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Phlegmasia cerulea dolens is the most severe form of phlegmasia. The extremity is grossly edematous, cold, and blue; there are often bullae and petechiae. At this point, the patient is at risk for neurovascular compromise and arterial insufficiency; if not treated, it can progress to venous gangrene involving the skin, subcutaneous tissue, and muscle. When venous gangrene occurs, it has a similar distribution with the cyanosis. Arterial pulses may be present when the venous gangrene is superficial. If the gangrene involves the muscular compartment, it may result in increased compartment pressures and a loss of palpable pulses. The arterial pulses are difficult to appreciate because of the significant edema, and use of a Doppler to detect arterial signals is appropriate.

  • Treatment is initiated with heparin. Thrombolytic therapy is initiated (if there are no absolute contraindications) to decrease the clot burden if there is impending or existing gangrene. After the acute phase, the patient is anticoagulated with low molecular weight heparin (LMWH) such as Lovenox and/or Coumadin. During the infusion of thrombolytics, the patient is in the ICU and monitored closely for bleeding. The patient’s neurologic status should be monitored for change in mental status or headache as there is a potential for intracranial bleeding. The extremity is monitored closely for changes in color, edema, sensation, and movement. Pain must be adequately controlled. Any puncture site is monitored for bleeding. The patient should be on bedrest with limited mobility, and skin integrity must be maintained. Surgical thrombectomy is done infrequently. Waist-high compression garments are used to control the edema. Improvement in the edema usually occurs 6 to 12 months after surgery as the body develops collateral pathways.