Journal of the Korean Pediatric Society 2001;44(7):827-831.
Published online July 15, 2001.
A Case of Crossed Branch Pulmonary Arteries in Dandy-Walker Malformation
Seonyoung Kim, Dongchul Park
Department of Pediatrics, College of Medicine, Inje University, Seoul, Korea
Dandy-Walker 기형에 동반된 교차성폐동맥분지 1례
김선영, 박동철
인제대학교 의과대학 소아과학교실
Abstract
Crossed pulmonary arteries is an uncommon anomaly in which the ostium of the left pulmonary artery originates superiorly and to the right of the right pulmonary artery. The pulmonary arteries then cross each other and supply their respective lungs. The recognition of this rare anomaly is important because of its association with significant cardiac and extracardiac congenital abnormalities. These congenital conotruncal malformations have long been considered part of the clinical spectrum of DiGeorge syndrome, velocardiofacial(Shprintzen) syndrome, and conotruncal face syndrome. More recent reports have shown that a high percentage of patients with these syndromes have microscopic deletions of chromosomal region 22q11. We experienced a case of crossed branch pulmonary arteries in Dandy-Walker malformation. The diagnosis of crossed branch pulmonary arteries was made by echocardiography and this case had no evidence of 22q11 deletion in high resolution chromosome study and fluorescence in situ hybridization. This case of both conotruncal malformation and Dandy-Walker malformaltion was due to an abnormal developmental process involving the neural crest. We report this case with related literature.
Key Words: Crossed branch pulmonary arteries, Dandy-Walker malformation, 22q11 deletion


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