Just wondering about KT?
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What Is KTS?
Klippel - Trenaunay Syndrome is a congenital vascular disorder of unknown cause. Klippel-Trenaunay (KTS) is characterized by a triad of signs: Port Wine Stain (capillary malformations) covering one or more limbs, vascular anomalies, usually venous varicosities or malformation and hypertrophy (enlargement of the limb) or atrophy (withering or smaller limb). KT involves the lower limbs in about 90% of the patients. In rare instances, there is an absence of Port Wine Stain and not all three abnormalities need always be present for the syndrome to exist. Each case of KT is different, with patients having varying abnormalities and severity. Other associations with KT can include internal organ involvement, hematuria (blood in the urine) rectal bleeding and vaginal bleeding. Bleeding from an abnormal lesion on the affected limb is also common. Patients may have symptoms including anemia, coagulation problems (blood clots) and platelet trapping in the affected limb. (from the Sturge-Weber Foundation at http://www.sturge-weber.com)
Although Klippel-Trénaunay Syndrome is a rare congenital (present at birth) disorder, it is the most common condition involving combined vascular malformations. The syndrome is characterized by a localized or diffuse capillary malformation (portwine stain) that overlies a venous malformation and/or lymphatic malformation with associated soft tissue and bone hypertrophy (excessive growth). The portwine stain is typically substantial, varicose veins are often quite numerous, and bone and soft tissue hypertrophy is variable. The affected limb is either larger or smaller than the unaffected limb. Hypertrophy occurs most commonly in the lower limbs, but may affect the arms, the face, the head or internal organs. Additionally, a wide range of other skeletal and skin abnormalities sometimes coexists.
photo of child's leg affected by KTS
Bony enlargement is usually not present at birth, but may appear within the first few months or years of life and may become particularly problematic during puberty. The affected area grows longer and thicker due to increased blood supply. Sometime after puberty and before age 30, the portwine stain develops small vesicles (blood-filled bubble-like lesions) that can bleed spontaneously.
What symptoms are associated with Klippel-Trénaunay Syndrome?
Symptoms vary according to the severity of the dominant vascular component and its location. If lymphatic malformations are dominant, soft tissue swelling and enlargement will occur. If venous malformations are dominant, episodes of painful thrombosis (clotting) will occur. This group of patients often experiences muscle cramping or joint pain when walking. When the lower gastrointestinal tract (intestines) is involved, rectal bleeding often occurs. When there is bladder involvement, blood is often seen in the urine.
In one type of Klippel-Trénaunay Syndrome, which is known as the Parkes-Weber variant, patients have arteriovenous fistulae (multiple arteriovenous connections), which can result in heart failure if untreated.
How is Klippel-Trénaunay Syndrome diagnosed?
In many patients, a thorough medical history and physical examination are sufficient to make the diagnosis. However, a number of imaging studies are useful when there are complications. Evaluation of the deep venous system can be done by Doppler ultrasonography (type of ultrasonography in which blood vessels are seen) and magnetic resonance imaging (MRI) studies. MRI is also helpful in imaging the soft tissue hypertrophy. Angiography is especially helpful in the diagnosis of arteriovenous fistulae that are seen in the Parkes-Weber variant of Klippel-Trénaunay Syndrome.
Careful clinical and radiologic assessment of the affected limb should be done at regular intervals to assess limb length discrepancy and to formulate an approach for prevention and treatment of overgrowth. For lower-limb overgrowth beyond a 2-centimeter (bit less than 1 inch) differential, orthopaedic intervention may be necessary.
What are the possible complications of Klippel-Trénaunay Syndrome?
Complications of the capillary malformation include skin breakdown and ulceration, bleeding and secondary infection. If the lesion extends deeper in tissue, internal organs such as the pleura (sacs which envelop the lungs), spleen, liver, bladder and colon may also be affected. When this happens, internal bleeding can occur.
Varicosities may affect the superficial and deep venous systems. Pain and lymphedema (swelling of extremities due to stoppage of lymph flow caused by malformed lymphatic vessels) are common. Complications due to varicosities include paresthesias (abnormal skin sensations such as burning or tingling), skin ulcers, pulmonary emboli (blood clots in the lungs), inflammation and clots of blood vessels in the legs, and cellulitis (skin and soft tissue infection).
Hypertrophy of a limb can lead to vertebral scoliosis, gait abnormalities and compromise of function.
How is Klippel-Trénaunay Syndrome managed?
Management of Klippel-Trénaunay Syndrome is dependent upon individual symptoms. Although both nonoperative and surgical approaches are used, treatment is primarily nonoperative and supportive.
Supportive Care
*
Compression therapy. Compression garments are often advised for chronic venous insufficiency, lymphedema, recurrent cellulitis and recurrent bleeding from capillary or venous malformations of the extremity. They also protect the limb from trauma. Intermittent pneumatic compression pumps may also provide benefit.
* Pain medication, antibiotics, and limb elevation. These treatments are all used to manage cellulitis.
* Anticoagulant therapy (the use of substances that prevent blood clotting). This approach is indicated in cases of acute thrombosis (clotting) and is also used as a preventive measure prior to surgical procedures.
* Heel inserts. These are sometimes used to manage limb length discrepancies that are less than 1 inch. For greater discrepancies, orthopaedic surgery may be considered.
Surgical Interventions
Laser therapy. Using the flashlamp pulsed-dye laser is often quite effective in lightening the color of the portwine stain. Many treatments are typically required to achieve a desirable result. Laser treatment is also indicated when there is ulceration, since it tends to effect quicker healing.
Surgery. Depending on individual circumstances and anatomical involvement, a number of surgical options are occasionally advised. These include vein ligation, vein stripping, vein resection, and in rare cases, amputation. Vein ligation is a procedure that clamps off a section of veins. The clamp prevents blood flow through the damaged section of veins and promotes blood flow through veins that are not damaged. Vein stripping uses a metal wire to remove varicosities from within the damaged vein. Vein resection is a procedure that removes a section of veins from the body. Amputation is a procedure that removes a portion or all of a limb.
Other Treatment
Sclerotherapy. This treatment consists of the injection of a chemical into the vein causing inflammation. As the inner wall of the vein becomes inflamed, blood is not permitted to flow through it. The vein then collapses and forms scar tissue.
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