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Definition
Biliary atresia is a condition in which the normal extrahepatic
biliary system is disrupted. Progressive damage of extrahepatic and
intrahepatic bile ducts secondary to inflammation, leading to fibrosis,
biliary cirrhosis, and eventual liver failure .
Incidence
Biliary atresia affects approximately 1 in 10,000-15,000 births and
occurs in 2 distinct forms: fetal-embryonic and postnatal.
Types
The fetal-embryonic form appears in the first 2 weeks of life and
10-20% of affected neonates have associated congenital defects.
The postnatal form of biliary atresia is typically found in neonates
and infants aged 2-8 weeks. Progressive inflammation and
obliteration of the extrahepatic bile ducts occur after birth. This form
is not associated with congenital anomalies, and infants may have a
short jaundice-free interval.
Classification:
Three main types of biliary atresia are surgicaly defined:
1 -In type I, the common bile duct is obliterated while the proximal bile
ducts are patent.
2 -In type II, atresia of the hepatic duct is seen. In type IIa, the cystic
and common bile ducts are patent, whereas in type IIb, the cystic,
common bile duct and hepatic ducts are all obliterated.
3-Type III atresia refers to discontinuity of both right and left hepatic
ducts to the level of the porta hepatis. Unfortunately, type III biliary
atresia is common, accounting for >90% of cases.
Pathophysiology
Infection with cytomegalovirus, group C rotavirus, and reovirus type
3 have been implicated in certain cases. Cholestasis almost
certainly contributes to ongoing hepatocellular and biliary damage.
Histologic findings on liver biopsy typically include acute or chronic
inflammatory change with obstruction, fibrosis, and the proliferation
of ductal and glandular element.
Investigations continue---Cholangiography
Duodenal intubation
To perform this study, a nasogastric tube is placed in the distal duodenum.
The absence of bilirubin in aspirated fluid suggests obstruction.
Liver biopsy
Liver Transplant
If there is still not enough bile flow with the Kasai
procedure, liver transplantation is a final option. A
liver transplant operation removes the damaged liver
and replaces it with a new liver from a donor.
After transplant surgery, the child's health may
improve quite quickly. However, the child's body
might reject the new organ. To prevent rejection, a
strict schedule of anti-rejection medications must be
taken.
After a transplant, ongoing lifelong care is required.
Frequent contact with physicians and other
members of the transplant team is also necessary.