All issues > Volume 42(10); 1999

Case Report

J Korean Pediatr Soc. 1999;42(10):1452-1458. Published online October 15, 1999.

Follow up of a Case of Primary Intestinal Lymphangiectasia

Su Jeong SJ Ha¹, Soo Jung SJ Lee¹, Dae Kyun DK Koh¹

¹Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea

Correspondence Soo Jung SJ Lee ,Email: 1

Abstract

Primary intestinal lymphangiectasia is a rare congenital disorder characterized by diffuse or localized ectasia of the enteric lymphatics, often in association with lymphatic abnormalities elsewhere in the body. The pathogenesis of these abnormal lymphatic structures is uncertain. Ectatic lymphatics may be located in the mucosa, submucosa, or subserosa, leading to a loss of protein and lymphocytes into the gut or the peritoneal cavity. The mechanism of this lymphatic loss is believed to be due to a rupture of lymphatics across the mucosa with subsequent leakage of the lymph into the bowel lumen. Presentation of primary intestinal lymphangiectasia may occur any time throughout infancy and childhood. Prominent clinical features include protein-losing enteropathy, lymphocytopenia, hypogammaglobulinemia, edema, growth failure, and chronic diarrhea. The mainstay of treatment for intestinal lymphangiectasia is the use of a low-fat, high- protein, medium-chain triglycerides diet. The natural history of this rare disorder is not well known as only a few cases have been reported in the literature. We present herein the follow up of a case with primary intestinal lymphangiectasia who responded to octreotide therapy but did not respond to dietary or antiplasmin therapy.

Keywords :Primary intestinal lymphangiectasia, Protein-losing enteropathy, Octreotide, Antiplasmin