Abstracts

Rationale: Sturge-Weber Syndrome (SWS) is a rare neurocutaneous disorder characterized by facial port-wine birthmark, vascular eye abnormalities, and leptomeningeal angioma. A recent published anonymous survey of patients with SWS brain involvement suggested a significant reduction in seizures and stroke-like episodes in patients on aspirin with minimal side-effects [Bay et al., J Child Neurol, 2011]. This study further addresses the incidence, severity, and type of side-effects associated with aspirin usage as well as the seizure characteristics of infants and children with SWS taking low-dose aspirin.Methods: Fifty-eight Sturge-Weber patients (34 male; ages 1 month to 12 years) on aspirin with brain-involvement, seen between 2002 and 2010, were analyzed in a retrospective chart review. Charts were evaluated for side of brain, eye, and skin involvement, age at which aspirin was started, and relevant side effects. A subset of seventeen subjects (9 male; ages 2 years to 14 years; 2 bilateral, 9 right-sided, 8 left-sided brain involvement) with three annual neurological exams was analyzed for seizure severity and management. Neuroscores [Lin et al., J Magn Reson Imaging, 2006], including seizure scores were assigned prospectively at each visit.Results: Out of fifty-eight SWS subjects with brain involvement that were started on aspirin, forty-nine reported no significant side-effects. Out of the nine subjects reporting complications, six reported only experiencing minor side-effects (nosebleeds, bruising). Three subjects reported serious side-effects including allergic rash, hematemesis, and subdural hematoma. Aspirin was stopped in six of the nine subjects due to side-effects or complications. For the seventeen subjects with yearly SWS Neuroscores, the median seizure score was one (no seizures in the past six months) at the first Neuroscore, two (breakthrough seizures less frequent than monthly in the past six months) a year later, and one at the final Neuroscore. At the end of the two year period, one subject (5.9%) was having monthly seizures, three (17.6%) had one cluster of breakthrough seizures in the last six months, and thirteen (76.5%) had no seizures in the six months prior to the Neuroscore.Conclusions: This study reviews aspirin use in a large population of SWS patients and its effect on seizures in a closely followed subset. While aspirin use in such a young population raises concerns regarding complications, only a minority of subjects in this study experienced side-effects. This suggests that aspirin should not be withheld due to concerns about complications. Data from the subset of all subjects on low-dose aspirin followed regularly over a 2 year period at the Hunter Nelson Sturge-Weber Center shows that these children started on a combination of aspirin and one or more anticonvulsants had well-controlled seizures. Ongoing studies will further evaluate clinical efficacy of aspirin in SWS in order to better assess the risks and benefits.