Phlegmasia Alba and Cerulea Dolens Treatment & Management

Treatment of phlegmasia alba dolens (PAD) or phlegmasia cerulea dolens (PCD) should be initiated as soon as the diagnosis is suspected. The patient is started on anticoagulation, and the involved extremity is elevated. Intravenous (IV) resuscitation is administered if there is significant fluid sequestration and the patient appears to have intravascular fluid depletion, as manifested by tachycardia, hypotension, and decreased urine output.

Heparin administration is initiated with an IV bolus of 80-100 U/kg, followed by continuous infusion at a rate of of 15-18 U/kg/hr.  The activated partial thromboplastin time (aPTT) should be monitored, with a goal in the range of 2.0-2.5 times the laboratory reference range. Platelet counts should be monitored to allow early detection of heparin-induced thrombocytopenia. Heparin drip is preferred because it has a shorter half-life and can be rapidly titrated in the event of bleeding or need for surgical intervention. The goal of early anticoagulation is to halt thrombus propagation and prevent pulmonary embolism (PE).

Low-molecular-weight heparins (LMWHs) have been found to be safe, effective, and convenient for use in PCD and result in shorter hospital stays because they can be used on an outpatient basis. ^{[12, 13]}

The oral factor Xa inhibitors rivaroxaban and apixaban are approved for treatment of deep venous thrombosis (DVT) and can potentially be used for treatment of phlegmasia. Alternative anticoagulation agents are longer-acting than heparin and should be started when patient is clinically stable and there is no concern for additional interventions.

If patients improve with elevation and anticoagulation and do not progress to critical limb ischemia, compression therapy with stockings or elastic bandages can help decrease edema and swelling and may be used as tolerated. Many physicians have the patient fitted for a prescription stocking while the limb is still severely edematous. This is inadvisable; instead, the patient may use nonprescription stockings or an elastic bandage, in combination with elevation, to minimize edema before being fitted for a prescription stocking.

Patients are treated with anticoagulation for at least 6 months.

Endovascular intervention

Catheter-directed thrombolytic therapy is the mainstay of therapy for the extensive DVT that is usually associated with phlegmasia. It is minimally invasive, effective, and safe, as shown in a 2012 systematic review of the literature. ^[14]

The patient is kept on heparin drip and brought to an angiography suite or a hybrid operating room. Under ultrasonographic guidance, the popliteal vein or a tibial vein is accessed and a 6-French sheath placed. The thrombus is crossed with a 0.035-in. wire, a multihole infusion catheter is placed in the vein, and an infusion of alteplase is started.

The patient is transferred to an intensive care unit (ICU) and typically receives alteplase infusion over a period of 2-4 days. 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.

A combination of pharmacomechanical thrombolysis using devices that macerate and aspirate the clot (eg, AngioJet [Boston Scientific, Marlborough, MA] or Trellis [Covidien, Minneapolis, MN]) or balloon angioplasty to break down the thrombus is performed. Venous occlusive lesions or areas of stenosis can be treated with stenting after dissolution of the acute thrombus.

In patients with tissue compromise because of extensive thrombosis, a more aggressive approach using pharmacomechanical thrombolysis ^[9] or aspiration thrombectomy ^{[5, 15]} during the first angiogram can provide enough outflow to avoid progression of ischemia. Placement of an inferior vena cava (IVC) filter decreases the risk of iatrogenic PE during thrombolysis but has not been shown to affect survival. ^[16]

Catheter-directed thrombolysis is contraindicated in certain patients. ^[17] Absolute contraindications include the folowing:

• Active bleeding
• Recent neurologic surgery (cranial or spinal)
• Trauma or cerebrovascular accident less than 2 months previously
• Brain tumor
• Severe hypertension
• Coagulopathy
• Allergy to thrombolytic agents

Relative contraindications include the following:

• Surgery less than 10 days previously
• Recent trauma or gastrointestinal hemorrhage
• Subacute bacterial endocarditis
• Severe liver or kidney disease
• Pancreatitis
• Pregnancy
• Any other scenario in which there seems to be a risk of hemorrhage that might be either excessive or difficult to control

In a study that included 692 patients with acute proximal DVT, Vedantham et al compared anticoagulation plus pharmacomechanical thrombolysis with anticoagulation alone, the primary outcome being development of the postthrombotic syndrome between 6 and 24 months of follow-up. ^[18] They found that the addition of pharmacomechanical catheter-directed thrombolysis to anticoagulation did not result in a lower risk of the postthrombotic syndrome but did result in a higher risk of major bleeding.

Endovascular iliocaval reconstruction has been described for the treatment of iliocaval thrombosis. ^[19]

Open surgery

Open surgical thrombectomy ^[20] is an alternative treatment for patients who cannot undergo thrombolysis and who require thrombus removal.

The patient is taken to the operating room. Placement of an IVC filter can protect the lungs from embolization during thrombectomy and should be done at the beginning of the procedure. An intraoperative Trendelenburg position may be used to decrease the risk of PE as well.

A longitudinal incision is made in the groin area to explore the femoral vein. A venotomy is performed to decompress the vein and allow passage of a Fogarty balloon catheter antegrade and retrograde in the vein to remove thrombus. Open access of the popliteal or tibial veins is sometimes needed to permit a more extensive thrombectomy and to facilitate pasage of the Fogarty balloons against the valves in the veins. A tourniquet can also be applied on the lower extremities with gradual compression to try to squeeze or “milk” thrombus out of the femoral venotomy. ^[21]

Transabdominal cavotomy and thrombectomy can also be performed. This approach permits better control of the cava above the thrombus and thus provides protection against PE, but it carries a higher morbidity.

Procedures that have been performed in an effort to decrease the rethrombosis rate include cross-pubic vein-to-vein reconstruction with polytetrafluoroethylene (PTFE) or the great saphenous vein (GSV) in conjunction with 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.

Surgical thrombectomy cannot open the small venules that are affected in venous gangrene and is therefore thought to be less effective than thrombolysis in clearance of acute thrombus.

For patients with acute compartment syndrome, ^[22] a four-compartment fasciotomy and decompression are required to prevent muscle necrosis. Finally, if all efforts fail, an amputation is required. It is best to delay the procedure as long as possible so as to reduce edema, allow venous channels to recanalize, and allow necrotic tissue to demarcate.