What is Pelvic Congestion Syndrome?

Pelvic Congestion Syndrome (PCS)

Pelvic Congestion Syndrome is a condition characterized by chronic pelvic pain caused by dilated and incompetent pelvic veins, primarily affecting premenopausal women, with diagnosis primarily made through Doppler ultrasound and treatment centered on ovarian vein embolization when conservative measures fail. 1

Definition and Clinical Features

Pelvic Congestion Syndrome is characterized by:

• Chronic pelvic pain lasting more than six months without evidence of inflammatory disease 2
• Pain that worsens with:
  • Prolonged standing
  • Sexual intercourse (dyspareunia)
  • Menstruation
  • Pregnancy 1, 3
• Associated symptoms include:
  • Post-coital ache
  • Dysmenorrhea
  • Bladder irritability
  • Rectal discomfort 2
  • Vulvar varices that may communicate with the saphenous vein 4

Pathophysiology

The exact etiology remains uncertain but is likely multifactorial:

• Valvular insufficiency in pelvic veins
• Venous obstruction
• Hormonal influences 3
• Most commonly affects multiparous women 2

Diagnosis

The American College of Radiology recommends a stepwise diagnostic approach:

1. First-line: Ultrasound with Doppler to document:

   • Engorged periuterine and periovarian veins (>8mm)
   • Low-velocity flow
   • Altered flow with Valsalva maneuver
   • Retrograde (caudal) flow of ovarian veins 1

2. Additional diagnostic modalities:

   • CT with contrast
   • MRI/MR Angiography
   • Venography with IVUS (considered gold standard) 1, 2

Important caveat: Incompetent and dilated pelvic veins are a common finding in asymptomatic women, making it challenging to determine which patients have chronic pelvic pain specifically related to PCS 3

Differential Diagnosis

It's essential to rule out other causes of chronic pelvic pain:

• Chronic pelvic inflammatory disease
• Endometriosis
• Adhesive disease
• Hydrosalpinx or pyosalpinx
• Interstitial cystitis/bladder pain syndrome
• Musculoskeletal disorders 1

Treatment Algorithm

1. First-Line: Conservative Management

• Venoactive agents
• Ergot alkaloid derivatives
• Compression garments 1
• Lifestyle modifications:
  • Avoiding prolonged standing
  • Regular exercise
  • Weight management
  • Application of heat or cold over painful areas
  • Pelvic floor muscle relaxation techniques 1

2. Second-Line: Pharmacological Options

• Amitriptyline
• Cimetidine (use with caution and under medical supervision) 1

3. Third-Line: Interventional Treatment

• Ovarian vein embolization (OVE):

  • Technical success rates: 96-100%
  • Symptomatic relief: 70-90% of cases 1, 2
  • Longest reported follow-up is five years 5
  • Potential complications include coil migration 5

• Other interventional options:

  • Internal iliac vein embolization
  • Left renal vein stenting for cases related to nutcracker syndrome 1

4. Surgical Options (Less Common Now)

• Hysterectomy combined with oophorectomy
• Open surgical ligation of ovarian veins
• Laparoscopic vein ligation 5, 4

Management Considerations

• Multidisciplinary approach involving gynecologists, pain specialists, and interventional radiologists is recommended for complex cases 1, 2

• Non-pharmacological interventions:

  • Cognitive behavioral therapy
  • Biofeedback therapy
  • Stress management practices (meditation, imagery) 1

• Dietary modifications:

  • Identifying and avoiding specific foods that worsen symptoms through an elimination diet approach 1

• Regular reassessment of treatment efficacy using validated tools:

  • Genitourinary pain index (GUPI)
  • Interstitial cystitis symptom index (ICSI)
  • Visual analog scale (VAS) 1

Important Clinical Considerations

• PCS is frequently misdiagnosed or overlooked as a cause of chronic pelvic pain 3, 2
• The presence of dyspareunia may indicate a poor prognosis, suggesting other coexisting causes of pelvic pain 6
• Under-diagnosis can lead to anxiety and depression 2
• Despite growing evidence supporting PCS as a clinical entity, the lack of prospective, multicenter randomized controlled trials remains a barrier to complete acceptance of the condition and its management 2