Superior vena cava syndrome presenting as chylothorax

Chylothorax caused by superior vena cava (SVC) syndrome is a rare but potentially life-threatening complication requiring a multidisciplinary diagnosis and management approach. We present a case of a 27-year-old female with end-stage renal disease who developed chylothorax secondary to SVC syndrome caused by venous stenosis from a tunneled hemodialysis (HD) catheter. The patient had a history of ongoing hemodialysis through a tunneled catheter placed in the right internal jugular vein approximately seven months before presentation. She presented with dyspnea, chest pain, and a large left-sided pleural effusion. A multidisciplinary diagnostic workup and management included 2 thoracentesis, pleural fluid studies, serial radiological tests, right and left heart catheterizations, and blood serum studies with flow cytometry. They revealed that SVC stenosis around the hemodialysis catheter was causing the patient's pathology. The patient underwent veno-plasty of the right SVC and brachiocephalic veins and replacement of HD catheter leading to the resolution of the chylothorax and significant improvement in respiratory symptoms. This report will highlight the approach to diagnosing and managing chylothorax and a review of existing medical literature.