Chylothorax in Steroid Resistant Nephrotic Syndrome: Difficult to Treat and Challenging Scenario

Abstract

Aim: The aim of the case study was to showcase treatment modalities in a child with nephrotic syndrome who presented with chylothorax. Presentation of Case: An 8-year-old male child, known case of nephrotic syndrome presented with recurrent breathlessness episodes. Initially, he had mild respiratory distress and X-ray showed mild pleural effusion which was managed with antibiotics and other supportive care. In a short time, he again presented with rapidly progressive breathlessness. On examination there was tachypnea, severe retractions, saturation of 80% and bilateral reduced air entry. Chest X-ray showed presence of massive bilateral pleural effusion and immediate pleural tapping was done and about 400-450ml of milky white fluid was drained on each side. Biochemical evaluation of the pleural fluid showed it was sterile with high levels of triglyceride and lymphocytes, which suggested the presence of chyle. The child was initially managed aggressively with intercostal drainage placement and high end antibiotics but later a multidisciplinary approach was required due to the recurrent presentation. Discussion: Steroid-resistant nephrotic syndrome with chylothorax is complex scenario. This case necessitated a multidisciplinary approach. A vital aspect of the management in this case was dietary modification. The child was started on a high calorie high protein low-fat, medium-chain triglyceride (MCT) based diet. MCTs are absorbed directly into the portal system, bypassing the intestinal lymphatics, and thus reducing thoracic duct flow and also takes care of nutritional requirement. Additionally, child was put on anti-tubercular therapy due to significant past history of tuberculosis. Conclusion: Chylothorax can also be a cause of sudden distress in nephrotic syndrome. A thorough, multidisciplinary approach was vital in devising an effective management strategy to treat chylothorax, infection, malnutrition and also steroid resistant nephrotic syndrome. Diet modification played a major role in management.