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May–Thurner syndrome
Other names	Iliac vein compression syndrome
Iliac veins
Specialty	Vascular surgery

May–Thurner syndrome (MTS), also known as the iliac vein compression syndrome, is a condition in which compression of the common venous outflow tract of the left lower extremity may cause discomfort, swelling, pain or iliofemoral deep vein thrombosis.

Specifically, the problem is due to left common iliac vein compression by the overlying right common iliac artery. This leads to stasis of blood, which predisposes to the formation of blood clots. Uncommon variations of MTS have been described, such as the right common iliac vein getting compressed by the right common iliac artery.

In the twenty-first century, the May–Thurner syndrome definition has been expanded to a broader disease profile known as nonthrombotic iliac vein lesions (NIVL) which can involve both the right and left iliac veins as well as multiple other named venous segments. This syndrome frequently manifests as pain when the limb is dependent (hanging down the edge of a bed/chair) and/or significant swelling of the whole limb.

Signs and symptoms

Because of its similarities to deep vein thrombosis (DVT), May–Thurner syndrome is rarely diagnosed amongst the general population. In this condition, the right iliac artery sequesters and compresses the left common iliac vein against the lumbar section of the spine, resulting in swelling of the legs and ankles, pain, tingling, and/or numbness in the legs and feet. The pain is often presented as dull, and may progress up and down the leg depending on the patient. Lower extremities may feel warm to the touch, and swelling may maintain or dissipate throughout the day.

Mechanism

In contrast to the right common iliac vein, which ascends almost vertically to the inferior vena cava, the left common iliac vein traverses diagonally from left to right to enter the inferior vena cava. Along this course, it goes under the right common iliac artery, which may compress it against the lumbar spine and limit the flow of blood out of the left leg. There are case reports of the inferior vena cava being compressed by the iliac arteries or right-sided compression syndromes, but the vast majority are on the left side. While this is the suspected cause of the syndrome, the left iliac vein is frequently seen to be compressed in asymptomatic patients, and considered an anatomic variant. A 50% luminal compression of the left iliac vein occurs in a quarter of healthy individuals. Compression becomes clinically significant only if it causes appreciable hemodynamic changes in venous flow or venous pressure, or if it leads to acute or chronic deep vein thrombosis (DVT).

In addition to compression, the vein develops intraluminal fibrous spurs from the effects of the chronic pulsatile compressive force from the artery. The narrowed turbulent channel predisposes the patient to thrombosis. The compromised blood flow often causes collateral blood vessels to form. These are most often horizontal transpelvis collaterals, connecting both internal iliac veins, thus creating outflow through the right common iliac vein. Sometimes vertical collaterals are formed, most often paralumbar, which can cause neurological symptoms, like tingling and numbness.

This compressed, narrowed outflow channel causes stasis of the blood, which is one element of Virchow's triad that precipitates DVT.

Diagnosis

It is important to consider May–Thurner syndrome in patients who have no other obvious reason for hypercoagulability and who present with left lower extremity thrombosis. To rule out other causes for hypercoagulation, it may be appropriate to check the antithrombin, protein C, protein S, factor V Leiden, prothrombin G20210A, and antiphospholipid antibodies.

Venography will demonstrate the classical syndrome when causing deep venous thrombosis.

May–Thurner syndrome in the broader disease profile, known as nonthrombotic iliac vein lesions (NIVLs), exists in the symptomatic ambulatory patient and these lesions are usually not seen by venography. Morphologically, intravascular ultrasound has emerged as the best current tool in the broader sense. Functional testing such as duplex ultrasound, venous and interstitial pressure measurement and plethysmography may sometimes be beneficial. Compression of the left common iliac vein may be seen on pelvic CT scan.

Treatment

Management of the underlying defect is proportional to the severity of the clinical presentation. Leg swelling and pain is best evaluated by vascular specialists (vascular surgeons, interventional cardiologists, and interventional radiologists) who both diagnose and treat arterial and venous diseases to ensure that the cause of the extremity pain is evaluated. The diagnosis needs to be confirmed with some sort of imaging that may include magnetic resonance venography, venogram and usually confirmed with intravascular ultrasound because the flattened vein may not be noticed on conventional venography. In order to prevent prolonged swelling or pain from the consequences of the backed up blood from the compressed iliac vein, flow needs to be improved out of the leg. Uncomplicated cases may be managed with compression stockings.

Severe May–Thurner syndrome may require thrombolysis if there is a recent onset of thrombosis, followed by angioplasty and stenting of the iliac vein after confirming the diagnosis with a venogram or an intravascular ultrasound. A stent may be used to support the area from further compression following angioplasty. As the name implies, there classically is not a thrombotic component in these cases, but thrombosis may occur at any time.

If the patient has extensive thrombosis, it may be appropriate to consider pharmacologic and/or mechanical (also known as pharmacomechanical) thrombectomy. This is currently being studied to determine whether this will decrease the incidence of post-thrombotic syndrome.

Epidemiology

May–Thurner syndrome is thought to represent between two and five percent of lower-extremity venous disorders. May–Thurner syndrome is often unrecognized. Current estimates are that this condition is three times more common in women than in men. The classic syndrome typically presents in the second to fourth decades of life. In the twenty-first century in a broader disease profile, the syndrome acts as a permissive lesion and becomes symptomatic when something else happens such as, following trauma, a change in functional status such as swelling following orthopaedic joint replacement.

See also

• Thoracic outlet syndrome (TOS) – Compression of the brachial plexus or subclavian vessels.
• Paget–Schroetter disease – Upper extremity deep vein thrombosis in the axillary or subclavian veins, related to TOS.
• Budd–Chiari syndrome – Venous compression or obstruction in the liver.
• Nutcracker syndrome - Compression of the left renal vein between aorta and upper mesenteric artery.

References

1. ^ Butros SR, Liu R, Oliveira GR, Ganguli S, Kalva S (2013). "Venous compression syndromes: clinical features, imaging findings and management". Br J Radiol. 86 (1030): 20130284. doi:10.1259/bjr.20130284. PMC 3798333. PMID 23908347.
2. ^ May R, Thurner J (1957). "The cause of the predominantly sinistral occurrence of thrombosis of the pelvic veins". Angiology. 8 (5): 419–27. doi:10.1177/000331975700800505. PMID 13478912. S2CID 6799302.
3. Fazel R, Froehlich JB, Williams DM, Saint S, Nallamothu BK (2007). "Clinical problem-solving. A sinister development – a 35-year-old woman presented to the emergency department with a 2-day history of progressive swelling and pain in her left leg, without antecedent trauma". N. Engl. J. Med. 357 (1): 53–9. doi:10.1056/NEJMcps061337. PMID 17611208.
4. Raju S, Neglen P (July 2006). "High prevalence of nonthrombotic iliac vein lesions in chronic venous disease: a permissive role in pathogenicity". J Vasc Surg. 44 (1): 136–43. doi:10.1016/j.jvs.2006.02.065. PMID 16828437.
5. Khalid, Sidra; Khalid, Aariez; Meridores, Tessy; Daw, Hamed (2018). "May-Thurner Syndrome: A Rare Cause of Deep Venous Thrombosis". Cureus. 10 (5): e2700. doi:10.7759/cureus.2700. PMC 6063391. PMID 30062074.
6. ^ Peters, Matthew; Khazi Syed, Rashad; Katz, Morgan; Moscona, John; Press, Christopher; Nijjar, Vikram; Bisharat, Mohannad; Baldwin, Drew (2012). "May-Thurner syndrome: a not so uncommon cause of a common condition". Proceedings (Baylor University. Medical Center). 25 (3): 231–233. doi:10.1080/08998280.2012.11928834. PMC 3377287. PMID 22754121.
7. ^ Marder VJ, Aird WC, Bennett JS, Schulman S. Hemostasis and Thrombosis: Basic Principles and Clinical Practice. 2013 ISBN 9781608319060
8. Neglén, Peter; Raju, Seshadri (April 2002). "Intravascular ultrasound scan evaluation of the obstructed vein". Journal of Vascular Surgery. 35 (4): 694–700. doi:10.1067/mva.2002.121127. PMID 11932665.
9. Mousa AY, AbuRahma AF (2013). "May-Thurner syndrome: update and review". Ann Vasc Surg. 27 (7): 984–95. doi:10.1016/j.avsg.2013.05.001. PMID 23850314.
10. Moudgill, Neil; Hager, Eric; Gonsalves, Carin; Larson, Robert; Lombardi, Joseph; DiMuzio, Paul (1 December 2009). "May-Thurner syndrome: case report and review of the literature involving modern endovascular therapy". Vascular. 17 (6): 330–335. doi:10.2310/6670.2009.00027. ISSN 1708-5381. PMID 19909680. S2CID 8475092.

External links

• May-Thurner syndrome at Who Named It?
• Vascular medicine – Vascular medicine and angiology related topics for experts and laypersons.

• Syndromes affecting the vascular system
• Diseases named after discoverers