 Texas Heart Institute educational programs on innovative technologies and techniques. The purpose of this presentation is to inform and educate the general public as well as the physicians and allied medical personnel on the latest advances in cardiovascular medicine. I'm your host. My name is Juan Mircresa. I'm an international cardiologist at Texas Heart Institute and Baylor College of Medicine. The topic today is incidents, causes, and treatment of pelvic congestion syndrome. Our special guest today is Dr. Breanna Costello, our international fellow at Texas Heart Institute and Baylor St. Luke's Medical Center. Thank you for having me, Dr. Crazier. It's a pleasure to be here and to talk about pelvic congestion syndromes. So as we start off, can you just maybe describe, give us a brief description of what pelvic congestion syndrome is and maybe how patients with this present? Very well. Pelvic congestion syndrome is commonly recognized as a persistence of chronic pelvic pain for longer than six months of duration in the absence of a known pelvic pathology. Now we all know that the venous system functions in the way that the blood is carried to the heart. So it means from the legs, from the abdomen, the blood should flow in the venous system towards the heart. What happens with pelvic congestion syndrome, this blood flow is reversed in the venous system. And as a matter of fact, the blood flows downward towards pelvis. This is very commonly seen whenever this particular syndrome exists in ovarian vein, this function where ovarian vein, typically left-sided ovarian vein, dilates or develops a valvular incompetence and then you get a varices in the abdomen and particularly in the pelvis that causes congestion and discomfort in the pelvic region. This condition is not very rare. As a matter of fact, up to 15% of women between the ages of 20 and 50 years of age will have varicose veins in the pelvis and up to 60% of those will experience some symptoms. Now it's interesting that over 10% of gynecological referrals are due to pelvic congestion syndrome and over 30% of pelvic pain is caused by pelvic congestion syndrome. So this entity is very common. Unfortunately it is not frequently diagnosed in early stages of this particular condition. So you said pelvic symptoms or pelvic pain. What are some other symptoms of pelvic congestion syndrome or what should we look out for our patients who have this type of you know issue? Well typically it's a chronic pelvic pain. Very frequently it's misdiagnosed as endometriosis and it can be accentuated before men's and also the patients experience dysmenorrhea. Frequently they also have this perunia, typically postcoital aching and discomfort or pain. Not infrequently they experience dysuria and worsening of stress incontinence in certain scenarios when the patients have well advanced pelvic congestion syndrome. This comfort also occurs with prolonged sitting or standing which is typically in a lot of professions like medical professions, surgeries or nurses and many other professions. Pain is also very commonly experienced in lower abdomen but also there is discomfort in the back in the large number of patients and hips as well. There are frequently encountered varicose veins in the vulva and vagina and also left lateral vaginal tendon is due to varicositis in that region and this is obviously related to malfunction and the valvular insufficiency of the left ovarian vein. Not infrequently also the patients will have hemorrhoids and symptoms related to hemorrhoids and also irritable bowel syndrome can be accentuated or caused by pelvic congestion syndrome. And then finally the patients will experience varicose veins and chronic venous insufficiency in the lower extremities, obviously more commonly in the left lower extremity because of the left sided predominance of this particular condition. Now what is not really well known, this particular entity or pelvic congestion syndrome also occurs in men and this is probably in most of the instances misdiagnosed or underdiagnosed but men will also experience chronic pelvic pain, pain in the lower abdomen or back or hips. They will also develop left sided varicose, frequently it is called the bag of worms and there will be scrotal varices, hemorrhoids are not uncommon, irritable bowel syndrome as well as dysuria and worsening of stress incontinence and in certain scenarios even erectile dysfunction and impotence in general but also other symptoms such as leg varicose veins and chronic venous insufficiency again more frequently present in the left lower extremity. You know it's interesting I wonder this is a touchy subject probably for both men and women so I wonder if that number you know the prevalence or the incidence is maybe even higher but a lot of patients are you know a little skittish about going to their doctor to address it because it's uncomfortable to talk about so it's very interesting. So why we talked a little bit about having pelvic congestion in men and women but why more in women than men, why you know is there an anatomic reason or what is what's the reason for that? Well this is not clearly understood but there are certainly some factors that are clearly explained it is not infrequently genetically transmitted type of entity or condition it is probably related more to pregnancy and issues related to pregnancy such as compression of the pelvic veins during pregnancy. There is also in women as you know we did present and discuss this in the previous presentation. May turn a syndrome or iliac vein compression syndrome is more commonly seen in women than men so this is another potential reasons. So as far as the causes of pelvic congestion syndrome is concerned we have two prominent factors that are playing a role. One is as we mentioned may turn a syndrome that's compressing either the left common iliac vein or iliac vein on the left or right side at any location and then another one is so called nutcracker syndrome which is a compression of the left renal vein by superior mesenteric artery and actually the left renal vein is being compressed between the superior mesenteric artery as we can see here and the abdominal aorta. So what is typically seen is with invasive or noninvasive evaluation enlarged left ovarian vein due to that compression and valvular incompetence of the left ovarian vein and then large varices as we can see here in the pelvis particularly around the uterus and urinary bladder. On the other hand may turn a syndrome is only related to compression of the iliac veins as we can see here on the bottom right image with the CT there is a severe compression of the left common iliac vein by the right common iliac artery that's crossing from the left side to the right side. Now as far as certain facts related to the pelvic congestion syndrome and the magnitude of this problem in my opinion this condition is the most under diagnosed and the most ignored and the most misdiagnosed and the most under treated medical condition to the best of my knowledge. And you touched on a few of these reasons but what do you think the biggest reason is for that? Well there are several factors that are playing a significant role but the most important one is delayed diagnosis obviously by patients and physicians lack of knowledge poor understanding of pelvic veins by clinicians. The average number of diagnostic tests prior to proper diagnosis is at least four and it takes on an average four years to establish the diagnosis that information is available in the literature. And several patients wait for over twenty years with symptoms to develop proper diagnosis and treatment of this condition. And you can imagine like you said a lot of these women have been pregnant or have gone through pregnancy so a lot of their symptoms could be just pushed aside as postpartum changes and uterus changes after delivering a baby but in fact maybe they could have this as the real etiology of their pain or discomfort so it's good to keep in mind. So what are the best tests in your opinion or in general to diagnose or work up this type of pelvic pain and diagnose the issue? You know it's sad to say and it's so disappointing that this condition is so poorly understood and misdiagnosed because we have simple tests that can clearly establish the diagnosis. Just a simple abdominal and pelvic duplex or ultrasound scan can reliably establish the diagnosis. Transvaginal duplex is also very useful to look at the varicose veins related to the post-pelic congestion syndrome and this can be done on outpatient basis in physician's office. It's painless, inexpensive and very reliable. But once we establish the diagnosis with ultrasound or duplex scan, additional tests might be of benefit. Unfortunately they're not frequently ordered and interpreted properly but for instance CT of the abdomen and pelvis and magnetic resonance particularly MRV of the venous system is very useful and clearly diagnostic of this condition and it can help us in differentiating the nutcracker syndrome or compression of the left iliac vein versus may turn a syndrome that also is commonly seen and a lot of patients actually will have both conditions at the same time. Can imagine the symptoms that you can have if you have both that would be pretty you know uncomfortable. Right. Alright so what is in your differential when you see a patient in the clinic with pelvic pain and you're maybe wondering if it could be pelvic congestion syndrome. One of the most common conditions is problems with some kind of arthritis or inflammatory process like lumbosacral spine degenerative arthritis, low back pain sciatica again due to this compression. Sacral iliac joint inflammatory arthritis and there are several conditions like that and several other ones that are maybe not clearly understood but inflammatory process of any kind including gastrointestinal type of chronic inflammatory disease would be very commonly be included in the differential diagnosis. Very good so when you've identified your patients of having pelvic condition syndrome what's the treatment options what do you talk with them about as possibilities to help with their symptoms. I usually use an algorithm in explaining to my patients and also to referring physicians how to evaluate and how to treat this condition and I certainly hope that this approach will help in establishing a diagnosis in great majority of patients without significant delay. So in my consideration any patient with chronic pelvic pain that cannot be explained with any other condition should be evaluated with abdominal and pelvic duplex scan and in women with transvaginal duplex scan and if there is evidence of compression either due to May Turner or the nutcracker and presence of varicositis then I would suggest to order either MRV or CTA. I prefer MRV because there is no radiation and it's as sensitive and diagnostic as CTA which obviously includes radiation. Especially in this young female population avoiding radiation would be ideal. So then once we establish the diagnosis that there is pelvic and left a renal vein abnormality from the MRV or CTA or noninvasive elevation then we have to establish the diagnosis is it related to May Turner or is it related to nutcracker or is it both conditions that are causing the problems. If we have a reflux typically like in a varian vein left a varian vein the treatment is simple and straightforward it's embolization of that insufficient and competent enlarged vein that has a lot of varicositis. So all of those conditions whether it's May Turner or nutcracker can be treated via endovascular interventions it is extremely rare that you need to resort to surgical treatment of this condition. That's great. So if there is compression obviously stenting is the treatment of choice. For May Turner. For May Turner obviously that's a must because you have to overcome that compression from the artery that has systemic pressure and venous pressure is very low. And also in certain scenarios when we have a nutcracker syndrome you have to stand the left renal vein and the indication for that would be only when a patient has symptoms related to that compression and the compression is putting a patient at risk of developing left renal vein thrombosis. So typically a patient will have dysuria they will have hematuria and left flank pain that will be persistent and in that kind of a situation stenting would be indicated. In your opinion what percentage of patients with pelvic congestion syndrome get stented in the renal vein is it a large proportion a large percentage or is it the you know the minority of patients that you actually have to stent the vein. Obviously this depends on the patient and patient symptoms and if there is any evidence of progression of disease and progression of symptoms but I would say that the great majority of patients will require treatment because this condition is progressive it gets worse over a period of time and in a lot of instances can be debilitating. So here we have actually the images of MRV and the treatment for the nutcracker syndrome in particular and what we can see here on the right hand side this patient already had coils in enlarged incompetent left ovarian vein and that usually should give alleviation of symptoms and improvement in a very short period of time in great majority of patients. All right so can you share with us maybe an experience or two with your treatment of pelvic congestion syndrome. Well as you know you are involved in several cases as a matter of fact one just yesterday so it's not uncommon to see this type of patients. I would like to share with the participants of this presentation one of the patients that we treated relatively recently and this patient was a 33 year old female with the history of two previous pregnancies complaining of abdominal lower back and left leg pain also she experienced dysparunia and had vaginal and vulvar varices which have been bothering her for a very long period of time since actually her teenage years. So she was quite incapacitated with her symptoms and was referred to us for further evaluation without proper diagnosis being established. She saw numerous practitioners including an orthopedic surgeon for evaluation of her lumbosacral spine several gynecologists and they did not establish proper diagnosis. So what we did is we evaluated her at our office obtained abdominal pelvic and vaginal ultrasound and then proceeded with MRV and we established a diagnosis that she had pelvic congestion syndrome. Now what was very interesting that she had a more advanced and more complex pelvic congestion syndrome because she had incompetent enlarged left ovarian vein with huge varices and also she had a late turner syndrome. Wow it's no wonder her symptoms were present for so long 20 years. So here we have the images of her CT angiogram of the abdomen and pelvis and we can see that her left ovarian vein was close to 11 millimeters in diameter which is probably three times larger than normal and also on the right hand side in the section right at the superior mesenteric and left renal vein we can see that the left renal vein is compressed to close to 90 percent with enlargement of the left renal vein post compression which had significant dilatation and also looking at her pelvic images we can see on this CT that she had large varicositis around her uterus and also urinally bladder not only on the left side but also on the right side and obviously the reason that the right side was enlarged as well with varicositis because the venous blood had to drain through the right side because there was severe compression of the left renal vein by the superior mesenteric artery. So here we can see the treatment. The treatment is pretty straightforward it's done on outpatient basis under local anesthesia. Typically we use five French catheters here we use the renal double curve diagnostic catheter we entered into the left renal vein and obtained an angiogram and we can see in the middle panel dilated the left ovarian vein that's also incompetent as we can see also on the right hand side. On the left hand side we can see a diagrammatic representation of what needs to be done basically placing coils and occasionally using sclerotherapy to thrombose that disease the incompetent ovarian vein. Now here we can see that the catheter is advanced distally to the distal part of the left ovarian vein and it's a showed with a red arrow and we can clearly see now on the middle panel that we have a huge varices in the pelvis around the uterus going from the left to the right and then we can see in the still image on the right hand side several actually varicositis that need to be addressed at the same time. So we proceeded with coil embolization of the left ovarian vein as we can see typically we start very distally to address all the varicositis that are clearly seen and gradually build up this endovascular coil occlusion all the way to a few centimeters from the origin of the left ovarian vein and then we can see on the completion angiogram that there is no longer flow in the left ovarian vein so that indicates a successful treatment of this particular condition in this particular patient. On the left hand side we can actually see on the still image evidence of compression of the left renal vein which we didn't stent at that time because the patient did not have any urinary symptoms such as left flank pain or hematuria and we typically like to reserve stenting only for patients that have obvious signs that the left renal vein is in jeopardy and at risk of thrombosis. Very good so for this patient did she improve or her symptoms better after this? Well she improved to a certain degree she had less back pain she had less ovarian left sided pelvic pain but she still had some symptoms and so she felt like she needed to have some other procedure done to address and correct this problem. Now what we decided to do is to wait for a few months because this condition requires a little bit longer treatment and follow up until you see significant improvements because there are numerous varicositis and they have to shrink over a period of time and typically the symptoms do not disappear within a few days or a month. So after two months she still had some symptoms that were obviously related to pelvic congestion syndrome and also iliac vein compression as you know as I mentioned previously she has or had a May Turner syndrome. So we proceeded with venogram and intra vascular ultrasound of the left iliac vein and on the left hand side we can see a still image where we can at least have a suspicion that there is compression of the left common iliac vein because of certain haziness or what we call pancaking of that vein that is being compressed by the right common iliac artery. We can see also endovascular coils in the left ovarian vein and we don't see any flow in it which is a good sign that we have at least achieved that successful treatment for the pelvic congestion syndrome. Now we also interrogated the left renal vein and we performed IVAS and we saw that there was no severe obstruction of the left renal vein. The flow was adequate but left ovarian vein was occluded as we can see it does not fill through that flow. We performed an intra vascular ultrasound of the left common iliac vein and we can see on the left hand panel there is severe compression of that vein shown by the arrow and where the catheter is. Yeah that's highly compressed wow. Right and then we measured it and actually the opening was somewhere in the range of two to three millimeters. Wow so what did you do next? Well obviously we had to address and correct this problem balloon angioplasty does not work for this condition. And that has to do with the systemic pressure of the artery. So we placed 18 millimeters in diameter stent as you can see here. There are several of them now available on the market that are dedicated for this particular application. We can see on the right hand side the completion angiogram showing excellent flow and on repeat IVAS there was no evidence of compression. And on the left hand side we can see balloon angioplasty being performed after the stent was deployed and using the intra vascular ultrasound now we can see that the left common iliac vein is fully open. There is no evidence of compression. On the right hand side we can see the glimpses of the stent in there and no evidence of compression. So this certainly had to help her so did you feel better after this? She felt better. Good. I was also encouraged. You felt better too. And I saw her about two months after the treatment and she said to me, which was relatively recently that she's totally symptom free. That's wonderful. Totally symptom free. Not only related to her back pain or pelvic pain or any pelvic discomfort but also total disappearance or left leg discomfort as well. That's great. That's a great end to that story. Well, thank you very much. This was great to be here with you today and learn about something that most of us don't think about on a daily basis. Well, thank you very much for being with me discussing this and I appreciate all of your questions. Great. Look forward to the next one.