Phlegmasa Cerulae Dolens: A case report

Abstract

Introduction

Phlegmasia (/flĕg-mā′zhə/: inflammation) cerulean (/sɪˈruːlɪən/: blue) dolens (/doleō/: pain) is a rare, potential limb-threatening complication of acute deep vein thrombosis characterized by marked swelling of the extremities with pain and cyanosis, which may progress to gangrene with high amputation and mortality rates. PCD is part of a clinical spectrum that ranges from phlegmasia alba dolens to venous gangrene.

Case report

An 80 years old lady was brought to the emergency department for sudden onset of dyspnoea and chest pain. Symptoms were preceded by the sudden onset of right-sided body weakness, which led to her being bedbound for ten days. Local examinations show diffuse swelling down from the thigh with purplish discolouration. However, the distal pulses are preserved with capillary refills time of less than 2 seconds.

Bedside Doppler ultrasound shows long segment thrombosis involving the right femoral until the popliteal vein, extending into the deep saphenous vein proximally. The patient was admitted and started on unfractionated heparin infusion. She was concurrently treated for cerebrovascular accident, aspiration pneumonia plus NSTEMI and was discharged well after ten days of admission with dual antiplatelet and apixaban.

Discussion

Clinical manifestations of PAD may be gradual or fulminant. Most cases are preceded by oedema, pain, and blanching without cyanosis. Diagnosis of PCD can be made on clinical grounds or by Doppler ultrasound, which the latter has the advantage of identifying and determining the extent of occlusion in both the arteries and the veins. The major amputation rate is 20–50%, and the death rate is 25–40%.

The goals of treatment in PCD are thrombus removal to improve venous patency, valve function and decrease the risk of recurrence and post-thrombotic syndrome. The first-line treatment is immediate limb elevation to an angle greater than 60 degrees above the heart level to prevent venous stasis and increase venous return. In PCD without signs of ischemia or gangrene, a systemic anticoagulant such as unfractionated heparin is recommended. However, if signs of arterial compromise are present, urgent referral to the interventional radiologist and vascular surgent for thrombolysis or thrombectomy (surgical or catheter-directed) is required to salvage the limb.