Phlegmasia Alba and Cerulea Dolens
- Author: Cassius Iyad Ochoa Chaar, MD, MS, FACS; Chief Editor: Vincent Lopez Rowe, MD more...
Phlegmasia stems from a Greek term (phlegma) meaning inflammation. It has been used in the medical literature in reference to extreme cases of lower-extremity deep venous thrombosis (DVT) that cause critical limb ischemia and possible limb loss. Phlegmasia alba dolens (PAD) describes the patient with swollen and white leg because of early compromise of arterial flow secondary to extensive DVT. This condition is also known as “milk leg,” especially as it affects women in the third trimester of pregnancy or post partum.
Phlegmasia cerulea dolens (PCD) is more advanced and considered a precursor of frank venous gangrene. It is characterized by severe swelling and cyanosis and blue discoloration of the extremity. It was first described by Hildanus in the 16th century. Later, the term PCD was first used by Gregoire in 1938. Its rarity notwithstanding, phlegmasia is a life-threatening condition. It is crucial for nonvascular specialists to be able to recognize this condition promptly and accurately; treatment is time-sensitive.
In PAD, the thrombosis involves only major deep venous channels of the extremity, thus sparing collateral veins. The venous drainage is decreased but still present; the lack of cyanosis differentiates this entity from PCD. In PCD, the thrombosis extends to collateral veins, resulting in severe venous congestion with massive fluid sequestration and more significant edema. Without established gangrene, these phases are reversible if proper measures are taken.
Of PCD cases, 40-60% also have capillary involvement, which results in irreversible venous gangrene that involves the skin, subcutaneous tissue, or muscle. Under these conditions, the hydrostatic pressure in arterial and venous capillaries exceeds the oncotic pressure, causing fluid sequestration in the interstitium. Venous pressure may increase rapidly, as much as 16- to 17-fold within 6 hours.[4, 5]
Fluid sequestration may reach 6-10 L in the affected extremity within days. Circulatory shock, which is present in about one third of patients, and arterial insufficiency may ensue. The exact mechanism for the compromised arterial circulation is debatable but may involve shock, increased venous outflow resistance, and collapse of arterioles due to increased interstitial pressure.
DVT affects as many as 2 million Americans every year. The majority of patients are treated medically, with anticoagulation being the mainstay of therapy to promote thrombus resolution and prevent propagation and embolization. Because phlegmasia is a rare condition, its incidence is unknown. The best estimate is derived from case series of patients treated for extensive DVT. In more recent series, 10-20% of patients undergoing thrombolysis with possible stenting for extensive DVT presented with phlegmasia.[6, 7, 8]
The prevalence is thought to be slightly higher in men than in women, with a male-to-female ratio of 1.5:1.
Despite all the therapeutic modalities currently available (see Treatment), PCD and venous gangrene remain life-threatening and limb-threatening conditions, with overall mortality in the range of 20-40%. Pulmonary embolism (PE) is responsible for 30% of the deaths reported from PCD. Overall, amputation rates of 12-50% have been reported among survivors. The postphlebitic sequelae are apparent in 60-94% of survivors. Strict adherence to the use of long-term compression stockings helps control chronic edema.[9, 3]
Haimovici H. Gangrene of the extremities of venous origin; review of the literature with case reports. Circulation. 1950 Feb. 1 (2):225-40. [Medline].
Gregoire R. La phlebite bleue (phlegmasia caerulea dolens). Presse Med. 1938. 46:1313-5.
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. [Medline].
Oguzkurt L, Ozkan U, Demirturk OS, Gur S. Endovascular treatment of phlegmasia cerulea dolens with impending venous gangrene: manual aspiration thrombectomy as the first-line thrombus removal method. Cardiovasc Intervent Radiol. 2011 Dec. 34 (6):1214-21. [Medline].
Mahomed A, Williams D. Phlegmasia caerulea dolens and venous gangrene. Br J Surg. 1996 Aug. 83 (8):1160-1. [Medline].
Knipp BS, Ferguson E, Williams DM, Dasika NJ, Cwikiel W, Henke PK, et al. Factors associated with outcome after interventional treatment of symptomatic iliac vein compression syndrome. J Vasc Surg. 2007 Oct. 46 (4):743-749. [Medline].
O'Sullivan GJ, Semba CP, Bittner CA, Kee ST, Razavi MK, Sze DY, et al. Endovascular management of iliac vein compression (May-Thurner) syndrome. J Vasc Interv Radiol. 2000 Jul-Aug. 11 (7):823-36. [Medline].
Nagarsheth KH, Sticco C, Aparajita R, Schor J, Singh K, Zia S, et al. Catheter-Directed Therapy is Safe and Effective for the Management of Acute Inferior Vena Cava Thrombosis. Ann Vasc Surg. 2015 Oct. 29 (7):1373-9. [Medline]. [Full Text].
Chinsakchai K, Ten Duis K, Moll FL, de Borst GJ. Trends in management of phlegmasia cerulea dolens. Vasc Endovascular Surg. 2011 Jan. 45 (1):5-14. [Medline].
Warkentin TE. Ischemic Limb Gangrene with Pulses. N Engl J Med. 2015 Aug 13. 373 (7):642-55. [Medline].
Kearon C, Kahn SR, Agnelli G, Goldhaber S, Raskob GE, Comerota AJ, et al. Antithrombotic therapy for venous thromboembolic disease: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th Edition). Chest. 2008 Jun. 133 (6 Suppl):454S-545S. [Medline].
Casey ET, Murad MH, Zumaeta-Garcia M, Elamin MB, Shi Q, Erwin PJ, et al. Treatment of acute iliofemoral deep vein thrombosis. J Vasc Surg. 2012 May. 55 (5):1463-73. [Medline].
Oğuzkurt L, Ozkan U, Gümüş B, Coşkun I, Koca N, Gülcan O. Percutaneous aspiration thrombectomy in the treatment of lower extremity thromboembolic occlusions. Diagn Interv Radiol. 2010 Mar. 16 (1):79-83. [Medline].
Sharifi M, Bay C, Nowroozi S, Bentz S, Valeros G, Memari S. Catheter-directed thrombolysis with argatroban and tPA for massive iliac and femoropopliteal vein thrombosis. Cardiovasc Intervent Radiol. 2013 Dec. 36 (6):1586-90. [Medline].
Working Party on Thrombolysis in the Management of Limb Ischemia. Thrombolysis in the management of lower limb peripheral arterial occlusion--a consensus document. J Vasc Interv Radiol. 2003 Sep. 14 (9 Pt 2):S337-49. [Medline].
Comerota AJ, Aziz F. Acute deep venous thrombosis: surgical and interventional treatment. Rutherford RB, Cronenwett JL, Johnston KW. Rutherford's Vascular Surgery. 7th ed. Philadelphia: WB Saunders; 2010. 793-810.