Superior Vena Cava Syndrome in a Child with Steroid Resistant Nephrotic Syndrome; A Case Report and Review of Literature

Abstract

Abstract: Nephrotic syndrome (NS) is one of the risk factors for thromboembolism (TE). We report here a rare case of a 6.5-year-old boy who presented with superior vena cava syndrome (SVCS) secondary to thrombosis during relapse of NS. He was diagnosed with primary steroid resistant nephrotic syndrome (SRNS) and was maintaining partial remission with triple regime immunosuppressive treatment for the last one year. He presented in the emergency department with generalized body swelling, more marked on face and neck for last one week. Initially managed as relapse of nephrotic syndrome but it was observed that his face and neck edema instead of disappearing, increased despite his pedal edema improving. This raised suspicion of neck vein thrombosis. Ultrasound doppler revealed sluggish flow in the internal jugular vein (IJV) and superior vena cava (SVC). CT angiogram further confirmed thrombosis in IJV extending to SVC. The patient was successfully treated with low molecular weight (LMW) heparin followed by maintenance anticoagulant therapy with rivaroxaban, a factor X inhibitor. Early consideration and intervention may prevent morbidity and mortality.