Flexible Bronchoscopy Diagnosis of Uncommon Congenital H-type Tracheoesophageal Fistula, Dual Fistulae, Bronchoesophageal Fistula, and Recurrence of Fistula in Children

Background: Interventional pediatric flexible bronchoscopy has many advantages over radiologic investigations in diagnosing uncommon congenital H-type tracheoesophageal fistula (TEF), dual TEF, bronchoesophageal fistula (BEF) and fistula recurrence including higher rates of identification and anatomic localization with guide wire cannulation. We compare the diagnostic utility of flexible bronchoscopy to radiologic techniques for congenital aerodigestive fistula. Methods: A single center retrospective review was completed of all cases of pediatric TEF and BEF diagnosed with flexible bronchoscopy between January 2000 and November 2020. Results: Fistulae were diagnosed 21 times in 18 patients at a median age of 1.22 years (interquartile range: 0.50 to 2.99). The median time from diagnosis to repair was 17.5 days (interquartile range: 5.5 to 43). Symptoms commonly related to fistula were found in all patients. Uncommon fistulae included single H-type TEF (n=10, 47.6%), dual H-type TEF (n=2, 9.5%), dual proximal and distal TEF with esophageal atresia (n=5, 23.8%), TEF recurrence (n=2, 14.3%), BEF (n=1, 4.8%), and a BEF recurrence (n=1, 4.8%). Flexible bronchoscopy confirmed the diagnosis in all fistulae using a guide wire cannulation or methylene blue dye injection. A combined procedure with simultaneous bronchoscopy and esophagoscopy was used for 6 fistulae. The positive examination rate was 75% for bronchoscopy compared with 2.6% for contrast swallow studies and 28.6% for tube esophagograms. Conclusions: Flexible bronchoscopy should be considered as a first line investigation in uncommon aerodigestive fistulae. In the absence of a skilled bronchoscopist, the best radiologic investigation is a pull-back tube esophagogram but may still require endoscopic confirmation at the time of fistula repair.