Introduction: To evaluate the medium to long-term outcomes after combined revascularization and indirect revascularization for pediatric hemorrhagic moyamoya disease (MMD).
Methods: A total of 29 consecutive pediatric patients (female to male: 1:1.4; mean age 12.5±3.2 years) with hemorrhagic MMD from October 2010 to August 2021 were enrolled in this study. All patients underwent combined revascularization (superficial temporal artery -middle cerebral artery + encephalo-duro-arterio-synangiosis(EDAS)) or EDAS alone and were followed up.
Results: Among the 29 patients, intracerebral hemorrhage (n=13, 44.8%) was the most common type of hemorrhage, followed by intraventricular hemorrhage (n=11, 37.9%). The mean follow-up duration was 62 months, with four patients lost to follow-up. Among the 25 patients with available follow-up data (including 11 with bilateral MMD and 14 with unilateral MMD), 11 patients (mean age 13.6±2.0 years) underwent combined revascularization, while 14 patients (mean age 11.9±3.8 years) received EDAS alone. Two patients (14.2%) treated with EDAS alone experienced rebleeding, resulting in an average annual incidence of 0.96%. The rebleeding site corresponded to the location of the initial preoperative bleeding. There were no incidents of rebleeding, ischemic events, or deaths observed during the follow-up period in both the combined revascularization group and the unilateral group.
Conclusion : Our research data show that revascularization surgery can prevent rebleeding in pediatric patients with hemorrhagic MMD in the medium to long term. For older age pediatric patients, we recommend combined revascularization more aggressively.