Phlegmasia Cerulea Dolens: Definition and Clinical Significance
Phlegmasia cerulea dolens (PCD) is a rare and life-threatening vascular emergency representing the most severe form of deep vein thrombosis, characterized by massive edema, severe pain, and cyanotic discoloration of the affected limb due to total or near-total occlusion of both deep and superficial venous systems. 1, 2
Pathophysiology and Clinical Presentation
PCD occurs when extensive venous thrombosis causes complete or near-complete occlusion of both the deep and superficial venous systems, leading to severe venous congestion and fluid sequestration 2
The massive venous hypertension can progress to arterial compromise through compartment syndrome, resulting in tissue ischemia and potential gangrene 2, 3
The condition carries a mortality rate of up to 40% and an amputation rate of up to 50% if not treated urgently 4, 3
Key Distinguishing Features
The triad of massive edema, cyanotic (blue) discoloration, and severe pain defines PCD clinically 1, 2
The American College of Radiology emphasizes differentiating PCD from acute arterial ischemia by the presence of massive edema accompanying the cyanotic discoloration 1, 4
PCD most commonly affects the left lower extremity, though upper extremity cases can occur, particularly in patients with indwelling devices like pacemakers 3, 5, 6
Predisposing Factors
Major risk factors include malignancy, hypercoagulable states, congestive heart failure, pregnancy, prolonged immobilization, and surgeries on the affected limb 7
Cancer patients are at particularly high risk, with cases reported in cervical cancer patients developing superior vena cava involvement 5
Clinical Urgency
PCD represents a true vascular emergency requiring immediate intervention to prevent progression to venous gangrene and limb loss 1, 4
The condition can progress from phlegmasia alba dolens (painful white leg from venous thrombosis) to PCD when cyanosis, worsening pain, loss of arterial pulses, or neurological deficits develop 2
Close monitoring for signs of compartment syndrome is essential, as fasciotomy may be required emergently 1, 2, 4