Esophageal atresia type C with overlapping long upper pouch: A rare variant
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Updated: Dec 18, 2025
Laparoscopic Choledochal Cyst Excision and Roux-en-Y Choledochojejunostomy in Adults
Published on: February 28, 2025
Samuel Negash1, Hiwote Girma1, Hanna Getachew Woldeselassie1
1Division of Pediatric Surgery, Department of Surgery, Addis Ababa University, Ethiopia.
Insights
Esophageal atresia, a common pediatric surgical condition, can present unusually, complicating diagnosis. Early recognition of rare types, like long upper pouch with distal fistula, is crucial for timely management and improved outcomes.
Area of Science:
- Pediatric Surgery
- Embryology
- Neonatal Medicine
Background:
- Esophageal atresia is a frequent pediatric surgical emergency, particularly in developing nations.
- Complex embryologic development of the esophagus and trachea results in diverse esophageal atresia anomalies.
- Long upper esophageal pouch with distal tracheoesophageal fistula represents a rare esophageal atresia variant.
Observation:
- A neonate presented with respiratory distress and vomiting, with initial diagnosis of esophageal atresia missed due to nasogastric tube placement.
- Diagnosis was confirmed as type C esophageal atresia with a long upper pouch via X-ray and esophagogram.
- Surgical intervention was delayed beyond the third week of life due to diagnostic challenges.
Findings:
- The case highlights diagnostic challenges posed by uncommon esophageal atresia presentations.
- Delayed diagnosis and intervention did not preclude survival in this specific case.
- This rare esophageal atresia variant appears to have a favorable prognosis despite diagnostic delays.
Implications:
- Increased physician awareness of rare esophageal atresia types is essential to prevent diagnostic delays.
- Timely diagnosis and management are critical for improving outcomes in esophageal atresia cases.
- This case underscores the importance of considering esophageal atresia even with atypical presentations.
Background:
Esophageal atresia is one of the most common pediatric surgical conditions with a high mortality in developing countries. The esophagus and trachea have a complex embryologic development leading to a wide spectrum of esophageal atresia anomalies. Long upper esophageal pouch with distal tracheoesophageal fistula is one of these rare varieties. Only a handful of case reports exist on the subject thus far. Most reports are from low income countries.
Case Presentation:
A neonate referred to our hospital after 2 weeks of life for excessive secretions, persistent respiratory distress and vomiting. Diagnosis of esophageal atresia was overlooked because nasogastric tube was advanced a considerable length. After repeat x-ray and esophagogram, we confirmed the diagnosis of type C esophageal atresia with a long upper esophageal pouch. Due to the dilemma in diagnosis, surgery was delayed after the 3rd week of life. Luckily the child survived even with extremely delayed intervention.
Conclusion:
Esophageal atresia can have an uncommon presentation which causes confusion in the diagnosis. Physicians need to be aware of its existence to avoid unnecessary delays in the management. Fortunately, this variant generally seems to have a good prognosis.
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