Spontaneous Perforation of the Extrahepatic Biliary Tree in Infancy

Abstract

Spontaneous perforation of the bile duct in infancy is rare, with less than 55 cases described in the literature to date. The authors report the case of a 30-day-old neonate who presented with a 2-week history of progressive abdominal distension and intermittent jaundice. Disofenin technicium 99m sequential scintiscanning provided a preoperative noninvasive confirmation of the diagnosis of biliary ascites secondary to spontaneous perforation of the extrahepatic biliary tract. Distal common bile duct atresia was identified intraoperatively, and end-to-side Roux-en-Y hepaticojejunostomy was performed, with an uneventful postoperative recovery. The etiology, diagnosis, and treatment of spontaneous neonatal biliary perforation is discussed.