Primary Intestinal Lymphangiectasia: A Case Report

Authors: Ridwaan Albeiruti1*, MD, Patrick Gleeson2, MD, Theodore Kelbel3, MD, Tracy Fausnight, MD3

Author Affiliations:

1Department of Medicine, West Virginia University, Morgantown, WV; Department of Internal Medicine, College of Human Medicine, Michigan State University, Grand Rapids, MI, USA; 2Department of Internal Medicine, Temple University, Philadelphia, PA, USA; 3Helen DeVos Children’s Hospital, Spectrum Health, Grand Rapids, MI, USA

Full Text Article PDF

*Corresponding Author: Ridwaan Albeiruti;

Key Words: primary intestinal lymphangiectasia; Waldmann’s disease; protein-losing enteropathy


Primary intestinal lymphangiectasia (Waldmann’s disease) is a rare protein-losing enteropathy which is mostly seen in young children. A 22-month-old male baby presented with a 1-week history of abdominal distension, chronic loose stools, recurrent ear infections, and failure to thrive. He had edematous eyelids and non-pitting edema of his hands and feet. The patient was diagnosed via endoscopic visualization and biopsy of the lymphangiectasia in the small bowel. He was managed through dietary restriction with a high-protein, low-fat diet. The patient subsequently had resolution of the diarrhea and an increase in albumin and total protein on labs. We describe a rare case of primary intestinal lymphangiectasia and highlight its clinical presentation, diagnosis, and treatment.

Published: Spring, 2019


1. Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann’s disease). Orphanet J Rare Dis 2008; 3: 5.
doi: 10.1186/1750-1172-3-5

2. Wen J, Tang Q, Wu J, Wang Y, Cai W. Primary intestinal lymphangiectasia: four case reports and a review of the literature. Dig Dis Sci 2010; 55(12): 3466–72. doi: 10.1007/ s10620-010-1161-1

3. Hokari R, Kitagawa N, Watanabe C, Komoto S, Kurihara C, Okada Y, et al. Changes in regulatory molecules for lymphangiogenesis in intestinal lymphangiectasia with enteric protein loss. J Gastroenterol Hepatol 2008; 23(7 Pt 2): e88–95. doi: 10.1111/j.1440-1746.2007.05225.x

4. Katoch P, Bhardwaj S. Lymphangiectasia of small intestine presenting as intussusception. Indian J Pathol Microbiol 2008; 51(3): 411–12.

5. Dierselhuis MP, Boelens JJ, Versteegh FG, Weemaes C, Wulffraat NM. Recurrent and opportunistic infections in children with primary intestinal lymphangiectasia. J Pediatr Gastroenterol Nutr 2007; 44(3): 382–5. doi: 10.1097/01. mpg.0000233192.77521.2f

6. Ingle SB, Hinge Ingle CR. Primary intestinal lymphangiectasia: minireview. World J Clin Cases 2014; 2(10): 528–33. doi: 10.12998/wjcc.v2.i10.528

7. Xinias I, Mavroudi A, Sapountzi E, Thomaidou A, Fotoulaki M, Kalambakas A, et al. Primary intestinal lymphangiectasia: is it always bad? Two cases with different outcome. Case Rep Gastroenterol 2013; 7(1): 153–63. doi: 10.1159/000348763