Biliary Atresia

Biliary Atresia is a topic covered in the Select 5-Minute Pediatrics Topics.

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Basics

Description

Biliary atresia (BA) is a congenital disease characterized by fibrosis, obstruction, and obliteration of the biliary system that is universally fatal without intervention.

Epidemiology

  • BA accounts for approximately 30% of cases of neonatal cholestasis.
  • BA is the most common cause of persistent cholestasis in infants and children and the most frequent indication for pediatric liver transplantation.
  • The disease affects 1:8,000 to 1:18,000 live births.

Risk Factors

Genetics

  • No single genetic mutation has been identified as the sole cause of BA, and there is no clear pattern of inheritance.
  • Genes influencing morphogenesis may contribute to pathophysiology.

Pathophysiology

  • Biliary obstruction begins at, or near, the time of birth and progresses throughout early infancy, leading to damage and ultimately scarring of liver parenchyma.
  • Approximately 20% of biliary patients have at least one other major congenital anomaly (i.e., embryonal form) including splenic malformation, interrupted inferior vena cava, midline liver, situs inversus, preduodenal portal vein, and intestinal malrotation.
  • More common form is the perinatal form that is not associated with malformations.

Etiology

Etiology is not completely defined, but many different pathogenic mechanisms have been proposed, including the following:

  • Perinatal infection of the liver and biliary tract with potential organisms including cytomegalovirus, rotavirus, and reovirus
  • Immune dysregulation
  • Defective morphogenesis
  • Environmental toxin exposure
  • Vascular insufficiency

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