Options for pelvic congestion syndrome

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Pelvic congestion syndrome (PCS) is a poorly understood and often overlooked etiology of chronic pelvic pain. Millions of women worldwide may develop chronic pelvic pain at some time in their life, and the occurrence may be as high as 39.1%.1

Chronic pelvic pain can be debilitating and accounts for 10% to 15% of all gynecologic visits.1 Managing this complex condition can be a challenge for the primary-care provider. When clinical and ultrasound examinations are normal, further diagnostic imaging can helpful to obtain the diagnosis. Once identified, PCS can be treated successfully with embolization therapy. 




Etiology


PCS is associated with dilated pelvic varices with reduced venous clearance, most often as a result of retrograde flow in an incompetent ovarian vein. The condition is seen more often in multiparous premenopausal women. A relationship between PCS and endogenous estrogen levels is suggested, as estrogen is known to weaken the vein walls.1

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The venous congestion stretches the inner surface of the ovarian vein, distorting both the endothelial and smooth-muscle cells. It is postulated that kinking of the ovarian vein leads to venous stasis, flow reversal, and subsequent varicosities.2 PCS can also be caused by external compression, such as that seen in nutcracker syndrome (compression of the left renal vein between the aorta and superior mesenteric artery) and May-Thurner syndrome (compression of the left iliac vein beneath the iliac artery). 


Clinical presentation


PCS is not easy to diagnose. Women typically complain of a dull, throbbing and achy pain in the vulvar region. This pain often worsens during or after intercourse or just before the onset of menses and/or increases throughout their day. Typically, women with PCS will not be symptomatic in the morning but will become so with prolonged standing or sitting.

The typical PCS patient may or may not have vulvar varicosities but often has varicose veins with the left leg presenting greater than the right. The varicosities usually extend along the medial aspect of the medial to posterior upper thigh and along the buttocks. 


Making the diagnosis more challenging is the vast array of the associated symptoms, including cyclic pain (with menstrual periods), dyspareunia, bladder irritability, GI symptoms and low back pain. Hemorrhoids and/or varicose veins of the perineum, buttocks or lower extremities may also be noted.1 Ovarian point tenderness on examination with a history of postcoital ache is said to be 94% sensitive and 77% specific for PCS.3

PCS is often diagnosed in women younger than age 45 years who have had more than one pregnancy. The ovarian veins increase in size during each pregnancy and do not return to normal in women with PCS. PCS is rarely diagnosed in nulliparous women. 


Patient evaluation


A detailed history and comprehensive examination is of paramount importance. The history should focus on the nature, intensity, pattern, location, duration and radiation of the pain, as well as any exacerbating and relieving factors. The relationship between the pain and a woman's menstrual cycle should also be discussed.

Screening for depression, personality disorders and domestic violence should be included. Women with any of these identifiers will have a higher incidence of somatic complaints and should be treated in conjunction with the assistance of a trained mental-health professional. 


The review of systems should involve a thorough discussion regarding the patient's sleep patterns, lifestyle (e.g., whether the pain affect activities of daily living), menstrual pattern, dyspareunia, urologic dysfunction and any GI issues. Irritable bowel syndrome (IBS) has been reported in 65% to 79% of women with chronic pelvic pain. All previous consultations, as well as diagnostic or therapeutic interventions, should be reviewed. 


In addition to an abdominal and pelvic examination, a thorough physical should include the neurologic (evaluating the thoracolumbar spine), cardiovascular, pulmonary and vascular (evidence of varicosities in the lower pelvis, buttocks, and legs) systems. Further testing should include a complete blood count, complete metabolic profile, urinalysis and an endocervical swab for chlamydia.


Differential diagnoses


The differential diagnoses for pelvic pain are vast, further adding to the complexity of the disorder. These diagnoses include endometriosis, chronic pelvic inflammatory disease, leiomyoma, adenomyosis, nutcracker syndrome, PCS, IBS, diverticulitis, diverticulosis, Meckel's diverticulum, interstitial cystitis, abnormal bladder function, chronic urethritis, fasciitis, nerve entrapment syndrome, hernia, scoliosis, spondylolisthesis, osteitis pubis, somatization, psychosexual dysfunction and depression. 


Imaging studies


Transvaginal ultrasound is helpful in identifying ovarian cysts or uterine leiomyomas. If the ultrasound results are inconclusive, an MRI of the pelvis (with and without contrast) is warranted. MRI is helpful with demonstration of the ovarian vein (Figure 1) and varicosities.

Figure 1

Dynamic imaging can confirm the active reflux from the ovarian vein. Bear in mind that the MRI is done in the supine position, which can alter the appearance of the varicosities in the pelvis. Whenever possible, the MRI should be scheduled for later in the day or early evening to coincide with a woman's increased perception of symptoms. 


Treatment options


After the diagnosis of PCS has been identified, medical treatment with nonsteroidal anti-inflammatory drugs may help relieve the patient's pain for the short term. Analgesia is a good first-line option, but if symptoms do not improve, a referral to an interventional radiologist should be made.

An interventional radiologist specializes in minimally invasive treatments using imaging for guidance. This clinician performs a thorough history and physical and reviews the imaging with the patient before scheduling her for pelvic venography. 


Pelvic venography an outpatient procedure and remains the gold standard for diagnosis of PCS. During venography, contrast dye is injected into the veins of the pelvis to make them visible via fluoroscopy, using the jugular vein for access. Performing the procedure with the patient placed on an incline will improve the accuracy of the diagnosis.

If this is not an option, a Valsalva maneuver is required and is often best achieved by having the patient blow into the tubing of a sphygmomanometer and hold the mercury at approximately 20 mm. If no reflux is present, a diagnosis of PCS is highly unlikely. If pelvic and/or ovarian varicosities are present (Figure 2), embolization of the offending abnormal vein can be performed. 


 

Figure 2

In this procedure, the interventional radiologist inserts a small guidewire and catheter into the faulty vein and embolizes it with coils, plugs, or sclerosant (Figure 3).

After treatment, women are discharged home the same day and able to return to regular activity the next day. Some patients may experience some mild cramping for 24 to 48 hours, which is managed well with ibuprofen. 


 

Figure 2

Outcomes


Embolization is successful in 98% to 100% of all PCS cases, with recurrence rates of less than 8%. Symptom improvement has been documented in 70% to 85% of women studied and can be expected within two to four weeks post procedure.4 Long-term follow-up data are minimal, however a study revealed that 83% of patients continued to remain symptom-free four years post treatment.4

Because it can negatively affect the quality of life and personal relationships of women and result in physical and psychological suffering, chronic pelvic pain is a significant health problem for many women. Patients with chronic pelvic pain report a high incidence of anxiety, depression and physical worries.5

Embolization offers a safe, effective and minimally invasive treatment option that can improve or resolve symptoms in women with chronic pelvic pain caused by PCS.

Debbie Semmel MSN, FNP-BC, is a family nurse practitioner at Duke University Medical Center, Division of Interventional Radiology, in Durham, N.C.


References


  1. Ignacio EA, Dua R, Sarin S, et al. Pelvic congestion syndrome: diagnosis and treatment. Semin Intervent Radiol. 2008;25:361-368. Available at www.ncbi.nlm.nih.gov/pmc/articles/PMC3036528/.

  2. Giacchetto C, Catizone F, Cotroneo GB, et al. Radiologic anatomy of the genital venous system in female patients with varicocele. Surg Gynecol Obstet. 1989;169:403-407. 

  3. Beard RW, Reginald PW, Wadsworth J. Clinical features of women with chronic lower abdominal pain and pelvic congestion. Br J Obstet Gynaecol. 1988;95:153-161.

  4. Kim HS, Malhotra AD, Rowe PC, et al. Embolotherapy for pelvic congestion syndrome: long-term results. J Vasc Interv Radiol. 2006;17(2 Pt 1):289-297.

  5. Walling MK, Reiter RC, O'Hara MW, et al. Abuse history and chronic pain in women: prevalences of sexual abuse and physical abuse. Obstet Gynecol. 1994;84:193-199.


All electronic documents accessed September 15, 2013.


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