title: Neurocutaneous melanosis and the Dandy-Walker complex: an uncommon but not so insignificant association.
abstract: 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.
authors.affiliation: Department of Pediatric Neurosurgery, Hôpital Roger Salengro, Centre Hospitalier Régional et Universitaire de Lille, Lille, France. dominique.marnet@laposte.net
authors.forename: Dominique
authors.initials: D
authors.lastname: Marnet
authors.forename: Matthieu
authors.initials: M
authors.lastname: Vinchon
authors.forename: Keyvan
authors.initials: K
authors.lastname: Mostofi
authors.forename: Benoit
authors.initials: B
authors.lastname: Catteau
authors.forename: Olivier
authors.initials: O
authors.lastname: Kerdraon
authors.forename: Patrick
authors.initials: P
authors.lastname: Dhellemmes
mesh_terms: 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