Iliofemoral DVT Progressing to Phlegmasia Cerulea Dolens in a Woman With May-Thurner Syndrome

Authors:
Lauren A. Hutson, MD, MBA; Braden Harold Danbury; and Tapasdip Maheshkumar Gajjar, MD

Citation:
Hutson LA, Danbury BH, Gajjar TM. Iliofemoral DVT progressing to phlegmasia cerulea dolens in a woman with May-Thurner syndrome [published online August 22, 2018]. Cardiology Consultant.

A 48-year-old woman with a history of left ankle tendonitis 3 months prior and long-term use of estrogen oral contraceptives presented to the emergency department (ED) with pain and swelling in her left leg.

History. The pain had begun the night before and had ascended from her left leg to include her groin and back. She had until recently been using an orthopedic boot and crutches as a result of her ankle tendonitis. She denied other recent trauma, chest pain, dyspnea, cough, hemoptysis, fever, chills, abdominal pain, nausea, and vomiting. She also denied recent surgical procedures, recent travel, prolonged immobilization, and any history of coagulopathies, cancer, or cardiac disease. She denied smoking.

Physical examination. Her vital signs were within normal limits, except for an oxygen saturation of 87%. Cardiac and respiratory examinations revealed no abnormalities. Her left lower extremity was nontender without palpable cords, exhibited nonpitting edema from groin to foot, and had soft compartments, palpable pulses, and full range of motion. The skin was dry and cooler to the touch than that of the right leg.

Doppler ultrasonography of the leg revealed slow venous blood flow, normal luminal compressibility, and color flow without evidence of thrombi in the common femoral, femoral, deep femoral, popliteal, peroneal, or posterior tibial veins. She was discharged home in stable condition with a compression stocking and instructions to follow up with her primary care physician.

The next morning, she presented to a different ED for worsening pain and swelling despite medication, compression, and elevation. Her vital signs were still normal; her oxygen saturation was then 96%. Cardiac and respiratory examination findings were benign. Her left lower extremity was edematous and tender from hip to foot, with diminished pulse in the left ankle and decreased capillary refill in the left foot.

Diagnostic tests. A complete blood cell count with differential revealed leukocytosis (17,900 white blood cells/µL) and neutrophilia (15,560 neutrophils/µL). Prothrombin time was 13.3 seconds, the international normalized ratio was 1.0, and the activated partial thromboplastin time was 29.3 seconds. The lactate level was elevated at 27.9 mg/dL.

Results of computed tomography arteriography (CTA) of the abdomen with runoff including extremities revealed edema around the left femoral vein and possible expansion, in addition to dilation of the left external iliac vein with edema in the surrounding tissues. The left common iliac vein could not be visualized clearly. Findings from venous ultrasonography of the left leg revealed an extensive occlusive deep-vein thrombosis (DVT). Findings of CTA of the chest were negative for pulmonary emboli.

An intravenous heparin drip was started. Her condition progressed with pain in her left lower abdomen and discoloration of her left leg (Figure 1). She was admitted to the intensive care unit with diagnoses of DVT of the left deep femoral vein, systemic inflammatory response syndrome, phlegmasia cerulea dolens of the left lower extremity, and possible May-Thurner syndrome (MTS).

Figure 1. The patient’s legs on presentation to the ED.

Cardiology and interventional radiology specialists were consulted and intervened with urgent revascularization. Venography findings showed that the clot extended proximally to the iliac veins with concurrent high-grade stenosis of the left common iliac vein. An overnight catheter-directed infusion of tissue plasminogen activator was started in addition to continued heparin and pain medications.

The next day, the laboratory values had improved, but she still reported having severe pain. The limb remained swollen and tender with intact sensation and diminished pulse at the ankle.

A thrombectomy and thrombolysis were performed on the left superficial femoral vein, the left common femoral vein, the left external iliac vein, and the left common iliac vein. Findings from venography of the inferior vena cava and iliac veins followed by intravenous ultrasonography confirmed compression of the left common iliac vein between the right common iliac artery and the spine, with resulting stenosis (Figure 2 and Video).

Figure 2. Image of venous flow during catheterization before stent placement.

Percutaneous transluminal valvuloplasty of the left common iliac vein, external iliac vein, and common femoral vein was done. A stent was deployed across the area of stenosis in the left common iliac vein and external iliac vein. Subsequent imaging showed excellent results (Figure 3 and Video). The patient reported relief of her pain following the procedure (Figure 4).

Figure 3. Image of venous flow during catheterization after stent placement.

Figure 4. The patient’s legs after treatment with catheter-directed tissue plasminogen activator.