A case of protein-losing enteropathy caused by intestinal lymphangiectasiain a preterm infant

Intestinal lymphangiectasia is characterized by obstruction of lymph draina ge from the small intestine and lacteal dilation that distorts the villus a rchitecture. Lymphatic vessel obstruction and elevated intestinal lymphatic pressure in turn cause lymphatic leakage into the intestinal lumen, thus r esulting in malabsorption and protein-losing enteropathy. Intestinal lympha ngiectasia can be congenital or secondary to a disease that blocks intestin al lymph drainage. We describe the first case of intestinal lymphangiectasi a in a premature infant. The infant presented with peripheral edema and low serum albumin; high fecal concentration of alpha (1)-antitrypsin documente d intestinal protein loss. Endoscopy showed white opaque spots on the duode nal mucosa, which indicates dilated lacteal vessels. Histology confirmed di lated lacteals and also showed villus blunting. A formula containing a high concentration of medium chain triglycerides resulted in a rapid clinical i mprovement and normalization of biochemical variables. These features shoul d alert neonatologists to the possibility of intestinal lymphangiectasia in newborns with hypoalbuminemia and peripheral edema. The intestinal tract s hould be examined for enteric protein losses if other causes (ie, malnutrit ion and protein loss from other sites) are excluded. The diagnosis rests on jejunal biopsy demonstrating dilated lymphatic lacteal vessels.