Biliary
atresia is a rare disease which affects newborns. It
causes
damage to the liver due to the
destruction of ducts which carry bile from the liver to
the small intestine. Biliary atresia is a progressive inflammatory
process that begins very soon after birth. In the most
common form, called extrahepatic biliary atresia, the
delicate ducts outside the liver are affected first. White
blood cells invade the ducts, which become damaged and may
whither or completely disappear. Bile is trapped inside
the liver and rapidly causes damage and scarring to the
liver cells. Further scarring of the liver tissue may
result in cirrhosis.
Signs and Symptoms:
Newborn
jaundice which does not improve by four weeks of age
especially if it appears after two weeks of age
Swollen
abdomen or enlarged liver
Dark urine
and pale, clay-coloured stools
Diagnosis:
Blood, urine and stool samples are
analyzed; the liver and bile ducts are examined with
ultrasound; a liver scan showing the liver and bile ducts is
performed; a liver biopsy is obtained.
Blood tests may
show abnormal results which indicate liver dysfunction with
increased bilirubin (a chemical marker in the blood for
jaundice).
A special needle
may be used to take a tiny piece of liver, which under a
microscope may indicate features typical of an obstruction to
the biliary system.
Surgical
exploration of the baby’s abdomen is necessary in most cases
of suspected biliary atresia to definitively make the
diagnosis.
Treatment:
The most common treatment is an
operation called the Kasai procedure (named for the surgeon
who developed it). In this operation the damaged duct
outside the liver is removed and replaced with a new
drainage system made from a piece of the small intestine.
This may partially or fully alleviate the jaundice, but it
will not reverse the liver damage that has already occurred
or prevent any low-grade ongoing damage. The Kasai procedure
is successful in one-third to one-half of all patients and
if jaundice is fully relieved, most children will grow and
develop quite normally. Cirrhosis, or scarring of the liver,
generally occurs despite a successful Kasai procedure.
Bile flow is re-established in approximately 80% of infants
who are operated on when younger than 3 months of age. Of
these, about 50% will have some bile drainage and as many as
30% will have complete bile drainage with a return to normal
bilirubin. About 20% of infants will not be helped by the
Kasai procedure. In these cases, the only other treatment
option is a
