Intracranial dural arteriovenous fistula associated with progressive cervical myelopathy and normal venous drainage of the thoracolumbar cord: case report and review of the literature

Abstract

Background Intracranial dural arteriovenous malformations draining into the perimedullary venous system are rare lesions. In these cases, the selective spinal catheterization of all vessels with potential of causing that malformation was negative, and additional cerebral angiography usually reveals the fistula. Because of venous congestion of the cord caused by the DAVF, a delayed drainage or stagnation of contrast material in the artery of Adamkiewicz was considered as a compelling angiographic disorder so far. Case Description We report about a 58-year-old patient with a DAVF of the right posterior fossa draining into the cervical and upper thoracic plexus of medullary veins, followed by progressive cervical myelopathy and a normal venous drainage of the artery of Adamkiewicz. Because of the failing endovascular treatment option, the neurosurgical intervention was performed. The fistula was explored and clipped without any complications. Immediately after operation, the patient reported an improvement of his neurological deficits. Conclusion About 38 cases of intracranial DAVFs draining into the perimedullary venous system are reported, but to our knowledge, this is the second one with a normal drainage of the artery of Adamkiewicz. The pathophysiological mechanisms, diagnostic procedures, and treatment modalities are discussed.