Revascularisation surgery for paediatric moyamoya disease: The Singapore experience

Moyamoya disease (MMD) is characterized by the spontaneous occlusion of the distal internal carotid arteries and resultant neo-angiogenesis of fragile collateral blood vessels. Direct and indirect revascularization surgeries have shown to effectively reduce stroke risks in paediatric MMD, whereby the latter is a more utilised technique in children. This study was undertaken to determine the outcomes of revascularization in Singapore's multi-ethnic, Southeast Asian paediatric population. This is an ethics-approved study conducted in Singapore's 2 tertiary children hospital units: KK Women's and Children's Hospital and National University Hospital. Sixteen patients with a diagnosis of ischaemic-type MMD are recruited between 01 January 2002 to 31 January 2019; and a total of 24 surgeries are undertaken (24 cerebral hemispheres). There are 2 cases of stroke within 30 days post-surgery. However, no stroke recurrence is observed beyond 30 days after surgery in all patients. Four patients reported recurrent transient ischaemic attack symptoms in the follow-up period ranging from 3 months to 12 years. Data analyses show a statistically significant improvement in modified Rankin's Scale (mMRS) in post-operative patients from baseline to discharge, and at 3 months after surgery. Our study also observes that predictors of recurrent ischaemic events include higher pre-operative MRS, Suzuki stage and perioperative infarction. To the authors' knowledge, this is the first study to date reporting the outcomes of revascularisation in a paediatric Southeast Asian cohort.