Cavernomas in Children

Cavernoma at pediatric age has been regarded as a relatively rare pathology, classified within the congenital vascular malformations of the central nervous system. Although majority of the cases are identified in adulthood due to expanding mass lesion or with the new onset of seizures following repeated hemorrhages. Advent of computed tomography (CT) and magnetic resonance imaging (MRI) their diagnosis both as an incidental finding and in childhood including infancy is increasing. Contrary to the other members of arteriovenous malformations of the brain, incidence, pathogenesis and the natural history of intracerebral cavernomas are not yet fully elucidated. Although they are originally regarded as congenital in origin with familial forms consisting almost one third of the diagnosed cases, documented cases of de novo formation after radiation therapy, stereotactic biopsy and association with developmental venous anomaly (DVA) bring controversy against the congenital hypothesis. Hemorrhage, as the main determinant of symptomatology in cavernomas is also diverse as it is almost always repetitive and intralesional resulting in expansion of the lesion rather than intraparenchymal disruption. Distinct pathology and pathogenesis of the cavernomas prevent sudden, life-threatening deterioration upon hemorrhage unlike true arteriovenous malformations. Nevertheless, seizures or progressive neurological deficits in a child have enough priority to treat a cavernoma to avoid neurocognitive decline even if incidentally detected. Surgical treatment is straightforward for symptomatic cases but a number of controversies exist related to the localization, multiplicity of the lesions especially in asymptomatic cavernomas. Regarding the brainstem, paraventricular or deep locations are more frequent in children, it is difficult to define a standard algorithm for surgical treatment of pediatric cavernomas.