Congenital Biliary Dilatation With Pre-Existing Diffuse Hepatolithiasis: A Challenging Case

Although postoperative intrahepatic stones are known to occur after surgery for congenital biliary dilation, preoperative diffuse hepatolithiasis is uncommon. We present a challenging case of a 14-year-old female with a Todani type IVa choledochal cyst complicated by numerous stones. She presented with abdominal pain, obstructive jaundice, and pancreatitis. To stabilize her condition, she was initially treated with endoscopic biliary and pancreatic stenting. Subsequently, laparoscopic radical surgery was performed. Although many stones were removed intraoperatively, complete clearance remained elusive despite repeated lavage and cholangioscopy. Following surgery, the patient recovered from a pelvic abscess and was discharged on postoperative Day 17. MRCP at 3 months showed residual stones, although the patient remained asymptomatic. This case highlights the limitations of laparoscopic surgery for congenital biliary dilation with advanced hepatolithiasis. The indications for open surgery should be carefully evaluated, and treatment strategies should anticipate the possibility of postoperative residual stones.