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.

Keywords

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

• References

• 1 Desmet VJ. Congenital diseases of intrahepatic bile ducts: variations on the theme “ductal plate malformation”. Hepatology 1992; 16 (4) 1069-1083
• 2 Alvarez F, Bernard O, Brunelle F , et al. Congenital hepatic fibrosis in children. J Pediatr 1981; 99 (3) 370-375
• 3 Kerr DNS, Okonkwo S, Choa RG. Congenital hepatic fibrosis: the long-term prognosis. Gut 1978; 19 (6) 514-520
• 4 Todani T, Watanabe Y, Urushihara N , et al Choledochal cyst, pancreatobiliary malunion, and cancer. J Hep Bil Pancr Surg 1994; 1 (3) 247-251
• 5 Todani T. Congenital choledochal dilatation: classification, clinical features, and long-term results. J Hepatobiliary Pancreat Surg 1997; 4: 276-282
• 6 Kobayashi S, Ohnuma N, Yoshida H , et al. Preferable operative age of choledochal dilation types to prevent patients with pancreaticobiliary maljunction from developing biliary tract carcinogenesis. Surgery 2006; 139 (1) 33-38
• 7 Perisic VN. Long-term studies on congenital hepatic fibrosis in children. Acta Paediatr 1995; 84 (6) 695-696
• 8 Ko JS, Yi NJ, Suh KS, Seo JK. Pediatric liver transplantation for fibropolycystic liver disease. Pediatr Transplant 2012; 16 (2) 195-200
• 9 Kaye AJ, Rand EB, Munoz PS, Spinner NB, Flake AW, Kamath BM. Effect of Kasai procedure on hepatic outcome in Alagille syndrome. J Pediatr Gastroenterol Nutr 2010; 51 (3) 319-321