II. 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

III. 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

IV. Differential Diagnosis

V. Imaging

  1. Extremity CT Venogram (preferred)
  2. Extremity MR Venogram

VI. 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

VII. Prognosis

  1. Untreated Phlegmasia (esp. cerulea) is associated with very high mortality (20 to 50%)

VIII. 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

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Related Studies

Ontology: Phlegmasia Alba Dolens (C0031556)

Definition (MSH) Inflammation that is characterized by swollen, pale, and painful limb. It is usually caused by DEEP VEIN THROMBOSIS in a FEMORAL VEIN, following PARTURITION or an illness. This condition is also called milk leg or white leg.
Concepts Disease or Syndrome (T047)
MSH D013924
SnomedCT 66923004
Portuguese Flegmasia Alba Dolens, Phlegmasia alba dolens
Spanish Flegmasia Alba Dolens, Flegmasia alba dolens, flegmasía alba dolorosa, pierna blanca dolorosa, pierna de leche, flegmasia alba dolens, flegmasia alba dolens (trastorno), flegmasía alba dolorosa (trastorno), leucoflegmasia dolorosa, leucoflegmasía dolorosa, flegmasia alba dolorosa
French Phlegmasia alba dolens, Phlegmatia alba dolens
German Phlegmasia alba dolens
Dutch phlegmasia alba dolens, alba dolens; phlegmasia, phlegmasia; alba dolens, Phlegmasia alba dolens
Japanese ユウツウセイハクコシュ, 有痛性白股腫
Czech Phlegmasia alba dolens, phlegmasia alba dolens
Italian Flegmasia alba dolens, Phlegmasia alba dolens
English legs white, leg white, phlegmasia alba dolens, Dolens, Phlegmasia Alba, phlegmasia alba dolens (diagnosis), Milk leg syndrome, Phlegmasia alba dolens, White leg, Phlegmasia alba dolens (disorder), phlegmasia; alba dolens, alba dolens; phlegmasia, white leg, Phlegmasia Alba Dolens
Hungarian Phlegmasia alba dolens
Norwegian Phlegmasia alba dolens

Ontology: Phlegmasia cerulea dolens (C0238367)

Concepts Disease or Syndrome (T047)
SnomedCT 66877004
English PHLEGMASIA CERULEA DOLENS, Phlegmasia caerulea dolens, phlegmasia cerulea dolens, phlegmasia cerula dolens, venous thrombosis deep vessels of lower extremity phlegmasia cerulea dolens, Phlegmasia cerulea dolens (diagnosis), Phlegmasia cerulea dolens, Blue phlebitis, Phlegmasia caerula dolens, Phlegmasia cerula dolens, Phlegmasia cerulea dolens (disorder), cerulea dolens; phlegmasia, phlegmasia; cerulea dolens
Dutch flegmasia cerulea dolens, flegmasia caerulea dolens, cerulea dolens; phlegmasia, phlegmasia; cerulea dolens
French Phlegmatia coerulea dolens, Phlegmatia caerulea dolens
German Phlegmasia coerulea dolens, Phlegmasia cerulea dolens
Italian Flegmasia cerulea dolens
Portuguese Flegmasia cerulea dolens, Flegmasia caerulea dolens
Spanish Flegmasía cerulea dolens, Flegmasia cerúlea dolens, flegmasia cerúlea dolens (trastorno), flegmasia cerúlea dolens, flegmasia cerúlea dolorosa, flegmasia cerúlea dolorosa (trastorno), pierna dolorosa azul
Japanese 有痛性青股腫, ユウツウセイセイコシュ
Czech Phlegmasia caerulea dolens, Phlegmasia cerulea dolens
Hungarian Phlegmasia cerulea dolens, Phlegmasia caerulea dolens

Ontology: alba dolens; phlegmasia, nonpuerperal (C1408363)

Concepts Disease or Syndrome (T047)
SnomedCT 155454004, 266328001
English Milk-leg, non puerperal, nonpuerperal; milk leg, phlegmasia; alba dolens, nonpuerperal, alba dolens; phlegmasia, nonpuerperal
Dutch alba dolens; phlegmasia, niet-puerperaal, niet-puerperaal; kraambeen, phlegmasia; alba dolens, niet-puerperaal