Dandy Walker syndrome: clinico pathological features and reevaluation of modes of treatment

P. W. Carmel, J. L. Antunes, S. K. Hilal, A. P. Gold

Research output: Contribution to journalArticlepeer-review

55 Scopus citations

Abstract

The clinical manifestations of 18 cases of the Dandy Walker syndrome are analyzed. Almost half of these children had associated congenital anomalies. Only 4 had the 'characteristic' radiologic findings. Direct surgical attack upon the cyst was not a successful form of treatment. Satisfactory treatment consisted of shunting of the ventricular system but the incidence of complications was high. There was a 27% mortality. The etiology and pathology are discussed. The common finding in all 5 patients who had autopsies was an abnormality in the roof of the 4th ventricle. The vermis was totally absent in 3 cases. In 1 case vermian segments could be recognized although they were all hypoplastic. In another case the rostral subdivisions were present, but the ventral subdivisions were missing. Associated with the maldevelopment of the vermian segments were abnormalities of the formation of the choroid plexus. In 2 cases, both with no apparent vermal formation, the choroid plexus was entirely absent as well. Clusters of cerebellar tissue were found in the cyst wall in 4 cases. These ectopic clusters contained cells that were clearly cerebellar in origin, but were otherwise structurally highly abnormal. Multiple cortical heterotopias were also present in the cerebellum and ventricular walls in 2 cases. The pathologic features of the autopsies of 5 patients are shown in a table.

Original languageEnglish (US)
Pages (from-to)132-138
Number of pages7
JournalSurgical Neurology
Volume8
Issue number2
StatePublished - 1977
Externally publishedYes

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

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