• These conditions are rare, and represent the more severe outcomes along the spectrum of DVT.
• They occur predominantly in the lower extremities, but can occur in the upper limbs as well. They are associated with hyper coagulable states including malignancy, pregnancy, recent trauma/surgery, use of exogenous hormones, periods of immobilization, inherited coagulopathies, APAS, cigarette smoking etc. (think Virchow’s triad of hyper-coagulability)
• The names describe the conditions: phlegmasia means swollen, alba means white, cerulean is a deep blue colour, and dolens means painful.
• They exist on a spectrum of severity with Alba Dolens occurring first, where there is extensive proximal occlusive clot most commonly in the ilio-femoral segment, with sparing of collaterals, therefore cyanosis does not occur. This has been colloquially referred to as “milk leg” because of the pale colour the leg turns. It has some association with pregnancy and is more common in the left leg due to the anatomy of the iliac vein and artery leading to greater compression of the venous return on the left side (May Thurner syndrome).
• Phlegmasia Cerulea Dolens is a progression of Phlegmasia Alba Dolens where the collaterals have also become fully occluded, therefore causing cyanosis.
• This extensive venous congestion leads to extravasation, and thirds spacing as pressure builds up.
• Increasing compartment pressures are eventually greater than the arterial pressure, which leads to hemodynamic instability.
• This condition is an emergency and if untreated leads to gangrene of venous insufficiency, limb loss, hemodynamic instability and collapse.
• Treatment is surgical.

Clinical presentation

• This may initially present similarly to a vascular or neurogenic claudication, so it is important to get a clear history of onset, duration, aggravating and alleviating factors etc.
• Occurs more often in the left leg (3-4x) than right leg.
• Upper limb involvement is very rare, <5% of cases.
• It is slightly more common in men than women.
• Highest incidence occurs between 50-69 years of age.
• Roughly 50% Cerulean Dolens cases are not preceded by Alba Dolens.
• Cyanosis begins distally and progresses proximally, motor and sensory deficits may develop and compartment syndrome can occur. Tissue ischemia occurs as this condition progresses and hemodynamic instability ensues.

• Measure leg circumferences (10cm below the tibial tuberosity). A difference greater than 3cm between calf circumferences suggests increased probability of DVT according to wells criteria (https://www.mdcalc.com/wells-criteria-dvt).
• Consider using a doppler to examine for dorsals pedis, posterior tibialis, and popliteal pulses as swelling may make these difficult to palpate.
• Ask about clotting risk factors and previous history of coagulopathy (consider using Wells DVT criteria to systematically ensure other factors increasings likelihood of coagulopathy have been discussed, link above).
• Look for the “milk leg triad” of Phlegmasia Alba Dolens: pain, swelling, and blanching of skin without cyanosis.
• In Phlegmasia Cerulea Dolens cyanosis happens. Worsening edema and cyanotic skin changes, such as bullae and necrosis, start appearing.
• The best study for evaluation is bedside ultrasound in the emergency department, since it is the most readily available.

• Treatment should not be delayed if suspected. The limb should be elevated to 60 degrees above level of the heart to encourage venous return and decrease edema.
• Anticoagulation should be initiated ideally with unfractionated heparin initial bolus of 10-15 units/kg, then as ongoing infusion titrated to PTT of 1.5-2.0 times lab control.
• If available, vascular surgery or interventional radiology should be contacted for consideration of Catheter directed thrombolysis, or thrombectomy.
• If neither of these are available and transfer is not possible, fibrinolytic agent could be considered if the patient does not have any absolute contraindications.

1. Perkins JM, Magee TR, Galland RB. Phlegmasia caerulea dolens and venous gangrene. Br J Surg. 2005;83(1):19-23.

   
   

2. Haimovici H. The ischemic forms of venous thrombosis, Phlegmasia cerulea dolens, Venous gangrene. J Cardiovasc Surg (Torino). 1965 Sep-Oct;5(6):Suppl:164-73.

   
   

3. Layne Gardella, A., & Faulk Affiliations, J. (2021). Phlegmasia Alba And Cerulea Dolens Continuing Education Activity.

   
   

4. baker, william, & kim, samuel. (2015, February 12). Risking Life and Limb: Management of Phlegmasia Alba and Cerulea Dolens. EMRA. https://www.emra.org/emresident/article/risking-life-and-limb-management-of-phlegmasia–alba-and-cerulea-dolens/

   
   

5. Stapczynski J, Ma J, Cline D, Cydulka R, Meckler G, & Tintinalli, E. (2011). Thromboembolism, Occlusive Arterial Disease. In E. Tintinalli (Ed.), Tintinalli’s (pp. 433–460). Mcgraw Hill.