The Update of Treatment for Primary Intestinal Lymphangiectasia
10.5223/pghn.2021.24.5.413
- Author:
Yiyoung KWON
1
;
Mi Jin KIM
Author Information
1. Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
- Publication Type:Review Article
- From:Pediatric Gastroenterology, Hepatology & Nutrition
2021;24(5):413-422
- CountryRepublic of Korea
- Language:English
-
Abstract:
Intestinal lymphangiectasia is a rare disease which is causing protein-losing enteropathy.Treatment of intestinal lymphangiectasia can be a challenge for clinicians because of the lack of specific guidelines regarding pharmacological indications. We sought to introduce a diagnostic approach and suggest guidelines for treatment. After exclusion of secondary intestinal lymphangiectasia, magnetic resonance lymphangiography is a promising tool for the assessment of abnormal lymphatic lesions in primary intestinal lymphangiectasia.Determining the extent of the lesion provides direction for treatment options. Focal shortsegment intestinal lymphangiectasia can be treated via intestinal resection or radiologic embolization after dietary therapy failure. Diffuse intestinal lymphangiectasia and extensive lymphangiectasia should be treated with several drugs with a full understanding of their mechanisms.
