Phlegmasia Alba and Cerulea Dolens

Phlegmasia Alba and Cerulea Dolens

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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. [1] It is characterized by severe swelling and cyanosis and blue discoloration of the extremity. It was first described by Hildanus in the 16th century. [2] Later, the term PCD was first used by Gregoire in 1938. [3] 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. [4] 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. [5, 6]

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. [7, 8, 9]

The prevalence is thought to be slightly higher in men than in women, with a male-to-female ratio of 1.5:1. [10]

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. [10, 4]

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Gregoire R. La phlebite bleue (phlegmasia caerulea dolens). Presse Med. 1938. 46:1313-5.

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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].

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[Guideline] 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].

[Guideline] Guyatt GH, Akl EA, Crowther M, Gutterman DD, Schuünemann HJ, American College of Chest Physicians Antithrombotic Therapy and Prevention of Thrombosis Panel. Executive summary: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest. 2012 Feb. 141 (2 Suppl):7S-47S. [Medline]. [Full Text].

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].

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Hage AN, Srinivasa RN, Abramowitz SD, Cooper KJ, Khaja MS, Barnes GD, et al. Endovascular iliocaval reconstruction for the treatment of iliocaval thrombosis: From imaging to intervention. Vasc Med. 2018 Jun. 23 (3):267-275. [Medline].

Comerota AJ, Aziz F. Acute deep venous thrombosis: surgical and interventional treatment. Cronenwett JL, Johnston KW, eds. Rutherford’s Vascular Surgery. 8th ed. Philadelphia: Elsevier Saunders; 2014. Vol 1: 792-810.

Cassius Iyad Ochoa Chaar, MD, MS, FACS Assistant Professor of Surgery, Section of Vascular Surgery, Yale University School of Medicine

Cassius Iyad Ochoa Chaar, MD, MS, FACS is a member of the following medical societies: American College of Surgeons, American Venous Forum, Association for Academic Surgery, Eastern Vascular Society, European Society for Vascular Surgery, New England Society for Vascular Surgery, Society for Vascular Surgery, Vascular and Endovascular Surgery Society

Disclosure: Nothing to disclose.

Savni Satoskar, MBBS Postdoctoral Fellow, Vascular Surgery Division, Yale School of Medicine

Savni Satoskar, MBBS is a member of the following medical societies: Indian Medical Association

Disclosure: Nothing to disclose.

Alan Dardik, MD, PhD, FACS Associate Professor, Department of Surgery, Section of Vascular Surgery, Yale University School of Medicine

Alan Dardik, MD, PhD, FACS is a member of the following medical societies: American College of Surgeons, Association for Academic Surgery, Association of VA Surgeons, Phi Beta Kappa, Society for Vascular Surgery, American Society for Cell Biology, Vascular and Endovascular Surgery Society, Eastern Vascular Society, Society for Vascular Medicine, Society for Clinical Vascular Surgery

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Vincent Lopez Rowe, MD Professor of Surgery, Program Director, Vascular Surgery Residency, Department of Surgery, Division of Vascular Surgery, Keck School of Medicine of the University of Southern California

Vincent Lopez Rowe, MD is a member of the following medical societies: American College of Surgeons, American Surgical Association, Pacific Coast Surgical Association, Society for Clinical Vascular Surgery, Society for Vascular Surgery, Western Vascular Society

Disclosure: Nothing to disclose.

Vincent Lopez Rowe, MD Professor of Surgery, Program Director, Vascular Surgery Residency, Department of Surgery, Division of Vascular Surgery, Keck School of Medicine of the University of Southern California

Vincent Lopez Rowe, MD is a member of the following medical societies: American College of Surgeons, American Surgical Association, Pacific Coast Surgical Association, Society for Clinical Vascular Surgery, Society for Vascular Surgery, Western Vascular Society

Disclosure: Nothing to disclose.

William H Pearce, MD Chief, Division of Vascular Surgery, Violet and Charles Baldwin Professor of Vascular Surgery, Department of Surgery, Northwestern University, The Feinberg School of Medicine

William H Pearce, MD is a member of the following medical societies: American College of Surgeons, American Heart Association, American Surgical Association, Association for Academic Surgery, Association of VA Surgeons, Central Surgical Association, New York Academy of Sciences, Society for Vascular Surgery, Society of Critical Care Medicine, Society of University Surgeons, Western Surgical Association

Disclosure: Nothing to disclose.

Timothy Liem, MD Clinical Assistant Professor of Surgery, Oregon Health and Science University; Consulting Surgeon, Department of Surgery, Division of Vascular Surgery, Legacy Emanuel Hospital and Good Samaritan Hospitals

Disclosure: Nothing to disclose.

Dina Rahhal, MD Postdoctoral Fellow in Transplant Immunology, Institute for Cellular Therapeutics, University of Louisville

Disclosure: Nothing to disclose.

Phlegmasia Alba and Cerulea Dolens

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