Phlegmasia Alba and Cerulea Dolens Clinical Presentation

Updated: Aug 13, 2018
  • Author: Cassius Iyad Ochoa Chaar, MD, MS, FACS; Chief Editor: Vincent Lopez Rowe, MD  more...
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Presentation

History and Physical Examination

Phlegmasia alba dolens (PAD) and phlegmasia cerulea dolens (PCD) predominantly affect the lower extremities, with fewer than 5% of cases believed to involve the upper extremities. In the lower extremities, left-side involvement is three to four times more common. PAD is known by the triad of edema, pain, and blanching (alba) without cyanosis.

The onset of symptoms may be gradual or fulminant. PAD precedes PCD in 50-60% of cases. With increasing cyanosis and progression, patients develop bullae, paresthesia, and motor weakness. Compartment syndrome may be precipitated by venous congestion. Venous gangrene and shock are the ultimate killers, and it is vital that patients be treated before reaching that stage.

Malignancy is the most common triggering factor and is present in approximately 20-40% of patients with PCD. [10] The presence of malignancy is also associated with higher mortality. Other associated risk factors include the following:

  • Hypercoagulable syndrome
  • Surgery
  • Inferior vena cava filter insertion
  • May-Thurner syndrome (compression of the left iliac vein by the right iliac artery)

Pregnancy has often been associated with PAD, especially during the third trimester, when the uterus is large enough to compress the left common iliac vein against the pelvic rim (ie, milk leg syndrome). Finally, 10% of patients with phlegmasia have no apparent risk factors.

Another condition that might present with limb ischemia in the setting of patent arterial supply is symmetric peripheral gangrene, a condition in which microvascular occlusion of venules and capillaries occurs in the setting of severe systemic infection and widespread inflammation. [11] In this condition, patients have systemic tissue ischemia and coagulopathy, resulting in autoamputation of distal digits in combination with disseminated intravascular coagulation (DIC). There is cyanotic discoloration of the tips of all four limbs (ie, acral cyanosis), as well as patches of cyanosis throughout the body (ie, nonacral cyanosis or purpura fulminans).