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Congenital disorders of the liver, biliary tract, and pancreas 

Congenital disorders of the liver, biliary tract, and pancreas

Congenital disorders of the liver, biliary tract, and pancreas

J.A. Summerfield


July 30, 2015: This chapter has been re-evaluated and remains up-to-date. No changes have been necessary.

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date: 30 March 2017

Congenital disorders of the liver, biliary tract, and pancreas are rare or very rare.

Biliary atresias present with cholestatic jaundice starting after the first 2 weeks of life and may eventually cause biliary cirrhosis and liver failure. Prognosis depends on the type of atresia: (1) intrahepatic—both nonsyndromic (with cirrhosis usually developing in late childhood, and fatal without liver transplantation) and syndromic (e.g. Alagille’s syndrome, due to mutation in the JAG1 gene, with dysmorphic and other features, and a tendency for recovery of liver function in adolescence), or (2) extrahepatic—which is usually fatal within 6 months of birth unless treated by hepatic portoenterostomy and/or liver transplantation.

Fibropolycystic diseases include polycystic liver, congenital hepatic fibrosis, congenital intrahepatic biliary dilatation (Caroli’s disease), choledochal cysts, and microhamartomas (von Meyenberg complexes). Patients may have more than one of these diseases, some of which may be complicated by biliary malignancy. Associated renal diseases can be severe.

Congenital pancreatic diseases comprise a heterogeneous group of conditions of varying severity, ranging from annular pancreas (where pancreatic tissue encircles the duodenum) that may be discovered incidentally, to pancreatic agenesis, which is usually fatal soon after birth.

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