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Klippel Trenaunay Weber syndrome

Case contributed by Nolan Walker
Diagnosis certain

Presentation

Known Klippel-Trénaunay-Weber syndrome. Increasing leg swelling. ? DVT. ? May-Thurner

Patient Data

Age: 60 years
Gender: Male

There are extensive varices throughout the right leg and thigh.  The deep and superficial venous systems of the right leg are significantly enlarged, however contrast is seen throughout both the deep and superficial systems with no filling defect demonstrated.  In particular the femoral vein,  profunda vein and popliteal vein are all patent. 
 
There is no evidence of May-Thurner syndrome in the abdomen.

Right limb hypertrophy noted, consistent with the known diagnosis of Klippel-Trenaunay-Weber Syndrome.

Case Discussion

The patient has a known diagnosis of Klippel-Trénaunay-Weber syndrome. Exhibiting the triad of:

  • port wine nevi.
  • bony or soft tissue hypertrophy of an extremity (localized gigantism).
  • varicose veins or venous malformations of unusual distribution.

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