Spontaneous closure of a superior sagittal sinus dural arteriovenous fistula with an extensive angioarchitectural network: A case report and systematic review of the literature

Abstract

Background: Intracranial dural arteriovenous fistulas (DAVFs) have been documented to occasionally spontaneously regress. However, the mechanism responsible for this occurrence remains speculative. Methods: We present a case of a Borden II – Cognard IIa+b DAVF involving the superior sagittal sinus (SSS) with bilateral external carotid artery supply that regressed spontaneously. A systematic literature review was conducted to explore the current theories explaining the spontaneous regression of DAVFs according to Preferred Reporting Items for Systematic reviews and Meta-Analyses guidelines. Results: A total of 26 studies and 54 cases were included in our results. Of the included cases, 57.14% of cases were Borden I, 16.33% were Borden II, and 26.53% were Borden III. Ruptured status or intracranial hemorrhage was documented in 24.1% of all cases, the majority of which (69.2%) were in cases with aggressive lesions (Borden II or greater). The most commonly involved location was the transverse sinus (38.89% of cases, n = 21), and the SSS was only involved in 12.96% of all cases. 50% of included cases proposed a mechanism responsible for spontaneous regression. The most frequently proposed mechanisms were thrombosis of the involved sinus/chronic inflammatory changes or direct endothelial injury, endoluminal stasis, and thrombogenic effects of contrast medium during angiography. We present the case of a 54-year-old woman with an aggressive ruptured DAVF that likely developed following a pediatric traumatic brain injury that was left untreated before she presented to our institution after significant delay. Her DAVF regressed on repeat angiography before neurovascular intervention without a clear identifying mechanism as proposed by the current literature. Conclusion: Our results suggest that spontaneous regression is not necessarily associated with lower risk DAVFs. The present case offers a unique long-term insight into the natural history of an aggressive ruptured DAVF of the SSS that regressed without intervention. Further research into the natural history of DAVFs will be helpful in deducing key factors leading to spontaneous regression.

Case report: Sphenoid wing dural arteriovenous fistula draining into ophthalmic veins inducing pulsatile tinnitus as the sole symptom and its spontaneous closure

“…In a recent systematic review of 54 spontaneous regression cases in the literature, the cases documenting the spontaneous regression of intracranial DAVFs were almost evenly distributed between low-risk (11,12). This lends support to the notion that lower-risk DAVFs do not necessarily indicate higher rates of spontaneous regression (12). Sinus thrombosis is recognized as a significant factor contributing to the spontaneous closure of DAVF.…”

“…In a recent systematic review of 54 spontaneous regression cases in the literature, the cases documenting the spontaneous regression of intracranial DAVFs were almost evenly distributed between low-risk lesions, comprising 57.14% classified as Borden I and 44.82% categorized as Cognard IIa or lower, and aggressive high-risk lesions, consisting of 42.58% categorized as Borden II or greater and 55.18% classified as Cognard IIb or greater ( 11 , 12 ). This lends support to the notion that lower-risk DAVFs do not necessarily indicate higher rates of spontaneous regression ( 12 ). Sinus thrombosis is recognized as a significant factor contributing to the spontaneous closure of DAVF.…”

“…However, alternative mechanisms, such as intracerebral hemorrhage resulting in mass effect and secondary vasospasm, have also been proposed as potential explanations for the spontaneous closure of DAVFs ( 11 ). The resolution of DAVF can transpire via either the direct closure of the shunt or an enlargement of the sinus due to structural alterations, subsequently leading to the compression of the arteriovenous shunts within the wall, resulting in DAVF occlusion ( 12 ). It is worth noting that the second scenario, however, was not observed in our specific case.…”