The Collateral Circulation in Moyamoya Disease: A Single-Center Experience in 140 Pediatric Patients

Area of Science:

• Neurology
• Pediatric Neurology
• Vascular Neurology

Background:

• Moyamoya disease is a rare cerebrovascular disorder characterized by progressive stenosis of the terminal internal carotid arteries.
• The role and clinical significance of collateral circulation patterns in pediatric moyamoya disease remain incompletely understood.
• Understanding collateral origins is crucial for assessing disease severity and predicting outcomes in affected children.

Purpose of the Study:

• To investigate the clinical features and significance of collateral circulation in pediatric moyamoya disease.
• To differentiate collaterals based on their anatomical origin: internal carotid artery and vertebral artery (ICA-VA) versus external carotid artery (ECA).
• To correlate collateral patterns with disease severity (Suzuki stage) and clinical presentation, specifically infarction.

Main Methods:

• Retrospective review of 140 pediatric patients diagnosed with moyamoya vasculopathy between 2009 and 2015.
• Inclusion criteria required digital subtraction angiography (DSA) for detailed vascular assessment.
• Spontaneous collaterals were categorized into ICA-VA-originated and ECA-originated groups.

Main Results:

• Collaterals originating from the ICA-VA system were identified in 87.1% of patients (222 hemispheres), with no significant difference in Suzuki stages compared to patients without these collaterals.
• Collaterals originating from the ECA system were present in 50.0% of patients (106 hemispheres).
• Patients with ECA-originated collaterals experienced significantly more infarctions than those without (25 vs. 12, P=0.021).

Conclusions:

• Collaterals originating from the external carotid artery (ECA) are significantly associated with advanced Suzuki stages in pediatric moyamoya disease.
• The presence of ECA-originated collaterals is linked to a higher incidence of infarction presentations in children.
• These findings highlight the clinical importance of ECA collaterals as a marker of disease severity and ischemic risk in pediatric moyamoya vasculopathy.