In my evolving understanding of leg and arm avms, I am learning that embolizations and sclerotherapy may do more harm than good if you have Klippel-Trenaunay or Parkes-Weber Syndrome. To complicate matters, doctors use different definitions of what constitutes KTS or PWS. Some doctors say if there is no portwine stain, it cannot be KTS or PWS -- not true. Other doctors say if there is no overgrowth of the limb (hypertrophy), it is not KTS or PWS -- this is a newer, narrower notion of these conditions which may exclude patients who are better served under the KTS/PWS umbrella.
Why does it matter what we call the condition? It matters because patients with KTS or PWS may have abnormalities in their circulatory system that can cause unacceptable side effects if these patients have sclerotherapy or embolizations. They made wind up with venous insufficiency and various circulation problems from treatment.
So, how do you know if you might have KTS or PWS? Look for:
Port-wine stain or "birthmark" on one side of the body -- usually but not always Venous malformations (KTS) or arteriovenous malformations (PWS) in the limb
Whole limb involvement
Vascular anomalies such as varicose veins
Lymphatic involvement
Soft tissue and bony hypertrophy (excessive growth of the soft tissue and/or bones), though there may be no hypertrophy, and there could be undergrowth
If you have avms or vms affecting a whole limb, please ask your doctor whether you may have KTS or PWS, and if the doctor says no, ask how the diagnosis was ruled out.
Thank you for sharing that information. My family has a strong history of severe varicose veins. So severe even the men have had to have surgery to treat them. I never heard there was even a remote connection with the types of vascular malformations we have (AVM survivors) and varicose veins. At age 77 my Mom ( who doesn't have an AVM but has had very bad varicose veins) was recently diagnosed with a fairly rare condition, Hemi-Facial Spasms which are believed to be caused by an enlarged vein near the brain stem putting pressure on facial nerves causing severe facial spasms. Maybe there is some kind of connection on some level.
Sirolimus is a drug therapy that some KTS patients are trying. Over the next few years, we should learn whether it can help stop the progression of KTS. Ask Dr. Mitchell about it.
I have been told my whole life that I have AVM, which effects my entire arm (spots in my elbow, wrist and various areas of my hand) - I have friends who have KTS and their extremity looks extremely different from mine. However, I do have port wine stains on my skin, bone overgrowth (I assume, my effected arm is longer than my other. Would this be worth questioning? I have been seeing the same doctors my whole life and I trust them entirely..Recently had sclerotherapy in May and still experience mobility restrictions in my fingers and extreme dry skin in the area they concentrated the surgery around.
hringioggrafir, KTS is so variable that one person's limb may look entirely different form another. Also, the appearance of the limb may change over time. I would suggest you join the Facebook group and take a look at some of the recent comments, and then post any questions you may have.
I recently came across these syndromes too and have been wondering if that may be something I have. I don’t have any port wine stains but my affected foot is significantly bigger than my AVM free foot. And from what I’ve read it’s some sort of genetic thing? My sister and my cousin have Reynaud’s and one of my aunts has some weird vascular tumor thing I keep forgetting to ask what it’s called but I’ve been wondering if there may be a connection. Some sort of genetic mutation or something…also I’ve had 5 embolizations (two recent ones) and they didn’t really tough my pain and I feel symptoms in my calf now
I agree with the information you provided, and I would like to add some comments. First, Klippel-Trenaunay syndrome [KTS] and Parkes Weber syndrome [PWS] are completely different conditions but are often confused. Both conditions involve the arms and legs, as you mentioned, and usually have a capillary malformation or port wine stain on the skin. However, KTS is a low-flow malformation including capillary, venous and lymphatic components, usually with overgrowth of the affected limb. Affected individuals often have abnormalities of the deep veins, including absence or under-development of the deep veins in association with varicose superficial veins. In such a situation, sclerotherapy or laser treatment of the superficial abnormal veins can worsen symptoms. KTS can cause severe lymphedema or even lymphatic cysts. Patients with KTS should be treated in a vascular anomalies center, not a standard vein center. PWS is a high flow vascular malformation affecting an arm or leg, usually with overgrowth and a port wine stain on the skin. In the most common form, the AVM consists of very extensive but tiny enlarged arteries and capillaries throughout the muscle and/or tissue under the skin. Because there may not be any direct arteriovenous connections, embolization or sclerotherapy can damage the tissues without leading to any improvement in symptoms. While this is the most common form of PWS, some patients with similar external appearance can have a more typical AVM, that can be treated with embolization. So again, it is best to see an expert for this type of condition.
The term "Klippel-Trenaunay Weber" [KTW] syndrome is incorrect, and is not used by vascular anomalies experts.
An easy way to tell the difference between KTS and PWS is to put your hand on the skin and feel the temperature. Patients with KTS usually have normal or slightly warm skin, while PWS causes the skin temperature to feel quite hot in the affected area.
Hello. I am so glad to have found this thread. I have a diagnosed AVM on my finger, extending down into my hand. I do have a port wine birthmark prominently on a foot. I have had one embolization surgery. No noticeable improvement. During a followup consultation, the surgeon refused to even estimate how many additional surgeries may be required, even saying he has patients who have had 30 - 40 surgeries. This is full on general w/ a catheter in my femoral artery, angiogram, and snaking the catheter all the way around to my finger. The idea and cost of the surgeries (with travel) is daunting, and it feels dangerous and intrusive to have that many surgeries. I have been having zero luck finding someone knowledgeable to even have a discussion with: what are my risks on no treatment? what are long term effects? Should amputation be considered? I only have occasional, throbbing pain and cramping associated with what I call “growth spurts.” There is bone deterioration. Can anyone recommend a starting point for me? I’m finding it near impossible to find the correct person to speak with. If I call the vascular people here, those doctors only work w/ brain AVMs. If I call a hand surgeon, they say they don’t treat AVMs. The specialist/surgeon in Denver was the only one I was able to engage with, but he is not very forthcoming with information and seems only interested in scheduling surgeries. Who can I call to have a conversation about options?
Good evening. I have a leg length discrepancy with my left leg 1cm longer than the right. I have always had one foot chubbier than the other and a year ago they found an AVM on the sole of my right foot. I don’t have any car on my body. Could it be one of these symptoms?
These are things you’ll need to discuss with your doctor. @Madere_dancermom is a member from a long time ago (and was one of our most active moderators) so unfortunately won’t answer these days.
It does seem relevant to discuss these conditions with your doctor.
