This week we recap the case of our second of three cases - a 61 year old male who presents to the ED with left leg pain.
The patient notes left leg pain that has been worsening over 2 days and now noticing some discoloration. Of note, he had a remote history of an operative repair of that femur, without peri-operative complications. On physical exam, you are able to palpate a dorsalis pedis and posterior tibial pulse, however notice a dark discoloration of the entire left leg up to the hip.
Phlegmasia Cerulea dolens
“The DVT that screams DVT”, Phlegmasia Cerulea Dolens (PCD) is a feared complication of deep venous thrombosis. This is a clot of the common femoral vein that causes significant edema, arterial vasoconstriction and ultimately gangrene of the soft tissues of the leg.  It is more common in those with concurrent malignancy or genetic predisposion to clotting (Factor V, Protein C or S deficiency, etc). PCD is clinically defined by an extremity which undergoes a dark coloration and is associated with ischemic-type pain in the leg.
Quick facts PCD
Left sided 3-4x more likely (iliac anatomy similar to May Thurner Syndrome)
Most commonly in the 40-50 year old population
Consider malignancy (20-40% with concurrent cancer)
~50% will have preceding phlegmasia cerulea alba (nonischemic version)
Venous duplex is the standard for identification of the clot, however the severity of clot should raise suspicion for a venous compression lesion as well as extending proximal clot, and CT venography is often warranted to evaluate for this. Additional concern for embolic phenomenon and screening for possible PE should be considered.
Indication for Clot Removal
Phlegmasia is an indication for removal of the clot burden, as it can rapidly progress to gangrene, compartment syndrome and limb loss.  This can occur via interventional mechanical removal, systemic or catheter directed thrombolysis depending on characteristics of the patient and the clot and should be done in conjunction with your surgical / radiology specialists. Systemic unfractionated heparin is reasonable initial therapy, although if there is evidence of arterial compromise or lack of improvement in 6-12 hours, invasive thrombolysis measures are warranted. Catheter directed thombolysis has should significant improvement in venous patency with an expected increase in bleeding events. 
Authored by Ryan LaFollette, MD
Chang F, Yeh G. Fulminant phlegmasia cerulea dolens with concurrent cholangiocarcinoma and a lupus anticoagulant: a case report and review of the literature. Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis 2014, 25 (5): 507-11
Lai H, Huang S. New England Journal of Medicine 2018 February 15, 378 (7): 658
Watson L, Broderick C, Armon M. Cochrane Database of Systematic Reviews 2014 January 23, (1): CD002783