Abstract

Background: Primary intestinal lymphangiectasia is a rare disease described in adults and children. It usually presents itself as an excessive protein loss into the gastrointestinal tract due to stasis and rupture of limph vessels; however, few reports in adults and very few in children have described associated obscure gastrointestinal bleeding.

Case report: we report a case of a 1-year-old child who was referred to our hospital for anasarca, significant pallor and weakness. Laboratory tests showed severe iron-deficiency anemia, hypoalbuminemia and hypogammaglobulinemia. No proteinuria was found in his urinalysis. Fecal occult blood was highly positive. Investigations were not indicative of an enteropathy. During diagnostic work-up, a 99mT-DTPA scintigraphy showed an abnormal diffusion of the radioisotope in the small bowel. An intraoperative endoscopy and the histopathology examination of the entero-biopsy specimens of the small bowel were indicative of primary intestinal lymphangiectasia. After two blood and multiple albumin transfusions, the patient was first started on parenteral nutrition and afterwards on a beneficial low-fat, high protein diet and medium chain triglycerides supplementation and discharged.

Conclusion: The current case highlights the importance of considering abnormalities of the lymphatic system as a cause of severe anemia in children.

Keywords:Intestinal lymphangiectasia, Anemia, Protein losing enteropathy, Children

Abbreviations: PIL: Primary Intestinal Lymphangiectasia; GI: Gastrointestinal; MCT: Medium Chain Triglycerides; PLE: Protein Losing Enteropathy; IBD: Inflammatory Bowel Disease