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let's move on to another cause of
congenital hyperbilirubinemia which is
biliary atresia this is a complete or
partial obstruction of the lumen of the
extra hepatic biliary tree within the
first three months of life so this is
not an intra hepatic problem this is an
extra hepatic problem and what we
typically see as you can see on the left
side of your slide is inflammation along
the biliary tree with this sort of Chain
of Lakes phenomenon where there are
multiple constrictions of the biliary
tract so let's go through how this
proceeds normal development of a biliary
tree and then a patient may have an
insult
generally perinatal II such as a viral
infection or some problem that causes
the bile duct to be inflamed some sort
of injury to the mucosa this results in
an inflammatory response in immunologic
response inside the duct and leads to
infiltration of the duct and obstruction
this duct reaction causes an epithelial
proliferation of the cells within the
duct which results in a progressive
sclerosis and fibrosis of the duct which
thus is going to obliterate these ducts
this usually happens within the first
month of life and leads to a colossus
stasis essentially an inability to get
bile through those ducts leading to a
backup which results in hepatocellular
injury and a secondary cirrhosis so if
you suspect biliary atresia what should
you do well of course it's important to
take a good history and typically these
are healthy newborns they're full-term
they have a normal weight gain they
don't have a history of a maternal
infection in utero because this is an
acute thing that happened after birth
they'll have a brief perinatal jaundice
and then be jaundice free
because that was an in unconjugated
hyperbilirubinemia and then they
developed the jaundice later generally
it's a progressive jaundice that happens
about 8 weeks out and these children
will often have a colic or pale stools
you'll see this because the child is not
able to extrude the bile which is the
main source of pigment in stool these
stools look almost chalky and white in
nature the child will also have dark
urine because there will be Billy Ruben
levels and spilling into their urine on
exam you will of course see the jaundice
you should see scleral icterus but
generally these children are very well
appearing there well appearing children
who are look ok they're just turning
yellow you may notice a soft tip Adam
eglee not a firm mass like thing but
generally a large liver and they may
have a mild spleen as well simply from
vascular back-up and generally you get
labs and you'll notice typically their
total bilirubin levels are around 7 to
10 with a direct level of around 3 to 6
and as you can see this clearly meets
criteria for conjugated
hyperbilirubinemia they should have a
bumped GGT because of that inflammation
to their biliary tract and an elk fossa
and of course a bumped ast and alt as
well as they're now developing hepatic
toxicity from the bile backup so we
diagnose this condition through
ultrasound primarily and if there's more
delineation that's required we can do a
HIDA scan as is pictured above in the
first picture or we can do an
intraoperative Qalandia gram which may
be useful at determining what type of
problem is truly going on the patient is
repaired through surgery and a Kasai
procedure or a Kasai Puerto and her
ostomy is performed where the liver is
essentially filleted open and directly
Anasta most to a piece of intestine
allowing those multiple
ducks to spill directly into the
intestinal vault usually through a ruin
why or some sort of similar surgical
procedure transplant may have to occur
and that is typically curative of the
problem
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you