Article
Long-term outcome in cerebral cavernous malformation-associated epilepsy in pediatric patients
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Published: | April 28, 2011 |
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Objective: Cerebral cavernous malformations (CCM) in children often become symptomatic with epileptic seizures. The current data on the surgical therapy of CCM-associated epilepsy is not sufficient concerning outcome for different epilepsy types, especially for functional and seizure outcome. Providing data on long-term outcome is essential since it has been shown that favorable seizure outcome may not be entirely stable.
Methods: Hospital records, surgical reports and -if available- cranial MRI were analyzed for type of seizure disorder, preoperative work-up, extent of resection and long-term follow-up.
Results: Overall, 31 children received surgical treatment for a CCM between 1990 and 2008. 18 patients presented with symptomatic epilepsy (5 single/sporadic seizures, 7 chronic, but not drug-resistant epilepsy, 6 drug-resistant epilepsy). Mean age was 14.27 years (range 3–18 years). Mean duration of seizures was 2.69 years. All CCM harbored a surrounding hemosiderotic rim. 15 CCM were located temporolaterally or in other neocortical regions (3 temporomesial). 5 patients had multiple CCM in whom the symptomatic CCM was removed. In 11 patients, detailed preoperative workup was performed and 3 patients underwent invasive EEG recording. In 11 cases extended lesionectomy (incl. rim /perilesional tissue/ bigger resections) was performed according to preoperative electrophysiological data and/or intraoperative ECOG. Due to proximity to eloquent brain regions, only an incomplete removal of the hemosiderotic rim was possible in 3 cases. Intraoperative neuromonitoring was used in 4 patients (2 MEP/SEP, 1 ECOG). Neuronavigation was used in 4 cases. One patient developed a visual field defect (quadrantanopia) as a calculated deficit. Mean follow-up of patients with single/sporadic seizures and chronic epilepsy was 11.7 years; regarding the long-term, all patients were seizure-free. Patients with drug-resistant epilepsy had a mean follow-up of 9.7 years and favorable long-term outcome (ILAE 1–3) was achieved in 83%.
Conclusions: Surgery on CCM-associated epilepsy in children is effective and safe. Children with drug-resistant epilepsy and even multiple CCM should be considered for surgery after a detailed epileptological workup. In terms of follow-up, long-term seizure outcome is stable.