Abstract

Abstract Aim This study presents a single institutional experience with H-type tracheoesophageal fistula (H-TEF). Methods Esophageal atresia (EA) patients who underwent primary surgery in a single center between 2006 and 2018 were retrospectively evaluated. Results Among 110 EA patients, 7 (6.4%) had H-TEF. There were 5 males and 2 females. The primary complaints were choking, cyanosis, and/or apnea with feeding, swallowing difficulty, recurrent bronchopneumonia, or bile in endotracheal tube. The relevant symptoms began during neonatal period with varying degrees of severity in all. The initial diagnosis was proximal esophageal web as demonstrated by contrast swallow and endoscopy in one patient; H-TEF was diagnosed during the course of treatment for the web. Hydrocephalus with severe neurological sequela unrelated to H-TEF and cardiac malformation were present in one. Contrast esophagography was employed in three patients for diagnosis two of which were diagnostic. All underwent diagnostic bronchoscopy. Delayed diagnosis (after 30 days of life) occurred in five (71%). The median age at operation was 3.8 months (11 days–33 months). The level of the fistulas was between C5 and T3. Preoperative bronchoscopic cannulation was successful in all but one. The operation was done via a right cervical approach in six patients and thoracotomy in one. All patients were followed up for at least one year and there was one recurrence detected five months after the first operation. She underwent a secondary surgery with a successful outcome. Right vocal cord paralysis was detected postoperatively in the patient with hydrocephalus. Conclusions H-TEF is a rare subtype of EA malformations with a delay in diagnosis in most cases. Suggestive clinical findings, especially neonatal respiratory symptoms should always be regarded as an indication for further investigations. Although contrast esophagograms may reveal the diagnosis, bronchoscopy is the gold standard diagnostic test in our practice. It should be done preoperatively in every patient not only for diagnostic confirmation but also for cannulation of the fistula to ease the operation. H-TEF might be considered a relatively innocent EA variant but its surgery is not devoid of complications. The patients should be followed up for recurrence and recurrent laryngeal nerve injury on the long term.

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