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Congenital isolated tracheo-oesophageal fistula – management and outcome

Aline Vaz-Silva1, Sara Carmo1, Catarina Ladeira1, Rafaela Murinello1, Cristina Borges1 , Oliveira Santos2, Paolo. Casella1

1 Serviço de Cirurgia Pediátrica, H. D. Estefânia, CHLC E.P.E.; 2 Serviço de Pediatria Médica, H. D. Estefânia, CHLC E.P.E.

4th World Congress of Pediatric Surgery, 13 a 16 de Outubro 2013, Berlim. (comunicação oral)


Background:  Congenital isolated tracheo-oesophageal fistula account for about 4% of tracheo-oesophageal malformations. The symptoms are usually present at birth, but may be intermittent and vary in severity - choking and cyanosis on feeding, recurrent lower respiratory tract infection and abdominal distension. The diagnosis can be made in the neonatal period, but delays are frequent. A high index of suspicion is required because the symptoms are not specific. Investigation can be made by upper gastrointestinal contrast study/tube esophagogram, esophagoscopy or bronchoscopy. Surgical treatment consists in the division of the fistula.

Methods:  A retrospective review of all cases of congenital isolated tracheo-oesophageal fistula that were diagnosed in a single terciary pediatric hospital over a 5 year period. Data gathered included age, co-morbidities, time to diagnosis, presenting symptoms, investigations, surgical repair and outcome.

Results: There were 6 patients in this series, 4 born prematurely (2 with severe associated co-morbidities).  The median age at diagnosis was 33 days (8-154 days). One patient presented with abdominal distension, 4 with cyanosis and choking on feeding, and all with recurrent lower respiratory tract infection. Diagnosis was made by bronchoscopy in all patients.  Five patients underwent surgical ligation of the fistula by a cervical approach. Two patients died, one pre-operatively and the other post-operatively. No patient presented with swallowing difficulties, recurrent respiratory tract infections or recurrence of the fistula in follow-up (13-62 months).

Conclusion: Diagnostic delay is common in congenital isolated tracheo-oesophageal fistula, due to lack of specificity of presenting symptoms. Bronchoscopy is a reliable exam for diagnosis. When not associated with severe co-morbidities the prognosis is good.