A Case of Primary Intestinal Lymphangiectasia

A 26-year-old male patient who had an 8 years history of recurrent peripheral edema with diarrhea and hypoproteinemia was evaluated. Endoscopic jejunal and ileal biopsy revealed markedly dilated mucosal lymph vessels with no evidence of inflammation. (99m)Tc-labeled human serum albumin (HSA) scintigraphy showed significant activity accumulating in the gastrointestinal tract to represent (99m)Tc-HSA leakage into the bowel lumen. A diagnosis of protein losing enteropathy and intestinal lymphangiectasia could be made. After treatment with a high protein and fat restricted diet, his symptoms subsided and the serum protein level was normalized.