Klippel-Trenaunay-Weber Syndrome

Klippel-Trenaunay-Weber Syndrome

Parkes Weber syndrome, PWS, Klippel-Trenaunay syndrome, KTS, Kasabach-Merritt syndrome, angioosteohypertrophy syndrome, cutaneous capillary malformation, congential vascular nevus, capillary hemangioma, port-wine stain, lymphedema

Synonyms

KTW syndrome, Klippel-Trenaunay syndrome, angio-osteohypertrophy syndrome, naevus vasculosus osteohypertrophicus; Parkes-Weber syndrome is a similar entity involving the presence of an arteriovenous malformation with high-flow vessel malformations and characteristic skin capillary changes, with associated skeletal or soft tissue hypertrophy (also known as haemangiectatic hypertrophy).

See also: Sturge-Weber syndrome

Clinical Features

First described by French physicians Klippel and Trenaunay in 1900, this syndrome is characterized by port-wine stain (capillary hemangioma), varicose veins and bony and soft tissue hypertrophy involving an extremity.

Other features involve lymphedema or lymphatic obstruction, cellulitis, chronic venous insufficiency, stasis dermatitis, poor wound healing, ulceration, thrombosis, and emboli.

Complications

Capillary hemangiomas (port-wine stain), stasis dermatitis, thrombophlebitis, cellulitis, limb disparity, and more serious sequelae such as thrombosis, coagulopathy, bleeding, pulmonary embolism, and congestive heart failure. (1), lymphedema is also a consistent complication.

Other indications may include asymmetric face, facial haemangiomas, advanced tooth eruption, macrocephaly, heterotopia, glaucoma, cutaneous pustules/ulcers

Etiology

Klippel Trenaunay appears to be a non-hereditary syndrome although there is still an ongoing discussion involving this.

Diagnosis

Simple diagnosis can be made by observation and examination. Venous and lymphatic complications are diagnosed using radiological tests such as lymphoscintigraphy, ultra-sounds, and MRI. Venograms, and arteriograms may also be used.

Treatment

Treatment is varied for the different symptoms present. Compression garments are used to control the lymphedema, prophylactic antibiotics are of course, used to treat the cellulitis and lymphangitis spells. Orthopedic procedures are available for the limb hypertrophy and wound treatment is sometimes necessary for ulcerations.

Other treatments for the symptoms or complications include lasers for the hemangiomas, and surgical intervention for the vascular anomalies may be called for.

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Klippel-Trenaunay-Weber Syndrome