Familial communicating hydrocephalus, posterior cerebellar agenesis, mega cisterna magna, and port-wine nevi. Report on five members of one family.

Abstract:

:This report deals with a family in which the maternal grandmother, the mother, and all three male children have port-wine nevi and mega cisterna magna. Two of the three male children have, in addition, congenital communicating hydrocephalus associated with agenesis of the posterior cerebellar vermis. This case of familial communicating hydrocephalus, posterior cerebellar vermis agenesis, port-wine nevus, and mega cisterna magna represents a new neurocutaneous syndrome, possibly transmitted as an autosomal dominant.

journal_name

J Neurosurg

journal_title

Journal of neurosurgery

authors

Nova HR

doi

10.3171/jns.1979.51.6.0862

subject

Has Abstract

pub_date

1979-12-01 00:00:00

pages

862-5

issue

6

eissn

0022-3085

issn

1933-0693

journal_volume

51

pub_type

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