Right esophageal lung with associated pulmonary vascular anomaly: A rare case report

Introduction and Importance: Congenital bronchopulmonary foregut malformation (CBPFM) is a rare anomaly in which either a lung, a lung lobe or a segment has a patent congenital communication to the upper gastrointestinal tract. In esophageal lung, an anomalous main bronchus arises from the esophagus rather than the trachea. Case presentation: A male neonate was admitted with respiratory distress immediately following birth. Radiographic evaluation included chest radiograph, chest Ultrasound, chest CT and an upper gastrointestinal (GI) contrast study. A completely opaque right hemithorax was initially visualized and identified as esophageal lung with associated anomalous partial systemic arterial supply and venous drainage. A right posterolateral thoracotomy with pneumonectomy of the hypoplastic lung was performed. However, the patient died of respiratory failure on the first postoperative day. Clinical discussion: Esophageal lung is an extremely rare form of CBPFM in which a main stem bronchus, usually the right one, is abnormally connected to the esophagus. The diagnosis is suggested by chest x-ray and confirmed by upper GI contrast study, which is also available in resource-poor settings. A CT scan with 3D volume rendered images can make a diagnosis, although difficulties remain. Pneumonectomy is the preferred treatment for most patients. Conclusion: Esophageal lung is a rare congenital abnormality with few reported cases. Radiologists and pediatricians should have a high index of suspicion for this anomaly in cases of neonatal respiratory distress and a persistently opacified lung with focal air bronchogram(s) and pursue a timely confirmatory contrast study.