Adult Primary Intestinal Lymphangiectasia; A Case Report

Primary intestinal lymphangiectasia is a rare congenital disorder leading to edema, hypoproteinemia, lymphocytopenia, and watery diarrhea. We here report a case of primary intestinal lymphangiectasia in a woman with peripheral edema and recurrent diarrhea in whom laparoscopic biopsy confirmed the diagnosis. In this report, a 21-year-old woman was referred to a tertiary hospital because of abdominal pain, lower extremity edema, and a history of chronic watery diarrhea from childhood. The patient was diagnosed as having protein losing enteropathy secondary to intestinal lymphangiectasia. Diagnosis was confirmed by laparoscopy and multiple deep intestinal biopsies were performed. The diagnosis of primary intestinal lymphangiectasia is usually neglected especially in adults. This differential diagnosis should be considered in any patients with a history of chronic diarrhea and hypoproteinemia. The correct clinical suspicion can properly guide physicians to the correct diagnosis. Diet intervention is the cornerstone of the medical management of primary intestinal lymphangiectasia, which is affected strongly with timely diagnosis.