Bronchopulmonary foregut malformations. A unifying etiological concept

Abstract

Two cases of congenital bronchopulmonary foregut malformation are reported and another 27 cases reviewed and the findings analyzed. The left lower lobe and the entire right lung are the most common locations of pulmonary involvement. The distal portion of the esophagus or cardioesophageal junction was the most common site of communication (83%). The majority of the patients (60%) presented in the first eight months of life, and the incidence in females was nearly twice that in males. Chronic cough, recurrent pneumonias and respiratory distress were the most common clinical findings, whereas two patients were totally asymptomatic. The esophagogram was the single most useful diagnostic procedure (82%). The microscopic structure of the congenital fistula resembled esophagus, bronchus or both. Surgical treatment was curative in most instances. Sime deaths occurred prior to corrective surgery, whereas the postoperative deaths in most instances were related to severe associated congenital anomalies. We believe a common embryologic pathogenesis leads to the formation of a variety of bronchopulmonary foregut malformations. These bronchopulmonary foregut malformations include intralobar and extralobar sequestrations, pulmonary sequestration with patent, or involuted--partial or complete--gastroesophageal communication, esophageal or gastric diverticula, and esophageal or bronchogenic duplication cysts.