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].
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.
Treatment
Treatment for Phlegmasia Alba Dolens focuses on restoring blood flow and preventing further clot formation. Anticoagulant medications, such as heparin, are typically administered to thin the blood and prevent new clots. In severe cases, thrombolytic therapy may be used to dissolve existing clots. Compression stockings and leg elevation can help reduce swelling. Surgical intervention may be necessary if there is a risk of tissue damage.
Prognosis
The prognosis for Phlegmasia Alba Dolens varies depending on the severity of the condition and the timeliness of treatment. With prompt and appropriate management, many patients recover without significant complications. However, if left untreated, it can lead to serious outcomes, including tissue death and potential amputation.
Etiology
Phlegmasia Alba Dolens is primarily caused by the formation of a blood clot in the deep veins of the leg. Risk factors include prolonged immobility, recent surgery, cancer, pregnancy, and certain genetic clotting disorders. It is important to identify and manage these risk factors to prevent the condition.
Epidemiology
Phlegmasia Alba Dolens is a rare condition, with a higher incidence in individuals with predisposing factors for DVT. It is more commonly observed in hospitalized patients, particularly those with cancer or undergoing major surgery. The condition can occur in both men and women, although it has historically been associated with postpartum women.
Pathophysiology
The pathophysiology of Phlegmasia Alba Dolens involves the obstruction of venous outflow due to a blood clot. This leads to increased pressure in the veins, causing fluid to leak into surrounding tissues and resulting in swelling. The reduced blood flow causes the skin to appear pale and cold, distinguishing it from other forms of DVT.
Prevention
Preventing Phlegmasia Alba Dolens involves addressing the underlying risk factors for DVT. This includes encouraging mobility, especially after surgery or during long periods of travel, using compression stockings, and managing medical conditions that increase clot risk. In high-risk patients, prophylactic anticoagulation may be considered.
Summary
Phlegmasia Alba Dolens is a serious condition characterized by leg swelling, pain, and pallor due to a blood clot in the deep veins. Prompt diagnosis and treatment are crucial to prevent complications. Understanding the risk factors and pathophysiology can aid in prevention and management.
Patient Information
If you experience sudden leg swelling, pain, and a pale appearance of the skin, it is important to seek medical evaluation. Phlegmasia Alba Dolens is a rare but serious condition that requires immediate attention. Treatment typically involves medications to thin the blood and reduce swelling. Early intervention can lead to a good outcome, so recognizing the symptoms and risk factors is key.
References
- 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.
- 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.
- Chinsakchai K, Ten Duis K, Moll FL, et al. Trends in management of phlegmasia cerulea dolens. Vasc Endovascular Surg. 2011; 45(1):5-14.
- Warkentin TE. Ischemic Limb Gangrene with Pulses. N Engl J Med. 2015;373(7):642-655.
- 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.
- Fraser JD, Anderson DR. Deep venous thrombosis: recent advances and optimal investigation with US. Radiology 1999;211(1):9-24.