Abstract

Moyamoya is a progressive cerebral angiopathy that entails a formidable natural history of repeated ischemic or haemorrhagic insults if not intervened. The potential advantages of direct/combined bypass can be harvested only if they are not outweighed by perioperative morbidity. Direct bypasses for the paediatric group have been less utilized because of small vessel calibres and an inherently more robust angioplasticity. This study was undertaken to examine the clinical and perfusion imaging outcomes of operated moyamoya disease or syndrome patients in Hong Kong’s Southeast Asian population. Comparison was made between direct/combined and indirect bypass cohorts. Subgroup analysis of the paediatric cohort was conducted to determine outcomes of a direct-bypass-first strategy. From November 2000 to September 2020, 86 hemispheres underwent revascularization at a tertiary neurosurgical unit with a mean clinical follow-up time of 70.0 months. 70.9% of the procedures involved direct bypasses. Direct/combined revascularization demonstrated superiority in restoring adequate cerebrovascular reserve capacity (CVRC) (63.2% vs 27.3%, p = 0.015), and freedom from transient ischemic attacks in the first 5 years post-operation by Kaplan-Meier plot with log-rank test (p = 0.038). Follow-up imaging revealed 96.5% of the bypass grafts remained patent. Direct/combined procedures significantly predicted restoration of adequate CVRC on follow-up perfusions scans by binary logistic regression (OR 4.57, p = 0.009). Compared to the adult cohort, direct bypasses in children carried no excessive perioperative morbidity. These results support a liberal bypass-first paradigm in both adult and paediatric cohorts.

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