What is Klippel-Trénaunay-Weber (KTW) syndrome and how does it cause venous insufficiency?

Updated: Sep 25, 2020
  • Author: Robert Weiss, MD; Chief Editor: William D James, MD  more...
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A less common cause of venous insufficiency is Klippel-Trénaunay-Weber (KTW) syndrome, which involves port-wine stains, varicose veins, and bony or soft-tissue hypertrophy. Patients with pure Klippel-Trénaunay syndrome have only venous involvement, whereas those with the Parkes Weber variant also have arteriovenous malformations.

The capillary hemangiomas (port-wine stains) of KTW syndrome, like those of other forms of venous insufficiency, can lead to local skin breakdown and ulceration, bleeding, and secondary infection. This can occur in any organ system of the body.

The sciatic vein is a large superficial vessel that is present during fetal development but usually does not persist. In patients with KTW syndrome, this vein may be noticed at birth, or it may become apparent later in life. The vein extends along the posterolateral aspect of the leg from the foot to the gluteal region. When present, it is invariably a reflux pathway rather than a pathway for antegrade flow.

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