Phlegmasia cerulea dolens: before and after lysis

Abstract

A 77-year-old woman, immobile due to advanced rheumatoid arthritis, presented to the emergency department with a 3-day course of a progressively painful and purple left lower extremity (Fig. 1a). On examination, the limb was discolored, swollen, warmer than the right leg, and very tender. The distal pulses were entirely absent, and only a faint left femoral pulse was palpable. Phlegmasia cerulea dolens (from Latin for inflamed, blue, and painful) was the clinical impression [1] and confirmed with Doppler ultrasound, which demonstrated an occlusive thrombus in the left femoral vein. Sensation and motor function of the limb remained intact. She was started on a heparin drip, and brought to interventional radiology for endovascular therapy. Fig. 1b shows a venogram of the left iliofemoral segment with extensive occlusive deep venous thrombus (DVT) that extended to the left popliteal vein (not shown).Catheter directed lysis was performed overnight resulting in Dopplerable distal pulses and improved symptoms; however, interval venography (not shown) revealed significant residual thrombus burden. The digital subtraction venogram of the left iliofemoral segment in Fig. 1c depicts improved patency and flow following subsequent endovascular mechanical thrombectomy. Fig. 1d shows the improvement in the patient's left leg 12 h later (36 h after presentation). Her pain resolved, and her distal pulses became palpable. She was discharged on warfarin. Although prolonged immobility was the presumptive risk factor for her, consultation with a hematologist/oncologist

Phelgmasia Cerulea Dolens Diagnosed by Point-of-Care Ultrasound

“…1 , 2 Prompt diagnosis and treatment is necessary to return circulation and prevent circulatory collapse, and a delay in treatment may result in loss of the patient’s limb or even death. 3 , 4 The condition can have either a gradual or a fulminant course. 5 …”

Phlegmasia cerulea dolens in a teenage boy found to have Factor V Leiden

“…Diagnosis is performed clinically with ultrasound and venography adjunctively. Treatment involves heparinisation, catheter-directed thrombolysis and thrombectomy 8 9. Non-gangrenous forms of PCD respond well to anticoagulation 10.…”

Phlegmasia cerulea dolens complicated by methylenetetrahydrofolate reductase genetic mutation

“…The PCD is clinically defined as a triad of acute extremity edema, cyanosis, and ischemic pain [5]. Ultrasonography is considered first-line imaging due to sensitivity and specificity in diagnosis of symptomatic proximal DVTs, 97% and 94% respectively; detection of occlusion in arteries and veins; and ability to characterize flow [7,8]. Alternative diagnostic imaging being CT scan and magnetic resonance imaging scan, with catheter venography and arteriography being the gold standard due to ability to confirm diagnosis, help direct treatment from mapping of circulation, and be therapeutic [8].…”