Phlegmasia cerulea dolens
Updates to Article Attributes
Phlegmasia cerulea dolens (Greek: "inflamed blue oedema"(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:
- malignancy
- prothrombotic state
- inferior vena cava filter
- trauma
- venous stasis
Clinical presentation
It is characterised by severe pain,a clinical triad of acute limb swelling, cyanosis, and oedemasevere acute pain.
Pathology
Complete/near-complete thrombotic occlusion of the affectedvenous 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
There is
- IV heparin
- angioplasty +/- stenting
- catheter-directed thrombolysis
- surgical thrombectomy
Complications
- significant risk of massive pulmonary embolism, even with anticoagulation
.Gangrene -
40-60% develop gangrene of the periphery
may occur3- due to increased compartmental pressures
resultingfrom severe venous congestion and oedema
- due to increased compartmental pressures
- 20-50% mortality 3
- amputation rate as high as 25% in survivors 4
History and etymology
From the Ancient Greek for "inflamed blue oedema".
-<p><strong>Phlegmasia cerulea dolens</strong> (Greek: "inflamed blue oedema") is an uncommon complication of <a href="/articles/deep-vein-thrombosis">deep venous thrombosis</a>, which results from extensive thrombotic occlusion of the major and collateral veins of an extremity (usually the legs).</p><h4>Clinical presentation</h4><p>It is characterised by severe pain, swelling, cyanosis, and oedema of the affected limb.</p><h4>Treatment and prognosis</h4><p>There is significant risk of massive <a href="/articles/pulmonary-embolism">pulmonary embolism</a>, even with anticoagulation.</p><p>Gangrene of the periphery may occur due to increased compartmental pressures resulting from severe venous congestion and oedema.</p>- +<p><strong>Phlegmasia cerulea dolens</strong> (PCD) is an uncommon complication of <a href="/articles/deep-vein-thrombosis">deep venous thrombosis</a>, which results from extensive thrombotic occlusion of the major and collateral veins of an extremity (usually the legs).</p><h4>Epidemiology</h4><p>Left leg is more commonly affected than the right <sup>4</sup>.</p><h5>Risk factors</h5><p>Risk factors for progression of deep venous thrombosis to phlegmasia cerulea dolens:</p><ul>
- +<li>malignancy</li>
- +<li>prothrombotic state</li>
- +<li><a href="/articles/vena-cava-filter">inferior vena cava filter</a></li>
- +<li>trauma</li>
- +<li>venous stasis</li>
- +</ul><h4>Clinical presentation</h4><p>It is characterised by a clinical triad of acute limb swelling, cyanosis and severe acute pain.</p><h4>Pathology</h4><p>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.</p><h4>Radiographic features</h4><h5>Doppler venous ultrasound</h5><p>Doppler ultrasound will support the diagnosis demonstrating massive superficial and deep venous thrombotic subtotal/total occlusion.</p><h4>Treatment and prognosis</h4><ul>
- +<li>IV heparin</li>
- +<li>angioplasty +/- stenting</li>
- +<li>catheter-directed thrombolysis</li>
- +<li>surgical thrombectomy</li>
- +</ul><h5>Complications</h5><ul>
- +<li>significant risk of massive <a href="/articles/pulmonary-embolism">pulmonary embolism</a>, even with anticoagulation</li>
- +<li>40-60% develop gangrene of the periphery <sup>3</sup><ul><li>due to increased compartmental pressures from severe venous congestion and oedema</li></ul>
- +</li>
- +<li>20-50% mortality <sup>3</sup>
- +</li>
- +<li>amputation rate as high as 25% in survivors <sup>4</sup>
- +</li>
- +</ul><h4>History and etymology</h4><p>From the Ancient Greek for "inflamed blue oedema".</p>
References changed:
- 2. Ikegami T, Kuriyama A, Matsukubo S. Phlegmasia cerulea dolens. (2016) QJM : monthly journal of the Association of Physicians. 109 (4): 281-2. <a href="https://doi.org/10.1093/qjmed/hcw016">doi:10.1093/qjmed/hcw016</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/26811544">Pubmed</a> <span class="ref_v4"></span>
- 3. Perkins JM, Magee TR, Galland RB. Phlegmasia caerulea dolens and venous gangrene. (1996) The British journal of surgery. 83 (1): 19-23. <a href="https://www.ncbi.nlm.nih.gov/pubmed/8653352">Pubmed</a> <span class="ref_v4"></span>
- 4. Onuoha CU. Phlegmasia Cerulea Dolens: A Rare Clinical Presentation. (2015) The American journal of medicine. 128 (9): e27-8. <a href="https://doi.org/10.1016/j.amjmed.2015.04.009">doi:10.1016/j.amjmed.2015.04.009</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/25910785">Pubmed</a> <span class="ref_v4"></span>