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Phlegmasia cerulea dolens: therapeutic considerations
F A Weaver1, P W Meacham, R B Adkins
1Division of Vascular Surgery, Vanderbilt University Medical Center, Nashville, Tenn.
Insights
Phlegmasia cerulea dolens (PCD) is a rare venous disorder. Systemic anticoagulation effectively treats non-gangrenous PCD, while combination therapy is best for severe cases or venous gangrene.
Area of Science:
- Vascular Surgery
- Hematology
- Critical Care Medicine
Background:
- Phlegmasia cerulea dolens (PCD) presents a significant therapeutic challenge due to its rarity and severity.
- Malignant conditions are frequently associated with PCD, impacting patient outcomes.
- Venous gangrene (VG) is a critical complication, significantly increasing mortality.
Purpose of the Study:
- To evaluate the effectiveness of different treatment modalities for Phlegmasia cerulea dolens.
- To identify optimal treatment strategies based on disease severity and presence of venous gangrene.
- To inform future therapeutic trial design for PCD.
Main Methods:
- Retrospective review of 16 PCD cases treated over 15 years.
- Analysis of treatment outcomes with intravenous heparin, venous thrombectomy, and thrombolytic therapy.
- Comparison with 38 cases from recent literature.
Main Results:
- Nongangrenous PCD responded well to systemic anticoagulation (heparin) in 53% of cases.
- Venous thrombectomy, alone or in combination, was successful in 50% of cases, including one with early VG.
- All five patient deaths occurred in those with venous gangrene, irrespective of treatment intensity.
Conclusions:
- Nongangrenous Phlegmasia cerulea dolens demonstrates a favorable response to systemic anticoagulation.
- Combination therapy with venous thrombectomy and heparin is recommended for severe ischemia, early venous gangrene, or refractory cases.
- Phlegmasia cerulea dolens with venous gangrene represents a lethal form with poor response to current therapies, necessitating aggressive future treatment strategies.
Abstract:
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.