A Case of Bronchopulmonary Foregut Malformation (BPFM) and its New Classification Based on Blood Supply and Bronchial Findings

Abstract

A case of bronchopulmonary foregut malformation (BPFM) which we treated in which the right bronchus was communicated with the esophagus was reviewed with respect to blood supply and bronchial findings in addition to 43 cases from the literature. A one year old boy with recurrent fever and coughing was found to have a bronchus-like structure communicating with the esophagus and occupying the right thoracic cavity by esophagogram and to have a blood supply to the lesion from the pulmonary artery and subphrenic artery by angiogram. The case was diagnosed as BPFM and treated by right pneumonectomy. The pathological findings revealed that the lesion involved only the lower and middle lobes with normal tracheal connections to the upper lobe. This was different from the operative findings of macroscopic involvement throughout the right lung. 44 cases of BPFM including the present case were reviewed and classified from the aspects of blood supply and bronchial findings. All of the cases were divided into two distinct groups according to the existence of bronchial defects. 1) "Pulmonary artery type" (22 cases) The blood supply comes from the pulmonary artery. There is a defect on the bronchial tree. The etiology may be due to the misdevelopment of the tracheoesophageal septum. 2) "Aorta type" (22 cases) The blood supply comes from an aberrant artery arising directly from the aorta. The bronchial tree is normal. The etiology may be due to the development of an accessory lung bud.