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Feedback on pelvic venous disorders

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"Original post, lightly edited for flow:"  

"My patient was a 37-year-old female, G0P0, with severe, debilitating chronic pelvic pain classic for pelvic venous disorder. Her pain was so severe that she was on disability and unable to work. 

Ultrasound imaging confirmed large left pelvic varices and CT demonstrated a significantly dilated left ovarian vein with extensive pelvic varices. CT also identified a retroaortic left renal vein, which raised concern for nutcracker physiology. The patient did not have flank pain or hematuria.

Diagnostic venogram performed with IVUS demonstrated a severely stenotic central left renal vein requiring prolonged wire and catheter manipulation to traverse what felt like a web or chronic changes. Nearly all of the left renal venous outflow was through a dilated left ovarian vein and pelvic plexus which received contribution from a large refluxing intraparenchymal renal vein. Simulated ovarian vein occlusion with a balloon catheter was performed and resulted in an increase in left renal vein pressure from 6 mmHg to 16 mmHg. 

Based on the venogram results, and after thorough discussion of all the options, I ultimately recommended surgical consultation and intervention. Surgery consultations offered splenorenal shunt creation, left renal auto transplant or nephrectomy for donation. The patient sought an additional web-based opinion from a vascular surgeon who recommended ovarian vein embolization. 

Due to confusion caused by the variety of opinions, this case was posted on SIR Connect for additional input from the IR community. Based on all of the gathered information, the patient ultimately chose to proceed with donor nephrectomy."

Thank you in advance.

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Author name

Allison Tan, MD

What is your general workup for patients referred to you for pelvic venous congestion? 

Many patients who are referred to me have already undergone extensive workup from our vulvovaginal specialist colleagues. Workup includes an extensive history and physical examination, including a pelvic exam by the patient’s gynecologist. Imaging studies help support the diagnosis and can include a pelvic ultrasound, CT or MRI, keeping in mind that negative imaging does not rule out a pelvic venous disorder. Venography is the gold standard to evaluate for the presence of ovarian vein reflux and has the benefit of being dynamic, allowing imaging with and without Valsalva maneuvers. However, due to the overlapping symptoms of pelvic venous disorders with other pelvic pathology, it is important to rule out other potential contributors to symptoms such as pelvic prolapse, genitourinary dysfunction and gastrointestinal issues.

Have you had experience with renal vein stenting in the setting of nutcracker or a retroaortic renal vein? If so, what are the important considerations and possible complications?

I personally have not had experience with renal vein stenting. Renal vein stent placement should be pursued with caution as it carries risks of stent malposition, thrombosis and migration.

In a case such as this, how important is it to obtain additional subspecialty opinions (transplant, vascular surgery, etc.) prior to treatment?

This case is an excellent example of a situation where multidisciplinary consultation is extremely important. The most important role of a physician is to educate the patient on all of the available treatment options to help them make the most informed decision possible. In this disease process, where there is no consensus on optimal treatment and possible treatments span specialties, I encourage patients to consult with different specialists to fully understand what each treatment entails, including the risks and benefits. Additionally, if you proceed with percutaneous management and have an anticipated complication that requires salvage from another specialist, it is prudent to have that specialist informed and on board before you proceed with your intervention in order to limit the time to transition of care.

What specifically prompted you to reach out regarding this case/topic?

This patient had sought multiple opinions and, as is common in this disease process, received differing recommendations that caused more confusion than clarity. I offered to cull opinions from the broader IR community through SIR Connect to see if we could find a dominant recommendation to help guide her decision-making.

What ended up being the outcome for this patient? Is there anything you would do differently in retrospect?   

After evaluating all of the options, this patient ultimately decided that surgical donor nephrectomy was the right decision for her. I do not think I would have done anything differently in this case. I discussed every option with her including the risks, benefits and unknowns surrounding the outcomes of each, and she ultimately made her own, fully educated decision. She trusted me to be honest and have her best interest in mind, not my own.

What post or posts were most valuable to you and why?  

I love the “how-I-do-it" posts on SIR Connect. When you train and work within the same system, as I have, exposure to different techniques is limited. SIR Connect reminds us that there are many ways to approach a problem. The SIR Connect forum provides an important setting for less formal and more fluid learning and exchange of ideas to occur.

Will you, or have you, changed your practice patterns based on responses on SIR Connect? Please describe any changes you are considering. 

Although I have not changed any major practice patterns based on SIR Connect threads, I do feel I have expanded my armamentarium based on the insights of my colleagues across the country.

Additional commentary:

It has been reported that approximately 14.7% of U.S. women of reproductive age will experience chronic pelvic pain (CPP).1 One of the frequently underdiagnosed etiologies of CPP are pelvic venous disorders (PeVDs). PeVDs in women can have variable presentations including chronic pelvic pain, lower extremity pain and swelling, flank pain, hematuria, and lower extremity/vulvar varicosities. In the past, a variety of terminology such as pelvic venous congestion, May-Thurner syndrome, nutcracker syndrome, etc. have led to imprecise patient diagnosis due to underlying pathophysiology or overlapping signs and symptoms. This in turn has led to confusion surrounding PeVD resulting in diagnostic error and suboptimal outcomes.2 Active efforts are underway to improve the definition of PeVD, disease classification, optimal diagnostic workup and optimization of endovascular treatment.

The Symptoms-Varices-Pathophysiology (SVP) classification system aims to define groups of patients with similar clinical characteristics of PeVD. There are three domains described in SVP including symptoms, varices and pathophysiology, including subclassification of pathophysiology into anatomic (A), hemodynamic (H) and etiologic (E) features of patient disease PVDAHE.3 The symptoms and varices are then broken down into what anatomic zone they involve as seen in Figure 1. For example, a patient with primary left ovarian vein reflux is S2 V2 PLGV, R, NT (left gonadal vein, reflux, nonthrombotic). Having a standardized classification system will allow for homogeneous patient populations for upcoming disease-specific clinical trials targeted at improving clinical decision-making.

When working up CPP, it is important to do a thorough history and physical, which likely includes high-level external gynecological assessment, as there is a broad differential diagnosis for CPP that can include multifactorial components.4 Often the first imaging test obtained in a woman with pelvic pain is a pelvic ultrasound, which may show periovarian and para-uterine varicosities. Additional cross-sectional imaging is often obtained to look for pelvic varices >5 mm or ovarian veins measuring >6 mm due to its association with reflux leading to pelvic venous hypertension.5 Diagnostic venography remains the gold standard approach in evaluating for venous reflux and measurement of pressure gradients.

The pathophysiology of PeVD is complex, and the relevant venous anatomy has many interconnections and anastomosis which leads to a broad spectrum of presenting symptoms. Often the pelvic veins can be thought of as “blood reservoirs” divided into the left renal vein, the superficial and deep veins of lower extremity and the parietal and visceral veins of the pelvis as seen in Figure 1. PeVD is secondary to venous incompetence leading to distension of a venous reservoir and venous hypertension. This activates nociceptors generating pain and generally occurs in the ovarian or internal iliac tributary veins. There are several venous “escape points” or communications between the pelvic veins and the superficial veins of the legs which can lead to vulvar and lower extremity varicosities. Symptom type and location depend on whether the distal reservoir affected has compensated or uncompensated physiology. For example, uncompensated reflux in the left ovarian vein will be transmitted to pelvic distal reservoir and can cause CPP. However, if compensation occurs via flow through “pelvic escape points” this may result in no CPP and instead pelvic origin vulvar and lower extremity varicosities.2,5,6,7,8

Ovarian vein and internal iliac vein embolization have been shown to be safe and effective for management of CPP. A large systematic review of 762 patients with CPP who underwent embolization and/or sclerosis of the internal iliac or ovarian veins reported symptomatic improvement in 697 (91.4%) of patients.9 Nonthrombotic iliac vein lesion (NIVL) can be associated with CPP and classically involves compression/occlusion of the left iliac vein between right iliac artery and the lumbar vertebrae. In a 2015 retrospective study reviewing outcomes of 18 patients with NIVL and 1 with suprarenal IVC stenosis, all who were treated with venous stenting, 15 of the 19 patients had complete resolution of their CPP.10 A similar retrospective study of 18 patients treated with stents for left renal vein stenosis or compression resulted in nine patients with complete resolution of symptoms and four patients with improvements of symptoms. The most common presenting symptoms were hematuria and flank pain. There was no stent migration and 2-year primary patency was 85%.11 The majority of the data regarding PeVD and embolization is retrospective in nature. Moving forward, randomized controlled trials are necessary to support existing data and aid in development of diagnostic/treatment algorithms. In summary, CPP secondary to PeVD is a complex process with varying presentations, symptoms and often confusing terminology which results in diagnostic errors. Although we have recently made great strides, continued work on imaging/diagnostic criteria, treatment algorithms and classification of disease is required.

References:

  1. Latthe P, Latthe M, Say L, et al. WHO systematic review of prevalence of chronic pelvic pain: a neglected reproductive health morbidity. BMC Public Health. 2006;6:177.
  2. Khilnani NM, Meissner MH, Learman LA, Gibson KD, Daniels JP, Winokur RS, et al. Research priorities in pelvic venous disorders in women: Recommendations from a multidisciplinary research consensus panel. J Vasc Interv Radiol. 2019;30:781–9.
  3. Meissner MH, Khilnani NM, Labropoulos N, Gasparis AP, Gibson K, Greiner M, Learman LA, Atashroo D, Lurie F, Passman MA, Basile A, Lazarshvilli Z, Lohr J, Kim MD, Nicolini PH, Pabon-Ramos WM, Rosenblatt M. The Symptoms-Varices-Pathophysiology classification of pelvic venous disorders: A report of the American Vein & Lymphatic Society International Working Group on Pelvic Venous Disorders. J Vasc Surg Venous Lymphat Disord. 2021 May;9(3):568–584. doi: 10.1016/j.jvsv.2020.12.084. Epub 2021 Jan 30. PMID: 33529720.
  4. Knuttinen MG, Machan L, Khilnani NM, Louie M, Caridi TM, Gupta R, Winokur RS. Diagnosis and management of pelvic venous disorders: AJR Expert Panel Narrative Review. AJR Am J Roentgenol. 2023 Nov;221(5):565–574. doi: 10.2214/AJR.22.28796. Epub 2023 Apr 5. PMID: 37095667.
  5. Steege JF, Siedhoff MT. Chronic pelvic pain. Obstet Gynecol 2014; 124:616–629.
  6.  Steenbeek M, van Der Vleuten CJM, Schultze Kool LJ, et al. Noninvasive diagnostic tools for pelvic congestion syndrome: a systematic review. Acta Obstet Gyenecol Scand. 2018;97:776–786.
  7. Kachlik D, Pechacek V, Musil V, et al. The venous system of the pelvis: New nomenclature. Phlebology. 2010;25:162–173.
  8. Greiner M, Dadon M, Lemasle P, et al. How does the pathophysiology influence the treatment of pelvic congestion syndrome and is the result long-lasting? Phlebology 2012;27(Suppl 1):58–64.
  9. Brown CL, Rizer M, Alexander R, Sharpe EE 3rd, Rochon PJ. Pelvic congestion syndrome: Systematic review of treatment success. Semin Intervent Radiol.2018; 35:35–40.
  10. Daugherty SF, Gillespie DL. Venous angioplasty and stenting improve pelvic congestion syndrome caused by venous outflow obstruction. J Vasc Surg Venous Lymphat Disord. 2015; 3:283–289.
  11. Avgerinos ED, Saadeddin Z, Humar R, Salem K, Singh M, Hager E, Makaroun M, Chaer RA. Outcomes of left renal vein stenting in patients with nutcracker syndrome. J Vasc Surg Venous Lymphat Disord. 2019 Nov;7(6):853–859. doi: 10.1016/j.jvsv.2019.06.016. Epub 2019 Aug 27. PMID: 31471277.

Disclaimer: This column represents the work and opinions of the contributing authors and do not necessarily reflect the views or policies of SIR. SIR assumes no liability, legal, financial or otherwise, for the accuracy of information in this article or the manner in which it is used. The statements made in the column are not intended to set a standard of care and should not be treated as medical advice nor as a substitute for independent, professional judgment. Please note: Due to recent law changes, certain procedures detailed within this article may not be legally permitted in your state. Please be sure to confer with your institution’s legal advisers.