What is massive ileofemoral Deep Vein Thrombosis (DVT) phlegmasia cerulea dolens?

From the Guidelines

Massive ileofemoral deep vein thrombosis (DVT) with phlegmasia is a severe and potentially limb-threatening condition that requires immediate medical attention to prevent complications such as venous gangrene, compartment syndrome, and limb loss. This condition is characterized by extensive blood clots in the iliac and femoral veins, causing extreme swelling, pain, and circulatory compromise in the affected leg. Phlegmasia refers to the severe manifestation of DVT, with phlegmasia cerulea dolens being the most serious form, characterized by bluish discoloration (cyanosis), severe pain, and significant swelling 1.

Key Considerations

• The treatment of massive ileofemoral DVT with phlegmasia typically involves systemic anticoagulation with heparin, along with consideration for more aggressive interventions such as catheter-directed thrombolysis, pharmacomechanical thrombectomy, or surgical thrombectomy in severe cases 1.
• Patients require hospitalization, elevation of the affected limb, pain management, and close monitoring for complications.
• The underlying cause of the extensive clotting should be investigated, as it may be associated with malignancy, thrombophilia, or other hypercoagulable states.
• Long-term anticoagulation for at least 3-6 months is necessary following the acute phase to prevent recurrence.

Treatment Options

• Systemic anticoagulation with heparin (unfractionated heparin at 80 units/kg bolus followed by 18 units/kg/hour infusion, adjusted to maintain aPTT 1.5-2.5 times normal) 1.
• Catheter-directed thrombolysis (using agents like alteplase at 0.5-1 mg/hour for 12-24 hours) 1.
• Pharmacomechanical thrombectomy or surgical thrombectomy in severe cases 1.

Recent Guidelines

• The NCCN Guidelines (Version 2.2024) recommend considering catheter-directed therapy (pharmacomechanical thrombolysis or mechanical thrombectomy) in appropriate candidates with proximal lower extremity DVT, including those with phlegmasia cerulea dolens 1.

From the Research

Definition of Massive Ileofemoral DVT Phlegmasia

• Massive ileofemoral DVT phlegmasia, also known as phlegmasia cerulea dolens (PCD), is a rare and severe complication of deep vein thrombosis (DVT) 2.
• It is characterized by a massive iliofemoral venous thrombosis that can lead to life- or limb-threatening conditions 2, 3.
• PCD is a condition where the leg turns blue or purple due to the lack of oxygen and nutrients, and it requires immediate attention and surgical intervention to prevent limb loss 2.

Causes and Risk Factors

• PCD can occur as a result of extensive DVT, which can be caused by various factors such as immobility, surgery, or trauma 2.
• The cessation of anticoagulant therapy, such as warfarin, can also increase the risk of developing PCD 2.
• Other risk factors include age, bedridden status, and underlying medical conditions such as dementia, hypertension, and atrial fibrillation 2.

Treatment Options

• Treatment options for PCD include catheter-directed thrombolysis (CDT), percutaneous mechanical thrombectomy (PMT), and surgical intervention 4, 3.
• CDT with argatroban and tissue plasminogen activator (tPA) has been shown to be a highly effective and safe approach for treating massive DVT 4.
• Surgical embolectomy is also a viable option in specific scenarios, such as simultaneous PCD and acute limb ischemia 3.
• Urgent fasciotomies can prevent limb amputation and mortality in cases of compartment syndrome secondary to PCD 5.