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KMID : 0357919810150040513
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Korean Journal of Pathology
1981 Volume.15 No. 4 p.513 ~ p.518
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Dandy-Walker Syndrome Associated with Polycystic Kidney
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조혜제/Hye Jae Cho
주중은/이정희/고일향/지제근/Jong Eun Joo/Jeung Hee Lee/Ill Hyang Ko/Je G. Chi
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Abstract
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Hydrocephalus associated with a posterior fossa cyst and hypoplasia of the cerebellar
vermis constitute a triad of Dandy-Walker syndrome. Since this syndrome was first
described by Sutton in 1887, it has been the source of much controversy not only as to
its embryogenesis but also concerning the correct diagnosis and surgical management.
Probably the most widely accepted view about the pathogenensis is a complex
developmental anomaly rather than previously believed atretic foramina of the 4th
ventricle.
This is an autopsy case report of a male infant who was born after 32 weeks of
gestation period and expired 90 minutes after the birth. The case showed the
characteristic findings of marked cystic dilatation of 4th ventricle and hypoplasia of the
cerebellar vermis. In addition, there were occipital meningoenceptalocele that was
connected with the roof of the attenuated cyst wall of fourth ventricle. There was also
bilateral polycystic kidney of Potter type 1.
This case is reported on the base of rarity of this interesting combination of two
anomalies.
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