Revisit on congenital bronchopulmonary vascular malformations: a haphazard branching theory of malinosculations and its clinical classification and implication.
We propose a haphazard branching theory to support the concept of bronchopulmonary malinosculations, and we apply this theory to classify congenital bronchopulmonary vascular malformation (BPVM) based on the anatomical results we have found. Between January 1990 and December 1997, a total of 22 pediatric patients (10 male and 12 female), aged 2 days to 14 years (median, 19.6 months), with congenital BPVM were enrolled in this retrospective study. Study modalities include the clinical features and plain chest films (n = 22) plus at least two of the following: echocardiography (n = 13), barium esophagraphy (n = 2), bronchoscopy (n = 4), contrast bronchography (n = 8), high-resolution direct coronal CT (n = 1) and electron beam or ultrafast CT (n = 1) of the chest, MRI (n = 10), MRA (n = 1), contrast cineangiocardiography (n = 9), surgery (n = 11), or autopsy (n = 2). The salient clinical features were recurrent lung infections in 14 patients, acute respiratory distress in 13, associated cardiovascular malformations in 8, dextroversion in 7, congestive heart failure in 7, dextrocardia in 4, and complex congenital heart diseases in 4. There were abnormal openings (malinosculations) of the pulmonary airway in 20 patients: to an artery in 12, to a vein in 8, and to the lung parenchyma in 9. These 22 patients with congenital BPVM can be classified into bronchial malinosculation (10 cases), arterial malinosculation (2 cases), and bronchoarterial malinosculation (10 cases). Congenital BPVM can be classified in terms of bronchopulmonary malinosculation based upon a haphazard branching theory, in which abnormal communications between two independent systems (primitive foregut system and aortic-pulmonary arch system) occurred coincidentally rather than causally.
Lee
Meng-Luen
ML
Division of Pediatric Cardiology, Department of Pediatrics, Changhua Christian Hospital, No. 135 Nanhsiao St., Changhua, Changhua 50050, Taiwan. 20540@cch.org.tw
Tsao
Lon-Yen
LY
Chaou
Wun-Tsong
WT
Yang
Albert D
AD
Yeh
Kun-Tu
KT
Wang
Jou-Kou
JK
Wu
Mei-Hwan
MH
Lue
Hung-Chi
HC
Chiu
Ing-Sh
IS
Chang
Chung-I
CI
D000293:Adolescent; D001028:Aortopulmonary Septal Defect / Q000000981:diagnostic imaging / Q000473:pathology; D001808:Blood Vessels / Q000002:abnormalities / Q000196:embryology; D001980:Bronchi / Q000098:blood supply* / Q000196:embryology; D001995:Bronchography; D001998:Bronchopulmonary Sequestration / Q000145:classification* / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008168:Lung / Q000098:blood supply* / Q000000981:diagnostic imaging / Q000196:embryology; D008297:Male; D012189:Retrospective Studies; D013624:Taiwan