Background: Paediatric moyamoya disease is characterized by chronic occlusive cerebral vasculature around the circle of Willis with the formation of collaterals. Its outcome is variable, depending on the disease severity on presentation, progression and timeliness of surgical intervention.

Objective: This study examines the outcome of a cohort of children with moyamoya disease, mostly managed conservatively.

Method: All children diagnosed with moyamoya disease in Penang Hospital, Malaysia were followed up longitudinally to monitor disease progression and outcome at last review.

Results: Of 7 patients (median age: 7.6 years, range: 1.3-17.8), five (71%) were female. Six had moyamoya disease and one with underlying Down syndrome. All of them were diagnosed in the first decade (median age – 5.4 years (range 0.3-9.7). Five and 2 patients presented with arterial ischemic stroke (AIS) and transient ischemic attack (TIA) respectively. Six (85%) had bilateral vascular disease. All of them were put on single anti-platelet agent (6 aspirin & 1 clopidogrel) on diagnosis. During mean follow-up of 3.8 years, serial MRAs showed progressive disease in two children. One had recurrent TIAs and the other one suffered from an episode of AIS. Revascularization surgery was performed on one of them. All the five patients with static disease were stable with aspirin. When last review, 3 had residual motor deficit and 2 with developmental delay / learning difficulty. The overall Pediatric Stroke Outcome Measure (PSOM) was low (0.1).

Conclusion: In the resource-limited areas, children with stable disease can safely be managed conservatively.