A Systematic Classification of the Congenital Bronchopulmonary Vascular Malformations: Dysmorphogeneses of the Primitive Foregut System and the Primitive Aortic Arch System

Purpose: We reviewed the cases of 33 patients from our clinic and 142 patients from the literature with congenital bronchopulmonary vascular malformations (BPVM), systematically analyzed the bronchopulmonary airways , pulmonary arterial supplies, and pulmonary venous drainages, and classified these patients by pulmonary malinosculation( PM). Materials and Methods: From January 1990 to January 2007, a total of 33 patients (17 men or boys and 16 women or girls), aged I day to 24 years (median, 2.5 months), with congenital BPVM were included in this study. Profiles of clinical manifestations, chest radiographs, echocardiographs , esophagographs, computer tomography (CT), magnetic resonance imaging (MRI) , magnetic resonance angiography (MRA ), cardiac catheterizations with angiography, contrast bronchographs, bronchoscopies, chromosomal studies, surgeries, and autopsies of these patients were analyzed to confirm the diagnosis of congenital BPVM. A total of 142 cases from the literature were also reviewed and classified similarly. Results: The malformations of our 33 patients can be classified as type A isolated bronchial PM in 13 patients, type B isolated arterial PM in three, type C isolated venous PM in two, type D mixed bronchoarterial PM in five, type F mixed arteriovenous PM in one, and type G mixed bronchoarteriovenous PM in nine. Conclusion: Dysmorphogeneses of the primitive foregut system and the primitive aortic arch system may lead to haphazard malinosculations of the airways, arteries, and veins of the lung. A systematic classification of patients with congenital BPVM is clinically feasible by assessing the three basic bronchovascular systems of the lung independently