PHLEGMASIA CERULEA DOLENS ASSOCIATED WITH OBSTRUCTIVE SHOCK

Abstract

TOPIC: Critical Care TYPE: Fellow Case Reports INTRODUCTION: Phlegmasia cerulea dolens (PCD) is a rare, fulminant condition caused by massive venous thrombosis associated with a high morbidity (amputation rates 20-50%) and mortality (25-40%)1. PCD often presents as a triad of limb swelling, acute ischemic pain, and reddish purple to blue skin discoloration1. This case presents a patient who developed PCD involving complete obstruction of the inferior vena cava (IVC) leading to obstructive shock. CASE PRESENTATION: Shortly after admission a 67 year old female with a relevant past medical history of metastatic tonsillar cancer on chemotherapy, Sjogren's disease, and previous venous thromboembolism (no longer on anticoagulation) developed acute onset of abdominal pain and hypotension associated with significant mottling and edema in her bilateral lower extremities. Six months prior to presentation she had an IVC filter placed. She developed worsening shock, acute kidney injury, and metabolic academia. A CT abdomen/pelvis revealed an extensive clot burden distal to the IVC filter with extension down through the bilateral iliac veins (img 1). Bilateral lower extremity venous duplex ultrasound scans showed extensive bilateral acute deep vein thromboses (img 2). Vascular surgery took the patient to the operating room for mechanical thrombectomy of the IVC and left iliac vein (img 3) and right above-the-knee amputation due to the severity of injury to right lower extremity. After returning from the operating room she developed worsening shock despite continued resuscitative measures, and after discussion with the patient's family her goals of care were transitioned to comfort-focused. DISCUSSION: Similar to DVT risk, factors associated with the development of PCD include congestive heart failure, immobility post-operatively, and malignancy1,2. Chemotherapy may increase the risk by up to six to seven times3. Initial management of PCD includes elevation of affected limbs, judicious fluid resuscitation, and anticoagulation1. Definitive therapeutic interventions include catheter-directed thrombolysis and surgical thrombectomy1. IVC filter presence is also a rare but important risk factor for the development of PCD associated with up to 3.2% of cases in one review1. The presence of an IVC filter may lead to the development of inferior vena cava syndrome which is often mistaken for other conditions. Complications associated with IVC filter presence also highlights the value of bedside ultrasound which could help to identify non-collapsible femoral veins. CONCLUSIONS: The clinical triad of PCD includes limb swelling, acute ischemic pain, and reddish purple to blue skin discoloration. Early recognition and treatment is critical to improved outcomes.Risk factors for the development of PCD are the same as those associated with DVT or PE IVC filter presence can be a nidus for thrombus development which can progress to the point of filter occlusion REFERENCE #1: Chinsakchai K, Ten Duis K, Moll FL, de Borst GJ. Trends in management of phlegmasia cerulea dolens. Vasc Endovascular Surg. 2011;45(1):5-14. doi:10.1177/1538574410388309 REFERENCE #2: Mohammed, M., Elhamdani, S., Abusnina, W., Majdi, A., & Yousef, S. (2018). Inferior Vena Cava Obstruction and Shock. Journal of emergencies, trauma, and shock, 11(2), 146–148. https://doi.org/10.4103/JETS.JETS_22_17 REFERENCE #3: Söderman, M., & Grimm, P. (2020). Phlegmasia cerulea dolens in a patient treated with carboplatin. BMJ case reports, 13(4), e233760. https://doi.org/10.1136/bcr-2019-233760 DISCLOSURES: No relevant relationships by Avraham Cooper, source=Web Response No relevant relationships by Sarah MacDowell, source=Web Response