Unusual variant of esophageal atresia and tracheo-esophageal fistula: A case report

Esophageal atresia (EA) is the most common congenital anomaly affecting the esophagus. We describe an unusual variant of EA, which shares similarities with Type A but features a mid-esophageal segment attached to the trachea. A 38-week gestational age baby girl had prenatal diagnoses of EA and congenital cardiac anomalies. Postnatal attempts to advance an orogastric tube were unsuccessful, and EA was confirmed by chest X-ray showing the tube at the T2/T3 level without distal bowel air. Further workup included bronchoscopy, which identified a posterior tracheal outpouching without an apparent connection to the esophagus, and Cardiac CT, which revealed a mid-esophageal segment with a tracheo-esophageal fistula. Workup was consistent with complex esophageal anatomy with distinct proximal and distal pouches and a mid-esophageal segment attached to the trachea. A gastrostomy tube was placed for decompression and enteral feeds. Surgical intervention included closure and excision of the mid-esophageal fistula. The gap between the proximal and distal pouches was impressive, requiring cervical esophageal mobilization and two esophageal myotomies to add length, and concluded in a primary esophago-esophagostomy. Postoperative complications included a contained leak at the anastomosis, which was managed conservatively with placement of a feeding tube past the anastomotic site and resolved after 4 weeks. The patient required multiple esophageal dilations during the first two years of life and achieved successful closure of the gastrostomy at 25 months of age without further complications. Awareness of rare subtypes of EA enables surgeons to anticipate and address unique challenges that may arise during surgical intervention.