Spontaneous Intrahepatic Biliary Duct Perforation in a 15-Month-Old Infant: A Case Report

Spontaneous bile duct perforation (SBDP) in infants is an exceedingly rare occurrence, with this being the first such case reported in the literature. A 15-month-old female infant presented with a 5-day history of progressive abdominal distension, constipation, abdominal pain, and poor feeding. Abdominal ultrasonography demonstrated a large intraperitoneal cystic lesion surrounding the left hepatic lobe. Clinical deterioration within 24 hours prompted a computed tomography scan, which confirmed a large cystic collection at the porta hepatis extending into the lesser sac. Emergency laparotomy revealed approximately 1 liter of bilious fluid within a contained pseudocyst extending from the porta hepatis to the lesser sac, with bile-stained bowel loops, and identified a perforation of the segment 3 intrahepatic bile duct. Intraoperative cholangiography localized the perforation and demonstrated a mildly dilated but otherwise normal biliary tree without distal obstruction or anatomical anomalies. The perforation was primarily repaired with interrupted sutures and reinforced with an omental patch, with placement of a cholecystostomy tube and abdominal drains. The post-operative course was uneventful, with reintroduction of feeds and removal of drains by day 10, and follow-up ultrasonography confirming resolution of fluid collections. Magnetic resonance cholangiopancreatography at 7 weeks showed mild residual biliary dilatation without obstruction or pancreaticobiliary maljunction. The child remained well at 2.5, 6, 12, and 18 months of follow-up, with normal clinical, biochemical, and radiological findings. Biliary perforation should be included in the differential diagnosis of children who develop abdominal pain and are found to have a perihepatic cystic mass.