Hepatomegaly
Hepatomegaly: Excerpt from The 5-Minute Pediatric Consult
John M. Good, MD
Hepatomegaly - BASICS
Hepatomegaly - description
Liver enlargement beyond age-adjusted normal values; can be a common component of many diverse disease processes seen in infants and children
Hepatomegaly - etiology
- Most cases of hepatic failure in children are owing to acute viral hepatitis.
- Toxic exposure accounts for 25% of cases, with the most common drug being acetaminophen.
Hepatomegaly - DIAGNOSIS
Hepatomegaly - signs & symptoms
A detailed history and physical will direct the practitioner to any additional laboratory testing or appropriate radiologic evaluation.
Hepatomegaly - history
- Prenatal history suggesting possible toxoplasmosis, other, rubella, cytomegalovirus, or herpes (TORCH) infection or HIV infection:
- TORCH infections and HIV may cause hepatomegaly.
- Liver involvement with HIV is usually secondary to disseminated opportunistic infections or neoplastic processes, rather than from the primary infection itself.
- Hepatitis C is the most common cause of transfusion-associated hepatitis; should be considered in any child who received transfusions before 1990
- If there is history of sexual activity or IV drug use, consider not only hepatitis B and HIV, but also gonococcal perihepatitis (Fitz-Hugh-Curtis syndrome) and syphilis.
- Foreign travel suggests increased risk for parasitic infections or liver abscess.
- Contaminated shellfish has been the source of several large outbreaks of hepatitis A.
- Many pharmaceuticals have hepatotoxic side effects; ask about nonprescription and recreational drug use, as vitamin A, alcohol, and certain mushroom species can be hepatotoxic.
- Other chronic illnesses:
- Patients with heart disease may have liver enlargement owing to CHF 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 hematopoiesis.
- Total parenteral nutrition: Cholestasis, bile duct proliferation, fatty infiltration, and early cirrhosis are all well-described complications.
- Pruritus can be a subtle sign of cholestasis.
Hepatomegaly - physical exam
- Liver edge:
- In children <2 years of age, the liver edge can extend 1–3 cm below the right costal margin in the midclavicular line.
- In older children, the liver edge rarely extends beyond 2 cm.
- Verify all suspected cases of hepatomegaly by checking the liver span.
- Signs of chronic liver disease:
- Liver is usually firm and enlarged, although actually may decrease in size eventually with advanced disease.
- Splenomegaly, caput medusae, spider angiomas, esophageal varices, and hemorrhoids suggest portal hypertension.
- Ascites may develop as a result of elevated hydrostatic pressures and decreased oncotic pressures secondary to hypoalbuminemia.
- Also look for signs of occult bleeding or bruising owing to impaired vitamin K production.
- Splenomegaly:
- In the context of chronic liver disease, implies portal hypertension
- In the context of other signs of viral illness such as adenopathy, fever, malaise, and pharyngitis, suggests acute viral hepatitis
- In the absence of these signs, suggests storage disease or hematologic malignancy
- Conditions that may mimic hepatomegaly by downwardly displacing a normal-sized liver:
- Pulmonary hyperinflation
- Subdiaphragmatic abscesses
- Retroperitoneal mass lesions
- Rib cage anomalies
- Clinical pearls:
- Until age 2, girls have a slightly larger liver span than boys.
- A Reidel lobe is a normal variant in which the right lobe of the liver appears elongated owing to its adhesion to the mesocolon.
- Administration of vitamin K in an attempt to correct PT can be a valuable assessment of the liver’s synthetic function.
- Fetor hepaticus is a sweetish odor that can be detected on the breath and urine of patients with liver failure.
- Asterixis or liver flap is rare in children.
Hepatomegaly - tests
All patients with hepatomegaly should have a laboratory evaluation, including CBC with differential, comprehensive metabolic panel (including liver function tests, total protein and albumin, total and direct bilirubin, basic electrolytes and glucose), a PT, and a PPT.
Hepatomegaly - lab
- CBC with differential
- Aminotransferase and alanine aminotransferase:
- Elevations reflect the amount of damage to hepatocytes.
- Elevations >1,000 indicate severe damage.
- PT and PTT:
- 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.
- β-Glutamyltransferase and alkaline phosphatase:
- Elevations of β-glutamyltransferase out of proportion to elevations in aminotransferase and alanine aminotransferase can indicate an obstructive or infiltrative abnormality.
- If an elevated β-glutamyltransferase is associated with elevations in bilirubin, cholesterol, and alkaline phosphatase, an obstructive process is more likely.
- Ammonia level: Rising ammonia levels with a prolongation of the PT and PTT suggest liver failure.
- Hepatitis profile: Should be obtained in all patients with appropriate prodromal illness
- Mono spot:
- Although this is a nonspecific heterophile antibody test for Epstein-Barr virus infection, it can be predictive in association with an elevation of the atypical lymphocyte count.
- High false-negative rate in children <4 years of age
- Epstein-Barr virus titer is the only confirmatory test.
- แα-Fetoprotein and carcinoembryonic antigen: Tumor markers for hepatoblastoma and hepatocellular carcinoma, respectively
- TORCH titers: Consider in newborns with hepatomegaly
- Serum immunoglobulins, antinuclear antibody, smooth muscle antibody, antimicrosomal antibody: Additional autoimmune evaluation is indicated for those patients with chronic active hepatitis.
- Serum ceruloplasmin level and urinary excretion of copper:
- 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.
Hepatomegaly - imaging
Abdominal ultrasound should be performed on all patients with acholic stools, asymmetric liver enlargement, or abdominal mass.
Hepatomegaly - differencial diagnosis
- Congenital/Anatomic:
- Alagille syndrome
- Biliary atresia
- Choledochal cyst
- Congenital hepatic fibrosis
- Obstruction of the common bile duct owing to stones, strictures, or tumors
- Infections:
- Viral infections:
- Hepatitis types A–E
- Cytomegalovirus
- Epstein-Barr virus
- Coxsackievirus
- Congenital infections:
- Toxoplasmosis
- Rubella
- Cytomegalovirus
- Herpes
- HIV
- Parasitic infections:
- Amebiasis
- Flukes
- Schistosomiasis
- Malaria
- Fungal disease:
- Candidiasis
- Histoplasmosis
- STDs:
- Gonococcal perihepatitis
- Syphilis
- HIV
- Zoonotic diseases: Brucellosis
- Leptospirosis
- Hepatic abscess
- Bartonella henselae
- Pasteurella multocida
- Tuberculosis
- Septicemia
- Toxic, metabolic, drugs:
- Drug-induced hepatitis:
- Acetaminophen
- Alcohol
- Corticosteroids
- Erythromycin
- Hypervitaminosis A
- Iron
- Isoniazid
- Nitrofurantoin
- Oral contraceptives
- Phenobarbital
- Valproate
- Trauma:
- Hemorrhage
- Subcapsular hematoma
- Traumatic cyst
- Tumor:
- Benign tumors:
- Hemangioma
- Hemangioendothelioma
- Mesenchymal hamartoma
- Focal nodular hyperplasia
- Adenoma
- Malignant tumors:
- Hepatoblastoma
- Hepatocellular carcinoma
- Metastatic tumors
- Histiocytic disease
- Genetic/Metabolic:
- แαAmyloidosis
- Beckwith-Wiedemann syndrome
- Chédiak-Higashi syndrome
- Crigler-Najjar syndrome
- Cystic fibrosis
- Diabetes mellitus
- Galactosemia
- GMGlycogen storage diseases
- Hematochromatosis
- Hereditary fructose intolerance
- Homocystinuria
- Lipidoses
- Mucopolysaccharidoses
- Urea cycle defects
- Wilson disease
- Zellweger syndrome
- Allergic/Inflammatory:
- Chronic active hepatitis
- Sclerosing cholangitis
- Sarcoidosis
- Systemic inflammatory disease:
- Juvenile rheumatoid arthritis
- Systemic lupus erythematosus
- Inflammatory bowel disease
- Miscellaneous:
- CHF
- Extramedullary hematopoiesis
- Pulmonary hyperinflation
- Restrictive pericarditis
- Veno-occlusive disease
- Malnutrition
- Reye syndrome
- Total parenteral nutrition
Indications for immediate hospitalization include:
- Persistent anorexia and vomiting
- Mental status changes
- Worsening jaundice
- Relapse of symptoms after initial improvement
- Known exposure to a liver toxin
- Rising PT
- Rising ammonia level
- Bilirubin >20 mg/dL
- Aspartate aminotransferase >2,000
- Development of new ascites
- Hypoglycemia
- Leukocytosis and thrombocytopenia
Hepatomegaly - bibliography
- Clayton PT. Diagnosis of inherited disorders of liver metabolism. J Inherit Metab Dis. 2003;26(2–3):135–146.
Roy C, Silverman A, Alagille D. Pediatric Clinical Gastroenterology. St. Louis: Mosby; 1995.
Hepatomegaly - CODES
Hepatomegaly - icd9
789.1 Hepatomegaly
Hepatomegaly - FAQ
- Q: Why does cholestasis cause pruritus?
- A: This probably reflects an abnormal accumulation of bile acids in the skin.
- Q: Do patients with chronic liver disease have any different 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 mediumchain triglycerides, which are more easily absorbed. There may also be higher than normal requirements of trace minerals.
- Q: What is the etiology of cholestasis owing to total parenteral nutrition?
- A: Certain amino acids present in total parenteral nutrition have been shown to increase the serum levels of bile acids, which may in turn affect peristalsis in the gallbladder. Fasting may also decrease the normal hormonal stimulation of bile secretion.
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Book Source Details
- Book Title: The 5-Minute Pediatric Consult
- Author(s): M. William Schwartz MD; et al.
- Year of Publication: 2008
- Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.
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- Hepatomegaly
- "Professional Guide to Signs & Symptoms (Fifth Edition)" (2006)
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- Hepatitis
- "The 10-Minute Diagnosis Manual: Symptoms and Signs in the Time-Limited Encounter" (2000)
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- "The 10-Minute Diagnosis Manual: Symptoms and Signs in the Time-Limited Encounter" (2000)
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- Hepatomegaly
- "The Diagnostic Approach to Symptoms and Signs in Pediatrics" (2006)
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: The 5-Minute Pediatric Consult
Authors: M. William Schwartz MD; et al.
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7577-9
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» Next page: Reye Syndrome (The 5-Minute Pediatric Consult)
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