A Case of Primary Intestinal Lymphangiectasia

Abstract

A 20-year-old female was admitted to our hospital because of bilateral lower limb edema for 2 months. Her symptom developed one month after the birth of her first child. Laboratory tests on admission showed hypoproteinemia (TP4.6g/dl, Alb2.4g/dl) without proteinuria or liver function disorder. Serum anti-nuclear antibody was negative. Her cardiac finding and thyroid function were normal.
Esophagogastroduodenoscopy showed scattered white spots in the second portion of duodenum. Biopsy specimen of the lesion demonstrated dilated lymphatics in the lamina propria microscopically. Abdominal image 5 minutes after intravenous injection of ¹¹¹In-transferrin revealed a leak of radioisotope into the upper gastrointestinal tract, indicating the presence of protein-losing gastroenteropathy. Other examinations showed neither neoplasm nor inflammatory disease, so she was diagnosed as primary intestinal lymphangiectasia. Elemental enteral alimentation with low fat diet led to increase her serum protein (TP6.0g/dl, Alb3.1g/dl) . Although a few other cases were reported, which presented protein-losing gastroenteropathy due to primary intestinal lymphangiectasia after delivery, the pathogenesis remains unclear.