What is pelvic congestion syndrome?

Pelvic Congestion Syndrome

Pelvic congestion syndrome is a disorder characterized by chronic pelvic pain caused by dilation, engorgement, and reflux of pelvic veins, primarily affecting premenopausal women and often resolving after menopause due to decreased estrogen levels. 1, 2

Definition and Pathophysiology

Pelvic congestion syndrome (PCS) is characterized by:

• Engorged periuterine and periovarian veins (typically >8mm in diameter)
• Venous reflux and altered flow patterns in pelvic vessels
• Chronic pelvic pain lasting more than 6 months without evidence of inflammatory disease 3

The exact etiology remains uncertain but is likely multifactorial, involving:

• Valvular insufficiency in ovarian and pelvic veins
• Venous obstruction (such as in nutcracker syndrome where the left renal vein is compressed)
• Hormonal influences, particularly estrogen, which may contribute to venous dilation 4

Clinical Presentation

Key clinical features include:

• Chronic pelvic pain that worsens with:
  • Prolonged standing
  • Sexual intercourse (dyspareunia)
  • Menstruation
  • Pregnancy 4
• Pain of variable intensity, often dull and aching
• Post-coital ache and discomfort
• Dysmenorrhea
• Bladder irritability and rectal discomfort
• Symptoms may worsen premenstrually 3
• External manifestations may include vulvar, perineal, or lower extremity varices 5

Diagnostic Approach

Imaging Studies

1. Ultrasound with Doppler (first-line imaging):

   • Can document engorged periuterine and periovarian veins (>8mm)
   • Evaluates for low-velocity flow and altered flow with Valsalva maneuver
   • Assesses for retrograde (caudal) flow of ovarian veins
   • Can identify direct connections between engorged pelvic veins and myometrial arcuate veins 1

2. MRI/MR Angiography:

   • Problem-solving examination when ultrasound is inconclusive
   • Diagnostic performance comparable to conventional venography
   • T2-weighted imaging can demonstrate pelvic varices
   • Time-resolved postcontrast T1-weighted imaging can directly demonstrate ovarian vein reflux 1

3. CT with contrast:

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

4. Venography:

   • Gold standard for definitive diagnosis
   • Usually performed immediately before potential intervention 3

Management Approaches

Conservative Management (First-Line)

• Venoactive agents: Improve venous tone and reduce congestion symptoms 2
• Ergot alkaloid derivatives: Reduce venous congestion through vasoconstriction 2
• Pain management: Amitriptyline starting at low doses (10mg) for chronic pain 2
• Compression garments: Help reduce pelvic venous pressure when standing 2
• Lifestyle modifications:
  • Regular low-impact exercise to improve circulation
  • Weight management
  • Avoiding prolonged standing
  • Dietary modifications 2

Interventional Treatment

For patients with persistent symptoms despite conservative management:

• Ovarian vein embolization:

  • Most frequently cited treatment for pelvic venous disease
  • Technical success rates of 96-100%
  • Provides symptomatic relief in 70-90% of cases
  • Low complication rates (transient pain common, <2% risk of coil migration) 1, 3

• Internal iliac vein embolization:

  • Often performed in addition to ovarian vein embolization
  • Safe and effective in treating pelvic venous insufficiency 1

• Left renal vein stenting:

  • For cases related to nutcracker syndrome
  • Less invasive alternative to open surgical approaches 1

Surgical Options

• Laparoscopic ovarian vein ligation:

  • Alternative to embolization
  • Complete resolution of symptoms reported in selected cases 1, 6

• Left renal vein surgery:

  • Bypass, transposition, or external stent placement
  • Being replaced by endovascular approaches due to lower morbidity 1

Important Clinical Considerations

• PCS is frequently underdiagnosed, accounting for up to 30% of patients with chronic pelvic pain 5
• Incompetent and dilated pelvic veins can be found in asymptomatic women, making accurate diagnosis challenging 4
• Dyspareunia may indicate a poor prognostic factor, suggesting other causes of pelvic pain may coexist with pelvic varicosities 7
• Symptoms often subside after menopause due to decreased estrogen levels 1
• A multidisciplinary approach involving gynecologists, vascular specialists, and pain specialists is essential for proper diagnosis and management 3

Differential Diagnosis

Other causes of chronic pelvic pain that should be ruled out include:

• Chronic pelvic inflammatory disease
• Endometriosis
• Adhesive disease
• Hydrosalpinx or pyosalpinx
• Vulvovaginal candidiasis
• Musculoskeletal disorders 1