Cavernous malformations in children: a review of the literature

Cavernous malformation (CM) is a rare vascular disorder of the brain in children. Its prevalence in the general population is in the range of 0.1 to 0.9%, with about one quarter of cases occurring in children. This descriptive review presents an analysis of 17 publications in English and Russian literature on the series of children with cavernomas of the central nervous system and describes their etiology, pathophysiology, clinical particulars, and methods of diagnosis, management, prognosis and outcomes in pediatric practice. In most of the published studies, surgery was performed for 35.3 to 100% of the CM diagnosed during a hemorrhage. The indications for surgical management of CM have been poorly described, and this issue remains unresolved so far. There is also no consensus on the treatment strategy for deeply located cavernomas (brainstem, thalamus and basal ganglia), asymptomatic supratentorial cavernomas and CM located in the functionally important zones of the cortex. Criteria for determination of the intervention volume in patients with CM and symptomatic epilepsy have not been determined, as well. All these issues require further evaluation in multicenter randomized trials