Multiple Spinal Perimedullary Arteriovenous Fistulae Associated with the Parkes-Weber Syndrome

We present a rare case of multiple spinal perimedullary arteriovenous fistulae associated with the Parkes-Weber (PW) syndrome.

A 31-year-old male known to have the PW syndrome involving the left leg since birth, presented with a 7-month-history of progressive myelopathy of the lower extremities and dysfunction of the bladder and bowel. Myelography demonstrated dilated intradural vessels. Angiography demonstrated two distinct single hole perimedullary arteriovenous fistulae near the conus at two different metameres. They were supplied by the left posterior spinal artery.

The patient was treated by transarterial embolisation using polyvinyl alcohol particles, which resulted in venous side occlusion of the fistulae. After the treatment, the patient developed transient worsening of the spasticity of the lower extremities, and was treated by heparinization. After heparinization, the patient partially recovered from the pre-embolisation status of his myelopathy.

The follow-up angiogram one year after the embolisation demonstrated persistent obliteration of both fistulae. At long-term follow-up, the patient can ambulate without assistance and work as a farmer.