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Klippel-Trenaunay syndrome

Please note the information provided is intended to support patients and is not a substitute for medical advice and treatment. We strongly recommend consultation with your doctor or health care professional, before using any information obtained from articles of this website.

Klippel-Trenaunay syndrome is a complex of congenital abnormalities affecting one or more limbs, and is usually characterized by the triad of (a) capillary malformations, (b) venous malformations and (c) bony and soft tissue hypertrophy of a leg. In some cases (20%) the abnormalities affect both legs or the pelvis and chest, while other abnormalities (e.g. of the fingers) may be present. There is no arteriovenous shunting.

The capillary malformations are of the port-wine stain type. Their depth is variable and may be limited to the skin or extend deeper to subcutaneous tissue, muscle and bone. Skin features are the first sign of the syndrome.

The venous malformations usually affect only superficial veins and present as varicose veins typically with a large, lateral, superficial, valveless vein which begins in the foot or the lower leg and travels proximally until it enters the thigh or the gluteal area. Sometimes, there are abnormalities of the deep veins, including aplasia, hypoplasia and ostruction.

Apart from the obvious disfigurement, there may be symptoms related to the varicose veins or to complications, such as bleeding, eczema (dermatitis) secondary to venous stasis, leg ulcer, superficial thrombophlebitis, deep venous thrombosis and pulmonary thromboembolism.

In uncomplicated cases without significant symptoms, treatment is conservative with graduated compression stockings. If surgical treatment is indicated, the patency of deep veins must have been confirmed first with venography (conventional or 3D-MRI).

Last modified 22/02/2014