Chapter 18 –Intrahepatic Biliary Tract Disease: SECONDARY BILIARY CIRRHOSIS Flashcards
What is the pathogenesis of SECONDARY BILIARY CIRRHOSIS?
Prolonged obstruction of the extrahepatic biliary tree results in profound hepatic alterations.
The most common cause of obstruction in adults is extrahepatic cholelithiasis (gallstones,
described later), followed by malignancies of the biliary tree or head of the pancreas, and strictures resulting from previous surgical procedures.
In SECONDARY BILIARY CIRRHOSIS what are theObstructive conditions in children?
- biliary atresia,
- cystic fibrosis,
- choledochal cysts (a cystic anomaly of the extrahepatic biliary tree, discussed later), and
- syndromes in which there are insufficient intrahepatic bile ducts (paucity of bile duct syndromes).
What is the initial morphologic features of cholestasis SECONDARY BILIARY CIRRHOSIS?
- entirely reversible with correction of the obstruction.
- However, secondary inflammation resulting from biliary obstruction initiates periportal fibrosis, which eventually leads to hepatic scarring and nodule formation, generating secondary biliary cirrhosis.
- Subtotal obstruction may promote secondary bacterial infection of the biliary tree (ascending cholangitis), which aggravates the inflammatory injury
What is the common culprit in secondary inflammation of SECONDARY BILIARY CIRRHOSIS?
. Enteric organisms such as coliforms and
enterococci are common culprits.
What is the appearance of SECONDARY BILIARY CIRRHOSIS end-stage obstructed liver?
yellow-green pigmentation that is
accompanied by marked icteric discoloration of body tissues and fluids.
On cut surface the liver is hard, with a finely granular appearance ( Fig. 18-30 ).
The histology is characterized by coarse fibrous septa that subdivide the liver in a jigsaw-like pattern.
Embedded in the septa are distended small and large bile ducts, which frequently contain inspissated
pigmented material.
There is extensive proliferation of smaller bile ductules, particularly at the interface between septa in former portal tracts and the parenchyma.
Cholestatic features in the parenchyma may be severe, with extensive feathery degeneration and
formation of bile lakes.
However, once regenerative nodules have formed, bile stasis may become less conspicuous.
Ascending bacterial infection incites a robust neutrophilic infiltration of bile ducts; severe pylephlebitis and cholangitic abscesses may develop.
FIGURE 18-30 Biliary cirrhosis. Sagittal section through the liver demonstrates the fine
nodularity and bile staining of end-stage biliary cirrhosis.
END
SECONDARY BILIARY CIRRHOSIS
PRIMARY BILIARY CIRRHOSIS (PBC)
