Hepatoportoenterostomy

A hepatoportoenterostomy, or Kasai portoenterostomy is a surgical treatment performed on infants with biliary atresia to allow for bile drainage. In these infants, the bile is not able to drain normally from the small bile ducts within the liver into the larger bile ducts that connect to the gall bladder and small intestine.

The surgery involves exposing the porta hepatis (the area of the liver from which bile should drain) and attaching part of the small intestine to the exposed liver surface. The rationale for this approach is that minute residual bile duct remnants may be present in the fibrous tissue of the porta hepatis and thus provide direct connection with the intrahepatic ductule system to allow bile drainage.^[1]

Prognosis

• If performed before 60 days of age, 80% of children achieve some bile drainage
• Prognosis is progressively worse the later surgery is done
• Post-operatively, cholangitis and malabsorption are common
• Many children with biliary atresia will require liver transplantation despite the attempted surgical repair

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