title: [Guidelines for the maintenance of Hippel-Lindau disease in daily practice. French Society of Neurosurgery].
abstract: The subject of the Teaching Course organized by the French Society of Neurosurgery during its Winter meeting in 1998 was phacomatosis. Special interest was given to the Von Hippel-Lindau (VHL) disease. Guidelines for the management of this disease are the following: The diagnosis is established in the presence of two major lesions, or with one major lesion associated with a familial history, or when a VHL gene mutation is detected. An isolated hemangioblastoma (HBM) is associated with the disease in 36% of the cases when located in the posterior fossa and in 64% of the cases when located in the spinal cord. The main causes of mortality of VHL are the following: HBM (61%), renal cancer (31%) and pheochromocytoma (PCC) (8%). The percentage of PCC in the French series is 24%. The percentage of Endolymphatic tumor in the French series is 4%. No surgery is recommended in case of asymptomatic HBM (but radiosurgery has to be evaluated). Follow-up requires regular clinical examinations, one ophthalmological examination per year, one abdominal ultrasonography per year, and one cerebral MRI every three years.
authors.affiliation: Service de Neurochirurgie, Hôpital Henri Mondor, Créteil.
authors.forename: P
authors.initials: P
authors.lastname: Decq
mesh_terms: D016543:Central Nervous System Neoplasms / Q000628:therapy; D005602:France / Q000453:epidemiology; D018325:Hemangioblastoma / Q000628:therapy; D006801:Humans; D007680:Kidney Neoplasms / Q000628:therapy; D010673:Pheochromocytoma / Q000628:therapy; D006623:von Hippel-Lindau Disease / Q000175:diagnosis / Q000235:genetics / Q000401:mortality / Q000628:therapy*
vernacular_title: Recommandations pour la prise en charge de la maladie de von Hippel-Lindau en pratique quotidienne.