[一例与先天性心脏病（右心室双出口、完全性心内膜垫缺损、房间隔缺损、肺动脉狭窄、动脉导管未闭）相关的中枢神经系统异常（胼胝体发育不全、脑室扩张、脑积水、先天性皮样窦）病例]
[A case of central nervous system anomalies (agenesis of corpus callosum, colpocephaly, hydrocephalus, congenital dermal sinus) associated with congenital heart disease(double-outlet right ventricle, complete endocardial cushion defect, atrial septal defect, pulmonary arterial stenosis, patent ductus arteriosus)].
作者信息
Oyama H, Numaguchi A, Sakurai H, Ichihara K, Ikeda A, Matsushima M, Maeda M, Inoue S, Iizuka H, Endoh O, Shibuya M
机构信息
Department of Neurosurgery, Chukyo Hospital, 1-1-10 Sanjo, Minami-ku, Nagoya 457-8510, Japan.
出版信息
No To Shinkei. 2001 Feb;53(2):179-84.
A case of central nervous system anomalies(agenesis of corpus callosum, colpocephaly, hydrocephalus, congenital dermal sinus) associated with congenital heart disease(double-outlet right ventricle, complete endocardial cushion defect, atrial septal defect, pulmonary arterial stenosis, patent ductus arteriosus) is reported. Female patient had been already diagnosed as hydrocephalus during pregnancy and ventricular drainage was performed soon after the delivery. Prostaglandin E 1 was also applied for heart disease, but saturation of O2 decreased to 80% on arterial blood gas analysis. Blalock-Taussig operation and ligation of ductus arteriosus was done 41 days after the delivery and ventricle-peritoneal shunt was also made for the progressive hydrocephalus on the same day. Chromosome analysis showed no abnormality. The genesis of this complicated brain and heart anomaly is discussed from the viewpoint of neural crest cell abnormality.
报告了一例伴有先天性心脏病（右心室双出口、完全性心内膜垫缺损、房间隔缺损、肺动脉狭窄、动脉导管未闭）的中枢神经系统异常（胼胝体发育不全、脑室扩张、脑积水、先天性皮样窦）病例。女性患者在孕期已被诊断为脑积水，出生后不久即进行了脑室引流。还对心脏病应用了前列腺素E1，但动脉血气分析显示氧饱和度降至80%。出生后41天进行了Blalock-Taussig手术和动脉导管结扎术，同一天还因进行性脑积水进行了脑室-腹腔分流术。染色体分析未显示异常。从神经嵴细胞异常的角度讨论了这种复杂的脑和心脏异常的发生机制。
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