Online Exclusives
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.Double H-type tracheoesophageal fistulas identified and repaired in 1 operation
Isolated tracheoesophageal (“H-type”) fistula is a relatively uncommon congenital anomaly that can be difficult to identify and, at times, challenging to repair. We present a very unusual case of an infant with 2 distinct H-type tracheoesophageal fistulas (TEFs) identified and repaired in 1 operation. A newborn male infant presented with coughing with feeds. Contrast esophagram demonstrated an intrathoracic H-type fistula without esophageal atresia. In the operating room, rigid bronchoscopy was performed, and a second TEF was identified in the cervical region.Flexible bronchoscopic cannulation of an isolated H-type tracheoesophageal fistula in a newborn
Congenital isolated H-type tracheoesophageal fistula (H-TEF) is a rare malformation of the airways. Surgery should not be delayed once the diagnosis is established. Identification of the fistula during surgery is a prerequisite for a successful outcome. Intubation or cannulation of the H-TEF with a catheter can help the surgeon to identify the fistula. A rigid bronchoscope is generally used for cannulation of the fistula. Cannulation of an H-TEF in a newborn with a flexible bronchoscope has the merit of simplicity and safety.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.Tripartite esophagus
Esophageal atresia can exhibit many variations as a result of embryological derangements. We present a variation not previously described.Neonatal appendicitis: a new look at an old zebra
Acute neonatal appendicitis is a rare condition associated with significant morbidity and mortality. The severity of this disease is caused by its tendency to occur more frequently in premature infants, an increased perforation rate with rapid progression to peritonitis, and delay in diagnosis and intervention. Although appendicitis in the perinatal period may occur as an isolated event, in many cases it occurs in association with other pathologic states, including prematurity, inguinal hernia, and others.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.Lung torsion after tracheoesophageal fistula repair: a case report and review of literature
Lung torsion is a very rare event that has been described after trauma, spontaneously, and post–thoracic surgery, with only 8 cases reported in the pediatric literature. We present the first case report of lung torsion complicating tracheoesophageal fistula repair. The diagnosis was suggested on chest ultrasonogram and Doppler and confirmed by computed tomographic scans. On exploration, a 90° rotation of the right middle and lower lobes in a clockwise direction was found. A complete interlobar fissure and an absent inferior pulmonary ligament were identified as predisposing factors.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.Tracheoesophageal fistula secondary to disk battery ingestion: a case report of gastric interposition and tracheal patch
The authors report a child with tracheoesophageal fistula secondary to disk battery ingestion. With respiratory compromise precluding expectant therapy and primary repair not achievable, gastric interposition and tracheal patch repair were undertaken in the acute phase. To the authors' knowledge, this is the first report of primary gastric interposition for traumatic tracheoesophageal fistula and suggest that immediate reconstruction can give an outcome at least as good as other reported approaches.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.Tracheoesophageal fistula in a child after blunt chest trauma
Traumatic tracheoesophageal fistula is a rare complication after blunt chest trauma, with all reported cases being more than 12 years of age. We report a 5-year-old boy with traumatic tracheoesophageal fistula after a blunt injury to the chest.Tracheal agenesis with unique anatomy
A premature infant with a unique form of tracheal agenesis is described. The combination of difficulty in intubation, abnormal course of the nasogastric tube on plain x-ray, and gastric perforation raised the suspicion of an upper airway malformation. Tracheal agenesis is an extremely rare, typically fatal, congenital anomaly with scattered case reports of its successful management. On many occasions, the diagnosis is a retrospective one at postmortem examination. The possibility of surgical correction rests on early diagnosis, anatomy, birth weight, and associated anomalies.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.Transillumination of H-type tracheoesophageal fistula using flexible miniature bronchoscopy: an innovative technique for operative localization
Precise localization of the fistula is the most important step in the operative strategy for dealing with H-type tracheoesophageal fistula. Bronchoscopic cannulation of the fistula with a Fogarty or ureteric catheter has been recommended to aid ready identification, but it is not always successful. We report an innovative technique that permitted localization of H-type fistula intraoperatively. A flexible pediatric 2.2-mm bronchoscope (Olympus BF Type N20) was steered through a standard endotracheal tube, and the fistula tract was illuminated, making its identification and subsequent repair straightforward.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.
