[Cerebral cavernomas in childhood. clinical presentation and diagnosis]. / Cavernomas cerebrales en la infancia. Presentación clínica y diagnóstico.

ABSTRACT

INTRODUCTION: Intracranial vascular malformations are congenital lesions due to alterations in the development of the arteriolocapillary network. Traditionally they are divided into four types according to their histological characteristics arteriovenous malformations, cavernous angiomas or cavernomas, venous angiomas and telangectasias. The cavernomas are multilobulated lesions which are clearly delimited and contain blood at different stages of evolution. Histologically they are composed of sinusoidal spaces lined by endothelium and closely interlinked, without intervening nervous tissue. They are usually found at a supratentorial level, and less frequently in the posterior fossa. PATIENTS AND METHODS: We made a retrospective review of the histories of 11 patients aged between 2 and 10 years, diagnosed as having intracranial cavernomas in which imaging techniques lead to the diagnosis of intracranial cavernoma. In two patients there were multiple cavernomas. We analysed the method of diagnosis and clinical features on presentation. RESULTS: The clinical features were varied. In six patients the onset of the disorder was with epileptic seizures, three had progressive neurological deficits, one patient had headache and vomiting which progressed to coma and another had a history of non specific fainting and deficit. All but one of the cavernomas were supratentorial. CONCLUSIONS: Cavernomas are a type of vascular malformation with specific histological features. The usual clinical features are convulsions and parenchymatous bleeding. They are generally supratentorial. The appearance of MR has permitted diagnosis of asymptomatic cavernomas and is currently considered to be the technique of choice for diagnosis.