[Central nervous system cavernomas in children].

UNLABELLED

Cavernomas represent 1.7 to 18% of all vascular malformations in children and 25% are observed in children under 18 years of age. Cases observed in neonates and infants have been published, but the mean age varies from 9.1 to 10.2 years. There is no predominance between boys and girls.

CLINICAL PRESENTATION

In children, hemorrhage is a common manifestation with an incidence varying from 27.3 to 78% versus 8 to 37% in adult patients. Isolated headaches occur in 2.8% of patients and elevated ICP is observed in 20.1%. Epilepsy is reported in 16 to 60% of children, depending on the series. Neurological deficits are observed in 22.7% of patients and are more severe for deep-seated and brainstem cavernoma. About 14.2% of the cases are discovered fortuitously in asymptomatic patients. Spinal cord deficits are observed in 5% of the cases.

LOCATION

Using data in the literature plus our personal series of 57 cases, 79.4% of lesions are in the supratentorial compartment and 20.6% in the posterior fossa, the majority located in the brainstem, most of them in the pons. Spinal cord cavernomas represent 5% and multiples cavernomas (12.6%) of the reviewed cases.

MANAGEMENT

Appropriate management of cavernomas has long been a subject of much debate. Today, a consensus has been reached to favor medical management of asymptomatic and non hemorrhagic lesions and surgical management of symptomatic and/or hemorrhagic cavernomas whatever the localization. Progress in neuroimaging, surgical mapping, intraoperative monitoring and microsurgical techniques has greatly contributed to improved approach to those lesions.

RESULTS

Results obtained in 217 cases were reviewed. Near 70% of the children are neurologically intact, 19.3% are improved or stable, 2.7% worsened and 1.13% died. Results for epilepsy are very encouraging, surgery is efficient in almost all the children except for temporal lobe cavernomas where invasive presurgical evaluation is recommended. Deep-seated and brainstem cavernomas can safely be removed in most of the cases. Only two children died from recurrent hemorrhage due to residual lesions.