Moyamoya disease: Experience with direct and indirect revascularization in 70 patients from a nonendemic region

Introduction: Moyamoya disease (MMD) is a chronic cerebrovascular disease characterized by stenosis/occlusion of bilateral terminal internal carotid arteries with the development of collaterals at base of the brain. We describe our experience of treating 70 patients (133 involved hemispheres, 108 hemispheres operated) surgically. Patients and Methods: Surgically treated patients with MMD from 2006 to 2014 were reviewed retrospectively. There were 70 patients (54 pediatric, 36 females, range 2-46 years). Seven had a unilateral disease. One hundred and eight of 133 hemispheres underwent surgery; 58 underwent combined revascularization (superficial temporal artery middle cerebral artery bypass and encephalo-duro-arterio-myo-synangiosis [EDAMS]); and, 50 underwent an indirect revascularization (EDAMS). 17/23 adult brain hemispheres and 41/85 pediatric hemispheres underwent combined revascularization. Results: The median follow-up was 15.9 months (range 3-62 months). The mean modified Rankin scale score at presentation and follow-up were 2.27 1.034 and 1.80 1.269, respectively. There was an overall significant improvement in the clinical status in the operated patients (P < 0.001) at follow-up. The clinical improvement (admission to follow-up) was better in pediatric patients compared to the adults (P < 0.001 vs. 0.769). The combined revascularization gave better clinical improvement than the indirect one (P = 0.024 vs. 0.0312). There were three postoperative strokes and one death. The morbidity rate was 2.8%, and the mortality rate, 1.4%. Other patients were symptom-free at the latest follow-up. Angiographic outcome did not statistically correlate with age, anastomosis type, or Suzuki grade, though there was trend toward better angiographic outcomes in patients who underwent a combined revascularization. Conclusion: Both the combined and indirect revascularization procedures are effective in treating MMD. Pediatric patients had a better clinical improvement after surgery than the adult patients . Patients undergoing combined revascularization had a better clinical status compared to those who only underwent indirect revascularization. Combined revascularization surgery should be the surgical strategy in all age groups as it is feasible in a significant proportion of pediatric patients too.