA) are often managed with gastrostomy and tube drainage of the proximal pouch for a number of months while awaiting definitive repair. Because of the risk of aspiration and need for complex nursing care, most remain hospitalized during this time. However, prolonged hospitalization utilizes scarce resources and may be difficult for many families. Methods: The authors report on 5 patients who were treated successfully at home while awaiting esophageal anastomosis. Results: Four patients had pure EA (one had a duodenal atresia), and one had a distal fistula. Gestational ages ranged from 31 to 41 weeks. All had a gastrostomy within days of birth. Age at definitive repair ranged from 6 to 12 months. Time at home while awaiting anastomosis ranged from 42 to 113 days. Care at home included nursing care, suction equipment and training, gastrostomy feeding, and ability to perform cardiopulmonary resuscitation. The only complications noted while at home consisted of ear infection in one patient and recurrent upper respiratory tract infections in another patient. Conclusions: Selected patients with long gap esophageal atresia can be treated safely at home while awaiting esophageal anastomosis. Success of this approach depends on a motivated, reliable family, and adequate support from community health care providers. J Pediatr Surg 38:705-708. [copy ] 2003 Elsevier Inc. All rights reserved.     

Endoscopic follow-up in esophageal atresia-for how long is it necessary?

Purpose: The aim of this study was to find out how long it is necessary to follow-up esophageal atresia patients endoscopically to prevent irreversible mucosal changes. Methods: Seventy-four of 79 long-term survivors with esophageal atresia and primary anastomosis underwent a total of 322 esophagogastroduodenoscopies during a follow-up period of 0.5 to 19 years (mean, 10.3) after the primary operation. For analysis, the biopsy findings were divided into 2 groups: good, histologically normal or only mildly inflamed mucosa; unfavorable, moderate or severe esophagitis or gastric metaplasia. Fundoplication irrespective of indications was considered unfavorable. The results were analyzed using actuarial survival analysis; the changing point was when a [ldquo ]good[rdquo ] turned into [ldquo ]unfavorable.[rdquo ] Results: Forty-five patients (61%) remained in the [ldquo ]good[rdquo ] group throughout the study period; 15 of those (20%) had completely normal findings. Nine patients (12%) had moderate, one (1%) had severe esophagitis, and 13 patients (18%) had gastric metaplasia. Fundoplication was performed on 21 patients (28%). Conclusions: About 40% of esophageal atresia patients eventually have significant esophageal mucosal pathology or need to have a fundoplication. The majority of these changes appear before the age of 3 years. Routine endoscopic follow-up of esophageal atresia patients is recommended at least to the age of 3 years. J Pediatr Surg 38:702-704. [copy ] 2003 Elsevier Inc. All rights reserved.     

Thoracoscopic repair of tracheoesophageal fistula in newborns

Background: Advancements in minimally invasive surgery in neonates have allowed even the most complex neonatal procedures to be approached using these techniques. Methods: During a period of 15 months, 8 patients born with a proximal esophageal atresia and a distal tracheoesophageal fistula underwent repair thoracoscopically. Weights ranged from 2.1 to 3.4 kg and operating times ranged from 55 to 120 minutes.Results: All procedures were completed successfully thoracoscopically, and there were no operative complications. One patient had a small leak on day 4 that resolved spontaneously on day 8. All other patients were shown to have a patent anastomosis with no leak by Barium swallow on day 5. Conclusion: This initial report shows that esophageal repair in the neonate is technically feasible and may provide advantages in terms of exposure and esophageal length, as well as the recognized advantages of avoiding a thoracotomy. J Pediatr Surg 37:869-872.     

Feasibility of thoracoscopic repair of esophageal atresia with distal fistula

Backgound/Purpose: Evaluation of the feasibility of thoracoscopic correction of esophageal atresia with distal fistula. Methods: Eight consecutive neonates with esophageal atresia and distal fistula were treated thoracoscopically. Mean birth weight was 3,048 g (range, 2,140 to 3,770). The patients were intubated endotracheally and placed in a [frac34] left prone position. Three cannulae were inserted along the inferior tip of the scapula. CO₂ was insufflated at a pressure of 5mm Hg and a flow of 0.5 L/min. The fistula was either clipped or ligated. The proximal esophagus was opened and an anastomosis was made over a 6F or 8F nasogastric tube with interrupted 5-0 Vicryl. Results: All procedures were completed thoracoscopically without major peroperative complications. The mean operating time was 198 minutes (range, 138 to 250). One patient had a major leak, resulting in a stormy postoperative course, but the leak healed on conservative treatment. This patient and 3 others had stenosis requiring dilatation, respectively, 3, 6, 12, and 1 times. The babies were fed after a median period of 8 days. The median hospital stay was 13 days. Conclusions: Thoracoscopic repair of esophageal atresia with distal fistula is feasible. Larger series are needed to determine the exact place of the thoracoscopic approach.     

Is contralateral exploration necessary in girls with unilateral inguinal hernia?

Background/Purpose: Routine contralateral groin exploration in girls with unilateral inguinal hernia (UIH) continues to be controversial. The aim of this study was to determine the incidence of contralateral hernia development in girls after UIH repair. Methods: Between 1972 and 2000, 391 girls underwent repair of UIH. Ninety-one (23%) of the 391 girls underwent routine contralateral exploration during UIH repair and were excluded from the study. The median age at operation in the remaining 300 girls was 3.3 years (range, 1 month to 14 years). Two hundred ten (70%) girls had right-sided and 90 (30%) had left-sided UIH. Familial history of hernia was identified in 8 (3%) patients. The follow-up ranged from 1.5 to 14 years. Mann-Whitney U test was used for intergroup comparison. Results: A contralateral hernia developed in 24 (8%) of the 300 patients who had unilateral repair of inguinal hernia. Median time from operation to occurrence of the contralateral hernia was 3 years (range, 1 to 4 years). Age at operation, side of hernia, and familial history did not influence the development of contralateral hernia. Conclusions: These data suggest that the low incidence of contralateral hernia development in girls undergoing UIH repair does not justify routine contralateral groin exploration. J Pediatr Surg 38:756-757. [copy ] 2003 Elsevier Inc. All rights reserved.     

Guide wire-assisted urethral dilatation for urethral strictures in pediatric urology

Purpose: The aim of this study was to report the results of 32 cases of dilatation of urethral stricture using a guide wire and sheath dilator technique supplemented by clean intermittent catheterization if further stabilization of the urethral stricture was felt warranted.Methods: The procedure involves insertion of a straight flexi-tip lubricated guide wire through the urethral stricture under cystoscopic guidance followed by insertion of a series of sheath dilators. Dilatation was followed by insertion of a Foley catheter, which was left in situ for 1 to 3 days. Patients underwent repeat cystoscopy to evaluate the urethra for recurrent stricture and those with a recalcitrant stricture were commenced on clean intermittent catheterization (CIC) to stabilize the narrowing.Results: Thirty-two patients were included. They have been followed up for up to 2 years after their last cystoscopy (mean, 16 months). Thirteen of 32 patients had more than 4 dilatations under anesthesia. Twelve patients had undergone CIC postoperatively. Complications included a urinary tract infection in 3 boys and bladder spasms in one. No false passage or sepsis occurred with this approach.Conclusions: Guide wire-assisted urethral dilatation helps avoid risks associated with blind dilatation techniques and appears to be a safe and simple alternative for management of urethral strictures in pediatric urology.     

Tracheoplasty for long segment congenital tracheal stenosis: analysis of 29 patients over two decades

Purpose: The authors determined the long-term outcome of patients who underwent surgical repair of long-segment congenital tracheal stenosis (LCTS) and compared the quality of cartilage graft and slide tracheoplasty techniques.Methods: Twenty-nine patients underwent surgical repair of LCTS at 29 days to 9 years of age. In 26 patients, more than 50% of the entire length of the trachea was involved. The early 12 patients (1981 through 1992) were treated with a costal cartilage graft for tracheal reconstruction. The remaining 17 patients (1997 through 2002) were treated with slide tracheoplasty. For both procedures, a median sternotomy was preferred because of convenience when using extracorporeal circulation and for simultaneous repair of associated cardiovascular anomalies.Results: Of the 12 patients who underwent a costal cartilage graft, 8 survived (66%). Six of these patients are free of disease, but 2 have persistent airway problems. Three patients died of residual stenosis and 1 of unrelated disease. Of the 17 patients who underwent slide tracheoplasty, 13 survived (76%), and 10 are free of disease. Three patients continue to require a stent because of tracheomalacia and granulation. Two patients died of cardiac failure, 1 of granulation, and 1 of aspiration pneumonia.Conclusions: The authors challenged LCTS with a unique surgical approach and succeeded in making an incurable disease curable. When the cartilage graft technique is compared with slide tracheoplasty, however, the latter is preferable because it preserves native tracheal tissue with less frequent complications.     

Esophageal motor function in congenital esophageal stenosis

Background/Purpose: Congenital esophageal stenosis (CES) is a rare condition that is associated with various foregut symptoms. The aim of the current study was to investigate esophageal motor function in pediatric patients with isolated CES.Methods: Four boys with CES (age, 3 weeks to 4 years old) were studied before treatment. The initial symptoms were dysphagia or stridor. The CES was caused by fibromuscular stenosis (FMS) in 2, tracheobronchial remnants (TBR) in 1, and membranous diaphragm (MD) in 1. An esophagram, endoscopy, 24-hour esophageal pH monitoring, and manometry were conducted.Results: The esophagram showed the stasis of contrast medium proximally to the distal esophageal narrowing in FMS/TBR patients. Endoscopic esophagitis was not found in any patients. Three patients were documented with pathologic esophageal acid exposure by 24-hour esophageal pH monitoring. Manometry showed that esophageal contractions predominantly were synchronous in FMS/TBR patients but were peristaltic in an MD patient. Basal lower esophageal sphincter (LES) pressure was at least 20 mm Hg in all. Swallow-induced LES relaxations were incomplete in FMS/TBR patients.Conclusions: The presence of gastroesophageal reflux and impaired esophageal motility are common in patients with CES.     

Aggressive conservative treatment of esophageal perforations in children

Background/Purpose: In contrast with adult patients in whom surgical closure of the defect is preferred, nonoperative treatment has been the usual approach for esophageal perforation (EP) in children. This report aims to assess whether this strategy stands the passage of time. Methods: We reviewed retrospectively the charts of 17 patients aged 5.3 [plusmn] 0.9 years (mean [plusmn] SD) treated at our institution for EP between 1991 and 2001. Results: Nineteen episodes of EP were caused by stricture dilation in 9 cases, foreign body extraction in 3, and blunt trauma and sclerosis of varices in 2 cases each. The remaining child had multiple gastrointestinal perforations in the course of chemotherapy for leukemia. Vigorous treatment, consisting of nasopharyngeal aspiration, wide spectrum antibiotics, prompt drainage of effusions and either parenteral or infraesophageal nutritition, was implemented immediately after diagnosis. Perforations were closed without direct surgery in 18 of 19 episodes (16 of 17 children). One or more pleural drains were inserted in 12 cases, and pericardial drainage was required once. Seven gastrostomies, 2 jejunostomies, and one esophagostomy were performed. Several major abdominal operations were necessary to repair concomitant lesions in a child who sustained severe blunt abdominal trauma and in the patient with leukemic perforations. All patients survived, and all recovered esophageal function. However, 2 with intractable lye strictures ultimately required esophageal replacement. The only patient in whom a direct approach for esophageal necrosis due to variceal endosclerosis was unavoidable, lost her organ and had a retrosternal colonic interposition after a successful portosystemic shunt. Excluding patients with other concomitant lesions and the patient who underwent surgery, median length of stay was 11 days (range, 6 to 47). Conclusions: Prompt and aggressive nonoperative treatment of esophageal perforations in children allows survival with conservation of the organ in most cases and remains, in the authors[apos ] hands, the first therapeutic choice at this age. J Pediatr Surg 38:685-689. [copy ] 2003 Elsevier Inc. All rights reserved.     

Esophageal foreign body obstruction after esophageal atresia repair

Background/Purpose: The aim of this study was to determine the incidence and risk factors for esophageal foreign body obstruction (EFBO) after esophageal atresia/tracheoesophageal fistula (EA-TEF) repair. Methods: A Case-control chart review was conducted from 1987 to 1999. Results: EFBO occurred in 14 of 108 patients (13%) with EA-TEF. Mean follow-up was 31 months. Alimentary debris was the most common foreign body. Mean time between operation and EFBO was 25 months. EFBO recurred in 50%. Sixty-one percent of patients required endoscopic FB removal. Esophageal stenosis was present in 57% of EFBO patients, and 80% of stenoses presented initially with EFBO. The incidence of gastroesophageal reflux (GER; 50%), type C anomaly (100%), anastomotic leak (21%), major associated anomalies (50%), and encephalopathy (14%) were not significantly different between the EFBO group and the total EA-TEF population. There was no difference in the need for antireflux surgery (57%) between those with EFBO and GER and the total EA/TEF population with GER. Conclusions: Esophageal foreign body obstruction occurs in at least 13% of patients after EA-TEF repair. Because no reliable risk factors were identified that could predict foreign body obstruction, all families should be warned about this possibility during the first 5 years, particularly once the child begins eating solid foods.     

Early surgical management of Klippel-Trenaunay syndrome in childhood can prevent long-term haemodynamic effects of distal venous hypertension

Purpose: The aim of this study was to evaluate the authors' preliminary experience in early surgical treatment of distal venous hypertension (DVH) in children affected by Klippel-Trenaunay syndrome (KTS). Methods: Clinical assessment, surgical management, and outcome of 29 children (18 girls, 11 boys) affected by KTS observed from October 1998 to October 2000 were reviewed retrospectively. Results: Patients ranged in age from 8 months to 17 years (median age at surgery, 10.3 years). The clinical findings are presented. Surgical treatments included stripping of persistent marginal vein (n = 16), multiple legation of bulky varicosities (n = 10), complementary sclerotherapy (n = 14) and laser photocoagulation (n = 13), and excision of associated lymphatic malformations (n = 5). No mortality or major postoperative morbidity occurred. Follow-up period ranged from 6 months to 2 years. Conclusion: These preliminary results suggest that early surgical management of DVH in KTS is safe and could be effective in preventing or minimising the long-term haemodynamic effects of DVH in absence of associated deep venous system anomalies.     

Femoral hernia repair with mesh-plug in children

Purpose: Femoral hernias (FH) are rarely seen in children, so there is no consensus on the age and sex distribution or the optimum method of repair. Recently, repair of the femoral hernias with mesh-plug has gained wide popularity in adults. The authors used this method in 4 children with FH and discuss its utilization in children. Methods: Four consecutive children with femoral hernia were treated using mesh-plug. Patients' characteristics and operative technique was described. Results: Plugging femoral defect with a mesh-plug was applied successfully in children. No complications were seen after a follow-up period for 2 years (range, 8 to 30 months). Conclusion: Repair of femoral hernias with mesh-plug is performed easily and successfully in children. J Pediatr Surg 37:1456-1458.     

Forme fruste choledochal cyst: long-term follow-up with special reference to surgical technique

Purpose: Forme fruste choledochal cyst (FFCC) is a choledochal cyst that has minimal or no dilatation of the extrahepatic bile duct (EHBD) and is associated with pancreaticobiliary malunion (PBMU). The authors reviewed the long-term outcome of their patients with FFCC.Methods: Inpatient and outpatient records of 281 patients with choledochal cyst were reviewed. In this study, minimal dilatation of the EHBD was defined as its maximum diameter being less than 10 mm.Results: There were 17 cases of FFCC identified. In all 17 patients, PBMU was present. The mean age at EHBD excision was 2.9 years. Fourteen patients had hepatico-jejunostomy, and three had hepatico-duodenostomy. The histology of the excised EHBD showed mucosal ulceration/sloughing (in 35.3% patients), fibrosis (52.9%), and inflammatory cell infiltration (41.2%). Over a mean postoperative follow-up period of 9.8 years, there have been no episodes of cholangitis or anastomotic stricture formation.Conclusions: The treatment of choice for FFCC in children is EHBD excision and hepatico-jejunostomy. There is little surgical morbidity if performed carefully.     

Outcome of pediatric live-donor liver transplantation-the Toronto experience

Background/Purpose: Live-donor liver transplantation (LDLT) has developed to address the critical shortage of cadaveric organs that accounts for 20% of children who die while awaiting for a liver transplant in Ontario each year. This report reviews the outcome of the pediatric recipients of LDLT at the authors[apos ] center. Methods: The charts of all children who received a LDLT between June 1996 and March 2002 were reviewed retrospectively. Results: Thirteen children (mean age, 3.6 years) underwent LDLT. All donors were parents except for one cousin. Ten grafts were left-lateral segments, 2 were right lobes, and 1 was a left lobe. Three patients required a SILASTIC^{[reg ]} (Dow Corning, Midland, MI) patch for delayed abdominal wall closure. Patient and graft survival rate was 100% with a median follow-up of 376 days. Major postoperative complications included biliary leaks (n [equals] 2), biliary strictures (n [equals] 1), portal vein thrombosis (n [equals] 1), and hepatic venous complications (n [equals] 1). There were no cases of hepatic artery thrombosis. Ten of 12 children became Positive for Epstein-Barr virus (EBV), and 3 of these patients had readily treatable post-transplant lymphoproliferative disorder. Conclusions: LDLT is an acceptable alternative to cadaveric transplantation for children with end-stage liver disease. J Pediatr Surg 38:668-671. [copy ] 2003 Elsevier Inc. All rights reserved.     

Congenital esophageal stenosis: Therapeutic strategy based on etiology

Background/Purpose: The diagnosis and treatment of congenital esophageal stenosis (CES) can be a vexing clinical problem. This study aims at determining the ideal therapeutic strategy for the management of CES. Methods: Medical records of patients with CES were reviewed retrospectively with regard to diagnostic method, therapy, and outcome. Results: During the last 29 years, 36 patients, aged 1 month to 9 years, were found to have CES. Diagnosis was made by fluoroscopy, esophagoscopy, endoscopic ultrasonography (EUS), and pathologic examination. Of the 36 patients, 15 had tracheobronchial remnants (TBR), 13 had fibromuscular stenosis (FMS), 5 had membranous stenosis (MS), and 3 had multiple stenoses (MPS). EUS was diagnostic in distinguishing TBR from FMS. All patients with TBR underwent operative repair in which 13 patients had resection and anastomosis with or without preoperative dilation. The 14th patient had enucleation and the 15th underwent myotomy. Postoperative dilation was needed 2.2 times for 2.1 months on average in this group of patients. Ten patients with FMS were treated by dilation only (a mean of 4.7 dilations was required for a duration of 2.6 years), whereas 3 patients underwent open surgical intervention. Most patients with MS or MPS were treated by dilation (with or without endoscopic resection of the membranes), whereas 2 patients had surgical intervention. All patients in this study are doing well after a mean follow-up period of 5.3 years (range, 0.5 to 25 years) without any further operative interventions. Conclusions: If the etiologic diagnosis of CES is unclear, EUS is useful in distinguishing TBR from FMS. This distinction is critical, because patients with TBR should undergo surgical resection, whereas most cases of FMS, MS, and MPS can be treated with esophageal dilation alone.