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Phlegmasia Alba Dolens

Phlegmasia alba dolens describes the initial stage in the presentation of deep iliac vein thrombosis with partial impairment of arterial flow resulting in edema and "milky" appearance of the involved limb. Predisposing conditions consist of malignancy, hypercoagulability, ulcerative colitis, heart failure, mitral valve stenosis, pregnancy, oral contraceptive use, surgery, immobilization and antiphospholipid syndrome.


Presentation

Phlegmasia alba dolens is usually seen in the lower extremities, especially in the left leg and consists of edema, pain and white color of the skin (caused by venous stasis, as a consequence of femoral vein thrombosis, thus the term "alba"), because cyanosis has not occurred yet, as phlegmasia alba precedes phlegmasia cerulea and arterial compromise is not as severe. The white color of the skin is caused by edema, not vasospasm, as initially postulated. Symptoms occur abruptly or gradually and progress to weakness, paresthesia, skin lesions like bullae and compartment syndrome, as collateral vessels become affected. The end stage is represented by venous gangrene and shock [1] [2]. Arterial pulses in the affected limb are weak or absent at this stage.

When facing phlegmasia alba dolens patients, the physician should search for and eliminate all the triggering factors highlighted above, keeping in mind that in one tenth of cases a predisposing factor cannot be found. Since malignancy is found in 20-40% of patients, this kind of pathology should be carefully looked for [3].

Clinical examination should differentiate this condition from symmetric peripheral gangrene, characterized by a patent arterial bed, occlusion of venules and capillaries [4] and disseminated intravascular coagulation. However, this disease also involves tegument area not belonging to limbs, making clinical differential diagnosis easier.

If the diagnosis is not established in this stage, the clinical status deteriorates due to progression to complications like pulmonary embolism and venous gangrene, potentially life- threatening conditions [5] [6].

Hypoxemia
  • 61 Solitary Pulmonary Nodule in a 58YearOld Male Case 42 483 Chapter 62 Persistent Jaundice in a 1MonthOld Infant Case 43 497 Chapter 63 Newborn Baby with a Large Abdominal Wall Defect Case 44 503 Chapter 64 Newborn Infant with Labored Breathing and Hypoxemia[books.google.com]
Vietnamese
  • Русский (Russian) ಕನ್ನಡ (Kannada) 한국어 (Korean) עברית (Hebrew) Український (Ukrainian) اردو (Urdu) Magyar (Hungarian) मानक हिन्दी (Hindi) Indonesia (Indonesian) Italiano (Italian) தமிழ் (Tamil) Türkçe (Turkish) తెలుగు (Telugu) ภาษาไทย (Thai) Tiếng Việt (Vietnamese[definitions.net]
Turkish
  • Deutsch (German) العربية (Arabic) Français (French) Русский (Russian) ಕನ್ನಡ (Kannada) 한국어 (Korean) עברית (Hebrew) Український (Ukrainian) اردو (Urdu) Magyar (Hungarian) मानक हिन्दी (Hindi) Indonesia (Indonesian) Italiano (Italian) தமிழ் (Tamil) Türkçe (Turkish[definitions.net]
Italian
  • Japanese) Português (Portuguese) Deutsch (German) العربية (Arabic) Français (French) Русский (Russian) ಕನ್ನಡ (Kannada) 한국어 (Korean) עברית (Hebrew) Український (Ukrainian) اردو (Urdu) Magyar (Hungarian) मानक हिन्दी (Hindi) Indonesia (Indonesian) Italiano (Italian[definitions.net]
Localized Edema
  • What first attracted my attention to phlegmasia alba dolens were its late complications, that is, the states of local edema, induration and ulceration which occur six months, a year, two years, even ten years later.[kundoc.com]
Labored Breathing
  • Breathing and Hypoxemia Case 45 510 Chapter 65 Vomiting in an Infant Case 46 516 Chapter 66 3YearOld Female with a 12Hour Onset of Abdominal Pain Vomiting and Fever of 385C Case 47 523 Chapter 67 4YearOld Boy with a Groin Mass Case 48 529 Chapter 68[books.google.com]
Hematochezia
  • Haug Browse recently published Learning/CME Learning/CME View all learning/CME CME Partial Oral versus Intravenous Antibiotic Treatment of Endocarditis Case 4-2019: An 18-Year-Old Man with Abdominal Pain and Hematochezia Bridging the Gap Challenge Yourself[nejm.org]
Pulse Deficit
  • Arterial pulses may be present when the venous gangrene is superficial; however, gangrene that involves the muscular compartment may result in increased compartment pressures and a pulse deficit.[thehealthscience.com]
Retinal Hemorrhage
  • Bagley Tucatinib, Trastuzumab, and Capecitabine for HER2-Positive Metastatic Breast Cancer Retraction: Retinal Hemorrhage from Blunt Ocular Trauma. N Engl J Med 2019;381:2252. J. Jinagal et al.[nejm.org]
Skin Lesion
  • Symptoms occur abruptly or gradually and progress to weakness, paresthesia, skin lesions like bullae and compartment syndrome, as collateral vessels become affected. The end stage is represented by venous gangrene and shock.[symptoma.com]
Skin Bullae
  • This process can result in massive fluid sequestration and the development of skin bullae and blebs.4 Further progression results in venous gangrene when muscle ischemia becomes infarction.[emra.org]
Excoriation
  • The dead subject presents a remarkable disfiguration of the countenance, tumefaction and discoloration of the external genitals, excoriation, ulcerative destruction of various characters, with or without laceration of the perineum, various vaginal discharges[chestofbooks.com]
Enlarged Uterus
  • In cases of pregnancy, it is most often seen during the third trimester, resulting from a compression of the left common iliac vein against the pelvic rim by the enlarged uterus.[en.wikipedia.org]
Incontinence
  • Monitoring Body Pressure Relief and Positioning Carts and Shelving Charting and Signage Daily Living Diagnostics Dietary Supplies Dining Aids Education and Training EMS Supplies Evaluation Tools Exam Room Supplies Furniture Glucose Monitoring Housekeeping Incontinence[berktree.com]

Workup

The diagnosis of Phlegmasia alba and cerulea dolens is a clinical one, but imaging methods can help establish the presence of venous thrombosis.

The most widespread diagnosis method is ultrasonography, with duplex ultrasonography being more accurate, if available. Deep vein thrombosis is suggested by a dilated, non-compressible vein with no spontaneous blood flow and hyperechoic masses inside, represented by thrombi [6]. Being a portable and noninvasive method, it tends to replace contrast venography, that still represents the gold standard for diagnosis in uncertain cases. Technical difficulties may be encountered when trying to perform a venography, as extensive thrombosis prevents proper visualization of the venous system; in this case, the venous system belonging to the affected leg can be approached via the contralateral femoral vein or via the upper extremity veins in order to obtain at least some information about the iliocaval system. In patients where the extent of the thrombosis cannot be properly evaluated, computer tomographic venography and magnetic resonance venography can be performed [5]. These methods have a supplementary advantage, represented by the fact that they can describe associated venous abnormalities or pelvic pathological processes compressing the iliac veins.

Mediastinal Mass
  • Chapter 57 Gastric Bypass Case 39 451 Chapter 58 Coronary Revascularization Case 40 463 Coronary Bypass 470 Chapter 60 Mediastinal Mass in a 61YearOld Male Case 41 475 Chapter 61 Solitary Pulmonary Nodule in a 58YearOld Male Case 42 483 Chapter 62 Persistent[books.google.com]

Treatment

  • [citation needed] Treatment[edit] The mainstay of the treatment of deep vein thrombosis is with anticoagulation, but in very large DVTs various other modalities may be used to reduce the risk of complications.[en.wikipedia.org]
  • Signs and tests : - For information on diagnosis, see: Deep venous thrombosis Treatment : - For information on treatment, see: Deep venous thrombosis Complications : - Continued clotting leads to increased swelling.[indiahospitaltour.com]
  • Treatment of this disease by following drugs: 1.Pulsatilla- It is the chief drug of this disease. If pain starts in the veins and feels coldness in that place; she does not want to cover his legs even after feeling cold in them.[jkhealthworld.com]
  • It is crucial for nonvascular specialists to be able to recognize this condition promptly and accurately; treatment is time-sensitive.[emedicine.com]
  • Furniture Wheelchair Wound & Scar Care Dental Education and Marketing Exam and Hygiene Gifts Home Care and Preventive Office Restorative and Specialties Safety Sterilization Treatment Laboratory Apparel Blood Bank Chemicals Chromatography Clinical Chemistry[berktree.com]

Prognosis

  • Each disease entity is introduced by an illustrative case presentation and is followed by a detailed topic review including clinical features, laboratory and imaging workup, treatment and prognosis, clinical pearls, discussion of differential diagnoses[books.google.com]
  • The prognosis is not very favourable, particularly when the treatment commences late.[chestofbooks.com]
  • […] 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] Prognosis[emedicine.com]

Etiology

  • Etiology The main causative factor in phlegmasia is massive thrombosis and occlusion of major venous channels with significantly compromised venous outflow. Multiple triggering factors exist.[thehealthscience.com]
  • Applicable To Deep vein thrombosis, postpartum Pelvic thrombophlebitis, postpartum Use Additional Use Additional Help Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10data.com]
  • It reviews the epidemiology, pathophysiology, etiology, physical examination, imaging modalities, diagnosis, and treatment of limb ischemia.[books.google.it]

Epidemiology

  • Epidemiology 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.[emedicine.com]
  • Epidemiology Frequency More than 600,000 cases of venous thromboembolism are estimated to occur annually in the United States.[thehealthscience.com]
Sex distribution
Age distribution

Pathophysiology

  • In addition, this book provides a basic knowledge of the pathophysiology of vascular disease, interventional techniques, and endovascular modalities of various disease categories.[books.google.ro]
  • Pathophysiology 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.[emedicine.com]
  • Pathophysiology In phlegmasia alba dolens, the thrombosis involves only major deep venous channels of the extremity, therefore sparing collateral veins.[thehealthscience.com]

Prevention

  • The next step in the disease progression is occlusion of the superficial venous system, thereby preventing all venous outflow from the extremity. At this stage it is called phlegmasia cerulea dolens.[en.wikipedia.org]
  • Technical difficulties may be encountered when trying to perform a venography, as extensive thrombosis prevents proper visualization of the venous system; in this case, the venous system belonging to the affected leg can be approached via the contralateral[symptoma.com]
  • A low-dose heparin drip is given at constant rate through the access sheath to prevent thrombosis around the sheath. The patient goes back for angiographic checks on a daily basis.[emedicine.medscape.com]
  • Lippincott Williams & Wilkins, ١٣‏/١١‏/٢٠١٢ - 884 من الصفحات Complications in Surgery, Second Edition offers authoritative recommendations for preventing and managing complications in all current general surgery procedures.[books.google.com]

References

Article

  1. Hasegawa S, Aoyama T, Kakinoki R, et al. Bilateral phlegmasia dolens associated with Trousseau's syndrome: a case report. Arch Phys Med Rehabil. 2008;89(6):1187-1190.
  2. Bhardwaj R, Kandoria A, Sharma RK, et al. A case of venous gangrene, treated successfully with thrombolytic therapy and skin grafting. J Assoc Physicians India 2008;56:640-642.
  3. Chinsakchai K, Ten Duis K, Moll FL, et al. Trends in management of phlegmasia cerulea dolens. Vasc Endovascular Surg. 2011; 45(1):5-14.
  4. Warkentin TE. Ischemic Limb Gangrene with Pulses. N Engl J Med. 2015;373(7):642-655.
  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;22(4):223-230.
  6. Fraser JD, Anderson DR. Deep venous thrombosis: recent advances and optimal investigation with US. Radiology 1999;211(1):9-24.

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Last updated: 2019-07-11 20:50