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An Unusual Cause of Pelvic Congestion Syndrome
VASCULAR DISEASE MANAGEMENT 2023;20(5):E88-E91
Abstract
Pelvic congestion syndrome (PCS) is widely thought to be due to ovarian or internal iliac vein reflux. We present a case where the symptoms of PCS resulted secondary to venous obstruction. A variant form of May-Thurner syndrome was identified on contrast-enhanced computed tomography, where the common trunk of the internal iliac veins was compressed by the right common iliac artery. The gonadal veins were dilated as an outflow tract. Stenting of the common trunk of the internal iliac veins was effective in managing PCS.
Introduction
Pelvic congestion syndrome (PCS) is common in multiparous women and is often overlooked and underdiagnosed.1 Dilated gonadal veins indicate venous reflux, which results in pelvic venous varicosities. Ovarian vein embolization is a well-established endovascular treatment for PCS.2,3
Case Report
A 31-year-old multiparous woman presented with chronic pelvic pain for 5 years with recent worsening. The pain was mostly on the left side with some left flank pain. She was diagnosed with PCS 5 months earlier and had undergone bilateral ovarian vein embolization. However, her pain scale had worsened from 6/10 to 8/10.
The patient’s previous contrast-enhanced computed tomography (CECT) scan was reviewed (Figure 1), showing duplication of the inferior vena cava (IVC). The left common iliac vein was continuing as the left IVC, which was communicating with the right IVC through retroperitoneal collaterals in the lumbar region. The left internal iliac vein (IIV) was joining the right IIV. The right IIV (a common trunk for both IIVs) was extrinsically compressed by the right common iliac artery (Figure 2). Both gonadal veins were dilated. Magnetic resonance imaging of the pelvis showed persistent flow voids in the parametrium and adnexa, consistent with pelvic venous congestion (Figure 1).
Endovascular treatment was planned. Under aseptic precautions, the right internal jugular vein was accessed and a 5F sheath was placed. Using a 0.035" guidewire and 5F multipurpose catheter, the left IIV was cannulated. The venogram (Figure 3) showed significant extrinsic compression stenosis in the junction of the common venous trunk, correlating with the CECT (Figure 2). Multiple venous collaterals were seen in the pelvis with stagnation of the contrast.
On-table intravascular ultrasound (IVUS) confirmed the findings (Figure 2). The diameter of the venous trunk was 13.4 mm caudal to the narrowing. There was a pressure gradient of 5 mm Hg across the narrowing. A 16 mm x 6 cm Wallstent endoprosthesis self-expanding stent (Boston Scientific) was deployed across the narrowed portion of the common venous trunk (Figure 4). The stent was opened with a 14 mm x 4 cm balloon. Check venogram showed a brisk antegrade flow. The pressure gradient had reduced to 2 mm Hg. The patient had an uneventful recovery and was asymptomatic on follow-up at 6 months.
Discussion
Patients with PCS usually present with otherwise unexplained chronic pelvic pain that has been present for more than 6 months, along with anatomic findings that include pelvic venous insufficiency and pelvic varicosities. It remains an underdiagnosed explanation for pelvic pain in young, premenopausal, usually multiparous females. Venography is usually necessary to confirm ovarian vein reflux. Embolization has been shown to be more effective than surgical therapy in improving symptoms in patients who fail hormonal therapy.1
CECT is the investigation tool of choice in diagnosing and planning treatment. The diagnostic criteria are dilated pelvic veins and gonadal veins measuring more than 5 mm.4,5 The reflux of the contrast during venography may not be apparent because patients are in a supine position during the procedure.6
Association of the anatomical variation, as in this patient, is extremely rare. In May-Thurner syndrome (MTS), patients present with left lower limb symptoms. Because the venous collaterals between the IIVs are present, the clinical manifestation of PCS may be less obvious because of MTS. In the index case, there was no compression over the left common iliac vein; instead, there was compression over the common venous trunk, draining both IIVs. This distinct entity may be called obstructive pelvic venous congestion syndrome for appropriate diagnosis and management.
Duplication of the IVC is an anatomical variation.7 Embryologically, it results when symmetrical pairs of the supracardinal veins do not fuse during the fourth to eighth week of gestation. An ascending interiliac communication between IIVs may be associated.8
The clinical presentation in this patient was similar to PCS, which is a common condition. However, the stagnation in the venous system was due to an obstruction to the pelvic venous outflow rather than venous reflux in the gonadal veins. The dilated gonadal veins represent an outflow tract. However, the principle of treatment is similar to treating MTS, with jugular venous access-facilitated venogram, IVUS, and stenting.
Adequate counseling is extremely important when dealing with such entities, as the literature regarding the outcome and long-term prognosis is lacking.
Conclusion
While evaluating clinically suspected PCS, anatomical variation in the pelvic venous anatomy should be looked for. Compression of bilateral IIV outflow tract, if present, may be effectively treated endovascularly by stenting. n
Disclosure: The authors have completed and returned the ICMJE Form for Disclosure of Potential Conflicts of Interest. The authors report no financial relationships or conflicts of interest regarding the content herein.
Manuscript accepted April 17, 2023.
Address for correspondence: Dr. Kumar Muthukumar, Christian Medical College, Room No. 1/13, X-Block, CMC Hospital Main Campus, Vellore, Tamil Nadu 632004, India. Email: drkmr1998@gmail.com
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