Unilateral contrast-enhancing pontomedullary lesion due to an intracranial dural arteriovenous fistula with perimedullary spinal venous drainage: the exception that proves the rule.

A large spectrum of possible diagnoses must be taken into consideration when a contrast-enhancing lesion of the pontomedullary region is found on MRI. Among these diagnoses are neoplastic, inflammatory, and infectious, as well as vascular pathologies. The authors report a rare case of an intracranial dural arteriovenous fistula (DAVF) with perimedullary spinal venous drainage (Cognard Type V) that initially presented as a unilateral contrast-enhancing pontomedullary lesion mimicking a brainstem neoplasm in a 76-year-old man. Following occlusion of the DAVF by transarterial embolization that resulted in clinical and radiological improvement, the fistula recurred 10 months later and was finally cured by a combined endovascularand surgical approach that resulted in complete occlusion. Clinical symptoms and MRI findings gradually improved following this treatment. A literature review on the MRI findings of Cognard Type V DAVF was performed. Centrally located medullary or pontomedullary edema represents the typical imaging finding, while unilateral edema as seen in the authors' patient is exceptionally rare. The hallmark imaging finding suggestive of DAVF consisting of perimedullary engorged vessels may not always be present or may only be very subtly visible. Therefore, the authors suggest performing contrast-enhanced MR angiography or even digital subtraction angiography in the presence of an unclear edematous brainstem lesion before scheduling stereotactic biopsy.