[Idiopathic perforation of the extrahepatic bile ducts in a nursing infant]

The report concerns a two-month-old suckling, affected on the 20th day of life with occurrence of icterus, dark urine and acholic stool, and on the 34th day--fever, vomiting and enlargement of the abdomen. Bile ascites is demonstrated by abdominal puncture, and perforation of the extrahepatic biliary ducts--by hepatobiliary scintigraphy. In the course of emergency laparotomy, diffuse bile peritonitis is established against the background of punctiform perforation at the site of d. cysticus and d. hepaticus communis union. Following sanation of the abdominal cavity, a draining catheter is inserted into the perforation area. The serious postoperative course, characterized by progressive hepatic failure, results in a fatal outcome on the third postoperative day. The etiopathogenesis, clinical symptomatology, timely diagnosis and effective operative management of this exceptionally rare surgical disease in children are discussed against the background of pertinent literature data survey.