Successful Ocreotide and Medium-Chain Triglyceride Therapy for Cylothorax in a Patient with Noonan Syndrome: Case Report

Abstract

Noonan syndrome (NS), is an autosomal dominant disorder commonly seen in childhood and is characterized by short stature, congenital heart defects and facial abnormalities (especially in adults) along with thoracic deformity. Lymphatic dysplasia can be seen with this syndrome causing chylothorax by development of fistulas between thoracic duct and pleural space or it can directly occur by malformation of thoracic lymphatic vessels. Hence, chylothorax can also be seen. Herein, we present a case with this syndrome and chylothorax secondary to possible lymphatic dysplasia. We achieved a great success with ocreotide and medium chain trigliserides in the management of chylothorax and hence suggest this therapy to other clinicians.