Progression of unilateral moyamoya disease resulted in spontaneous occlusion of ipsilateral cavernous dural arteriovenous fistula: Case report

Interv Neuroradiol. 2016 Jun;22(3):362-4. doi: 10.1177/1591019916632371. Epub 2016 Feb 24.


The pathogenic association between cavernous dural arteriovenous fistula (CDAVF) and moyamoya disease remains unclear. This unusual case is the first report of a progression of unilateral moyamoya disease resulting in the spontaneous occlusion of ipsilateral CDAVF. A 52-year-old woman presented with two-week spontaneous exophthalmos, chemosis and tinnitus, and cerebral angiography showed a right CDAVF coexisting with ipsilateral moyamoya disease. Transvenous approaches through the inferior petrosal sinus and facial vein were attempted but failed. However, a progression of the moyamoya disease and disappearance of the CDAVF were observed on one month follow-up angiogram in accordance with the resolution of clinical symptoms. This extremely rare coincidental presentation may have deeper pathogenic implications. This case report may give a clue to the underlying mechanism of the progression of moyamoya disease and occlusion of the CDAVF.

Keywords: Cavernous arteriovenous fistula; moyamoya disease; spontaneous occlusion.

Publication types

  • Case Reports

MeSH terms

  • Biomarkers / analysis
  • Cavernous Sinus / diagnostic imaging
  • Central Nervous System Vascular Malformations / diagnostic imaging
  • Central Nervous System Vascular Malformations / etiology*
  • Central Nervous System Vascular Malformations / therapy
  • Cerebral Angiography
  • Diagnosis, Differential
  • Disease Progression
  • Embolization, Therapeutic
  • Female
  • Humans
  • Magnetic Resonance Angiography
  • Middle Aged
  • Moyamoya Disease / complications*


  • Biomarkers