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Advances in the treatment of phlegmasia cerulea dolens
D B Hood1, F A Weaver, J G Modrall
1Department of Surgery, University of Southern California, Los Angeles 90033-4612.
Insights
Phlegmasia cerulea dolens (PCD) is a severe deep vein thrombosis. Aggressive anticoagulation and extremity elevation are effective initial treatments, with thrombolysis beneficial for non-responders.
Area of Science:
- Vascular Surgery
- Hematology
Background:
- Phlegmasia cerulea dolens (PCD) is a rare, severe form of lower extremity deep venous thrombosis.
- PCD can lead to limb ischemia, gangrene, amputation, and death.
- Optimal treatment strategies for PCD remain unclear.
Purpose of the Study:
- To evaluate the effectiveness of different therapeutic regimens for phlegmasia cerulea dolens.
- To identify patient characteristics and outcomes associated with PCD treatment.
Main Methods:
- Retrospective review of 12 patients with PCD over 10 years.
- Treatment included bedrest, fluid resuscitation, extremity elevation, heparin, and urokinase therapy.
- Outcomes assessed included resolution, complications, and mortality.
Main Results:
- Eighteen lower extremities were affected in 12 patients.
- Eight patients had comorbidities like malignancy or postoperative status.
- Five patients resolved with anticoagulation alone; two improved with urokinase; four patients died.
Conclusions:
- Initial treatment with extremity elevation, fluid resuscitation, and anticoagulation is effective for many PCD cases.
- Selective thrombolytic therapy improves outcomes in non-responsive patients.
- Venous thrombectomy is a last resort for patients with thrombolysis contraindications.
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.