Recurrent Strictly Unilateral Extra-Axial Intracranial Hemorrhage in Severe Hemophilia A Without Inhibitors Revealing a Suspected Dural Arteriovenous Fistula: A Case Report

Abstract

Intracranial hemorrhage is a feared complication of hemophilia A, and recurrence may prompt evaluation beyond systemic hemostatic factors. We report an 18-year-old male with severe hemophilia A and no current inhibitors who experienced three episodes of spontaneous, strictly right-sided extra-axial intracranial hemorrhage (epidural and subdural) over four years. Each event presented with headache and vomiting without antecedent trauma and was managed conservatively with high-dose factor VIII replacement and close neurologic monitoring, resulting in complete clinical recovery and radiologic resolution. After the third ipsilateral event, repeated inhibitor testing remained negative, and systemic risk factor evaluation was unrevealing. Brain magnetic resonance angiography (MRA) demonstrated an ectatic right parietal cortical venous structure draining into the superior sagittal sinus with suspected arteriovenous shunting, raising concern for a dural arteriovenous fistula (DAVF). Digital subtraction angiography (DSA) was recommended for definitive diagnosis, but was not immediately available, and the patient is awaiting transfer. This case highlights that recurrent strictly unilateral extra-axial hemorrhage in hemophilia should prompt investigation for a focal structural or vascular etiology such as DAVF, particularly in resource-limited settings.