May – Thurner Syndrome: A case based review

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2014 Sameer Gupta, Taslima Bhuiyan et al.
Journal of Heart and Circulation
Research Article
Open Access
May – Thurner Syndrome: A Case Based Review
Sameer Gupta ¹,²*, Taslima Bhuiyan²,*, Amit R Shah¹, Ross T Lyon¹, Aksim Rivera¹ and Seth I Sokol¹,²
1
Department of Medicine and Surgery, Division of Endovascular Services, Jacobi Medical Center, Bronx, N.Y.
2
Department of Medicine, Division of Cardiology, Montefiore Medical Center and the Albert Einstein College of Medicine, Bronx, N.Y.
Keywords: May Thurner syndrome; Iliac vein compression;
Deep vein thrombosis.
Introduction
May-Thurner syndrome is a disorder of left common iliac
vein compression by an overriding right common iliac artery that leads
to deep venous thrombosis (DVT). This syndrome was first described
in 1957 by May and Thurner [1], but has remained under-recognized
as an etiology for DVT. Even though the anatomical variant is
common, not all patients will develop symptoms and therefore the
prevalence of this syndrome is difficult to assess. Patients may present
with acute DVT or signs and symptoms of chronic venous
hypertension such as venous reflux or hyper pigmented skin changes.
The diagnosis is made clinically based on history and exam along with
the aid of imaging modalities to document iliac vein compression by
the iliac artery. Treatment of May-Thurner syndrome includes
anticoagulation in addition to more definitive therapy to correct the
anatomical disorder with endovascular stenting or open surgical
repair. We present a case of May-Thurner syndrome followed by a
review of the current literature on the subject
Case Report
Medical Center (Bronx, N.Y.) with pain and swelling to his left lower
extremity for 4 days. The patient was a waiter by profession and
reported the onset of pain after prolonged standing during a long work
day. Initially his leg was mildly sore and over the next few days he
developed pain in the left lower back, thigh and leg with associated
swelling. The pain acutely worsened the night before presentation with
difficulty ambulating and moving his leg. He denied any injury or
recent prolonged travel requiring his leg to be immobile. His family
history was non-contributory. He denied any medication or
recreational drug use. His vitals were stable and his BMI was 17.7
kg/m2. On exam, his left leg was warm and tensely swollen from the
ankle to the mid-thigh, and diffusely tender to palpation. His distal
pulses were intact. His strength, reflexes and sensation were also intact.
His complete blood count, basic metabolic profile and PT/INR were
normal.
An X-ray did not show any evidence of fracture. A venous
Doppler demonstrated extensive deep vein thrombosis (DVT)
extending from the popliteal vein to the common femoral vein with a
dilated external iliac vein without flow (Fig 1 A,B). Incidentally he was
noted to have concern for left hydronephrosis. A CT Angiogram
demonstrated the right common iliac artery over-riding a dilated left
common iliac vein (Fig 2).
A 28 year old East Asian male with no significant past
medical history presented to the Emergency Department at Jacobi
Fig 1 A – Dilated and non-compressible common femoral vein
(White Arrow) with thrombus
J Heart Circ
Fig 1 B- Color Doppler demonstrating flow in the common femoral
artery (A) and no flow in the femoral vein (V).
Vol. 1. Issue. 1. 17000101
Citation: Sameer Gupta, Taslima Bhuiyan, Amit R Shah, Ross T Lyon, Aksim Rivera, et al. (2014) May – Thurner Syndrome: A Case Based Review. J
Heart Circ 1(1): 17000101.
Page 2 of 5
Figure 2 – CT Angiogram demonstrating right common iliac
artery (arrow) crossing the left common iliac vein (arrow head). Also
incidentally noted to have massive asymptomatic left hydronephrosis.
Interventional Therapy
Given the extent of DVT, patient’s young age, good health
and high risk for post-thrombotic syndrome, catheter-directed
thrombolysis was initiated after informed consent was obtained. The
patient was brought to the cardiac catheterization laboratory and
placed in the prone position on the table. Ultrasound- guided access
was obtained in the left popliteal vein using a micropuncture kit (Cook
Right
Left
Fig 3A- Venogram demonstrating occlusion of left femoral vein with
heavy thrombus burden (3A, arrow) and patent deep collateral vein.
Fig 4A- Venogram after thrombolysis demonstrating residual
stenosis at the left CIV-IVC junction (white arrow).
J Heart Circ
Medical, Bloomington, IN). A venogram was performed through the
micropuncture sheath which confirmed a large clot burden (Fig 3A)
and collaterals from the left iliac to the right iliac veins (Fig 3B). A 7
French Glide sheath (Terumo Corporation, Tokyo, Japan) was placed
in the popliteal vein and a 0.035” Glidewire (Terumo Corporation,
Tokyo, Japan) was advanced through the thrombus into the inferior
vena cava. An EKOS catheter (EKOS Corporation, Bothell, WA) was
placed. A 10mg bolus of tissue plasminogen activator (TPA) was given
followed by an infusion at a rate of 1.5mg/hr. Heparin at 500units/hr
was given via the side port of the venous sheath. After 8 hours, the
patient was taken back to the catheterization laboratory and a repeat
venogram demonstrated marked reduction in clot burden with mild
residual clot in the femoral vein and external iliac vein and reduction
of collateral flow but severe stenosis at the left common iliac vein
(CIV) - IVC junction. Manual thrombectomy was performed using a 7
French Hockey stick catheter (Cordis Corporation, Bridgewater
Township, New Jersey) followed by balloon angioplasty of the left CIV
stenosis with a 10mm x 60mm Mustang balloon (Boston Scientific,
Natick, MA) and 14mm x 40mm Atlas balloon (Bard Peripheral
Vascular Inc, Temple, AZ). Repeat venogram demonstrated resolution
of thrombus with residual stenosis at the left CIV-IVC junction (Fig
4A). A 16mm x 60mm self-expanding Wall stent (Boston Scientific,
Natick MA) was therefore deployed at the area of stenosis extending
into the IVC (Fig 4B). The stent was post dilated successfully.
Right
Left
Fig 3B- Venogram in the AP projection demonstrating thrombus in
the Left iliac vein(arrow head) with collaterals from the left to right
venous system (Arrow)
Fig 4B- Venogram after stent placement (Arrow) demonstrating
brisk flow across the area of stenosis with no residual stenosis or
clot. Stent extends into IVC and is anchored against wall.
Vol. 1. Issue. 1. 17000101
Citation: Sameer Gupta, Taslima Bhuiyan, Amit R Shah, Ross T Lyon, Aksim Rivera, et al. (2014) May – Thurner Syndrome: A Case Based Review.
J Heart Circ 1(1): 17000101.
Page 3 of 5
After stent placement the patient received therapeutic anticoagulation
with enoxaparin and was bridged to therapeutic warfarin. A venous
Doppler the next day demonstrated no residual venous thrombosis.
The pain and swelling in his left leg improved dramatically and he was
discharged home on post- operative day three with compression
stockings. He followed up in clinic and a repeat ultrasound at 2 weeks
demonstrated patent stent with no clot (Fig 5).
A hypercoagulability workup, including Factor V Leiden,
Antithrombin III, homocysteine and prothrombin gene mutation, was
negative.
Pathophysiology
May-Thurner syndrome occurs when the iliac vein
develops evidence of venous hypertension or deep venous thrombosis
due to chronic compression by an overlying right common iliac artery
against the lumbar vertebrae [5]. Although there are other parts of the
body where an artery apposes a vein, the vein is simply displaced in
those circumstances and therefore does not feel the effects of arterial
pulsation or compression. In the case of May-Thurner syndrome, the
position of the iliac vein in between the right iliac artery and vertebral
body leads to the compression effect. It has been postulated that chronic
compression and arterial pulsations lead to the development of
endovascular intimal proliferation causing venous “spur” formation,
impaired venous return and blood stasis leading to thrombus
formation. Autopsy studies of patients with lower extremity DVT
revealed presence of large amounts of elastin and collagen in the
thickened venous wall, without inflammatory cellular infiltration or
scar tissue. There are three morphologic types of May-Thurner
syndrome: focal compression by the overlying iliac arteries, diffuse
atrophy and cordlike obliteration of the iliac vein. Diffuse atrophy and
cordlike obliteration represent chronic effects of focal compression
leading to fibrosis. Once the venous walls have formed spurs leading to
luminal narrowing, the process is felt to be irreversible. Patients with
venous spurs may remain asymptomatic for an extended time due to
the formation of collateral venous drainage.
Literature Review
Left common iliac vein compression is the most common
variant seen in May-Thurner, but there are other less
common variants, including left common iliac vein compression
from a left common iliac artery, compression of the right common
iliac vein by the right internal iliac artery and right-sided MayThurner in a patient with a left-sided IVC [6]. There are also reports
of thrombophilia in patients with May-Thurner and some
experts have advocated performing a hypercoagulable work-up
in all patients who present with this syndrome [7].
Prevalence
Clinical Manifestations
May-Thurner syndrome is a rare condition whereby the
left common iliac vein is compressed in between an overriding right
common iliac artery and the posterior vertebral spine leading to
DVT and or chronic venous hypertension. The true prevalence of this
anatomical variant is unknown, but quoted to be as high as 22%
based on autopsy series [2,3]. Despite this, the syndrome remains
under-recognized, mainly because a small percentage of those with
DVT have this anatomical variant (2-3%) [4]. It is seen
predominantly in women in the second to fourth decade of life, after
prolonged immobilization, use of oral contraceptives or multiple
pregnancies. Only 30% of those with May-Thurner syndrome are
male.
The clinical presentation of May-Thurner can vary widely. There are
many patients with iliac vein compression without any symptoms
while others may present with chronic venous insufficiency and/or
DVT, the hallmark of May-Thurner syndrome. Patients will present
with unilateral lower extremity edema and pain, predominantly on
the left side. Some also present with venous claudication. However,
because of the chronic and progressive nature of the disease process,
patients may have stigmata of post-thrombotic syndrome such as
venous reflux, hyperpigmentation of the skin, varicose veins
(retroperitoneal and pudendal venous collaterals to shunt the distal
iliofemoral venous system), chronic leg pain, phlebitis,
lipodermatosclerosis and recurrent skin ulcers [5,8].
Fig 5- Follow up ultrasound at 2 weeks with patent stent (arrow)
within the left EIV extending into the CIV and normal phasic flow
(arrowhead).
J Heart Circ
Vol. 1. Issue. 1. 17000101
Citation: Sameer Gupta, Taslima Bhuiyan, Amit R Shah, Ross T Lyon, Aksim Rivera, et al. (2014) May – Thurner Syndrome: A Case Based
Page 4 of 5
Review. J Heart Circ 1(1): 17000101.
Kim et al. have described clinical stages of iliac vein compression.
Stage I is asymptomatic iliac vein compression. Stage II is
development of venous spur. Stage III is development of left iliac vein
DVT. Patients with DVT in the past are at increased risk for
developing recurrent DVT. It has also been noted that patients with
DVT and who have spur formation in the vein had a 72% of
recurrence despite treatment with anticoagulation. Although DVT
may be common in May-Thurner syndrome, very few patients
progress to large pulmonary embolism. One study found an inverse
correlation between the severity of stenosis and symptomatic
pulmonary embolism in these patients [9]. This may be due to a
“protective” effect of luminal narrowing from spur formation and
possible trapping of large emboli that may otherwise go to the lungs
[10].
Diagnosis
The diagnosis of May-Thurner is based on presence of
DVT and subsequent confirmation of the anatomy of the iliac arteries
and veins. This syndrome should be suspected in patients who present
with recurrent DVT without another apparent etiology or have
extensive DVT on the left side. The presence of DVT can be
confirmed non-invasively with lower extremity ultrasound. With
regards to confirming venous compression, there have been various
proposed methods such as measuring pressure differentials between
the two iliac veins at rest (difference of 2 mmHg) and with exercise
(difference of 3 mmHg). Others have utilized inferior vena cava
pressures as a surrogate for contra lateral iliac vein pressure. Imaging
modalities are increasingly used to make the diagnosis. While lower
extremity vascular ultrasound will confirm presence of DVT, it will
not provide information on the anatomical position of the iliac
arteries and veins or presence of spurs. Abdominal helical CT and
MRI venography, and intravenous ultrasound have been utilized for
diagnosis of DVT and delineation of venous anatomy consistent with
May-Thurner syndrome with good yield compared to the gold
standard of traditional invasive venography. MRI can also provide
accurate estimation of collateral blood flow. Intravascular ultrasound
plays an important role in May-Thurner because it has the ability to
assess luminal diameter and presence of spurs and aid in endovascular
intervention. One must keep in mind that there may be significant
variability in the degree of venous compression noted in these imaging
modalities in the same patient depending on volume status. However,
in true May-Thurner, the vein should remain narrowed regardless of
volume status and patient position on serial imaging [11]. CT and
MRI venography are also useful to evaluate for alternative etiologies of
DVT (tumor, lymphadenopathy and hematoma) [8].
J Heart Circ
Treatment
The goal of treatment in May-Thurner syndrome is to
reduce the incidence of post-thrombotic syndrome (PTS) by
eliminating obstructing thrombus and correcting the precipitating
factor. Most studies evaluating treatment for iliac vein compression
syndrome are either retrospective or observational [5,12].
Anticoagulation with unfractionated heparin, low molecular
heparin and warfarin is the mainstay of treatment for patients with
DVT to reduce propagation of thrombus and prevent recurrence. If
early clot lysis can be achieved with catheter directed thrombolysis
or mechanical thrombectomy, the incidence of PTS can be reduced
[13-15]. Despite the success of these measures in routine iliofemoral
DVT, clot lysis therapy is inadequate for patients with MayThurner syndrome [14,16, 17].
Surgical treatments to relieve the obstruction include
a variety of novel techniques that include creation of a bypass
(the Palma cross over), iliac vein patch angioplasty and
relocation of the artery behind the vein [10,18]. These
procedures incur a high morbidity and patients still need longterm anticoagulation. With the improvement in percutaneous
endovascular techniques, balloon angioplasty and stenting have
become the standard of care [10,17]. An endovascular approach
reduces morbidity and permits assessment of stenosis
characteristics and collateral circulation during the procedure. In
cases of large thrombus burden mechanical and catheter
directed thrombolysis (CDT) are also be employed to improve
clinical symptoms and vessel patency [19].
irst Ādescribed by erger, Āstent placement for iliocaval
stenosis is now being used routinely [14,17,20]. Isolated balloon
angioplasty and or CDT alone has a high re-occlusion rate (100% in
one study)[1] Ā suggesting it should be supplemented with stent
placement to improve patency. The technical success of the
endovascular approach is upwards of 96% with a 1 year primary and
secondary patency rate of 58-100% and 76-98% [1,21,22] Ā The
durability, long length and flexibility of self-expanding stents make
them the preferred stent compared to balloon expandable stents in the
treatment of iliocaval stenosis. The average stents used are 1 Āto
1mm Āx Ācm Wall stents. The stented area should cover the area of
stenosis and if needed can be extended proximally into the IVC [1]. Ā
tenting Āinto the IVC has shown to be safe with no increased risk of
contralateral iliac vein occlusion [23]. When successfully treated,
patients experience some immediate relief in their symptoms that may
not be reflected in the conventional hemodynamic measurements
[1,23]. Ā In the absence of randomized trials, expert opinion
recommends 3- Ā months of anticoagulation followed by lifelong
antiplatelet therapy if no evidence of hypercoagulability is found.
Vol. 1. Issue. 1. 17000101
Citation: Sameer Gupta, Taslima Bhuiyan, Amit R Shah, Ross T Lyon, Aksim Rivera, et al. (2014) May – Thurner Syndrome: A Case Based
Review. J Heart Circ 1(1): 17000101.
Page 5 of 5
Conclusion
May-Thurner syndrome is an underappreciated cause of
DVT and chronic venous insufficiency. The syndrome should be
suspected in all young and middle aged patients who present with
left sided ileo-femoral DVT or isolated left sided chronic venous
disease. We advocate an aggressive approach in appropriate
candidates utilizing catheter directed thrombolysis, and mechanical
thrombectomy followed by angioplasty and stenting of the common
iliac vein. Although surgical interventions are available, there is
significant more risk associated with surgical repair compared to
endovascular techniques.
References
1. May R, Thurner J (1957) The cause of the predominantly sinistral
occurrence of thrombosis of the pelvic veins. Angiology 8: 419-427.
2. Mathur M, M Cohen, R Bashir (2014) May-Thurner syndrome.
Circulation 129: p. 824-5.
3. Foit NA, Chen QM, Cook B, Hammerberg EM (2013) Iliofemoral deep
vein thrombosis after tibial plateau fracture fixation related to
undiagnosed May-Thurner syndrome: a case report. Patient Saf Surg
7: 12.
4. Pallavi R, S Chaudhari (2014) The Eye cannot see what the Mind does
not know: An unusual cause of deep venous thrombosis. Am J Med.
5. Wael Ibrahim, Zakareya Al Safran, Hosam Hasan, Wael Abu Zeid
(2012) Endovascular management of may-thurner syndrome. Ann
Vasc Dis 5: 217-221.
6. Fretz V, CA Binkert (2010) Compression of the inferior vena cava by
the right iliac artery: a rare variant of May-Thurner syndrome.
Cardiovasc Intervent Radiol 33: 1060-1063.
7. Sharma R, W Joshi (2008) A case of May-Thurner syndrome with
antiphospholipid antibody syndrome. Conn Med 72: 527-530.
8. Wu WL, Tzeng WS, Wu RH, Tsai WL, Chen MC, et al. (2012)
Comprehensive MDCT evaluation of patients with suspected MayThurner syndrome. AJR Am J Roentgenol 199: 638-645.
9. Chan KT, Popat RA, Sze DY, Kuo WT, Kothary N, et al. (2011) Common
iliac vein stenosis and risk of symptomatic pulmonary embolism: an
inverse correlation. J Vasc Interv Radiol 22: 133-341.
10. Brazeau NF, Harvey HB, Pinto EG, Deipolyi A, Hesketh RL, et al.
(2013) May-Thurner syndrome: diagnosis and management. Vasa
42: 96-105.
11. McDermott S, Oliveira G, Ergül E, Brazeau N, Wicky S, et al. (2013) MayThurner syndrome: can it be diagnosed by a single MR venography
study? Diagn Interv Radiol 19: 44-48.
12. DeRubertis BG, Alktaifi A, Jimenez JC, Rigberg D, Gelabert H, et al. (2013)
Endovascular management of nonmalignant iliocaval venous lesions.
Ann Vasc Surg 27: 577-586.
13. Benjamin O Patterson, Robert Hinchliffe, Ian M Loftus, Matt M
Thompson, Peter JE Holt, et al. (2010) Indications for catheter-directed
thrombolysis in the management of acute proximal deep venous
thrombosis. Arterioscler Thromb Vasc Biol 30: 669-674.
14. Kim JY, Choi D, Guk Ko Y, Park S, Jang Y, et al. (2006) Percutaneous
treatment of deep vein thrombosis in May-Thurner syndrome.
Cardiovasc Intervent Radiol 29: 571-575.
15. Enden T, Haig Y, Kløw NE, Slagsvold CE, Sandvik L, et al. (2012) Longterm outcome after additional catheter-directed thrombolysis versus
standard treatment for acute iliofemoral deep vein thrombosis (the
CaVenT study): a randomised controlled trial. Lancet 379: 31-38.
16. Peters M, Syed RK, Katz M, Moscona J, Press C, et al. (2012) May-Thurner
syndrome: a not so uncommon cause of a common condition. Proc (Bayl
Univ Med Cent) 25: 231-233.
17. Firas F Mussa, Eric K Peden, Wei Zhou, Peter H Lin, Alan B Lumsden, et
al. (2007) Iliac vein stenting for chronic venous insufficiency. Tex Heart
Inst J 34: 60-66.
18. Palma, EC, R Esperon, (1960) Vein transplants and grafts in the surgical
treatment of the postphlebitic syndrome. J Cardiovasc Surg (Torino) 1:
94-107.
19. Patel NH, Stookey KR, Ketcham DB, Cragg AH (2000) Endovascular
management of acute extensive iliofemoral deep venous thrombosis
caused by May-Thurner syndrome. J Vasc Interv Radiol 11: 1297-1302.
20. Berger A, Jaffe JW, York TN (1995) Iliac compression syndrome treated
with stent placement. J Vasc Surg 21: 510-514.
21. Binkert CA, Schoch E, Stuckmann G, Largiader J, Wigger P, et al. (1998)
Treatment of pelvic venous spur (May-Thurner syndrome) with selfexpanding metallic endoprostheses. Cardiovasc Intervent Radiol 21: 2226.
22. Mousa AY, AbuRahma AF (2013) May-Thurner syndrome: update and
review. Ann Vasc Surg 27: 984-995.
23. Neglén P, Hollis KC, Olivier J, Raju S (2007) Stenting of the venous
outflow in chronic venous disease: long-term stent-related outcome,
clinical, and hemodynamic result. J Vasc Surg 46: 979-990.
*Corresponding Authors: 1. Sameer Gupta, Departments of Medicine and Surgery, Division of Endovascular Services, Jacobi
Medical Center, Department of Medicine, Division of Cardiology, Montefiore Medical Center and the Albert Einstein College of
Medicine, Bronx, N.Y.
2. Taslima Bhuiyan, Departments of Medicine and Surgery, Division of Endovascular Services, Jacobi Medical Center, Department
of Medicine, Division of Cardiology, Montefiore Medical Center and the Albert Einstein College of Medicine, Bronx, N.Y,
Received Date: April 30, 2014, Accepted Date: May 22, 2014, Published Date: June 17, 2014.
Copyright: © 2014 Sameer Gupta, Taslima Bhuiyan et al. This is an open access article distributed under the Creative Commons
Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is
properly cited.
Citation: Sameer Gupta, Taslima Bhuiyan, Amit R Shah, Ross T Lyon, Aksim Rivera, et al. (2014) May – Thurner Syndrome: A
Case Based Review. J Heart Circ 1(1): 17000101.
J Heart Circ
Vol. 1. Issue. 1. 17000101