Approach to a child with hepatomegaly Dr. Shreedhar Paudel May, 2009

Approach to a child with hepatomegaly Considering the history and hepatomegaly found on the exam, what are your differentials? How would you evaluate this child? What is your diagnosis? What is the treatment and prognosis for this infection?

Hepatomegaly Liver size may be estimated by degree of extension of liver edge below costal margin or by span of liver dullness to percussion In children liver edge may be normally palpable up to 2 cm below right costal margin

Hepatomegaly… This assessment of liver size may be unreliable if other non hepatic factors (e.g., lung disease) push diaphragm lower and thus the liver below the costal margin Liver span – a more reliable measurement of liver size – distance determined by percussion between upper margin of liver dullness and its lower edge – Span of 5–7cm is normal in infants and children, whereas 8–10 cm is normal in adolescents

Hepatomegaly… Principal Causes of Hepatomegaly – Infection/inflammation Hepatitis – Viral – Bacterial – Other infections – Toxic Cholangitis

Hepatomegaly… Principal Causes of Hepatomegaly – Hemolytic anemia – Cardiac disorders – Trauma – Bile duct obstruction Biliary atresia – Vascular disorders Budd-Chiari syndrome Veno-occlusive disease

Hepatomegaly… Principal Causes of Hepatomegaly – Metabolic disorders Wilsons disease Disorders of carbohydrate metabolism – Galactosemia – Hereditary fructose intolerance – Glycogen storage disease Disorders of amino acid metabolism – Tyrosinemia – Urea cycle defects Disorders of lysosomal storage – Mucopolysaccharidoses – Lipidoses – Glycoprotein disorders

Hepatomegaly… Principal Causes of Hepatomegaly – Systemic disorders Obesity Diabetes mellitus Cystic fibrosis Malnutrition Connective tissue diseases

Hepatomegaly… Investigations – CBC and differential Reticulocyte count – Liver function tests Serum aspartate aminotransferase (SGOT) Alanine aminotransferase (SGPT) Alkaline phosphatase Total protein Albumin Bilirubin – Serum alpha 1 -antitrypsin – Serum ceruloplasmin

Hepatomegaly…… Prothrombin and activated partial thromboplastin times Selection of radiographic imaging procedures(e.g., abdominal U/S and CT) depends on suspected diagnosis Percutaneous liver biopsy is diagnostic of many disorders Investigations should be tailored to suspected diagnosis