Biliary Atresia - Dr Rohit Bhaskar

Biliary Atresia
©2021 DR ROHIT BHASKAR PT HTTPS://WWW.PT-PEDIA.COM/

• Biliary atresia is characterized by
obliteration or discontinuity of the
extrahepatic biliary system, resulting in
obstruction to bile flow.
• Most common surgically treatable
cause of Cholestasis in newborns
• If not corrected
–Secondary biliary cirrhosis

• The biliary system originates from the
hepatic diverticulum of the foregut
at 4 weeks’ gestation.
• This differentiates into cranial and
caudal components, which give
rise to the intrahepatic and
extrahepatic bile ducts,
respectively.

Isolated Biliary Atresia Associated with situs inversus
or polysplenia/asplenia with or without
other congenital anomalies
Postnatal form Fetal /embryonic form
Accounts for 65-90% of cases 10-35% of cases.

• Type I
– obliteration of the common bile duct
– the proximal ducts are patent
• Type II
– Type IIa is atresia of the hepatic duct, with cystic
bile ducts found at the porta hepatis
– Type IIb is atresia of the cystic duct, common
bile duct, and hepatic ducts

• Type III
– >90% of patients
– atresia occurs at porta hepatis.
• Shouldnot be confused with
intrahepatic biliary hypoplasia,
– Group of distinct and surgically
noncorrectable disorders.

• Multifactorial
– Genetic
– Inflammatory
– infectious
• Infectious agents causing bile duct
obliteration (H/P evidence of inflammatory
lesion)
– reovirus type 3
– rotavirus and
– cytomegalovirus (CMV)

• Other causes include
– bile duct ischemia,
– abnormal bile acid
metabolism,
– pancreaticobiliary
maljunction
– environmental toxins

Congenital Anomalies Associated with Biliary
Atresia
Malrotation
Preduodenal portal vein
Polysplenia
Interrupted inferior vena cava
Azygous continuation
Cardiac malformations

• Highest in Asian populations
• Biliary atresia occurs in between 1 in 10,000 and 1 in
• 16,700 live births
• More common in females than in males
• Long -term survival rate for infants with biliary atresia following
portoenterostomy
– 47-60% at 5 years
– 25-35% at 10 years
• The fetal/perinatal form is evident within the first 2 weeks of life.
• The postnatal type presents in infants aged 2-8 weeks.

• History
– jaundice, dark urine, and light stools.
– In most cases, acholic stools are not
noted at birth but develop over the first
few weeks of life.
– Appetite, growth, and weight gain may
be normal during the first few weeks of
life.

• Hepatomegaly may be present early, and
the liver is often firm or hard to palpation.
• Splenomegaly is common,
– Enlarging spleen suggests progressive cirrhosis with
portal hypertension
• In fetal/neonatal form
(polysplenia/asplenia syndrome),
– midline liver may be palpated in the epigastrium .
• Cardiac murmurs, associated
cardiac anomalies

• Labs
1. Serum bilirubin (total and direct)
• infants show only moderate elevations in total
bilirubin, which is commonly 6-12 mg/dL, with
the direct fraction comprising 50-60% of total
serum bilirubin.
2. Alkaline phosphatase (AP), 5'
nucleotidase, gamma-glutamyl
transpeptidase(GGTP), serum
aminotransferases, serum bile acids

• 3. Serum alpha1-antitrypsin with Pi
typing:
• Alpha1-antitrypsin deficiency is the most
common inherited liver disease that
presents with neonatal cholestasis.
• 4. Sweat chloride (Cl):
– Biliary tract involvement is a well-recognized
complication
of cystic fibrosis (CF)

• Ultrasonography
– exclude specific anomalies of the
extrahepatic biliary system, particularly
choledochal cysts.
– In biliary atresia it may demonstrate
absence of the gallbladder and no
dilatation of the biliary tree.
– sensitivity and specificity do not exceed
80%

• gallbladder length less
than
1.9 cm,
• a thin or indistinct
gallbladder wall,
• an irregular and
lobular contour
• 97% sensitive and 100%
specific for biliary
atresia.

• Hepatobiliary scintiscanning
– using technetium-labeled diisopropyl iminodiacetic acid
(DISIDA) nuclear scintiscan,
– intestinal excretion of radiolabel confirms patency of the
extrahepatic biliary system.
• If time allows, all jaundiced infants undergoing
hepatobiliary scintigraphy should be pretreated with
phenobarbital (5 mg/kg/day) for 5 days before the
study.
– sensitivity of hepatobiliary scintigraphy is high (~100%).
– The specificity is 93% specific, and 94.6% accurate in
diagnosing biliary atresia following pretreatment with
phenobarbital

• reliability of the scintiscan is diminished at very high
conjugated bilirubin levels (>20 mg/dL).
• 10% rate of false-positive or false-negative diagnostic
errors

• MRCP
– incomplete visualization of the
extrahepatic biliary system
– sensitivity and specificity of 90%
and 77%, respectively.

• Endoscopic retrograde cholangiopancreatography
(ERCP)
• Percutaneous liver biopsy
• most accurate nonsurgical diagnostic test
• differentiate between obstructive and
hepatocellular causes of cholestasis,
• with 90% sensitivity and specificity for biliary atresia
– bile ductular proliferation in the liver biopsy is
considered diagnostic for biliary atresia
• Intraoperative cholangiography:
– demonstrates anatomy and patency of the extrahepatic
biliary tract.

• Surgical intervention is the only mechanism for a
– definitive diagnosis (intraoperative cholangiogram)
– therapy (Kasai portoenterostomy).
• Preoperative oral supplementation with fat-soluble
vitamins or an intramuscular injection of vitamin K (1
mg) should be considered.
• During the operation, the fibrotic biliary tract
remnant is identified, and the patency of the
biliary system is assessed

• In cases in which biliary patency is
associated with ductal hypoplasia, further
surgical intervention is not indicated,
• Bile may be collected to evaluate for
disorders of bile acid metabolism.
• In most cases of atresia, dissection into the
porta hepatis and creation of a Roux-en-Y
anastomosis with a 35-cm to 40-cm
retrocolic jejunal segment is the procedure
of choice.

• Infant should be placed supine
• Right upper abdominal incision given
• LHQ is searched for spleen
– Absence of the spleen or the finding of
polysplenia can alert the surgeon to the
presence of important associated anomalies
• Next, the liver, biliary structures, and porta
are inspected
– Liver is nodular and fibrotic with greenish colour

• If Rudimentary fibrous gallbladder is noted at
initial exploration and if it clearly has no
lumen,
– diagnosis of biliary atresia has been confirmed
and the operation can proceed with dissection
of the portal plate
• If G.B is Normal then fluid in lumen of G.B is aspirated
– If fluid is white proceed for portal plate dissection
– If fluid is dark or there is any doubt intraoperative
cholangiogram is done

If contrast flows freely into the
duodenum and into
intrahepatic bile ducts, then
patency of the biliary tree has
been established and the
diagnosis of biliary atresia
excluded.
In this scenario, a wedge liver
biopsy should
be performed, the
cholangiogram catheter
removed, the cholecystotomy
closed, and the operation
concluded

• The peritoneum overlying the
hepatoduodenal ligament is opened to
allow identification of the structures in this
area.
• Fibrous remnant of the distal common bile
duct is often present here.
• As dissection continues proximally, the biliary
remnant develops into a cone of fibrotic
tissue that is located at the bifurcation of the
main portal vein into its left and right
branches.
– most important landmark during the dissection of
the portal plate and should be the goal of every
dissection

• The bifurcation of the portal vein
should be used as a landmark in
such cases where the biliary
remnants and portal plate are
difficult to identify.

• After portal plate dissection roux limb is
constructed.
• Proximal jejunum is identified and transected about
10 centimeters distal to the ligament of Treitz
• The distal end, destined for the right upper quadrant,
is oversewn and the Roux limb is measured to 40 to
50 centimeters. At this location,
an end-to-side jejunojejunostomy is created with
interrupted absorbable sutures.
• The oversewn end of the Roux limb is carefully
brought into the right upper quadrant via a small
defect created in the avascular portion of the
transverse mesocolon.

• The side of the Roux limb is opened,
and the portoenterostomy is
created.
• Mesenteric defect created by
construction of the Roux limb and to
anchor the Roux limb to the edges of
the defect in the transverse
mesocolon.
• Small closed suction drain is placed
near the portoenteric anastomosis,
and a wedge liver biopsy is routinely
performed

• Drainage of gastric secretions with a
nasogastric tube should continue for
the first 48 hours, then removed
• NPO can be broken at 2nd or 3rd day.
• Drain removed at 5th P.O.D
• Antibiotics, a choleretic agent, fat-
soluble vitamin supplements, and
an oral steroid taper is used
routinely.

Postoperative Medical Regimen After Hepatic
Portoenterostomy
• Ursodiol (Actigall)—10-15 mg/kg/dose BID day
• Trimethoprim-Sulfamethoxazole—2.5 mg/kg/day based on
Trimethoprim component dosing
• Vitamins ADEK drops—1 mL/day
• Prednisone—2 mg/kg/day and taper over 6 weeks

• Cholangitis- 33 to 60% of patients following
portoenterostomy.
– Treat with I/V fluids, broad spectrumm antibiotics,
choleretic agents and steroids.
• Anastomosis failure to achieve adequate bile
drainage.
• Portal hypertension occur in more than 34-76% of
infants.
– Eventually require liver transplant.
• Progressive liver disease .

• Hepatocellular carcinoma
• Progressive fibrosis and biliary
cirrhosis develop in children who
do not drain bile,
– liver transplantation is the only option
for long- term survival.

THANK YOU

Biliary Atresia - Dr Rohit Bhaskar

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