SECONDARY BILIARY CIRRHOSIS

Prolonged obstruction of the extrahepatic biliary tree results in profound alteration of the liver itself. 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. Obstructive conditions in

children include biliary atresia, cystic fibrosis, choledochal cysts (a cystic anomaly of the extrahepatic biliary tree, see later), and syndromes in which there are insufficient intrahepatic bile

ducts (paucity of bile duct syndromes).[39] The initial morphologic features of cholestasis were described earlier and are 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. Enteric organisms such as

coliforms and enterococci are common culprits.

Morphology.

The end-stage obstructed liver exhibits extraordinary yellow-green pigmentation and 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 septae 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 and edema, particularly at the

interface between septa (formerly 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

Figure 18-30Biliary cirrhosis. Sagittal section through the liver demonstrates the fine nodularity and bile staining of end-stage biliary cirrhosis.

Figure 18-31Primary biliary cirrhosis. A portal tract is markedly expanded by an infiltrate of lymphocytes and plasma cells. The granulomatous reaction to a bile duct undergoing

destruction (florid duct lesion) is highlighted by the arrowheads.

Figure 18-32Primary sclerosing cholangitis. A bile duct under-going degeneration is entrapped in a dense, "onion-skin" concentric scar.

Figure 18-33Bile duct anomalies. The morphologic features of the four major groups are diagrammed, along with apparent patterns of inheritance and associations with polycystic kidney

disease. PV, portal vein. HA, hepatic artery.

Figure 18-34Hepatic circulatory disorders. The forms and clinical manifestations of impaired blood flow are contrasted.

Figure 18-35Liver infarct. A thrombus is lodged in a peripheral branch of the hepatic artery and compresses the adjacent portal vein; the distal hepatic tissue is pale, with a hemorrhagic

margin.

Figure 18-36Centrolobular hemorrhagic necrosis. The cut liver section, in which major blood vessels are visible, is notable for a variegated, mottled, red appearance (nutmeg liver).

Figure 18-37Budd-Chiari syndrome. Thrombosis of the major hepatic veins has caused extreme blood retention in the liver.

Figure 18-38Veno-occlusive disease. A reticulin stain reveals the parenchyma framework of the lobule and the marked deposition of collagen within the lumen of the central vein.

Figure 18-39Eclampsia. Subcapsular hematoma dissecting under Glisson's capsule in a fatal case of eclampsia. (Courtesy of Dr. Brian Blackbourne, Office of the Medical Examiner, San

Diego, CA.)

Figure 18-40Focal nodular hyperplasia. A, Resected specimen showing lobulated contours and a central stellate scar. B, Low-power photomicrograph showing a broad fibrous scar with

hepatic arterial and bile duct elements and chronic inflammation, present within hepatic parenchyma that lacks the normal sinusoidal plate architecture (H&E).

Figure 18-41Nodular regenerative hyperplasia. Autopsied liver showing diffuse nodular transformation.

Figure 18-42Liver cell adenoma. A, Resected specimen presenting as a pendulous mass arising from the liver. B, Microscopic view showing cords of hepatocytes, with an arterial vascular

supply (arrows) and no portal tracts.

Figure 18-43Hepatocellular carcinoma. A, Autopsied liver showing a unifocal, massive neoplasm replacing most of the right hepatic lobe in a noncirrhotic liver; a satellite tumor nodule is

directly adjacent. B, In this microscopic view of a well-differentiated lesion, tumor cells are arranged in nests, sometimes with a central lumen, one of which contains bile (arrow). Other

tumor cells contain intracellular bile pigment.

Figure 18-44Fibrolamellar carcinoma. A, Resected specimen showing a demarcated nodule in an otherwise normal liver. B, Microscopic view showing nests and cords of malignantappearing

hepatocytes separated by dense bundles of collagen.

Figure 18-45Cholangiocarcinoma. A, Autopsied liver showing a massive neoplasm in the right hepatic lobe and innumerable metastases permeating the entire liver. B, Microscopic view

showing tubular glandular structures embedded in a dense sclerotic stroma.

Figure 18-46Multiple hepatic metastases from a primary colon adenocarcinoma.

Figure 18-47Normal gallbladder histology. The undulating mucosal epithelium overlies a delicate lamina and only one smooth muscle layer. This is different from elsewhere in the gut,

where two muscle layers exist (muscularis mucosa and muscularis propria).

Figure 18-48Phrygian cap of the gallbladder; the fundus is folded inward.

TABLE 18-12-- Risk Factors for Gallstones

Cholesterol Stones

Demography: Northern Europe, North and South America, Native Americans, Mexican Americans

Advancing age

Female sex hormones

••Female gender

••Oral contraceptives

••Pregnancy

Obesity

Rapid weight reduction

Gallbladder stasis

Inborn disorders of bile acid metabolism

Hyperlipidemia syndromes

Pigment Stones

Demography: Asian more than Western, rural more than urban

Chronic hemolytic syndromes

Biliary infection

Gastrointestinal disorders: ileal disease (e.g., Crohn disease), ileal resection or bypass, cystic fibrosis with pancreatic insufficiency

Date: 2016-04-22; view: 708