Congenital malformation of the lymphatics of the small intestine.

Of 300 congenital malformations of the lymphatics of the small intestine investigated, 120 were operated upon. Intestinal lymphography shows no injection of the cisterna chyli and histology proves that the mesenteric lymph nodes are abnormal. The induced hyperlipidemia test permits a biochemical diagnosis. Modifications of the flow of the chyle secondary to the hypoplasia of the cisterna chyli were studied: (1) in the abdominal cavity, (2) in the extraperitoneal region and the lower limb, (3) in the thorax, especially the chyle drainage channels from the diaphragm towards the cervical region. Our investigations have established that the following diseases are produced by malformation of the lymphatics of the small intestine: protein losing enteropathy, chyloperitoneum, chyluria, lymphedema with chyle reflux, chylothorax, chylopericardium, chyle reflux in the pulmonary lymphatics, hypoproteinemia and food allergies. A better understanding of the pathophysiology of the malformations of the intestinal lymphatics permits a more rational treatment of the diseases produced by this anomaly.