Incidence of late cerebrovascular events after direct bypass among children with moyamoya disease: a descriptive longitudinal study at a single center.

BACKGROUND: The potential for late cerebrovascular events following surgical revascularization presents a challenge in the treatment of pediatric moyamoya disease. Limited information is available on the incidence of such events after direct bypass. The objective of this descriptive study was to examine the incidence of late cerebrovascular events after direct bypass for pediatric moyamoya disease.

METHODS: The study cohort comprised consecutive patients with moyamoya disease who had undergone direct bypass at less than 18 years of age in the authors' institute between 1978 and 2003. They were prospectively followed until the end of the study period or, if applicable, the time of death.

RESULTS: Fifty-six of 58 enrolled patients (96.6%) were followed for a mean period of 18.1 years. Four patients experienced late cerebrovascular events, comprising one stroke and three hemorrhages, an average of 13 years after surgery, one of whom experienced a fatal second hemorrhage. The only late ischemic stroke in the cohort occurred after a severe head injury and emergent craniotomy. The incidence of late cerebrovascular events was 0.41% per year (95% confidence interval, 0.15-1.08); 10-year, 20-year, and 30-year cumulative incidences were 1.8%, 7.3%, and 13.1%, respectively.

CONCLUSIONS: Despite the efficacy of surgical revascularization, pediatric patients remain at risk of future cerebrovascular events, especially hemorrhage, after reaching adulthood and thus require careful long-term follow-up.