Phlegmasia cerulea dolens

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Phlegmasia cerulea dolens
DiseasesDB 32484
MedlinePlus 000200

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Usama Talib, BSc, MD [2]

Overview

Phlegmasia cerulea dolens is a rare but severe form of deep venous thrombosis which results from extensive thrombotic occlusion of the major extremity veins and their collaterals.

Pathogenesis

Phlegmasia cerulea dolens is characterized by sudden severe pain, swelling, cyanosis and edema of the affected limb. Cyanosis is pathognomic for Phlegmasia cerulea dolens. It is a a part of spectrum of changes associated with underlying deep vein thrombos.

  • DVT results in occlusion of the vasculature, thus hindering the normal drainage of deoxygenated blood to the heart.
  • Based on the duration, burden of clot and vascular morphology the overlying skin may appear normal or have whitish or bluish discoloration.
  • Whitish discoloration of skin secondary to an underlying DVT, compromising arterial supply and thus resulting in sequestration of fluid, this is referred as Phlegmasia alba dolens.
  • If not addressed promptly, Phlegmasia alba dolens can progress to complete obstruction of collateral, resulting in venous congestion and bluish discoloration (cyanosis), referred as Phlegmasia cerulea dolens.

There is a high risk of massive pulmonary embolism, even under anticoagulation. Foot gangrene may occur and is often followed by circulatory collapse and shock. Delay in treatment can be fatal or can lead to loss of limb.

Treatment options

  • Phlegmasia cerulea dolens is the only indication of thrombolysis or thrombectomy in patients with deep vein thrombosis.
  • Initiation of heparin followed by assessment for further management with thrombolysis or thrombectomy is done based on clinical presentation.
  • Use of IVC filter is based on physician preference and a consensus does not yet exist on its use.
  • See Deep vein thrombosis

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References

  • Barham, Kalleen (2007 Jan 18). "Images in Clinical Medicine: Phlegmasia Cerulea Dolens". The New England Journal of Medicine. 356 (3): e3. PMID 17229945. Unknown parameter |coauthors= ignored (help); Check date values in: |date= (help)