Surgical revascularization versus nonsurgical management in children with symptomatic moyamoya arteriopathy: a North American multicenter cohort study.

OBJECTIVE

Moyamoya arteriopathy is a leading cause of cerebral ischemia in children. In pediatric patients with symptomatic or progressive moyamoya disease, surgical revascularization is the mainstay of treatment; however, there is limited comparative evidence demonstrating an advantage of surgical revascularization over nonsurgical management in North American cohorts. The authors aimed to determine whether surgery reduces the risk of subsequent ischemic events in North American children with symptomatic ischemic moyamoya arteriopathy compared to nonsurgical management alone.

METHODS

Patients treated conservatively at the Sainte-Justine University Hospital Center in Montréal, Canada, were compared to patients treated surgically through indirect revascularization, predominantly performed at the Seattle Children's Hospital. Data were acquired via retrospective review of patients treated at both institutions. Time to event analysis via multivariable Cox regression for postoperative ipsilateral ischemic stroke or transient ischemic attack (TIA) recurrence was performed.

RESULTS

Forty-nine patients with a median (IQR) age of 6.0 (3.0-9.0) years were included. Twenty-four patients (49.0%) were treated nonsurgically, while 25 patients (51.0%) were treated surgically. Among the 42 patients with subtype data available, 15 (35.7%) had moyamoya disease and 27 (64.3%) had moyamoya syndrome. On Kaplan-Meier analysis, patients who underwent nonsurgical management had 12-, 24-, and 60-month recurrence-free rates from an ipsilateral ischemic event of 52.8%, 52.8%, and 31.7%, respectively. In contrast, patients who underwent surgical management had 12-, 24-, and 60-month recurrence-free rates from an ipsilateral ischemic event of 87.7%, 83.0%, and 83.0%, respectively. In the entire cohort, surgical intervention (HR 0.15, 95% CI 0.04-0.51) and White race (HR 0.18, 95% CI 0.06-0.56) were both independently associated with longer time to ipsilateral ischemic event recurrence.

CONCLUSIONS

Pediatric patients with symptomatic ischemic moyamoya arteriopathy treated nonsurgically had worse outcomes compared with those managed surgically. These findings support pursuing surgical revascularization therapy in North American children and youth with symptomatic ischemic moyamoya arteriopathy.