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Visual Diagnosis

Case: A 52-year-old man with history of hypertension presents to the ED with a diffusely swollen, tight, painful, and discolored left leg. Symptoms began suddenly 6 hours earlier while walking his dog, and he denies any trauma or injury. He went to sleep, hoping this leg would improve, but he was woken by severe pain and presented to the ED for evaluation. He now has numbness in the entire extremity, and the discoloration is deeply purple. The patients states that he has had no difficulties with his legs in the past, no claudication, no history of DVT or PE, and no known clotting abnormalities. He also denies tobacco use/abuse, history of varicose veins, or family history of clotting abnormalities. What is the diagnosis?

Acute appendicitis ultrasound in a 13-year-old-boy
Case and photo submitted by Dr. Misha Ownbey.

Diagnosis:

This patient has phlegmasia cerulea dolens (PCD).

PCD is the term used to describe lower-extremity DVT that causes limb ischemia, and is characterized by severe swelling and blue discoloration of the extremity. It is the more severe form of phlegmasia alba dolens (PAD), and can lead to venous gangrene. PAD/PCD mostly affects the lower extremities, with left-side involvement being more common. The condition is more prevalent in males, with the most common risk factor being malignancy. Onset may be gradual or fulminant, and with progression, patients may develop bullae, paresthesias, motor weakness, and compartment syndrome. Forty to 60% of cases have capillary involvement, which may result in irreversible venous gangrene. Amputation rate is 12% to 50%, and overall mortality is 20% to 40%, with pulmonary embolism causing 30% of deaths from PCD. The diagnosis is made clinically in patients who have extensive DVT on imaging. Treatment should be initiated as soon as the diagnosis is suspected, with anticoagulation and elevation of the affected extremity. The goal of anticoagulation is to halt propagation of the thrombus and prevent PE.

Clinical Practice Pearls:

  • Suspect PCD clinically in patients with characteristic findings of severe swelling, blue discoloration, severe pain, and paresthesias in the lower extremity.
  • Males are affected more than females 1.5:1.
  • Initiate treatment early with anticoagulation, elevation of the extremity, pain control, resuscitative measures, and consultation to vascular surgery.
  • Catheter-directed thrombolytic therapy is the mainstay of therapy for extensive DVT that is associated with PCD.
  • May-Thurner syndrome is a rare causative condition. Venous obstruction of the left lower extremity due to compression of the left common iliac vein between the right common iliac artery and a vertebral body.

Futher Reading:

  • Mahomed A, Williams D. Phlegmasia caerulea dolens and venous gangrene. Br J Surg. 1996;83(8):1160-1161.
  • Chinsakchai K, Ten Duis K, Moll FL, de Borst GJ. Trends in management of phlegmasia cerulea dolens. Vasc Endovascular Surg. 2011;45(1):5-14.

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