Role of Interventional Cardiologists in Lower Limb Swelling/DVT -Case Series on May-Thurner Syndrome and its Variants

Reddy, Natraj Setty, Srinivas, Raghu, Patil, Shankar, Kumar, Nagesh, and Manjunath: Role of Interventional Cardiologists in Lower Limb Swelling/DVT -Case Series on May-Thurner Syndrome and its Variants

Authors

BACKGROUND

May–Thurner syndrome is caused by compression of the left common iliac vein by the right common iliac artery, resulting in symptoms of venous obstruction, insufficiency, leg ulcers, varicosities and deep vein thrombosis. The incidence rate of May–Thurner syndrome is unknown and perhaps ranges from 22 to 32% according to the autopsy studies in the early twentieth century. However, May–Thurner syndrome related deep venous thrombosis accounts for only 2%-3% of all lower limb deep vein thrombosis. It is also known as iliac vein compression syndrome or Cockett’s syndrome.1 McMurrich initially reported the association of common iliac vein compression with an increased incidence of left lower deep vein thrombosis, thought to be congenital.2 Ehrich postulated acquired degenerative obstruction at the origin of the left common iliac vein.3 May and Thurner described compression of the left common iliac vein from the right common iliac artery, resulting in mural spur formation and deep vein thrombosis.4

CASE PROFILE

We report a case series of 9 women patients aged 20-40 years, of which 6 patients had left lower limb swelling and 3 patients had right lower limb swelling. The demographic characteristics of the patients are shown in Table 1. There was no history of smoking, bleeding disorders in the family, no history of connective tissue disorder, malignancy, hypercoagulable syndrome, trauma, surgery, travel, recurrent miscarriage, and no history of consumption of oral contraceptive pill, routine blood examinations were normal, chest X-ray is normal, coagulation profile includes protein C, protein S, antithrombin III, and antinuclear antibodies (ANA) were normal, comprehensive metabolic (Homocysteine, apo-lipoproteins, thyroid) panel, ECG, 2D Echocardiograph were within normal limits. On examination 6 patients had left lower limb swelling and 3 patients had right lower limb swelling. Lower extremity Doppler was significant for extensive thrombosis of left common femoral, Iliac, femoral, great saphenous, posterior tibial, popliteal, and peroneal vein as shown in Table 2. All the patients underwent peripheral angiogram which revealed compression of the left common iliac vein by the right common iliac artery in 6 patients and right common iliac vein compression by a right common iliac artery in 3 patients. 3 patients underwent catheter-directed thrombolysis with streptokinase with catheter laid across femoral and iliac veins with a closer monitoring of complete blood count and coagulation profile. Subsequently, 2 patients underwent ballooning angioplasty with good iliac and pelvic venous runoff and 6 patients underwent percutaneous transluminal angioplasty (PTA) with stenting and one patient had completely recanalized vessel after catheter-directed thrombolysis details shown in Table 2. All the patients were started with anti-coagulants (Dabigatran) for a period of 6 months because it is provoked deep vein thrombosis advised Compression stock and repeated venous Doppler study performed during follow up.

Table 1

The demographic characteristics of the patients.

Patient NoAgeSexPresenting complaintsH/o DM/HTNPrior IHD
I33FLeft lower limb DVT.NoNo
II40FLeft lower limb DVT.NoNo
III21FLeft lower limb DVT.NoNo
IV35FRight lower limb DVT.NoNo
V38FRight lower limb DVT.NoNo
VI28FRight lower limb DVT.NoNo
VII42FLeft lower limb DVT.NoNo
VIII31FLeft lower limb DVT.NoNo
IX40FLeft lower limb DVT.NoNo
Table 2

Patients interventional profiles.

Patient no.Peripheral angiographyLesion/ ThrombosisThrombolysisPredilatation with balloonRevascularization/StentPost dilatation with bollonResultsFigures
1ILIAC, FEMORAL AND POPLITEAL VEINLeft lower limb DVT.-8X80mm
Cuba balloon
Self Expanding Stent 14 * 120 Mm Wall Stent14*40 Balloonbrisk antegrade flow with a good run off in common iliac veinsFigure 1A: Peripheral Angiography showing Iliac and femoral vein occlusion.
https://s3-us-west-2.amazonaws.com/jourdata/jcdr/JCardVascDisRes-9-2-92_g000.jpg
Figure 1B: Peripheral Angiography showing Final result with a good runoff in common iliac veins.
https://s3-us-west-2.amazonaws.com/jourdata/jcdr/JCardVascDisRes-9-2-92_g001.jpg
2ILIAC AND FEMORAL VEINLeft lower limb DVT.CATHETER DIRECTED LYSIS6X40mm Cuba balloon9*60 Balloon Expandable Stent-brisk antegrade flow with a good runoff in common iliac veinsFigure 2A: Peripheral Angiography showing iliac, femoral and popliteal vein occlusion.
https://s3-us-west-2.amazonaws.com/jourdata/jcdr/JCardVascDisRes-9-2-92_g002.jpg
Figure 2B: Peripheral Angiography Showing final result with the antegrade flow and good runoff in common iliac veins.
https://s3-us-west-2.amazonaws.com/jourdata/jcdr/JCardVascDisRes-9-2-92_g003.jpg
3ILIACLeft lower limb DVT.CATHETER DIRECTED LYSIS16X30mm Cuba balloon16*30 Balloon Expandable Stent-good Pelvic venous runoffFigure 3A: Peripheral Angiography showing iliac vein occlusion.
https://s3-us-west-2.amazonaws.com/jourdata/jcdr/JCardVascDisRes-9-2-92_g004.jpg
Figure 3B: Peripheral Angiography showing final Results with good Pelvic venous runoff.
https://s3-us-west-2.amazonaws.com/jourdata/jcdr/JCardVascDisRes-9-2-92_g005.jpg
4ILIAC, POPLITEAL VEINRight lower limb DVT.CATHETER DIRECTED LYSIS---Good Pelvic venous runoffFigure 4A: Peripheral Angiography showing iliac and popliteal vein occlusion.
https://s3-us-west-2.amazonaws.com/jourdata/jcdr/JCardVascDisRes-9-2-92_g006.jpg
Figure 4B: Peripheral Angiography showing final Results good Pelvic venous runoff.
https://s3-us-west-2.amazonaws.com/jourdata/jcdr/JCardVascDisRes-9-2-92_g007.jpg
5ILIAC, FEMORAL AND POPLITEAL VEINRight lower limb DVT.-6X40mm Cuba Balloon18*90 Balloon Expandable Stent18*65 BalloonGood Pelvic venous runoffFigure 5A: Peripheral Angiography showing femoral and popliteal vein occlusion.
https://s3-us-west-2.amazonaws.com/jourdata/jcdr/JCardVascDisRes-9-2-92_g008.jpg
Figure 5B: Peripheral Angiography showing iliac, final results with the antegrade flow and good runoff in common iliac veins.
https://s3-us-west-2.amazonaws.com/jourdata/jcdr/JCardVascDisRes-9-2-92_g009.jpg
6ILIACRight lower limb DVT.-6X40mm Vatsmed Balloon18*90 Balloon Expandable Stent18*65 BalloonGood Pelvic venous runoffFigure 6A: Peripheral Angiography showing Iliac vein occlusion.
https://s3-us-west-2.amazonaws.com/jourdata/jcdr/JCardVascDisRes-9-2-92_g0010.jpg
Figure 6B: Peripheral Angiography showing Final result with good Iliac and pelvic venous runoff..
https://s3-us-west-2.amazonaws.com/jourdata/jcdr/JCardVascDisRes-9-2-92_g0011.jpg
7ILIACLeft lower limb DVT.-6X40mm Vatsmed Balloon--Good Iliac and pelvic venous runoffFigure 7A: Peripheral Angiography showing Iliac vein occlusion.
https://s3-us-west-2.amazonaws.com/jourdata/jcdr/JCardVascDisRes-9-2-92_g0012.jpg
Figure 7B: Peripheral Angiography showing Final result with good Iliac and pelvic venous runoff.
https://s3-us-west-2.amazonaws.com/jourdata/jcdr/JCardVascDisRes-9-2-92_g0013.jpg
8ILIACLeft lower limb DVT.-6X30mm Cuba Balloon--Good Iliac and pelvic venous runoffFigure 8A: Peripheral Angiography showing Iliac vein occlusion.
https://s3-us-west-2.amazonaws.com/jourdata/jcdr/JCardVascDisRes-9-2-92_g0014.jpg
Figure 8B: Peripheral Angiography showing Final result with good Iliac and pelvic venous runoff.
https://s3-us-west-2.amazonaws.com/jourdata/jcdr/JCardVascDisRes-9-2-92_g0015.jpg
9ILIACLeft lower limb DVT.-6X40mm Cuba Balloon16*60 Balloon Expandable Stent8*60 BalloonGood Iliac and pelvic venous runoffFigure 9A: Peripheral Angiography showing Iliac vein occlusion.
https://s3-us-west-2.amazonaws.com/jourdata/jcdr/JCardVascDisRes-9-2-92_g0016.jpg
Figure 9B: Peripheral Angiography showing Final result with good Iliac and pelvic venous runoff.
https://s3-us-west-2.amazonaws.com/jourdata/jcdr/JCardVascDisRes-9-2-92_g0017.jpg

DISCUSSION

May-Thurner syndrome was first described in 1957 when it was noted that 22% of 430 cadavers on autopsy possessed an anatomical variant in which an overriding right common iliac artery caused compression of the left common iliac vein against the lumbar spine. This compression is associated with intimal hyperplasia, which creates the potential for venous stasis and subsequently deep vein thrombosis.5 Left iliac vein compression is the most common variant seen in May-Thurner syndrome; however, several other variants have been described in the literature. Compression of the left common iliac vein by the left internal iliac artery,6 compression of the right common iliac vein by the right internal iliac artery,7 compression of the IVC by the right common iliac artery8 and right-sided May-Thurner syndrome in a patient with a left-sided IVC9 have all been described. Despite the relatively high incidence of this anatomical variation, the clinical prevalence of May-Thurner syndrome -related DVT is surprisingly low.10 It is thought that this low occurrence rate may be an underestimate of the actual prevalence due to missed diagnoses.11 Usually seen in females between the age group of 20–40 years, the trauma from chronic arterial pulsation of the right common iliac artery is believed to cause deposition of elastin and collagen in left common iliac vein resulting in a spur formation.5 Patients with May-Thurner syndrome usually present with acute or chronic unilateral left lower extremity swelling and pain. Some patients may present with skin pigmentation, chronic leg pain, and swelling, recurrent skin ulcers.12 Few cases of iliac vein rupture due to May-Thurner syndrome have also been reported.13 The clinical stages of iliac vein compression were described by Kim et al.14 and include Stage I, asymptomatic iliac vein compression; Stage II, development of a venous spur; Stage III, development of left iliac vein DVT. CT venography, MR venography, intravenous ultrasound, or conventional venography can be used to confirm the diagnosis of May-Thurner syndrome in the suspected cases. With simple doppler ultrasound, it is very difficult to find pathology in the iliac vessels though there have been few case reports where the diagnosis of the May-Thurner syndrome was suspected on Doppler examination of the iliofemoral vessels.5 May and Thurner,4 have advocated the use of pressure differentials to support the diagnosis of hemodynamically significant obstruction. They have suggested that a pressure differential between the two iliac veins of 2 mmHg at rest or 3 mmHg with exercise is significant and that an exaggerated pressure response to exercise is a marker of significant obstruction. Other authors have utilized inferior vena cava pressure as a surrogate for contralateral iliac vein pressures with the assumption that there should be little or no gradient between the inferior vena cava and the iliac vein unless an obstruction is present.15 May-Thurner syndrome is treated only when it is symptomatic. Previously open surgical procedures were done for repair of May-Thurner syndrome but with the advancement of technology less invasive endovascular repair has traditionally followed. The treatment is the removal of the clot with pharmacomechanical thrombolysis and mechanical thrombectomy to prevent post-thrombotic syndrome and to repair the anatomical defect with the use of stents and balloon angioplasty.16

CONCLUSION

May-Thurner syndrome is a common cause of left lower extremity swelling and deep vein thrombosis; however, it is underdiagnosed. In patients presenting with left-sided Deep Vein Thrombosis without obvious etiology, especially in young women, May-Thurner syndrome should be considered as a possibility. Thrombolysis with Endovascular intervention and stenting remains the choice for successful outcomes.

CONFLICT OF INTEREST

The authors declare no conflict of interest.

ABBREVIATIONS

DVT

Deep Vein Thrombosis

CT Venography

Computed Tomography Venography

PTA

Percutaneous Transluminal Angioplasty

IVC

Inferior Vena Cava.

SUMMARY

May-Thurner syndrome is a common cause of left lower extremity swelling and deep vein thrombosis. A Thrombolysis with Endovascular intervention and stenting remains the choice for successful outcomes.

REFERENCES

1. 

Cockett FB, Thomas ML , authors. The iliac compression syndrome. Br J Surg. 1965;52(10):816–21

2. 

McMurrich JP. , author. The occurrence of congenital adhesions in the common iliac veins, and their relation to thrombosis of the femoral and iliac veins. Am J Med Sci. 1908;135(3):342–5

3. 

Ehrich WE. , author. A frequent obstructive anomaly of the mouth of the left common iliac vein. Am Heart J. 1943;26(6):18–31

4. 

May R, Thurner J , authors. The cause of the predominantly sinistral occurrence of thrombosis of the pelvic veins. Angiology. 1957;8(5):419–27

5. 

Oguzkurt L, Ozkan U, Tercan F, Koc Z , authors. Ultrasonographic diagnosis of iliac vein compression (May-Thurner) syndrome. Diagnostic and Interventional Radiology. 2007;13(3):152–5

6. 

Kölbel T, Lindh M, Akesson M, et al. , authors. Chronic iliac vein occlusion: midterm results of endovascular recanalization. J Endovasc Ther. 2009;16(4):483–91

7. 

Dheer S, Joseph AE, Drooz A , authors. Retroperitoneal hematomacaused by a ruptured pelvic varix in a patientwith iliac vein compression syndrome. J VascIntervRadiol. 2003;14(3):387–90

8. 

Molloy S, Jacob S, Buckenham T, et al. , authors. Arterial compression of the right common iliac vein; an unusualanatomical variant. CardiovascSurg. 2002;10(3):291–2

9. 

Fretz V, Binkert CA , authors. Compression of the inferior venacava by the right iliac artery: a rare variant of May-Thurner syndrome. CardiovascInterventRadiol. 2010;33(5):1060–3

10. 

O’Sullivan GJ, Semba CP, Bittner CA, Kee ST, Razavi MK, Sze DY, Dake MD , authors. Endovascular management of iliac vein compression (May-Thurner) syndrome. J VascIntervRadiol. 2000;11(7):823–36

11. 

Thijs W, Rabe KF, Rosendaal FR, Middeldorp S , authors. Predominance of left-sided deep vein thrombosis and body weight. J ThrombHaemost. 2010;8(9):2083–4

12. 

Baron HC, Shams J, Wayne M , authors. Iliac vein compression syndrome: a new method of treatment. American Surgeon. 2000;66(7):653–5

13. 

Kim YH, Ko SM, Kim HT , authors. Spontaneous rupture of the left common iliac vein associated with May Thurner syndrome: successful management with surgery and placement of an endovascular stent. The British Journal of Radiology. 2007;80(956):e176–e179

14. 

Kim JY, Choi D, Ko YG, et al. , authors. Treatment of May-Thurner syndrome with catheter-guided local thrombolysisand stent insertion. Korean Circ J. 2004;34(7):655–9

15. 

Ferris EJ, Lim WN, Smith PL, et al. , authors. May-Thurnersyndrome. Radiology. 1983;147:29–31

16. 

Moudgill N, Hager E, Gonsalves C, Larson R, Lombardi J, Dimuzio P , authors. May-Thurner syndrome: case report and review of the literature involving modern endovascular therapy. Vascular. 2009;17(6):330–5