Abstract
Sickle cell disease (SCD) is an autosomal recessive hematological disorder, characterized by sickling of the red blood cells in response to a hypoxic stress and vaso-occlusive crises. It is associated with moyamoya-like changes on cerebral angiographic imaging in 43% of patients. Cerebral aneurysms, arteriovenous malformations, and dural arteriovenous fistulas (AVFs) have been described in association with SCD and moyamoya disease. However, the description of a pial AVF (pAVF) in a patient with SCD and/or moyamoya formation has not yet been reported. The authors present the case of a 15-year-old boy with SCD-associated moyamoya disease harboring a pAVF who developed a de novo venous aneurysm 8 months after undergoing indirect superficial temporal artery-middle cerebral artery (MCA) bypass that was complicated by bilateral ischemia of the MCA territory. The pAVF was successfully treated with transarterial embolization using Onyx. The authors describe the possible pathophysiological mechanisms and management strategies for this rare occurrence.
Original language | English (US) |
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Pages (from-to) | 207-211 |
Number of pages | 5 |
Journal | Journal of Neurosurgery: Pediatrics |
Volume | 16 |
Issue number | 2 |
DOIs | |
State | Published - Aug 2015 |
Keywords
- Encephaloduroarteriosynangiosis
- Moyamoya syndrome
- Onyx
- Pial arteriovenous fistula
- Sickle cell anemia
- Vascular disorders
ASJC Scopus subject areas
- Surgery
- Pediatrics, Perinatology, and Child Health
- Clinical Neurology