• Journal of neurosurgery · May 2004

    Case Reports

    Neurocutaneous melanosis associated with Dandy-Walker malformation and a meningohydroencephalocele. Case report.

    • Masayuki Arai, Kazuhiko Nosaka, Kengo Kashihara, and Yasuharu Kaizaki.
    • Department of Neurosurgery, Fukui Prefectural Hospital, Fukui City, Japan. myarai@yahoo.co.jp
    • J. Neurosurg. 2004 May 1; 100 (5 Suppl Pediatrics): 501-5.

    AbstractNeurocutaneous melanosis and Dandy-Walker malformation are both forms of rare congenital neurodysplasia. Interestingly, 8 to 10% of patients with neurocutaneous melanosis also harbor an associated Dandy-Walker malformation, indicating that these developmental abnormalities share a common origin. The authors describe a case of neurocutaneous melanosis associated with Dandy-Walker malformation and an occipital meningohydroencephalocele with a giant melanotic nevus. Multiple congenital liver masses were also observed in the infant. The occipital nevus was totally excised, and ventriculoperitoneal and cyst-peritoneal shunts were created to prevent subsequent hydrocephalus. Findings in this case support the possibility that excessive melanocytes hinder normal mesenchymal development, causing Dandy-Walker malformation and an occipital meningocele.

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