Cavernous Malformations of the Central Nervous System in the Pediatric Age Group

Abstract

Objective: The main clinico-diagnostic features, risk factors and associated diseases of cavernous malformations (CMs), also called cavernous angiomas or cavernomas, of the central nervous system (CNS) in children are described, and the most relevant differences compared to the affected adult population are pointed out, focusing on the management of pediatric patients harboring cranial and spinal CMs. Materials: This was a retrospective study of a series of 42 children symptomatic for CMs of the cranial and spinal compartments (35 supratentorial brain lesions, 5 infratentorial and 2 in the spinal region) operated on between 1975 and 2005, with a clinical follow-up ranging from 12 to 192 months. The results were compared with those found in the most recent literature dealing with pediatric CMs of the CNS. Results: Surgical treatment produced excellent or good results in 69% of our 42 children. Unchanged neurological deficits were observed in 23.8% of cases, while morbidity from surgical procedures was 7.14%. Mortality was absent in this series. These surgical results are comparable with those found in the literature, where morbidity and mortality rates from surgery are 8.8 and 1.13%, respectively, and are mostly associated with procedures for the excision of deep, critically located cavernomas. Conclusion: CMs represent the most common CNS vascular lesion in children, although their incidence is 4 times lower than that of the adult population. The natural history of pediatric CMs throughout the neuraxis seems to be more aggressive than in adult patients; these lesions have higher rates of growth and hemorrhage, larger dimensions and often atypical radiological pictures at diagnosis. Beside the familial form of the disease, which is more often associated with multiple lesions and an earlier age of clinical presentation, the major risk factor for CMs in children seems to be radiotherapy for CNS tumors. Furthermore, a greater number of CMs coexistent with mixed angiomatous lesions have been reported in children than in adults. Surgical results are related to the preoperative neurological status of the children; symptomatic patients who are operated on early, before they develop severe neurological deficits or long-standing seizures, may achieve the best clinical outcome. Radiosurgery does not seem to be advisable in children as an alternative treatment for deep CMs or those causing epilepsy.

Intracranial cavernous malformation in children: a single-centered experience with 30 consecutive cases

“…In the current study, 56.6% of patients suffered from seizures, which is significantly higher than in earlier studies 2,[6][7][8]10,17,19 , although Acciarri et al 9 reported 70% of seizures in a series of 42 children with CMs.…”

“…Exception is made for multiple asymptomatic cerebellar CMs 4 . Cerebellar cavernomas represent 0.0 to 13.8% in earlier studies 3,6,[8][9][10][17][18][19] , in our series there were two patients in this group (6.6%).…”

“…Table 2 summarizes previous studies on pediatric CM. Note that only ten series were according to the selection criteria and therefore included in this review 2,3,[6][7][8][9][10]12,17,19 .…”

“…Brainstem CMs constitute 0 to 21% of all cavernomas in different pediatric series 3,6,[8][9][10][17][18][19] . Clinical presentation is generally acute and severe due to peculiar localization, despite frequent small amount of hemorrhage.…”

“…Pediatric CMs may have characteristics that differ from those of adults in clinical presentation and treatment, such as higher rates of hemorrhage [6][7][8][9][10] and larger dimensions 9 . Even though there are not many reports about exclusive pediatric CMs, only a few large series have yet been reported, and ideal group classification has not yet been defined.…”