ABSTRACT

This chapter focuses on the medical, open surgical, and endovascular treatment of chylous disorders. Chylous disorders develop because of abnormal circulation of chyle, the lipid- and protein-rich lymph fluid collected by the mesenteric lymphatic system. Primary chylous disorders are fortunately rare and they are usually caused by congenital lymphangiectasia. Etilefrine, a sympathomimetic drug that causes smooth muscle contraction of the thoracic duct (TD), has helped in the management of post-operative chylous disorders, but has not been used for primary chylous effusions. Pre-operative lymphangiography may localize the site of the chylous fistula or document occlusion of the TD. Thoracentesis is diagnostic but rarely therapeutic, as chyle from the TD or large intercostal, mediastinal, or diaphragmatic collaterals will re-accumulate. Multiple case reports have been published illustrating the role of thoracoscopic TD ligation in the treatment of chylothorax. Percutaneous TD embolization to treat chylothorax due to a leaking TD was first described by Cope in 1998.