From the Guidelines
Phlegmasia cerulea dolens is a medical emergency that requires immediate anticoagulation and consideration of catheter-directed therapy (CDT) or pharmacomechanical thrombolysis (PMT) in patients at risk of limb loss. This condition is characterized by sudden, painful swelling of the affected limb with a bluish discoloration due to extensive venous thrombosis. The management of phlegmasia cerulea dolens involves a multidisciplinary approach, including anticoagulation, elevation of the affected limb, and pain control 1.
Key Considerations
- Anticoagulation is the cornerstone of treatment, and patients should be started on intravenous unfractionated heparin, followed by oral anticoagulants like warfarin or direct oral anticoagulants 1.
- CDT or PMT should be considered in patients with limb-threatening ischemia, as it can help to rapidly restore blood flow and prevent long-term complications such as postthrombotic syndrome 1.
- Surgical thrombectomy may also be considered in selected patients, particularly those with severe limb ischemia or those who have failed CDT or PMT 1.
- The use of graduated compress stockings (GCS) is not recommended for routine use in patients with deep vein thrombosis, as it has not been shown to reduce the incidence of postthrombotic syndrome 1.
Treatment Approach
- Patients with phlegmasia cerulea dolens should be hospitalized for initial management, with close monitoring of their condition and adjustment of their treatment as needed 1.
- A multidisciplinary team, including vascular surgeons, interventional radiologists, and hematologists, should be involved in the management of these patients to ensure that all treatment options are considered and that the patient receives the best possible care 1.
- The goal of treatment is to rapidly restore blood flow to the affected limb, prevent long-term complications, and improve the patient's quality of life 1.
From the Research
Definition and Characteristics
- Phlegmasia cerulea dolens (PCD) is a rare condition of acute deep vein thrombosis, characterized by marked swelling of the affected limb with pain and cyanosis 2.
- It is associated with high mortality and morbidity rates, and early diagnosis and treatment are crucial for preventing potential limb loss or even death 2, 3.
- The main predisposing factors for the disease are neoformative processes, hypercoagulable states, congestive heart failure, pregnancy, prolonged immobilization, and surgeries on the affected limb 4.
Diagnosis
- The diagnosis of PCD is clinical, and it is associated in most cases with pulmonary embolism 4.
- Point-of-care ultrasound (POCUS) can expedite the diagnosis, leading to earlier life- and limb-saving treatment 5.
- Computed tomography and angiography can also be used to confirm the diagnosis and demonstrate extensive venous occlusion 2.
Treatment
- Treatment options for PCD include anticoagulation with heparin, local thrombolysis, systemic fibrinolysis, surgical thrombectomy, fasciotomy, and inferior vena cava filter 4, 6.
- Catheter-directed thrombolysis (CDT) and mechanical thrombectomy can be effective in treating PCD, and prompt action is essential regardless of the treatment modality 2, 3.
- The choice of treatment depends on the severity of the condition and the experience of the surgeon, and advances in minimally invasive techniques have not yet resulted in outcome improvements compared with traditional surgical thrombectomy 6.
Prognosis and Complications
- PCD is associated with a high rate of amputation and mortality, and underlying malignancy, pulmonary embolism, and PCD severity are the vital factors that predict the outcome of PCD 3, 6.
- Irreversible cases may require amputation, and treatment should be initiated as soon as possible to prevent potential complications 4, 6.