Teaching Case: Improvement of Chronic Chylous Pleural Effusion Using a Restricted Fat Diet and Medium Chain Triglycerides in a Patient with Congenital Lymphangiectasia

Abstract

Congenital lymphangiectasia is a rare disease of the lymphatic system often causing respiratory symptoms from chylous pleural effusions. Treatment options for these effusions include drainage by multiple thoracenteses or chest thoracostomy tube placement. Previous studies have reported the efficacy of surgical intervention with adjunctive nutrition therapy using total parenteral nutrition (TPN) in decreasing chylous effusions. Few reports, however, describe the use of very low-fat diets with medium chain triglycerides (MCT) as a therapeutic option. Objective: In order to help manage recurrent pleural effusions in a 28-year-old woman with a history of congenital lymphangiectasia, an oral diet restricted in fat using MCT was administered. Methods: The patient was treated with a very low-fat diet, with fat consumption approximately 5% of total caloric intake using food sources and MCT oil. Her diet was supplemented with a multivitamin and linoleic essential fatty acid. Results: After implementation of this diet, the patient has had a stable, small pleural effusion and has not required repeat thoracentesis for more than 24 months. Conclusion: Oral diet therapy restricted in fat using MCT oil may be a beneficial first intervention before considering the use of long-term TPN in patients with chylous pleural effusions from congenital lymphangiectasia.