Phlegmasia, Phlegmasia Alba Dolens, Milk Leg Syndrome, Phlegmasia Cerulea Dolens, Copper Leg Syndrome, Venous Gangrene

  • Pathophysiology
  1. Phlegmasia (milk leg)
    1. Venous outflow obstruction
      1. Typically left sided due to left common iliac vein course beneath the left common iliac artery
    2. Progression along a spectrum
      1. Phlegmasia Alba Dolens
      2. Phlegmasia Cerulea Dolens
      3. Venous Gangrene
  2. Phlegmasia Alba Dolens (Milk Leg Syndrome)
    1. Pale white, painful, severely edematous extremity (milk leg)
    2. Proximal deep vein obstruction with patent collateral veins
  3. Phlegmasia Cerulea Dolens (Copper Leg Syndrome)
    1. Cyanotic or blue, severely edematous extremity (copper leg) with obstructed collateral veins
    2. Pain out of proportion to appearance
    3. Like Alba Dolens, Cerulea Dolens is associated with proximal deep vein obstruction
      1. However, collateral or superficial veins are also obstructed leading to greater venous obstruction and Cyanosis
  4. Venous Gangrene
    1. Thrombosis extends into the capillaries
    2. Associated with Septic Shock and need for Limb Amputation
  5. May-Thurner Syndrome
    1. Left common iliac vein compressed by right femoral artery and spurring from Vertebrae
    2. Presents with unilateral left Leg Edema and venous Hypertension
  • Causes
  1. Ilio-femoral Deep Vein Thrombosis
    1. Malignancy
    2. Hypercoagulable State
    3. Trauma
  2. Anatomic Compression
    1. May-Thurner Syndrome (typically left-sided)
    2. Pregnancy
  • Differential Diagnosis
  • Imaging
  1. Extremity CT Venogram (preferred)
  2. Extremity MR Venogram
  • Management
  1. Vascular surgery Consultation in all cases
  2. Phlegmasia Alba Dolens
    1. Medical management in most cases (if DVT)
  3. Phlegmasia Cerulea Dolens
    1. Emergent surgical thrombectomy or
    2. Emergent directed Thrombolytics
  • Prognosis
  1. Untreated Phlegmasia (esp. cerulea) is associated with very high mortality (20 to 50%)
  • References
  1. Jhun and Herbert in Herbert (2014) EM:Rap 14(5): 8
  2. Jean-Louis and Sethuraman (2023) Crit Dec Emerg Med 37(7): 4-11