What is pelvic congestion disorder?

Pelvic Congestion Syndrome: Definition, Diagnosis, and Management

Pelvic congestion syndrome (PCS) is a chronic pelvic pain condition caused by engorged and refluxing pelvic veins, characterized by dilated periuterine and periovarian veins, often associated with retrograde flow in the ovarian veins. 1

Definition and Pathophysiology

• PCS is defined as chronic pelvic pain (lasting at least 6 months) resulting from reflux or obstruction of the gonadal, gluteal, or periuterine veins, sometimes associated with perineal or vulvar varices 2
• It can also be caused by compression of the left renal vein between the superior mesenteric artery and the aorta (nutcracker syndrome) 2
• Multiple investigators have identified estrogen overstimulation as a contributing factor in pelvic venous disorders 1
• PCS accounts for up to 30% of patients presenting with chronic pelvic pain but is frequently underdiagnosed 2

Clinical Presentation

• Pain characteristics:
  • Variable intensity, typically worse in pre-menstrual period
  • Exacerbated by walking, standing, and fatigue
  • May be continuous (69% of patients) or intermittent 3, 4
• Associated symptoms:
  • Post-coital ache
  • Dysmenorrhea
  • Dyspareunia (reported in 60% of patients in some studies)
  • Bladder irritability
  • Rectal discomfort 3, 4
• Many women with PCS have morphologic findings of polycystic ovarian syndrome (enlarged ovaries with exaggerated central stroma and multiple small peripherally located follicles), but without the typical clinical features of hirsutism and amenorrhea 1
• Symptoms may subside after menopause in some women due to decreased estrogen stimulation 1

Diagnostic Approach

Imaging Modalities

• Ultrasound with Doppler is the initial imaging study of choice 1

  • Findings include:
    • Engorged periuterine and periovarian veins (≥8 mm)
    • Low-velocity flow
    • Altered flow with Valsalva maneuver
    • Retrograde (caudal) flow of the ovarian veins
    • Direct connection between engorged pelvic veins and myometrial arcuate veins 1

• MRI/MR angiography:

  • Diagnostic performance comparable to conventional venography
  • Considered the problem-solving imaging examination when ultrasound findings are nondiagnostic or inconclusive
  • Time-resolved postcontrast T1-weighted imaging can directly demonstrate ovarian vein reflux 1

• CT with contrast:

  • May demonstrate engorged periuterine and periovarian veins
  • Can identify venous anatomic variants and compression of the left renal vein
  • Lacks capacity to provide dynamic flow information compared to ultrasound or MRI 1

• Selective ovarian venography:

  • Considered the gold standard for definitive diagnosis
  • Usually performed immediately before treatment 5, 4

Treatment Options

Endovascular Treatment

• Ovarian vein embolization (OVE) is the most favored treatment option for patients with PCS 2, 4

  • Technical success rates of 96-100%
  • Long-term symptomatic relief in 70-90% of cases
  • Low complication rates 4
  • Can be performed on an outpatient basis 3
  • Internal iliac vein embolization (in addition to ovarian vein embolization) has been shown to be safe and effective 1

• For patients with nutcracker syndrome:

  • Endovascular stenting of the left renal vein has shown promise in alleviating symptoms as an alternative to open surgery 2

Other Treatment Approaches

• Medical management:

  • Analgesics and hormonal therapy have been used but with limited success 3, 5

• Surgical options (less commonly used now):

  • Hysterectomy combined with oophorectomy
  • Open surgical ligation of ovarian veins
  • Laparoscopic vein ligation 6, 5

Clinical Pitfalls and Considerations

• PCS is frequently underdiagnosed, with patients often seeing multiple specialists before diagnosis 4
• Many women with pelvic varices do not have PCS symptoms - the presence of varices alone is not diagnostic 6
• Under-diagnosis can lead to anxiety and depression 4
• A multidisciplinary approach involving gynecologists, vascular specialists, pain specialists, and radiologists is vital 4
• There is a lack of clear definition and high-quality evidence in the clinical domain of pelvic venous disorders 1
• The condition is referred to by various terms in literature, causing confusion in diagnosis and management 4
• Symptoms may persist in women with pelvic varicosities measuring over 5 mm at ultrasound even after treatment 3

Follow-up Considerations

• Follow-up should include assessment of symptom improvement and ultrasound examination to evaluate reduction in periovarian varicosities 3
• The longest duration of follow-up currently reported in literature is five years 6
• Potential complications of embolization include migration of coils used to occlude veins 6