Case Presentation: A 58-year old female with history of breast cancer and uterine fibroid, presented to the ED with worsening right leg pain and swelling associated with red skin discoloration and blanching skin. There was no history of fever, dyspnea, coughs, chest pain, hemoptysis, diaphoresis, palpitations, oral contraceptives or tobacco use. She has family history of Factor V Leiden mutation, however patient has never been tested. On examination she had stable vital signs and found to have edema of right lower extremity which was larger than the left along with the phlegmasia. Labs were without leukocytosis, anemia, or electrolyte derangements. Partial thromboplastin time (PTT), International normalized ratio (INR), troponin and pro-B-type natriuretic peptide (pro-BNP) were within normal limits. Duplex study of the right lower extremity revealed extensive acute right deep vein thrombosis (DVT) involving the right external iliac, femoral, and deep femoral veins. Subsequent CT angiogram of the chest revealed bilateral lower lobe segmental pulmonary emboli (PE). Abdominal and pelvic CT confirmed DVT, involving the right common iliac, external iliac veins, the right common femoral, femoral, extending into profunda veins and the right greater saphenous vein. The uterus was markedly enlarged, containing a 13.9 x 12.1 x 16.2 cm mass, compressing right common iliac vein. During the course of the treatment she was started Heparin infusion. Patient’s right lower extremity edema worsened significantly with markedly darker skin coloration suggestive of impending necrosis and limb compromise. Therefore, she required catheter directed thrombolysis of right popliteal vein as well as mechanical thrombectomy was performed of aforementioned veins. Patient’s right leg returned to its normal and pain-free after the procedure. She was subsequently discharged on subcutaneous low molecular heparin. Molecular genetics results later revealed heterozygous Factor V Leiden mutation, compound heterozygous MTHFR mutation (C677T and A1298C). Prothrombin G20210A mutation was negative.
Discussion: May-Thurner Syndrome is a condition where there is an extrinsic compression of iliocaval venous territory by arterial system against the bony prominence. Most commonly this variant is seen on left side which led to VTE of the lower extremity. In our case presentation following the same May-Thurner physiology, however, here the enlarged fibroid uterus compressed into right common iliac artery as well as right common iliac vein, causing venous stasis over a significant length along the right common iliac vein, mimicking May Thurner but in the uncommon extremity. Thrombolysis and anticoagulation were performed to salvage the limb from necrosis and to prevent respiratory compromise from PE.
Conclusions: The utmost importance of this case for the hospitalist is the early recognition and intervention of unusual presentation of MTS-like symptoms, especially with VTE risk factors. The end result was right lower extremity venous claudication and asymptomatic PE, which could have resulted in loss of her right lower extremity and respiratory compromise. Vigilant recognition of risk factors, early initiation of anticoagulation, and rapid coordination to thrombolysis is critical for clinician to save the limb.