say this 3 times, fast: Phlegmasia Ceruleans Dolens

15 Aug

RAGING HYPOTHETICAL: 

Your next patient is a 32 year old female with history of Factor V Leiden complicated by previous DVT and PE with sudden onset, excruciating right leg pain.  No injuries or trauma to extremity.

On exam, she’s tachycardic, and in extreme distress from her leg pain.  The leg, for the record, is bluish, cold, swollen, extremely tender to palpation, and a tiny bit pulseless.  

What’s going on here?

 

Phlegmasia Ceruleans Dolens

Phlegmasia cerulea dolens (“painful blue edema”) is an uncommon manifestation of deep-vein thrombosis.

often presents in the acute setting with

  • sudden and dramatic onset of intense pain,
  • violaceous cyanosis,
  • woody edema,
  • ecchymoses,
  • loss of arterial pulsations,
  • and sometimes gangrene.

first described by R. Gregoire in 1938.

extremely rare, with less than 100 cases being reported in the literature prior to 1967.

proposed mechanism is a massive thrombosis compromising venous outflow of a limb, which causes ischemia.

exact mechanism for the compromised arterial circulation is debatable but may involve

  • shock,
  • increased venous outflow resistance,
  • collapse of arterioles due to increased interstitial pressure.
  • Vasospasm of the resistance vessels.

 

associated with:

  • malignancy (most often)
  • femoral vein catheterization,
  • IVC filter placement,
  • heparin-induced thrombocytopenia,
  • antiphospholipid syndrome,
  • surgery,
  • heart failure,
  • pregnancy,
  • sepsis.

high probability of developing a PE if not already present at time of diagnosis.

Intervention is usually required in order to prevent gangrene.

Treatment consists of a multimodal approach involving

  • systemic anticoagulation,
  • catheter-directed thrombolysis,
  • systemic thrombolysis,
  • surgical thrombectomy.

 

BOTTOM LINE:

Ultimately it’s a massive DVT and often concurrent PE, but will likely need to evaluate arterial flow since it’s presentation mimics arterial occlusion.

Either way (if there’s arterial occlusion or massive DVT/Phlegmasia Ceruleans Dolens), Vascular and/or IR will need to be involved quickly, and anticoagulation (e.g. heparin) will be part of the treatment course.

Main difference with Phlegmasia Ceruleans Dolens will be thinking about and looking for the DVT/PE.

 

Submitted by K. Dabrowski,

 

References:  Br J Surg. 1996 Jan;83(1):19-23. Phlegmasia caerulea dolens and venous gangrene. Perkins JMMagee TRGalland RB. Nuffield Department of Surgery, University of Oxford, UK.; J Spinal Cord Med. 2008; 31(4): 398–402. PMCID: PMC2582430 Phlegmasia Cerulea Dolens: Rare Complication of Vena Cava Filter Placement in Man With Paraplegia Kazuko Shem, MD; Br Med J. 1967 March 25; 1(5542): 714. PMCID: PMC1840986 Phlegmasia cerulea dolens.; medscape; picture.

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