Incompetence of the sphincter of Oddi in the newborn.

Abstract

Roentgenographic recognition of air-filled structures in the hepatic area is always an important observation. Gas in the portal vein has been distinguished from gas in the biliary tree by its “peripheral fine radicle distribution” according to Susman and Senturia (8) and “visualization of gas in the outermost 2 cm (of the liver)” by Sisk (7). These criteria have proved reliable in the adult. This report presents two cases of incompetence of the sphincter of Oddi in the newborn, both associated with duodenal atresia and annular pancreas. To the best of my knowledge, this has not been previously reported in the English literature. Furthermore, the established criteria in adults for distinguishing gas in the bile ducts from gas in the portal vein do not seem valid in pediatric patients. Case Reports Case I: A two-day-old girl with persistent bilestained vomiting. A maternal history of excessive weight gain and hydramnios was reported. Physical examination revealed a dehydrated infant with a distended stomach, flat lower abdomen, and hypoplasia of the right radius. X-ray films of the abdomen in the supine and erect positions disclosed a good example of the “double bubble” sign observed in duodenal obstruction. Air in the lower bowel was thought to be secondary to a saline enema (Fig. 1, A). A barium enema examination excluded malrotation and assured patency of the colon (Fig. 1, B). On all films, gas with a branching pattern and peripheral (1 cm from liver margin) distribution was evident in the area of the liver. With a diagnosis of duodenal atresia, laparotomy was undertaken at three days of age. An annular pancreas was identified and an end-to-end duodeno-duodenostomy was performed. There was no evidence of leakage at the end of the procedure. The postoperative course was complicated by recurrent seizures and aspiration pneumonia. No sign remained of the duodenal dilatation or biliary air. No free peritoneal air was evident. Late in the first postoperative day the patient died of cardiac arrest. The pertinent gross autopsy findings included peritonitis with a 1 mm defect in the suture line through which stomach contents poured freely. Bile was easily expressed from the gallbladder into the duodenum through a normal ampulla of Vater. The suture line did not involve the ampulla. Marked hypoplasia of the right radius was present. Case II: A one-day-old female infant admitted because of bile-stained vomiting. Physical examination disclosed signs of mongolism (the mother was thirty-eight years old). X-ray films revealed a typical “double bubble.” Air in the lower gastrointestinal tract was due to a saline enema which had been performed in the referring hospital. An upper gastrointestinal series was undertaken via a nasogastric tube.