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CASE REPORT
Year : 2021  |  Volume : 18  |  Issue : 2  |  Page : 159-161

May–thurner syndrome in a 31-year-old female with chronic deep vein thrombosis: A case report and review of the literature


1 Department of Internal Medicine, Cardiology Unit, General Amadi Rimi Specialist Hospital, Katsina, Nigeria
2 Department of Radiology, General Amadi Rimi Specialist Hospital, Katsina, Nigeria

Date of Submission11-Oct-2020
Date of Decision02-Nov-2020
Date of Acceptance01-Dec-2020
Date of Web Publication10-Dec-2021

Correspondence Address:
Dr. Muhammad Nazir Shehu
Department of Internal Medicine, Cardiology Unit, General Amadi Rimi Specialist Hospital, Katsina
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/njbcs.njbcs_29_20

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  Abstract 


MayThurner syndrome (MTS) also known as iliocaval compression, occurs as a result of left common iliac vein compression by the right iliac artery. The syndrome is characterised by persistent leg swelling, venous ulcer, deep-vein thrombosis (DVT) and pulmonary embolism. However, it is rarely diagnosed with one of the causes of DVT. We hereby present a 31-year-old woman with persistent left lower limb DVT caused by MTS.

Keywords: Deep vein thrombosis, may-thuner syndrome, pulmonary embolism, third decade, women


How to cite this article:
Shehu MN, Saliu A. May–thurner syndrome in a 31-year-old female with chronic deep vein thrombosis: A case report and review of the literature. Niger J Basic Clin Sci 2021;18:159-61

How to cite this URL:
Shehu MN, Saliu A. May–thurner syndrome in a 31-year-old female with chronic deep vein thrombosis: A case report and review of the literature. Niger J Basic Clin Sci [serial online] 2021 [cited 2022 Jan 17];18:159-61. Available from: https://www.njbcs.net/text.asp?2021/18/2/159/332187




  Introduction Top


MayThurner syndrome (MTS) also known as iliocaval compression or iliac vein compression syndrome is caused by compression of the left common iliac vein by the right common iliac artery.[1],[2],[3] It occurs due to an anatomical variant in which the right common iliac artery crosses over and compresses the left common iliac vein.

The syndrome was first described in 1957 by May and Thurner after observing the fact that pelvic vein thrombosis is found five times more common over the left leg and in the majority of cases it affects the left common iliac vein.[1] The obstruction of the common iliac vein is said to occur due to trauma caused by arterial pulsation with subsequent collagen and elastin deposition.[4] The venous thrombus formation is aided by continued trauma and intimal proliferation.[5] Although the anatomical variant of MTS is said to be present in 20% of the population, MTS is rarely considered as one of the differentiavls in the diagnosis of deep vein thrombosis (DVT).[1] We, therefore, present a case of MTS complicated by DVT and pulmonary embolism (PE) in a 31-year-old woman.


  Case Report Top


A 31-year-old woman presented with 5 weeks history of left lower limb swelling and 3 days history of cough, chest pain and breathlessness. The patient was delivered a baby boy through spontaneous vaginal delivery 5 weeks before presentation. Left lower limb pitting pedal oedema to the thigh was documented. She was discovered to be tachycardic and tachypneic; with a heart rate of 120 beats/min and a respiratory rate of 52 cycles/min, respectively. In addition, she was found to be in mild respiratory distress, with SpO2 of 97% at room air.

Chest X-ray revealed left-sided pleural effusion and D-Dimer was raised (6 mg/L). Doppler ultrasound scan of the left lower limb showed left femoral vein thrombosis [Figure 1]a and [Figure 1]b. Computed tomographic (CT) – venography of the pelvis showed thrombosis of the left superficial femoral and left common iliac veins up to the point where it goes behind the right common iliac artery [Figure 2]a, [Figure 2]b, [Figure 2]c. The lumen of the left common iliac vein is narrowed and compressed at the point where it crossed under the right common iliac artery [Figure 2]d, this has confirmed the presence of the clinically suspected MTS. There was no evidence of proximal progression of the thrombus beyond this point. CT pulmonary angiography showed complete occlusion of a subsegmental pulmonary artery, with evidence of pulmonary infarction at the peripheral region of the basal left lung [Figure 3]a, [Figure 3]b, [Figure 3]c.
Figure 1: (a) Colour Doppler ultrasound image showing complete thrombosis of the left superficial femoral vein. (b) Colour Doppler ultrasound image showing thrombosis of the left common femoral vein. (c) Colour Doppler image of left superficial femoral vein, acquired 2 months later and shows evidence of recanalisation following treatment. (d) Spectral Doppler image of the superficial femoral vein, acquired 2 months later showing evidence of recanalisation following treatment

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Figure 2: (a-c) Axial computed tomographic images at the level of the proximal common femoral vessels showing compression of the left common iliac vein by the right common iliac artery. (d) Axial computed tomographic image showing thrombosis of the left common iliac vein which appears non-enhancing and smaller than the right counterpart

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Figure 3: (a) A is saggital contrast-enhanced chest computed tomographic that shows complete occlusion of a subsegmental pulmonary artery in the left lung (arrow) seen as a hypodense non-enhancing soft-tissue density mass within the mid and distal length of the vessel while its proximal portion is disproportionately dilated. (b and c) A triangular area (arrow) of non-enhancing pulmonary tissue in the peripheral region of the basal left lung into which the thrombosed vessel leads, in keeping with pulmonary infarction

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The patient was admitted into the medical ward and was managed by a multidisciplinary team of specialists. She did well on admission and repeated CT pulmonary angiography showed normal study after 3 weeks of management with dabigatran. However, repeat Doppler scan of the affected limb showed persistent DVT with only recanalisation of the affected vessel [Figure 1]c and ][Figure 1]d even after 16 weeks on anti-coagulant. The patient was eventually referred for an interventional procedure of clots removal, angioplasty and stent insertion. As at the time of writing this report, the patient was doing well 5 months post-diagnosis on dabigatran 150 mg PO BID and is preparing for the interventional procedure at the referral centre.


  Discussion Top


Based on autopsy findings, the incidence rate of MTS has been shown to range between 22% and 32%.[5] However, only 2%–3% of lower extremities deep venous thromboses are clinically related to MTS. This may not be unconnected to the missed diagnosis of MTS owing to the fact that 57% of the lower extremities DVT occur over the left side.[4] MTS is five times more common in females than in males.[4] Thus, most of the MTS patients are females, often use contraceptives or have a history of recent pregnancy.[4] Cases of DVT are often attributed to these additional risk factors, hence the reason for the missed diagnosis of MTS. Our patient started having symptoms at 34 weeks gestation and MTS was diagnosed after delivery. Delay in diagnosis usually halts further evaluation of the cause of DVT in the affected patients leading to recurrence and complications such as PE, iliac vein rupture with attending morbidity and mortality.

The clinical features of MTS usually occur among women in their second and third decades of life.[6] However, majority with anatomical variants of MTS do not develop symptoms.[6] Our patient first developed symptoms at the age of 31 years. Symptoms usually occur after significant narrowing of the left common iliac vein. Common symptoms include leg swelling, discoloration, ulcers and DVT. These clinical features may occur gradually or acutely over weeks and hours, respectively. Clinical features typically occur on the left lower limb and pelvis, however, occurrence over the right side has rarely been reported.[6] There might also be features of venous intermittent claudication over the thigh. Grievous complications include PE and rupture of the iliac vein. The patient presented in this report had left lower limb common iliac and femoral veins DVT, leg swelling and PE.

The diagnosis is made after clinical suspicion with Doppler ultrasound scan of the affected limb usually to detect absence or presence of DVT. CT scan or magnetic resonance imaging (MRI) can be used to visualise the compression of the veins and DVT.

Venography and intravenous ultrasound can be used to visualise the veins better than MRI or CT scans. The patient we presented had MTS confirmed by Doppler, USS, CT scan and venography. Treatment includes prolonged use of anticoagulant, angioplasty and stent insertion. Rarely open surgical thrombectomy with re-positioning of the right common iliac artery away from the left iliac vein can be offered. This surgical therapy is usually reserved for those with failure of angioplasty and stent insertion. Long-term anti-coagulation and elastic stocking compression are advocated following the interventional procedure.


  Conclusion Top


Although clinically symptomatic MTS is rare, it is important to always consider MTS as one of the differentials of lower extremities DVT, particularly in young females. This will prevent recurrence of DVT, PE as well as associated morbidity and mortality.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Acknowledgement

We highly appreciate the efforts and support of Abubakar Lawal and Aminu Ibrahim as well as the entire staff of the Department of Internal Medicine, General Amadi Rimi Specialist Hospital Katsina toward the successful completion of this study.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
May R, Thurner J. The cause of the predominantly sinistral occurrence of thrombosis of the pelvic veins. Angiology 1957;8:419-27.  Back to cited text no. 1
    
2.
Cockett FB, Thomas ML. The iliac compression syndrome. Br J Surg 1965;52:816-21.  Back to cited text no. 2
    
3.
Taheri SA, Williams J, Powell S, Cullen J, Peer R, Nowakowski P, et al. Iliocaval compression syndrome. Am J Surg 1987;154:169-72.  Back to cited text no. 3
    
4.
O'Sullivan GJ, Semba CP, Bittner CA, Kee ST, Razavi MK, Sze DY, et al. Endovascular management of iliac vein compression (May-Thurner) syndrome. J Vasc Interv Radiol 2000;11:823-36.  Back to cited text no. 4
    
5.
Shivani K, Payal S, Aparna N, Nwabundo N, Usman M, Nasir H. May-Thurner Syndrome: A case report and review of the literature. Hindawi Publ Corp 2013;2003:1-5.  Back to cited text no. 5
    
6.
Liddell RP, Evans NS. May-Thurner syndrome. Vasc Med 2018;23:493-6.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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