Introduction:

Phlegmasia cerulea (PC) is a severe form of deep vein thrombosis. In the setting of massive venous thrombosis and severe ischemia, catheter-directed thrombolysis (CDT) or trombectomy is mandatory. We report three cases of women with PC managed with venous thrombectomy after failure of CDT.

Case reports:

1: 20 years-old, with recent intake of oral contraceptive, referred with acute onset of limb swelling, pain and a cold left lower extremity associated with foot pallor, paresthesia and numbness. Doppler ultrasound revealed occlusive thrombosis of the entire deep venous system and the great saphenous vein (GSV). Anticoagulation (AC) and CDT were started. However due to increasing levels of transaminases, creatine kinase and myoglobin, CTD was stopped and venous thrombectomy was proposed. A retrievable inferior vena cava filter (IVC) was implanted and venous surgical trombectomy. The completion venography showed a Cockett compression that was treated with stenting of the left iliac vein. Thrombophilia tests were positive for anticardiolipin antibodies an hyperhomocysteinemia. At 3-years follow-up, the patient is asymptomatic and under AC. The 3-year Doppler showed normal patency for the iliac stent and a mild femoropopliteal vein insuffiency.

2: 19 year-old, taking oral contraceptives, with acute onset of PCD with acute thrombus in the left iliac, femoral, popliteal veins. CDT was started at admittance but stopped after two days because of very low values of serum fibrinogen and persistence of occlusive thrombus in the iliac vein. A retrievable IVC filter was placed and the thrombus removed with surgical thrombectomy. Phlebography showed no significant residual thrombus and no signs of compression were present. At 1 month follow-up, the patient presented without leg edema or venous claudication symptoms. Thrombophilia testing is awaited.

3: 54 year-old who presented with low back pain, worsening left leg pain and swelling with a cyanosed and colder foot. At doppler ultrasound there were monophasic arterial flow in the left leg. After exclusion of arterial embolism, the first therapeutic approach was CDT, but it was also stopped due to very low fibrinogen levels and an ineffective thrombus lysis in venography controls. After implantation of a retrievable IVC, surgical thrombectomy via femoral vein was performed, with successful thrombus removal. Venography showed Cockett syndrome and a stent was implanted. At 6 months the patient remained without major symptoms, and Doppler confirmed stent patency with non residual obstruction or venous insufficiency.

Conclusion:

Awareness and timely diagnosis of phlegmasia cerulea is necessary to ensure prompt intervention to prevent loss of limb. When CDT is not effective, surgical thrombectomy remains successfully alternative. Iliac venous stenting complement is also crucial to treat associated Cockett syndrome. Endovascular thrombectomy devices may be a reasonable alternative to surgical thrombectomy.

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