Clinical features and outcomes of paediatric moyamoya vasculopathy in Malaysia

• Main Authors: Lim, Wei Kang, Ong, Lai Choo, Tan, Khian Aun, Li, Limin, Teh, Chee Ming, Heng, Hock Sin, Murugesu, Sumitha, Fong, Choong Yi
• Format: Article
• Published: ASEAN Neurological Association 2022
• Subjects: R Medicine; RC Internal medicine; RC0321 Neuroscience. Biological psychiatry. Neuropsychiatry; RJ Pediatrics
• Online Access: http://eprints.um.edu.my/40904/

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.