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

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.