title: Neurocutaneous melanosis in association with the Dandy-Walker complex.
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.
authors.affiliation: Department of Dermatology, Oregon Health Sciences University, Portland.
authors.forename: J N
authors.initials: JN
authors.lastname: Kadonaga
authors.forename: A J
authors.initials: AJ
authors.lastname: Barkovich
authors.forename: M S
authors.initials: MS
authors.lastname: Edwards
authors.forename: I J
authors.initials: IJ
authors.lastname: Frieden
mesh_terms: D001932:Brain Neoplasms / Q000150:complications / Q000175:diagnosis*; D003616:Dandy-Walker Syndrome / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D008545:Melanoma / Q000150:complications / Q000175:diagnosis*; D008548:Melanosis / Q000150:complications / Q000175:diagnosis*; D009508:Nevus, Pigmented / Q000150:complications / Q000151:congenital / Q000175:diagnosis*; D012878:Skin Neoplasms / Q000150:complications / Q000151:congenital / Q000175:diagnosis*; D013702:Temporal Lobe