Biliary Atresia

dr. Boris Crossley Nainggolan

 Introduction
• Biliary atresia is characterized by obliteration or
discontinuity of the extrahepatic biliary system,
resulting in obstruction to bile flow.

• Most common surgically treatable causeof

Cholestasis in newborns

• If not corrected

–Secondary biliary cirrhosis

 Embryology
• 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.
 Groups
Isolated Biliary Atresia Associated with situsinversus
or polysplenia/asplenia with or without
other congenital anomalies
Postnatal form Fetal /embryonic form
Accounts for 65-90% of cases 10-35% of cases.
 Kasai classification system
• Type I
– obliteration of the common bileduct
– the proximal ducts arepatent

• Type II
– Type IIa is atresia of the hepatic duct, with cystic
bile ducts found at theporta 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.

• Should not be confused with intrahepatic

biliary hypoplasia,
– Group of distinct and surgically noncorrectable
disorders.
Variants of ductal anatomy in biliary
atresia
 Pathophysiology/etiology
• 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 BiliaryAtresia

Malrotation

Preduodenal portal vein

Polysplenia

Interrupted inferior vena cava

Azygous continuation

Cardiac malformations
 Epidemiology
• Highest in Asian populations
• Biliary atresia occurs in between 1 in 10,000 and 1in
• 16,700 live births

• More common in females than inmales

• Long -term survival rate for infants withbiliary 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.

 Diagnosis
• 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 benormal

during the first few weeks of life.
 Physical findings
• 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
 Imp points
• Direct hyperbilirubinemia is always an abnormal
finding and may be present from birth in the
fetal/embryonic form.
• Consider biliary atresia in all neonateswith
direct hyperbilirubinemia.
• Physiologic unconjugated hyperbilirubinemia
rarely persists beyond 2 weeks.
– Infants with prolonged physiologic jaundice must be
evaluated for other causes.
• Ahigh index of suspicion is key to makinga
diagnosis

– because surgical treatment by age 2 months has

clearly been shown to improve

• the likelihood ofestablishing bile flow

• prevent the development of irreversiblebiliary

cirrhosis.
 Work Up
• 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 serumbilirubin.

2. Alkaline phosphatase (AP), 5' nucleotidase,

gamma-glutamyl transpeptidase(GGTP), serum
aminotransferases, serum bile acids
• 3. Serum alpha1-antitrypsin with Pityping:
• 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)
 Imaging studies
• Ultrasonography
– exclude specific anomalies of the extrahepatic
biliary system, particularly choledochal cysts.

– In biliary atresia it may demonstrate absenceof

the gallbladder and no dilatation of the biliary
tree.
– sensitivity and specificity do not exceed80%
 Gallbladder ghost triad
• 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 iminodiaceticacid
(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
– disadvantages
• 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 theliver biopsy is
considered diagnostic for biliary atresia
• Intraoperative cholangiography:
– demonstrates anatomy and patency of the extrahepatic
biliary tract.
 Management
• 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 acidmetabolism.

• In most cases of atresia, dissection into the

porta hepatis and creation of a Roux-en-Y
anastomosis with a 35-cm to 40-cmretrocolic
jejunal segment is the procedure ofchoice.
 Kasai procedure
• 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 portaare
inspected
– Liver is nodular and fibrotic with greenishcolour
• 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 isaspirated

– 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 fibrotictissue that
is located at the bifurcation of the main portal vein
into its left and rightbranches.
– 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 aredifficult
to identify.
• After portal plate dissection roux limbis
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 rightupper quadrant via a small
defect created in the avascular portion of the
transverse mesocolon.
• The side of the Roux limb is opened, andthe
portoenterostomy is created.
• Mesenteric defect created by construction of
the Roux limb and to anchor the Roux limbto
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
 POSTOPERATIVECARE
• 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 ADEKdrops—1 mL/day

• Prednisone—2 mg/kg/day and taper over 6 weeks

 Tid bits
• Pigmented stools either immediately or within the
first 10 to 14 days after surgery suggest successful
bile flow from the liver to the intestinal tract

• Half will continue to drain bile well, with efficient

clearance of jaundice and normal development.This
is considered a “cure,” and liver transplantation is
not necessary

• In other half liver failure will evetually develop

slowly  Liver transplantation

• In 15 to 30% early liver transplatation isrequired

 When to operate
• Goal is to operate before 70 to 90 days of life.
It is important to note, however, that hepatic
portoenterostomy is not contraindicated after
90 days of age.
• A study of older children (100 days)revealed
acceptable survival rates, supporting the use
of the procedure, if possible, even in older
infants
 Complications
• Cholangitis 33 to 60% of patientsfollowing
portoenterostomy.
– Treat with I/Vfluids, broad spectrumm antibiotics,
choleretic agents and steroids.

• Anastomosis failure to achieve adequatebile

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 notdrain bile,
– liver transplantation is the only option forlong-
term survival.
Available at surgicalpresentations