Childhood Moyamoya: Looking Back to the Future

Area of Science:

• Neurology
• Vascular Biology
• Pediatric Neurology

Background:

• Moyamoya is a chronic, progressive arteriopathy affecting the circle of Willis.
• It leads to a network of collateral vessels, creating a characteristic "puff of smoke" angiographic appearance.
• This condition presents a lifelong risk of stroke and neurological decline, with a peak incidence in children aged 5-10 years.

Purpose of the Study:

• To summarize the current understanding of Moyamoya disease and syndrome.
• To highlight the clinical presentation, diagnostic features, and management strategies.
• To identify areas for future research in Moyamoya disease.

Main Methods:

• Review of existing literature on Moyamoya disease and syndrome.
• Analysis of clinical presentation, diagnostic criteria, and treatment outcomes.
• Identification of knowledge gaps in pathophysiology and management.

Main Results:

• Moyamoya disease is distinct from Moyamoya syndrome, which is associated with other disorders.
• Geographic and ethnic variations in incidence exist, with a higher prevalence in East Asia.
• Current management focuses on antiplatelet therapy and surgical revascularization due to the lack of disease-modifying treatments.

Conclusions:

• Moyamoya disease and syndrome require lifelong management due to persistent stroke risk.
• Further research is crucial to understand its pathological basis and optimize surgical timing and outcomes.
• There is an unmet need for treatments that can slow the progression of this childhood arteriopathy.