Neurocutaneous melanosis associated with Dandy-Walker malformation and a meningohydroencephalocele. Case report.
Neurocutaneous 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.
Arai
Masayuki
M
Department of Neurosurgery, Fukui Prefectural Hospital, Fukui City, Japan. myarai@yahoo.co.jp
Nosaka
Kazuhiko
K
Kashihara
Kengo
K
Kaizaki
Yasuharu
Y
D003616:Dandy-Walker Syndrome / Q000150:complications*; D004677:Encephalocele / Q000150:complications* / Q000196:embryology / Q000601:surgery; D005260:Female; D005315:Fetal Diseases / Q000000981:diagnostic imaging; D006801:Humans; D006849:Hydrocephalus / Q000517:prevention & control; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D008588:Meningocele / Q000150:complications* / Q000196:embryology / Q000601:surgery; D020752:Neurocutaneous Syndromes / Q000150:complications* / Q000151:congenital / Q000601:surgery; D009508:Nevus, Pigmented / Q000150:complications* / Q000151:congenital / Q000601:surgery; D010784:Photomicrography; D011247:Pregnancy; D014463:Ultrasonography