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

We present a case of a 51-year-old female who presented for evaluation of a large scalp mass found to have a different quartet of vascular malformations- a persistent scalp arteriovenous malformation (sAVM) with sinus pericranii, an inoperable intracranial SM-V brain arteriovenous malformation (bAVM), and a Cognard I dural arteriovenous fistula (dAVF). This is the first reported instance with four distinct vascular pathologies. We review the etiologies of multiple vascular abnormalities in the cerebral circulation that could contribute to this patient's findings and review strategies for treatment. We conducted a retrospective review of the clinical and angiographic records for a single adult female patient, including a management approach and an in-depth literature review. Given the high baseline vascularity of these complex lesions, surgery was not considered the initial therapy. We focused primarily on the sAVM with a staged embolization involving both transarterial and transvenous approaches. Transarterial coil embolizes 5 feeding artery branches of the right external carotid artery, followed by transvenous coil embolization into the common venous pouch accessed through the transosseous sinus pericranii via the SSS, dramatically reduced the size and filling of the large sAVM and eliminated a significant 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 continued new development of collaterals. Ultimately the patient elected to forego further treatment for her sAVM. To our knowledge, there has not been another report of a single adult patient with a quartet of vascular malformations in the literature. Treatment paradigms for sAVMs are limited to case reports and small series; however, we purport that the most successful therapeutic approaches are multimodal and likely should incorporate surgical resection when feasible. We emphasize the caution required for patients with multiple other underlying intracranial vascular malformations. The altered intracranial flow dynamics can drastically hinder the success of a unimodal approach involving endovascular therapy alone.