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Neurocutaneous Melanosis in Association with the Dandy‐Walker Complex
Author(s) -
Kadonaga Julie N.,
Barkovich A. James,
Edwards Michael S. B.,
Frieden Ilona J.
Publication year - 1992
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.1992.tb00323.x
Subject(s) - medicine , cerebellar vermis , dandy–walker syndrome , fourth ventricle , hydrocephalus , anatomy , cerebellum , fornix , macrocephaly , pathology , subarachnoid space , hamartoma , magnetic resonance imaging , radiology , cerebrospinal fluid , hippocampus
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.