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06-04-2008, 09:18 AM
Incidence: Atresia is present in one per 14 000 live births, and affects male and females equally.
Aetiology and Pathology:
The extrahepatic bile ducts are progressively destroyed by an inflammatory process which starts around the time of birth. Intrahepatic changes also occur and eventually result in biliary cirrhosis and portal hypertension.
The untreated child dies before the age of 3 years of liver failure or hemorrhage.
Associated anomalies include (in about 20 per cent of cases) cardiac lesions, polysplenia, situs inversus, absent vena cava and a preduodenal portal vein.
The inflammatory destruction of the bile ducts has been classified into three main types :
Type I - atresia restricted to the common bile duct.
Type II - atresia of the common hepatic duct.
Type III - atresia of the right and left hepatic ducts.
m Clinical Features
About one-third of cases are jaundiced at birth. In all cases, jaundice is present by the end of the first week and deep­ens progressively.
The meconium may be a little bile stained but later the stools are pale and the urine is dark.
Prolonged steatorrhoea gives rise to osteomalacia (biliary rickets).
Pruritis is severe. In some cases clubbing and skin xanthomas, probably related to a raised serum cholesterol.
m Differential Diagnosis:
This includes any form of jaundice in a neonate giving a cholestatic picture.
Liver biopsy and radio­nuclide excretion scans are essential.
m Treatment:
I- If a patent segment of proximal bile duct is found (10 percent of type I lesions), a direct Roux-en-Y anastomosis will achieve bile flow in 75 per cent, but progressive fibrosis results in disappointing long-term results.
II- Type II and III are treated by radical excision of all bile-duct tissue up to the liver capsule. A Roux-en-Y loop of jejunum is anastomosed to the exposed area of liver capsule above the bifurcation of the portal vein.
III- Liver transplantation may be considered in the failures.
SOURCE: DR. AYMAN SALEM'S BOOK
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