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NEUROCUTANEOUS MELANOSIS IN ASSOCIATION WITH THE DANDY-WALKER COMPLEX PEDIATRIC DERMATOLOGY KADONAGA, J. N., Barkovich, A. J., Edwards, M. S., Frieden, I. J. 1992; 9 (1): 37-43

Abstract

An infant had a giant congenital nevus, neurocutaneous melanosis (NCM), and a Dandy-Walker malformation of the brain. The diagnosis of NCM was suspected at 6 weeks of age when macrocephaly was noted, resulting in the discovery of hydrocephalus and a Dandy-Walker malformation. Serial magnetic resonance imaging scans demonstrated so-called T1 shortening in the pia or subarachnoid spaces surrounding the cerebellar vermis and in the temporal lobes anterior to the temporal horns. Eventually, a biopsy-proved melanoma developed in the anterior temporal lobe, in an area previously noted to have T1 shortening. Since meningeal cells have been shown experimentally to play a critical role in cerebellar development, we hypothesize that the association of NCM with a Dandy-Walker malformation may be due to meningeal melanosis disrupting the normal development of the cerebellum and fourth ventricle.

View details for Web of Science ID A1992HJ83100007

View details for PubMedID 1574474