| Hepatomegaly | ||
John M. Good
| Database Differential Diagnosis Approach to the Patient Data Gathering Physical Examination Laboratory Aids Emergency Care/Referral Common Questions and Answers Bibliography |
| DATABASE | ||
DEFINITION
Liver enlargement beyond age-adjusted normal values. Can be a common component of many diverse disease processes seen in infants and children.
| DIFFERENTIAL DIAGNOSIS | ||
CONGENITAL/ANATOMICAL
INFECTIONS
TOXIC, METABOLIC, DRUGS
TRAUMA
TUMOR
GENETIC/METABOLIC
ALLERGIC/INFLAMMATORY
MISCELLANEOUS
| APPROACH TO THE PATIENT | ||
| DATA GATHERING | ||
HISTORY
Question: Any prenatal history suggesting possible TORCH infection or
HIV infection?
Significance: TORCH infections and HIV may cause
hepatomegaly, although the liver involvement with HIV is usually secondary to
disseminated opportunistic infections or neoplastic processes, rather than from
the primary infection itself.
Question: Any history of blood product
transfusions?
Significance: Hepatitis C is the most common cause of
transfusion-associated hepatitis and the diagnosis should be considered in any
child who had received transfusions prior to 1990.
Question: Any history of sexual activity or intravenous drug
use?
Significance: Consider not only hepatitis B and HIV, but also
gonococcal peri-hepatitis (Fitz-Hugh–Curtis syndrome), and syphilis.
Question: Any foreign travel?
Significance: Increased risk
for parasitic infections or liver abscess.
Question: Any shellfish ingestion?
Significance:
Contaminated shellfish has been the source of several large outbreaks of
hepatitis A.
Question: What medications is the patient
taking?
Significance: Many pharmaceuticals have hepatotoxic side
effects. Remember to ask about non-prescription and recreational drug use, as
vitamin A, alcohol, and certain mushroom species (Amanita phalloides) can
be hepatotoxic.
Question: Any other chronic illnesses present?
Significance:
Patients with heart disease may have liver enlargement due to congestive heart
failure. Patients with cystic fibrosis can have focal biliary cirrhosis.
Patients with diabetes mellitus often have hepatomegaly secondary to increased
glycogen secretion. Severely anemic patients have hepatomegaly because of
extramedullary hematopoesis.
Question: Has the patient received total parenteral nutrition
(TPN)?
Significance: Cholestasis, bile duct proliferation, fatty
infiltration, and early cirrhosis are all well-described complications of
TPN.
Question: Any itching?
Significance: Puritis can be a subtle
sign of cholestasis.
| PHYSICAL EXAMINATION | ||
Finding: Where is the liver edge?
Significance: In children
younger than 2 years of age, the liver edge can extend from 1 to 3 cm below the
right costal margin in the mid-clavicular line. In older children, the liver
edge rarely extends beyond 2 cm. Verify all suspected cases of hepatomegaly by
checking the liver span.
Finding: What are signs of chronic liver
disease?
Significance: The liver is usually firm and enlarged, though
actually may decrease in size eventually with advanced disease. Splenomegaly,
caput medusae, spider angiomas, esophageal varices, and hemorrhoids suggest
portal hypertension. Ascites may develop due to elevated hydrostatic pressures
and decreased oncotic pressures secondary to hypoalbuminemia. Also look for
signs of occult bleeding or bruising due to impaired vitamin K production.
Finding: Is splenomegaly present?
Significance: Splenic
enlargement in the context of chronic liver disease implies portal hypertension.
Splenomegaly in the context of other signs of viral illness like adenopathy,
fever, malaise, and pharyngitis suggests acute viral hepatitis. Splenomegaly in
the absence of these signs suggests storage disease or hematologic
malignancy.
Finding: Are there conditions present that may be mimicking
hepatomegaly?
Significance: Pulmonary hyperinflation, subdiaphragmatic
abscesses, retroperitoneal mass lesions, or rib cage anomalies may all
downwardly displace a normal-sized liver mimicking hepatomegaly.
| LABORATORY AIDS | ||
Test: Aminotransferase and alanine aminotransferase (AST and
ALT).
Significance: Elevations reflect the amount of damage to
hepatocytes. Elevations >1000 indicate severe damage.
Test: Prothrombin time and partial prothrombin time (PT and
PTT)
Significance: Good indicators of the liver’s synthetic function.
Elevations can occur with an acute injury or illness. Combined with albumin
level, this test can be a sensitive indicator of chronic liver disease as
well.
Test: Gamma-glutamyl transferase (GGT) and alkaline
phosphatase
Significance: Elevations of GGT out of proportion to
elevations in AST and ALT can indicate an obstructive or infiltrative
abnormality. If an elevated GGT is associated with elevations in bilirubin,
cholesterol, and alkaline phosphatase, an obstructive process is more
likely.
Test: Ammonia level
Significance: Rising ammonia levels with
a prolongation of the PT and PTT suggests liver failure.
Test: Hepatitis profile
Significance: Should be obtained in
all patients with appropriate prodromal illness.
Test: Mono spot
Significance: While this is a non-specific
heterophile antibody test for EBV infection, it can be predictive in association
with an elevation of the atypical lymphocyte count. There is a high
false-negative rate as well in children less than 4 years of age. EBV titer is
the only confirmatory test.
Test: Alpha-fetoprotein (AFP) and carcinoembryonic antigen
(CEA)
Significance: Tumor markers for hepatoblastoma and
hepatocellular carcinoma, respectively.
Test: TORCH titers
Significance: Consider in newborns with
hepatomegaly.
Test: Serum immunoglobulins, anti-nuclear antibody (ANA), smooth
muscle antibody (SMA), anti-microsomal antibody, etc.
Significance:
Additional autoimmune evaluation is indicated for those patients with chronic
active hepatitis.
Test: Abdominal ultrasound
Significance: Should be performed
on all patients with acholic stools, asymmetric liver enlargement, or abdominal
mass.
Test: Serum ceruloplasmin level and urinary excretion of
copper
Significance: Decreased ceruloplasmin levels and increased
urinary excretion of copper characterize Wilson disease, especially after the
administration of oral D-penicillamine. Consider the diagnosis for patients with
unexplained liver disease.
| EMERGENCY CARE/REFERRAL | ||
Indications for immediate hospitalization include:
| COMMON QUESTIONS AND ANSWERS | ||
Q: Why does cholestasis cause puritis?
A: This probably
reflects an abnormal accumulation of bile acids in the skin.
Q: When following patients with chronic liver disease, are there any
differences in their nutritional needs?
A: Patients may have impaired
fat absorption and, therefore, may have deficiencies of fat soluble vitamins A,
D, E, and K, which may become evident as anemia, neuropathy, rickets, pathologic
fractures, visual disturbances, or skin changes. Also consider supplementing the
diet with medium chain tryglycerides, which are more easily absorbed. There may
also be higher than normal requirements of trace minerals as well.
Q: What is the etiology of TPN cholestasis?
A: Certain amino
acids present in TPN have been shown to increase the serum levels of bile acids,
which may in turn affect peristalsis in the gall bladder. Fasting may also
decrease the normal hormonal stimulation of bile secretion.
Clinical Pearls
| BIBLIOGRAPHY | ||
Roy C, Silverman A, Alagille D. Pediatric clinical gastroenterology. St. Louis: Mosby, 1995.
Walker WA, Mathia RK. Hepatomegaly: an approach to differential diagnosis? Pediatr Clin North Am 1975;22:929–942.
Copyright
© 2000 Lippincott Williams & Wilkins
M. William
Schwartz, Louis M. Bell, Jr., Peter M. Bingham, Esther K. Chung, David F.
Friedman and Andrew E. Mulberg, The 5 Minute Pediatric Consult