Treatment of a cerebral pial arteriovenous fistula in a patient with sickle cell disease-related moyamoya syndrome: case report

Anna Lo Presti, Alexander G. Weil, Aria Fallah, Eric Peterson, Toba N. Niazi, Sanjiv Bhatia

Research output: Contribution to journalArticle

3 Scopus citations

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 languageEnglish (US)
Pages (from-to)207-211
Number of pages5
JournalJournal of Neurosurgery: Pediatrics
Volume16
Issue number2
DOIs
StatePublished - Aug 1 2015

Keywords

  • AED = antiepileptic drug
  • AVF = arteriovenous fistula
  • AVM = arteriovenous malformation
  • dAVF = dural AVF
  • encephaloduroarteriosynangiosis
  • HHT = hemorrhagic hereditary telangiectasia
  • ICA = internal carotid artery
  • MCA = middle cerebral artery
  • moyamoya syndrome
  • Onyx
  • pAVF = pial AVF
  • pial arteriovenous fistula
  • SCD = sickle cell disease
  • sickle cell anemia
  • STA = superficial temporal artery
  • vascular disorders

ASJC Scopus subject areas

  • Medicine(all)

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