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Treatment of Rare Vascular Conditions (Dr. Clair 10/15/12)

Monday, October 15, 2012 - 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's syndrome, Klippel Trenaunay Syndrome, Fibromuscular Disease, Hypercoagulable syndromes, and many others. Dr. Clair, Department Chairman of Vascular Surgery answers your questions about the treatment of rare vascular conditions.

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Fibromuscular Dysplasia (FMD)

SarahK: What is the Cleveland Clinic's FMD team doing to develop and publish a standard of care for patients with fibromuscular dysplasia so that doctors outside of the Cleveland Clinic can better treat their patients? Where does Cleveland Clinic stand on federal regulations regarding clinical trials within the rare disease community, orphan drugs and off-label uses?

Dr__Clair: Our FMD team, headed by Dr. Heather Gornik, is collaborating in writing a guidelines document for treating patients with FMD. As for clinical trials for rare diseases and orphan drugs, I cannot comment on a Cleveland Clinic position, but I can say that we participate extensively in clinical trials for both common and rare diseases to try and advance care for patients and understanding of disease.

SherryG: This question is for Dr. Daniel Claire concerning his chat on October 15, 2012, Treatment of Rare Vascular Conditions. My husband, age 62, was recently diagnosed with Fibromuscular Disease and also had endovascular surgery to repair an aneurysm in his right iliac artery. His father died of an abdominal aneurysm when he was 54 and our daughter, age 37, had a stroke due to narrowness in her carotid artery at the age of 5. Her daughter was also born with only one kidney. Could you discuss the hereditary implications of this disease and if it could be related to all of our family's medical problems? Thank you so much! -Sherry

Dr__Clair: As of right now we don't know of a hereditary cause or link for FMD. But, our participation in the FMD registry is designed to help us sort this out. Clearly from your own family history there must be some families who are at risk for genetic transmission of this process or something like it.

danb63: I have FMD and suffering from major headaches and neck pain I have a dissected carotid artery, I have read that BOTOX injections are used for Migraines. What is your thought?

Dr__Clair: If your headaches are migraines and not related to the dissection, perhaps Botox would be helpful. You should be evaluated in a headache center to see what the underlying cause of your headaches is.

AfternoonNapper: Regarding physical activity after dissection - many FMD patients have been told to discontinue activities such as yoga or high-impact activities or those that require a prolonged period of holding the neck at sharp angles. Do you agree with this?

Dr__Clair: It is easy to modify yoga positions to not have sharp head angles. High impact activities such as kick boxing or neck manipulation by chiropractors should be avoided with this diagnosis.

Zimbabe: I live in Portland OR, have FMD in Renals, Carotids, Iliac and vertebrals. So far I have been seeing a very good Nephrologist for the blood pressure issues, and a Neurologist for the rest but not with any great satisfaction. Would you suggest a different specialist? by chance any recommendations?

Dr__Clair: There are specialists who treat FMD - I would suggest you go to and speak with the team there. They can provide you with suggested specialists.

cdrgarey: My husband was recently diagnosed with FMD. Our daughter, age 37, had a stroke when she was 5 because of narrowness in her carotid artery. Could you recommend any tests that she should have for possible FMD?

Dr__Clair: Ultrasound of carotid and renal arteries is a good easy screening test.

AfternoonNapper: I would like to note that there are organizations other than FMDSA that are focused on FMD. Please also visit

Dissected Carotid Artery

ChuckNYM: Hi - I am a 60 yo male with a dissected carotid artery. I have run marathons - very very active. I am on Coumadin. I would like to get back to training for a marathon but have stopped this right now. My doctor said carotid stent is high risk and I don't really have the indication for it as a dissection. Not really sure what to do as my life as it was, is at a standstill. Suggestions?

Dr__Clair: Coumadin is the correct therapy for you initially and normally it is continued for only a year with transition to antiplatelet agents such as aspirin and plavix. It would be important to know what the cause of the carotid artery dissection was - ie. traumatic or due to FMD.

In most instances I don't believe that patients need to alter their activities including exercise as long as they are adequately treated. I would agree with not placing a stent at this point unless there was some indication such as neurological symptoms related to the dissection.

May-Thurner Syndrome

PhyllisH: Are you seeing an increase in patients with May-Thurner Syndrome, also known as Iliac Vein Compression Syndrome, and have the majority of these patients presented with DVT?

Dr__Clair: I would not say we have seen an increase in May-Thurner Syndrome because we do already see a large number of patients with this. We have seen an increase in identification of it I believe both inside and outside of our institution. Unfortunately most patients do come in with DVT but a significant number do present with unilateral leg swelling and pain on the left side.

SarahV: How important is it to take blood thinners after stent placement for May Thurner syndrome? I have developed an allergic reaction to plavix after taking it for 3 weeks after stent placement and now only taking adult strength aspirin.

Dr__Clair: It is not clear what the requirements are for anticoagulation after venous stent placement. I normally keep patients on coumadin for 6 months but some have reported using just aspirin as the single strategy. I would be sure to let the physician know who placed the stent that you are having trouble maintaining the medical regimen they have prescribed.

SarahV: Hello, I am from the May-Thurner Google Group. There are several members at our forum, me included, who suffer from both May-Thurner and Nutcracker syndrome and subsequent pelvic congestion. According to Monedero et al from Madrid, 10% of the May-Thurner patients also have Nutcracker compression. All of us are slim with no belly fat. Have you had patients who display both of these syndromes and if so, what is your experience of the renal vein stenting? Sofia

Dr__Clair: I have seen also an increase incidence of Nutcracker Syndrome or left renal vein compression in patients with May Thurner. Not all patients are symptomatic from this renal vein compression. We have stented patients for nutcracker syndrome with May Thurner and without May Thurner and the results to date appear to be very good but we do not have long term data regarding this. Since stenting does not take away the surgical option for treating this - I have suggested this as a first line of therapy for patients I have treated with nutcracker syndrome.

Vein Stent

mariannejohanna: I have an occluded iliac vein stent. The Mayo Clinic could not open it with a guide wire. Can you open these stents with spectranetics?

Dr__Clair:  In the last year alone, I have treated 6 patients with occluded venous stents and have been able to get them open. In most instances this does not require a laser and can provide long term patency as long as the stented segment is successfully able to be dilated. There is one patient in the last year that I have been unable to re-establish venous flow around an occluded stent. I am not sure the laser has any benefit.

mariannejohanna: Can collateral veins provide enough flow for the rest of my life after my iliac stent occluded completely. I do have pelvic discomfort, but I can life with that.

Dr__Clair: Collateral veins can provide adequate drainage from the leg but you will likely always have increased swelling and pressure in that leg compared to the other. Occluded iliac veins even after failed stents can still be re-opened. I would suggest it is worth an attempt at a center that has experience with complex vascular problems.

cdrgarey: During my husbands endovascular surgery to place a stent in his right iliac artery, they also had to block off his right interior iliac artery. He is now experiencing new pain in both his hips. Could this be related to his surgery?

Dr__Clair: This could be related to the procedure without question. In some instances the internal iliac artery can be reopened to re-establish normal pelvic flow.

willsingh: Hi Dr. Clair my cousin has a stenosis of his right and left jugular vein as seen on MRI(not just ultrasound) of the Veins can you or another vascular surgeon at Cleveland Clinic open them up with a catheter a place a stent? thank you -Will

Dr__Clair: If the veins are just narrowed some your cousin may not need therapy. If they are narrowed to the point they are causing symptoms such as superior vena cava syndrome they can be simply dilated with angioplasty. Stents in these vessels do not tend to have the same kind of long term results we see in stents in other places.

Multiple Sclerosis (MS) and Vein Stent

willsingh: I already submitted this question: my brother has MS and has stenosis of his right and left jugular veins as seen on MRI of the veins (not just ultrasound) would you or another vascular surgeon at your clinic be willing to open them up with a catheter and place a stent?

Dr__Clair: The concept of CCSVI as a cause of MS has been suggested but never proven. A number of physicians have made millions treating patients with angioplasty and stent without clearly proven benefit in MS patients. If your brother has this, he should be enrolled in a randomized trial to assess whether this treatment actually has any benefit to MS patients. I would check for more information.

willsingh: My cousin and us appreciate your reply but with all due respect, you talked about making millions, the only ones making millions are the drug companies from treating this as an immunolical problem (that no one has proven) and not a vascular problem which has been proven on MRI. Thank you

Dr__Clair: It would be great if one of the drug or device companies would sponsor a randomized trial and perhaps your family member would benefit from this knowledge.

The exact cause of MS clearly has not been proven and the association with central venous hypertension has been proposed based upon some anatomic modeling that was noted in the 1980s. The Italian physician who reported this association reported a registry of patients treated for MS with venous stents. His wife was one of the patients treated. In his results never revealed any functional improvement in the patients treated. There was subjective symptomatic improvement noted by the patients. Even he, as the initial physician reporting these results, has recommended further study through a randomized trial. I think we would all hope that we could either prove or refute the benefits of this approach. A scientific exploration through a randomized control trial would be needed to accomplish this.

Klippel-Trenaunay-Weber (also known as K-T Syndrome)

charleskt: Hi. My dad was diagnosed with Klippel-Trenaunay-Weber. What is the prognosis for him? Will he be able to walk without pain?

Dr__Clair: KT is an unusual venous malformation syndrome that affects one lower extremity and is associated with a port wine stain of the trunk. Previous thinking was that these patients could not be treated for fear of bleeding or damage to an absent deep vein system.

Our own experience shows that most of these patients have a deep vein system that is intact and thus with care treatment of this supervenous malformation can be undertaken safely. This can improve walking, pain and functional capacity.

It is unusual to be diagnosed with this late in life.

Blood Clots

David78: I had a large blood clot to my right leg after an accident. I am on coumadin and wear compression socks. It helps but as soon as I take the socks off, my foot and leg swells up until I put the socks back on. The swelling is uncomfortable but I don't like to wear the socks at night. Any more permanent fixes?

Dr__Clair: Depending upon where the venous obstruction that you still have there are potential options for treatment especially if this involves the vessels in your pelvis. This can be well treated with venous stents in many situations.

Arterial Venous Malformation (AVM)

STS2554: Hi. My sister has been having migraines and she was found to have an arterial venous malformation. Can you tell me more about this diagnosis? Is it treated by a vascular surgeon? She is currently seeing a migraine specialist and we are figuring out next steps.

Dr__Clair: An arterial venous malformation or AVM is an abnormal growth of blood vessels directly connecting arteries and veins. If your sister's problems are due to an AVM in the brain these are usually treated by a neuro-interventionalist.

If the AVM is not in the CNS (central nervous system) it is most often addressed by a vascular surgeon.

Ehlers Danlos

CharlotteAK: My grandson who is 23 years old is undergoing tests for Ehlers Danlos syndrome. His kidney function has decreased due to dissected renal arteries. He needs to speak to a surgeon with experience in this area. What do they do for dissected arteries in someone with this condition? I am worried.

Dr__Clair: The testing for Ehlers Danlos is clearly what should be done for your grandson but dissected renal arteries can be seen with a number of other conditions such as FMD. He should be seen in a large center that deals with a lot of genetic abnormalities affecting the venous and arterial system. We see a lot of patients with that condition at Cleveland Clinic.

Nutcracker Syndrome

Earlene: My 27 year old daughter has been complaining of mid abdominal pain, nausea vomiting, and blood in her urine and left side pains. Her physician order a CT scan (CT Abdomen W/+ w/o contrast). The results from the scan notes” The abdominal aorta is of normal caliber. The celiac axis, SMA and IMA appear normally opacified. There is abrupt change in caliber of the left renal vein as it passes the aortmesenteric angle.”IMPRESION:THERE IS APPEARANCE OF MILD DILATATION OF THE LEFT RENAL VEIN PROXIMAL TO THE AORTOMESENTERIC ANGLE. CORRELATION FOR NUTCRACKER SYNDROME IS SUGGESTED.Are there other test that needs to be gone to confirm nutcracker?

Dr__Clair: Your daughter's symptoms sound potentially suggestive of nutcracker syndrome. The most common cause of this problem is renal stones. If this has been ruled out, then an ultrasound to look at the renal vein and its flow pattern would be helpful. Often review of this and a CT scan (which she had) can be indicative of this diagnosis. I would reiterate that renal stones be ruled out before being treated for nutcracker syndrome which is a less common diagnosis.

Earlene: Suppose my daughter does have Nutcracker Syndrome what are her treatment options? And with Nutcracker being so rare could this be treated at Cleveland? FYI Reading the post from Sarah V…My daughter is 5’6” and weighs 102…

Dr__Clair: The initial thing here is to confirm the diagnosis of nutcracker syndrome and if your daughter is symptomatic from it she can be treated with stenting to resolve the renal vein compression. This has been very effective in our hands.

Fistula post Cardiac Catheterization

clara: I have been told a fistula where a catheterization has been done, and I need surgery. I have had 8 stents since 2009 - the 8th one 2 weeks ago. They want to try and wait due to the Plavix and new stent. What symptoms would one have with this condition, and why is the surgeon concerned? Is this unusual to happen. During the cath the doctor hit and artery or vein ( I am not explaining it correct) and had to stop and do it the next day in the other leg. I don't know if this was the cause or not. Thank you.

Dr__Clair: First, if you have had 8 stents over 2 years - you should seek a second opinion to make sure you are optimizing your alternative therapies to minimize progression of atherosclerosis. This would include lifestyle modification, blood pressure control and cholesterol management.

Second, regarding the fistula, it is something that is not infrequent with catheterization procedures. They rarely require any therapy and can often be simply followed. If the leg is tremendously swollen, or there is evidence of markedly increased flow through the fistula, then you may need something done. But - again - this is unusual.


jillrn: Dr. Clair, thank you for being here today! Can you please tell me about treatment for lymphedema of the face s/p neck surgery for cancer and subsequent radiation. How do I get started on treatment, appointments, etc?

Cleveland_Clinic_Host: Here is an online resource that might be helpful to you.

Reviewed: 10/12

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