A 30‐year‐old man with history of HIV and metastatic Kaposi sarcoma presented with 3 days of worsening pain, bilateral swelling, and progressive purplish discoloration of his lower extremities. Initial blood pressure was 95/56 mm Hg, heart rate 138 beats/minute, and respiratory rate 22/minute. Both legs were markedly swollen, dusky, and tender to midthigh. Bilateral lower‐extremity pulses were nonpalpable but could not be fully assessed due to the swelling. During initial evaluation the patient had a pulseless cardiac arrest necessitating CPR and intubation. Lower‐extremity ultrasound with Doppler revealed extensive deep venous thromboses extending from the bilateral common femoral veins to the inferior vena cava, which, given the patient's hemodynamic state, was consistent with phlegmasia cerulea dolens (PCD). The patient was started on IV fluid and heparin, and soon after, norepinephrine was added for pressure support. The dusky, purplish lower‐extremity discoloration continued to progress proximally up to his lower abdomen (Figure). As preparation for emergent thrombectomy was initiated the patient had another pulseless cardiac arrest and could not be resuscitated.
PCD consists of a triad of severe extremity swelling, cyanosis, and pain, providing the basis for its name. PCD is caused by large deep venous thromboses, typically of the lower extremities, which create severe, sudden venous hypertension leading to tissue ischemia, massive interstitial fluid accumulation, and, potentially, circulatory collapse. Risk factors include malignancy, pregnancy, surgery, recent venous instrumentation, and conditions predisposing to hypercoagulability. Patients present with tense, mottled, purple extremities (bilateral in 26%) that progress proximally at varying rates. Although diagnosis can often be made on clinical presentation alone, Doppler ultrasound represents a rapid, noninvasive confirmatory test. PCD is divided into 3 stages: noncomplicated (cyanosis only); impending venous gangrene (blistering skin, diminished distal pulses, and decreased sensory/motor function); and venous gangrene (absent distal pulses and no sensory/motor function accompanied by gangrene). With each stage there is a progressive increase in amputation (7%, 20%, and 43%, respectively) and mortality (0%, 20%, and 57%, respectively). Early intervention consisting of extremity elevation, IV fluid and heparin administration is imperative. Patients should be monitored for signs of compartment syndrome. If there is no improvement within 6‐12 hours, catheter‐directed thrombolysis, angioplasty, or surgical thrombectomy should be considered.
PCD should be suspected in patients with severe extremity swelling, cyanosis, and pain.