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176

Section 7: Digestive system

LIVER

Chapter 176

Cirrhosis

DEFINITION

Cirrhosis is dened histologically as the presence of brosis and


regenerative nodules in the liver (Fig. 1761). It can be classied as micronodular, macronodular, and mixed; however, each
form may be seen in the same patient at different stages of the
disease. Cirrhosis manifests clinically with portal hypertension,
hepatic encephalopathy, and variceal bleeding.
PHYSICAL FINDINGS AND CLINICAL PRESENTATION

Variable with etiology and stage of cirrhosis


SKIN: Jaundice (Fig. 1762), palmar erythema (alcohol abuse)
(Fig. 1763), spider angiomata (Fig. 1764), ecchymosis
(thrombocytopenia or coagulation factor deciency), dilated abdominal wall veins, xerotic eczema (Fig. 1765),
dilated supercial periumbilical vein (caput meduse) (Fig.
1766), increased pigmentation (hemochromatosis [see

Fig 1761

Fig 1763

Advanced cirrhosis after longstanding chronic hepatitis C.

Palmar erythema (liver palms). Gross reddening of the thenar and hypothenar eminences and ngers with sparing of the center of the palm.

(From Silverberg SG: Principles and Practice of Surgical Pathology and


Cytopathology, 4th ed. Philadelphia, Churchill Livingstone, 2006.)

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Fig. 1801]), xanthomas (primary biliary cirrhosis; see Fig.


1791), needle tracks (viral hepatitis)
EYES: Kayser-Fleischer rings (corneal copper deposition seen in
Wilsons disease [see Fig. 403]; best diagnosed with slit
lamp examination), scleral icterus
BREATH: Fetor hepaticus (musty odor of breath and urine
found in cirrhosis with hepatic failure)
CHEST: Possible gynecomastia in men
ABDOMEN: Tender hepatomegaly (congestive hepatomegaly),
small, nodular liver (cirrhosis), palpable, nontender gallbladder (neoplastic extrahepatic biliary obstruction), palpable spleen (portal hypertension), venous hum auscultated
over periumbilical veins (portal hypertension), ascites (portal hypertension, hypoalbuminemia) (Fig. 1767)

(From Forbes A, Misiewicz JJ, Compton CC, et al [eds]: Atlas of Clinical


Gastroenterology, 3rd ed. Edinburgh, Elsevier Mosby, 2005.)

Fig 1762

Fig 1764

Jaundice.

Spider angioma on the arm.

(From Swartz MH: Textbook of Physical Diagnosis, 5th ed. Philadelphia,


WB Saunders, 2006.)

(From Callen JP, Jorizzo JL, Bolognia JL, et al: Dermatological Signs of Internal Disease, 3rd ed. Philadelphia, WB Saunders, 2003.)

Chapter 176: Cirrhosis

Fig 1765

Fig 1767

Dilated abdominal wall veins along with xerotic eczema associated with
cirrhosis and portal hypertension.

Ascites.

(Courtesy of Neil Fenske, MD, Tampa, FL.)

176

(From Swartz MH: Textbook of Physical Diagnosis, 5th ed. Philadelphia,


WB Saunders, 2006.)

Secondary biliary cirrhosis, obstruction of the common bile


duct (stone, stricture, pancreatitis, neoplasm, sclerosing
cholangitis)
Drugs (e.g., acetaminophen, isoniazid, methotrexate, methyldopa)
Hepatic congestion (e.g., CHF, constrictive pericarditis, tricuspid insufciency, thrombosis of the hepatic vein, obstruction of the vena cava)
Primary biliary cirrhosis
Hemochromatosis
Chronic hepatitis B or C
Wilsons disease
Alpha-1 antitrypsin deciency
Inltrative diseases (amyloidosis, glycogen storage diseases,
hemochromatosis)
Nutritional: jejunoileal bypass
Others: parasitic infections (schistosomiasis), idiopathic
portal hypertension, congenital hepatic brosis, systemic
mastocytosis, autoimmune hepatitis, hepatic steatosis, IBD

LABORATORY TESTS

Fig 1766
Abdominal venous patterns
(From Swartz MH: Textbook of Physical Diagnosis, 5th ed. Philadelphia,
WB Saunders, 2006.)

RECTAL EXAMINATION: Hemorrhoids (see Fig. 1691) (portal


hypertension), guaiac-positive stools (alcoholic gastritis,
bleeding esophageal varices, PUD, bleeding hemorrhoids)
GENITALIA: Testicular atrophy in males (chronic liver disease,
hemochromatosis)
EXTREMITIES: Pedal edema (hypoalbuminemia, failure
of right side of the heart), arthropathy (see Fig. 1803)
(hemochromatosis)
NEUROLOGIC: Flapping tremor, asterixis (hepatic encephalopathy), choreoathetosis, dysarthria (Wilsons disease)
CAUSE

Alcohol abuse

Decreased Hgb and Hct, elevated MCV, presence of stomatocytes (Fig. 1768), increased BUN and creatinine
(the BUN may also be normal or low if the patient has
severely diminished liver function), decreased sodium
(dilutional hyponatremia), decreased potassium (as a result of secondary aldosteronism or urinary losses).
Decreased glucose in a patient with liver disease is indicative
of severe liver damage
Other laboratory abnormalities:
1. Alcoholic hepatitis and cirrhosis: there may be mild
elevation of ALT and AST, usually 500 IU; AST ALT (ratio 2:3).
2. Extrahepatic obstruction: there may be moderate elevations of ALT and AST to levels 500 IU.
3. Viral, toxic, or ischemic hepatitis: there are extreme elevations (500 IU) of ALT and AST.
4. Transaminases may be normal despite signicant liver
disease in patients with jejunoileal bypass operations or
hemochromatosis or after methotrexate administration.

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176

Section 7: Digestive system

21. Elevated level of serum globulin (especially


-globulins), positive ANA test may occur with autoimmune hepatitis.
IMAGING STUDIES

Fig 1768
Stomatocytes in a patient with liver disease.
(From Young NS, Gerson SL, High KA [eds]: Clinical Hematology. St Louis,
Mosby, 2006.)

5. Alkaline phosphatase elevation can occur with extrahepatic obstruction, primary biliary cirrhosis, and primary
sclerosing cholangitis.
6. Serum LDH is signicantly elevated in metastatic disease of the liver; lesser elevations are seen with hepatitis,
cirrhosis, extrahepatic obstruction, and congestive hepatomegaly.
7. Serum -glutamyl transpeptidase (GGTP) is elevated in
alcoholic liver disease and may also be elevated with cholestatic disease (primary biliary cirrhosis, primary sclerosing
cholangitis).
8. Serum bilirubin may be elevated; urinary bilirubin can
be present in hepatitis, hepatocellular jaundice, and biliary
obstruction.
9. Serum albumin: signicant liver disease results in hypoalbuminemia.
10. Prothrombin time: an elevated PT (INR) in patients
with liver disease indicates severe liver damage and poor
prognosis.
11. Presence of hepatitis B surface antigen implies acute or
chronic hepatitis B.
12. Presence of antimitochondrial antibody suggests primary biliary cirrhosis, chronic hepatitis.
13. Elevated serum copper, decreased serum ceruloplasmin,
and elevated 24-hours urine is indicative of Wilsons disease.
14. Protein immunoelectrophoresis may reveal decreased 1 globulins (-1 antitrypsin deciency), increased IgA (alcoholic cirrhosis), increased IgM (primary biliary cirrhosis), increased IgG (chronic hepatitis, cryptogenic cirrhosis).
15. An elevated serum ferritin and increased transferrin
saturation are suggestive of hemochromatosis.
16. An elevated blood ammonia suggests hepatocellular
dysfunction; serial values, however, are generally not useful
in following patients with hepatic encephalopathy, because
there is poor correlation between blood ammonia level and
degree of hepatic encephalopathy.
17. Serum cholesterol is elevated in cholestatic disorders.
18. Antinuclear antibodies (ANA) may be found in autoimmune hepatitis.
19. Alpha fetoprotein: levels 1000 pg/mL are highly suggestive of primary liver cell carcinoma.
20. Hepatitis C viral testing identies patients with chronic
hepatitis C infection.

618

Ultrasonography is the procedure of choice for detection of


gallstones and dilation of common bile ducts.
CT scan is useful for detecting mass lesions in the liver and
pancreas, assessing hepatic fat content, identifying idiopathic hemochromatosis, diagnosing of Budd-Chiari syndrome, dilation of intrahepatic bile ducts, and detection of
varices and splenomegaly.
Technetium-99m sulfur colloid scanning is infrequently
used. It can be useful for diagnosing cirrhosis (there is a
shift of colloid uptake to the spleen, bone marrow), identifying hepatic adenomas (cold defect is noted), diagnosing
Budd-Chiari syndrome (there is increased uptake by the
caudate lobe).
ERCP is the procedure of choice for diagnosing periampullary carcinoma, common duct stones; it is also useful in diagnosing primary sclerosing cholangitis.
Percutaneous transhepatic cholangiography (PTC) is useful
when evaluating patients with cholestatic jaundice and dilated intrahepatic ducts by ultrasonography; presence of
intrahepatic strictures and focal dilation is suggestive
of primary sclerosing cholangitis (PSC).
Percutaneous liver biopsy (Fig. 1769) is useful in evaluating hepatic lling defects, diagnosing hepatocellular disease
or hepatomegaly, evaluating persistently abnormal liver
function tests, and diagnosing hemachromatosis, primary
biliary cirrhosis, Wilsons disease, glycogen storage diseases,
chronic hepatitis, autoimmune hepatitis, inltrative diseases, alcoholic liver disease, drug-induced liver disease, and
primary or secondary carcinoma.

TREATMENT

Treatment varies with etiology of cirrhosis


Avoid any hepatotoxins (e.g., ethanol, acetaminophen);
improve nutritional status.
Remove excess body iron with phlebotomy and deferoxamine in patients with hemochromatosis.
Remove copper deposits with D-penicillamine in patients
with Wilsons disease.
Long-term ursodiol therapy will slow the progression of
primary biliary cirrhosis. It is, however, ineffective in primary sclerosing cholangitis.
Glucocorticoids (prednisone 20 to 30 mg/day initially or
combination therapy or prednisone and azathioprine) is
useful in autoimmune hepatitis.
Liver transplantation may be indicated in otherwise healthy
patients (age 65 years) with sclerosing cholangitis, chronic
hepatitis cirrhosis, or primary biliary cirrhosis with prognostic information suggesting 20% chance of survival without
transplantation; contraindications to liver transplantation
are AIDS, most metastatic malignancies, active substance
abuse, uncontrolled sepsis, and uncontrolled cardiac or pulmonary disease.
Treatment of complications of portal hypertension (ascites,
esophagogastric varices, hepatic encephalopathy, and hepatorenal syndrome).

Chapter 176: Cirrhosis

176

C
Fig 1769
Biopsy needles. (A) Chiba needles for aspiration biopsy. (B) Automated biopsy needle (Temno, Bauer). (C) Close-up view of notched cutting tip of
Temno needle.
(From Grainger RG, Allison DJ, Adam A, Dixon AK [eds]: Grainger and Allisons Diagnostic Radiology, 4th ed. Philadelphia, Churchill Livingstone, 2001.)

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