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SIB CASES OF DANDY-WALKER SYNDROME Kei-ichi Saitoh 1 , Toshihiko Kawamoto 1 , Tsutomu Ebina 2 , Teruhiko Tanaka 2 1Division of Pediatrics, Aomori Prefectural Central Hospital 2Division of Neurosurgery, Aomori Prefectural Central Hospital pp.875-879
Published Date 1978/8/1
DOI https://doi.org/10.11477/mf.1406204290
  • Abstract
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Two children of normal parents had "Dandy-Walker syndrome" (cystic dilatation of the 4th ventricle with incomplete development of the cere-beller vermis).

Both cases were admitted to our hospital in neo-natal period, because of apneic spell. Air study showed the enlargement of the 4th ventricle in both cases.

One case (22 days after birth, male infant) was operated by occipital craniotomy, and cystic mem-brane of the roof of the 4th ventricle was removed. But his head was growing rapidly in size, which suggested hydrocephalus. He died of pneumonia at 70 days after V-P shunt operation. At autopsy, defect of the cerebellar vermis and tonsil, involv-ing the enlargement of the 4th ventricle was found.

Another case, younger sister of the previous one, was complicated by occipital meningocele, which did not communicated with ventricular system. Her occipital tumor was removed, and now, she is growing normally in body size, but does not react to the visual stimuli with considerable psychomotor retardation.

In our cases, occulsion of the foramina Luschka and Magendie was not present.

So, we have come to the conclusion that primary underdevelopment of the cerebellum is of major importance for understanding of the etiopathoge-nesis of this syndrome.


Copyright © 1978, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 2185-405X 印刷版ISSN 0006-8969 医学書院

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