The pattern of scarring and gross appearance of the liver can be used to classify cirrhosis into three groups:

a. Micronodular (Laënnec’s)

b. Macronodular

c. Mixed

Morphologic classification seldom permits the determination of the specific etiology. However, micronodular cirrhosis is most commonly seen as the consequence of alcoholic liver disease, and macronodular and mixed cirrhosis are the result of most other inflammatory or infiltrative diseases of the liver.

a. Alcohol

b. Viral hepatitis B, C, and D—most common causes of cirrhosis in the United States

c. Drug- or toxin-induced

d. Hemochromatosis

e. Wilson’s disease

f. α₁-Antitrypsin deficiency

g. Autoimmune hepatitis

h. Nonalcoholic steatohepatitis (NASH)

i. Biliary obstruction

i. Primary biliary cirrhosis (without extrahepatic bile duct obstruction)

ii. Secondary biliary cirrhosis (with extrahepatic bile duct obstruction)

j. Venous outflow obstruction

i. Budd-Chiari syndrome

ii. Venoocclusive disease

k. Cardiac failure

i. Chronic right-sided failure

ii. Tricuspid insufficiency

l. Malnutrition

i. Jejunoileal bypass surgery

ii. Gastroplasty

m. Miscellaneous

i. Schistosomiasis

ii. Congenital syphilis

iii. Cystic fibrosis

iv. Glycogen storage disease (type IV)

n. Idiopathic

Most symptoms found in patients with cirrhosis are nonspecific. Fatigue, malaise, and loss of vigor are common. There may be other symptoms caused by a complication of the disease leading to cirrhosis or of cirrhosis itself. These are discussed in the corresponding chapters or sections.

No physical abnormality establishes the diagnosis of cirrhosis.

a. Characteristic findings include palmar erythema, spider angiomata, gynecomastia, testicular atrophy, Dupuytren’s contractures, and findings due to portal hypertension such as splenomegaly, ascites, esophageal varices, and prominent superficial veins of the abdominal wall (caput medusae).

b. The liver in most patients with cirrhosis is enlarged and palpable below the costal margin. The left lobe often extends to the left upper quadrant below the xiphoid process. A small, hard, shrunken liver is a sign of very advanced cirrhosis. The liver edge in most patients with cirrhosis feels firm. Occasionally, regenerating nodules may be palpable in macronodular cirrhosis. Micronodules are not palpable.

Alteration of hepatic blood flow: portal hypertension

Reduction in functional cell mass

a. Decreased synthesis: albumin, coagulation proteins, other proteins

b. Decreased detoxification: bilirubin, ammonia, drugs

Deposition of collagen in the space of Disse with consequent narrowing of the sinusoids.

Distortion of sinusoids and the hepatic venous system by regenerating nodules.

The distortion of the hepatic parenchyma results in not only the development of portal hypertension but also intrahepatic intravascular shunts between portal venules through sinusoids to hepatic venules. Up to one third of the hepatic blood flow may bypass the functioning liver tissue because of these shunts.

Portal vein (prehepatic)

Intrahepatic (presinusoidal, sinusoidal, postsinusoidal)

Hepatic veins (posthepatic)

Collateral circulation and varices

Ascites

Congestive splenomegaly

Encephalopathy

The first step is to ensure adequate circulation with transfusion of blood, fresh-frozen plasma, and, if necessary, platelets. Because patients with liver disease often have deficiency of clotting factors, the infusion of fresh blood or fresh-frozen plasma is important.

After the vital signs are stabilized, the site and cause of the bleeding should be established by endoscopy. If bleeding is too brisk and endoscopic diagnosis is not possible, angiography may be performed to determine the site of bleeding and the vascular anatomy of the portal circulation.

Once the diagnosis of active variceal bleeding is made, there are several therapeutic options. The treatment of choice is endoscopic sclerotherapy or endoscopic variceal banding.

If these methods are not immediately available, medical drug therapy, balloon tamponade, or transhepatic variceal obliteration may be used. Surgical therapy with a portosystemic shunt (PSS) carries a very high mortality but may be lifesaving. Transjugular introduction of an expandable stent (transjugular intrahepatic portosystemic shunt [TIPS]) into the liver may create a PSS with much less morbidity or mortality.

the direct injection of a sclerosing agent into the esophageal varices, is effective in the immediate control of variceal bleeding. This technique is preferentially used as an initial therapy before the infusion of vasopressin or balloon tamponade. The sclerosants most commonly used are tetradecyl, sodium morrhuate, and ethanolamine oleate. The sclerosing agent is injected directly into the variceal wall or into the mucosa between the varices. It causes clotting of the varices and a severe necrotizing inflammation of the esophageal wall followed by a marked fibrotic reaction.

After control of the bleeding, the endoscopic sclerotherapy is repeated at weekly or monthly intervals until the varices are totally obliterated, leaving a scarred esophagus. Sclerotherapy of gastric varices has not been shown conclusively to be effective and may result in gastric ulceration. The complications of endoscopic sclerotherapy of esophageal varices include ulceration, hemorrhage, perforation, stricture, and pleural effusion. Sclerotherapy controls acute variceal hemorrhage in 80% to 90% of patients. Chronic sclerotherapy that obliterates the esophagus and varices decreases the risk of rebleeding.

has been shown to be as effective as or slightly more effective than injection sclerotherapy in the initial treatment of bleeding esophageal varices. The technique requires expertise and a
cooperative patient. Tracheal intubation and sedation of the patient may be necessary.

Although endoscopic banding or sclerotherapy is widely accepted as the treatment of choice for acutely bleeding esophageal varices, drug therapy may be a useful adjunctive treatment, particularly in severe hemorrhage and when bleeding is present from sites inaccessible to sclerotherapy (e.g., portal hypertensive gastropathy, gastric fundal varices, and varices in the more distal gastrointestinal tract). Several agents have been evaluated in the setting of acute portal hypertensive bleeding: vasopressin and its analogs with or without short-acting nitrates and somatostatin and its analog octreotide.

a. Vasopressin (Pitressin). Parenteral vasopressin results in constriction of the splanchnic blood flow and subsequent decrease in portal venous pressure. There is no clear evidence that direct infusion of vasopressin into the superior mesenteric artery is more effective or less toxic than intravenous (IV) administration of the drug. The IV route is preferred initially.

A continuous infusion of 0.4 unit per minute (or up to 0.9 U/min if necessary) is given for 4 to 12 hours with subsequent gradual decrease in the dose for duration up to 36 to 48 hours. The complications of vasopressin therapy are generalized vasoconstriction leading to myocardial and peripheral ischemia, lactic acidosis, cardiac arrhythmias, and hyponatremia (antidiuretic hormone effect).

b. Short-acting nitrates. The addition of nitroglycerin administered via transdermal, sublingual, or IV routes reduces the peripheral vasospastic effects of vasopressin and lowers the portal pressure further via direct vasodilation of portosystemic collaterals. The dosages are as follows: transdermal: 10 mg applied to skin q12h; sublingual: 0.6 mg every 30 minutes; IV: 40 µg/min increasing to 400 µg/min, adjusting doses to keep systolic blood pressure greater than 90 mmHg.

c. Somatostatin. Somatostatin appears to be highly selective in its ability to reduce splanchnic blood flow and hence reduce portal pressure. It has been shown to be as effective as vasopressin with considerably fewer hemodynamic effects. It can be administered for prolonged periods. The possible side effects are nausea, abdominal pain, and minor disturbances in glucose tolerance with prolonged use. Octreotide, the synthetic somatostatin analog, appears to be as effective as somatostatin. The dosages are as follows: somatostatin: 250-µg IV bolus followed by 250 µg per hour IV continuous infusion up to 5 days; octreotide: 50-mg IV bolus followed by 50 mg per hour IV. In cases of severe bleeding, the bolus dose may be repeated and the dose of somatostatin or octreotide in the continuous infusion may be doubled.

Octreotide has replaced vasopressin in the treatment of bleeding esophageal varices.

The Sengstaken-Blakemore (SB) and the Minnesota tube consist of two balloons, an elongated esophageal and a round gastric balloon, with orifices in the tube to suction the stomach and upper esophagus of collecting secretions. Variceal tamponade with the SB tube stops the bleeding, at least temporarily, in more than 90% of patients. Many difficulties that have been associated with the procedure can be avoided if the patient is monitored in an intensive care unit. The proper procedure requires inserting the tube through either the mouth or the nose, inflating the gastric balloon with 250 to 300 mL of air, and positioning the balloon tightly against the gastroesophageal junction. In most patients, this procedure alone stops the bleeding. If the bleeding persists, the esophageal balloon must be inflated to a pressure of 30 to 40 mmHg. The main complications of the SB tube are esophageal or gastric ischemia, rupture, and aspiration. Because the chance for complications from the SB tube increases with the length of time the balloon is kept inflated, the balloon should be deflated after 24 hours. If bleeding has stopped, the SB tube may be removed in another 24 hours.

of the varices with either a sclerosant or embolization controls active variceal bleeding 70% of the time. However, bleeding usually recurs. It should be used only as a secondary approach after other therapy has failed or for bleeding gastric varices in patients who are poor surgical risks.

Recurrent or continued bleeding may indicate a need for a PSS with surgical decompression of the portal venous pressure. This major surgery, when performed on an emergency basis, carries a mortality of 40%. If the surgery can be performed electively, mortality declines substantially. PSS procedures do not appear to prolong survival, but they do prevent subsequent bleeding. Because PSS diverts much of the blood away from the liver into the vena cava, the underperfusion of the liver results in liver failure and intractable hepatic encephalopathy in most of the patients. A variation of the PSS, the distal splenorenal shunt with concomitant gastroesophageal devascularization, selectively decompresses esophageal varices while maintaining mesenteric blood flow to the liver. In many studies, the use of the distal splenorenal shunt has been shown to reduce the incidence of severe encephalopathy as a late complication following surgery compared to other PSSs. This procedure is technically difficult, however, and is not advised in the presence of significant nonresponsive ascites, which it tends to worsen.

Emergency PSSs

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