Hospital Medicine

Hepatomegaly

I. Problem/Condition.

Hepatomegaly is a condition in which the liver is enlarged, generally greater than 15 centimeters in the midclavicular line. However, normal liver size varies by person depending on the person’s sex, age, height, weight, and body size. Hepatomegaly develops primarily as a result of a pathologic process which should be investigated further. Hepatomegaly may be suggested from the physical examination or found on imaging studies. Like any physical or radiologic finding, the clinical context of this finding should guide further work-up and treatment.

II. Diagnostic Approach

A. What is the differential diagnosis for this problem?

There are various possible etiologies for an enlarged liver. Generally, this can be broken down into the following categories: infiltrative disease, vascular or congestive hepatopathy, hepatitis (infectious and non-infectious), cholestatic disease, and nodules or space-occupying lesions (see Liver nodules section).

Infiltrative processes include Wilson’s disease (abnormal copper deposition) and hemochromatosis (abnormal iron deposition). Gaucher’s disease is a liposomal storage disorder which ultimately causes hepatomegaly due to accumulation of lipid-laden macrophages and resultant inflammation in the liver (and spleen); some cases of Gaucher’s are not detected until adulthood. Amyloidosis and glycogen storage disorders may also cause liver enlargement.

When vascular flow out of the liver is compromised, as by acute blood clot formation in the hepatic veins as in Budd-Chiari syndrome, the liver may become enlarged and tender. Obstruction of the inferior vena cava can also cause hepatomegaly. Congestive hepatopathy results from passive liver congestion due to poor cardiac output. Tricuspid regurgitation and constrictive pericarditis may also cause hepatomegaly and are distinguished by the presence of pulsations on palpation.

Hepatitis, or inflammation in the liver, can also cause hepatomegaly. The causes of hepatitis are varied and include infections (various viruses), medications, alcohol, autoimmune disorders, and nonalcoholic steatohepatitis.

Cholestatic disease, such as cholangitis, can cause liver enlargement which is generally tender and associated with elevated alkaline phosphatase and bilirubin levels.

There are malignant and nonmalignant causes for liver nodules (see Liver nodules section).

Other miscellaneous causes of hepatomegaly include sarcoidosis, lymphoma, leukemia, and extramedullary hematopoiesis.

B. Describe a diagnostic approach/method to the patient with this problem

Should you suspect hepatomegaly based on physical examination, liver ultrasound or computed tomography scan can be used to confirm the diagnosis. Ultrasound is the preferred imaging modality given its lack of radiation and lower cost, but computed tomography scanning can better identify parencyhymal abnormalities and focal lesions.

If the liver is in fact not enlarged by imaging, consider displacement of the liver due to small body habitus, adjacent mass, flattened diaphragm such as in chronic obstructive pulmonary disease or asthma, or subdiaphragmatic abscess as the etiology for the palpable liver.

In the setting of true hepatomegaly without obvious cause (such as decompensated congestive heart failure), you should obtain transaminases, bilirubin, and alkaline phosphatase. Elevated transaminases can be further investigated with viral serologies, though multiple other etiologies can cause elevated transaminases.

Ultimately, the patient may require liver biopsy to determine the etiology of hepatomegaly.

1. Historical information important in the diagnosis of this problem.

A complete history can focus the clinician as to which laboratory and radiologic studies will be necessary. Any prior history of liver disease or known cirrhosis is important; other chronic medical problems, such as congestive heart failure, prior deep venous thrombosis, or known malignancy, may also tailor your differential. The patient should give a full social history, including recent travel, alcohol intake, use of intravenous drugs, and sexual exposures. Medications taken, especially including new medications, is important as drug-induced hepatitis is a possibility.

2. Physical Examination maneuvers that are likely to be useful in diagnosing the cause of this problem.

Liver palpation

There are two main techniques for hepatic palpation.

Some experts tout beginning by palpating the right lower quadrant with the finger pads. You should ask the patient to inhale slowly in order to lower the edge of the liver (by flattening the diaphragm) and during exhalation, slowly move the hands superiorly in approximately 2-centimeter intervals in order to touch the inferior edge of the liver. If the liver edge is not felt, this portion of the examination is complete.

Liver percussion

If the liver edge is felt, you then proceed to percuss the superior edge of the liver by starting in the midclavicular line at approximately the third rib. Dullness to percussion generally indicates the superior margin of the liver. You can also percuss the inferior margin of the liver by starting in the right lower quadrant and gently percussing superiorly along the midclavicular line until dullness is appreciated.

Take note, however, that palpation of the liver edge does not necessarily indicate hepatomegaly; similarly, hepatomegaly may be suggested by increased size by percussion, but this is not definite proof of liver enlargement.

Skin examination

Pay particular attention to palmar erythema, gynecomastia, or spider angioma which may indicate chronic liver disease.

Evidence of congestive heart failure

Lower extremity edema, ascites, or elevated jugular venous pressure may indicate congestive heart failure. Tricuspid regurgitation or restrictive pericarditis may cause a palpable pulsating liver edge, though data is limited on exactly how to correlate this finding.

Confirming the diagnosis

3. Laboratory, radiographic and other tests that are likely to be useful in diagnosing the cause of this problem.

Though palpation of a liver edge on examination increases the chances of hepatomegaly, confirmation should be obtained by diagnostic imaging with ultrasound or computed tomography scan when appropriate.

Diagnostic criteria

C. Criteria for Diagnosing Each Diagnosis in the Method Above.

Vascular diseases

Budd-Chiari syndrome is diagnosed using ultrasound with Doppler, computed tomography, or magnetic resonance imaging (see Budd-Chiari syndrome section). Congestive hepatopathy from heart failure or tricuspid regurgitation can be diagnosed with echocardiogram (see heart failure section).

Hepatitis

Hepatitis is suggested by elevated transaminase levels (see hepatitis section).

Cholestatic diseases

Cholestatic disease is suggested by elevated alkaline phosphatase and bilirubin levels. Liver ultrasound can help identify dilated bile ducts (see sections on hyperbilirubinemia and elevated alkaline phosphatase).

Liver nodules

Liver nodules will be seen on imaging with either ultrasound, computed tomography, or magnetic resonance imaging (see liver nodules section).

Infiltrative diseases

(see infiltrative liver disease section)

D. Over-utilized or “wasted” diagnostic tests associated with the evaluation of this problem.

A radioisotope scan of the liver is generally not needed as computed tomography and ultrasound provide adequate imaging.

III. Management while the Diagnostic Process is Proceeding

A. Management of Clinical Problem: Hepatomegaly.

There is no acute management of hepatomegaly per se, but acute medical issues leading to hepatomegaly such as acute decompensated congestive heart failure, sepsis, or acute Budd-Chiari disease should be recognized and treated promptly.

B. Common Pitfalls and Side-Effects of Management of this Clinical Problem

Acute, life-threatening disorders such as acute decompensated congestive heart failure and acute thrombosis should be diagnosed and treated as soon as possible. Hepatomegaly can lead to splenomegaly which may cause pancytopenia (which may lead to additional problems).

There are no specific medications to treat hepatomegaly. The underlying cause of hepatomegaly must be addressed.

IV. What’s the evidence?

Naylor, CD, Simel, DL, Rennie, DL. "Does this patient have hepatomegaly". The Rational Clinical Examination: Evidence-Based Clinical Diagnosis. McGraw-Hill. 2009.

Healy, PM, Jacobson, EJ. "Hepatomegaly". Common Medical Diagnoses: An Algorithmic Approach (ed 4). Saunders Elsevier. 2006.

Noel, RJ, Balistreri, WF, Friedman, LS, Keeffe, EB. "Pediatric liver disease". Handbook of Liver Disease (ed 2). Churchill Livingstone. 2004.

Schiff, ER, Sorrell, MF, Maddrey, WC. Schiff’s Diseases of the Liver (ed 10). Volume 1 and Volume 2. Lippincott Williams and Wilkins. 2007.

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