title: Treatment of a cerebral pial arteriovenous fistula in a patient with sickle cell disease-related moyamoya syndrome: case report.
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.
authors.affiliation: Division of Pediatric Neurosurgery, Department of Neurosurgery, Miami Children's Hospital, University of Miami/Miller School of Medicine;
authors.forename: Anna
authors.initials: A
authors.lastname: Lo Presti
authors.affiliation: Division of Pediatric Neurosurgery, Department of Neurosurgery, Miami Children's Hospital, University of Miami/Miller School of Medicine;
authors.forename: Alexander G
authors.initials: AG
authors.lastname: Weil
authors.affiliation: Division of Pediatric Neurosurgery, Department of Neurosurgery, Miami Children's Hospital, University of Miami/Miller School of Medicine;
authors.forename: Aria
authors.initials: A
authors.lastname: Fallah
authors.affiliation: Division of Neuroendovascular Surgery, Department of Neurosurgery, University of Miami/Miller School of Medicine, Miami, Florida.
authors.forename: Eric C
authors.initials: EC
authors.lastname: Peterson
authors.affiliation: Division of Pediatric Neurosurgery, Department of Neurosurgery, Miami Children's Hospital, University of Miami/Miller School of Medicine;
authors.forename: Toba N
authors.initials: TN
authors.lastname: Niazi
authors.affiliation: Division of Pediatric Neurosurgery, Department of Neurosurgery, Miami Children's Hospital, University of Miami/Miller School of Medicine;
authors.forename: Sanjiv
authors.initials: S
authors.lastname: Bhatia
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications*; D017542:Aneurysm, Ruptured / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D004621:Embolization, Therapeutic; D006801:Humans; D002532:Intracranial Aneurysm / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D002538:Intracranial Arteriovenous Malformations / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D008279:Magnetic Resonance Imaging; D008297:Male; D020768:Middle Cerebral Artery / Q000601:surgery; D009072:Moyamoya Disease / Q000209:etiology / Q000601:surgery*; D010841:Pia Mater / Q000098:blood supply; D020521:Stroke / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D013699:Temporal Arteries / Q000601:surgery
keywords: AED = antiepileptic drug; AVF = arteriovenous fistula; AVM = arteriovenous malformation; HHT = hemorrhagic hereditary telangiectasia; ICA = internal carotid artery; MCA = middle cerebral artery; Onyx; SCD = sickle cell disease; STA = superficial temporal artery; dAVF = dural AVF; encephaloduroarteriosynangiosis; moyamoya syndrome; pAVF = pial AVF; pial arteriovenous fistula; sickle cell anemia; vascular disorders