Pancreatic Head Cancer and Intrahepatic Cholangiocarcinoma Occurring After Surgery for Congenital Biliary Dilatation Associated With Pancreaticobiliary Maljunction: A Case Report

TL;DR

A patient with a history of congenital biliary dilatation and pancreaticobiliary maljunction developed two cancers years after surgery, but remained cancer-free following complex reoperations.

Contribution

Demonstrates the long-term cancer risk in CBD/PBM patients and the feasibility of reusing a preserved Roux limb in complex reoperations.

Findings

A patient with congenital biliary dilatation developed pancreatic head cancer and intrahepatic cholangiocarcinoma years after initial surgery.

Reusing a preserved Roux limb during complex reoperations proved feasible and effective.

Patients with CBD and PBM remain at lifelong risk of malignancy, especially if surgery is performed in adulthood.

Abstract

Congenital biliary dilatation (CBD), frequently associated with pancreaticobiliary maljunction (PBM), predisposes patients to biliary tract malignancies due to chronic reflux of pancreatic juice into the biliary epithelium. Although biliary diversion reduces carcinogenic risk, malignancy may still arise many years after surgery. A 69-year-old woman underwent choledochal cyst excision and Roux-en-Y hepaticojejunostomy at age 52. Seventeen years later, she developed pancreatic head adenocarcinoma, treated with subtotal stomach-preserving pancreaticoduodenectomy (SSPPD) while preserving and reusing the original Roux limb. Six months later, she developed intrahepatic cholangiocarcinoma, requiring an extended right hepatectomy with reconstruction using the same preserved Roux limb. She remains disease-free 18 months after surgery. Patients with CBD and PBM remain at lifelong risk of malignancy, especially when surgery is performed in adulthood. Reuse of a preserved Roux limb is a feasible and effective strategy in complex reoperations.

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