• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.5
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• pp.413-422
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• 2021

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 short-segment 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.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.2
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• pp.253-259
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• 2004

Primary intestinal lymphangiectasia is a congenital lymphatic disorder in which intestinal lymphatic channels are dilated and ruptured resulting in loss of protein, lipid, and lymphocyte into the intestine or peritoneum. As a result, hypoalbuminemia, generalized edema, diarrhea are clinically manifested. We report a case of primary intestinal lymphangiectasia with generalized edema which occurred in a 7-year old boy who was treated with lipid restriction diet with medium chain triglyceride oil supplement.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.2 no.1
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• pp.93-98
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• 1999

We experienced a case of primary intestinal lymphangiectasia in a 20-month-old boy, which was confirmed gastrofiberscopically and histologically only after a high-fat meal before the procedure. A characteristic endoscopic finding was the appearance of tiny white dots scattered in the duodenal mucosa, which were proved dilated lymph vessels in the lamina propria on histological examination. Treatment with low-fat diet and medium chain triglycerides supplementation was satisfactory.