Pelvic Congestion Syndrome

Erin Johnson had been dealing with chronic pelvic pain since she was 16 years old. Over the years, she underwent many CT scans and X-rays, and received many different diagnoses.

“It was a powerful ache in my lower abdominal and back area that would get worse throughout the day,” says Erin.

The mysterious pain worsened in her twenties after she had children, ultimately resulting in the removal of her gall bladder. Still, the pain persisted.

“I loved going on runs, but the pain would get worse when I’d start running,” Erin adds.

At one point her gynecologist suggested that Pelvic Congestion Syndrome (PCS) may be the culprit. After some research, Erin found the similarities between the symptoms of PCS and her own were uncanny. She was advised to consult with an interventional radiologist. A CT scan was completed that noted that some of the veins in her abdomen were bigger than they should be, which is a red flag for Pelvic Congestion Syndrome. 

Chronic pelvic pain is defined as “non-cyclic” pain lasting greater than six months. It is generally a frustrating diagnosis and leaves patient searching for answers for years. 

Researchers are not sure exactly how many people in the United States have chronic pelvic pain. Because it is often linked to other disorders, such as endometriosis or vulvodynia, chronic pelvic pain may be misdiagnosed as another condition, making it difficult to estimate reliable prevalence rates. According to one study, about 15% of women of childbearing age in the United States reported having pelvic pain that lasted at least 6 months. Worldwide, the rates of chronic pelvic pain for women of childbearing age range from 14% to 32%  Between 13% and 32% of these women have pain that is severe enough to cause them to miss work.  Unfortunately 60% of women in chronic pelvic pain never receive a specific diagnosis.

In the past, a diagnosis of chronic pelvic pain left many patients frustrated with few treatment options and a lack of available resources. Their physicians were likewise perplexed, despite the endless acquisition of negative laboratory and imaging data as well as inconclusive consultations obtained. In the last 10 years, improved scientific understanding and increased physician awareness have lessened the confusion surrounding this condition.

While there has been an increase in understanding of and treatment of pelvic pain issues, many aspects of it remain underappreciated. One such diagnosis, Pelvic congestion syndrome, appears to continue to be an underdiagnosed and undertreated disease.

Men and women can develop pelvic congestion syndrome. Typical age of onset is between 20 and 45 years old. Often patients will complain of a severe dull ache or pain in the abdomen, pelvis, groin, and/or genitalia. This may be a result of pelvic varicosities, which present similar to varicose veins of the legs. Because of vein wall weakness and inability of the it to return blood effectively, pressure in the vein increases as blood pools or remains in the pelvic vein. This may cause pressure pain and throbbing that can contribute to nerve irritation in the pelvic region. Risk factors may include obesity, pregnancy, sedentary lifestyle, constipation, prostatitis, surgeries, ovarian varicosities, and endogenous estrogen levels.

Male pelvic congestion syndrome is underrepresented in medicine and may be mistakenly referred to as prostatitis syndrome. Male pelvic congestion syndrome can be caused by pelvic varicosities and surgery such as varicocelectomy and can be related to varicoceles of the scrotum, prostatitis syndrome, and pelvic floor muscle dysfunction. 50 % of male infertility cases involve pelvic congestion syndrome. Testicular atrophy may also occur.  In men this is often diagnosed through presentation of visible varicosities on the scrotum cavarello.

Patients with pelvic congestion syndrome generally report that the dull pain is chronic but that it worsens in certain situations, including:

• after standing up for a long time
• in the evenings
• during and after sexual intercourse
• in women in the late stages of pregnancy
• in women during the days leading up to menstruation

Besides the pain, patients may experience other PCS symptoms as well as different combinations of these symptoms. The severity can also vary quite widely between individuals. These symptoms can include:

• dysmenorrhea (painful menstruation)
• abnormal bleeding during menstruation
• backache
• depression
• fatigue
• varicose veins around the vulva or scrotum, buttocks, and legs
• abnormal vaginal discharge
• swelling of the vagina or vulva
• tenderness of the abdomen
• increased urination
• irritable bowel symptoms
• hip pain

The diagnosis of this is generally one of exclusion. The first step in the treatment of PCS related to chronic pelvic pain requires a multidisciplinary approach because the differential diagnosis is quite long and varied. For women evaluation by an obstetrics/gynecology specialist is a fundamental part of the assessment, but input from other specialties including anesthesiology, gastroenterology, general surgery, neurology, hematology/oncology, psychiatry, and urology may also be necessary. The standard workup usually includes an abdominal and pelvic examination, Pap smear test, routine laboratory blood work, and some cross-sectional imaging.

• Bowel pathology
• Cancer/metastases
• Endometriosis
• Fibroids
• Fibromyalgia
• Neurologic pathology
• Orthopedic pathology
• Ovarian cyst
• Pelvic congestion syndrome
• Pelvic inflammatory disorder
• Bowel pathology
• Cancer/metastases
• Endometriosis
• Fibroids
• Fibromyalgia
• Neurologic pathology
• Orthopedic pathology
• Ovarian cyst
• Pelvic congestion syndrome
• Pelvic inflammatory disorder

After a physical examination, a Pap test to rule out cervical cancer, and routine laboratory bloodwork, a cross-sectional imaging study is obtained to be certain that there is not a pelvic tumor. If the clinical symptoms are those of chronic pelvic pain, worse when sitting or standing, and sometimes also associated with varicose veins in the thigh, buttock regions, or vaginal area, the possibility of ovarian vein and pelvic varices must be considered.

Once tests have been performed to exclude other causes of chronic pelvic pain, definite tests that can be done to establish a diagnosis of pelvic congestion syndrome include:

•  Ultrasound: This non-invasive procedure is typically the first diagnostic test done to prove that a woman is suffering from pelvic congestion syndrome. The ultrasound can either be abdominal or transvaginal and may reveal the presence of dilated pelvic or ovarian veins suggestive of pelvic congestion syndrome.
• CT venogram: The CT venogram is a non-invasive diagnostic test, just like the ultrasound. This procedure involves the use of a CT scan machine to scan the pelvic and ovarian veins. It is better than the ultrasound in visualizing abnormally dilated pelvic or ovarian veins.
• MRI venogram: This is similar to the CT venogram, the only difference being that an MRI machine is used to perform the testing rather than a CT machine.
• Pelvic venography: This is the gold standard in the diagnosis of pelvic congestion syndrome. With pelvic venography, a contrast dye is placed using local anesthetic into the common femoral or internal jugular vein. An x-ray machine is then used to take several images as the dye spreads throughout the venous system. Abnormal vessels can then be easily located and visualized. Any areas of obstruction are also highlighted by the contrast as well.
• Of importance to note, pelvic congestion syndrome is often missed on imaging due to the supine positioning, typically used for testing, which may cause a decrease in venous distention.

After diagnosis treatment options vary. Some options include:

• Non-steroidal anti-inflammatory drugs (NSAIDs): NSAIDs can be taken to provide symptomatic relief from the chronic pain that is associated with pelvic congestion syndrome. However, these medications do not address the underlying cause of the pelvic pain, which is the insufficiency of the pelvic or ovarian veins.
• Medroxyprogesterone acetate (MPA): This suppresses ovarian function and increases venous contraction, thereby enhancing the flow of blood back to the heart.
• Goserelin: This is a Gonadotropin-releasing hormone (GnRH) analogue which functions similar to Medroxyprogesterone acetate in increasing venous contraction as well as suppressing ovarian function.
• Foam sclerotherapy: With this procedure, the provider inserts a catheter close to the dilated pelvic or ovarian veins. When this is done, a substance known as a sclerosant is injected into these veins which then seals up the veins. The pain symptoms are improved once all the dilated veins are completely sealed up.
• Pelvic vein embolization: Female pelvic vein embolization is the gold standard in the treatment of pelvic congestion syndrome. Typically, a catheter is placed through the internal jugular, femoral, subclavian, or brachial veins and directed to the site of the dilated pelvic or ovarian veins. Tiny coils are then inserted into the dilated veins which then results in the development of clots. These clots then seal off the dilated veins and provide relief from pelvic congestion syndrome.

Pelvic congestion can lead to chronic pelvic pain, pain with intercourse, increased urinary frequency and urgency, infertility, erectile dysfunction, and possible back and leg pain. Sometimes patients complain of a dull ache that is aggravated by physical activity (especially with standing). Physical therapy can help improve symptoms of this diagnosis through myofascial release, massage, lymph drainage, postural modifications, patient education, biofeedback stretching and strengthening exercises. 

So how did things work out for Erin? She elected to undergo ovarian vein embolization. The minimally invasive procedure closes off faulty veins so they can no longer enlarge with blood and cause pain. Typically, patients are in the hospital for no more than two or three hours and can resume normal activity almost immediately.

“The very next day I went for a run and had no pain at all,” Erin shares. “I’ve lived through decades of strict medication and diet regimens. I’ve had countless vacations and family events ruined by pain. And now that pain is gone.”