Esophageal lung, pulmonary artery sling and congenital tracheal stenosis: A case report

Introduction: Esophageal lung is rare bronchopulmonary foregut malformation where a main stem bronchus is connected anomalously to the esophagus. We present a challenging diagnostic workup of a 14-month-old who was referred following multiple complicated hospitalizations for recurrent respiratory distress. Case presentation: A male infant born at 37 weeks was noted to be hypoxemic at birth and subsequently diagnosed with right-sided pulmonary hypoplasia, absent right main stem bronchus, a left-sided non-obstructive pulmonary artery sling, and possible tracheal stenosis via computed tomographic angiography (CTA). After a stay in the neonatal intensive care unit for persistent hypoxemia, he was discharged home on oxygen. At 4.5 months, the patient developed severe respiratory distress requiring extracorporeal membrane oxygenation, which was complicated by a stroke. During this admission, repeat CTA showed a broncho-esophageal fistula, concerning for esophageal lung. The patient was seen in consultation at our institution at 14 months given persistent respiratory insufficiency. He underwent repeat endoscopy with our multi-disciplinary Aerodigesive Clinic, confirming mild tracheal stenosis and esophageal lung. A right pneumonectomy was recommended. During the pneumonectomy, the right bronchus was confirmed to originate from the distal esophagus on endoscopy. Intraoperative bronchoscopy showed a patent central airway and left bronchopulmonary tree. The patient recovered well post-operatively without complications and was discharged home on room air. Conclusion: Diagnosing esophageal lung can be difficult and requires thorough evaluation of the adjacent anatomy, oftentimes requiring multidisciplinary evaluation with multiple radiologic and endoscopic modalities.