Images in Emergency Medicine: Phlegmasia Cerulea Dolens



Joel T Levis, MD, PhD, FACEP, FAAEM; Danny L Sam, MD, FACP

Perm J 2013 Winter; 17(1):68

https://doi.org/10.7812/TPP/12-036

Abstract

Phlegmasia cerulea dolens (PCD) is a rare form of massive venous thrombosis of the lower extremities associated with a high degree of morbidity including venous gangrene, compartment syndrome, and arterial compromise.1 Risk factors for PCD include malignancy, immobility, heart failure, heparin-induced thrombocytopenia, prothrombin states (eg, antiphospholipid syndrome), pregnancy, surgery and venous instrumentation (eg, placement of central venous catheters and inferior vena cava filters).2 Clinically, PCD is characterized by sudden pain, swelling, purple ecchymosis, and arterial ischemia with loss of distal pulses.3 Edema develops rapidly, and the skin of the affected extremity is usually tense, firm, and tender to palpation. Doppler ultrasound of the affected extremity should be used to confirm the diagnosis of PCD, and initial treatment includes bed rest, elevation of the affected limb, and systemic anticoagulation with heparin.2 Catheter-directed thrombolysis and venous thrombectomy should also be considered as early treatment options for PCD.4

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References
1.    Bang C. Lower and upper extremity deep venous thrombosis evaluation. In: Dogra V, Rubens DJ, editors. Ultrasound secrets. 1st edition. Philadelphia: Hanley & Belfus; 2004. p 337.
2.    Suwanabol PA, Tefera G, Schwarze ML. Syndromes associated with the deep veins: phlegmasia cerulea dolens, May-Thurner syndrome, and nutcracker syndrome. Perspect Vasc Surg Endovasc Ther 2010 Dec;22(4):223-30. DOI: https://doi.org/10.1177/1531003511400426
3.    Sarwar S, Narra S, Munir A. Phlegmasia cerulea dolens. Tex Heart Inst J 2009;36(1):76-7.
4. Mumoli N, Invernizzi C, Luschi R, Carmignani G, Camaiti A, Cei M. Phlegmasia cerulea dolens. Circulation 2012 Feb 28;125(8):1056-7. DOI: https://doi.org/10.1161/CIRCULATIONAHA.111.051912

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