Extrahepatic bile duct fibroma in an infant: a case report

Abstract Background Extrahepatic bile duct tumor in neonates and infants is extremely rare. Fibroma in neonates and infants has been reported in different body parts but not in the bile duct. We report a 2-month-old girl with common bile duct (CBD) fibroma. Case presentation A 2-month-old girl presented with jaundice and pale stool for 15 days. Her perinatal period was uneventful. On examination, we found the girl deeply icteric and having hepatomegaly but no palpable lump. Laboratory investigations revealed direct hyperbilirubinemia with elevated liver enzymes. Ultrasonography revealed hepatomegaly with focal dilatation of the extrahepatic bile duct (2.1 × 0.5 cm). Our preliminary diagnosis was a type I choledochal cyst. Considering the clinical scenario, we planned for exploration without further imaging. On exploration, we found the liver blackish and a hard mass involving the common CBD and cystic duct. The gall bladder was filled with clear mucus. After excising the mass, the gall bladder, and CBD, we reconstructed the anatomy with Roux-N-Y hepaticojejunostomy. The postoperative period was uneventful except for minor bile leakage during the first four postoperative days, which stopped spontaneously. Histopathology revealed spindle-shaped fibrous tissue admixed with collagenous tissue, compatible with a fibroma. A liver biopsy showed the features of cirrhosis. However, liver function tests were normal 2 weeks after surgery. The baby was thriving at 6 months of follow-up, hepatomegaly was resolved, and there was no clinical or radiological sign of recurrence. Conclusions CBD fibroma is a rare cause of obstructive jaundice in neonates and infants. The earliest possible excision of the tumor and bile drainage restoration are necessary to halt the progression of liver damage.