Phlegmasia cerulea dolens: therapeutic considerations.

Phlegmasia cerulea dolens (PCD) is a rare venous disorder that continues to be a major therapeutic challenge. We reviewed 16 cases of PCD treated during the past 15 years; 11 of the patients were male, and the average age of all the patients was 59 years. Malignant disease was the most common underlying condition (seven patients). Venous gangrene (VG) was present in seven extremities. Three treatment methods were used alone or in combination--intravenous heparin, venous thrombectomy, and thrombolytic therapy. Heparin was used initially in 13 patients; it yielded a successful result in seven (53%) patients, none of whom had VG. Venous thrombectomy was done in six patients; in three it was the primary procedure, in two it followed failure of heparin, and in one it followed failure of both heparin and thrombolytic therapy. Venous thrombectomy was successful in three (50%) patients, one of whom had early VG. Thrombolytic therapy was used on one occasion in conjunction with both heparin and venous thrombectomy, without benefit. Five patients died, all with VG, three after heparin only, one after heparin and venous thrombectomy, and one after all three treatment methods. Review of the 38 cases reported in the recent literature shows comparable results. These data suggest that nongangrenous forms of PCD respond well to systemic anticoagulation. Combination therapy using venous thrombectomy and heparin is indicated for severe ischemia, early VG, or failure of PCD to improve after six to 12 hours of heparin therapy. Phlegmasia cerulea dolens with VG is the lethal form of the entity and responds poorly to established therapy. Future therapeutic trials need to consider aggressive use of thrombolysis with or without thrombectomy.