What are the CT (Computed Tomography) findings for pelvic congestion syndrome in patients with Ehlers-Danlos syndrome?

CT Findings for Pelvic Congestion Syndrome in Patients with Ehlers-Danlos Syndrome

Contrast-enhanced CT of the abdomen and pelvis is the most appropriate imaging modality for evaluating pelvic congestion syndrome in patients with Ehlers-Danlos syndrome, which may demonstrate engorged periuterine and periovarian veins, venous anatomic variants, and occasional compression of the left renal vein resulting in asymmetric left-sided pelvic varicosities. 1

Specific CT Findings in Pelvic Congestion Syndrome

When evaluating patients with Ehlers-Danlos syndrome (EDS) for pelvic congestion syndrome, CT imaging typically reveals:

• Engorged periuterine and periovarian veins (typically ≥8 mm in diameter) 1
• Venous anatomic variants that may contribute to venous congestion 1
• Compression of the left renal vein (nutcracker phenomenon) resulting in asymmetric left-sided pelvic varicosities 1
• Dilated arcuate veins within the myometrium 1

Special Considerations for Ehlers-Danlos Syndrome

Patients with vascular Ehlers-Danlos syndrome require particular attention during imaging and management due to:

• Increased vascular fragility that may lead to spontaneous vessel rupture even without significant dilation 1
• Arterial and venous tortuosity that may be more pronounced than in the general population 1
• Risk of vascular complications during invasive procedures 1
• Potential for multiple vascular abnormalities throughout the body 1

Imaging Protocol Recommendations

For optimal evaluation of pelvic congestion in EDS patients:

1. Use contrast-enhanced CT of the abdomen and pelvis as the primary imaging modality 1
2. Consider including the entire abdomen in the scan to evaluate the ovarian vein drainage into the renal vein or vena cava 1
3. Obtain multiplanar reformations to better visualize vascular anatomy 1
4. Consider low-radiation alternatives when available, especially for young patients requiring repeated imaging 1

Limitations of CT for Pelvic Congestion Syndrome

It's important to note that CT has certain limitations:

• CT lacks the capacity to provide dynamic flow information compared to ultrasound or MRI 1
• Cannot directly demonstrate altered flow with Valsalva maneuver or retrograde (caudal) flow of the ovarian veins 1
• May not detect subtle venous abnormalities in early disease 1

Alternative Imaging Modalities

While CT is valuable, other imaging modalities may provide complementary information:

• MRI/MR angiography: Provides superior assessment of flow dynamics and is comparable to conventional venography for identifying pelvic venous disorders 1
• Doppler ultrasound: Can document flow alterations with Valsalva maneuver and retrograde flow 1
• Venography: Remains the gold standard but is typically reserved for cases requiring intervention 2

Clinical Correlation

The diagnosis of pelvic congestion syndrome in EDS patients should correlate imaging findings with clinical symptoms:

• Chronic pelvic pain exacerbated by prolonged standing 2, 3
• Pain worsening during menstruation 3
• Post-coital ache and dyspareunia 2
• Symptoms often more pronounced in multiparous women 4, 5

Monitoring and Follow-up

For patients with vascular EDS and pelvic congestion syndrome:

• Annual surveillance imaging is recommended to monitor any dilated or dissected vascular segments 1
• More frequent imaging may be warranted with rapid progression of symptoms or vascular changes 1
• Imaging every 2 years may be appropriate when initial imaging is normal 1

Pitfalls and Caveats

• Dilated pelvic veins may be present in asymptomatic women (12% overall, 21% in premenopausal women) 5, 3
• Vascular abnormalities in EDS may rupture even without significant dilation, requiring careful monitoring 1
• Invasive procedures carry increased risk in vascular EDS due to tissue fragility 1
• Avoid arterial puncture when possible; noninvasive vascular imaging is preferred 1

By understanding these specific CT findings and considerations in the context of Ehlers-Danlos syndrome, clinicians can better identify and manage pelvic congestion syndrome in this high-risk population.