Online Exclusives
Esophageal atresia, small omphalocele and ileal prolapse through a patent omphalomesenteric duct: A methamizole embryopathy?
Newborns prenatally exposed to methimazole (active metabolite of carbamizole) for maternal hyperthyroidism may present some disorders in common, but the phenotype is not well defined. Choanal atresia is the most frequent, and other anomalies such as esophageal atresia and aplasia cutis were described with this embryopathy. Additionally, patent omphalomesenteric duct or Meckel's diverticulum in similar association was reported in some patients. The predisposed genetic background has to be considered.Esophageal atresia with proximal tracheoesophageal fistula: A missed diagnosis
This retrospective study was performed to compare the relative incidence of esophageal atresia (EA) with proximal tracheoesophageal fistula (PTEF) at our institution with those reported in literature and to test the hypothesis that our higher relative incidence is caused by the routine use of tracheoscopy.Foregut duplication cyst associated with esophageal atresia and tracheoesophageal fistula: A case report and literature review
A case of esophageal atresia associated with a foregut duplication cyst is reported and the literature reviewed. This is the first documented occurrence in conjunction with Down syndrome and the second case where both anomalies were treated at the initial surgery.Congenital bronchopulmonary foregut malformation initially diagnosed as esophageal atresia type C: challenging diagnosis and treatment
Communicating bronchopulmonary foregut malformations are extremely rare congenital malformations, characterized by a communicating fistula between an isolated part of the respiratory system and the esophagus or the stomach. In this article, we present a case of esophageal atresia type C, later diagnosed as a rare form of a communicating bronchopulmonary foregut malformation, an esophageal atresia combined with right main bronchus originating from the lower esophagus. Therapeutic resection of the right lung was complicated by postpneumonectomy syndrome.Eosinophilic esophagitis after esophageal atresia: is there an association? Case presentation and literature review
Eosinophilic esophagitis (EoE) is a relatively new condition resulting in dysphagia or symptoms resembling gastroesophageal reflux disease, symptoms that also are common in patients with a history of esophageal atresia. We present 2 patients with persistent dysphagia after repair of esophageal atresia that was caused by EoE. Although the exact etiology and pathogenesis of EoE remain unclear, it is now generally accepted that it is the result of a T-helper cell 2–type immune response with a crucial role for the eosinophil-specific chemotaxis factor eotaxin 3 and eosinophils.Novel use of glycopyrrolate (Robinul) in the treatment of anastomotic leak after repair of esophageal atresia and tracheoesophageal fistula
Anastomotic leak after tracheoesophageal fistula repair is a well-known complication and can represent a challenging clinical scenario. We present the case of an infant girl with VACTERL syndrome who underwent repair of a type C esophageal atresia and tracheoesophageal fistula repair, which was complicated by an anastomotic leak. Glycopyrrolate (Robinul), an anticholinergic agent, was successfully used to decrease copious salivary secretion and promote spontaneous closure of the leak. This report represents the first description in the medical literature of the use of glycopyrrolate in the treatment of an esophageal anastomotic leak.Dumping syndrome after esophageal atresia repair without antireflux surgery
In childhood, the surgical treatment of gastroesophageal reflux is the main cause of dumping syndrome. We report the cases of 2 children with esophageal atresia who presented with dumping syndrome without any precipitating known factors, such as gastroesophageal reflux surgery or associated microgastria. Our data suggest (1) that dumping syndrome can occur after primary anastomosis of esophageal atresia without antireflux surgery and (2) that dumping syndrome should be considered in every child treated surgically for esophageal atresia presenting with digestive symptoms, malaise, failure to thrive, or refusal to eat.Heterotopic pancreas of the esophagus and stomach associated with pure esophageal atresia
Esophageal atresia with or without tracheoeosphageal fistula is a frequent congenital malformation that may be associated with other congenital anomalies. The combination of heterotopic pancreas of the esophagus and pure esophageal atresia, however, is rare. We treated a patient with esophageal atresia without tracheoesophageal fistula (type A) associated with heterotopic pancreas of the esophagus and stomach who developed a gastric perforation.Tripartite esophagus
Esophageal atresia can exhibit many variations as a result of embryological derangements. We present a variation not previously described.Asymmetric sweating and flushing in infants with esophageal atresia
Of 136 infants with repaired esophageal atresia, one presented an unilateral facial flushing and 2 presented a flushing and sweating of one half of the body. The topography of these disorders and/or the associated clinical manifestations suggest that the asymmetry may be related to an instability of unilateral autonomic centers more than to a surgical injury of upper thoracic sympathetic chain during esophageal repair.Tracheal agenesis and esophageal atresia with proximal and distal bronchoesophageal fistulas
Tracheal agenesis (TA) is an extremely rare, typically fatal congenital tracheal malformation. Lack of prenatal symptoms and emergent presentation usually lead to a failure to arrive at the correct diagnosis and manage the airway properly before the onset of irreversible cerebral anoxia. Esophageal atresia (EA) encompasses a group of congenital anomalies comprising an interruption of the continuity of the esophagus with or without a persistent communication with the trachea. In 86% of cases, there is a distal tracheoesophageal fistula (TEF); in 7%, there is no fistulous connection, whereas in 4%, there is a TEF without atresia.Staged esophageal lengthening with internal and subsequent external traction sutures leads to primary repair of an ultralong gap esophageal atresia with upper pouch tracheoesophagel fistula
Primary repair of very long gap esophageal atresia (EA) with almost complete absence of thoracic esophagus has usually been thought impossible. Thus, esophageal replacement with colon or gastric interposition seemed inevitable. In contrast, J. Foker described a technique of lengthening the pouches with traction sutures and making primary repair possible. To contribute clinical experience to this discussion, we report about esophageal elongation in a child with long gap EA and an upper pouch tracheoesophageal fistula (TEF).Feingold syndome: a rare but important cause of syndromic tracheoesophageal fistula
Esophageal atresia (EA) and tracheoesophageal fistula (TEF) are common congenital malformations and are associated with additional anomalies in approximately half of cases. Feingold syndrome is an important genetic cause of syndromic EA-TEF to consider in patients with associated microcephaly and digital anomalies. We present a case report of a male infant with EA-TEF, microcephaly, subtle facial dysmorphism, dysplastic kidney, short fifth fingers, second finger clinodactyly, and increased spacing between the first and second toes bilaterally.Use of Polyflex Airway stent in the treatment of perforated esophageal stricture in an infant: a case report
Anastomotic stricture is a common sequela after primary repair of esophageal atresia with esophagoesophagostomy. Esophageal perforation secondary to dilatation of the stricture, and refractory stricture are not uncommon. We present a case using a Polyflex Airway stent (Boston Scientific, Natick, MA) as an alternative treatment of esophageal stricture and perforation in an infant.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.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.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.Development of an adenocarcinoma of the esophagus 22 years after primary repair of a congenital atresia
Esophageal cancer development after previous atresia repair is extremely rare in young patients. We present the clinical course of a patient who developed an adenocarcinoma of the esophagus at the age of 22 years, after repair of a tracheoesophageal fistula with esophageal atresia in the neonatal period. She developed a stricture of the esophageal anastomosis requiring frequent dilatations. Six years after an antireflux procedure because of a difficult treatable severe gastroesophageal reflux, an advanced adenocarcinoma was detected at the site of the end-to-end anastomosis of the previous atresia.Azygos lobe associated with esophageal atresia: a trap for the unwary
Presence of an associated azygos lobe may cause problems during thoracotomy on the right side for esophageal atresia. Awareness of the anomaly allows its recognition and appropriate management.High-grade congenital esophageal stenosis owing to a membranous diaphragm with tracheoesophageal fistula
Gross E–type congenital esophageal atresia associated with congenital esophageal stenosis is extremely rare. In a male infant born at 36 weeks of gestation, bubbly vomiting was noted after birth. X-ray films of the chest and abdomen showed coil-up sign of the nasogastric tube and gas in the stomach and small intestines were recognized, so gross C–type esophageal atresia was suspected and surgery was performed on the first day of life. Surgery revealed the presence of a tracheoesophageal fistula in the upper esophagus and membranous stenosis on the distal side.Esophageal atresia and severe respiratory failure—cuffed pediatric tracheal tubes as an additional therapeutic option?
This article describes the use of a cuffed pediatric endotracheal tube occluding selectively the air leakage resulting from tracheoesophageal fistula in a preterm neonate with esophageal atresia and severe respiratory failure due to respiratory distress syndrome. The gastric distension resolved completely within 4 hours. Surgical correction was performed on the third day of life after respiratory stabilization.
