Pelvic Congestion Syndrome

Pelvic congestion syndrome (a pelvic venous disorder) is chronic pelvic pain caused by venous incompetence and dilation of the ovarian and internal iliac veins and pelvic venous plexuses, leading to pelvic venous stasis. It occurs in multiparous women of reproductive age (typically 20 to 45 years) and accounts for 15 to 30 percent of chronic pelvic pain in this group.

Mechanisms:

• Incompetent or absent valves in the ovarian veins (about 15 percent of women have absent valves, left more than right)
• Venous reflux
• Hormonal effect: oestrogen causes venodilation, and pregnancy raises venous capacity and pressure
• Pregnancy, with compression by the gravid uterus
• Venous obstruction: nutcracker syndrome (left renal vein compression between aorta and superior mesenteric artery), May-Thurner syndrome (left common iliac vein compression by the right common iliac artery), or retroaortic left renal vein

Pelvic veins above 5 to 6 mm in diameter with reflux are significant.

• Dull, aching, dragging pelvic pain or heaviness that is unilateral or bilateral, lasting more than 6 months, worse at the end of the day, with prolonged standing or sitting, after intercourse (postcoital ache lasting hours), during menses and premenstrually, and relieved by lying down.
• Dyspareunia, dysmenorrhoea, and urinary urgency.
• Visible varicose veins of the vulva, perineum, buttocks and thighs (from pelvic origin, in about 50 percent), and lower limb varices recurring after surgery.
• Pain on bimanual examination, tender ovaries, and cervical or adnexal tenderness; the pain may be reproduced by pressure over the ovarian point.
• Nutcracker syndrome: left flank pain and haematuria, and left varicocele in men.
• Normal examination otherwise; symptoms are often disproportionate to findings.

• Transvaginal and transabdominal Doppler ultrasound: dilated, tortuous pelvic veins (above 5 to 6 mm), slow flow (below 3 cm/s) or reflux on Valsalva, polycystic appearing ovaries, dilated arcuate veins crossing the myometrium; examination in the standing position increases sensitivity.
• CT or MR venography: pelvic veins above 6 mm and ovarian vein above 8 mm, with tortuous parauterine veins; identifies compression syndromes (nutcracker, May-Thurner).
• Catheter venography (the gold standard) with selective ovarian and internal iliac vein injection: ovarian vein above 6 mm, retrograde flow, delayed contrast clearance (over 20 seconds), contrast filling of pelvic plexus and crossover to the contralateral side; performed at the same time as therapeutic embolisation.
• Exclusion of other causes of chronic pelvic pain: endometriosis, adenomyosis, ovarian pathology, adhesions, interstitial cystitis, and irritable bowel syndrome; laparoscopy may be needed, though in a supine position veins collapse and findings are less visible.

• Medical therapy: medroxyprogesterone acetate 30 mg daily orally for 6 months. It reduces venous congestion and ovarian function, with improvement in about 70 percent but recurrence after stopping.
• Goserelin 3.6 mg every 28 days for 6 months (with add back therapy).
• Venoactive drugs (micronised purified flavonoid fraction 500 mg twice daily for 6 months, diosmin 600 mg daily, or sulodexide), and analgesics (paracetamol, non-steroidal anti-inflammatory drugs).
• Lifestyle measures: avoiding prolonged standing, leg elevation, compression garments, weight loss, avoiding constipation.
• Endovascular embolisation is the preferred treatment for refractory pain. Use transfemoral or transjugular catheterisation under local anaesthetic. Embolise the refluxing ovarian veins (and internal iliac tributaries when needed) using coils (3 to 8 mm), plugs (Amplatzer vascular plug), glue or sclerosant foam (sodium tetradecyl sulfate 3 percent foam, polidocanol), generally with coils plus sclerosant.
• Symptoms improve in 70 to 85 percent (with complete relief in about 50 percent). Recurrence or persistent symptoms require reintervention.
• Embolisation complications: coil migration, vein perforation, thrombophlebitis, post-embolisation syndrome (pain, fever and nausea for 3 to 5 days), contrast reaction, and radiation. Advise patients to avoid heavy exertion for 1 week.
• Stenting when significant compression exists: for May-Thurner syndrome, an iliac vein stent with dual antiplatelet or anticoagulation for 3 to 6 months; for nutcracker syndrome, a left renal vein stent, or transposition. Anticoagulation is not otherwise needed for pelvic venous disorder.
• Surgery is reserved for refractory cases after childbearing, usually after embolisation fails, with removal of all dilated veins. Options are laparoscopic ovarian vein ligation or resection (success about 75 percent), and hysterectomy with bilateral salpingo oophorectomy (success about 75 to 85 percent).
• Treat coexisting varicose veins of pelvic origin by sclerotherapy of the vulval or leg varices after the pelvic source is closed.
• Give hormone replacement after oophorectomy. Continue pain control and counselling. Avoid oestrogen containing contraception in women with severe symptoms.
• Outcome: good in most. Recurrence after embolisation is 10 to 20 percent. Pregnancy after embolisation is possible.