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A Case of Holoprosencephaly Accompanied with Dysgenesis of the Cerebellum

  • Hiroshi Tokimura
  • Koki Kadota
  • Koichi Uetsuhara
  • Hiroshige Oda
  • Koji Todoroki
  • Masatoshi Tamura
  • Tetsuhiko Asakura

Abstract

A case of holoprosencephaly with dysgenesis of the cerebellum was reported. In computerized tomography scan, supratentorial structure had a huge monoventricle and very thin cerebral cortex in the frontal base. Tentorium was elevated to high level and in the posterior fossa, dysgenesis of cerebellum and large cerebrospinal fluid space were present. In left carotid angiogram, the azygous anterior cerebral artery ran along the frontal base. These investigations revealed that this case was a semilobar type of holoprosencephaly and an anomaly of the posterior fossa had the features of Dandy-Walker syndrome. For the progressive hydrocephalus, a ventriculo-peritoneal shunt was performed. Postoperatively, left inversed brachial angiogram was examined. From the left vertebral artery to the vertebral venous plexus, shunts were formed by radicular arteries. This finding suggests that there might be some vascular accidents in the developmental stage in such a case. In the cases of holoprosencephaly, the posterior fossa are usually normal. Therefore, such findings in our case seemed to be rare. Information from angiographical investigations should contribute to the understanding of such a case.

Key words

Holoprosencephaly Dandy-Walker syndrome Hydrocephalus Vascular accident 

Copyright information

© Springer-Verlag Berlin Heidelberg 1990

Authors and Affiliations

  • Hiroshi Tokimura
    • 1
  • Koki Kadota
    • 1
  • Koichi Uetsuhara
    • 1
  • Hiroshige Oda
    • 1
  • Koji Todoroki
    • 1
  • Masatoshi Tamura
    • 1
  • Tetsuhiko Asakura
    • 1
  1. 1.Department of Neurosurgery, Faculty of MedicineUniversity of KagoshimaKagoshimaJapan

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