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이리라(Ri Ra Lee),이동규(Dong Gyu Lee),손성민(Sung Min Son),박재동(Jae Dong Park),김태상(Tae Sang Kim),김익수(Il Soo Kim) 대한산부인과학회 2000 Obstetrics & Gynecology Science Vol.43 No.12
The Dandy-Walker syndrome is a developmental disorder of brain characterized by cystic deformity of fourth ventricle, agenesis or hypoplasia of cerebellar vermis, and hydrocephalus. This sydrome is frequently associated with central nervous system malformation and systemic anomalies. The etiology is not clearly established. The pathogenesis of the Dandy-Walker syndrome is originally postulated to be congenital atresia of the foramina of Magendie and Luschka with obstructive hydrocephalus predominantly affecting the fourth ventricle. Subsequent experimental and clinicopathologic studies suggested that the Dandy-Walker syndrome represents a complex developmental anomaly of the rhombencephalon rather than the sequelae of foraminal obstruction. The prenatal diagnosis of the Dandy-Walker syndrome depends on the demonstration of a posterior fossa cyst communicating with the fourth ventricle through a defect of the cerebellar vermis. We experienced two cases of prenatally detected Dandy-Walker syndrome: A case was Dandy-Walker malformation associated with congenital polycystic kidney and the other was Dandy-Walker variant associated with Trisomy 13(Patau syndrome). We present these cases with a brief review of literatures.