Neurocutaneous melanosis and the Dandy-Walker complex: an uncommon but not so insignificant association.
BACKGROUND
Neurocutaneous melanosis represents a rare congenital but nonheritable phakomatosis defined as the association of giant or multiple congenital nonmalignant melanocytic nevi with leptomeningeal melanosis or melanoma of the central nervous system.


METHODS
We describe the case of an adolescent with a giant congenital bathing trunk melanocytic nevus who developed progressive intracranial hypertension due to leptomeningeal melanosis confirmed by surgical biopsy. Brain and spine magnetic resonance images showed posterior fossa malformation compatible with the Dandy-Walker complex, hydrocephalus, and extensive enhancement of posterior fossa then spine. Shunt placement, corticotherapy, and chemotherapy were attempted leading to transient relief but the boy died 12 months after the onset of primary neurological symptoms.


CONCLUSIONS
We discuss diagnosis, pathogenesis, management, and prognosis in the light of data from the recent literature.


CONCLUSIONS
Neurocutaneous melanosis is considered to follow from neurulation disorders which could account for associated developmental malformations as the so-called Dandy-Walker complex. Cutaneous lesions are usually recognized at birth whereas neurological manifestations develop later. Numerous neurological symptoms have been reported according to extent of leptomeningeal and parenchymal infiltration. Whether magnetic resonance imaging of the neuroaxis represents the choice radiological exam, definite diagnosis relies upon the histological data obtained by mean of biopsy. Once symptomatic, surgical and medical measures remain palliative since death occurs within 3 years.
Marnet
Dominique
D
Department of Pediatric Neurosurgery, Hôpital Roger Salengro, Centre Hospitalier Régional et Universitaire de Lille, Lille, France. dominique.marnet@laposte.net
Vinchon
Matthieu
M
Mostofi
Keyvan
K
Catteau
Benoit
B
Kerdraon
Olivier
O
Dhellemmes
Patrick
P
D000293:Adolescent; D003616:Dandy-Walker Syndrome / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D017809:Fatal Outcome; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008548:Melanosis / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D020752:Neurocutaneous Syndromes / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D017287:Ventriculoperitoneal Shunt