1758 H-type Tracheoesophageal Fistula: A Rare Cause of Cough and Dysphagia in Adults

Abstract

INTRODUCTION: Tracheoesophageal fistula (TEF) without associated esophageal atresia (H-type) is a rare congenital anomaly, accounting for ∼4% of esophageal malformations. It is defined as a connection between the posterior tracheal and anterior esophageal walls and is typically diagnosed before age 3. However, it can occasionally be seen in adults with chronic cough and respiratory infections. We present a 38-year-old female with a new diagnosis of H-type TEF. CASE DESCRIPTION/METHODS: A 38-year-old female (G16P12-0-3-12) with multiple prior admissions for hypoxia (presumed due to asthma and aspiration in the setting of achalasia) was admitted with HELLP syndrome. Prior pulmonary function tests showed severe airway obstruction and echocardiogram showed normal left and right ventricular systolic function. She reported coughing when supine and with meals, a history of congenital lung abnormality, severe infancy pneumonia, upper lobe resection, diagnosis of achalasia, and yearly admissions for pulmonary issues. After delivery, she was transferred to the pulmonary service for management of her hypoxia. A TEF without inflammation was noted on chest CT and confirmed by EGD and bronchoscopy. EGD showed a 3 mm proximal esophageal fistula. Bronchoscopy demonstrated a normal oropharynx and vocal cords (VC), erythematous larynx, inflamed arytenoids, a 4 mm proximal TEF without mucosal abnormalities, and right upper lobe (RUL) absence. A swallow study showed aspiration on the posterior side of the trachea presumed due to the TEF. Surgical repair rather than endoscopic clip or stent placement was determined to be the best treatment via cervical approach esophagoplasty, muscle flap reconstruction TEF repair, and laparoscopic Heller myotomy with Dor fundoplication. DISCUSSION: H-type TEF is rare and can mimic chronic lung disease due to aspiration, recurrent infection, and bronchiectasis. It must be considered in adults with recurrent respiratory infections, coughing with eating/drinking, and rarely, hemoptysis. A chest CT, bronchoscopy, esophagoscopy, and barium study are crucial to confirm the diagnosis. While endoscopic clip closure of fibrotic TEFs has been successful in 62%, it may obliterate surgical tissue planes. Surgery is the optimal treatment for H-type TEF and reported to be 75-94% successful. As H-type TEF disrupts myenteric plexus development with impaired esophageal peristalsis and lower sphincter dysfunction, a Heller myotomy and Dor fundoplication should be offered.