Biliary Atresia
Persistent
conjugated hyperbilirubinemia (greater than 20% of total or 1.5 mg%) should be
evaluated.
evaluation includes
history and
physical exam,
partial and total
bilirubin determination,
type and blood
group,
Coomb's test,
reticulocyte cell
count
peripheral smear.
Cholestasis means
a reduction in bile flow in the liver, which depends on the biliary excretion
of the conjugated portion.
Reduced flow causes retention of biliary
lipoproteins that stimulates hypercholesterolemia causing progressive damage to
the hepatic cell, fibrosis, cirrhosis and altered liver function tests.
Biliary Atresia
(BA) is the most common cause of direct (conjugated) hyperbilirubinemia in the
first three months of life.
It is
characterized by progressive inflammatory obliteration of the extrahepatic bile
ducts, predominance of female patients.
The disease is the
result of an acquired inflammatory process with gradual degeneration of the
epithelium of the extrahepatic biliary ducts causing luminal obliteration,
cholestasis, and biliary cirrhosis.
The timing of the
insult after birth suggests a infectious etiology obtained transplacentally.
Almost 20% of
patients have associated anomalies such as:
polysplenia,
malrotation, situs inversus, preduodenal portal vein and absent inferior vena
cava.
Histopathology
progressive
destruction,
scar formation,
and chronic granulation tissue of bile ducts.
Physiologic
jaundice of the newborn is a common, benign, and self-limiting condition.
In BA the patient
develops jaundice by the second week of life.
The baby looks
active, not acutely ill and progressively develops acholic stools, choluria and
hepatomegaly.
Non-surgical causes
of cholestasis present as a sick, low weight infant who is jaundiced since
birth.
The evaluation of
the cholestatic infant
Sreen for perinatal
infectious (TORCH titers, hepatitis profile),
metabolic
(alpha-1-antitrypsin levels),
systemic and
hereditary causes.
Total bilirubin in
BA babies is around 6-10 mg%, with 50-80% conjugated.
Liver function
tests are nonspecific.
Lipoprotein-X
levels greater than 300 mg
Gamma Glutamyl
Transpeptidase (GGT) above 200 units
The presence of the yellow bilirubin pigment
in the aspirate of duodenal content excludes the diagnosis of BA.
Ultrasound study
of the abdomen = imaging study done to evaluate the presence of a gallbladder,
identify intra or extrahepatic bile ducts dilatation, and liver parenchyma
echogenicity.
The postprandial
contraction of the gallbladder eliminates the possibility of BA
Nuclear studies of
bilio-enteric excretion (DISIDA) after pre-stimulation with phenobarbital for
3-5 days is the diagnostic imaging test of choice.
The presence of
the radio-isotope in the GI tract excludes the diagnosis of BA.
Percutaneous liver
biopsy should be the next diagnostic step.
The
mini-laparotomy is the final diagnostic alternative.
Those infant with
radiographic evidence of patent extrahepatic biliary tract has no BA.
Medical management
of BA in our hospital is
Antibiotics
Cefotaxime and ampicillin for 14 days parenterally
Vitamin A,D,(once
a month and K( once a week) injections
Vitamin E orally
daily
removing the
obliterated extrahepatic biliary system,
anastomosing the
most proximal part to a bowel segment.
patients may
develop portal hypertension = manifest esophageal varices, hypersplenism, and
ascites.
Hepatic
transplantation if progressive liver failure
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