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European J Pediatr Surg Rep. 2013; 01(01): 043-045
DOI: 10.1055/s-0033-1341420
Case Report
Georg Thieme Verlag KG Stuttgart · New York

Recurrent Cholangitis with Congenital Hepatic Fibrosis and Pancreaticobiliary Maljunction after Roux-en-Y Reconstruction

Tokio Sugiura
1   Department of Pediatrics and Neonatology, Nagoya City University, Nagoya, Japan
,
Takeshi Endo
1   Department of Pediatrics and Neonatology, Nagoya City University, Nagoya, Japan
,
Koichi Ito
1   Department of Pediatrics and Neonatology, Nagoya City University, Nagoya, Japan
,
Kenji Goto
1   Department of Pediatrics and Neonatology, Nagoya City University, Nagoya, Japan
,
Yoko Sato
2   Department of Pediatric Surgery, Nagoya City West Medical Center, Nagoya, Japan
,
Satoshi Kondo
3   Department of Pediatric Surgery, Nagoya City University, Nagoya, Japan
,
Tatsuya Suzuki
4   Department of Pediatric Surgery, Fujita Health University School of Medicine, Toyoake, Japan
,
Takashi Hashimoto
4   Department of Pediatric Surgery, Fujita Health University School of Medicine, Toyoake, Japan
› Institutsangaben
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Publikationsverlauf

30. Dezember 2012

16. Januar 2013

Publikationsdatum:
19. März 2013 (online)

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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.

Keywords

congenital hepatic fibrosis - pancreaticobiliary maljunction - cholangitis - Roux-en-Y reconstruction - liver transplantation
 

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