Advances in the treatment of phlegmasia cerulea dolens

Abstract

Phlegmasia cerulea dolens (PCD) is an uncommon, severe form of lower extremity deep venous thrombosis characterized by extremity swelling, cyanosis, and pain. Progression of the thrombotic process may result in extremity gangrene, amputation, and death. The relative value of specific therapeutic regimens in the treatment of this disease remains uncertain. Twelve patients, 9 females and 3 males, with PCD were treated during a 10-year period. Eighteen lower extremities were involved. Pre-existing conditions included malignancy (eight), postoperative state (four), diabetes (three), previous deep venous thrombosis (three), and hypercoagulation (two). Venous gangrene was present in four patients. All patients were treated initially with bedrest, fluid resuscitation, extremity elevation, and systemic high-dose heparin therapy. Five patients had complete resolution with this regimen alone. One patient required cessation of heparin therapy due to heparin-induced thrombocytopenia and developed gangrenous toes. Two patients whose condition failed to respond to heparin therapy underwent catheter-based delivery of urokinase with marked clinical improvement. Four patients, two with venous gangrene, died, three of whom had disseminated malignant disease. A significant percentage of patients with PCD will respond to extremity elevation, fluid resuscitation, and aggressive systemic anticoagulation therapy. Thrombolytic therapy selectively administered is beneficial in patients whose disease fails to respond promptly. Venous thrombectomy should be reserved for patients with contraindications to thrombolysis.