Management of Esophageal Lung in a Patient With VACTERL Anomalies

Esophageal lung is a rare type of bronchopulmonary foregut malformation where an anomalous main bronchus arises from the esophagus rather than the trachea. This differentiates from an esophageal bronchus where a lobar bronchus arises from the esophagus. Fewer than 30 of these anomalies have been reported in the literature. A female infant was born at 35 weeks gestational age and found to have multiple congenital abnormalities including cleft palate, long-gap esophageal atresia, tracheoesophageal fistula (TEF), imperforate anus, and renal anomalies. She initially underwent thoracoscopic ligation of TEF with colostomy and mucus fistula creation. Bronchoscopy found no right mainstem bronchus, and subsequent computed tomography scan was consistent with possible esophageal bronchus. Esophagoscopy through the gastrostomy discovered a fistulous connection between the distal esophagus and anomalous main right bronchus.Right thoracotomy with pneumonectomy of the hypoplastic lung was performed. She subsequently underwent vaginostomy for hydrometrocolpos. Her proximal esophagus was diverted with a cervical esophagostomy for control of secretions. She also required tracheotomy and is currently at home on minimal ventilator settings awaiting staged reconstruction.Esophageal lung is a rare congenital abnormality with few reported cases. Surgical treatment with pneumonectomy is often required, and pediatric surgeons should be familiar with these congenital bronchopulmonary malformations.