Clinical features and outcomes of paediatric moyamoya vasculopathy in Malaysia

Abstract

Background: Moyamoya vasculopathy (MMV) is a chronic progressive occlusive cerebrovascular disease causing recurrent strokes in children. We describe the clinical features, treatment and outcomes of childhood MMV in Malaysia; and compared their neurological outcome with the published literature.

Methods: A retrospective study between 2005–2020 of Malaysian children with MMV seen at 4 tertiary Malaysian hospitals. Their post-stroke outcomes were assessed using the modified Rankin scale (mRS) and Pediatric Stroke Outcome Measure (PSOM).

Results: Twenty one cases were included with a median age of presentation at 5.8 years and a median follow-up period of 4.6 years. The female-to-male-ratio was 2:1. All patients had acute ischaemic cerebrovascular accidents. The anterior cerebral circulation was affected in all cases with additional three having also posterior circulation involvement. Among 17 (81%) patients neuroimaging showed bilateral moyamoya. Eight (30%) had preceding febrile illness and the commonest presenting symptom was hemiparesis seen in 8 (38%). Seven (35%) had surgical revascularisation whilst the remainder opted for conservative management. Nineteen (90%) achieved a good stroke outcome but 2 had a poor PSOM outcome and 1 also had a poor mRS outcome. Both these patients declined surgical revascularisation.

Conclusion: Our cohort demonstrated the following MMV features: young age at the first presentation, female and Chinese preponderance; the main type of MMV was moyamoya disease; and ischaemic stroke as the commonest presentation. The overall neurological and functional outcomes were good despite the parental preference on medical therapy over surgical revascularization.