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Moyamoya Disease in Children: Results From the International Pediatric Stroke Study
Sarah Lee1, Michael J Rivkin2, Adam Kirton3
11 Department of Neurology & Neurological Sciences, Stanford University, Stanford, CA, USA.
Insights
Moyamoya disease affects children, often causing ischemic strokes. Surgical revascularization significantly reduces the risk of recurrent strokes in pediatric patients with this rare condition.
Area of Science:
- Neurology
- Pediatric Stroke
- Vascular Neurology
Background:
- Moyamoya disease is a rare, progressive cerebrovascular disorder.
- It primarily affects children and can lead to recurrent ischemic strokes.
- A significant proportion of pediatric moyamoya disease cases are associated with underlying syndromes.
Purpose of the Study:
- To characterize pediatric patients diagnosed with moyamoya disease using international stroke registry data.
- To identify risk factors associated with stroke recurrence in this population.
- To evaluate the effectiveness of surgical revascularization in preventing recurrent strokes.
Main Methods:
- Retrospective analysis of data from the International Pediatric Stroke Study (2003-2013).
- Inclusion criteria: children over 28 days old diagnosed with moyamoya disease.
- Data collected included demographics, stroke type, presence of moyamoya syndrome, treatment, and recurrence events.
Main Results:
- 174 children from 32 sites and 14 countries were included.
- The majority (90%) presented with ischemic stroke; 20% experienced stroke recurrence over a median follow-up of 13 months.
- Surgical revascularization was associated with a significantly lower rate of stroke recurrence (P = .046).
Conclusions:
- Moyamoya disease represents a notable cause of arterial stroke in children.
- Surgical revascularization is an effective intervention for reducing stroke recurrence in pediatric moyamoya disease.
- One-third of pediatric moyamoya disease cases are linked to syndromic conditions, highlighting the need for comprehensive evaluation.
Abstract:
This study aimed to describe children with moyamoya disease from an international multicenter stroke database, and explore risk factors for stroke recurrence. We reviewed data of children >28-days old with moyamoya disease enrolled in the International Pediatric Stroke Study from January 2003 to March 2013. A total of 174 children from 32 sites and 14 countries had moyamoya disease; median age 7.4 years, 49% male. Of these, 90% presented with ischemic stroke, 7.5% with transient ischemic attack, and 2.5% with hemorrhagic stroke. One-third of patients had moyamoya syndrome. Stroke recurrence was 20% over median follow-up of 13 months; 9% had multiple recurrences. Children treated with surgical revascularization were less likely to have stroke recurrence ( P = .046). Moyamoya disease accounted for 8% of arterial strokes in this international pediatric stroke registry. One-third of pediatric patients with moyamoya disease have an underlying syndromic condition. Surgical revascularization is effective at reducing the incidence of stroke recurrence.
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