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Extradural tumor causing spinal cord compression in Klippel-Trenaunay-Weber syndrome
D A Carter1, K Kim, R A Brinker
1Department of Neurological Surgery, Medical College of Ohio, Toledo 43699, USA.
Surgical Neurology
|March 1, 1995
Summary
Myelopathy is rare in Klippel-Trenaunay-Weber syndrome, but this case shows spinal cord compression from an angiomyolipoma. This highlights a new association with segmental vascular abnormalities in Klippel-Trenaunay-Weber syndrome.
Area of Science:
- Neurology
- Vascular Malformations
- Genetics
Background:
- Klippel-Trenaunay-Weber syndrome (KTWS) is a rare congenital disorder characterized by port-wine stains, venous and lymphatic malformations, and limb hypertrophy.
- Myelopathy, or spinal cord dysfunction, is an uncommon complication of KTWS, typically linked to spinal vascular malformations.
Observation:
- A patient diagnosed with Klippel-Trenaunay-Weber syndrome presented with symptoms of spinal cord compression.
- Diagnostic imaging revealed a spinal extradural mass lesion identified as an angiomyolipoma causing the compression.
Findings:
- This case describes a previously unreported association between Klippel-Trenaunay-Weber syndrome and spinal extradural angiomyolipoma.
- The presence of an angiomyolipoma causing spinal cord compression is consistent with the known segmental vascular abnormalities in KTWS.
Implications:
- This finding expands the spectrum of neurological complications associated with Klippel-Trenaunay-Weber syndrome.
- It underscores the importance of considering and investigating spinal pathologies, including vascular tumors like angiomyolipomas, in KTWS patients presenting with neurological deficits.