Patient Presenting with Nonpitting Edema Diagnosed with MTS

Phil Vinall

doi:10.1177/155989771424021

Summary

May-Thurner syndrome (MTS), also known as Cockett syndrome or ileocaval compression syndrome, is a vascular anomaly first described in 1956 by R. May and J. Thurner. This syndrome frequently involves pain and significant swelling of the whole limb; however, not all patients present with symptoms, which can include left leg pain and edema, pulmonary embolism, deep vein thrombosis, varicose veins, and chronic ulcerations. This article presents a case of a male patient aged 16 years without past medical history who was referred to her practice after 10 months of pain and severe progressive nonpitting edema of the left leg.

Thrombotic Disorders

Thrombotic Disorders
Cardiology

May-Thurner syndrome (MTS), also known as Cockett syndrome or ileocaval compression syndrome, is a vascular anomaly first described in 1956 by R. May and J. Thurner. This syndrome frequently involves pain and significant swelling of the whole limb; however, not all patients present with symptoms. When they do, besides pain and swelling, symptoms may include left leg pain and edema, pulmonary embolism, deep vein thrombosis, varicose veins, and chronic ulcerations.

Ramona A. Lappot Guzmán, MD, Medicina Cardiovascular Asociada, Santo Domingo, Dominican Republic, described a case of a male patient aged 16 years without past medical history who was referred to her practice after 10 months of pain and severe progressive nonpitting edema of the left leg. On examination, the swelling presented distally from the left thigh to the ankle. There was tenderness to the palpation but normal pulses, reflexes, and strength.

Ultrasonography showed a huge amount of thrombi in the left superficial femoral vein and the anterior tibia vein that persisted even after aggressive warfarin therapy. Extensive workup for thrombophilia, malignancies, and autoimmune diseases yielded negative results. Computed tomography angiography of the pelvis revealed inadequate venous opacification at the level of the iliac veins but no thrombosis, masses, or anatomic defects. Simultaneous venography and arteriography revealed normal anatomy of the arteries but did show an extrinsic compression of the left common iliac vein at the entry of the vena cava that was caused by an overlying right common iliac artery. This led to a diagnosis of MTS.

Treatment consisted of placing a vena cava filter below the renal veins and a stent where the left common iliac vein was compressed. Good expansion, anterograde flow, and a lumen opening of 95% of normal were achieved. The patient was discharged 3 days later without complications and with considerable edema reduction. There was good long-term recovery, although the patient remains on warfarin.

Once the condition has been identified (best by venography), traditional therapy is anticoagulation and, recently, endovascular procedures. MTS should be considered when dealing with left leg pain.

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