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Key Features

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Essentials of Diagnosis
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  • Extrahepatic neonatal cholestasis is characterized by

    • Complete and persistent cholestasis (acholic stools) in the first 3 months of life

    • Lack of patency of the extrahepatic biliary tree proved by intraoperative, percutaneous, or endoscopic cholangiography

    • Firm to hard hepatomegaly

    • Typical features on histologic examination of liver biopsy tissue

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General Considerations
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  • Progressive fibroinflammatory obliteration of the lumen of all, or part of, the extrahepatic biliary tree presenting within the first 3 months of life

  • Three types

    • Isolated (84% of cases): meconium and initial stools are usually normal in color, suggesting early patency of the ducts

    • BA with at least one malformation but without laterality defects (6%; cardiovascular, gastrointestinal, or genitourinary defects): bile duct presumably did not develop normally and is associated with other nonhepatic congenital anomalies

    • BA splenic malformation syndrome associated with laterality defects and polysplenia or asplenia (4–10%): supports an embryonic origin of BA

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Demographics
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  • Incidence highest in Asians, African Americans, and preterm infants

    • 1:6600 in Taiwan

    • 1:18,000 in Europe

    • 1:12,000 in United States

  • Slight female predominance

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Clinical Findings

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Symptoms and Signs
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  • Jaundice may be noted in the newborn period or develop by age 2–3 weeks

  • Urine stains the diaper

  • Stools are often pale yellow, gray, or acholic

  • Firm hepatomegaly is common

  • By age 6 months, the growth curve reveals poor weight gain

  • Symptoms of portal hypertension (splenomegaly, ascites, variceal bleeding) may develop in the first year of life

  • Pruritus, digital clubbing, failure to thrive, bone fractures, and bleeding complications may also occur later in childhood

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Differential Diagnosis
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  • Bile duct paucity

  • Metabolic liver disease (particularly α1-antitrypsin deficiency)

  • Choledochal cyst

  • Intrinsic bile duct obstruction (stones, bile plugs)

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Diagnosis

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Laboratory Findings
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  • No single laboratory test consistently differentiates BA from other causes of complete obstructive jaundice

  • Aminotransferases are only moderately elevated in BA

  • Serum albumin and blood clotting factors are normal early in the disease

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Imaging
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  • Ultrasonography

    • Should be performed to exclude the presence of choledochal cyst and identify intra-abdominal anomalies

    • A triangular cord sign in the hepatic porta suggests BA

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Diagnostic Procedure
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  • Intraoperative cholangiogram should be performed as soon as possible if BA cannot be excluded by diagnostic evaluation and percutaneous liver biopsy

  • Liver biopsy specimens (particularly if obtained after age 6 weeks) can differentiate intrahepatic causes of cholestasis from BA in over 90% of cases

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Treatment

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  • Hepatoportoenterostomy (Kasai procedure)

    • Standard therapeutic procedure

    • Should not be undertaken in infants older than age 4 months because the likelihood of bile drainage at this age is very low

  • Portocholecystostomy (gallbladder Kasai procedure) may be performed if the gallbladder is present and the passage ...

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