Hrev_master Emergency Care Journal 2024; volume 20:12877 [Emergency Care Journal 2024; 20:12877] [page 78] A painful massive cyanotic edema of the left leg Alessandra Marchetti, Guglielmo Guarona, Fabrizio Elia Emergency Medicine, San Giovanni Bosco Hospital, Torino, Italy Question Given these results, which is the possible diagnosis? 1. Cellulitis 2. Lymphedema 3. Phlegmasia Cerulea Dolens 4. Acute arterial occlusion Answer The correct answer is Phlegmasia Cerulea Dolens (PCD). PCD is a rare, life-threatening massive deep vein thrombosis of the limbs, with a higher incidence rate in the lower extremities (90%), particularly the left leg. The iliofemoral segment is almost always involved and occluded in the lower extremities due to the anatom- A 70-year-old woman presented to our Emergency Department because of acute pain and swelling in her left lower limb. She suf- fered from depressive syndrome and hypertension, and she had a previous history of breast and uterine cancer. She denied fever and trauma. Her medication included olmesartan/ hydrochlorothiazide and unspecified antidepressants. Physical examination revealed blood pressure of 90/60 mmHg, heart rate of 78 bpm, peripheral oxygen saturation of 94% in room ambi- ent, and unilateral edema of the left lower limb with bluish skin discoloration, decreased peripheral sensitivity, and weak periph- eral pulses. Laboratory findings showed White Blood Cell count (WBC) 5.53x109/L, Hemoglobin (Hb) 12.5 g/dL, Platelet count (PLT) 137,000/µL, International Normalized Ratio (INR) 1.22, glucose 449 mg/dL, creatinine 1.19 mg/dL (nv 0.5-0.96 mg/dL), C-reactive protein 0.26 mg/dL (nv<1.0 mg/dL), D-dimer 28,920 ng/mL (nv<500 ng/mL), fibrinogen 182 mg/dL (nv 200-400 mg/dL). Correspondence: Alessandra Marchetti, Emergency Medicine, San Giovanni Bosco Hospital, Torino, Italy. Tel.: +39-3206055080. E-mail: alessandra.marchetti93@gmail.com Key words: acute arterial occlusion, cellulitis, lymphedema, deep vein thrombosis. Contributions: all the authors made a substantive intellectual contri- bution. All the authors have read and approved the final version of the manuscript and agreed to be held accountable for all aspects of the work. Conflict of interest: the authors declare no potential conflict of interest. Funding: none. Availability of data and materials: all data underlying the findings are fully available upon reasonable request to the corresponding author. Ethics approval and consent to participate: the article contains no personal medical information, and the patient is not identifiable. The photos are original and there are no copyright constraints. Informed consent: the patient provided consent for access to medical records at the time of admission. Received: 29 July 2024. Accepted: 29 August 2024. Early view: 26 September 2024. This work is licensed under a Creative Commons Attribution 4.0 License (by-nc 4.0). ©Copyright: the Author(s), 2024 Licensee PAGEPress, Italy Emergency Care Journal 2024; 20:12877 doi:10.4081/ecj.2024.12877 Publisher's note: all claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher. Non -co mmerc ial us e o nly ical relationship between the right iliac artery overlying the left iliac vein.1 PCD is characterized by complete obstruction of the venous outflow and consequent increased compartment pressure with limb ischemia and venous gangrene.2 Its most pathognomonic feature is the presence of cyanosis. Paraesthesia and motor weak- ness can be present if edema causes severe arterial compromise and compartment syndrome. PCD is more common in women than men. The highest inci- dence is in the fifth and sixth decades of life. It is a real medical emergency with a high incidence of mortality and limb loss that requires immediate evaluation and treatment.3 The risk of limb amputation and the estimated mortality are 20-50% and 20-40%, respectively.4-6 Given the involvement of the iliofemoral segment, PCD is associated with significant post-thrombotic morbidity and high recurrence rates if not treated adequately. The etiology remains unknown in approximately 10% of cases. The leading causes are malignancy (20-40%), followed by hyper- coagulable disorders, venous stasis or insufficiency, use of hor- monal therapy or oral contraceptives, prolonged immobilization, May-Thurner syndrome (left iliac vein compression between the overlying right iliac artery and the lumbar spine), spinal surgery, trauma, pregnancy, IVC filter placement, central venous catheteri- zation, and others (i.e., inflammatory bowel disease, heart fail- ure).2-8 The American Society of Vascular Surgery suggests remov- ing the thrombus directly (grade 1A) or using medicine to remove the thrombus (grade 2C), if available.7 The evidence regarding PCD management is insufficient, and a universal consensus on a standard procedure has not yet been reached. Consequently, per- sonnel from each center administrates different treatments based on their experience and available resources.2 In this case report, a Computed Tomography (CT) scan of the chest, abdomen, and lower limbs was performed and showed mas- sive venous thrombosis of the left iliac vein and hypoplastic infe- rior vena cava; pulmonary embolism was excluded. Unfractionated heparin was started, and vasopressors were admin- istered because of ongoing hemodynamic instability. The patient underwent pharmaco-mechanical catheter-directed thrombolysis, and a self-expanding stent was placed in the common iliac vein. Unfortunately, the procedure was complicated by hemorrhagic shock and disseminated intravascular coagulation. The patient passed away shortly after admission. References 1. Gardella L, Faulk J. Phlegmasia Alba and Cerulea Dolens. 2022. Available from: https://www.ncbi.nlm.nih.gov/ books/NBK563137/ 2. Said A, Sahlieh A, Sayed L. A comparative analysis of the effi- cacy and safety of therapeutic interventions in phlegmasia cerulea dolens. Phlebology 2021;36:392-400. 3. Chinsakchai K, Ten Duis K, Moll FL, de Borst GJ. Trends in management of phlegmasia cerulea dolens. Vasc Endovascular Surg 2011;45:5-14. 4. Oguzkurt L, Tercan F, Ozkan U. Manual aspiration thrombec- tomy with stent placement: rapid and effective treatment for phlegmasia cerulea dolens with impending venous gangrene. Cardiovasc Intervent Radiol 2008;31:205-8. 5. Oguzkurt L, Ozkan U, Demirturk OS, Gur S. Endovascular treatment of phlegmasia cerulea dolens with impending venous gangrene: manual aspiration thrombectomy as the first-line thrombus removal method. Cardiovasc Intervent Radiol 2011;34:1214-21. 6. Elsaid AS, Alqattan AS, Elashaal E, et al. The ugly face of deep vein thrombosis: Phlegmasia cerulea dolens – case report. Int J Surg Case Rep 2019;59:107-10. 7. Meissner MH, Gloviczki P, Comerota AJ, et al. Early thrombus removal strategies for acute deep venous thrombosis: clinical practice guidelines of the Society for Vascular Surgery and the American Venous Forum. J Vasc Surg 2012;55:1449–146. 8. Mumoli N, Invernizzi C, Luschi R, et al. Phlegmasia cerulea dolens. Circulation 2012;125:1056-7. Images in emergency [page 79] [Emergency Care Journal 2024; 20:12877] Non -co mmerc ial us e o nly