Pelvic congestion syndrome

What is pelvic congestion syndrome?

DEFINITION

Pelvic congestion syndrome (PCS) is broadly defined as chronic pelvic pain (CPP) caused by dilated and dysfunctional pelvic veins. It is difficult to diagnose because of overlapping symptoms seen with other causes of pelvic pain.

Synonyms

• PCS
• Pelvic congestive syndrome
• Pelvic venous syndrome
• Pelvic venous insufficiency
• Pelvic varices
• Pelvic vascular dysfunction

Pelvic congestion syndrome is a condition characterized by chronic dull pelvic pain, pressure, and heaviness that persists for >6 months’ duration, secondary to congested pelvic veins. It usually affects multiparous women of reproductive age.

On CTA and MRA, imaging findings may include dilated gonadal veins (>6 mm in caliber), prominent (>4 mm) serpentine periuterine or periovarian veins, perineal varices, retrograde flow of contrast material into the gonadal veins, or reversal of blood flow in the gonadal veins on TOF MRA. Associated findings of nutcracker syndrome may also be present.

It is important to note that dilated gonadal and periuterine veins are commonly seen in asymptomatic parous women, and therefore in isolation (i.e., without associated clinical symptoms or signs) do not indicate presence of pelvic congestion syndrome.

EPIDEMIOLOGY & DEMOGRAPHICS

• •Typically reported in reproductive-age women, most commonly in those ages 20 to 30 yr. Has never been reported in postmenopausal women. Pelvic varices are reported in up to 30% of CPP patients with no other obvious pathology; however, dilated vessels have been found in asymptomatic women as well.
• •Etiology is multifactorial, and it is thought that there are both mechanical and hormonal factors leading to vein reflux or obstruction with resulting dilation. Estrogen, which acts as a venous dilator, is considered a possible cause, because PCS is not typically seen in women after menopause. Mechanical causes may be secondary to intrinsic vein disorders (valve incompetence, etc.), vascular compression (nutcracker syndrome, May-Thurner syndrome, etc.), or extrinsic compression (endometriosis, tumors, etc.). PCS is also generally associated with multiparity; however, the syndrome has also been found to occur in nulliparous women.

PHYSICAL FINDINGS & CLINICAL PRESENTATION

• •There are no universally accepted or well-defined clinical criteria for the diagnosis of PCS. It is generally described as CPP (at least 6 mo duration) and evidence of pelvic vascular insufficiency/dilation on imaging, in the absence of other causes for pain.
• •Often manifests after first pregnancy and worsens with subsequent pregnancies.
• •Common presenting features include sensation of a dull ache or heaviness in the abdomen and pelvis, exacerbated by long periods of standing/walking, that improves when person is in a supine position. Typically worsens at the end of the day. May be exacerbated before or during menses. Pain during intercourse, with prolonged pain after intercourse (postcoital ache), is very typical.
• •Bimanual exam showing marked ovarian tenderness, cervical motion tenderness, and uterine tenderness supports a diagnosis of PCS in patients with characteristic symptoms.
• •Gluteal, vulvar, and/or thigh varices may also be present.

ETIOLOGY

One postulated mechanism for pain symptoms is that local stasis and stretch of the venous walls release nociceptive factors, contributing to inflammation and pain. Another theory is that enlarged veins may cause irritation of adjacent nerves.

DIAGNOSIS

Differential Diagnosis

The differential diagnosis for CPP is broad and includes gynecologic, urologic, gastrointestinal, musculoskeletal, neurologic, and psychologic disorders. Common causes of CPP include endometriosis, interstitial cystitis, irritable bowel syndrome, pelvic floor myalgia, and myofascial pain.

Workup

• •Detailed history and physical examination, imaging, and evaluation for other causes of CPP are part of the workup. Evaluation often requires a multidisciplinary approach and may include trials of medical management for other common pain conditions.
• •There are no specific laboratory tests for PCS.

Imaging Studies

• •Isolated findings on imaging are nondiagnostic for PCS, as pelvic varices are estimated to be present in 9.9% of the general population. Asymptomatic patients should not be treated for PCS.
• •There are no firm, agreed-upon criteria for imaging findings. Pelvic ultrasound, MRI, and CT, are all considerations for imaging workup. Catheter venography is considered the gold standard for diagnosis of PCS; however, it is typically preceded by less invasive imaging and reserved for cases in which intervention is planned with concurrent transcatheter embolization or sclerotherapy.
• •Pelvic ultrasound is considered first line for CPP, as it can be helpful in ruling out other diagnoses, as well as assess valve competence through position changes and color Doppler. Patel et al proposed the following diagnostic criteria:
  • 1.Tortuous parametrial/adnexal pelvic veins with diameter >4 mm
  • 2.Tortuous and dilated arcuate veins in myometrium that communicate with the varicose veins in the adnexa
  • 3.Slow blood flow (>3 cm/s) or reversal of blood flow in the left ovarian vein, with/without Valsalva maneuver
  • 4.Polycystic appearance of ovaries, without history of amenorrhea or hirsutism (found in up to 50% of PCS patients)

• MRI and CT offer cross-sectional imaging and survey of the surrounding tissue, thus allowing for evaluation of external venous compression (e.g., nutcracker syndrome, compressive tumors). Both are superior to ultrasonography in identifying tortuous, dilated pelvic and ovarian veins, and broad ligament vascular congestion. However, they are expensive and may require radiation (CT), although they are less invasive than catheter venography. Additionally, it can be difficult to assess direction of blood flow to diagnose reflux.
• •Diagnostic laparoscopy may miss up to 80% to 90% of PCS cases, secondary to variceal decompression from supine and Trendelenburg positioning, and gas insufflation of the abdominal cavity. It can assist in ruling out other causes of CPP such as endometriosis or adhesive disease.

TREATMENT

• •There is no standard approach to treatment, and management is individualized based on symptoms.
• •Referral to multidisciplinary teams, including gynecology, GI, pain specialists, physical therapy, and interventional radiology, may be indicated when the diagnosis is suspected to evaluate for other causes of pelvic pain.
• •Medical management is considered first line as risks are low. Inducing a hypoestrogenic state, via ovarian suppression, is the primary goal of medical management. Evidence for efficacy of medical management is limited, but pain improvement has been shown with medroxyprogesterone acetate, GnRH agonists, and the etonogestrel implant. There is emerging evidence that micronized purified flavonoid fraction (approved for chronic venous insufficiency) may improve symptoms in PCS.
• •Surgical management: Options currently available are removal of reproductive organs and alteration of venous blood flow. Of these options, hysterectomy has not been shown to consistently improve pain symptoms and is typically reserved for refractory cases.
  • 1.Ovarian vein embolization has become the preferred treatment, with a mean success rate of 75%, low recurrence rate (5%), and relative safety.
  • 2.Surgical ligation of the ovarian vein (either via laparoscopy or laparotomy) has shown pain improvement in 67% to 75% of patients; however, because surgical approach can only address a limited number of vessels, there may be increased risk for recurrence. Additionally, surgery is associated with longer hospital stays, recovery times, and higher risk of morbidity. However, surgery can concomitantly address other causes of pain (endometriosis, adhesions, etc.).

PEARLS & CONSIDERATIONS

• •Pelvic congestion syndrome is a controversial condition describing the association between chronic pelvic pain and pelvic varices without clear diagnostic criteria.
• •The differential diagnosis for CPP is broad, and medical treatment for PCS overlaps with methods that are effective for other common causes of CPP.
• •Some evidence suggests long-term symptom improvement with embolization or ligation.

SUGGESTED READINGS

• Akhmetzianov R., et al.: Clinical efficacy of conservative treatment with micronized purified flavonoid fraction in female patients with pelvic congestion syndrome . Pain Ther 2021; 10 (2): pp. 1567-1578.
• Bendek B., et al.: Comprehensive review of pelvic congestion syndrome: causes, symptoms, treatment options . Curr Opin Obstet Gynecol 2020; 32: pp. 237-242.
• Borghi C., Dell’Atti L.: Pelvic congestion syndrome: the current state of the literature . Arch Gynecol Obstet 2016; 293: pp. 291-301.
• Daniels J.P., et al.: Effectiveness of embolization or sclerotherapy of pelvic veins for reducing chronic pelvic pain: a systematic review . J Vasc Interv Radiol 2016; 27: pp. 1478-1486.