The absence of clinically significant tracheomalacia in patients having esophageal atresia without tracheoesophageal fistula.

The association of tracheomalacia (TM) with esophageal atresia (EA) has been well described. This study attempted to find a correlation between the severity of TM and the presence or absence of an associated tracheoesophageal fistula (TEF) in patients with EA. A review of all patients presenting at this institution with EA through the years 1970 to 1990 was carried out. Six patients with EA without fistula (type A) and 61 patients having EA with fistula (types B, C, D, and E) were analyzed. Five of the six type A patients required esophageal dilatations and antireflux procedures; none had clinically significant TM. Thirty-five of the 61 patients with fistula required esophageal dilatation, and 8 had an antireflux procedure; 11 (18%) required either long-tube tracheostomy or aortopexy for TM. This small study supports a recent hypothesis of different embryological events resulting in different types of esophageal and tracheal anomalies. When EA occurs without fistula, it appears that no significant alteration in tracheal development occurs.     

Triple fistula: management of a double tracheoesophageal fistula with a third H-type proximal fistula

Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is a relatively common congenital condition in which there have been several described anatomical variants. The most common type, EA with distal TEF, comprises more than 75% of cases in many reports. Less commonly, a smaller proximal pouch fistula (H-type) will be associated with this most common variant in 1.4% of these cases. Only 2% of all cases of EA/TEF will have 2 large fistulas between the trachea and esophagus in which the end of the upper esophageal pouch connects terminally to the midtrachea and the distal esophagus arises from the trachea near the carina. Here we describe the management of an infant with this type of EA/TEF who was also found to have an H-type TEF of the proximal trachea. The combination of this type of EA/TEF with an associated H-type TEF or “triple fistula” has been previously described in the literature in only 1 other patient.     

Successful thoracoscopic repair of esophageal atresia with tracheoesophageal fistula in a newborn with single ventricle physiology

A neonate with VACTERL association including tricuspid atresia was scheduled for thoracoscopic esophageal atresia with tracheoesophageal fistula (EA/TEF) repair and laparoscopic gastrostomy tube placement. In addition to standard noninvasive monitoring, arterial blood pressure, central venous pressure, and cerebral oxygen saturation were monitored. Gastric distension resulting from positive pressure ventilation prevented laparoscopic gastrostomy tube placement. Thoracoscopy with a CO2 insufflation pressure of 6 mm Hg at low flow (1 L/min) was well tolerated hemodynamically despite hypercarbia and cerebral oxygen saturation was maintained. Careful monitoring and good communication were critical to the safe management of this single ventricle patient during thoracoscopic EA/TEF repair. IMPLICATIONS: Esophageal and tracheoesophageal fistula in conjunction with single ventricle physiology carries a significant risk of mortality. We present the anesthetic management of a neonate with unpalliated tricuspid atresia who underwent thoracoscopic tracheoesophageal fistula repair.     

A new variant of esophageal atresia with distal tracheo-antral fistula associated with congenital intrathoracic stomach and situs inversus

Esophageal atresia (EA) with distal tracheoesophageal fistula (TEF) is the most common anatomic pattern within congenital anomalies of the esophagus. Also, more than 50% of the infants with esophageal atresia have other congenital malformations. To our knowledge, this is the first case report of EA with distal tracheo-antral fistula associated with congenital intrathoracic stomach and situs inversus (SI).     

A gasless abdomen does not exclude distal tracheoesophageal fistula: the value of a repeat x-ray

A “gasless abdomen” in babies with esophageal atresia (EA) has traditionally been thought to imply absence of a distal tracheoesophageal fistula (TEF). We present 3 neonates with a provisional diagnosis of EA, who had a gasless abdomen on an initial x-ray taken within the first 4 hours of life. These children were subsequently shown to have gas in the stomach associated with a distal TEF. Two children were examined on subsequent repeat x-ray of the abdomen, when gas was demonstrated in the stomach. Another child had a laparotomy for a gastrostomy when a distended stomach was found. This baby went on to have a thoracotomy and a routine repair of the distal TEF and an esophageal anastamosis. Based on our data of 65 cases of EA and distal TEF, it is recommended that an x-ray of the abdomen is repeated before surgery is undertaken if the gasless abdomen is documented during the first 4 hours of life.     

The use of biosynthetic mesh to separate the anastomoses during the thoracoscopic repair of esophageal atresia and tracheoesophageal fistula

Recurrent tracheoesophageal fistula following the repair of esophageal atresia and tracheoesophageal fistula (EA/TEF) is a difficult complication to manage, which makes prevention the dominant concern of surgeons performing the primary repair. To this end, the surrounding pleural tissues are usually brought over the tracheal closure to prevent the development of a recurrence during the open repair. This maneuver is not usually feasible when using the thoracoscopic approach. Therefore, in this paper, we describe a case in which we interposed a biosynthetic mesh between the esophageal and tracheal suture lines during the thoracoscopic repair of EA/TEF on a 2.9-kg newborn girl.     

Outcomes of multi-gestational pregnancies affected by esophageal atresia – tracheoesophageal fistula

BackgroundContemporary outcomes of infants with esophageal atresia with or without tracheoesophageal fistula (EA/TEF) from multi-gestational pregnancies compared to those of singleton pregnancies have not been reported.MethodsA single-center retrospective review of EA/TEF patients born from 1999 to 2013 was performed. Patient demographics, gestational age (GA), birth weight, associated anomalies, requirement for gastrostomy tube and mortality were reviewed.ResultsSingleton EA/TEF patients outnumbered those from multi-gestational pregnancies nearly 10:1 (214 vs 22 patients). EA/TEF patients from multi-gestational pregnancies were more likely to be premature (77% vs. 32%), have lower birth weight (mean 1766 g vs. 2695 g), have associated duodenal atresia (18% vs. 6%) and require gastrostomy tube (41% vs. 33%) for feeding challenges compared to EA/TEF singletons. Mortality was also significantly greater for multi-gestational EA/TEF patients compared to singleton EA/TEF patients (18% vs. 6%). Conclusion: EA/TEF infants from multi-gestational pregnancies have greater clinical complexity and mortality than singleton EA/TEF patients. Parents of EA/TEF multi-gestational infants should be appropriately counseled and supported.     

Thoracoscopic repair of esophageal atresia and tracheoesophageal fistula: a multi-institutional analysis

OBJECTIVES: For the past 60 years, successful repair of esophageal atresia (EA) and distal tracheoesophageal fistula (TEF) has been performed via a thoracotomy. However, a number of reports have described adverse musculoskeletal sequelae following thoracotomy in infants and young children. Until now, only a few scattered case reports have detailed an individual surgeon's success with thoracoscopic repair of EA/TEF. This multi-institutional review represents the largest experience describing the results with this approach. METHODS: A cohort of international pediatric surgeons from centers that perform advanced laparoscopic and thoracoscopic operations in infants and children retrospectively reviewed their data on primary thoracoscopic repair in 104 newborns with EA/TEF. Newborns with EA without a distal TEF or those with an isolated TEF without EA were excluded. RESULTS: In these 104 patients, the mean age at operation was 1.2 days (+/-1.1), the mean weight was 2.6 kg (+/-0.5), the mean operative time was 129.9 minutes (+/-55.5), the mean days of mechanical ventilation were 3.6 (+/-5.8), and the mean days of total hospitalization were 18.1 (+/-18.6). Twelve (11.5%) infants developed an early leak or stricture at the anastomosis and 33 (31.7%) required esophageal dilatation at least once. Five operations (4.8%) were converted to an open thoracotomy and one was staged due to a long gap between the 2 esophageal segments. Twenty-five newborns (24.0%) later required a laparoscopic fundoplication. A recurrent fistula between the esophagus and trachea developed in 2 infants (1.9%). A number of other operations were required in these patients, including imperforate anus repair in 10 patients (7 high, 3 low), aortopexy (7), laparoscopic duodenal atresia repair (4), and various major cardiac operations (5). Three patients died, one related to the EA/TEF on the 20th postoperative day. CONCLUSIONS: The thoracoscopic repair of EA/TEF represents a natural evolution in the operative correction of this complicated congenital anomaly and can be safely performed by experienced endoscopic surgeons. The results presented are comparable to previous reports of babies undergoing repair through a thoracotomy. Based on the associated musculoskeletal problems following thoracotomy, there will likely be long-term benefits for babies with this anomaly undergoing the thoracoscopic repair.     

Management of patients with combined tracheoesophageal fistula,esophageal atresia,and duodenal atresia

INTRODUCTION

Patients with combined esophageal atresia (EA), tracheoesophageal fistula (TEF), and duodenal atresia (DA) pose a rare management challenge.

PRESENTATION OF CASE

Three patients with combined esophageal atresia (EA), tracheoesophageal fistula (TEF), and duodenal atresia safely underwent a staged approach inserting a gastrostomy tube and repairing the EA/TEF first followed by a duodenoduodenostomy within one week. None of the patients suffered significant pre- or post-operative complications and our follow-up data (between 12 and 24 months) suggest that all patients eventually outgrow their reflux and respiratory symptoms.

DISCUSSION

While some authors support repair of all defects in one surgery, we recommend a staged approach. A gastrostomy tube is placed first for gastric decompression before TEF ligation and EA repair can be safely undertaken. The repair of the DA can then be performed within 3–7 days under controlled circumstances.

CONCLUSION

A staged approach of inserting a gastrostomy tube and repairing the EA/TEF first followed by a duodenoduodenostomy within one week resulted in excellent outcomes.     

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.     

Esophageal atresia with double tracheoesophageal fistula

Ninety cases of esophageal atresia (EA) with double tracheoesophageal fistula (TEF) from the literature and 4 new cases are examined. The incidence may be more common than is generally recognized with incidences up to 5.3% reported. The pre-, intra-, and postoperative diagnosis may be difficult, and half have been missed initially with almost half of these being first recognized at autopsy. During repair of EA and TEF the proximal esophagus should be mobilized looking for a proximal fistula. Many proximal TEF are missed at the initial operation and discovered in the postoperative period. The symptoms, diagnosis and treatment of the unrecognized proximal TEF are similar to that for isolated TEF. Results should be good since this problem occurs in large babies with fewer and less complex associated conditions.     

Surgical problems in patients with VATER-associated anomalies

From 1960 through 1980, 14 patients with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) with two or more other associated anomalies of VATER have been seen. Seventy percent of patients were preterm or low in birth weight. In addition to the esophageal abnormality, vertebral anomalies were found in 7 patients, anorectal anomalies in 11, limb anomalies in 9 and renal anomalies in 9 patients. Three patients had associated duodenal atresia and 4 had cardiac anomaly. Eight patients are dead, 5 are living, and 1 is lost to follow-up. Six of the eight deaths occurred during 1960 to 1970. Preliminary gastrostomy was done in all patients. Primary esophageal repair was carried out in 9 patients--5 died and 4 survived. Three patients had delayed esophageal repair and two of these patients survived. Cervical esophagostomy was done in one patient with EA without TEF after unsuccessful repeated bouginage for elongation of the upper esophageal pouch. Three of the survivors showed growth retardation--below 5th percentile. Aggressive nutritional support and management of chronic problems related to the anomalies are important.     

Appendiceal Mass in a Neonate After Surgery for Esophageal Atresia and Tracheoesophageal Fistula: Report of a Case

A 23-day-old girl presented with abdominal distension and vomiting. She had been previously operated on for esophageal atresia and tracheoesophageal fistula (EA/TEF) when she was 2 days old. An immediate laparotomy revealed an appendiceal mass caused by perforated appendicitis. The occurrence of appendicitis and an appendiceal mass is extremely rare in neonates and this may be the first such report in the world literature.     

Coexisting congenital diaphragmatic hernia, esophageal atresia, and tracheoesophageal fistula: a case report and review of the literature

Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) and congenital diaphragmatic hernia (CDH) are two relatively common neonatal conditions. The coexistence of the two conditions in the same newborn is extremely rare. This is a case report of a newborn with CDH and concurrent EA and TEF. Aspects of diagnosis and the literature on the subject are also reviewed.     

Tracheoesophageal fistula and associated congenital heart disease: implications for anesthetic management and survival

BACKGROUND: Infants with tracheoesophageal fistula (TEF) and/or esophageal atresia (EA) frequently have other associated congenital anomalies which can have a significant impact on their anesthetic care and survival to discharge. METHODS: A medical record review and retrospective data analysis were performed in a university affiliated children's hospital of all infants undergoing TEF/EA repair between January 1998 and July 2004. The incidence of intraoperative complications during the TEF repair and overall survival to hospital discharge was compared in two groups of infants: 26 patients with TEF/EA and coexisting congenital heart disease (CHD), and 27 patients with TEF/EA and no CHD. RESULTS: The overall incidence of intraoperative critical events during repair of TEF/EA was significantly higher in infants with associated cardiac pathology (P = 0.003). Six of 53 infants died during hospitalization (overall mortality, 11.3%) and all had associated cardiac pathology. In comparison with nonductal-dependent lesions, the presence of a ductal-dependent cardiac lesion appeared to significantly increase patient mortality (57% vs. 10%, P = 0.028). CONCLUSIONS: Low birth weight (<1500 g) and associated cardiac pathology were found to be independent predictors of mortality in infants undergoing surgery for TEF/EA repair. The presence of a ductal-dependent cardiac lesion further increased the risk of morbidity and mortality, in addition to necessitating special anesthesia considerations.     

Congenital esophageal atresia successfully treated by early ligation of a tracheoesophageal fistula and delayed repair of the esophagus in a premature infant: Report of a case

We report herein the case of a premature infant with esophageal atresia (EA) and a tracheoesophageal fistula (TEF) associated with cardiac anomalies who was successfully treated by an early ligation of the TEF following gastrostomy, and delayed repair of the esophagus. A 1212-g male was born prematurely at 31 gestational weeks, at which time he was diagnosed as having EA with TEF and patent ductus arteriosus (PDA), ventricular septal defect (VSD), and atrial septal defect (ASD). A gastrostomy was initially performed but following extubation he gradually became tachypneic. A chest roentogenogram revealed atelectasis and ground-glass appearance, and reintubation was required. Ligation of the TEF was performed 53h after his birth. Following the improvement of his respiratory condition through ventilatory support and the intratracheal administration of pulmonary surfactant, he underwent repair of the esophagus on the 6th day of life. Postoperatively, he suffered from heart failure, but was treated with peritoneal dialysis and pharmacological closure of the PDA. Weaning the infant from the ventilator proved difficult, but it was finally achieved when he had reached a weight of 2268g at 3 months of age by enteral feeding. Our experience of this case demonstrates that early ligation of TEF should be performed for a premature infant with EA and TEF before respiratory distress syndrome (RDS) has developed. If a gastrostomy is required to prevent gastric distention, it should be followed by simultaneous or immediate ligation of the TEF.     

Tracheoesophageal fistula after primary repair of type C esophageal atresia in the neonatal period: recurrent or missed second congenital fistula

Purpose

The aim of the study is to investigate whether a tracheoesophageal fistula (TEF) found after the primary repair of type C esophageal atresia (EA) is a recannulation of the original fistula, a missed proximal fistula, or other rare foregut malformation.

Methods

Between 2000 and 2009, 143 different types of patients with EA were admitted in our hospital. Seven patients (2 from our series, 5 referred to us by other hospitals with the history of primary repair of type C EA) had late presenting TEF. Esophagogram, 3-dimensional computed tomographic (CT) reconstruction, bronchoscopy, and reoperation were performed to confirm the TEF. Their medical records were reviewed and summarized.

Results

Persistent feeding or respiratory problems were the common symptom. The mean age of the first appearance was 17 ± 26 (1-63) months. Preoperative diagnosis was made by esophagograms and bronchoscopy in 6 patients. Reoperations were performed in all patients through thoracotomy. Missed proximal TEF shown as a distinct fistula above the primary anastomosis without much adhesion was confirmed in 5 cases. A recurrent TEF was found in 1 case. A case of communicating bronchopulmonary foregut malformation was confirmed by 3-dimensional CT reconstruction and reoperation.

Conclusion

A missed proximal TEF after repair of EA may be misdiagnosed as a recurrent TEF. Accurate preoperative diagnosis depends on combined evaluations of radiologic contrast study, 3-dimensional CT, and bronchoscopy.     

Health-related quality of life in adult patients with esophageal atresia--a questionnaire study

Background/Purpose

Long-term sequelae caused by associated anomalies or respiratory and gastrointestinal disorders are common after the repair of esophageal atresia (EA). The aim of this study was to assess the effect of these sequelae on the health-related quality of life (HRQoL) of patients with EA.

Methods

A questionnaire including a 36-item Gastrointestinal Quality of Life Index, a 15-item Respiratory Symptoms-Related Quality of Life Index, and a 36-item psychosocial survey and a symptoms query was sent to 159 patients with EA with or without tracheoesophageal fistula (TEF), who were operated on between 1949 and 1979, and to 400 healthy control subjects. A 36-item Health Survey Form was sent to patients with EA only and the results were compared with values of the general Finnish population.

Results

A total of 128 patients with EA (80%) and 162 control subjects (41%) returned the questionnaire. Median age [patients with EA, 38 (range, 24-54) years; control subjects, 36 (20-56) years] and sex distribution (M/F of patients with EA, 57:70; control subjects, 63:99) were comparable. Of the 128 patients, 115 (91%) had EA with a distal TEF, 8 (6%) had EA without TEF, 3 (2%) had EA with proximal and distal TEF, and 2 (1%) had TEF without EA. The types of esophageal repair were distributed as follows: primary end-to-end anastomosis (n = 112), colon interposition (n = 10), gastric tube (n = 3), thoracic skin tube (n = 1), and fistula closure only (n = 2). Thirty-eight patients had significant associated anomalies. Gastrointestinal Quality of Life Index scores did not differ statistically between patients with EA and control subjects. However, the incidence of regurgitation (17% vs 10%) and dysphagia (10% vs 2%) was significantly higher in patients with EA (P < .5). Low Respiratory Symptoms-Related Quality of Life Index was found in 10 of 128 patients with EA and in 3 of 163 control subjects (P < .05). Psychosocial survey scores and the incidence of acquired diseases did not differ between the groups (P = NS). The 36-item Health Survey Form indicated low HRQoL in 19 (15%) of patients with EA (expected value, 16%). Of the 19 patients with EA with low HRQoL, 8 (42%) had significant congenital or EA-associated diseases and 11 (58%) had acquired diseases. The type of EA or esophageal conduit was not related to HRQoL.

Conclusions

Most adult survivors of EA or TEF repair have a normal quality of life. Morbidity from esophageal functional disorders and respiratory disorders with or without acquired diseases impairs HRQoL in 15% of patients with EA.     

A new variant of esophageal atresia associated with esophageal heterotopic pancreas

An infant with esophageal atresia (EA) and absence of gas on abdominal radiographs was found to have an obliterated distal tracheoesophageal fistula (TEF). Preoperative bronchoscopy and surgical exploration found that the reason for gasless abdomen was the atresia of the distal portion of lower esophagus, which also contained heterotopic pancreatic tissue. The type of trachea/esophageal anomaly found in this patient, with the association of esophageal heterotopic pancreas (EHP), has not yet been reported in the literature.     

Vocal cord dysfunction following esophageal atresia and tracheoesophageal fistula (EA/TEF) repair

BackgroundThe purpose of this study was to determine risk factors and long-term outcomes in patients with esophageal atresia +/?tracheoesophageal fistula (EA/TEF) with vocal cord dysfunction (VCD) owing to recurrent laryngeal nerve (RLN) injury.MethodA retrospective chart review was performed for EA/TEF patients repaired at our institution from 1999 to 2014 (REB #1000032265).ResultsOf 197 patients, 22 (11.2%) were diagnosed with VCD by indirect laryngoscopy following EA/TEF repair. Aspiration was documented on video swallow study for 21 patients, and as a result, 13 required thickened feeds and 8 required gastrostomy tube feeds. Of the 16 H-type TEF patients, 8 (50%) had VCD. Following discharge, 20 (90.9%) patients with VCD eventually tolerated full feeds orally without aspiration but only 8 (36.4%) had documented recovery of vocal cord movement at long-term follow up (mean 452 days). Overall, patients with VCD were more likely to have feeding modifications, increased exposure to radiological studies, and increased frequency of Otolaryngology follow-up compared to EA/TEF patients without VCD.ConclusionEA/TEF patients are at risk for VCD. Clinical improvement did not always correlate with recovery of VC motion. Strategies to minimize RLN damage will improve outcomes and quality of life for EA/TEF patients.Level of evidenceLevel III.