title: Recurrent Cholangitis with Congenital Hepatic Fibrosis and Pancreaticobiliary Maljunction after Roux-en-Y Reconstruction.
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.
authors.affiliation: Department of Pediatrics and Neonatology, Nagoya City University, Nagoya, Japan.
authors.forename: Tokio
authors.initials: T
authors.lastname: Sugiura
authors.affiliation: Department of Pediatrics and Neonatology, Nagoya City University, Nagoya, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Endo
authors.affiliation: Department of Pediatrics and Neonatology, Nagoya City University, Nagoya, Japan.
authors.forename: Koichi
authors.initials: K
authors.lastname: Ito
authors.affiliation: Department of Pediatrics and Neonatology, Nagoya City University, Nagoya, Japan.
authors.forename: Kenji
authors.initials: K
authors.lastname: Goto
authors.affiliation: Department of Pediatric Surgery, Nagoya City West Medical Center, Nagoya, Japan.
authors.forename: Yoko
authors.initials: Y
authors.lastname: Sato
authors.affiliation: Department of Pediatric Surgery, Nagoya City University, Nagoya, Japan.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Kondo
authors.affiliation: Department of Pediatric Surgery, Fujita Health University School of Medicine, Toyoake, Japan.
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Suzuki
authors.affiliation: Department of Pediatric Surgery, Fujita Health University School of Medicine, Toyoake, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Hashimoto
keywords: Roux-en-Y reconstruction; cholangitis; congenital hepatic fibrosis; liver transplantation; pancreaticobiliary maljunction