Oesophageal lung with systemic arterial blood supply

An adolescent girl with congenital bronchopulmonary-foregut malformation (BPFM) is reported. The patient had a hypoplastic, non-functioning right lung with the main bronchus originating from the oesophagus. The pulmonary artery of the "oesophageal lung" was supplied by systemic circulation from the descending aorta, and the venous flow drained through a hypoplastic right pulmonary artery (RPA) into the large left pulmonary artery (LPA). This case was a rare type of left-to-right shunt and demonstrates that oesophagography, pulmonary angiography and aortography are important in demonstrating the haemodynamics involving this malformation.