All issues > Volume 35(12); 1992

Case Report

J Korean Pediatr Soc. 1992;35(12):1737-1743. Published online December 15, 1992.

A Case of Intestinal Lymphangiectasia

Yae Kyung YK Suh¹, Kyung Hee KH Park¹, Chul Ho CH Jang¹, Bum Soo BS Park¹, Jeong Kee JK Seo¹, Sung Hae SH Park², Je Geun JG Chi², Kyung Mo KM Yeon³, Kwi Won KW Park⁴

¹Department of Pediatrics, Seoul National Universty College of Medicine, Seoul, Korea
²Department of Pathology, Seoul National Universty College of Medicine, Seoul, Korea
³Department of Radiology, Seoul National Universty College of Medicine, Seoul, Korea
⁴Department of Pediatric Surgery, Seoul National Universty College of Medicine, Seoul, Korea

Abstract

Intestinal lymphangiectasia is a primary or secondary disorder of the gastrointestinal tract, which is associated with lymphatic dysfuction and protein-losing enteropathy. It's clinical manifestations vary widely, but the main symptoms are abdominal distention, edema, abdominal pain and growth failure. We described a 15-yr-old boy, who suffered from above symptoms since third year of his age and diagnosed by duodenal endoscopy which showed numerous dilated lymphatics in the propria associated with a mild chronic inflammation. The additional findings were lymphopenia, increased α1-antitrypsin clearance and lymphatic flow obstruction on lymphangiography at thoractic duct-left subclavian vein junction area. The exploratory laparotomy was done and the entire small bowel was severely adhered and encapsulated with fibrinoid membrane, so adhesiolysis was impossible. The medium chain triglyceride was given for his medical treatment and generalized edema with abdominal distension was improved. This is the first biopsy proven case of intestinal lymphangiectasia in Korea. So we report this case with a review of related literatures.

Keywords :Intestinal lymphangiectasia, Protein-losing enteropathy