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Revascularisation surgery for paediatric moyamoya disease: The Singapore experience
Jamie Ah Ong1, Sharon Yy Low2, Wan Tew Seow2
1Yong Loo Lin School of Medicine, National University of Singapore, NUHS Tower Block, 1E Kent Ridge Road Level 11, 119228, Singapore.
Abstract:
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.
Insights
Revascularization surgery significantly improves outcomes for children with Moyamoya disease (MMD), reducing stroke risk and improving functional status. This study highlights the effectiveness of these procedures in a Southeast Asian pediatric cohort.
Area of Science:
- Neurology
- Neurosurgery
- Pediatric Stroke
Background:
- Moyamoya disease (MMD) involves artery blockages in the brain, leading to fragile new blood vessels and increased stroke risk, particularly in children.
- Revascularization surgery, especially indirect methods, is a key treatment for pediatric MMD to improve blood flow and prevent strokes.
Purpose of the Study:
- To evaluate the outcomes of revascularization surgery for ischemic-type Moyamoya disease in Singapore's diverse pediatric population.
- To assess the safety and efficacy of surgical interventions in reducing stroke recurrence and improving functional outcomes in young MMD patients.
Main Methods:
- Retrospective analysis of 16 pediatric patients (24 hemispheres) undergoing revascularization between 2002 and 2019 at two tertiary hospitals.
- Assessment of stroke events, transient ischemic attacks, and modified Rankin's Scale (mRS) scores pre- and post-operatively.
Main Results:
- A statistically significant improvement in modified Rankin's Scale (mRS) was observed from baseline to discharge and at 3 months post-surgery.
- Two strokes occurred within 30 days post-surgery, with no recurrence beyond this period. Four patients experienced recurrent transient ischemic attacks.
- Higher pre-operative mRS, Suzuki stage, and perioperative infarction were identified as predictors of recurrent ischemic events.
Conclusions:
- Revascularization surgery is effective in improving functional outcomes and reducing long-term stroke risk in pediatric Moyamoya disease patients in a Southeast Asian cohort.
- While early postoperative strokes can occur, the procedure demonstrates significant benefits for long-term neurological recovery and quality of life.
