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  • Title: Neurocutaneous melanosis associated with Dandy-Walker malformation. case report and review of the literature.
    Author: Berker M, Oruckaptan HH, Oge HK, Benli K.
    Journal: Pediatr Neurosurg; 2000 Nov; 33(5):270-273. PubMed ID: 11155066.
    Abstract:
    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.
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