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KMID : 0357919800140020085
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Korean Journal of Pathology
1980 Volume.14 No. 2 p.85 ~ p.88
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An Autopsy Case of Dandy-Walker Syndrome
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Abstract
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Hydrocephalus associated with a posterior fossacyst and hypoplasia of the cerebellar
vermis constitute a traid of Dandy-Walker syndrome. This syndrome was first described
by Sutton in 1887. In 1914 Dandy & Blackfan described a case of 13 year old child and
thought that these finding were due to atresia of the foramina of Luschka and
Magendie. Taggart and Walker, D'Agostino, Giebson and others supported the concept
of congenital atresia of the foramina of Luschka and Magendie. However many other
authors rejected this concept, and especially Brodal and Hauglie Hanssen pointed out the
cerebellar anlage is fused before the foramina normally become patent and thus there
was no cause-result relationship between foramina atresia and vermal hypoplasia. They
and Gardner thought that this abnormality was the result of preexisting and persistent
hydrocephalus and Benda concluded that the entity is a complex developmental
anormaly.
There were not a few literature-reported cases in other countries, but in Korea the
literature is scarce.
We describe an autopsy case of Dandy-Walker syndrome associated with malforma-
tions of the brain and additional systemic anomalies, which may be related to acute
carbon monoxide poisoning during the early pregnancy period.
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KEYWORD
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