Structural causes of ischemic and hemorrhagic stroke in children: moyamoya and arteriovenous malformations

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Purpose of review

Moyamoya and arteriovenous malformations represent, respectively, significant sources of ischemic and hemorrhagic stroke in children after the first year of life. Although rarely encountered in routine pediatric practice, the potential severe morbidity of these entities, coupled with the typical acuity of their presentation, merit ongoing awareness of current relevant diagnostic and therapeutic strategies.

Recent findings

Mutations in RNF213, ACTA2, and GUCY are implicated in moyamoya. Several common pediatric conditions – trisomy 21, sickle-cell disease, and neurofibromatosis type I – demonstrate an increased risk of moyamoya development. Advances in imaging have improved the diagnosis of moyamoya and surgical revascularization has been further supported as the primary treatment.

Recent findings

Genetic associations with arteriovenous malformations (AVMs) are few outside of hereditary hemorrhagic telangiectasia. Within this population, the majority harbor mutations in ENG and ACVRL. Once screened, if no AVM is found, repeat scanning may not be needed for 5 years. Trauma and infection may not be ‘triggers’ for inducing hemorrhage in patients with untreated AVMs. If found in children, evidence supports the treatment of the AVM, ideally with surgery.


Moyamoya and AVMs are rare but important causes of stroke in children. If identified, it is important to refer the children to pediatric centers with experienced neurovascular teams. Surgical revascularization of moyamoya and resection of AVMs should be strongly considered, including incidentally discovered asymptomatic disease.

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