Intracranial dural arteriovenous fistula (DAVF) with perimedullary venous drainage may cause brainstem swelling and represent a diagnostic challenge. A 66-year-old man presented to the emergency room with recurrent vertigo, minimal truncal ataxia with a wide-based gait, and a slightly impaired tandem gait. Brain magnetic resonance imaging (MRI) revealed a hyperintense lesion in the left pontomedullary area on T2-weighted images (T2WIs) with partial gadolinium enhancement, but without increased signals on diffusion-weighted images. Abnormal serpentine flow void vessels surrounding the medulla and upper cervical spinal cord were initially overlooked but discovered later. An angiogram revealed DAVF with feeders from the right occipital artery and the meningeal branch of the right distal vertebral artery with drainage into the anterior medullary venous system and the perimedullary veins. The patient underwent a successful transarterial endovascular embolization and improved gradually. A brain MRI at 3-month follow-up revealed a residual hyperintense signal on the T2WIs in the left lower medulla. Six cases of patients exhibiting DAVF with isolated unilateral brainstem swelling from the literature were reviewed. Isolated unilateral brainstem swelling due to intracranial DAVF with perimedullary venous drainage is extremely rare and might mimic a tumor on MRI. Abnormal serpentine flow void vessels on the surface of the brainstem or spinal cord are crucial diagnostic clues.
Keywords: brainstem swelling; dural arteriovenous fistula; perimedullary venous drainage; venous congestion.