Long gap esophageal atresia: an Australian experience

Background

The diagnosis of long gap esophageal atresia (LGEA) may preclude immediate primary anastomosis. We reviewed our experience with this entity for a period of 10 years.

Methods

A retrospective review was undertaken of the medical records of all patients managed for esophageal atresia (EA)/tracheoesophageal fistula (TEF) during the period from 1991 to 2001 at the Children's Hospital at Westmead, Sydney, Australia. Esophageal atresia was defined as long gap when primary repair was considered technically impossible by the surgeon. Also, a questionnaire was sent to all the general pediatric surgeons in Australia to explore their attitude toward LGEA management.

Results

One hundred three patients with EA were managed for that period, 17 (16%) of them were defined as LGEA, with mean gap of 5 cm (SD, 1cm). Eight patients (47%) had TEF. Sixteen patients had gastrostomy tube (GT) insertion at a mean age of 4 days. Six patients had esophagostomy at a mean age of 27 days. Thirteen patients had EA repair at a mean age of 146 days. Four patients died before repair and 2 after repair secondary to associated anomalies. Fifty percent of Australian Pediatric Surgeons (APS) responded to the questionnaire. Forty percent defined LGEA as a gap more than 3 to 4 vertebral bodies, whereas 24% considered the absence of TEF as an indication of LGEA. Fifty-six percent of APS will measure the gap on preoperative chest x-rays, and 80% will assess the gap at thoracotomy for ligation of TEF. Ninety-two will measure the gap by inserting a bougie into the upper pouch and into the lower esophagus via the GT. If LGEA was diagnosed, all APS will perform GT with delayed repair. Seventy-two percent of APS will attempt delayed primary repair within 3 to 6 months of age. Seventy-six percent will perform hiatal dissection, and 48% will use upper pouch myotomies. Forty-eight percent will perform gastric pull up, and 32% will use gastric tube for esophageal replacement.

Conclusions

Long gap esophageal atresia represents a surgical challenge. Mortality rate is high secondary to associated anomalies. There is no consensus among APS regarding the definition of LGEA. In general, the consensus of APS would be that the preservation of the patient's own esophagus should be attempted before considering the use of an esophageal replacement.     

Oral feeding outcomes in infants with esophageal atresia and tracheoesophageal fistula

Purpose

The purpose of this study was to explore oral feeding outcomes in infants born with type-C esophageal atresia and tracheoesophageal fistula (EA/TEF).

Esophageal atresia and tracheoesophageal fistula with distal esophageal stenosis: preoperative diagnosis

This is a presentation of a case of preoperative diagnosis of apparent esophageal atresia and an apparent distal and tracheoesophageal fistula from a peripheral hospital. After surgery, histopathologic findings showed the absence of cartilage and bronchial glands, suggesting the etiology as a fibromuscular stenosis.     

Congenital laryngeal atresia associated with esophageal atresia and tracheoesophageal fistula: a case of long-term survival

Congenital laryngeal atresia (LA) is a life-threatening anomaly in which appropriate perinatal management is essential for survival. The authors report a neonate with LA associated with esophageal atresia (EA) and tracheoesophageal fistula (TEF) who was successfully resuscitated by emergent tracheostomy. Before birth, the patient had a diagnosis of EA based on the findings of polyhydramnios and absent stomach bubble. Immediately after birth, severe respiratory distress, cyanosis, and sternal retraction were evident. Because either endotracheal or esophageal intubation was unsuccessful, an emergent tracheostomy was performed. A direct laryngoscope revealed a subglottic atresia with normal appearance of the vocal cords. Repair of EA with TEF was performed on the third day of life, and the postoperative course was uneventful. In 2 years follow-up, the patient has no mental retardation nor central nervous impairment. Because of the presence of TEF, the antenatal ultrasonogram did not demonstrate the characteristic findings of the congenital high airway obstruction syndrome. This case represents one of the very few reported cases of successful resuscitation of a neonate with an unanticipated LA in which emergent airway management is required immediately after birth.     

Laryngotracheobronchoscopy prior to esophageal atresia and tracheoesophageal fistula repair—its use and importance

Background

Pure esophageal atresia (EA) and esophageal atresia with tracheoesophageal fistula (EA-TEF) are commonly associated with various anomalies. Associated anomalies, especially those of upper airways may alter the management strategies. This study was designed to find out the role of preoperative laryngotracheobronchoscopy (LTB) just prior to the standard surgical procedure.

Study design

This was a retrospective study. The data of all the newborn babies (n = 88) with a provisional diagnosis of EA or EA-TEF with preoperative rigid LTB, using 2.5/3.0/3.5 F rigid bronchoscope were analyzed. This additional procedure entailed documenting the abnormalities, endoscopic lavage and noting the site of the fistula. The fistula was cannulated by 3.0 F ureteric catheter just prior to the standard surgical procedure. Management strategies were changed as per the additional findings.

Results

Out of 88 patients, 77 had EA-TEF while 11 had pure EA. LTB was performed in all of them. Additional findings in bronchoscopy were noted in 18 (20.46%) babies. These additional findings were: fistula at unusual site in 12, laryngotracheal cleft in 2 and vallecular cyst in 1 neonate. The diagnosis of pure EA turned out to be EA-TEF in 3 cases. Unusual fistula sites were carinal/subcarinal in 4/12 (33.33%), upper pouch fistula in 1/12 (8.33%), double fistula in 2/12 (16.67%) and fistula from main bronchus in 5/12 (41.67%) cases.

Conclusions

LTB performed just prior to the definitive surgical procedure in EA and EA-TEF would diagnose, document and may aid in the surgical management strategies.     

Thoracoscopic repair of a type D esophageal atresia in a newborn with complex congenital heart disease

This report describes a case of thoracoscopic repair of esophageal atresia with a rare type D tracheoesophageal fistula in a child with complex congenital heart disease. We demonstrate the feasibility of thoracoscopic repair and anesthetic management in a child with complex congenital heart disease.     

Repair of esophageal atresia with tracheoesophageal fistula via thoracotomy: a contemporary series

Background

A recent series detailing thoracoscopic repair of esophageal atresia with tracheoesophageal fistula (EA/TEF) reported lower complication rates compared with historic controls. This study provides a contemporary cohort of patients repaired via thoracotomy for comparison with the recent large multi-institutional thoracoscopic series.

Methods

Records of patients with EA/TEF between 1993 and 2008 were reviewed. Attention was focused on demographics and complications including anastomotic leak, recurrent fistulae, stricture formation, and need for fundoplication.

Results

Seventy-two patients underwent repair of EA/TEF via thoracotomy. Complication rates in the current series compared with the thoracoscopic series were anastomotic leak, 2.7% versus 7.6%; recurrent fistulae, 2.7% versus 1.9%; stricture, 5.5% versus 3.8%; and need for fundoplication, 12% versus 24%. Differences in complication rates did not reach statistical significance. Two children in this cohort developed mild scoliosis attributed to congenital vertebral anomalies, neither of whom required intervention.

Conclusions

Thoracoscopic repair of EA/TEF yielded complication rates similar to this contemporary series; however, trends toward increased anastomotic leaks and greater need for fundoplication were noted. No musculoskeletal sequelae were directly attributable to thoracotomy.     

The effect of a right-sided aortic arch on outcome in children with esophageal atresia and tracheoesophageal fistula

During repair of an esophageal atresia with tracheoesophageal fistula (EA/TEF), a right-sided aortic arch (RAA) presents a technical challenge. We reviewed our experience with EA/TEF focusing on the impact of RAA.

Methods

A retrospective review of patients with EA/TEF at our institution from 1990 to 2004 was performed. Of 61 patients, 53 had a left aortic arch (LAA) and 8 had RAA.

Results

The mean gestational age and birth weight were similar between patients with LAA and RAA. There were more vascular rings in patients with RAA than with LAA (37.5% vs 3.8%, P = .009). A preoperative echocardiogram correctly identified the location of the arch in all with LAA but only 62.5% of those with RAA. Operative complications occurred in 25% of patients with RAA and 11% of patients with LAA.

Conclusions

Management of a child with RAA and EA/TEF is technically challenging. Preoperative identification of the great vessel anomalies may impact operative decision making. Therefore, careful diagnostic evaluation of a patient with an aortic arch that cannot be easily defined by ECHO should be considered before definitive repair.     

Repair of long gap esophageal atresia without anastomosis

Background/Purpose

Repair of long gap esophageal atresia represents a challenge. Several different techniques may be used. We describe 5 cases of long gap esophageal atresia managed successfully with suture approximation without anastomosis.

Methods

Retrospective review identified 5 newborns (4 males; 1 female) with long gap esophageal atresia treated with suture approximation and subsequent endoscopic and fluoroscopic placement of string for guided dilatations. Three babies had esophageal atresia without fistula, and 2 had the common type with proximal atresia and distal tracheoesophageal fistula. The babies with pure esophageal atresia had delayed repair, and those with the common type had repair 2 days after birth. All had a gastrostomy for feedings.

Results

All 5 babies recovered uneventfully. Three babies had spontaneous fistulization that allowed easy placement of guide wire and string. Two other babies required endoscopic and fluoroscopic combined fistula creation bypassing a long needle from the upper pouch to the lower one. Initially, all had string-guided dilatations that were subsequently converted to balloon dilatations. All babies had a functioning esophagus and did not need any further surgical intervention. An average of 8 postoperative dilatations were needed.

Conclusions

The baby's own functional esophagus is superior to any esophageal replacement. Familiarity with different techniques to preserve it is therefore important. Suture approximation without anastomosis is a safe technique that can be applied to long gap esophageal atresia. The downside of this technique is a prolonged hospital stay, multiple dilatations, prolonged fasting, and therapy to learn to eat orally.     

Thoracoscopic repair of esophageal atresia with distal fistula

Background: Esophageal atresia (EA) has always been considered the hallmark of pediatric surgery. In the past decade, mortality was primarily the result of associated diseases, and operative morbidity had greatly improved. Yet the consequences of opening the thoracic cavity remained unchanged. In the era of endoscopic surgery, a thoracic approach to EA has become feasible, but is it of benefit for the patient? Methods: Between May 2000 and June 2002, 13 neonates underwent thoracoscopic repair of EA. There were 12 boys and 1 girl. Mean gestational age was 36.9 weeks. Mean weight was 3093 g. Eleven children had associated anomalies. Results: All of the procedures were performed thoracoscopically. There were no intraoperative complications, although anastomosis was difficult in one patient due to an extensive distance between the two stumps. Mean operating time was 2.6 h (range, 1.45–3.5). Five short-term postoperative complications occurred. Four of the early patients had stenosis due to a too-small incision in the proximal pouch, which needed one or more dilatations. One of these children, as well together as one other child, had anastomotic leakage, which was treated conservatively. Late complications consisted of gastroesophageal reflux (n = 5) and tracheomalacia (n = 1); these conditions required endoscopic correction in, respectively, two and one cases. Feeding by nasogastric tube was started after 3.5 days (mean), and total oral feeding was possible after 8.6 days (mean). Mean hospitalization was 12.2 days. Mean follow-up was 15.2 months. Scar formation was minimal, and the thoracic cage was preserved. Conclusion: The feasibility of thoracoscopic repair of EA has already been demonstrated. Today, its results in terms of operating time, feeding, hospital stay, and postoperative complications are equal to open procedures. Its advantages include better cosmesis and preservation of the thorax.     

Triad of tracheoesophageal fistula-esophageal atresia, pulmonary hypoplasia, and duodenal atresia

We describe 3 cases of tracheoesophageal fistula with esophageal atresia who also had right pulmonary hypoplasia-aplasia and duodenal atresia. We review the current literature and propose that this may be a distinct association.     

Long-term analysis of children with esophageal atresia and tracheoesophageal fistula

Background/purpose

For children with esophageal atresia (EA) or tracheoesophageal fistula (TEF), the first years of life can be associated with many problems. Little is known about the long-term function of children who underwent repair as neonates. This study evaluates outcome and late sequelae of children with EA/TEF.

Methods

Medical records of infants with esophageal anomalies (May 1972 through December 1990) were reviewed. Study parameters included demographics, dysphagia, frequent respiratory infections (> 3/yr), gastroesophageal reflux disease (GERD), frequent choking, leak, stricture, and developmental delays (weight, height < 25%, < 5%, respectively).

Results

Over 224 months, 69 infants (37 boys, 32 girls) were identified: type A, 10 infants; type B, 1; type C, 53; type D, 4; type E, 1. Mean follow-up was 125 months. During the first 5 years of follow-up, dysphagia (45%), respiratory infections (29%), and GERD (48%) were common as were growth delays. These problems improved as the children matured.

Conclusions

Children with esophageal anomalies face many difficulties during initial repair and frequently encounter problems years later. Support groups can foster child development and alleviate parent isolationism. Despite growth retardation, esophageal motility disorders, and frequent respiratory infections, children with EA/TEF continue to have a favorable long-term outcome.     

Outcomes in esophageal atresia and tracheoesophageal fistula

Background/Purpose: The purpose of this analysis was to investigate outcomes in newborns with esophageal atresia (EA) or tracheoesophageal fistula (TEF) with respect to prognostic classifications and complications.Methods: Charts of all 144 infants with EA/TEF treated at British Columbia Children’s Hospital (BCCH) from 1984 to 2000 were reviewed. Patient demographics, frequency of associated anomalies, and details of management and outcomes were examined.Results: Applying the Waterston prognostic classification to our patient population, survival rate was 100% for class A, 100% for class B, and 80% for class C. The Montreal classification survival rate was 92% for class I and 71% for class II (P = .08). Using the Spitz classification, survival rate was 99% for type I, 84% for type II, and 43% for type III (P < .05). The Bremen classification survival rate was 95% “without complications” and 71% “with complications.” Complications included stricture (52%), gastroesophageal reflux (31%), anastomotic leakage (8%), recurrent fistula (8%), and pneumonia (6%). Seventeen patients underwent fundoplication for gastroesophageal reflux, 16 pre-1992 and one post-1992.Conclusions: Comparing the major prognostic classifications, the Spitz classification scheme was found to be most applicable. In our institution, the trend in management of gastroesophageal reflux after repair of EA/TEF has moved away from fundoplication toward medical management.     

Supraclavicular approach to cervical esophageal atresia with tracheoesophageal fistula

This is a report of a patient with esophageal atresia with a blind upper pouch and a lower pouch fistula, both in the neck. The preoperative radiologic appearance of an air-filled esophagus behind the trachea suggested a higher than normal fistula. Preoperative bronchoscopy confirmed the cervical position of the fistula and, as a result, a supraclavicular incision was used to approach both the atresia and fistula.     

Complete vs partial fundoplication in children with esophageal atresia

Purpose

The purpose of the study was to compare outcomes after partial vs complete fundoplication in patients with prior esophageal atresia repair.

Methods

All patients undergoing fundoplication following esophageal atresia repair at a tertiary care pediatric hospital from 1987 to 2006 were retrospectively reviewed. All children had at least 1 year of follow-up postfundoplication.

Results

Of 47 children, 31 (66%) had a partial fundoplication and 16 (34%) had complete fundoplication. Demographics, presence of tracheoesophageal fistula, early complications of esophageal atresia repair, gastroesophageal reflux symptoms before fundoplication, and operative details of fundoplication were statistically similar between groups, except for the frequency of hiatus repair during fundoplication (23% vs 69%, P = .004). Patients were followed for a median of 4.98 years (range, 1-17.8 years). Postfundoplication symptoms of vomiting (39% vs 31%), dysphagia (45% vs 38%), retching (10% vs 25%), abnormal findings on barium study, and need for reoperation (19% vs 13%) were not statistically different between groups. However, a greater proportion of children undergoing partial fundoplication achieved long-term symptom- and medication-free recovery (52% vs 13%, P = .012).

Conclusions

Our data suggest that partial fundoplication is associated with a greater likelihood of symptom- and medication-free recovery than complete fundoplication in children with previously repaired esophageal atresia.     

The management of long gap esophageal atresia

Background

Present management of esophageal atresia has enabled the survival rate to approach 95%. Controversy remains concerning the many options for the surgical management of long gap esophageal atresia without tracheoesophageal fistula and represents the difficulty of this pathology. In the last couple of years, we have had a nonexplained outbreak of cases of long gap esophageal atresia without tracheoesophageal fistula. This article reports our experience in the management of these children.

Material and Methods

It is a retrospective study of all cases of long gap esophageal atresia without tracheoesophageal fistula managed in our institution since 1992, focusing on the antenatal period, delivery with weight and term, the associated malformations, the initial management, and the definitive surgery. Mann-Whitney U test was used for statistical analysis.

Results

Ten cases (8.7%) of long gap esophageal atresia according to Ladd's classification, 6 during the past 2 years, were taken in charge at Robert Debré Hospital between 1992 and 2002. There were 4 girls and 6 boys. Ten had a prenatal diagnosis of esophageal atresia. The average birth weight was 2496 g (range, 1400-3400 g) with an average term of 36.6-week gestation (range, 31.5-39.6). Delayed reconstruction was done in all children between 41 and 147 days of life (average of 102 days). Six had a direct anastomosis and 4 had a colonic esophagoplasty (3 with an esogastric disconnection during the same procedure). The average follow-up was 60 months (range, 27-133). There was 1 death owing to adenovirus infection at 5 years of age. Four children required a Nissen fundoplication for severe gastroesophageal reflux. At least, 2 children presented an anastomotic stricture which required pneumatic dilatations.

Conclusion

Treatment options for long gap esophageal atresia generally require several stages over several months. We propose, for their management, a direct anastomosis at 4 months of age whenever it is possible. If not, we use a colonic esophagoplasty with an esogastric disconnection to control the gastroesophageal reflux which is responsible for strictures and respiratory impairment and does not obstruct the aperistaltic tube.     

Esophageal atresia without distal tracheoesophageal fistula: high incidence of proximal fistula

Background

This retrospective study was performed to test our suspicion that the incidence of esophageal atresia with proximal fistula in our institution is much higher than is generally reported.

Methods

The charts of all patients with esophageal atresia and/or tracheoesophageal fistula admitted in the period 1982 to 2000 were analyzed. The type of atresia and/or tracheoesophageal fistula was noted, and the relative incidence was calculated and compared with the relative incidence in a cumulative series of 3492 patients taken from 9 published studies.

Results

In the period under study, 123 patients with esophageal atresia and/or tracheoesophageal fistula were identified. The relative incidence of esophageal atresia without distal fistula was statistically not different (10.6% in the present series against 8.49% in the reference group). A statistically significant difference in the relative incidence of esophageal atresia with proximal fistula, however, was found: 5.69% in the present series against 1.05% in the reference group (P < .0001). Looking at the subgroup of patients without a distal fistula, more than half of the patients did have a proximal fistula.

Conclusions

The relative incidence of esophageal atresia with proximal fistula in this series of children with esophageal atresia and/or tracheoesophageal fistula is significantly higher than reported in the literature. This is on the account of the subgroup of patients without a distal fistula in which the incidence of a proximal fistula is more than 50%. Especially in this subgroup, the existence of a proximal fistula should be ruled out preoperatively.     

Potential hazards of contrast study diagnosis of esophageal atresia

Delay in the diagnosis of esophageal atresia (EA) is rare. We present a child with EA and distal tracheoesophageal fistula who was diagnosed 9 days from birth after a contrast study performed at the referring hospital. This article aims to highlight the potential hazards of using contrast to diagnose EA.     

MSCT及后处理技术诊断食管闭锁并气管食管瘘

目的探讨MSCT及后处理技术诊断先天性食管闭锁合并气管食管瘘(EA-TEF)的价值。方法收集8例临床疑诊为EA-TEF的新生儿,于手术前行食管造影及MSCT扫描并进行重建,重建方法包括MPR、MinIP、VR等;将术前MSCT诊断结果与手术结果进行对照研究。结果 8例中,1例为EA-TEFⅠ型,7例为Ⅲ型(其中Ⅲa型4例,Ⅲb型3例);5例伴有其他先天性畸形。利用MPR、VR重建图像测得的闭锁食管上下盲端的距离与手术结果基本相符,7例仿真内镜图像能明确显示瘘管开口。结论 MSCT及其后处理技术可以准确诊断EA-TEF并进行分型,显示闭锁食管上下盲端情况及气管-食管瘘口位置可靠,还能显示肺部病变及其他伴随畸形。