A systematic classification of the congenital bronchopulmonary vascular malformations: dysmorphogeneses of the primitive foregut system and the primitive aortic arch system.
OBJECTIVE
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).


METHODS
From 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.


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.


CONCLUSIONS
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.
Lee
Meng-Luen
ML
Department of Pediatrics, Division of Pediatric Cardiology, Changhua Christian Hospital, No. 135, Nanhsiao St., Changhua 50050, Taiwan. ferdielee@yahoo.com
Lue
Hung-Chi
HC
Chiu
Ing-Sh
IS
Chiu
Han-Yao
HY
Tsao
Lon-Yen
LY
Cheng
Ching-Yuan
CY
Yang
Albert D
AD
D000293:Adolescent; D000328:Adult; D001013:Aorta, Thoracic / Q000002:abnormalities*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007422:Intestines / Q000002:abnormalities* / Q000098:blood supply*; D008168:Lung / Q000002:abnormalities* / Q000098:blood supply*; D008297:Male; D054079:Vascular Malformations / Q000145:classification*