Neurocutaneous melanosis associated with Dandy-Walker malformation - Case report and review of the literature

Berker M., Oruckaptan H., Oge H., Benli K.

PEDIATRIC NEUROSURGERY, vol.33, no.5, pp.270-273, 2000 (SCI-Expanded) identifier

  • Publication Type: Article / Review
  • Volume: 33 Issue: 5
  • Publication Date: 2000
  • Doi Number: 10.1159/000055968
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.270-273
  • Hacettepe University Affiliated: No


Neurocutaneous melanosis is a rare dysmorphogenesis associated with single or multiple giant pigmented cutaneous nevi and diffuse involvement of the leptomeninges anywhere in the central nervous system (CNS), It is interesting that almost 8-10% of patients had associated Dandy-Walker malformation in the literature, suggesting a common origin of the developmental abnormalities, In this article, we present a 2-year-old patient with neurocutaneous melanosis associated with Dandy-Walker malformation, We reviewed the literature and discuss the pathogenesis based on the preferred hypotheses so far. Copyright (C) 2001 S. Karger AG. Basel.