Blood in the lymph (hemochylia) and intestinal lymphangiectasia associated with Lutembacher's syndrome

A 31 year old man presenting with Lutembacher's syndrome and protein-losing enteropathy is described. Reversal of the protein-losing enteropathy 7 months after cardiac surgery suggested that it was secondary to the cardiac disorder. Spontaneous rupture of a congenital aneurysm of the splenic artery created a spleno-portal arteriovenous fistula which caused a massive sequestration of blood into the dilated abdominal lymphatic system, severe hypovolemic shock and death.The pathogenesis and relation of two rare disorders of the lymphatic system, contamination of the lymph with blood (hemochylia) and intestinal lymphangiectasia are discussed.