Congenital intestinal lymphangiectasia: report of a case

ABSTRACT

Congenital intestinal lymphangiectasia is a rare protein-losing enteropathy that usually affects children and young adults. Major symptoms include peripheral edema, mild non-bloody diarrhea, and chylous effusions that may develop during the course of the disease. In this disorder intestinal lymphatic vessels show fibrous occlusions that lead to pressure elevation of the lymphatic flow and rupture of the small lymphatic vessels. Transudation of lymph fluid into the different layers of the intestinal wall and lumen then occurs