ADVANCES IN THE TREATMENT OF PHLEGMASIA-CERULEA-DOLENS

Phlegmasia cerulea dolens (PCD) is an uncommon, severe form of lower e xtremity deep venous thrombosis characterized by extremity swelling, c yanosis, and pain. Progression of the thrombotic process may result in extremity gangrene, amputation, and death. The relative value of spec ific therapeutic regimens in the treatment of this disease remains unc ertain. 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 pa tients had complete resolution with this regimen alone. One patient re quired cessation of heparin therapy due to heparin-induced thrombocyto penia and developed gangrenous toes. Two patients whose condition fail ed to respond to heparin therapy underwent catheter-based delivery of urokinase with marked clinical improvement. Four patients, two with ve nous gangrene, died, three of whom had disseminated malignant disease. A significant percentage of patients with PCD will respond to extremi ty elevation, fluid resuscitation, and aggressive systemic anticoagula tion therapy. Thrombolytic therapy selectively administered is benefic ial in patients whose disease fails to respond promptly. Venous thromb ectomy should be reserved for patients with contraindications to throm bolysis.