Pelvic Congestion Syndrome (PCS), belongs to the group of pelvic venous syndromes along with vulvar varicosities, a widely overlooked and poorly understood disorder.
PCS mainly presents as chronic pelvic pain or discomfort associated with prolonged standing or sexual intercourse largely in women’s population presenting with periovarian varicosities on imaging studies.
Multiparous women in their reproductive years are mainly affected as no cases have been diagnosed in menopausal women. PCS is present in up to 30 percent of patients suffering from chronic pelvic pain in which etiology can’t be identified.
While the etiology of PCS is still a mystery. Many physicians have noticed increased diameter, valve incompetence, and reflux of the ovarian veins in women with PCS thus the primary etiology currently revolves around underlying venous pathology.
Combination of hormonal and anatomic factors result in venous insufficiency of internal iliac and ovarian veins ultimately leading to periovarian pelvic varicosities.
Ovarian vein dilatation, stasis, and/or reflux on pelvic venography are common findings among multiparous premenopausal women, and most are asymptomatic. No clear causal relationship has been established, but improvement of symptoms in those who are symptomatic after administering venoconstrictors (dihydroergotamine) or after performing ligation/embolization of ovarian vein has been noted.
The prevalence of PCS among multiparous women can be due to the fact that pelvic vein capacity increase up to 50 percent during pregnancy thus precipitating to venous incompetence and reflux in postpartum period
PCS is more frequent on the left side due to compression of left renal vein between aorta and superior mesenteric artery (nutcracker phenomenon[MEH1] ) or valvular incompetence of ovarian vein due to valve absence which is more prevalent on the left side.
Estrogen acts as a venodilator hence absence of PCS among menopausal women.
Diagnosis and diagnostic evaluations in PCS:
Diagnosis is based on combination of unique symptoms, detecting pelvic vein dilatation or incompetence, and tenderness on physical examination. Usually diagnosis of exclusion.
Clinical manifestations PCS:
Pain lasting at least 6 month, onset most often during or after pregnancy, may worsen with more pregnancies. Factors that may worsen symptoms may include prolonged standing, walking, intercourse, or exercises that increase intraabdominal pressure (crunches, lower abs strengthening). Most often presents unilaterally.
Cervical motion tenderness (CMT), ovarian tenderness, and uterine tenderness with palpation. These physical findings can be present with other medical conditions such as pelvic inflammatory disease thus unspecific and undiagnostic for PCS.
The presence of pelvic venous changes on imaging studies helps support the diagnosis because incompetent and dilated ovarian veins are non-specific for PCS.
Usually is the preliminary test performed as it cheap, noninvasive, and harmless to the patient. USG can eliminate several potential causes of pain from the differential such as neoplasms and certain uterine pathology. In PCS, the left ovarian vein is dilated with reversed caudal flow. In addition, the presence of tortuous and dilated pelvic venous plexuses, dilated arcuate veins crossing the uterine myometrium, and variable duplex waveforms in varicoceles during the Valsalva’s maneuver help support the diagnosis.
Ovarian and internal iliac venography in symptomatic patients are recommended. The procedure involves catheterization of the right and left ovarian veins via percutaneous femoral or jugular approach. Ovarian venograms and patients with PCS usually demonstrate abnormally dilated ovarian veins (more than a 10 mm in diameter), slow blood flow, reflux causing retrograde fill and congestion of the ovarian venous plexus.
CT and MRI
Those imaging studies identify tortuous, dilated pelvic and ovarian veins, broad ligament vascular congestion, and ovarian varicoceles better than ultrasonography.
Characteristic pelvic venous changes can sometimes be visualized at laparoscopy laparotomy which exclude other causes of chronic pelvic pain.
Management of PCS:
PCS without vulvar varices:
Short trial of medical therapy with medications such as medroxyprogesterone acetate, etonogestrel implant, or goserelin. This is supported by a trial in which women treated with medroxyprogesterone complained of rapid return of pain after cessation of medication
Invasive treatment is reserved for women who do not respond to pharmaceutical therapy, and those include embolization or sclerotherapy of ovarian veins, laparoscopic or open ligation of the ovarian veins, and hysterectomy with bilateral salpinoophorectomy (BSO) for women who no longer desire to have children.
PCS with vulvar varices:
Embolization is preferred because it is safe, well tolerated, minimally invasive approach. Direct vulvar sclerotherapy or local excision of varices can be performed for persistent disease
PCS with simultaneous left ovarian vein compression syndrome “nutcracker syndrome”:
Results from compression of the left renal vein at the origin the superior mesenteric artery. This entrapment can cause symptoms of pelvic congestion with retrograde venous flow and dilated gonadal vein. Medical options include embolization of gonadal veins, laparoscopic gonadal vein ligation, or treatment of the renal vein compression.
Summary and conclusion:
Pelvic congestion syndrome:
The etiology of PCS is unclear, there’s no definitive diagnostic criteria, and the optimum treatment is uncertain.
PCS is characterized by a dull ache or heaviness exacerbated by menstrual cycle, prolonged standing, postural changes, or any activity that increase intra-abdominal pressure, and after sexual activity. Dysmenorrhea, deep dyspareunia, and urinary urgency are also present in some patients with PCS
The diagnosis of PCS is a diagnosis of exclusion and is based on a combination of characteristic symptoms, tenderness on physical examination, and documentation of pelvic vein dilation or incompetence using imaging studies.
Diagnostic laparoscopy can be used to exclude other medical causes of pelvic pain.
Despite limited clinical response, trial of hormonal medical therapy is recommended. Patients who don’t respond to medical therapy can qualify for embolization or sclerotherapy. For specific patient criteria with both PCS and left ovarian vein compression syndrome, the treatment approach includes embolization of gonadal veins or laparoscopic gonadal vein ligation, treatment of the renal vein compression, or both.
- Venbrux AC, Lambert DL. Embolization of the ovarian veins as a treatment for patients with chronic pelvic pain caused by pelvic venous incompetence (pelvic congestion syndrome). Curr Opin Obstet Gynecol 1999; 11:395.
- Scultetus AH, Villavicencio JL, Gillespie DL, et al. The pelvic venous syndromes: analysis of our experience with 57 patients. J Vasc Surg 2002; 36:881.
- Chung MH, Huh CY. Comparison of treatments for pelvic congestion syndrome. Tohoku J Exp Med 2003; 201:131.
Mahmoud El Hajj
M. Akram Khan, MD, MBA, FACC, FSCAI