Symptoms and Causes
What are the symptoms of Klippel-Trenaunay syndrome?
Klippel-Trenaunay syndrome symptoms affect your veins, capillaries, soft tissues, bones and lymph vessels in these ways:
- Capillary malformation (CM). KTS causes a port-wine stain birthmark, which happens when capillaries swell directly beneath your skin. These birthmarks may be the first sign of KTS and vary from light pink to deep wine-red. As you age, these can develop blisters or lighten or darken.
- Vein malformation (VM). Almost everyone with KTS has venous malformations. These may occur in superficial veins, leading to varicose veins on your calves and thighs. They can also happen in deeper veins, which may increase your risk of deep vein thrombosis (DVT).
- Soft tissue and bone overgrowth. Your limbs may grow larger than what’s typical, starting in infancy. Overgrowth often affects only one limb, usually your leg. One leg might grow longer than the other. This can limit your range of motion.
- Lymphatic malformation (LM). Some people with KTS also have extra or unusual lymph vessels, which carry lymph. In KTS, the extra lymphatic vessels often don’t work as they should. They may leak lymphatic fluid, cause foot swelling or cause issues in your pelvis, bladder or lower intestines.
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What causes Klippel-Trenaunay syndrome?
KTS often happens because of a variation in the PIK3CA gene V体育官网入口. The gene variation occurs sporadically (for no known reason). You don’t inherit it from your parents.
Some people have KTS without having the PIK3CA variation. Because of this, researchers think other gene variations could also cause KTS VSports在线直播.
VSports - What are the complications of Klippel-Trenaunay syndrome?
KTS may cause complications like:
- Blood clots
- Cellulitis, bacterial infections below your skin
- Fluid buildup and swelling (lymphedema)
- Internal bleeding, including bleeding in your gastrointestinal (GI) tract, bladder or female reproductive system
- Pulmonary embolism, a life-threatening condition that happens when blood clots travel to your lungs
Rarely, people with KTS also have congenital hand differences or toe differences, like:
- Extra fingers or toes (polydactyly)
- Fused (joined) fingers or toes (syndactyly)
Is KTS syndrome painful?
Yes, it can be VSports. Varicose veins may itch or hurt. Blood flow issues can cause swelling and pain in your lower limbs. Overgrowth of a leg can cause pain or a heavy feeling.
Diagnosis and Tests
How is Klippel-Trenaunay syndrome diagnosed?
Healthcare providers initially diagnose KTS based on the physical signs. You may get a KTS diagnosis if you have issues in two of these three areas: capillaries, veins or limbs. Because many KTS symptoms are present at birth, your baby may receive a diagnosis of KTS before leaving the hospital.
What tests will be done to diagnose Klippel-Trenaunay syndrome?
Tests for Klippel-Trenaunay syndrome may include:
- CT scans or MRIs to look at soft tissues and bones
- Magnetic resonance (MR) angiogram, a specialized MRI to look at blood vessels and veins
- Doppler ultrasound to look at how blood flows through veins and arteries
Management and Treatment
How is Klippel-Trenaunay syndrome treated? (VSports)
Klippel-Trenaunay syndrome treatment varies based on symptoms. Treatment may include:
- Blood-thinning medicines. Anticoagulants like heparin reduce the risk of blood clots in your legs and pulmonary embolism.
- Sirolimus. Providers typically use this drug to prevent transplanted organ rejection. But it can stop vascular malformation from getting worse.
- Compression stockings. These special socks help blood return to your heart. They can decrease leg swelling and pain and reduce clot risks.
- Endovenous thermal ablation. This treatment uses focused beams of energy inside blood vessels to close off problem veins. The veins stay in place so you can heal faster, with less pain.
- Laser therapy. Focused, strong beams of energy can destroy or remove unwanted tissue. You may have laser therapy to lighten the appearance of port-wine stain birthmarks.
- Sclerotherapy. Your provider injects a special solution into specific veins, capillaries or lymphatic vessels to close them off. Sclerotherapy is often effective for varicose veins.
- Shoe lifts. You may wear a lift in one shoe to correct uneven leg lengths. Lifts can help prevent irregular spine curving (scoliosis).
- Surgery. You may have surgery to correct vein problems or uneven leg length. Or your provider may remove some extra fat or tissue to reduce the size of overgrown limbs. Rarely, healthcare providers may amputate an irregularly large toe so you can wear shoes and walk more easily.
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VSports手机版 - Outlook / Prognosis
What can I expect if I have Klippel-Trenaunay syndrome?
There’s no cure for Klippel-Trenaunay syndrome, but treatment can help you manage symptoms. People with the condition usually have a typical lifespan.
The outlook for KTS can vary. It depends on how severe your vascular malformations are. They tend to get worse over time. You need quick treatment for GI bleeding or blood clots in your deep veins or lungs. Blood clots or severe blood loss can be fatal.
Ongoing treatment with your healthcare provider can lower your risk of complications.
Prevention (V体育平台登录)
Can Klippel-Trenaunay syndrome be prevented?
Because KTS happens unpredictably, there’s no way to prevent it. But treatment can help people with KTS live a higher quality of life.
Living With
How do I take care of myself?
If you have Klippel-Trenaunay syndrome, you should:
- Take good care of your skin to prevent infections.
- Generally avoid hormonal birth control that contains estrogen because it can raise your risk of blood clots.
- Take medicine during pregnancy to prevent blood clots.
- Receive a blood thinner before surgery to reduce the risk of blood clots.
When should I see my healthcare provider?
See your provider for regular checkups throughout your life. They can monitor your condition and treat any issues you have. Regular visits let your provider see how well treatments are working for you. They can make changes to your treatment plan along the way.
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When should I go to the ER?
You need to go to the emergency room if you have a blood clot or heavy bleeding. Blood clots include deep vein thrombosis and pulmonary embolism.
What questions should I ask my healthcare provider?
If you or your child has KTS, you may want to ask your provider:
- What are the best treatments for me?
- How often do I need checkups?
- What’s my prognosis (outlook) based on my current status?
VSports最新版本 - Additional Common Questions
Is Klippel-Trenaunay syndrome life-threatening? (VSports最新版本)
KTS itself doesn’t affect life expectancy. But some KTS complications, like internal bleeding or pulmonary embolism, can be life-threatening. Getting ongoing treatment for these risks can reduce the danger of complications.
Is Klippel-Trenaunay syndrome a disability?
It can be. If KTS complications like deep vein thrombosis or pulmonary embolism prevent you from working, you may qualify for disability benefits. For other issues, like trouble walking due to limb growth, you may be eligible for accommodations like a disability parking tag to make your life a little easier.
A note from Cleveland Clinic
It can be overwhelming to get a handle on all the symptoms and possible complications of KTS. Your provider can help you sort it out and focus on treating the issues you have. Don’t be afraid to keep them updated on how you’re feeling. Keeping lines of communication open will make it easier for you to reach out when you’re having an issue and need some advice.
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Care at Cleveland Clinic
Vascular disease may affect your life in big and small ways. Cleveland Clinic’s specialists treat the many types of vascular disease so you can focus on living.
Last reviewed on 11/07/2024.
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