Progressive Intracranial Arteriovenous Fistula Over Forty Years: A Case Report

TL;DR

A rare case of a slowly progressing brain arteriovenous fistula over 40 years is reported, highlighting the need for long-term monitoring and timely treatment.

Contribution

This case report documents the unusual long-term progression of a dural arteriovenous fistula with atypical clinical features.

Findings

The patient presented with scalp bleeding from a vascular mass without neurological deficits.

Surgical ligation of external carotid artery feeders led to clinical improvement.

The fistula showed progressive changes over four decades with no typical dAVF symptoms.

Abstract

This report highlights the rare progression of a dural arteriovenous fistula (dAVF) over four decades, evolving from an initially benign presentation to aggressive hemorrhagic features. A 58-year-old male presented with uncontrollable scalp bleeding secondary to a pulsating mass on the right temporal area. Diagnostic cerebral angiography revealed a complex right transverse sinus dAVF with multiple arterial feeders, primarily from the bilateral superficial temporal arteries (STA) and occipital arteries via the external carotid arteries (ECA). Internal carotid artery (ICA) injections failed to adequately opacify due to advanced atherosclerotic changes. No cortical venous reflux or venous ectasia was identified; instead, a serpiginous network of ECA feeders was observed, likely due to collateral development following a prior ligation in 1979. Clinically, the patient’s only presenting complaint was persistent scalp hemorrhage from the vascular mass. He exhibited no neurological deficits preoperatively, and no steal phenomena or other typical dAVF-related symptoms were observed. The hemorrhage, initially manifesting as anemia, was managed conservatively with transfusions before definitive treatment. Surgical ligation of the ECA feeders was performed via an open technique. As this did not involve an endovascular embolization, no immediate postoperative angiogram was obtained. The patient showed significant clinical improvement and was discharged in a stable condition without any neurological deficits. This report underscores the importance of patient compliance, long-term follow-up, and timely neurosurgical intervention in managing progressive dAVFs, particularly those evolving over decades with atypical presentations.

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