Neurological Surgery No Shinkei Geka Volume 20, Issue 10 (October 1992)

Holoprosencephaly Accompanied with Dysgenesis of the Cerebellum Nobuko YAMASHITA 1 , Ken KAMIYA 1 , Hajime NAGAI 1 1Department of Neurosurgery, Nagoya City University Medical School Keyword: Holoprosencephaly , Dandy-Walker syndrome , Hydrocephalus pp.1097-1101
Published Date 1992/10/10
  • Abstract
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We have encountered 9 cases of holoprosencephaly, four of them with abnormalities of the posterior fossa. Three cases of semilobar type had severe cerebellar hypoplasia and a large cyst continuing to the fourth ven-tricle. They were diagnosed as Dandy-Walker syndrome. A case of lobar type had a relatively mild form of cere-bellar hypoplasia and it was thought to be a Dandy-Walker variant. MRI was able to clarify the anatomical relationship of the intracranial construction, for example monoventricle, dorsal sac or large cyst in the posterior fossa, and it was very useful for the diagnosis of these congenital anomalies. All of them showed hydrocephalus and required treatment with ventriculoperitoneal or cyst-peritoneal shunt.

With the availability of MRI, easy diagnosis of the mild forms of holoprosencephaly has been facilitated, and accompanying anomalies are also able to be dia-gnosed. Up to now, the structure of the posterior fossa in holoprosencephaly was thought to be normal. However, several cases of holoprosencephaly accompanied with cerebellar hypoplasia and cystic lesion of the post-erior fossa, revealed by MRI or autopsy, have been re-ported. It was suggested that holoprosencephaly with anomalies of the posteior fossa may be less rare than has been thought, so far.

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Neurological Surgery 脳神経外科
20巻10号 (1992年10月)
電子版ISSN 1882-1251 印刷版ISSN 0301-2603 医学書院