Phlegmasia Cerulea Dolens: A Contemporary Narrative Review

Abstract

Phlegmasia cerulea dolens (PCD) is the most severe form of deep venous thrombosis, defined by extensive iliofemoral and collateral venous obstruction causing profound venous hypertension, massive edema, and rapidly progressive tissue ischemia. Mortality remains at approximately 20 to 40%, and amputation rates are high when diagnosis or intervention is delayed. This narrative review summarizes current evidence on epidemiology, diagnostic pitfalls, and evolving management strategies. PCD commonly occurs in the context of anatomical venous abnormalities, malignancy, surgery, trauma, pregnancy, or thrombophilia. Clinical features frequently mimic acute arterial occlusion, and color-based descriptors may be unreliable, particularly in patients with darker skin tones. Objective assessment using clinical, imaging, and serial biochemical findings is essential. Immediate anticoagulation is the foundation of early care, but conservative management rarely reverses venous hypertension. Timely escalation to catheter-directed thrombolysis, pharmacomechanical thrombectomy, or combined surgical approaches improves venous patency and limb salvage. Fasciotomy is required when compartment syndrome develops, guided by clinical and biochemical evidence of evolving ischemia. Key knowledge gaps include optimal sequencing of decompression and endovascular therapy, validation of biochemical thresholds for intervention, and development of inclusive diagnostic criteria. Prospective multicenter studies are needed to inform standardized treatment pathways.