eMedicine Specialties > Vascular Surgery > Medical Topics

Phlegmasia Alba and Cerulea Dolens: Treatment

Author: Alan Dardik, MD, PhD, FACS, Associate Professor, Department of Surgery, Section of Vascular Surgery, Yale University School of Medicine
Coauthor(s): Dina Rahhal, MD, Postdoctoral Fellow in Transplant Immunology, Institute for Cellular Therapeutics, University of Louisville
Contributor Information and Disclosures

Updated: Sep 10, 2009

Treatment

Medical Therapy

The standard treatment of phlegmasia and venous gangrene is still evolving. The optimal therapeutic modality remains under debate. So far, the results of treatment have been moderately successful. For phlegmasia alba dolens and mild nongangrenous forms of phlegmasia cerulea dolens (PCD), conservative medical treatment, such as steep limb elevation, anticoagulation with intravenous administration of heparin, and fluid resuscitation, should be the initial course of therapy.

Initiate heparin administration with an intravenous bolus of 80-100 U/kg, followed by a continuous infusion of 15-18 U/kg/h. Frequently monitor the activated partial thromboplastin time (aPTT), with a goal range of 2-2.5 times the laboratory reference range. Frequently monitor platelet counts to allow the early detection of heparin-induced thrombocytopenia.

The purpose of rapid heparin anticoagulation is to decrease the risk of proximal clot propagation or thromboembolism. Heparin does not directly affect limb swelling. The best nonsurgical method to decrease edema is steep leg elevation.

Recent studies have demonstrated that low molecular weight heparins are safe and effective in the treatment of proximal deep venous thrombosis (DVT) and pulmonary embolism (PE); however, no good evidence supports the use of these newer agents in phlegmasia and venous gangrene.³

If heparin-induced thrombocytopenia occurs, immediately discontinue the use of heparin and replace it with an alternative anticoagulant. Danaparoid and lepirudin are effective alternative agents; however, heparin-associated antibodies exhibit a 10-19% cross-reactivity with danaparoid. Thus, perform cross-reactivity testing before the initiation of danaparoid in patients with these antibodies. Lepirudin is a direct thrombin inhibitor that does not demonstrate any cross-reactivity. The recommended dosage of lepirudin in patients without renal failure is 0.4 mg/kg as an intravenous bolus followed by a continuous infusion of 0.15 mg/kg/h. Use aPTT to monitor therapy, with a goal range of 2-2.5 times the laboratory reference range.

Continue long-term anticoagulation with warfarin (or other coumarin derivatives) for at least 6 months. Life-long anticoagulation is recommended in patients with hypercoagulable states.

Patients should wear long-term prescription compression stockings with at least 30-40 mm Hg of graded pressure. Many physicians erroneously have the patient fitted for a prescription stocking while the limb is still severely edematous. Instead, the patient may use nonprescription stockings or an elastic bandage, in combination with elevation, to minimize edema prior to being fit for a prescription stocking.

Surgical Therapy

Surgical thrombectomy performed through a femoral venotomy allows instant decompression of the venous hypertension. An intraoperative Trendelenburg position may be used to decrease the risk of PE. Transabdominal cavotomy and thrombectomy is an alternative approach that permits better control of the cava above the thrombus and, thus, provides protection against PE. Procedures that have been performed in an effort to decrease the rethrombosis rate include (1) cross-pubic vein-to-vein reconstruction with polytetrafluoroethylene (PTFE) or the greater saphenous vein (GSV) or (2) the creation of an arteriovenous fistula between the femoral artery and the GSV. These adjuvant procedures may be especially beneficial in cases that involve proximal iliofemoral vein constriction, damage, or external compression.

Concomitant administration of heparin and long-term anticoagulation are mandatory. Regardless, thrombectomy in patients with PCD is associated with a high rate of rethrombosis. Surgical thrombectomy cannot open the small venules that are affected in venous gangrene, and it does not prevent valvular incompetence or postphlebitic syndrome. The incidence of postphlebitic syndrome may be as high as 94% among survivors.

For the above reasons, thrombolysis seems to be an attractive alternative in the management of PCD and venous gangrene. In 1970, Paquet was the first to use thrombolysis for the treatment of PCD.⁴ Some authors propose catheter-directed thrombolysis directly into the vein with high doses of urokinase or tissue plasminogen activator (t-PA). Other authors support the method of intra-arterial low-dose thrombolysis via the common femoral artery, reasoning that the arterial route delivers the thrombolytic agent to the arterial capillaries and, subsequently, to the venules. The intra-arterial approach seems to be more effective in cases with venous gangrene. Systemic thrombolysis has also been used. Many authors have strongly recommended the insertion of a vena caval filter prior to initiation of thrombolytic therapy. Combine thrombolysis with heparin administration and long-term oral anticoagulation.

Fasciotomy alone or in conjunction with thrombectomy or thrombolysis reduces compartmental pressures; however, it significantly increases morbidity because of the prolonged wound healing and the risk of infection.

Finally, if all efforts fail and amputation is required, delay the procedure as long as possible. Take all precautions to reduce edema, allow venous channels to recanalize, and allow necrotic tissue to demarcate.

Preoperative Details

Patients who require emergent venous thrombectomy should have heparin continued throughout the perioperative period. Banked red blood cells should be available. The proximal extent of the thrombosis must be defined using a combination of venous ultrasonography for infrainguinal veins and retrograde venography of the iliac veins and inferior vena cava using a jugular or contralateral femoral approach. If the thrombosis extends into the iliac veins and vena cava, preparations should be made to control the cava via a right retroperitoneal incision. A high-quality fluoroscopy unit should be available to aid in catheter manipulation and completion venography.

Intraoperative Details

Operative exposure depends on the proximal and distal extent of the thrombus. The involved veins should be controlled proximally and distally prior to venotomy.

Iliac venous thrombectomy should be performed with large-bore thrombectomy balloon catheters (as large as 10F). Extension of the thrombus into the inferior vena cava may require proximal control of the cava. The anesthesiologist should apply positive airway pressure while the thrombus is being extracted from the iliocaval system. Digital subtraction venography should be performed to ascertain completeness of the thrombectomy.

Infrainguinal extraction of the thrombus is aided by the intraoperative placement of an Esmarch bandage from foot to thigh. Thrombectomy balloon catheters (3F and 4F) are passed through the femoral veins and, possibly, the posterior tibial veins as well.

Thrombolytic agents may be administered intraoperatively through the posterior tibial veins. t-PA is the most commonly used agent and may be administered intraoperatively.

After the thrombectomy is performed, an arteriovenous fistula should be constructed, connecting the proximal greater saphenous vein or one of its larger tributaries to the superficial femoral artery in an end-to-side fashion.

Completion venography should be performed to exclude the presence of residual thrombus or proximal venous stenosis. If one is present, balloon angioplasty with or without stent placement may be necessary.

When percutaneous endovascular therapy is performed as a single treatment modality, and many centers are now reporting this as a first-line therapy, the popliteal veins are usually accessed with duplex ultrasonography as an aid. Prone positioning is rarely necessary. If extensive thrombus is present, access via the posterior tibial vein is usually successful. A 6-F sheath is usually adequate. An infusion wire is passed through the thrombus just to its proximal extent, often into the vena cava. Infusion is usually performed in the most proximal segment first, usually in the iliac veins. 

A common protocol is to infuse tPA (1 mg/hr) through the infusion wire as well as through the sheath for 24 hours, then to change the sheath perfusion to lower dose heparin after 24 hours. The infusion is then performed in the superficial femoral and popliteal vein segments. Clinical improvement is often noted with clearing of the profunda venous segment. Performance of simultaneous percutaneous mechanical thrombectomy is controversial and may not give better results than postprocedure balloon dilation.

Postoperative Details

Intravenous heparin is administered throughout the postoperative period to prolong the aPTT (2-2.5 times the reference range for aPTT). This is continued until the patient is adequately anticoagulated with warfarin or one of the coumarin derivatives (international normalized ratio [INR] range of 2.0-3.0). The optimal duration of oral anticoagulation is not established.

A sequential compression device is also placed, or, at a minimum, an ace bandage is placed for control of edema. Once the edema is at its minimum, the patient may be fitted for a thigh-length compression stocking. Ambulation is encouraged, if the patient is able.

Complications

The incidence of postphlebitic syndrome may be as high as 94% among survivors. Pulmonary embolism is common, and prophylactic placement of an inferior vena cava filter is recommended in most cases. Thrombectomy in patients with phlegmasia cerulea dolens (PCD) is associated with a high rate of rethrombosis. Amputation and death are common.

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