ABSTRACT
Peri-medullary arteriovenous fistula (PMAVF) is a rare spinal vascular malformation that manifests as progressive neurologic deficits or hemorrhage in the spinal canal. We report a case of high-flow PMAVF in a child, with a single feeder artery and a large venous pouch, which was successfully treated with transarterial endovascular intervention. A 2-year-old boy was referred with a progressive 2-year history of myelopathy. The MRI revealed a large venous pouch at the midthoracic spinal cord with segmental surrounding edema. A spinal angiogram confirmed high-flow PMAVF with a single feeder artery from the anterior radiculomedullary artery, draining into the peri-medullary vein. The patient underwent transarterial embolization at the distal feeder artery, resulting in gradual motor strength improvement. PMAVF is classified as type IV spinal vascular malformation, usually presenting as a large, high-flow fistula with multiple feeders, although there was only one in this case. PMAVFs are intradural and may cause severe neurologic deficits due to mass effect, venous congestion, or hemorrhage, hence requiring prompt treatment. Treatment options for PMAVF include microsurgery, endovascular intervention, or a combination of the 2. Endovascular intervention with coil or liquid embolic material is considered first-line treatment for IVc PMAVF, and effective in type IVb with good clinical outcome. PMAVF is a rare spinal vascular malformation commonly manifesting as severe neurologic deficits but has the potential of favorable outcomes with endovascular therapy. This case demonstrates a unique angioarchitecture of high-flow PMAVF with a single feeder artery and large venous pouch, treated successfully with endovascular therapy.
