Clinical and Imaging Outcomes After Combined Direct and Indirect Bypass for Pediatric Moyamoya Disease: A Single Center Retrospective Series

Abstract

INTRODUCTION Moyamoya disease is a progressive cerebrovascular arteriopathy of unknown etiology, causing narrowing and eventual occlusion of the internal carotid arteries and their branches. Surgical revascularization is a key component of the overall treatment strategy. The goal of this study was to determine outcomes and complications related to direct vascular bypass in a pediatric cohort with Moyamoya disease. METHODS All pediatric patients (<18 yr of age) treated with direct bypass combined with encephalomyosynangiosis between 2006 and 2017 at the authors’ institution were included in the cohort. Demographic features, treatment, and outcomes were determined retrospectively from clinical reports available through the electronic medical record. RESULTS A total of 18 patients (11 females and 7 males) underwent 32 bypass procedures with a median follow-up of 2.2 yr (range 22 d 8.6 yr). Mean age at operation was 8.3 yr (range 1.4-16.9 yr). Preoperative Suzuki stage 1, 2, 3, 4, and 5 were seen in 2, 7, 7, 12, and 4 hemispheres, respectively. By last follow-up, 3 patients had evidence of new clinical ischemic stroke, all occurring within 30 d of surgery. Favorable outcomes (pediatric modified Rankin scores 0-2) at last follow-up were seen in 15 patients (83.3%). There was 1 death in the cohort due to gastrointestinal bleeding related to underlying medical comorbidities. On review of imaging at last follow-up for surviving patients, 29 of 30 direct arterial bypasses were patent; 12 and 5 patients had stable and worse imaging findings, respectively, when compared to immediate postoperative scans. CONCLUSION Combined direct and indirect revascularization procedures may be successfully applied to pediatric patients with Moyamoya disease of all age groups. The direct bypass component provides immediate revascularization and is associated with high rates of patency on follow-up imaging.

Implementation and Rationale for a Unified Clinical and Imaging Protocol for Evaluation and Treatment of Moyamoya Angiopathy: A Single Institutional Experience

“…Although which revascularization technique to utilize should be determined within the context of each unique patient, current literature demonstrates the superiority of direct bypass to indirect bypass in terms of improving angiographic collateralization, functional cerebrovascular hemodynamics, as well as decreasing the rate of post-operative cerebral events (30,32,(35)(36)(37)(38). This pattern seems to be true for pediatric MMD patients as well (31,39), although, as compared to adults, pediatric patients are more likely to benefit from indirect revascularization procedures alone (36). Given these data, our institutional protocol calls for direct revascularization, either alone or with combined indirect revascularization, in cases of an acceptable donor artery as a first-line option in patients who are surgical candidates.…”

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“…The rare disease is progressive, with an appearance of a puff of smoke in the brain area [5]. It is unfortunate that moyamoya is not curable [6]. However, experts suggest that a cerebral revascularization and vascular bypass surgery are considered effective as they help in diverting blood to nearby muscles from the blood vessel, with oxygen finally reaching the oxygen stared brain areas, also reducing the risks of stroke and long-term drastic outcomes [7].…”