Abstract
A 1-year-old girl had pancreaticobiliary maljunction, a choledochal cyst, and polycystic kidney. At the age of 4 years, she was treated by resection of the choledochal cyst and Roux-en-Y reconstruction because of the cyst's risk of cancer. She was diagnosed as having congenital hepatic fibrosis based on the histological findings. Postoperatively, she suffered recurrent fever of unknown origin, refractory to several antibiotics. At the age of 6 years, she underwent living donor liver transplantation from her father. Multi-drug-resistant Pseudomonas aeruginosa was cultured in the recipient's liver. After liver transplantation, she had no episodes of recurrent fever. Roux-en-Y reconstruction should be avoided for ductal plate malformations such as congenital hepatic fibrosis.
Highlights
Congenital hepatic fibrosis (CHF) is a rare disorder of the portobiliary system characterized histologically by defective remodeling of the ductal plate, abnormal branching of the intrahepatic portal veins, and progressive fibrosis of the portal tracts.[1]
Roux-en-Y reconstruction should be avoided for ductal plate malformations such as congenital hepatic fibrosis
Complete choledochal cyst resection and Roux-en-Y reconstruction were performed in a 4-year-old girl with Pancreaticobiliary maljunction (PBM) and a choledochal cyst
Summary
Congenital hepatic fibrosis (CHF) is a rare disorder of the portobiliary system characterized histologically by defective remodeling of the ductal plate, abnormal branching of the intrahepatic portal veins, and progressive fibrosis of the portal tracts.[1]. A 1-year-old girl had pancreaticobiliary maljunction, a choledochal cyst, and polycystic kidney.
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