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Logo of thijTexas Heart Institute JournalSee also Cardiovascular Diseases Journal in PMCSubscribeSubmissionsTHI Journal Website
 
Tex Heart Inst J. 2009; 36(1): 76–77.
PMCID: PMC2676521

Phlegmasia Cerulea Dolens

Raymond F. Stainback, MD, Section Editor
Department of Adult Cardiology, Texas Heart Institute and St. Luke's Episcopal Hospital, 6624 Fannin Street, Suite 2480, Houston, TX 77030

A 71-year-old man was admitted to the hospital for severe community-acquired pneumonia. He had a history of chronic obstructive pulmonary disease and atrial fibrillation and was on chronic warfarin therapy. His international normalized ratio (INR) was 9.05. We started the patient on antibiotics and withheld the warfarin. Blood cultures grew Streptococcus pneumoniae. On the 2nd day of hospitalization, the patient's mental status deteriorated. Therefore, after normalization of the INR with fresh frozen plasma and vitamin K, a lumbar puncture was performed to rule out meningitis. Cerebrospinal fluid analysis showed nothing unusual. On day 3, the patient developed shortness of breath and painful swelling of both lower extremities with bluish discoloration of the skin (Fig. 1A). Later, he developed blisters on his feet and dark blue mottled patches on the soles of both feet (Fig. 1B). Multidetector computed tomographic (CT) pulmonary angiography showed a central filling defect in the superior segment of the right lower pulmonary lobe; delayed images of the pelvis and lower extremities without additional contrast material showed bilateral veno-occlusive disease (Fig. 2). The CT examination enabled rapid diagnosis of both pulmonary embolism and deep venous thrombosis at 1 time. Doppler ultrasonography of the lower extremities confirmed extensive deep venous thrombosis. The diagnosis of phlegmasia cerulea dolens was made. Systemic and local tissue-plasminogen activator infusion and AngioJet® thrombectomy (AngioJet® Rheolytic™ Thrombectomy System, Possis Medical, Inc.; Minneapolis, Minn) were used to improve venous outflow. Anticoagulation was resumed, and the patient's condition improved.

figure 19FF1
Fig. 1 A) Swollen and bluish appearance of the legs. B) Patchy distribution of superficial venous gangrene on the soles of both feet.
figure 19FF2
Fig. 2 A) Computed tomographic pulmonary angiography with contrast material shows a pulmonary embolus (arrow), and B) delayed image of the pelvis shows occluded femoral veins bilaterally (arrows).

Comment

Phlegmasia cerulean dolens is a severe form of deep venous thrombosis characterized by severe venous outflow obstruction, marked limb swelling, pain, bluish discoloration, and even venous gangrene if the condition is untreated.1 Triggering factors include malignancy, femoral vein catheterization, heparin-induced thrombocytopenia,2 antiphospholipid syndrome, surgery, heart failure, and pregnancy. The cause in our patient could have been the rapid reversal of anticoagulation in the setting of decreased mobility and a generalized inflammatory state due to bacteremia. Systemic inflammation has been shown to result in activation of coagulation due to tissue-factor–mediated thrombin generation and down-regulation of physiological anticoagulant mechanisms.3

The development of a standard treatment for phlegmasia cerulea dolens is ongoing. Intervention is usually required in order to prevent gangrene. Treatment consists of a multimodal approach, including systemic anticoagulation, catheter-directed thrombolysis, systemic thrombolysis, surgical thrombectomy, and AngioJet thrombectomy.4,5 The advantages of thrombolysis over the other methods are wider distribution of the thrombolytic agent with blood flow and the resultant beneficial effect on both large and small venous channels. In our patient, the combination of systemic thrombolysis and AngioJet thrombectomy resulted in symptomatic improvement and the prevention of gangrene.

Acknowledgments

The authors thank Drs. Saleem O. Farooqui and Frank D. Parks of the Department of Radiology, University of Tennessee Health Science Center (Memphis) for their contributions to this manuscript.

Footnotes

Address for reprints: Salman Sarwar, MD, The University of Tennessee Health Science Center, 842 Jefferson Ave., Suite A 607, Memphis, TN 38103. E-mail: ude.memtu@rawrass

References

1. Mahomed A, Williams D. Phlegmasia caerulea dolens and venous gangrene. Br J Surg;83(8):1160–1. [PubMed]
2. Warkentin TE, Elavathil LJ, Hayward CP, Johnston MA, Russett JI, Kelton JG. The pathogenesis of venous limb gangrene associated with heparin-induced thrombocytopenia. Ann Intern Med 1997;127(9):804–12. [PubMed]
3. Levi M, van der Poll T, Buller HR. Bidirectional relation between inflammation and coagulation. Circulation 2004;109 (22):2698–704. [PubMed]
4. Patel NH, Plorde JJ, Meissner M. Catheter-directed thrombolysis in the treatment of phlegmasia cerulea dolens. Ann Vasc Surg 1998;12(5):471–5. [PubMed]
5. Chaer RA, Dayal R, Lin SC, Trocciola S, Morrissey NJ, McKinsey J, et al. Multimodal therapy for acute and chronic venous thrombotic and occlusive disease. Vasc Endovascular Surg 2005;39(5):375–80. [PubMed]

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