A Vascular Quartet: Scalp Arteriovenous Malformation, Sinus Pericranii, Dural Arteriovenous Fistula, and Brain Arteriovenous Malformation in a Single Patient

Introduction: We review the etiologies of multiple vascular abnormalities in the cerebral circulation contributing to the finding of a distinct quartet of vascular malformations in a single adult patient - a persistent scalp AVM with sinus pericranii, an inoperable intracranial SM-V bAVM, and a Cognard I dAVF.

Methods: Retrospective review of the clinical and angiographic records for a single adult female patient including management approach and an in depth review of the literature.

Results: Given the high baseline vascularity of these complex lesions, surgery was not considered as the initial therapy. We focused primarily on the sAVM with a staged embolization involving both transarterial and transvenous approaches. Transarterial coil embolization of 5 feeding artery branches of the right external carotid artery followed by transvenous voil embolization into the common venous pouch accessed through the transosseus sinus pericranii via the SSS dramatically reduced the size and filling of the large sAVM and eliminated a major source of hypertensive venous outflow. Serial endovascular treatments of her sAVM led to a significant reduction in size and pulsatility and the pain from tenderness to palpation was concurrently decreased. Despite multiple treatments, serial angiographic evaluations of her scalp lesion showed persistent new development of collaterals. Ultimately the patient relected to forego further treatment for her sAVM.

Conclusion : To our knowledge, there has not been another report of a single adult patient who possesses a quartet of vascular malformations within the literature. Treatment paradigms for sAVMs are limited to case reports and small series, however we purport the most successful therapeutic approaches are multimodal and likely should incorporative surgical resection when feasible. We emphasize the caution required for cases in which patients have multiple other underlying intracranial vascular malformations, as we believe the altered intracranial flow dynamics can drastically hinder the success of a unimodal approach involving endovascular therapy alone.