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Multiple spinal perimedullary arteriovenous fistulae associated with the parkes-weber syndrome. A case report
1Endovascular Surgery Center, Institute for Neurology and Neurosurgery, Beth Israel Medical Center North Division, Albert Einstein College of Medicine; New York, NY, USA.
Summary:
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.
Insights
This study details a rare case of spinal perimedullary arteriovenous fistulae in a patient with Parkes-Weber syndrome. Transarterial embolization successfully treated the condition, leading to improved mobility and function.
Area of Science:
- Neurology
- Vascular Surgery
- Radiology
Background:
- Parkes-Weber syndrome is a rare congenital disorder characterized by arteriovenous malformations.
- Spinal perimedullary arteriovenous fistulae are uncommon vascular lesions affecting the spinal cord.
Purpose of the Study:
- To report a rare case of multiple spinal perimedullary arteriovenous fistulae associated with Parkes-Weber syndrome.
- To describe the diagnostic and therapeutic approach for this complex condition.
Main Methods:
- Diagnostic imaging included myelography and spinal angiography.
- Treatment involved transarterial embolization using polyvinyl alcohol particles.
- Post-treatment management included heparinization for transient neurological worsening.
Main Results:
- Angiography confirmed two distinct perimedullary arteriovenous fistulae supplied by the left posterior spinal artery.
- Transarterial embolization achieved venous side occlusion of the fistulae.
- The patient experienced partial recovery from myelopathy after treatment and demonstrated persistent obliteration of fistulae at one-year follow-up.
Conclusions:
- Multiple spinal perimedullary arteriovenous fistulae can occur in the context of Parkes-Weber syndrome.
- Transarterial embolization is an effective treatment modality for spinal perimedullary arteriovenous fistulae.
- Successful treatment can lead to significant functional recovery and improved quality of life.
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