1281 Patient Presentation and Clinical Characteristics of Cerebral Cavernous Malformations in Pediatric Patients

INTRODUCTION: Cerebral cavernous malformations (CCMs) occur in both pediatric and adult patients. However, the characteristics of CCMs have not yet been thoroughly described in children. METHODS: Using radiology records, 237 patients with CCMs between May 1991 and December 2022 were identified and retrospectively reviewed for demographics, imaging, and clinical characteristics. Pediatric patients were defined as patients with age<18y at time of diagnosis. The Mann-Whitney and chi-square tests were used to compare variables between pediatric and adult patients. RESULTS: 15 pediatric patients were identified (M/F=5/10). Compared to adults, pediatric patients trended toward higher rates of multiple CCMs (33.3% vs 18%, p=0.26), and had significantly higher rates of familial cerebral cavernous malformations (FCCM) determined by family history, imaging, and genetic testing (33.3% vs 5.4%, p<0.001). Pediatric patients were more often diagnosed due to seizure (53.3% vs 9.5%, p<0.001) and presented with larger lesions (median 24mm vs 8mm, p<0.001). CCMs did not differ significantly in location (supratentorial: 53.3% vs 70.3%, cerebellum 6.7% vs 6.8%, brainstem 6.7% vs 5%) or rates of associated developmental venous anomaly (33.3% vs 44.6%). There was a greater incidence of radiographic hemorrhage (80% vs 32.9%, p<0.001) and history of seizure (53.3% vs 18%, p=0.003), but no significant difference in rate of neurological deficit (13.3% vs 32.4%, p=0.21). Pediatric patients underwent surgical resection of CCMs at a higher rate (66.7% vs 11.7%, p<0.001), and operated pediatric patients had improved mean Modified Rankin Scale from 2.11 at first presentation to 0.89 at last follow up. CONCLUSIONS: Pediatric patients with CCMs were more likely to have FCCM and larger CCMs. These patients underwent higher rates of resection. Indications for resection include long natural history risk, higher rates of seizures, higher rates of previous hemorrhage, and resection corresponded to improved long-term neurologic status.