Abstract
In this article, the authors review the literature related to long-term outcome of pediatric moyamoya disease, focusing on late cerebrovascular events and social outcome of pediatric patients once they reach adulthood. Late-onset de novo hemorrhage is rare but more serious than recurrence of ischemic stroke. Long-term follow-up data on Asian populations suggest that the incidence of de novo hemorrhage might increase at age 20 or later, even more than 10 years after bypass surgery. Social adaptation difficulty, possibly related to cognitive impairment caused by frontal ischemia, continues in 10-20% of patients after they reach adulthood, even if no significant disability is present in daily life. A treatment strategy aimed at improving long-term outcome and careful follow-up might be required.