Neurocutaneous melanosis in association with Dandy-Walker malformation: case report and literature review.

Abstract

Neurocutaneous melanosis (NCM) is a rare congenital noninheritable phacomatosis characterized by large and/or numerous cutaneous congenital melanocytic naevi (CMN) in combination with melanocytic leptomeningeal tumours. Dandy-Walker malformation (DWM) consists of a cystic dilatation of the fourth ventricle communicating with the posterior fossa, and a high insertion of the tentorium and hypoplasia/aplasia of the cerebellar vermis (partially caused by Zic1(+/-)Zic 4(+/-) on 3q2). An association of NCM and DWM is very rare, with only 15 previously reported cases to our knowledge. We present an 8-year-old girl with multiple CMN and DWM. A ventriculoperitoneal shunt operation was performed when she was 1 day old. Her neurological symptoms to date comprise headaches, nausea and vomiting as a result of ventriculoperitoneal shunt dislocation at the age of 4 years. The diagnosis is provisional asymptomatic multiple CMN-type NCM in association with DWM.

• Authors: S Schreml, B Gruendobler, J Schreml, M Bayer, E Ladoyanni, L Prantl, G Eichelberg
• Journal: Clinical and experimental dermatology (Clin Exp Dermatol) Vol. 33 Issue 5 Pg. 611-4 (Aug 2008) ISSN: 1365-2230 [Electronic] England
• PMID: 18477004 (Publication Type: Case Reports, Journal Article, Review)

Topics

• Child
• Dandy-Walker Syndrome (complications, diagnosis)
• Female
• Humans
• Magnetic Resonance Imaging
• Melanosis (complications, diagnosis, surgery)
• Neurocutaneous Syndromes (complications, diagnosis, surgery)
• Tomography, X-Ray Computed
• Ventriculoperitoneal Shunt