Introduction: Pediatric moyamoya patients have little long-term data to inform them about what to expect after revascularization beyond a 5-year window. Here we present a series of pediatric patients treated with indirect revascularization with long-term clinical and radiographic follow-up.
Hypothesis: We assessed the hypothesis that indirect revascularization, such as pial synangiosis, provides durable clinical and radiographic protection from stroke in pediatric moyamoya patients.
Methods: Retrospective case series. Records of all children with moyamoya surgically revascularized by the senior author prior to 1996 (20 years ago) were analyzed.
Results: 37 patients (27F/12M) were identified, all diagnosed under age 22 years. Most common presentations included ischemic symptoms and 30 patients (81%) had a radiographic stroke prior to surgery. Bilateral disease existed in 36 patients; 1 unilateral. Average age at surgery was 8 years (range=1-22 yr.). Surgery consisted of 25 EDAS (encephalo-duro-arterio-synangiosis) and 48 pial synangiosis.
Average follow-up=16 years (range=5.5-31.8 years). Only one stroke occurred during this period (2.7% of patients). Two deaths occurred, 1 from a malignant meningioma at age 27 and the other from aneurysmal hemorrhage after intracranial radiation for tumor at age 40. In all other patients, mRS scores were stable or improved at most recent follow-up.
Descriptive data less amenable to analysis but subjectively helpful to patients includes information on patient life experiences. A total of 91% completed high school, 90% are employed, 56% completed college and 3 patients have graduate degrees. Four of the female patients had successful vaginal child births.
Conclusions: Revascularization confers durable protection from stroke for pediatric patients on long-term follow up. These data suggest that pediatric patients do very well after treatment and support the use of interventions such as pial synangiosis.