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

Abstract

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.