Failure of blind small bowel biopsy in the diagnosis of intestinal lymphangiectasia

A case of primary intestinal lymphangiectasia is presented in which multiple blind peroral jejunal biopsies were unable to document any abnormality, despite strongly suggestive clinical history and radiographic findings. Endoscopically directed biopsy was necessary to document the characteristic pathologic lesion. This report documents the importance of endoscopy in the diagnosis of intestinal lymphangiectasia when clinical history is suggestive of intestinal lymphangiectasia but standard small bowel biopsy fails to show any abnormality.