Phlegmasia Cerulea Dolens With Compartment Syndrome.

Abstract

Introduction: Phlegmasia cerulea dolens (PCD) is a rare, severe form of deep vein thrombosis (DVT) characterized by extensive venous thrombosis and significant venous congestion, leading to swelling, cyanosis, and severe pain. Unlike typical DVT, PCD causes secondary arterial compromise via small arteriole collapse, resulting in critical limb ischemia and potential venous gangrene. This compromise, coupled with increased compartment pressures and fluid sequestration, makes PCD a true emergency requiring immediate intervention to prevent irreversible damage and limb loss.

Case presentation: A 58-year-old woman with a complex medical history presented with severe left hip and lumbar pain following trauma. Examination revealed a swollen, cold, cyanotic, and painful left leg with weak pulses indicating compromised blood flow. Computed tomography confirmed extensive DVT from the left common iliac to the tibial veins, consistent with PCD and concerning for compartment syndrome. Given the critical presentation, fasciotomy was performed without obtaining compartment pressure measurements. The patient received anticoagulation and underwent inferior vena cava filter placement, attempted thrombectomy, and subsequent fasciotomy. Despite these measures, persistent ischemia necessitated a left above-the-knee amputation. Incidental cholangiocarcinoma was subsequently diagnosed, likely contributing to her thrombotic condition.

Conclusions: This case highlights the need for prompt diagnosis and intervention in PCD and its progression to compartment syndrome. Underlying malignancy can complicate the clinical course, necessitating a multidisciplinary approach to care. Immediate anticoagulation, thrombus removal, and supportive measures are essential in preventing further complications and improving outcomes.