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).
Left leg is more commonly affected than the right 4.
Risk factors for progression of deep venous thrombosis to phlegmasia cerulea dolens:
- prothrombotic state
- inferior vena cava filter
- venous stasis
It is characterized by a clinical triad of acute limb swelling, cyanosis and severe acute pain.
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.
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
- 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".
- 1. Barham, Kalleen; Tina Shah (2007-01-18). "Images in Clinical Medicine: Phlegmasia Cerulea Dolens". The New England Journal of Medicine. 356 (3): e3. doi:10.1056/NEJMicm054730 - Pubmed citation
- 2. Ikegami T, Kuriyama A, Matsukubo S. Phlegmasia cerulea dolens. (2016) QJM : monthly journal of the Association of Physicians. 109 (4): 281-2. doi:10.1093/qjmed/hcw016 - Pubmed
- 3. Perkins JM, Magee TR, Galland RB. Phlegmasia caerulea dolens and venous gangrene. (1996) The British journal of surgery. 83 (1): 19-23. Pubmed
- 4. Onuoha CU. Phlegmasia Cerulea Dolens: A Rare Clinical Presentation. (2015) The American journal of medicine. 128 (9): e27-8. doi:10.1016/j.amjmed.2015.04.009 - Pubmed