Natural history of cavernomas of the central nervous system

We present a critical review of the literature on the central nervous system cavernomas in order to highlight their natural history and to define the most appropriate management of these rare lesions. The prevalence is now estimated from 0.3 to 0.7% in the general population without any significant difference by gender; 25% of cases are pediatric. Two forms of the disease can be described: sporadic forms in 80% of cases, characterized by isolated or rare lesions and familial dominant autosomic forms characterized by multiple and evolutive lesions. The incidence is not well known, the consultation of the French PMSI database suggests that 50 to 100 cases are operated on each year (1 to 2 per million). Cavernomas are dynamic lesions: growing in many cases, seldom remaining quiescent and disappearing in rare cases. The anatomical evolution is more pejorative in familial forms. "De novo" cases are now well known, either in familial or sporadic forms and after radiotherapy. Many lesions are totally asymptomatic, but the frequency of symptomatic forms is debated in the literature from 3 to 90%... The hemorrhagic risk is evaluated from 0,5 to 3% each year, depending on the localization, and the risk of rebleeding is more important but not well known. The epileptic risk is correlated to the localization, more frequent for temporal and frontal lesions from 4,5 to 11% each year, but these data are controversial. The natural history depends on the topography: hemispheric, deep-seated, brain stem, cerebellum or intramedullary and in pediatric situations. Each situation will be treated in this report.