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Primary antiphospholipid syndrome with moyamoya-like vascular changes
F Booth1, R Yanofsky, I B Ross
1Department of Pediatrics, University of Manitoba, Winnipeg, Manitoba, Canada.
Pediatric Neurosurgery
|November 5, 1999
Summary
Antiphospholipid syndrome (APS) in a child caused moyamoya-like changes. Anticoagulation therapy reversed these cerebrovascular abnormalities, improving blood flow and preventing further vascular damage.
Area of Science:
- Neurology
- Vascular Medicine
- Pediatrics
Background:
- Antiphospholipid syndrome (APS) is an autoimmune disorder associated with an increased risk of thrombosis.
- Moyamoya disease is a rare, progressive cerebrovascular disorder characterized by stenosis of the terminal internal carotid arteries.
- Cerebrovascular changes resembling moyamoya have been reported in association with APS.
Observation:
- A pediatric patient presented with clinical and radiological evidence of moyamoya-like cerebrovascular changes.
- The patient was diagnosed with antiphospholipid syndrome (APS).
Findings:
- Treatment with anticoagulation led to the reversal of the moyamoya-like cerebrovascular changes.
- Resolution of thrombus formation was observed, suggesting improved cerebrovascular circulation.
- Despite the risk of hemorrhage from collateral vessels, anticoagulation was deemed beneficial.
Implications:
- Anticoagulation therapy may be a viable treatment to prevent the progression of vascular abnormalities in pediatric APS.
- This case highlights the potential for reversible cerebrovascular changes in APS with appropriate management.
- Further research is warranted to explore the efficacy and safety of anticoagulation in similar pediatric cases.