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).

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

• inferior vena cava filter

• trauma

• venous stasis

Clinical presentation

Phlegmasia cerulea dolens is characterised 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 oedema, and a secondary compartment syndrome with obstruction of arterial supply, and ultimately ischaemic 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 oedema

• 20-50% mortality ³

• amputation rate as high as 25% in survivors ⁴

History and etymology

From the Ancient Greek for "inflamed blue oedema".