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332a) This glass slide is a section of liver obtained from a surgical explant liver specimen. Based on the features seen in this slide, the patient who underwent liver transplantation was most likely:

65-year-old man with bleeding varices, increased serum aminotransferases AST and ALT and low albumin 53-year-old woman who has chronic hepatitis B and a 5.0 cm. mass in the right lobe of the liver. 2-year-old boy with hepatosplenomegaly and markedly increased serum alphafetoprotein 9-year-old boy with splenomegaly and bleeding varices, normal liver function tests, and no coagulopathy or encephalopathy. 41-year-old woman with chronic biliary stricture after a previous laparoscopic cholecystectomy

332b) This glass slide is a section of liver obtained from a surgical explant liver specimen. Which one of the following topics is LEAST relevant to the pathogenesis and manifestations of the liver disease seen here?

Primary cilia Fibrocystin Ductal plate malformations Portal hypertension Jaundice

332c) This glass slide is a section of liver obtained from a surgical explant liver specimen. Based on the features seen in this slide, the patient who underwent liver transplantation was MOST likely:

65-year-old man with bleeding varices, increased serum aminotransferases AST, and ALT and low albumin. 53-year-old woman who has chronic hepatitis B and a 5.0 cm mass in the right lobe of the liver. 2-year-old boy with hepatosplenomegaly and markedly increased serum alphafetoprotein. 9-year-old boy with splenomegaly and bleeding varices, normal liver function tests, and no coagulopathy or encephalopathy. 41-year-old woman with chronic biliary stricture after a previous laparoscopic cholecystectomy.

Show Diagnosis/Topic

• Congenital hepatic fibrosis (CHF) is one of the fibropolycystic liver diseases, which stems from abnormalities of remodeling and maturation of the embryonic bile duct plates (ductal plate malformations or DPMs).

• The characteristic pathology includes numerous bile ducts of irregular contour, angulation, and dilatation (sometimes with bile) embedded in portal and periportal fibrosis, with interspersed normal lobules of liver parenchyma.

• CHF is a cause of portal hypertension in pediatrics, with well preserved synthetic function.

• The presentation of CHF may be in infants (organomegaly, respiratory distress, systemic hypertension), juvenile form (portal hypertension with hepatosplenomegaly and/or bleeding varices) or may be asymptomatic.

• CHF is considered a variant of autosomal recessive polycystic kidney disease (ARPKD) in which mutation of the PKHD gene that encodes fibrocystin/polyductin on the bile duct epithelial primary cilium contributes to the abnormal remodeling of the bile duct plates.

• Routine serum liver function tests are typically normal in CHF, although alkaline phosphatise occasionally is elevated.

• Patients with CHF are not jaundiced and there is no histological cholestasis (except for the occasional inspissated bile seen in the ductal plate malformations).

Everson GT, Taylor MR, Doctor RB: Polycystic disease of the liver. Hepatology 2004;40:774-782.

Nakanuma Y, Harada K, Sato Y, Ikeda H: Recent progress in the etiopathogenesis of pediatric biliary disease, particularly Caroli’s disease with congenital hepatic fibrosis and biliary atresia. Histol Histopathol 2010;25: 223-235.

Raynaud P, Tate J, Callens C, et al: Classification of ductal plate malformations based on distinct pathogenic mechanisms of biliary dysmorphogenesis. Hepatology 2011;53:1959-1966.

Summerfield JA, Nagafuchi Y, Sherlock S, Cadafalch J, Scheuer PJ: Hepatobiliary fibropolycystic diseases. A clinical and histological review of 51 patients. J Hepatol 1986;2:141-156.

Anatomic Pathology: Liver Pathology

332a) This glass slide is a section of liver obtained from a surgical explant liver specimen. Based on the features seen in this slide, the patient who underwent liver transplantation was most likely:

332b) This glass slide is a section of liver obtained from a surgical explant liver specimen. Which one of the following topics is LEAST relevant to the pathogenesis and manifestations of the liver disease seen here?

332c) This glass slide is a section of liver obtained from a surgical explant liver specimen. Based on the features seen in this slide, the patient who underwent liver transplantation was MOST likely:

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