Klippel Trénaunay syndrome (KTS or KT), previously Klippel–Trénaunay–Weber syndrome and at times angioosteohypertrophy syndrome and hemangiectatic hypertrophy, is a infrequent congenital health condition where blood vessels and/or lymph vessels don’t form properly. The 3 chief attributes are nevus flammeus (port-wine blot), venous and lymphatic malformations, and soft-tissue hypertrophy of those affected limb. [two] it’s comparable to, though obviously different from, the less common Parkes-Weber syndrome.
The classical triad of Klippel-Trenaunay syndrome is composed of:
- vascular malformations of the capillary, venous and lymphatic vessels;
- varicosities of unusual distribution, particularly the lateral venous anomaly; and
- unilateral soft and skeletal tissue hypertrophy, usually the lower extremity.
Signs and symptoms of Klippel Trénaunay Syndrome
The birth defect is diagnosed by the presence of a combination of these symptoms (often on approximately ¼ of the body, though some cases may present more or less affected tissue):
- One or more distinctive port-wine stains with sharp borders
- Varicose veins
- Hypertrophy of bony and soft tissues, that may lead to local gigantism or shrinking, most typically in the lower body/legs.
- An improperly developed lymph system
Sometimes, port-wine stains (capillary port wine kind) could be absent. Such cases are extremely rare and might be categorized as “irregular Klippel–Trenaunay syndrome”.
The illness most frequently presents with a combination of both. People who have venous involvement experience improved complications and pain, like venous ulceration at the lower extremities.
People that have big AVMs are in danger of formation of blood clots at the vascular lesion, which might migrate into the lungs (pulmonary embolism). When there’s large-volume blood circulation throughout the lesion, “high-output heart failure” can grow as a result of inability of the heart to create adequate cardiac output.
Treatment of Klippel Trénaunay Syndrome
KTS is a complex syndrome, and no single treatment is applicable for everyone. Treatment is decided on a case-by-case basis with the individual’s doctors.
At present, many of the symptoms may be treated, but there is no cure for Klippel–Trenaunay syndrome.
