Anatomic Causes

1. Extrahepatic biliary atresia. The most common cause of neonatal cholestasis; more common in females. Underlying process is progressive destruction of the biliary tree lumen, resulting in complete obliteration by 3 months of age. Although injury seems to begin in the extrahepatic biliary tree, it progresses to include intrahepatic biliary radicals as well. Etiology is undetermined. Approximately 10% of patients have the embryonic form of disease with associated anomalies, including polysplenia or asplenia, preduodenal portal vein, situs inversus, and malrotation. Most patients present with jaundice and acholic stools between the 2nd and 4th weeks of life.

2. Gallstone disease. Seen in patients with hemolytic disease, hypothyroidism, cystic fibrosis, or those receiving prolonged diuretic or parenteral nutrition therapy.

3. Choledochal cyst. Essentially a cystic outpouching of the biliary tree; there are five different types.

4. Other. Sclerosing cholangitis, infectious cholangitis, inspissated bile plugs, spontaneous bile duct perforation, biliary hypoplasia, Caroli disease, extrinsic compression of bile ducts, congenital hepatic fibrosis.

B. Infectious Causes. Include cytomegalovirus (CMV), rubella, hepatitis A though G, herpesviruses (simplex, zoster, HHV 6), adenovirus, enteroviruses, Epstein-Barr virus (EBV), reovirus 3, parvovirus B19, HIV, bacterial sepsis, E coli UTI, cholangitis, syphilis, listeriosis, tuberculosis, toxoplasmosis. Most of these patients present with other signs of infection. Laboratory evaluation usually reveals evidence of hepatocellular injury with elevated ALT and AST in addition to cholestasis.

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