Phlegmasia cerulea dolens (PCD) is an uncommon complication of deep venous thrombosis, which results from extensive thrombotic occlusion of the major and collateral veins of an extremity (usually the legs).
On this page:
Epidemiology
Left leg is more commonly affected than the right 4.
Risk factors
Risk factors for progression of deep venous thrombosis to phlegmasia cerulea dolens 5:
malignancy
prothrombotic state
trauma
venous stasis
Clinical presentation
Phlegmasia cerulea dolens is characterized by a clinical triad of 5:
acute limb swelling
cyanosis
severe acute pain
Pathology
Complete/near-complete thrombotic occlusion of the venous drainage of the limb leads to massive fluid stasis and edema, and a secondary compartment syndrome with obstruction of arterial supply, and ultimately ischemic injury.
Radiographic features
Doppler venous ultrasound
Doppler ultrasound will support the diagnosis demonstrating massive superficial and deep venous thrombotic subtotal/total occlusion.
Treatment and prognosis
IV heparin
angioplasty +/- stenting
catheter-directed thrombolysis
surgical thrombectomy
Complications
significant risk of massive pulmonary embolism, even with anticoagulation
-
40-60% develop gangrene of the periphery 3
due to increased compartmental pressures from severe venous congestion and edema
20-50% mortality 3
amputation rate as high as 25% in survivors 4
History and etymology
From the Ancient Greek for "inflamed blue edema".