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Cerebral cavernous malformations (cavernomas) in the pediatric age-group

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Abstract

Cavernomas are vascular malformations composed of a compact mass of sinusoidal-type vessels that are immediately contiguous with one another and have no intervening parenchyma. Cavernous malformations were previously held to be a rare pathology occurring predominantly in adults. New neuroradiological techniques such as computed tomography (CT) and magnetic resonance imaging (MRI) demonstrate, on the contrary, that these lesions are also more frequent during childhood than was previously thought. In our institution we observed 17 cases of cavernous malformations in patients aged between 18 months and 16 years, 16 of whom were diagnosed after 1982. In 4 of these cases there was a documented familial history; in 2 multiple malformations were present. The most common site was the subcortical frontal region, but in 1 case the malformation was located in the pineal region. The most frequent (in 12 cases) initial symptom was hemorrhage, with the characteristics of an intracerebral hematoma. In 4 cases the initial symptom consisted of epileptic fits and 2 of these patients subsequently suffered hemorrhage. In one case the symptoms observed were those of an expansile process. All our patients underwent cerebral angiography and only in 1 case did this show a vascular abnormality. CT, performed in 16 patients, gave positive results in all cases. MRI, performed in 12 patients, gave highly significant images in all cases. Radical surgical removal of the malformation was performed in 15 of the 17 patients, and the results can be considered excellent in the majority of cases. Cavernous malformations are, therefore, more frequent lesions than had previously been thought, especially in pediatric patients. MRI offers the most conclusive means of diagnosis and, considering the possibility of family predispositions, this is also the method to be used in screening a patient's family members. The operation is easy, at least for the most frequent location, and is justified in view of the risk of hemorrhage and relapses.

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Mazza, C., Scienza, R., Beltramello, A. et al. Cerebral cavernous malformations (cavernomas) in the pediatric age-group. Child's Nerv Syst 7, 139–146 (1991). https://doi.org/10.1007/BF00776709

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  • DOI: https://doi.org/10.1007/BF00776709

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