Pelvic Venous Congestion Syndrome: Finding Answers For Pelvic Pain
Pelvic venous congestion syndrome, or PVCS, although easy to describe can be challenging to diagnose. In fact, PVCS is often misdiagnosed or never diagnosed at all.
What is PVCS? Veins have one-way valves that help pump blood back toward the heart. If the valves are weak or damaged, blood can flow in the wrong direction and pool in the legs and feet, often causing them to swell. When this happens near the pelvis, it is called pelvic venous congestion syndrome. Simply put, varicose veins can also develop internally, in the pelvis, uterus and ovaries.
Diagnosing PVCS
Internal varicose veins can cause symptoms similar to those in the legs. Patients will often have visible varicose veins on their upper legs or labia, but not always. The main symptom is pelvic pain that lasts for six months or more.
Patients with PVCS report a prolonged deep and dull ache, often associated with movement, posture, and activities that increase abdominal pressure. Similar to varicose veins in the leg, the achiness that increases with prolonged standing can often be relieved by lying flat or elevating the legs.
Chronic pelvic pain (CPP) occurs below the belly button in the pelvis. The pain may be unilateral or bilateral – meaning on one side of the pelvis or both – and is often more pronounced on one side than the other. The pain is chronic and appears to have no obvious source. Symptoms may also include rectal discomfort or increased urinary frequency, bloating or gastrointestinal (GI) symptoms.
According to some studies, more than 26% of women suffer from CPP, but data about its prevalence is incomplete. When taking into account the number of factors that could also cause chronic pelvic pain, one can see how misdiagnosis by medical professionals might occur.
For example, 71 percent of cases of chronic pain in the pelvis may be associated with dyspareunia (pain during sexual intercourse), 66 percent of cases may be due to dysmenorrhea (pain associated with menstruation), and 65 percent of cases of chronic pelvis pain are diagnosed as postcoital ache (pain following sexual intercourse).
Conditions range from problems in the gastrointestinal tract to gynecologic diseases and urologic abnormalities. While some of these conditions are easily diagnosed, other causes of chronic pelvic pain are extremely difficult to recognize and have often been underdiagnosed or overlooked—even though studies show that PVCS occurs in up to 30 percent of patients who have chronic pelvic pain.
A heightened awareness
Dr. Joseph Gerding, a physician at Spectrum Medical Group in Maine, has Board certifications in both Interventional Radiology and Phlebology. According to Dr. Gerding, an evaluation by a gynecologist is a fundamental part of the patient's assessment in diagnosing chronic pelvic pain.
He believes, however, that input from other specialties – such as gastroenterology, urology, oncology, phlebology, and interventional radiology – may also be necessary because the reasons behind the pain are so many and varied. In addition, said Dr. Gerding, "a heightened awareness and clinical suspicion for the specific symptomatology of PVCS may bring about a more rapid progression toward treatment."
PVCS usually affects women who have previously been pregnant, because the ovarian and pelvic veins widened during pregnancy to accommodate the increased blood flow from the uterus. After the pregnancy, some of these veins remain enlarged and fail to return to their previous size, causing them to weaken and allow blood to pool or flow in the wrong direction.
Similar to varicose veins in the legs, venous congestion in the pelvis often first manifests during or after a pregnancy and worsens with subsequent pregnancies. Risk factors for PVCS may include a family history of the condition, hormonal influence, pelvic surgery, multiple pregnancies, a retroverted (tilted) uterus, and a history of varicose veins. African American women and women over 35 years of age have a lower risk of developing this condition.
How do you know?
Diagnosis begins with a physical examination. According to Dr. Gerding, in a patient with characteristic symptoms, the examination will typically indicate ovarian tenderness, cervical motion tenderness, and uterine tenderness with direct palpation (feeling with the hands). In one study, the combination of tenderness with a history of postcoital ache was 94 percent sensitive and 77 percent specific for discriminating PCVS from other pelvic conditions.
After an initial exam, a number of non- or minimally-invasive diagnostic tests can be performed to determine whether chronic pelvic pain is a result of pelvic varicose veins. These tests include pelvic ultrasound, pelvic venography, Computed Tomography (CT), and Magnetic Resonance Imaging (MRI).
Pelvic ultrasound: There are three diagnostic criteria for establishing the diagnosis of PVCS: (a) tortuous pelvic veins with diameters greater than 4 mm, (b) slow blood flow (about 3 cm/s), and (c) dilated veins in the outer one-third of the myometrium (arcuate veins) that communicate between bilateral pelvic varicose veins. Ultrasound (US) can also be used to exclude pelvic neoplasms (tumors) and uterine pathology as potential causes of pain.
Cross-sectional Imaging: Computed Axial Tomography (CAT), and Magnetic Resonance Imaging (MRI) are noninvasive methods that use special x-ray equipment to create detailed scans of areas inside the body. CAT and MRI identify pelvic venous changes better than US, and they may be indicated as part of the evaluation of pelvic pain to exclude or to further characterize pelvic pathology other than pelvic varices, or pelvic varicose veins.
Venography: Many investigators recommend selective ovarian and internal iliac venography in patients with symptoms of PVCS. The minimally invasive procedure involves inserting a very thin catheter into the femoral or jugular vein in order to inject a colored fluid into the ovarian veins. The venogram shows which way the blood is flowing, as well as how quickly.
Venography is more sensitive than US since it can be performed in the upright or semi-upright position with a tilting table. The Society for Vascular Surgery (SVS) and the American Venous Forum (AVF) clinical practice guidelines recommend retrograde ovarian and internal iliac venography as the test of choice for the diagnosis of pelvic venous disorders.
Laparoscopy: Characteristic pelvic venous changes can sometimes be visualized at laparoscopy, a surgical procedure in which a fiber-optic instrument is inserted through the abdominal wall to view the organs in the abdomen. However, laparoscopy is less sensitive than venography for the diagnosis of PCS. As with pelvic US, diagnostic laparoscopy is generally utilized to exclude other causes of chronic pelvic pain.
CAN MEN SUFFER FROM PVCS?
There is a male equivalent of pelvic venous congestion: varicocele (pronounced var-i-koh-seel). A varicocele is a dilation and reflux of the veins within the scrotum (the pouch of skin that holds the testicles), and it is similar to a varicose vein that might develop in the leg.
According to Dr. Joseph Gerding, although there are similarities to venous reflux in the sex organs of men and women, there are, of course, differences. When a woman has varicosities in the pelvis, they communicate with the internal iliac veins, which then communicate with veins down the legs. That means that when you see a woman with varicose veins in the legs, it is possible to trace the problem to veins in the pelvis.
In men, the testicular veins don't have that same kind of communication with the legs, because those veins are external to the body and thus more isolated from other veins. In other words, testicular varicose veins in men and varicose veins in their lower limbs are not often interrelated.
Varicocele occurs in 15-20% of males after puberty, but only 2-10% of men with a varicocele experience symptoms. When symptoms do appear, they may include testicular atrophy (testicles that decrease in size and remain small) or, more rarely, pain that increases with standing over a prolonged period of time but is relieved after lying down.
Varicoceles are also a common cause of low sperm production and decreased sperm quality, which can cause infertility, but not all varicoceles affect sperm production. Most varicoceles develop over time, sometimes enlarging and becoming more noticeable. Varicocele might cause one testicle to swell, almost always on the left side.
Treatment for varicoceles is similar to women with PVCS: surgical ligation or percutaneous (through the skin with a needle) venous embolization. Typically, a urologist or interventional radiologist can perform the ligation as treatment.
Fortunately, most varicoceles are easy to diagnose and many don't need treatment because they don't cause any symptoms. However, if someone is experiencing pain or swelling in the scrotum, he should contact a physician to rule out other conditions, some of which may require immediate attention.
Treatment for PVCS
There are a number of treatments for those diagnosed with pelvic venous congestion syndrome: medical, surgical, and minimally invasive. (In Maine, there aren't many medical professionals who regularly treat this disorder.)
One medical approach is the use of pharmacologic agents to suppress ovarian function, such as medroxyprogesterone or gonadotropin-releasing hormone. Though it may offer short-term pain relief, the long-term effectiveness of these drugs has not been proven. Additionally, women treated with medroxyprogesterone acetate reported rapid return of pain after cessation of treatment, which further suggests that hormonal therapy suppressed pain rather than cured it long-term. (The findings from relevant studies are limited, due to their small sizes and lack of a placebo groups.) The risks of medical therapy are low.
Surgical approaches include hysterectomy with unilateral or bilateral salpingo-oophorectomy (BSO) and ovarian vein ligation and excision with interruption of as many collateral veins as possible. Hysterectomy with BSO may be helpful for women with PVCS, but the data is inconsistent. In one study of women who underwent this procedure, 67 percent of patients reported no pain at one year, while a different study reported a non-significant trend toward improvement at one year. Hysterectomy with BSO is an option for women who have failed other treatment modalities, but women should be counseled that the surgery may not resolve their pain.
Surgical ligation of the ovarian veins has been associated with improvement in pain in approximately 75 percent of patients. The goal is to isolate the ovarian vein significantly above the pelvic brim (the edge of the pelvic cavity) and before the vein becomes substantially dilated.
Ovarian vein embolization (OVE) is a same-day treatment typically performed by interventional radiologists. The physician gains access either through a large vein in the groin or the neck by using a small catheter, which is a flexible tube like a strand of spaghetti. The catheter is moved through the vein to the enlarged pelvic veins, allowing the introduction of embolic agents, which are medications that cause the vein to seal off and relieve the painful pressure.
This treatment is less expensive than surgery and much less invasive. In addition, the reported technical success rates of ovarian vein embolization range from 89-100% with clinical success rates of 58-100%. Complication rates of 4-8% have been reported. According to the SVS/AVF clinical practice guidelines, embolization of refluxing ovarian veins with coils, plugs, or sclerotherapy (usually in combination), has become the standard approach for management of PVCS.
Index of suspicion
Historically, women with chronic pelvic pain have been frustrated by their lack of understanding about why they suffer from pain and available resources for treating it. PVCS is a diagnosis of exclusion. It's not the first thing that's thought of as the source of a woman's pain. Once gynecologists exclude ovarian cysts, infection, adhesions, endometriosis and other well-recognized gynecological disorders, they may never consider pelvic venous congestion.
With their deep understanding of the venous system – and access to it through imaging – phlebologists are almost always the specialists who recognize refluxing pelvic veins. They work closely with interventional radiologists to confirm a diagnose and to make a plan for treatment.
But Dr. Gerding believes that recognizing pelvic vein issues doesn't have to be limited to vein specialists and radiologist: "It takes a heightened awareness, education, and an 'index of suspicion.'"
RESOURCES
- Alebtekin Ahangari, BSc. PT. Prevalence of Chronic Pelvic Pain Among Women: An Updated Review. Pain Physician 2014; 17:E141-E147
- O’Brien MT, Gillespie DL. Diagnosis and treatment of the pelvic congestion syndrome. J Vasc Surg Venous Lymphat Disord. 2015 Jan;3(1):96-106. doi: 10.1016/j.jvsv.2014.05.007. Epub 2014 Jun 25.
- Whiteley MS, Lewis-Shiell C, Bishop SI, Davis EL, Fernandez-Hart TJ, Diwakar P, Beckett D. Pelvic vein embolisation of gonadal and internal iliac veins can be performed safely and with good technical results in an ambulatory vein clinic, under local anaesthetic alone – Results from two years’ experience. Phlebology. 2018 Sep;33(8):575-579. doi: 10.1177/0268355517734952. Epub 2017 Oct 9.
- RadiologyInfo.org. Ovarian Vein Embolization. bit.ly/2CrVzJn