How can we optimize the long-term outcome in children with intracranial cavernous malformations? A single-center experience of 61 cases

OBJECTIVETo provide a treatment algorithm for pediatric patients with intracranial cavernous malformations (CMs) based on our experience.METHODS Patients <18 years of age who were treated either surgically or conservatively at the authors’ institution between 1982 and 2019 were retrospectively evaluated. RESULTSA total of 61 pediatric patients were treated at the authors’ institution: 39 with lobar CMs, 18 with deep CMs, including 12 in the brainstem and 6 in the basal ganglia, and 4 with CMs in the cerebellar hemispheres. Forty-two patients underwent surgery and 19 were treated conservatively. The median follow-up time was 65 months (1-356 months). In surgically treated patients, lesions were larger (2.4cm vs 0.9cm, p<0.001). In patients with lobar CMs, seizures were more common (72% vs 21%, p=0.003) in the surgery group than in conservatively managed patients. In deep CMs modified Rankin scale (mRS) was higher (4 vs 1, p=0.003) in the surgery group than in conservatively treated patients. At time of last follow-up no differences in Wieser outcome class I was seen (86% vs 67%) in lobar CMs and mRS scores had aligned between the treatment groups in deep CMs (1 vs 0). We encountered no new permanent neurological deficit at time of last follow-up. We propose a treatment algorithm according to lesion location and size, burden of symptoms, epilepsy workup, and further clinical course during observation.CONCLUSIONA conservative management is safe in pediatric patients with asymptomatic CMs. Gross total resection should be the aim in patients with symptomatic lobar CMs. A less aggressive approach with subtotal resection, when required to prevent neurological compromise, sustainably improves neurological outcome in patients with deep CMs.