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

Abstract

PurposeWe 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 MethodsFrom January 1990 to January 2007, a total of 33 patients (17 men or boys and 16 women or girls), aged 1 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.ResultsThe 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.ConclusionDysmorphogeneses 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.

Pulmonary vascular anomalies: a review of clinical and radiological findings of cases presenting with different complaints in childhood

“…Their etiology is not completely known; on the other hand, some genetic factors are thought to play a role in the formation of disease. Lee et al 3 divided bronchopulmonary vascular malformations systematically into seven subcategories (Type A-G) with the "malinosculations theory ". Shields et al 4 , however, created three main categories, as anomalies originating from the pulmonary arteries, pulmonary veins, and lymphatic vessels.…”

“…Pulmonary arteriovenous malformations are generally congenital lesions caused by abnormal capillary development [1][2][3][4] . Their etiology is not completely known; on the other hand, some genetic factors are thought to play a role in the formation of disease.…”

“…This difference arises from the relationship between the lung's one or more components' with the other. Major pulmonary vascular abnormalities can be classified as interruption of the main pulmonary artery or its absence, the emergence of the left pulmonary artery in the right pulmonary artery, pulmonary venous drainage abnormalities, and pulmonary arteriovenous malformations [1][2][3][4] . Pulmonary arteriovenous malformations (PAVMs) are caused by the incomplete formation of vascular septa dividing the primitive connections between the vein-artery plexus or its disintegration.…”

Congenital bronchopulmonary vascular malformations, "sequestration" and beyond

“…[ 4 ] Lee et al ., further refined the concept of malinosculation and classified it as bronchopulmonary malinosculations [ Table 2 ]. [ 5 ] This classification is a systematic approach for the evaluation of BPVMs, taking into account isolated and concurrent abnormalities of airway, arteries, and veins [ Figure 1 ].…”

Congenital Malformations and Developmental Anomalies of the Lung

“…Langston proposed a classification system based on pathologic features. The term pulmonary 'maliosculation' ('mal'-abnormal, 'osculum'-mouth) has also been proposed to describe the abnormal connections or openings of different components of the bronchopulmonary-vascular complex [57,59].…”