INTESTINE Vol.21 No.6(2-6)

Theme Reviewing inflammatory diseases of small bowel
Title Clinical features of intestinal lymphangiectasia and treatment
Publish Date 2017/11
Author Masanao Nakamura Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine
Author Naoki Ohmiya Department of Gastroenterology, Fujita Health University School of Medicine
Author Yoshiki Hirooka Department of Endoscopy, Nagoya University Hospital
Author Hidemi Goto Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine
[ Summary ] Intestinal lymphangiectasia (IL) is the primary etiology of protein-losing enteropathy, which is a rare and occasionally life-threatening syndrome characterized by enteric loss of plasma proteins in abnormal amounts. IL can be diagnosed through histopathological findings, which exhibit dilated lymphatic vessels in the small bowel mucosa and submucosa. Double-balloon enteroscopic findings are classified into two categories : the white villi type and the non-white villi type. The white villi type is defined as having white plaques. The white-tipped villi are scattered in the small bowel (termed typical lymphangiectasia). The non-white villi type is often defined as apparently being normal. However under more detailed observation, low and round villi of normal color are diffused in the small bowel. Corticosteroid response is better in the non-white villi type. Treatment for IL includes diet therapy, medication, and surgical treatment, although there is not a good standardized therapy. We should be aware that opportunistic infections occur in IL patients with weakened immune systems.
back