Síndrome de Klippel-Trenaunay-Parkes-Weber com angiomatose medular

We describe a 24-year-old woman who presented with twice previously unverified subarachnoid hemorrhages from the conus medullaris spinal arteriovenous malformation with Parkes-Weber-syndrome. Spinal MRI examination is considered to be necessary for the diagnosis of Klippel-Trenaunay-Weber syndrome. For diagnosis of the spinal cord arteriovenous malformation, it is indispensable to search carefully for the presence of accompanying lesions. Transarterial glue embolization is effective for the management of the spinal vascular lesion.

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

Niimi

Ito

Tone

et al. 1998

Interv Neuroradiol

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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.

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Síndrome de Klippel-Trenaunay-Parkes-Weber com angiomatose medular

Abstract: É relatado um caso de síndrome de Klippel-Trénaunay-Parkes-Weber associada a angiomatose medular. O autor salienta ter encontrado na literatura somente uma referência com tal associação.

Cited by 6 publications

References 3 publications

Klippel–Trenaunay Syndrome

Klippel–Trenaunay Syndrome

Conus Medullaris Spinal Arteriovenous Malformation in a Patient with Klippel-Trenaunay-Weber Syndrome

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

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