A Case of Intestinal Lymphangiectasia

Yim, Hyung Eun; Jung, Min Ji; Yoo, Kee Hwan; Hong, Young Sook; Lee, Joo Won; Kim, Soon Kyum

Abstract

Intestinal lymphangiectasia, one of the protein-losing gastroenteropathies, is an uncommon disease characterized by dilated intestinal lymphatics, enteric protein loss, edema, hypoalbuminemia, and lympocytopenia. Small bowel biopsy and CT have been used to confirm the diagnosis of intestinal lymphangiectasia. Small bowel biopsy shows collections of abnormal dilated lacteals in submucosa with distortion of villi and CT findings have been described as diffuse nodular thickening of the small bowel and as linear hypodense streaking densities in the small bowel caused by dilated lymphatic channels. Demonstration of increased enteric protein loss using 51Cr-, 131I- or 99mTc-labeled albumin, timed measurement of fecal excretion of radioactivity or by measuring fecal clearance of alpha 1-antitrypsin can also help the diagnosis. We experienced a rare case of intestinal lymphangiectasia in an eight year old boy who presented with facial edema, abdominal distension and intermittent diarrhea. We report a patient with intestinal lymphangiectasia, in whom abdominal CT, 99mTc-labeled albumin scintitigraphy, and stool alpha 1-antitrypsin measurement played key roles in determining the diagnosis. A brief review of literature was made.