Long proximal pouch oesophageal atresia with tracheo-oesophageal fistula, a diagnostic dilemma: case report

Background: Oesophageal atresia is a congenital abnormality commonly encountered in neonates. Classification proposed by Gross is the most frequently used. It divides the oesophageal anomalies into five types. Type C-proximal oesophageal atresia and distal tracheo-oesophageal fistula-is the most common. Dafoe and Ross described a rare subtype with a long proximal pouch. Only 11 cases of a long proximal pouch have been reported in the English literature so far. We describe a neonate with long proximal pouch oesophageal atresia with distal tracheo-oesophageal fistula emphasizing the difficulty in arriving at the diagnosis. Case presentation: A 1-week-old neonate presented with feed intolerance and failure to advance orogastric tube into the stomach. Water soluble upper gastrointestinal tract contrast revealed a blind ending proximal oesophagus at the level of the diaphragm. Gastric volvulus was considered as a diagnosis. Patient underwent a laparotomy where a normal stomach was found. Bubbles were seen coming from the fashioned gastrostomy with each inspiration. This prompted us to consider the possibility of a missed oesophageal atresia with distal tracheo-oesophageal fistula. Diagnosis was confirmed on bronchoscopy and right thoracotomy. The tracheo-oesophageal fistula was ligated and a cervical oesophagostomy and Stamm gastrostomy were performed due to irreparable tear in the long upper pouch. Conclusion: This rare subtype of long upper pouch oesophageal atresia poses a diagnostic dilemma that can result in delay in the diagnosis and management. This diagnosis should be suspected if the tip of the orogastric/feeding tube is seen to be far below the level of the carina.