Pediatr Gastroenterol Hepatol Nutr.  2021 Sep;24(5):413-422. 10.5223/pghn.2021.24.5.413.

The Update of Treatment for Primary Intestinal Lymphangiectasia

• Kwon Y
• Kim MJ

• KMID: 2520136
• DOI: http://doi.org/10.5223/pghn.2021.24.5.413

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.

Keyword

Lymphangiectasis; Therapeutic embolization; Octreotide; Sirolimus; Everolimus