Atresia Bilier

Biliary atresia is a congenital condition in which the

pathway for bile to drain from the liver to the intestine
is undeveloped.
It is the most common lethal liver disease in children

Type 1 (3%): Atresia limited to

common bile duct
Type 2 (6%): Cyst in the liver hilum
communicating with "hairy"
intrahepatic bile ducts
Type 3 (19%): Gallbladder, cystic duct
and common bile duct patent
Type 4 (72%): Complete extrahepatic
biliary atresia
Atresia bilier adalah bagian dari Kolestasis Pada Bayi

Kolestasis adalah gangguan

pembentukan, sekresi dan
pengaliran empedu mulai dari
hepatosit, saluran empedu intrasel,
ekstrasel dan ekstra-hepatal.

menyebabkan perubahan indikator

biokimia, fisiologis, morfologis, dan
klinis karena terjadi retensi bahanbahan larut dalam empedu.

Dikatakan kolestasis apabila kadar

bilirubin direk melebihi 2.0 mg/dl atau 20%
dari bilirubin total.

Beberapa penelitian pada bayi yang akan

lahir dengan atresia bilier: didapatkan
level enzim digestive yang dihasilkan oleh
defekasi fetus dalam cairan amnion yaitu
kadar gamma glutamyl transpeptidase
rendah pada usia 18 minggu

Etiologi

Etiologi tidak diketahui

Adanya gangguan pada pembentukan duktus bilier

pada trimester pertama

Beberapa penelitian menyebutkan gangguan ini

disebabkan oleh infeksi virus seperti
cytomegalovirus, respiratory synctial virus, epsteinBarr virus dan human papiloma virus. Tetapi tidak
ada hubungannya dengan virus hepatitis A, B,C

Genetik : pada beberapa kasus familial, adanya

insiden HLA B12 dan haptotype A9B5 dan A28-B35

DIAGNOSIS

Prenatal diagnosis: Types 1 and 2 of BA

(billiary atresia), which are rare, can be
suspected on prenatal USG if a cystic
structure is detected in the liver
hilum : post-natal check-up has to
distinguish a cystic form of BA, which
requires urgent surgery, and a choledocal
cyst, whose treatment can usually be
delayed.

Clinical features: After birth, the clinical triad

of BA is :
1) jaundice which lasts after 2 weeks of life.
2) decoloured (white) stools, and dark urines
3) hepatomegaly.
4) The general condition of the child is
usually good, as well as growth in weight
and height. Late symptoms are:
splenomegaly (portal hypertension),
ascitis, hemorrhage, which can be
intracranial (lack of absorption of vitamine
K).

Ultrasonography: Ultrasonography of the liver

is performed after 12 hours fasting (with IV
fluids infusion): it shows no bile duct
dilatation.

BA can be suspected
if the gallbladder is shrunken despite fasting
if the liver hilum appears exceedingly
hyperechogenic ("triangular chord sign")
if there is a cyst in the liver hilum,
if features of the polysplenia syndrome are
identified: multiples spleens, preduodenal
portal vein, absence of retrohepatic vena cava

Cholangiography: In the cases where the

gallbladder seems normal on US scans, a
cholangiography is needed to assess the
morphology and permeability of the biliary
tree.

Liver biopsy: The main features suggesting

BA are biliary plugs, ductular proliferation,
portal oedema and/or fibrosis. As in any
other cause of neonetal cholestasis, giant
cell transformation may be observed

Others: Liver function tests show

cholestasis (with elevated cholesterol and
g GTs). Hepatobiliary scintigraphy (HIDA
scans) shows failure to excrete the marker
in the intestine, but this can also be
observed in any severe neonatal
cholestasis. Moreover, scintigraphy can be
falsely reassuring in the early stage of BA.

DAT (duodenal aspiration test)

Treatment:
The current management of BA patients
involves two steps:
1) in the neonatal period, the Kasai
operation, which aims at restoring a biliary
flow to the intestine.
2) subsequent liver transplantation in case
of failure of the Kasai operation to restore
the biliary flow and/or complications of the
biliary cirrhosis