Combination of Perioperative Cilostazol and Aspirin in Pediatric Moyamoya: A Case Series

Background: Cilostazol is a phosphodiesterase III inhibitor that causes mild platelet aggregation inhibition and vasodilation. Observational studies have demonstrated benefit in adult moyamoya arteriopathy (MMA). Safety, tolerability, and efficacy of cilostazol has not been reported in pediatric MMA patients, where aspirin has been the most frequently used antiplatelet agent. Methods: We reviewed pediatric patients at two institutions who received cilostazol and low-dose aspirin for MMA or steno-occlusive disease. Intraoperative complications, postoperative bleeding, and strokes were evaluated. Results: We identified 13 patients between ages 6 months to 21 years, ten (77%) who started cilostazol before revascularization surgery and three (23%) who started cilostazol after surgery. No patient had an increase in ischemic symptoms after initiation of cilostazol. One patient had a spontaneous parenchymal hemorrhage intraoperatively, and one patient had postoperative strokes. Up to 1 year follow-up on cilostazol, 8 (61%) patients reported no transient ischemic attacks or strokes. In this report of cilostazol use in pediatric patients with MMA undergoing direct or indirect revascularization surgery, we observed a low rate of bleeding and other adverse events. No instances of steal phenomena from cilostazol were identified. Conclusion: These data suggest that off-label cilostazol may be safely used in children with MMA. Larger studies are warranted to evaluate the safety and efficacy of cilostazol in pediatric MMA/steno-occlusive disease.