Pelvic congestion syndrome

Pelvic congestion syndrome
A very large (9cm) fibroid of the uterus which is causing pelvic congestion syndrome as seen on X-ray computed tomography
Classification and external resources
Specialty
ICD-9-CM	625.5
Patient UK	Pelvic congestion syndrome

Pelvic congestion syndrome (also known as pelvic vein incompetence) is a chronic medical condition in women caused by varicose veins in the lower abdomen. The condition causes chronic pain, often manifesting as a constant dull ache, which can be aggravated by standing. Early treatment options include pain medication, alternative therapies such as acupuncture, and suppression of ovarian function. Surgery can be done using noninvasive transcatheter techniques to embolize the varicose veins. Up to 80% of women obtain relief using this method.

The condition can occur as a result of pregnancy or for unknown reasons. The presence of estrogen in the body causes vasodilation, which can result in the accumulation of blood in the veins in the pelvic area. Estrogen can weaken the vein walls, leading to the changes that cause varicosities. Up to 15% of all women have varicose veins in the abdominal area, but not all have symptoms.

Pathophysiology

Multiple factors contribute to the pathogenesis of PCS. The characteristic severe dull aching pain of PCS is thought to be a direct result of the presence of ovarian and pelvic varicosities, much like the leg pain resulting from lower extremity varicose veins. Multiparous women seem to be predisposed to develop PCS. In patients with multiple previous pregnancies, there may have been a significant increase in intravascular volume at each term of gestation. Vein capacity can increase by 60%. Over time, venous distension can render the valves incompetent. Additionally, the weight gain and anatomic changes in the pelvic structures during pregnancy may cause chronic intermittent venous obstruction. Blood pooling in the pelvic and ovarian veins may cause further engorgement, thrombosis, and mass effect on nearby nerves, collectively contributing to pelvic pain.3 The majority of women affected are premenopausal, and a relationship between PCS and endogenous estrogen levels is suggested, as estrogen is known to weaken the vein walls.

Obstructing anatomic anomalies may also lead to secondary PCS. In patients with a retroaortic left renal vein, there may be obstruction of the left ovarian vein leading to symptomatic pelvic varices. Additionally, the left ovarian vein and the left renal vein may by compressed by the superior mesenteric artery (Nutcracker phenomenon) as well. Finally, compression from the right common iliac artery on the left common iliac vein against the spine and pelvic brim is known to cause iliofemoral deep venous thrombosis (May–Thurner syndrome) as well as the pelvic varices of PCS.

Signs and symptoms

Women with this condition experience a constant pain that may be dull and aching, but is occasionally more acute. The pain is worse at the end of the day and after long periods of standing, and sufferers get relief when they lie down. The pain is worse during or after sexual intercourse, and can be worse just before the onset of the menstrual period.

Women with pelvic congestion syndrome have a larger uterus and a thicker endometrium. 56% of women manifest cystic changes to the ovaries, and many report other symptoms, such as dysmenorrhea, back pain, vaginal discharge, abdominal bloating, mood swings or depression, and fatigue.

Diagnosis

A very large (9cm) fibroid of the uterus which is causing pelvic congestion syndrome as seen on ultrasound

Diagnosis can be made using ultrasound or laparoscopy testing. The condition can also be diagnosed with a venogram, CT scan, or an MRI. Ultrasound is the diagnostic tool most commonly used. Recent research from a leading pelvic venous unit has suggested that Transvaginal Duplex Ultrasound scanning is the "Gold Standard" test for pelvic venous reflux. The same research group has shown that the size of the veins - as shown by venography and also used as the diagnostic criteria in CT and MRI - is not relevant and only Trannsvaginal Duplex Ultrasound shows the venous reflux that causes the problem.

The diagnosis of pelvic congestion syndrome is established by the demonstration of multiple dilated, tortuous parauterine veins with a width >4 mm or an ovarian vein diameter greater than 5–6 mm 4.

Ultrasound

• ovarian vein >5–6 mm (positive predictive value of 71–83%)
• may show multiple dilated veins in the adnexae with reversed venous flow on colour Doppler, especially after Valsalva maneuver
• the venous calibres may increase on real time during Valsalva

CT

Contrast enhanced CT typically shows dilated pelvic and ovarian veins. The supine position during scanning may underestimate the size of venous dilatation.

MRI

MR venography

May show dilated veins. Time of flight (TOF) imaging can be performed where contrast is not required.

Signal characteristics

• T1: seen as flow voids
• T2: mostly high signal but but can vary dependent on velocities from low signal to iso signal
• GE: high signal

Treatment

Treatment options for PCS remained elusive until recently, due to controversial diagnostic methods and poor understanding of its etiology ranging from psychosomatic origin to vascular causes. Since Topolanski-Sierra first noted an association in the 1950s between chronic pelvic pain and ovarian and pelvic varices,11 many treatment modalities have been proposed. Medical management with hormone analogues and analgesics, surgical ligation of ovarian veins, hysterectomy with or without bilateral salpingo-oophorectomy and transcatheter embolization have been described in the literature as treatment options for patients with PCS today.

Medical treatment of PCS includes psychotherapy, progestins, danazol, phlebotonics, gonadotropins receptor agonists (GnRH) with hormone replacement therapy (HRT), dihydroergotamine, and nonsteroidal antiinflammatory drugs (NSAIDS). Specifically, the literature supports use of medroxyprogesterone acetate (MPA), or the GnRH analogue goserelin in an effort to suppress ovarian function and/or increase venous contraction. MPA may be given orally 30 mg/day for 6 months. Goserelin acetate is dosed as an injection of 3.6 mg monthly over a 6-month period. As chemical ovarian ligation has numerous side effects, estrogen replacement or “add-back” therapy is frequently required as well.12

In the 1980s, surgical treatment was described by Rundqvist et al., which consisted of extraperitoneal resection of the left ovarian vein, which proved to be useful in relieving symptoms of PCS.13 Subsequent studies described anatomic abnormalities with the proposed etiology being incompetent ovarian veins. This notion was supported by the fact that surgical treatment such as ventrosuspension of the retroverted uterus and hysterectomies proved to be of little benefit. Despite the curative intent of hysterectomy, studies reported residual pain in 33% of patients and a 20% recurrence rate,14,15 which led to the advent of surgical ligation or resection of ovarian veins. More recently, laparoscopic ligation bilateral ovarian veins has been gaining popularity among laparoscopic gynecologists, but surgical experience of ovarian vein ligation is anecdotal with only a few available case studies.3,16 The procedure is performed in a supine position with insufflation of pressurized carbon dioxide into the peritoneal cavity, forcing venous effacement and decompression, which potentially underestimates the number of actual varices thereby decreasing procedural efficacy.9 In addition, laparoscopy is an invasive procedure that generally requires anesthesia and may be associated with significant morbidity, poor cosmesis, and a hospital stay of at least 2 days.9

To improve clinical efficacy and reduce perioperative and postoperative morbidity, percutaneous pelvic vein embolization therapy has been utilized (Figs. 3 and and4).4). Since its introduction in 1993 by Edwards et al., this modality has revolutionized the treatment of PCS.17 The procedure is usually performed at the time of diagnostic venography using a variety of embolic agents, including sclerosant foam and coils. In several published series in the 1990s, success rates for reduction of chronic pelvic pain ranged from 50 to 80%. With advancements in technique, clinical success is achieved in 70 to 85% of treated patients.9,18,19 Kim et al. found significant improvement in 83% of women in their overall pain perception levels with a mean of 45 months of long-term follow up.18

See also

• Interventional radiology
• Ovarian vein syndrome
• Nutcracker syndrome