Primary intestinal lymphangiectasia: A rare cause of diarrhea in adults diagnosed by capsule endoscopy and double balloon enteroscopy

Abstract

AbstractPrimary intestinal lymphangiectasia (PIL) or Waldmann’s disease is a rare protein-losing enteropathy presenting with diarrhea. The etiology and prevalence of PIL remain unknown. <200 cases have been reported in the literature so far. Diagnosis of intestinal lymphangiectasia is difficult as there are no serological or radiological tests available. Small bowel imaging modalities like capsule endoscopy and double balloon enteroscopy have increased the chances of diagnosis of PIL due to direct visualization of small bowel. Diagnosis is confirmed by characteristic histopathological finding, which includes dilated intestinal lymphatics with broadened villi of the small bowel. We report a case of a patient with chronic diarrhea who was extensively worked up before he was finally diagnosed to have PIL involving the small bowel by performing balloon enteroscopy-guided biopsy.