A Case of Primary Intestinal Lymphangiectasia

  • Rajshri P Damle Assistant professor Department of Pathology ACPM Medical college Dhule 424001
  • Kishor H Suryawanshi
  • N V Dravid
  • D V Newadkar
Keywords: Intestinal lymphangiectasia, Diarrhea, Edema


Primary intestinal lymphangiectasia (PIL) is an uncommon disorder and important cause of protein losing enteropathy due to congenital malformation or obstruction of intestinal lymphatic drainage. No accurate serological or radiological tests available for the diagnosis of intestinal lymphangiectasia. Endoscopy and biopsy are currently the only diagnostic modality with certainty. Hence histopathological examination is the gold standard for the diagnosis of intestinal lymphangiectasia which shows dilated intestinal lymphatics with broadened villi of the small bowel. Herein we report a case of intestinal lymphangiectasia in a 4-year-male child presented with chronic diarrhea and edema. Patient was treated by resection of small intestine and postoperatively patient showed improvement with small bowel movements and disappearance of edema.


1. Waldmann TA, Steinfeld JL, Dutcher TF, Davidson JD, Gordon RS Jr. The role of the gastrointestinal system in “idiopathic hypoproteinemia”. Gastroenterology 1961; 41:197-207

2. Fang YH, Zhang BL, Wu JG, Chen CX. A primary intestinal lymphangiectasia patient diagnosed by capsule endoscopy and confirmed at surgery: A case report. World J Gastroenterol 2007;13: 2263-5.

3. Freeman HJ, Nimmo M. Intestinal lymphangiectasia in adults. World J Gastrointest Oncol 2011; 3:19-23.

4. Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann’s disease). Orphanet J Rare Dis 2008; 3:5

5. Mistilis SP, Skyring AP, Stephen DD. Intestinal lymphangiectasia mechanism of enteric loss of plasma protein and fat. Lancet 1965; 1:77-9.

6. 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;55:3466-72

7. Hauser B, Moreels T, Urbain D, Van Marck V, Pletincx M, Devreker T, et al. Intestinal lymphangiectasia. J Pediatr Gastroenterol Nutr. 2009 ;48(2):125.

8. Levine C. Primary disorders of the lymphatic vessels – A unified concept. J Pediatr Surg 1989; 24: 233-24.
Case Report