Revascularization for moyamoya disease: five-year follow-up

Area of Science:

• Neurology
• Pediatric Neurosurgery
• Vascular Neurology

Background:

• Moyamoya disease is a rare, progressive cerebrovascular disorder characterized by stenosis of the terminal internal carotid arteries and the development of collateral circulation.
• Severe moyamoya disease in pediatric patients presents significant challenges due to the risk of ischemic or hemorrhagic stroke.
• Early diagnosis and intervention are crucial for managing moyamoya disease in children to prevent neurological deficits.

Observation:

• A 4-year-old child with severe moyamoya disease affecting both anterior and posterior circulations was evaluated.
• The patient underwent encephaloduroarteriosynangiosis, a surgical procedure to improve cerebral blood flow.
• Serial angiography was performed over a 5-year follow-up period to monitor disease progression and treatment efficacy.

Findings:

• The surgical intervention led to early clinical stabilization in the pediatric patient.
• Despite pre-existing neurological deficits, the child demonstrated normal or superior intellectual development post-surgery.
• Follow-up angiography revealed the formation of significant direct anastomotic channels, indicating successful collateral development.

Implications:

• Encephaloduroarteriosynangiosis can be an effective treatment for severe moyamoya disease in children, promoting long-term clinical stability.
• The development of robust collateral circulation post-surgery supports favorable neurodevelopmental outcomes, even in the presence of early deficits.
• This case highlights the potential for surgical revascularization to alter the natural course of moyamoya disease and improve quality of life in affected children.