A 37yearold woman with a past medical history of depression, anxiety and migraine, who presented to emergency complaining of severe left leg pain and swelling which is sudden in onset. Pain is 10/10, worse with walking extends from calf and to distal leg. She was hemodynamically stable. Her left extremity was pale, white, swollen and dorsalis pedis pulse was diminished. Her laboratory data at admission showed lactic acid of 8, Ddimer of 4300 with normal hematology and metabolic profile. Her US of lower extremity showed occlusive deep venous thrombosis extending from the left external iliac vein into the left popliteal vein. Venography showed a cast of thrombus filling in the left deep system from the common femoral vein to the mid popliteal vein. Diffuse left iliac vein stricture likely the etiology of the patient’s DVT and consistent with MayThurner syndrome. Pt has signs of left leg ischemia. An immediate thrombectomy was performed with a stent placement. CT chest did not show PE and CT abd/pelvis did not reveal any mass. Coagulation profile did not show any abnormality and factor V and antithrombin 3 mutation was negative. Pts left leg pain immediately got better after thrombectomy with stenting and her pulse were full and color skin turned red. Pt was started on anticoagulation and discharged on warfarin.
This is the rare presentation of deep venous thrombosis, which occluded venous system to the extent that it caused obstruction of blood supply to lower limb. Fabricus Hildanus first described this entity in the 16th century. Precipitating factors include malignancy, femoral vein catheterization, heparininduced thrombocytopenia, antiphospholipid antibody syndrome. In MayThurner syndrome, there is compression of the left common iliac vein by the overlying right common iliac artery. It occurs only in left leg. This is the first case of MayThurner syndrome causing plegmasia cerulea dolens. Prompt diagnosis and treatment initiation are paramount in order to prevent progression to venous gangrene and the need for amputation and possible death of the patient who presents with this condition.
Phlegmasia alba dolens is a spectrum of disease related to Deep venous thrombosis. It is a sudden onset of occlusion of deep venous system. The leg turns edematous and white (alba). As the superficial venous supply occludes, it affects the arterial flow and called plegmasia cerulean dolens as there are signs of leg ischemia. MayThurner syndrome is a rare condition in which blood clots occur in the iliofemoral vein due to compression of the common venous outflow tract of the left lower extremity.
To cite this abstract:Roer D, Sohail M. A Case of Maythurner Syndrome Associated with Phlegmasia Cerulea Dolens. Abstract published at Hospital Medicine 2012, April 1-4, San Diego, Calif. Abstract 97765. Journal of Hospital Medicine. 2012; 7 (suppl 2). https://www.shmabstracts.com/abstract/a-case-of-maythurner-syndrome-associated-with-phlegmasia-cerulea-dolens/. Accessed September 17, 2019.