Skip to main content

A rare case of left inferior vena cava presenting with May-Thurner syndrome

Abstract

Background

May–Thurner anomaly is characterized as external venous compression by the arterial framework against hard bony structures. This chronic anatomical lesion infrequently leads to deep vein thrombosis in the lower extremity, and it may lead to leg swelling as a long-term post-thrombotic complication. Left iliac vein compression may not be as uncommon as was previously thought, and it typically occurs in women more than men. Congenital anomalies of venous tree are not rare; they exist in 8.7% of the general population.

Case-presentation

We herein present the first case of right-sided May Thurner Syndrome in a patient with IVC anomalies. In our patient, both common iliac veins formed the left-sided IVC, which extended to the hemiazygos vein and the superior vena cava. Additionally, there was a right-sided suprarenal IVC, which extended to the right atrium.

Conclusion

Understanding the proper anatomy in May-Thurner syndrome helps in better decision making for management of disease pathophysiology.

Peer Review reports

Introduction

May–Thurner anomaly is characterized as external venous compression by the arterial framework against hard constructions (Dogan and Boke 2005; Kibbe et al. 2004). This starts a confined central stenosis of the common iliac vein. Since compression in most scenarios happens against lower lumbar vertebrae, MTS should be suspected in patients with scoliosis or widened periumbilical veins (Steinberg and Jacocks 1993a; Moudgill et al. 2009). This chronic anatomical lesion infrequently leads to deep vein thrombosis (DVT) in the lower extremity, and it may result in leg swelling as a long-term post-thrombotic complication (Moudgill et al. 2009; Oteros Fernandez et al. 2008; Murphy et al. 2009; Patel et al. 2000). Frequently, iliac vein compression is left-sided; nonetheless, in some infrequent presentations, it involves the right-sided venous structures (Spentzouris et al. 2014; Ang et al. 2013). Congenital anomalies of the venous tree are not rare in that they exist in 8.7% of the general population (Truty and Bower 2007). It is particularly imperative to detect an IVC anomaly before vascular procedures or other interventional strategies because sudden entanglements can emerge (Mano et al. 2004).

Herein, we present the first case of MTS in a patient with IVC anomalies. In our patient, both common iliac veins formed the left-sided IVC, which extended to the hemiazygos vein and the superior vena cava (SVC). There was also a small right suprarenal IVC in the normal position with 1 retroaortic right renal vein. These anomalies led to an atypical MTS and compression in the right-sided venous system which crossed to the left and into the IVC.

Case presentation

The patient was a 43-year-old woman with recurrent but non-documented right leg DVT during past 13 years. She used intermittent oral anticoagulation some years after first episode of her DVT and she complained of worsening right leg swelling, pain, formation of new varicose veins, and fatigue.

She had no specific medical or surgical illness and she have had 2 successful and uneventful pregnancies with normal vaginal delivery in spite of poor compliance to medical surveillance and lack of anticoagulant regimen during pregnancy period.

The patient was referred to our endovascular clinic for further assessment. In her physical examination, the right lower extremity was edematous with some hyperpigmentation and.

telangiectasia on her right leg. Additionally, prominent varicose veins were noticed on the right side. There were no active or healed ulcers on either lower extremity. Other cardiovascular examinations were unremarkable especially in terms of right-sided heart failure. The Villalta score for the further assessment of post-thrombotic syndrome based on its latest variables was 12, indicating a moderate post-thrombotic syndrome score (Kahn 2009; Kahn et al. 2009).

The patient underwent a Doppler ultrasonography of both lower extremities, which revealed chronic post-thrombotic changes in the deep venous system with evidence of blunted pulse-Doppler waves of the right-sided veins. For detailed imaging of these findings, we had a computed tomography (CT) venography of both lower extremities depicted (Fig. 1).

Fig. 1
figure 1

The image A illustrates the schematic anatomy of the patient’s IVC and BC are reconstructed images of patient’s CT venography, IVC, Inferior vena cava; RA, Right atrium; RV, Right ventricle; SVC, Superior vena cava

Unpredictably, the reconstructed image showed chronic post-thrombotic changes in the right lower extremity including right femoro-popliteal veins with the formation of a left-sided IVC after the union of both iliac veins, which extended to the hemiazygos vein. As the patient’s symptoms persisted enduringly, we decided to perform venography to determine the precise pathology of these changes. Preprocedural cardiac evaluations showed no abnormal echocardiographic or electrocardiographic findings. As is shown in Fig. 2, the lower extremity venous system after the iliac veins formed the left-sided IVC. Chiming in with the CT venography findings, the left-sided IVC extended to the hemiazygos vein and terminated at the SVC. On the other side, there was a suprarenal right-sided IVC, which joined the hepatic venous system and finally drained into the right atrium. There was also some evidence of compression on the right iliac vein and the proximal part of the left-sided IVC, suggesting the atypical MTS as the main cause of the patient’s chronic symptoms. We decided to perform balloon venoplasty for symptom relief and we deferred the stenting for next session if the patient symptoms does not relieve with venoplasty and compression therapy. The final venography demonstrated the acceptable outflow of both IVCs. The balloon venoplasty of the compressed venous system improved our patient’s venous hypertension as was reflected by improvements in both her subjective symptoms and her Villalta score. The patient was discharged the following day with oral anticoagulants, and she was asked to return for a follow-up the next month for the final approach like stenting if her symptoms persist.

Fig. 2
figure 2

The images depict the patient’s venography (upper images) and venoplasty (lower images). In figure “A” the arrow shows compression on right iliac vein and in “B” the IVC anomaly is imaged and in “C” the wire path shows the connection of left sided IVC with hemiazygus vein and in “D” we perfomed a prolonged balloon inflation and “E” demonstrates the final acceptable angioplasty result

Discussion

In the most common variant of left iliac vein compression as a venous anomaly, it is mostly asymptomatic and because of this innocent presentation, it is usually underdiagnosed. In its symptomatic type, hypertension in the left lower extremity venous system occurs because of compression by the overriding iliac artery with or without left iliofemoral DVT (Suwanabol et al. 2010). Manifestations include left leg swelling, pain, and skin manifestations of venous stasis or thrombosis due to the mechanical venous obstruction of the left iliac vein by the right iliac artery and the underlying vertebral body. Such manifestations occur secondary to intimal hyperplasia from the repeated trauma, leading to venous narrowing due to intraluminal webs and fibrosis (Kibbe et al. 2004). MTS is mostly asymptomatic, which renders an accurate incidence estimation of this anomaly challenging. MTS may not be as uncommon as was previously thought, and it typically occurs in women more than men (Kim and Porter 1992).

MTS or iliac vein compression syndrome (IVCS) is not always simple compression, but sometimes it constitutes additional evidence of venous scarring and associated vessel changes, leading to symptoms of persistent venous hypertension (Steinberg and Jacocks 1993b). The clinical presentation consists of signs and symptoms of acute (52%) or chronic DVT, and the findings are often attributable to venous hypertension. The most prevalent findings are edema (90%), venous dilations (33.5%), persistent discomfort (89.7%), and recurring ulcers (5.1%). PTS (symptoms and signs of chronic venous insufficiency) is a result of the extension of the MTS sequeale and the postponement of its invasive treatment, since medical therapy alone is mostly insufficient for long-term maintenance (Hulsberg et al. 2016).

Various variants of MTS have been reported in the literature as atypical forms of this syndrome: compression of the left common iliac vein by the left internal iliac artery, compression of the right common iliac vein by the right internal and right common iliac arteries, and compression of the IVC by the right common iliac artery (Molloy et al. 2002; Fretz and Binkert 2010).

Kim et al. (Kim et al. 2018) were first to present a unique congenital anomaly of the IVC in which a left interrupted IVC extended to the hemiazygos vein and then the SVC before it drained into the right atrium. They described the anomaly, in which case both common iliac veins formed the left-sided IVC. Another component of the anomaly was a small right suprarenal IVC in the normal position. One retroaortic right renal vein formed the communicating point between the larger left IVC and the smaller right suprarenal IVC. Notably, the reported rare double IVC anomaly by Kim and colleagues had no more pathology and disease; however, unfortunately, our patient developed an unusual and atypical form of IVCS. Her right iliac vein was compressed between the left iliac artery and the spine, and she experienced mild-to-moderate post-thrombotic symptoms after some episodes of DVT.

The stenting of MTS is deemed safe, efficacious, and durable in both post-thrombotic patients and those treated for edema alone (Oteros Fernandez et al. 2008; Hager et al. 2013). Because stenting for MTS has been shown to have low morbidity and good patency with relief of symptoms, any patient with symptoms and identifiable iliac vein compression should be considered for stenting. The decision for intervention should be driven primarily by symptoms of venous outflow obstruction and guidance by history and physical examination. History of DVT may also increase the clinical indication for stenting (Salahuddin and Armstrong 2020; Kalantar et al. 2019). The balloon venoplasty of the compressed venous system improved our patient’s venous hypertension as was reflected by improvements in both her subjective symptoms and her Villalta score. We decided to follow our patient next weeks to assess angioplasty alone result. The mild nature of post-thrombotic symptoms in our patient based on the Villalta score prompted us to manage her without stenting and reserve the more definitive endovascular approach if the symptoms persisted or worsened (Kahn et al. 2009).

Conclusion

Understanding proper anatomy in May-Thurner syndrome as a frequent venous disease is fundamental. In our case, a very rare variation accompanied the patient’s symptoms and it was obviated by balloon angioplasty. Also these patients need a careful surveillance to diagnose future symptoms earlier.

Availability of data and materials

All data are available if need for publication.

Abbreviations

MTS:

May-Thurner syndrome

IVC:

Inferior vena cava

DVT:

Deep vein thrombosis

SVC:

Superior vena cava

CT:

Computed tomography

RA:

Right atrium

RV:

Right ventricle

SVC:

Superior vena cava

IVCS:

Iliac vein compression syndrome

References

  • Ang WC, Doyle T, Stringer MD (2013) Left-sided and duplicate inferior vena cava: a case series and review. Clin Anat 26:990–1001

    Article  Google Scholar 

  • Baron HC, Shams J, Wayne M (2000) Iliac vein compression syndrome: a new method of treatment. Am Surg 66:653–655

    CAS  PubMed  Google Scholar 

  • Cockett FB, Thomas ML, Negus D (1967) Iliac vein compression. eIts relation to iliofemoral thrombosis and the postthrombotic syndrome. Br Med J 2:14e9

    Article  Google Scholar 

  • Dogan OF, Boke E (2005) Three cases with may-Thurner syndrome: a possibly under-reported disorder. Vasa 34:147e51

    Article  Google Scholar 

  • Fretz V, Binkert CA (2010) Compression of the inferior vena cava by the right iliac artery: a rare variant of may-Thurner syndrome. Cardiovasc Intervent Radiol 33(5):1060–1063. https://doi.org/10.1007/s00270-009-9671-y Epub 2009 Aug 13. PMID: 19680718

    CAS  Article  PubMed  Google Scholar 

  • Hager E, You T, Tahara R, Dillavou E, Al-Khoury G, Marone L, Makaroun M, Chaer R (2013) Outcomes of endovascular intervention for MayThurner syndrome. J Vasc Surg 1:270–275

    PubMed  Google Scholar 

  • Hulsberg PC, McLoney E, Partovi S, Davidson JC, Patel IJ (2016) Minimally invasive treatments for venous compression syndromes. Cardiovasc Diagn Ther 6:582–592

    Article  Google Scholar 

  • Kahn SR (2009) Measurement properties of the Villalta scale to define and classify the severity of the post-thrombotic syndrome. J Thromb Haemost 7(5):884–888

    CAS  Article  Google Scholar 

  • Kahn SR, Partsch H, Vedantham S, Prandoni P, Kearon C (2009) Subcommittee on control of anticoagulation of the scientific and standardization Committee of the International Society on thrombosis and Haemostasis. Definition of post-thrombotic syndrome of the leg for use in clinical investigations: a recommendation for standardization. J Thromb Haemost 7(5):879–883

    CAS  Article  Google Scholar 

  • Kalantar KR, Malek H, Amin A (2019) Chronic thromboembolic pulmonary hypertension versus fibrosing mediastinitis. Anatol J Cardiol 21(2):E4–E5. https://doi.org/10.14744/AnatolJCardiol.2018.12258

    Article  Google Scholar 

  • Kibbe MR, Ujiki M, Goodwin AL et al (2004) Iliac vein compression in an asymptomatic patient population. J Vasc Surg 39:937e43

    Article  Google Scholar 

  • Kim DOD, Porter DH (1992) Venographic anatomy, technique and interpretation. In: Kim DOD (ed) Peripheral vascular imaging and intervention. Mosby-Year Book, Saint Louis, pp 269–349

    Google Scholar 

  • Kim SS, Shin HC, Hwang JA, Jou SS, Lee WH, Choi SY, Park CH (2018) Various congenital anomalies of the inferior vena cava: review of cross-sectional imaging findings and report of a new variant. Abdom Radiol 43(8):2130–2149. https://doi.org/10.1007/s00261-017-1430-y PMID: 29264715

    Article  Google Scholar 

  • Mano A, Tatsumi T, Sakai H et al (2004) A case of deep venous thrombosis with a double inferior vena cava effectively treated by suprarenal filter implantation. Jpn Heart J 45(6):1063–1069

    Article  Google Scholar 

  • Molloy S, Jacob S, Buckenham T, Kham KT, Taylor RS (2002) Arterial compression of the right common iliac vein; an unusual anatomical variant. Cardiovasc Surg 10(3):291–2922

    CAS  Article  Google Scholar 

  • Moudgill N, Hager E, Gonsalves C et al (2009) May-Thurner syndrome: case report and review of the literature involving modern endovascular therapy. Vascular 17:330e5

    Article  Google Scholar 

  • Murphy EH, Davis CM, Journeycake JM et al (2009) Symptomatic ileofemoral DVT after onset of oral contraceptive use in women with previously undiagnosed may-Thurner syndrome. J Vasc Surg 49:697e703

    Google Scholar 

  • Oteros Fernandez R, Bravo Rodriguez F, Delgado Acosta F et al (2008) May-Thurner syndrome and surgery for scoliosis [in Spanish]. Radiologia 50:245e7

    Article  Google Scholar 

  • Patel NH, Stookey KR, Ketcham DB et al (2000) Endovascular management of acute extensive iliofemoral deep venous thrombosis caused by may-Thurner syndrome. J Vasc Interv Radiol 11:1297e302

    Google Scholar 

  • Salahuddin T, Armstrong EJ (2020) Intervention for Iliofemoral deep vein thrombosis and may-Thurner syndrome. Interv Cardiol Clin 9(2):243–254. https://doi.org/10.1016/j.iccl.2019.11.003 Epub 2020 Jan 30. PMID: 32147124

    Article  PubMed  Google Scholar 

  • Spentzouris G, Zandian A, Cesmebasi A et al (2014) The clinical anatomy of the inferior vena cava: a review of common congenital anomalies and considerations for clinicians. Clin Anat 27:1234–1243

    Article  Google Scholar 

  • Steinberg JB, Jacocks MA (1993a) May-Thurner syndrome: a previously unreported variant. Ann Vasc Surg 7:577e81

    Article  Google Scholar 

  • Steinberg JB, Jacocks MA (1993b) May-Thurner syndrome: a previously unreported variant. Ann Vasc Surg 7:577–581

    CAS  Article  Google Scholar 

  • Suwanabol PA, Tefera G, Schwarze ML (2010) Syndromes associated with the deep veins: phlegmasia Cerulea Dolens, may-Thurner syndrome, and nutcracker syndrome. Perspect Vasc Surg Endovasc Ther 22:223–230

    Article  Google Scholar 

  • Truty MJ, Bower TC (2007) Congenital anomalies of the inferior vena cava and left renal vein: implications during open abdominal aortic aneurysm reconstruction. Ann Vasc Surg 21:186–197

    Article  Google Scholar 

Download references

Acknowledgements

None.

Funding

none.

Author information

Authors and Affiliations

Authors

Contributions

JM: procedure, PS: data collection, BM: procedure, KR: CT assessment, EK: writing and editing. The author(s) read and approved the final manuscript.

Authors’ information

Cardiovascular intervention research center, Rajaie cardiovascular medical and research center, Iran university of medical sciences, Tehran, Iran.

Corresponding author

Correspondence to Ehsan Khalilipur.

Ethics declarations

Ethics approval and consent to participate

A consent form has been provided before any procedure and it was signed by the patient.

Consent for publication

A consent form has been provided before any procedure and it was signed by the patient.

Competing interests

None.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Moosavi, J., Sadeghipour, P., Mohebbi, B. et al. A rare case of left inferior vena cava presenting with May-Thurner syndrome. CVIR Endovasc 5, 31 (2022). https://doi.org/10.1186/s42155-022-00305-2

Download citation

  • Received:

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1186/s42155-022-00305-2