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May Thurner Syndrome and Other Rare Vascular Conditions (Dr. Clair 03/03/14)

Monday, March 3, 2014 - Noon


The Department of Vascular Surgery at Cleveland Clinic has earned an international reputation for excellence in patient care, surgical results, and clinical research. Our surgeons specialize in the diagnosis and management of vascular disease, including rare disorders such as May-Thurner syndrome (MTS), Klippel-Trenaunay (K-T) syndrome, median arcuate ligament syndrome, vascular issues affecting athletes such as cyclist induced iliac endofibrosis, congenital vascular defects, and many others. Dr. Clair, Chairman of Vascular Surgery answers your questions.

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Mesenteric Ischemia

Jdog_holland: I've been recently diagnosed with Mesenteric ischemia. Is this surgery so specialized that I should seek out a major vascular center such as yours? I live close to Philadelphia and within relatively easy reach of New York city and Baltimore. What would you recommend?

Daniel_Clair,_MD: Mesenteric ischemia is a lack of adequate blood flow reaching the intestine. It is not so much a diagnosis, as it is a symptom. That is, it is not a cause, it is a result of a vascular problem. The symptom of mesenteric ischemia is the end result of something leading to a diminished amount of blood flow reaching your intestines. There are a number of different diagnoses which can lead to this problem, but by far, the most common cause is atherosclerosis, or cholesterol plaque building up in your arteries and leading to narrowing of the arteries. It is important to understand what the cause of your mesenteric ischemia is first before informing you as to where would be best to go, however, no matter what the cause, I would recommend you seek treatment at a vascular center where they have a significant experience in treating patients with this problem. This would normally be a place with a large vascular surgical experience, such as a place like here at Cleveland Clinic or if you live closer, then perhaps the University of Pennsylvania, New York Hospital, or Columbia Presbyterian Hospital.

Aberrant Subclavian Artery

justmeAmy: Hello. I am a fit 38 year old woman, mom of 2 young kids and I previously worked as a PA. I was diagnosed ten years ago, incidentally, with a right sided aorta with a left aberrant subclavian artery originating from a Kommerell Diverticulum, with impingement on my trachea. No one suggested a vascular eval and I was lead to believe it was nothing to be concerned about. I now have a new primary care doc who felt it necessary to reinvestigate this anomaly as my shortness of breath has been increasing. I have done some research and am terrified by the statistics of 18-50% of KDs resulting in dissection or rupture and death. Does this mean I will likely be facing corrective surgery in the near future? How do I know when the risk of surgery is less than the risk of rupture? I also deal with an undiagnosed slowly progressive polyneuropathy of both legs, unresponsive to IVIG and with hyperactive patellar reflexes. Could this be related in any way to a vascular condition? Thank you!

Daniel_Clair,_MD: I think your new Primary Care physician is correct. The risk associated with your vascular anomalies and the symptoms of shortness of breath, mandate a further investigation, and frankly, you will likely be under the care of a vascular specialist for some time. This is not because you cannot get better, but because it is important to make sure you are followed carefully. Nearly all patients with symptoms related to the vascular anomalies you have will require corrective surgery, but in someone like yourself, who is young and otherwise fit, these risks are low and recovery times are relatively fast. Risks related to the problem are assessed normally with CT scans to assess the arch vessels and their diameters, and surgery is usually recommended a bit earlier than perhaps with some other defects, because the risks are relatively low. In many instances, there may need to be a series of procedures, which are utilized to minimize the risk and impact of a single large surgery. This is an abnormality with which you certainly will want to be cared for at a very experienced facility.

As for the potential relationship to the polyneuropathy you have to the vascular anomaly, my initial answer is probably not, but an evaluation at an experienced center should include a neurologic assessment with the anomaly you have in mind.

TimV: I am looking for treatment options for an aberrant right subclavian artery. I have dysphagia and dyspnea. Surgery? I am exploring my options and where to go for this treatment.

Daniel_Clair,_MD: Aberrant right subclavian artery is an unusual diagnosis that is normally treated at specialized centers, such as we have here. It can be associated and often is associated with other vascular anomalies. In most settings, if the problem is only an aberrant right subclavian artery, surgery can be performed through an incision on the neck to resolve the symptoms of compression of your airway and esophagus (swallowing tube).

Atherosclerosis or Blockage

5jake925: What are the indications of vascular problems, arterial blockages, in cases where there are no symptoms. What testing is advised and how often after the age of 75?

Daniel_Clair,_MD: Vascular blockage from cholesterol or atherosclerosis is not uncommon after the age of 75. In our society, that is probably present in about 15% of the population. In most instances, if the blockage does not cause symptoms, then the treatment is really aimed at reducing the risk of heart disease and the presence of the atherosclerosis. This is done by a combination of lifestyle changes (smoking cessation, exercise, and dietary changes) along with medical therapy to manage blood pressure and cholesterol. The combination of these efforts can significantly reduce your risk in both the short and long term.

Femoral Artery Disease

JimS: I have 100% blockage of my femoral arteries. My Dr. wants to do by-pass surgery, but, being diabetic I worry about complications. Are there other less invasive laser type treatments?

Daniel_Clair,_MD: Blockage of a femoral artery can be effectively treated with bypass but there certainly are less invasive methods of therapy. The choice between surgery and minimally invasive therapy depends upon the extent of the blockage, the location of the blockage, and the projected outcomes from the two forms of therapy. If there are no symptoms related to the blockage, it is not necessary to have something done. If, however, you have a wound of the limb that is not healing because of poor blood flow, then some form of revascularization should be done. If you are anxious about the bypass, my own thought would be to get a second opinion. It would be helpful if this opinion could be offered by someone who can offer both minimally invasive treatments and bypass surgery.

Arterial Malformation

CynthiaK: I have multiple arterial malformations in my leg arteries that are causing considerable pain in the top of my legs, groin area. I would like to come for an evaluation but wonder what that would entail and if you could treat at the same appointment?

Daniel_Clair,_MD: Arterial malformations are abnormal communications between the arteries and veins that lead to dilation of vessels and abnormally increased blood flow through the area of the abnormality. In most instances, these malformations can be treated with minimally invasive therapy, but it is important that the individual treating these has a broad experience in order to assure you will achieve relief. It is important to understand as well that cure from arterial venous malformation is not the goal - this can rarely be achieved. The real goal here is to resolve symptoms and restore normal function and this can often be achieved. The evaluation would consist of ultrasound and possible CT angiography to assess the extent of the abnormality. If you are coming from a distance, we can often arrange for treatment to be performed within a day or two of the evaluation to limit your travel. It is not unusual to need more than one treatment in order to successfully resolve the issues.

Carotid Artery Disease

YvonneG: How dangerous is removing plaque from a carotid artery if there is already a diagnosis of severe coronary artery disease and the patient has had two CABG done, the last one in 2012?

Daniel_Clair,_MD: The procedure to remove plaque from a carotid artery is called carotid endarterectomy. The risk of stroke from this procedure varies depending upon whether the patient is symptomatic from this narrowing or not. In asymptomatic individuals, the risk of stroke should be about a half of a percent and more important than this situation, the risk of heart attack can be minimized by correct pre-operative preparation and perioperative management. It is also important to recognize that in many instances, patients who have been told they need this operation actually simply need good medical management. If you have been informed that you need this procedure, it is not unreasonable to obtain a second opinion.

Chronic Venous Ulcer

Melody G: My dad has a chronic venous ulcer of the leg treated with a skin graft? The ulcer is about 75% healed but has been treating it since last Fall. Is that normal?

Daniel_Clair,_MD: It is unusual to have a wound last this long. It would be important in this situation to assure that there is normal flow in the arteries and veins supplying this leg. In addition, if this is a chronic venous ulcer, any therapy should be combined with compression therapy and elevation in order to resolve the pressure on the wound. If there is infection or inadequate blood flow to the leg, healing will not progress. It is therefore imperative that this wound be assessed for these two particular issues at this point.

Vein Disease

Jim R: I have been diagnosed with vascular disease in my legs. I was told that the valves in my veins in my legs do not operate as they should. They want to laser the veins and also strip some. My question after reading your website, is it possible to repair the valves? This option was not mentioned by my doctor. I have type 2 diabetes. I went to the doctor when one of the veins burst and bled in one of my legs after getting out of the shower.

Daniel_Clair,_MD: It sounds like you have superficial valvular dysfunction which is ordinarily NOT treated by valve repair, but in the manner that has been offered to you. It is difficult to know whether that reflux is responsible for the episode that you had but if your vascular specialist is convinced that these are related, then the treatment offered seems reasonable.

donnaedge: I had a venogram in February of this year. Later vein studies were done at the same facility. Ultrasounds and venogram results contradict each other. Is this common and what should I believe?

Daniel_Clair,_MD: If the physician who ordered these tests has not discussed this discrepancy with you and clarified what he or she thinks is going on, I would recommend a second opinion or re-evaluation. We would be happy to offer that to you here.

Deep Vein Thrombosis (DVT)

DonnaEdge: My doctor does not feel I am a candidate for stenting surgery due to a left leg DVT and PE's in 2201. I have been on Warfarin since 2001. After a recent venogram, I found i have a completely occluded clot in my groin, upper thigh and behind my left knee. I have considerable pain. Why didn't the Warfarin do its job? Thank you.

Daniel_Clair,_MD: Warfarin, or Coumadin is a blood thinner that keeps an individual from getting clot, but it does not have any effect on clots already in place. In most instances, it will not dissolve the clot in your veins. With this said, in many individuals, the body’s own clot busting proteins may resolve some of the clots in veins in your legs, but it sounds like this has not happened for you. I wish we could explain why some times these clots resolve and in some circumstances, like yours they don’t, but we cannot be sure why these things happen as they do. In most instances, even with the clot you have in place from some time ago, we can resolve these issues with stents. I do think this would be helpful for your leg. I would be more than happy to review studies or to see you and discuss options available to you.

Compartment Syndrome

ChristinaO: I have had compartmental release on my legs both sides two times and the swelling is recurring. My doctors are concerned and not sure what to do, I think they want to refer me to another center. How is this normally treated when it comes back?

Daniel_Clair,_MD: Compartment syndrome without a known cause is unusual to say the least. Recurrent compartment syndrome is even more unusual. In a situation like this, there should be clear confirmation that pressures within the compartment are elevated and are associated with the symptoms that you are having. In some situations, the solution for compartment syndrome alone can simply be modifications to your ambulation style, your shoes, or physical inserts which can help. In your particular situation, I would recommend a second opinion at an experienced center to confirm this diagnosis.

Median Arcuate Ligament Syndrome (MALS)

CathyS: Does any of your Vascular Surgeons have experience with Celiac Artery Decompression when the Ligament is compressing the artery? If so do you use the laparoscopic approach or full surgery? My daughter has been diagnosed with this and we are at our wits end in trying to find the best place that is fairly close to Louisville, KY to have this done. We see that Cleveland Clinic is rated #2 in the country for Gastro. Since this is a Gastro/Vascular issue, hoping you can help.

Daniel_Clair,_MD: We actually do have a vascular surgeon, Dr. W. Michael Park. He has a particular interest in this disorder and sees a number of patients annually for this problem. He works in conjunction with our minimally invasive general surgeons to perform the surgery, if needed, through a laparoscopic or minimally invasive approach.

Dolphin13: Regarding median arcuate ligament syndrome - a Doppler ordered by a Gastroenterologist at the Mayo Clinic showed a PSV of 140 cm s/inspiration and a PSV of 274/expiration, near occlusion of the vessel with end expiration. Testing was done to determine origin of extreme abdominal pain/cause of gastroparesis. However, the Vascular Surgeon there is skeptical of MAL syndrome causing Gastro issues and stated it is a major open surgery. All other conditions have been ruled out e.g. neurological autoimmune, pelvic muscle dysfunction. Additionally, I've received a trigger point steroid injection into the abdomen with no resolution to pain.

  1. Does Cleveland Clinic perform Celiac Artery Decompression?
  2. If so, are you using the Laparoscopic approach or full blow open surgery?
  3. Either way, which Vascular Surgeon (if not yourself) should I contact there for a second opinion. Being from northern Kentucky you are much closer that the Mayo Clinic in Minnesota.

Daniel_Clair,_MD: Please see above about Dr. Park and his practice involving patients with MALS.

May Thurner Syndrome (MTS)

PhillipL: I have May-thurner and a blockage in my iliac vein that has been there for over five years. The Dr. tried doing catheter directed therapy and it was unsuccessful. What is another treatment to open the blockage? I am on blood thinners.

Daniel_Clair,_MD: Open surgery can be done in this situation if needed, essentially doing a venous bypass. But in this situation, I would recommend another attempt at interventional therapy to solve this problem. I personally see a large number of patients who have had attempts in the past that have failed and have been able to treat these individuals with minimally invasive methods to restore normal flow in the venous system.

CharlaD: I have been diagnosed with May Thurners. I am on blood thinners for DVT in leg and lungs. They did a venous Doppler and said I need a stent and have May Thurners. Is that the treatment you would suggest?

Daniel_Clair,_MD: If you are completely asymptomatic from the compression of your iliac vein, then you do not really need to have treatment. If, however, you have significant symptoms of the left leg from venous compression or obstruction, then re-establishing normal venous flow through stenting would be the initial treatment I would recommend. Once again, as with other less common conditions, I would make sure the person treating you has a fairly broad experience in treating patients with venous occlusive disease. It's perfectly fine to ask your physician about his or her experience in placing stents and patient outcomes.

MarianneL: Hello doctor. My daughter had a stent put in her iliac vein for May Thurner and had relief for some time, and now it is back. Is that common? Do they restent or is surgery the next step?

Daniel_Clair,_MD: It is not common for the symptoms to return after placement of the stent to resolve May-Thurner Syndrome. I have seen this in patients who have either an abnormal clotting system or something externally beside the artery compressing the venous stent. Here, I think it is mandatory that your daughter be evaluated at a facility that has extensive experience with this problem, like we do here at Cleveland Clinic.

mariannejohanna: Hi, I had an iliac stent placed in December 2010. It has been completely occluded for two years now. The Mayo Clinic tried to open it a year ago and could not. Are there any new procedures to open older occluded stents? Marianne in Cape Coral.

Daniel_Clair,_MD: We have treated a number of patients who have failed stents and have been able to re-open them. In many instances, patients have had one or more attempts that have failed. We have a variety of techniques that can enhance our outcomes and have trained many surgeons including those at the Mayo Clinic on these techniques.

donnaedge: I have recently been diagnosed with May-Thurner's syndrome and have a few questions. What are my lifelong consequences of this disease? What is the general treatment choices for May_Thurners Syndrome? If my left iliac vein is occluded and my doctor could not stent it, what are my other choices for treatment? Is my life expectancy shortened by my recent diagnosis of May Thurners syndrome? I am wondering if there is any treatment for pain in the leg as result of May-Thurners syndrome?

Daniel_Clair,_MD: May-Thurner Syndrome can be treated with both minimally invasive (stent therapy) or open bypass surgery, but in nearly all instances, we have been able to achieve success with stenting. Our long-term success is excellent with patency of over 80% beyond five years and most patients do not need any further treatment after this. The diagnosis when adequately treated has no proven impact on life expectancy or function. Resolution of the venous compression is usually the best treatment for the pain but compression therapy of the effected limb adds additional benefit, and in some instances, may be all that an individual needs.

Janellemc: I am a 43 year old female. About a year ago, I had a blood clot on my left groin area. My vascular surgeon's hypothesis was, that a clot formed behind my cellulitis infection in my right calf and shot up to my groin area, oral bc was also a contributing factor. When he went in to place filter and break up clot he diagnosed me with May Thurner syndrome, was going to place stents when he went back in to break up clot. At the time I was in no condition to question this. I had previously had no symptoms of May Thurner syndrome. will the stents be ok for the rest of my life? Do they need to be checked on a regular basis? My surgeon does not communicate this information with me. Since the procedure which was last April, I have been in pain of various degrees and locations. Mostly hip area pain, comes and goes on the right and left side. Also the pain occurs in my calves. Are you familiar with pains after stent placed? i am puzzled by the pains, i never had previous to the procedure.

Daniel_Clair,_MD: When stents are placed for May Thurner and the blockage is resolved, symptoms are usually minimal. Some of what you re describing, that is, bilateral groin and calf pain do not seem typical of May-Thurner Syndrome and make me concerned that other may be some other etiology for your pain. Your stents, in particular, if they are patent, should be checked at least every one to two years and in most instances, you can expect excellent long term patency and normal function.

leesahbee: As a person with MTS, I've found that I'm unable to get a sense of how well -- quality of life wise -- I can expect to do with venous stents. Do they experience wear and tear and will "break down" in their performance over time? I have been stented six years now and am not anticoagulated and do very well. But, what should I expect 20 years from now? Thanks.

Daniel_Clair,_MD: This is a great question! We have patients who have had stents in over 12 years without problems and with long-term success who are managed without anticoagulants as you are. It appears at least preliminarily, that you can expect a normal long life with long term - potentially 20 years or longer - success with these stents.

Santenkraam: Hello, I'm a Dutch woman, 39 years old. I wonder, after stenting, what can I expect? I had a deep-vein thrombosis in my left leg (from under my knee up to my bellybutton) five years ago and known, for three months, that I suffer from MTS. In a couple of months I will receive surgery at the university hospital in Maastricht, the Netherlands. The blood vessel that suffered from thrombosis will be cleaned, the vessel that is obstructed by MTS will be stented. It's hard for me to stand upright and to sit. I also have less energy than I used to have, because of the pain I guess, but maybe also because the vessel is obstructed. Do you think after surgery these problems will be solved? Or is MTS something that will be part of my life forever?

Daniel_Clair,_MD: In most instances, when stenting successfully resolves the blockage, patients can and should expect resolution of their pain, improved physical function, and long term success of the stents.

Janellemc: I have been diagnosed with May Thurner syndrome, i question if I also have Nutcracker syndrome. Can you explain the symptoms of this disorder. Treatment?

Daniel_Clair,_MD: Nutcracker Syndrome is an unusual condition where the mesenteric artery (the artery that supplies blood to your intestines) compresses the vein draining the left kidney. In every situation where a patient has Nutcracker Syndrome, there should be blood (this may be only microscopic evidence of blood) in the urine. In the absence of the finding of blood in the urine, the diagnosis of Nutcracker Syndrome is unlikely. The most common symptoms of Nutcracker Syndrome are hematuria or blood in the urine and left flank pain from increase venous pressure on the left kidney. The treatment may be venous stenting of the left renal vein, but it is important that the diagnosis be fully confirmed before proceeding with this. Again, it is always best to be evaluated and treated in an experienced center.

Klippel-Trenaunay syndrome (K-T)

tnjeff: I am a 34 year old male that has K-T syndrome that moderately affects the entire right side of my body, i.e., overgrowth of right arm and leg. I cannot seem to find anyone in the Nashville area that is well versed in the condition as you seem to be at CC. Is there anyone you can recommend?

Daniel_Clair,_MD: I don't know of anyone in Nashville who deals with this problem regularly, but I do think it is important that you receive treatment and evaluation from someone who is well versed in dealing with patients with this problem. We'd be happy to offer you an evaluation/second opinion for this problem.

Cynthia1974: Hello, my son (2-1/4 yrs.) has K-T lower left limb. Surgeons are proposing a debulking in his foot. The extra bulk is lymphatic. He has had five incidents of cellulitis, so I am worried about infections from surgeries. What are the risks I should discuss with the surgeons. I am not convinced on intervention at this stage as he is running, jumping, climbing with no pain.

Daniel_Clair,_MD: Especially in this situation with K-T in a child, it is very important that you assure the individuals treating your son have a broad experience with this problem. I share your concern regarding the potential for infection with surgical debulking but peri-operative infection risk can be minimized with careful technique.

Undiagnosed Symptoms

DeannaR: I have been sick for about six years with symptoms from pain all over my body, serious stomach issues, weight loss, headaches, nausea...etc. My doctor did a cat scan and told me that my mesenteric artery is narrowing and that I need to see a specialist. I have been having horrible chest pain, my breasts have swollen a size larger than they were and are in extreme pain, my back legs and arms hurt terribly. My neck feels very tight and hurts when I swallow. I am seeing the specialist on Tuesday, March 4, but I am wondering if this is getting to the point where I should be admitted for surgery. How much longer should I lie around in all this pain before it becomes dangerous. I have been sick for six years with this problem and it has gotten even worse this past month. Please let me know what I should do. Thank you.

Daniel_Clair,_MD: This is a complicated problem and from your description of your issues, I really cannot be certain of what the underlying diagnosis is. It sounds as if you need to see an internist and perhaps a rheumatologist but I would not recommend surgery as an initial step here as I am not sure what we would operate on. If the mesenteric vascular disease progresses, this can be addressed in several ways, but it is imperative that we understand the disease leading to these difficulties before we simply try to treat this narrowing in the mesenteric vascular system.

Cardiology Question

5jake925: I have been diagnosed with a left branch block, trifascicular block. I have no symptoms. Is there a concern? What is the appropriate follow up.

Daniel_Clair,_MD: Your issues seem mostly related to your heart's electrical system, not the vascular system. I would recommend an opinion from a cardiologist with specific reference to an electrophysiologist or EP cardiologist.

Moderator: I'm sorry to say that our time is now over. Thank you again Dr. Clair for taking the time to answer our questions. If you have additional questions, please go to to chat online with a heart and vascular nurse. Get the latest news and views from the specialists at the Miller Family Heart & Vascular Institute at Cleveland Clinic on our regularly updated blog! We explore current topics related to research, Cleveland Clinic physician perspectives on breaking news stories and offer an outlet for Cleveland Clinic heart and vascular patients to share their story with readers.

Reviewed: 03/14

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