Long Gap Esophageal Atresia and Esophageal Replacement: Moving Toward a Separation?

Transcription

1 Long Gap Esophageal Atresia and Esophageal Replacement: Moving Toward a Separation? By P. Bagolan, B.D. Iacobelli, P. De Angelis, G. Federici di Abriola, R. Laviani, A. Trucchi, M. Orzalesi, and L. Dall Oglio Rome, Italy Background/Purpose: Treatment of long gap esophageal atresia (EA) is still a major challenge. Gastric transposition and colon interposition are the 2 most popular choices for esophageal replacement, but there is general agreement that the child s own esophagus is the best. The aim of the study was to critically evaluate the feasibility and outcome of primary repair of long gap EA with or without tracheoesophageal fistula (TEF) by direct esophago-esophageal anastomosis as the only technique. Methods: Seventy-one neonates with EA / TEF were considered. Nineteen cases were classified as long gap ( 3 cm). All infants underwent either primary or shortly delayed repair. In the latter group, a gastrostomy was performed along with an x-ray evaluation of the gap a few days before surgery (mean age, 46.4 days). To avoid disruptive anastomotic force, all infants were kept paralyzed and mechanically ventilated for an additional 6 days after esophageal anastomosis. Before starting feeding, postoperative esophagogram was done on day 7. Endoscopy was done routinely, starting 1 month after surgery; ph monitoring was conventionally performed at 1 year of age or even earlier, should gastroesophageal reflux disease (GERD) be suspected. Follow-up ranged from 11 months to 7 years. Results: In all 19 long gap EA infants an esophago-esophageal anastomosis was performed. Six of them (31%) required an anterior esophageal flap to bridge residual gap. Complications included minor anastomotic leak in 2 cases and anastomotic stricture ( 5mm) in 12 (80%) cases, which were treated with an average of 5 dilatations (1 of which with resection of the stricture). GERD occurred in 8 cases (53.3%), of which, 3 required fundoplication. None of the patients had esophageal swallowing difficulties or persistent dysphagia. Two children experienced food aversion. Mean hospital stay was 66.2 (22 to 230) days. There were 4 deaths (very low birth weight, 1; associated anomalies, 1; and late sepsis, 2). Conclusions: Considering heat gap determination remains imprecise, it seems possible to conclude that in a wellestablished tertiary care level referral center: (1) long gap EA could be treated successfully with primary repair and anastomosis; (2) strictures and GER represent the most frequent postoperative problem, but additional procedures required seem acceptable to maintain the patient s own esophagus and avoid replacement; (3) esophageal substitution in long gap EA should be reserved for cases in which a previous attempt of esophageal reconstruction failed. J Pediatr Surg 39: Elsevier Inc. All rights reserved. INDEX WORDS: Esophageal atresia, tracheoesophageal fistula, long gap, esophageal replacement. TREATMENT of long gap esophageal atresia (LEA) is still a major challenge. Options for esophageal reconstruction have long included use of native esophagus or replacement with stomach, colon, or small intestine. 1,2 Moreover, esophageal replacement often has to be delayed to 6 to 9 months of age. Although it is generally accepted that the best results are obtained if native esophagus is preserved, this may be difficult to achieve. Technical refinements have been advised to mobilize and From the Neonatal Surgery Unit and Neonatal Intensive Care Unit, Department of Medical and Surgical Neonatology, Bambino Gesú Children s Hospital, Rome, Italy. Presented at the XLIX Meeting of the British Association of Paediatric Surgeons, Cambridge, England July 23-26, Address reprint requests to Pietro Bagolan, Newborn Surgery Unit, Bambino Gesù Children s Hospital, P.zza S. Onofrio, 4, Rome, Italy Elsevier Inc. All rights reserved /04/ $30.00/0 doi: /j.jpedsurg lengthen both esophageal pouches, including circular myotomies, 3 esophageal flap, 4 continuous suture traction with reoperation, 5 and multistaged extrathoracic elongation of the proximal esophagus. 6 Our protocol for treatment of EA was updated in 1995 to either avoid any esophageal replacement or long-term delayed reconstruction in both LEA and normal gap esophageal atresia (NEA). The aim of this work was to critically review our surgical experience of the last 7 years to evaluate (1) feasibility of primary repair of LEA as a standard repair, (2) effectiveness of surgical refinements such as esophageal flap or tractions, and (3) different results in terms of outcome comparing LEA with NEA. MATERIALS AND METHODS From January 1995, 13 (18%) infants with LEA, with or without tracheoesophageal fistula (TEF), were treated at our center; these were part of a series of 71 consecutive patients with EA. Six further patients with LEA, 4 type A and 2 type C, were referrals after a failed surgical repair. Patients with H fistula and congenital stenosis without atresia 1084 Journal of Pediatric Surgery, Vol 39, No 7 (July), 2004: pp

2 LONG GAP ESOPHAGEAL ATRESIA 1085 Table 1. Comparison Between LEA and NEA (71 Cases) Long Gap ( 3 cm) No Long Gap P Value Total number Type A 8 B 1 C D 3 Birth weight (g) 2, , Not Significant Gestational age (wk) Not Significant Associated Anomalies Not Significant Early complications 7 12 Not Significant Leakage 2 1 Refistula 2 Post-op pneumonia 3 5 Sepsis 2 4 Death Late complications Stricture 12 (80%) 27 (60%) Not Significant Number of dilations 5.5 (2-10) (1-10) Dilations interval (d) Stent Malnutrition ( 25 ) 5 (33%) 4 (7.6%) Not Significant Esophagitis 8 (53.3%) 20 (40%) Not Significant Bronchopneumopathy 8 (53.3%) 21 (42%) Not Significant Fundoplication 3 (20%) 6 (12%) Not Significant Follow-up (mean time) 30 mo (11 mo-7yr) 25 mo (11 mo-7yr) were excluded from the current report. All cases were classified into anatomic anomaly type based on the Gross classification. 7 Several parameters, including gap length, birth weight, gestational age, associated anomalies, postoperative complications, and medium-term outcome were considered. To define the level of fistula, when present, as well as to rule out Gross type B and D EA, tracheoscopy was always performed before operation. When a deep fistula (close to the carina or lower) was present, it was occluded (by a 3.5F Fogarty catheter, the balloon inflated with 0.2 ml of saline solution) to make easier the mechanical ventilation, avoiding gastric overdistension and gastroesophageal reflux. The catheter then was removed at the same time of surgical closure of the fistula. Gap length was evaluated in 2 different ways. Preoperative Evaluation In type A and B, this was done under general anesthesia both after gastrostomy and at the time of esophageal reconstruction. For this purpose, a radiopaque 10F feeding tube and a No.5 Hegar dilator were advanced under gentle pressure into the proximal and the distal pouch, respectively. The residual gap then was measured under fluoroscopy and expressed in terms of vertebral bodies. Intraoperative Evaluation This was performed in type C and D EA after wide dissection and gentle traction of upper and lower pouch. Thus, the gap was expressed in centimeters Long gap was defined as a gap 3 vertebral bodies or greater than 3 cm. Why this gap was arbitrary assumed as a long gap is later reasoned. The esophagus was approached extrapleurally (excluding 2 cases in which a previous attempt of reconstruction has been already made elsewhere) on the side opposite the aortic arch. All patients underwent primary esophago-esophageal anastomosis (and TEF closure) immediately after birth (type C and D) or shortly delayed (4 to 6 weeks) after an initial gastrostomy (type A and B). When, despite extensive mobilization and traction of both pouches, the remaining gap was deemed too wide for direct anastomosis, an upper esophageal flap was raised and tubulized. In all cases, a nasogastric transanastomotic probe was positioned. To minimize disruptive anastomotic forces (ie, crying, hiccup), all infants with LEA were postoperatively kept paralyzed, sedated, and mechanically ventilated for 6 days. A paranastomotic chest drain was left on site until the esophagogram was performed. The transanastomotic tube was removed and contrast study done on postoperative day 7 to rule out anastomotic leak before starting feeding. According to the definition of stricture (anastomotic diameter 5 mm), all patients with LEA, even if asymptomatic, underwent a further contrast study and endoscopy 1 month after surgery to document anastomotic stricture or esophagitis. In this high-risk group of children, if esophageal stenosis was confirmed, a further endoscopy was performed 3 months after surgery. When the size of esophageal anastomosis was 5 mm in diameter, dilatations were carried out under general anaesthesia with Savary bougie and guide wire. The upper gastrointestinal endoscope was the GIFN30 (Olympus, outer diameter 5 mm) or GIFXPI60 (6 mm). In the presence of recurrent (more than 5 dilatations) stenosis, a silicone esophageal stent was left in situ for 40 days. In the presence of signs and symptoms of gastroesophageal reflux disease (GERD), 24-hour ph monitoring was also considered. Because of the high stricture rate and GER reported in LEA, endoscopy and ph monitoring were further planned at 1 year of age. Patients with LEA and NEA were compared then with regard to birth weight, gestational age, associated malformations, and complications such as leakage, stenosis, GERD, and failure to thrive (Table 1). Attention was also directed to differences between patients with LEA requiring esophageal flap and all remaining patients with LEA (Table 2); the same differences were evaluated with regard to patients with

3 1086 BAGOLAN ET AL Table 2. LEA and Esophageal Flap Upper Flap T-T P Value Total number 6 13 Birth weight (g) Not Significant Gestational age (wk) Not Significant Associated anomalies 5 (83.3%) 8 (61.5%) Not Significant Gap length (cm) 3.6 (3-5.3) (3-6.4) 0.7 Not Significant Leakage 1 1 Not Significant Death 1 3 Not Significant Stricture 4 (80%) 8 (80%) Not Significant Stricture length (1-1.5) Number of dilations (0-8) 2.8 Not Significant Dilations interval (d) (15-60) 14.9 Not Significant Stent 2 (33%) 2 (15%) Not Significant Reanastomosis 1 0 Not Significant Nissen 1 2 Not Significant type A-LEA and the remaining ones (Table 3). T test, 2, Pearson test, and Fisher s Exact test were used for statistical analysis with a significance level of P less than.05. RESULTS Seventy one patients with EA with or without TEF were treated during the 7-year study period: 8 were type A (11.3%), 1 was type B (1.4%), 59 were type C (69%), and 3 were type D (4.3%). Our study group is of 19 newborns affected by LEA (Table 1). Thirteen infants were boys and 6 girls. The mean gestational age was 34 weeks (range, 29 to 40 weeks), the mean birth weight was g (range, 880 to 3830 g). Thirteen (68.5%) infants had associated anomalies. Urogenital, cardiovascular, and skeletal malformations were most frequently observed in 4, 3, and 3 patients, respectively. Intestinal atresia occurred in 2 cases. One patient had a Silver Russell syndrome and type A EA. LEA occurred in 10 type C (52.6%), 8 type A (42.1%), and 1 type B patients, respectively. Gap ranged from 3.0 to 6.4 cm per vertebral bodies. Twelve infants, 8 type A, 3 type C (2 referred from elsewhere, 1 with duodenal and multiple ileal atresia), and 1 type B had a Stamm gastrostomy performed as initial step. In all 19 patients, direct end-to-end anastomosis was performed at a mean age of 48.5 days (range, 1 to 202 days). The longest delay for reconstruction (202 days) was in a baby affected by Silver Russel Syndrome, type A EA, and poor general conditions, referred to us from another institution at age of 90 days. In all subjects, extensive mobilization of both pouches was required: esophageal flap had to be constructed in 6 patients (31.5%; 3 type A and 3 type C). No azygos vein anomalies were noted; preoperative chest x-ray followed by echocardiography showed 2 aortic arches on the right side. No intraoperative deaths were observed, and in no instance did complications demand switching to esophageal substitution. Early Postoperative Complications Early complications occurred in 7 of 19 patients (36.8%); these consisted of minor anastomotic leak (2 cases), postoperative pneumonia (3 cases), and sepsis (2 Table 3. LEA: Type A versus B and C Type A Type B and C P Value Total number 8 11 Birth weight (g) Not significant Gestational age (wk) Not significant Associated anomalies 4 9 Not significant Gap length (cm) 3.8 (3-6.4) (3-5.3) 0.6 Not significant Leakage 1 1 Not significant Death 1 3 Not significant Stricture 5 7 Not significant Stricture length Not significant Number of dilations Not significant Dilations interval (d) Not significant Stent 3 1 Not significant Reanastomosis 1 0 Fundoplication 1 2 Not significant

4 LONG GAP ESOPHAGEAL ATRESIA 1087 cases). There were 4 deaths not related to surgery (21%); 1 occurred in a type A EA. Causes of death included late (day 90 postoperative) postoperative sepsis, cardiac anomaly and sepsis (day 21 postoperative), necrotising enterocolitis and peritonitis (day 30), severe bronchial anomalies (day 18). In all newborns, esophagogram proved normal, and 3 of 4 were feeding normally. In the 15 remaining cases, mean follow-up was 2.5 years (11 months to 7 years). Average length of stay was 66.2 days (22 to 230 days). Late Complications Strictures (Table 1, 5 mm) occurred in 12 cases (80%). An average of 5.5 dilations (range, 2 to 10 dilations) was required. Dilations failed in 4 patients (2 with esophageal flap and 2 with direct anastomosis) who required silicone stent placement. These patients did well after stent removal. In 1 patient, recurrence of esophageal stenosis and failure to thrive demanded anastomotic resection, esophago-esophageal anastomosis, and Nissen fundoplication. Reflux esophagitis occurred in 8 of 15 cases (53%), and Nissen fundoplication was performed in 3 of them. The 5 remaining were treated only medically. Failure to thrive ( 25th percentile) occurred in 5 patients (33%). None of the patients reported swallowing difficulties or persistent dysphagia. Two children experienced food aversion, which necessitated nocturnal feeding supplementation through the gastrostomy over the first 6 and 11 months, respectively. No statistically significant difference (Table 1) was observed between LEA and NEA with regard to considered parameters, with exception of mean time between dilatations (23.3 v days), total number of dilatations (5.5 v. 3), necessity of esophageal stent (4 v. 0), and death (4 v. 2). A higher rate of anastomotic strictures and GERD was noted in LEA patients, although this difference did not reach statistical significance. Comparison between patients with LEA requiring upper esophageal flap (6 patients) and the remaining ones (13 patients) is shown in Table 2. Stricture length was significantly higher in newborns with esophageal flap (2.2 v. 1.0 cm). Length of stricture affected neither mean number nor interval between dilatations. No differences with regard to parameters considered in Table 3 were seen when comparing patients who had type A LEA with the remaining ones with long gap. DISCUSSION Definition of long gap is very variable in the literature. Long gap has been arbitrarily defined as either 2 or more centimeters or 2 vertebral body spaces between the 2 esophageal pouches. 1,2,5,8-13,15 Other investigators define a gap of 3.5 cm or more an ultra long gap. 5,14 Gaps 2 cm or less are considered amenable to primary repair; however, when the gap is 3 cm or more, the complication rate is significantly higher. 1,12,13,16 Spitz 2 in 1998 recommended establishing a cervical esophagostomy and an esophageal replacement if the gap exceeds the length of 6 vertebral bodies. Hagberg et al in reported a group of OA with a 3-cm gap and a complication rate of 100%. Thus, the advantages of the native esophagus, which most of the surgeons consider infinitely better than any replacement that can be constructed, 18 seem to outweigh complications encountered for gaps longer than 3 cm or 3 vertebral spaces. 12 That is the reason for which we arbitrarily assumed the 3-cm gap as the length limit to define a long gap. Esophageal replacement often exhibits early and late problems because of the loss of the transposed segment and to the presence of the GE junction in the chest or neck with possible metaplasia, Barrett s esophagus; 12,19-23 there has also been evidence of dysplasia 24,25 evolving into squamous cell carcinoma. 26 Appignani et al 27 reported 8 gangrenous bypass of 117 patients operated on in the last 20 years of left transverse colon transposition. In the series of Schier et al, 25 of 8 colonic transpositions, there were 3 necroses. In the same report, the 2 replacements with small bowel had to be abandoned. Ruangtrakool and Spitz 28 reported 65% postoperative complications of 100 gastric transposition; the same investigators noted that respiratory ones were the most common in EA patients. In other series postoperative complications of gastric transposition have been reported in 5 of 6 cases. 30 Moreover, these children eat later and worse than after an esophageal reconstruction. 12,16,28 Esophageal replacement for type A esophageal atresia was reported recently in few cases: 3 of 23 cases in the series of Rescorla et al, 16 whereas in other reports with a total number of 27 LEA cases, no replacement was required. 1,5,14,32 Early repair of long gap esophageal atresia with colon 35,36 has been reported as a feasible and functional technique. Therefore, it seems reasonable to conclude that nowadays there is no substitute for a congenitally deficient esophagus when all attempts at primary anastomosis have been exhausted. 29 Stomach and colon interpositions have developed frequent early and late problems. For this reason, the term replacement is probably better than substitution. Primary repair of ultra long gap EA without lengthening procedure was reported by Boyle et al 14 in 1994 in 8 of 58 babies with EA and gaps ranging from 3.5 to 6 cm. 14 Despite severe anastomotic tension in all cases, there were no anastomotic leaks, disruptions, recurrent tracheoesophageal fistulas, or deaths. Sixty-two percent of these infants needed a Nissen fundoplication. More recently, Foker et al 5 reported a series of 70 infants (with

5 1088 BAGOLAN ET AL Fig 1. Preoperative tracheoscopy. The Fogarty 3.5 catheter has been advanced into the esophageal fistula at the carina. or without TEF) observed from 1976 to 1997 in which a primary esophageal anastomosis was performed for full spectrum of OA. Primary repair was performed with no surgery-related deaths and 11% later deaths. There were no discernible anastomotic leaks and one late recurrent TEF. In this series, the gap length ranged from insignificant to 6.8 cm. The entire spectrum of anomaly and presentation was considered, including long gap EA initially treated with gastrostomy and cervical esophagostomy (the latter not observed in our current series). More antireflux procedures and more dilatations were needed for very long gap cases to relieve stricture. Delayed primary anastomosis in cases of pure EA was reported also from Puri and Khurana. 32 Primary reconstruction with the native esophagus is technically easy and helps avoid most of the problems associated with replacements; therefore, it always is recommended for NEA repair, but agreement is still not reached for LEA. To avoid any esophageal replacement or long-term delayed reconstruction even in LEA, surgical protocol for treatment of EA has been modified since As a matter of fact, our Department of Medical and Surgical Neonatology is a tertiary care center. Cervical esophagostomy was always avoided and tracheoscopy always performed to define the level of the lower fistula, as well as to occlude it, if necessary, (Fig 1) and rule out Gross type B and D EA. All babies underwent operation within 72 hours after birth, which included either prompt esophago-esophageal anastomosis with TEF closure (type C and D) or gastrostomy and shortly delayed (4 to 6 weeks) esophageal anastomosis (type A and B). Time is commonly used to allow the segments to grow, and we believe that this is true in type A and B EA, in which the lower esophagus and stomach are hypoplastic, 33 such as any postatretic intestinal loop. The hypoplasia is probably secondary to complete lack of amniotic fluid transit because of to the absence of lower TEF. Following this rational we, as other investigators, 1 do not perform inferior pouch bouginage but do encourage large bolus gastrostomy feedings with the object of promoting gastro-esophageal reflux in the belief that the spontaneous growth of the lower esophagus may be caused and improved by reflux. The length of the lower esophagus is evaluated at the time of gastrostomy. Further evaluation is performed after 4 weeks, with gentle pressure, to make a decision regarding the definitive esophageal reconstruction. In this preoperative measurement of the gap, the spine is used as the reference point for estimating the gap between the pouches (Fig 2). On the contrary, in type C (and D) EA we assume that anastomosis is always feasible at birth, and TEF has to be closed as soon as possible. As a consequence, long gap is not easily presumable in these cases because the preoperative measurement is not possible. Thus, exact gap assessment is an intraoperative procedure, and the evaluation of the gap is made by measuring the length in centimeters. When the gap is still too long, after wide dissection of esophageal pouches and gentle intraoperative traction by few minutes (Fig 3 A&B),an upper esophageal flap is raised to avoid 2-stage primary anastomosis, which would imply 6 to 7-day sedation and paralyzation, mechanical ventilation, external esophageal traction, and rethoracotomy. 5 Regardless of whether primary (soon after birth) or delayed (4 to 6 weeks), esophageal anastomosis was always accomplished in our series, both in NEA or in Fig 2. Preoperative evaluation of the gap. Radio-opaque feeding tube and mega-dilator (see test) are gentle advanced into the esophageal pouches. The gap is 4 vertebral bodies (white arrows). Sinostosis of 2 ribs due to a previous surgical repair.

6 LONG GAP ESOPHAGEAL ATRESIA 1089 Fig 3. (A) Intraoperative traction of the esophagus to bridge a gap too long after wide dissection of the pouches (white arrow, upper esophageal pouch). (B) Primary reconstruction and transanastomotic feeding tubes. LEA. After 1995, none of 71 infants with EA needed to switch to esophageal replacement regardless of the length of the gap. To support the hypothesis that primary reconstruction in LEA is not only feasible but is also safe as in NEA, early and late complications of patients affected by LEA and NEA were compared; this was done to confirm that final outcome of primary reconstruction is similar in both groups. Even though a definitive conclusion is not possible in such a small cohort of patients (Table 1) no significant differences were found with regard to anastomotic leak and stenosis, recurrent fistula, postoperative pneumonia, fundoplication, esophagitis, and malnutrition (weight, 25 percentile). Birth weight and gestational age were lower in LEA. Mean number of dilatations was higher and interval between dilatation shorter in LEA (5.5 and , respectively). Eighty percent of LEA needed to be dilated if compared with 60% of NEA. Mortality rate was significantly higher in the LEA group, although this was not related to surgery. Patients needing upper esophageal flap did not have stricture more frequently than LEA patients without flap (Table 2); this finding has been confirmed recently by others. 5,6 Nevertheless, one of them required resection of the stricture, esophago-esophageal anastomosis, and Nissen fundoplication at 8.5 months of age. The anterior esophageal flap was associated recently with considerable morbidity, but a satisfactory outcome was achieved in long-term survivors. 31 Of 25 infants with EA reported by Davenport and Bianchi 4 in which the anterior flap was performed, 23 (92%) retained their esophagus and had no long-term swallowing problems. With preservation of the upper pouch and delayed primary repair, new problems may arise such as longer hospital stay, delay of the normal feeding, risk of aspiration. Total parenteral nutrition, prolonged ventilatory support, and intensive care monitoring are possible disadvantages of such a policy. 1 Long-term follow-up studies have shown that the majority of children with LEA have normal growth curves. 32,34 Even if the incidence of postoperative complications such as GERD is still high, gastroesophageal reflux was documented in other series of LEA in 54% to 100% of cases, and fundoplication was required in 33% to 100% of patients. 1,5,12,20,26 In our series, esophagitis and recurrent broncopneumonia (Table 1) were more common in LEA (53%) than in NEA (40%). Likewise, malnutrition ( 25th percentile) was more frequent in LEA patients (33% v. 7.6%) (Table 1). In the current series, patients with type A LEA (8 patients) were also compared with the remaining ones (11 patients) affected by LEA (Table 3). None of the considered parameters appeared significantly different. Two children experienced food aversion, which necessitated nocturnal feeding supplementation through the gastrostomy during the first 6 and 11 months, respectively. Swallowing difficulties never occurred, and no infant in this series experienced inability to start normal oral feeding early in life as it has been reported with different surgical procedures of esophageal elongation or replacement. 6,25,28 Even though series in literature of pure EA are too small to draw any definitive conclusions, our data suggest that primary repair in LEA might be as feasible as in NEA. There is not an ideal method for treatment of long gap EA. Because of differences in patient selection and in gap measurement, different series are difficult to compare. Sepsis and severe associated anomalies still remain the major concern for a successful outcome. We are in agreement with others that the best tissue for esophageal reconstruction is esophageal tissue. In our series, no patient of 71 consecutive cases of EA needed esophageal replacement. All patients with long gap EA could be

7 1090 BAGOLAN ET AL treated successfully with a final result comparable with that of patients with normal gap EA. Thus, primary repair of long gap EA is always feasible as a standard repair. Cervical esophagostomy has to be avoided in newborns affected by long gap EA. Even if upper esophageal flap has not been uniformly successful in the literature as it was in our series, it represents a useful adjustment to the surgical armamentarium to help avoid 2-stage primary anastomosis with external traction, which would imply longer sedation and paralyzation. On these findings, other replacements using stomach, jejunum, or colon may be further abated in the future and reserved for babies in which a previous attempt of esophageal reconstruction failed. 1. Myers NA, Beasly SW, Auldist AW, et al: Oesophageal atresia without fistula-anastomosis or replacement. Pediatr Surg Int 2: , Spitz L, Ruangtrakool R: Esophageal substitution. Semin Pediatr Surg 2: , Giacomoni MA, Tresoldi M, Zamana C, et al: Circular myotomy of the distal oesophageal stump for long gap oesophageal atresia. J Pediatr Surg 36: , Davenport M, Bianchi A: Early experience with oesophageal flap oesophagoplasty for repair of oesophageal atresia. Pediatr Surg Int 5: , Foker JE, Linden BC, Boyle EM, et al: Development of a true primary repair for the full spectrum of oesophageal atresia. Ann Surg 226: , Kimura K, Nishijima E, Tsugawa C, et al: Multistaged extratoracic oesophageal elongation procedure for long gap oesophageal atresia: Experience with 12 patients. J Pediatr Surg 36: , Gross E: Atresia of the Esophagus. The Surgery of Infancy and Childhood. Philadelphia, PA, Saunders, 1953, pp Dessanti A, Caccia G, Iannuccelli M, et al: Use of Gore-Tex surgical membrane to minimize surgical adhesions in multistaged extrathoracic oesophageal elongation for esophageal atresia. J Pediatr Surg 35: , Holder TM, Aschcraft KW, Sharp RJ, et al: Care of infants with oesophageal atresia, tracheooesophageal fistula and associated anomalies. J Thorac Cardiovasc Surg 94: , Engum SA, Grosfeld MD, West MD, et al: Analysis of morbidity and mortality in 227 cases of oesophageal atresia and/or tracheoesophageal fistula over two decades. Arch Surg 130: , Louhimo L, Lindahl H: Esophageal atresia. Primary results of 500 consecutively treated patients. J Pediatr Surg 19: , Ein Sh, Shandling B, Heiss K: Pure esophageal atresia: Outlook in the 1990s. J Pediatr Surg 28: , Brown AK, Tam PKH: Measurement of gap length in oesophageal atresia: A simple predictor of outcome. J Am Coll Surg 182:41-45, Boyle EM Jr, Irwin ED, Foker JE: Primary repair of ultra-longgap oesophageal atresia: Results without a lengthening procedure. Ann Thorac Surg 57: , Hands LJ, Dudley NE: A comparison between gap length and Waterston classifications as guides to mortality and morbidity after surgery for oesophageal atresia. J Pediatr Surg 21: , Rescorla FR, West KW, Scherer LR III, et al: The complex nature of type A (long-gap) oesophageal atresia. Surgery 116: , Hagberg S, Rubenson A, Sillen U, et al: Management of longgap esophagus: Experience with end-to-end anastomosis under maximal tension. Prog Pediatr Surg 19:89-92, Lindhal H, Rintala R, Louhimo I: Oesophageal anastomosis REFERENCES without bouginage in isolated atresia Do the segment really grow while waiting? Z Kinderchir 42: , Stringer MD: Oesophageal substitution. Pediatr Surg Int 11:213, Spitz L: Gastric transposition for oesophageal replacement. Pediatr Surg Int 11: , Randolph JG: The reversed gastric tube for oesophageal replacement in children. Pediatr Surg Int 11: , Scharli AF: Esophageal reconstruction by elongation of the lesser gastric curvature. Pediatr Surg Int 11: , Raffensperger JG, Luck SR, Reynolds M, et al: Intestinal bypass of the esophagus. J Pediatr Surg 31:38-47, Davenport M, Hosie GP, Tasker RC, et al: Long-term effects of gastric transposition in children: A physiological study. J Pediatr Surg 31: , Schier F, Korn S, Michel E: Experience of a parent support group with long-term consequences of esophageal atresia. J Pediatr Surg 36: , Deurloo JA, van Lanschot JJB, Drillenburg P, et al: Esophageal squamous cell carcinoma 38 years after primary repair of esophageal atresia. J Pediatr Surg 36: , Appignani A, Lauro V, Prestipino M, et al: Intestinal bypass of the oesophagus: 117 patients in 28 years. Pediatr Surg Int 16: , Ruangtrakool R, Spitz L: Early complications of gastric transposition operation. J Med Assoc Thai 83: , Othersen B, Hebra A, Tagge EP: Esophageal replacement for atresia without fistula. Semin Pediatr Surg 7: , Macksood DJ, Blane CE, Drongowski RA, et al: Complications after gastric transposition in children. Can Assoc Radiol J 48: , Brown AK, Gough MH, Nicholls G, et al: Anterior flap repair of oesophageal atresia: A 16-year evaluation. Pediatr Surg Int 10: , Puri P, Khurana S: Delayed primary esophageal anastomosis for pure esophageal atresia. Semin Pediatr Surg 7: , Kimble RM, Harding JE, Kolbe A: The vulnerable stomach in babies born with pure oesophageal atresia. Pediatr Surg Int 15: , Puri P, Ninan GK, Blake NS, et al: Delayed primary anastomosis in oesophageal atresia: 18 months to 11 years follow-up. J Pediatr Surg 27: , Lipshutz GS, Albanese CT, Jennings RW, et al: A strategy for primary reconstruction of long gap oesophageal atresia using neonatal esophagoplasty: A case report. J Pediatr Surg 34:75-78, Yamataka A, Wang K, Kobayashi H, et al: Transplantation of newborn esophagus: An experimental study. J Pediatr Surg 36: , Vargus Gomez M: Esophageal replacement in patients under 3 months of age. J Pediatr Surg 29: , 1994