Clinical outcomes after revascularization for pediatric moyamoya disease and syndrome: A single-center series. (September 2020)

• Record Type: Journal Article
• Authors: Morshed, Ramin A.; Abla, Adib A.; Murph, Daniel; Dao, Jasmin M.; Winkler, Ethan A.; Burkhardt, Jan-Karl; Colao, Kathleen; Hetts, Steven W.; Fullerton, Heather J.; Lawton, Michael T.; Gupta, Nalin; Fox, Christine K.
• Is Part Of: Journal of clinical neuroscience. Volume 79(2020)
• Page Start: 137
• Page End: 143
• Publication Date: 2020-09
• Subjects: Moyamoya disease -- Direct and indirect revascularization technique -- STA-MCA bypass -- Vascular disorders; Brain -- Surgery -- Periodicals; Neurosciences -- Periodicals; Nervous system -- Surgery -- Periodicals; Brain -- surgery -- Periodicals; Neurosurgical Procedures -- Periodicals; Neurosciences -- Periodicals; Electronic journals; 616.8
• DOI: 10.1016/j.jocn.2020.07.016
• Languages: English
• ISSNs: 0967-5868

Abstract

Highlights: The rate of direct bypass failure in children is low (5.7%). A combined revascularization technique is feasible even for very young children. High preoperative PSOM predicts worse good clinical outcome on follow-up. Children < 5.4 years old with moyamoya have worse clinical outcomes. Abstract: Moyamoya is a progressive cerebrovascular arteriopathy that affects children of any age. The goal of this study was to determine imaging and clinical outcomes as well as complication rates in a pediatric cohort undergoing either a combined direct/indirect or indirect-only revascularization approach. Patients with moyamoya disease or syndrome ≤ 18 years of age at the time of initial surgery were identified, and clinical data were collected retrospectively. Over a 12-year period, 26 patients underwent revascularization procedures on 49 hemispheres with a median follow-up of 2.6 years from surgery. Median age at surgery was 7.3 years (range 1.4–18.0 years). Thirty-three hemispheres (67.3%) underwent combined revascularization with a direct bypass and encephalomyosynangiosis, and sixteen hemispheres (32.7%) underwent indirect-only revascularization. The rate of 30-day perioperative complication was 10.2%, and the rate of postoperative clinical stroke by end of follow-up was 10.2% by hemisphere. There was a 5.7% rate of intraoperative bypass failure requiring conversion to an indirect revascularization approach. On follow-up imaging, 96.9% of direct bypasses remained patent. On multivariate analysis, higher preoperative Pediatric Stroke Outcome Measure (PSOM) scores were associated with lower rates of good clinical outcome on follow-up (unit OR 0.03; p = 0.03). Patients with age < 5.4 years had lower rates of good clinical outcome on follow-up. In this North American cohort, both combined direct/indirect and indirect only revascularization techniques were feasible. However, younger children < 5.4 years of age have worse outcomes than older children, similar to east Asian cohorts.