A 57-year-old male who works as a truck driver with a history of hypertension, type 2 diabetes, and recent COVID-19 infection presents with right lower extremity pain for two hours. He reports experiencing one day of dull aching of the right leg, then being suddenly awakened with the abrupt onset of severe right leg pain and shortness of breath. He denies chest pain. EMS reports a pulseless and painful blue leg en route. The patient denies any history of trauma, irregular heartbeat, or anticoagulation.
Vitals: T 97.5°F; BP 120/78; HR 102; RR 20; oxygen saturation 100%
General: Writhing and moaning in pain
Cardiovascular: Tachycardic; 2+ pulses in all extremities except for the right lower extremity
Pulmonary: No respiratory distress
Hemoglobin: 12.9 g/dL
INR: 1.02
Phlegmasia cerulea dolens
When a patient presents with a painful, pulseless extremity, acute limb ischemia, with etiologies including aortic dissection, arterial thromboembolism, and phlegmasia dolens, is of the highest concern. Unlike a typical deep venous thrombosis (DVT), phlegmasia dolens is a DVT that causes complete occlusion, resulting in venous congestion and hypoperfusion. Risk factors for phlegmasia dolens and DVT are the same; this patient had both a sedentary occupation and recent COVID-19 as risk factors.
Phlegmasia is usually characterized early on with pale discoloration (alba) due to patency of collaterals and later with blue/cyanotic discoloration (cerulea) after complete occlusion of the venous system. It is important to remember this exam finding might be limited in pigmented skin. Venous gangrene and compartment syndrome can be delayed exam findings. The exam should include immediate evaluation of pulses with doppler and compartment checks. Imaging modalities are controversial and should not delay vascular surgery consultation. Bedside ultrasound can be rapidly performed for clot evaluation, but CT venogram would be the preferred method for surgical planning. A common femoral vein DVT can be seen on the accompanied ultrasound and CT images. The limb should be elevated, and heparin infusion should be initiated. Surgical consultation should include a discussion of thrombectomy or catheter-directed thrombolysis.
Take-Home Points
- Phlegmasia cerulea dolens is an uncommon complication of DVT that presents with a discolored, painful, pulseless extremity, and is associated with high morbidity and mortality.
- Initial management includes vascular surgery consultation, elevation of the extremity, and heparinization.
- Baker, William, and Samuel Kim. “Risking Life And Limb: Management Of Phlegmasia AlbaAnd Cerulea Dolens”. Emra.Org, 2020, https://www.emra.org/emresident/article/risking-life-and-limb-management-of-phlegmasia–alba-and-cerulea-dolens/.
- Gardella L, Faulk J. Phlegmasia Alba And Cerulea Dolens. 2020 Oct 12. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan–. PMID: 33085284.
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