Abstract
While prognostic information for Dandy-Walker syndrome and non-progressive cerebellar
ataxia/cerebellar hypoplasia is available, surprisingly scant literature reports are
found for space-occupying posterior fossa arachnoid cysts (PFAC). We describe the
outcome of patients with symptomatic PFAC shunted as infants. Only 11 children were
seen over a 20-year period (1980 - 1999). Cyst location was retrocerebellar (n = 8),
supravermian (n = 2) and in the cerebellopontine angle (n = 1). Motor and cognitive
impairments in one child were interpreted as the result of neurosurgical complications
(intraventricular and intracerebral haemorrhage). One 6-year-old boy had mild truncal
ataxia and mild cognitive delay. The 9 other patients had no evidence of ataxia or
clumsiness and had average, some even above average, cognitive ability and scholastic
achievements. Epilepsy was not a feature. We conclude that the prognosis of PFAC for
cognitive development and neurological signs is favourable.
Key words
Arachnoid Cyst - Posterior Fossa Cyst - Cerebellum - Outcome - Children
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1 Results were presented in part at the annual meeting, Gesellschaft für Neuropaediatrie,
Recklinghausen on March 29, 2001
Prof. Dr. E. Boltshauser
University Children's Hospital
Steinwiesstrasse 75
8032 Zurich
Switzerland
Email: eugen.boltshauser@kispi.unizh.ch