Congenital Bronchoesophageal Fistula Diagnosed in Late Adulthood

Abstract

Introduction: A 69-year-old man with a history of right thoracotomy with decortication and drainage of empyema 34 years ago, presented with a 25-year history of dysphagia and coughing up of food and liquids and recurrent respiratory infections. EGD 8 years ago showed possible achalasia and mid-esopha-geal diverticulum. Recent evaluation for pneumonia included barium esophagram showing a fistula from the lower esophagus to the right lower lobe bronchi. CT showed right lower lobe bronchiectasis, infiltrate and dilated esophagus but did not visualize a fistula. EGD revealed a 3mm fistula in the middle third of the esophagus and no other lesions in the esophagus. Bronchoscopy showed no endobronchial lesions but could not initially identify the fistula site. EGD was performed simultaneously with bronchoscopy and saline with methylene blue was injected through the esophageal side of the fistula using an ERCP cannula. Bronchoscopy revealed passage of air bubbles and methylene blue through a bronchus [posterior subsegment of the superior basal segment of right lower lobe (B8a)], localizing the exact fistula site. He had surgical repair involving division of the fistula tract with intercostal muscle-flap interposition. There was no persistent fistula on EGD and esophagram. Congenital bronchoesophageal fistula (BEF) is rare in adults but can be unrecognized for years, with symptom duration varying from 5-50 years before diagnosis. Age at diagnosis ranges from 9-83 years. Symptoms include cough, frequent respiratory infections, hemoptysis and bouts of cough when swallowing liquids (Ohno’s sign). Barium esophagram is the most sensitive test compared to endoscopy and bronchoscopy. Complications include recurrent pneumonia, abscess and bronchiectasis. Definitive treatment involves surgical closure of the fistula tract, with interposition of viable tissue such as intercostal muscle to prevent refistulization. The insidious course of congenital BEF, the absence of surrounding inflammation and adherent lymph nodes, and our patient’s history support a congenital nature of the fistula. In summary, we illustrated a case of congenital bronchoesophageal fistula diagnosed in late adulthood by barium esophagram, confirmed by simultaneous EGD and bronchoscopy and managed by surgical repair.Figure 1: (A) EGD of esophageal side of fistula. (B) Bronchoscopy showing methylene blue leaked through bronchial side of fistula when methylene blue was instilled into the fistula during EGD.