BILIARY ATRESIA

MANGULABNAN C., MANLAPAZ L., MARASIGAN M., MARCIAL

S., MARI S., MARZAN J., MATIBAG R., MATURAN J., MEDINA
D. MEDINA M., MERCADO K., MIGUEL C., MIMAY K., MONERA
O., MONTARDE M., MUPAS J., NAVAL J.
 PATHOGENESIS
• most
important surgical cause of jaundice in the
newborn period

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• incidence is approximately 1 in 20,000

•characterized by an obliterative process of the

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extrahepatic bile ducts and associated with hepatic
fibrosis

•etiology is unknown

• In the classic textbook Abdominal Surgery of Infancy

and Childhood, Ladd and Gross described the cause
of biliary atresia as an ". . . arrest of development
during the solid stage of bile duct formation."
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CLINICAL PRESENTATION
•Jaundice

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•a constant finding, is usually present at birth or
shortly thereafter, but may go undetected or
may be regarded as physiologic until the child is
2 or 3 weeks old

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•acholic, grey-appearing stools, secondary to
obstructed bile flow

•manifest progressive failure to thrive, and if

untreated, progress to develop stigmata of liver
failure and portal hypertension, particularly
splenomegaly and esophageal varices
 KASAI
CLASSIFICATION
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SYSTEM
widely used and divides cases

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of biliary atresia according to
their location and degree of
pathology
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TYPE 1: CBD is obliterated
proximal bile ducts are patent
TYPE 2: atresia of the hepatic duct is
seen, cystic bile ducts at porta
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hepatis
TYPE 2A: cystic and CBD are patent

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TYPE 2B: cystic, CBD and hepatic ducts
are all obliterated
Type 3: atresia refers to discontinuity of
both R and L hepatic ducts to the
level of the porta hepatis
common, ( >90% of cases)
 ULTRASOUND
• Detect associated anomalies such as:

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- abdominal polysplenia
- vascular malformation

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• Assess for the presence of other
causes of biliary obstruction including
choledochal cyst

• Gallbladder is either not visualized or

a micro gallbladder is seen
 ULTRASOUND

ULTRASONOGRAPHIC
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TRIANGULAR CORD (UTC)

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- represents a cone-shaped
fibrotic mass cranial to the
bifurcation of the portal vein

Initial procedure of choice in

investigating Biliary Tract
Obstruction
TRIANGULAR CORD

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 HEPATOBILIARY UTZ
ADVANTAGES

•Rapid
•Simultaneous scanning of GB, liver, bile
ducts, pancreas
•Accurate identification of dilated bile
ducts
•Not limited by jaundice, pregnancy
•Guidance for fine-needle biopsy

LIMITATIONS

•Bowel gas
Hepatobiliary scintigraphy
•with technecium-labeled
iminodiacetic derivatives
•used to differentiate biliary
atresia from non-
obstructive causes of
cholestasis
•administration of
phenobarbital (5mg/kg/day)
for 5 days before the scan
is recommended
Hepatobiliary scintigraphy

•Hepatic uptake of the agent is normal in

patients with biliary atresia but excretion
into the intestines is absent

•Obtaining a follow-up scan after 24 hours

is of value to determine patency of the
biliary tree

•Sensitive but not specific test for biliary

atresia
LIVER BIOPSY
may be ordered to diagnose or stage any
of the following conditions or disorders

• jaundice
• cirrhosis
• repeated abnormal results from liver
function tests
• alcoholic liver disease
• unexplained swelling or enlargement
of the liver (hepatomegaly)
LIVER BIOPSY
may be ordered to diagnose or stage
any of the following conditions or
disorders

•suspected drug-related liver damage

such as acetaminophen poisoning
• hemochromatosis
• intrahepatic cholestasis
• hepatitis
• primary cancers of the liver
(hepatomas, cholangiocarcinomas, and
angiosarcomas)
LIVER BIOPSY
may be ordered to diagnose or stage
any of the following conditions or
disorders

• metastatic cancers of the liver

• post-liver transplant to measure graft
rejection
• fever of unknown origin
• suspected tuberculosis, sarcoidosis, or
amyloidosis
• genetic disorders such as Wilson’s
disease
PERCUTANEOUS LIVER BIOPSY
PERCUTANEOUS LIVER BIOPSY

•mainstay of oncologic diagnosis for more

than two decades, since the advent of
ultrasound and computed tomography
(CT)

•basic principles of fine needle aspiration

and core needle biopsy can be applied to
almost any site in the body, with subtle
differences in technique depending on the
organ being investigated and the imaging
modality utilized
ADVANTAGES OF PLB

•Outpatient procedure — patient is

typically
home within 4 hours

• Decreased costs

• Local anesthesia or intravenous

sedation, rather than general anesthesia

• Fewer complications
 PATIENT PREPARATION

•Most procedures can be performed using IV

sedation and local anesthesia

•General anesthesia may be preferable in some

cases, including pediatric patients

•All patients require observation for a few hours

following the procedure
PATIENT PREPARATION

•Pertinent patient history includes

bleeding dyscrasias and use of
blood thinning medications,
including aspirin

•Contraindications: uncorrected
coagulopathy and lack of a safe
route to pass a needle

•limit food or drink for a period of 4-

8 hours before the biopsy
 CONTRAINDICATIONS:
PERCUTANEOUS LIVER BIOPSY
•platelet count < 50,000

•PT > 3s over the reference interval,

indicating a possible clotting
abnormality

•a liver tumor with a large number of

veins

•large amount of abdominal fluid

(ascites)
 CONTRAINDICATIONS:
PERCUTANEOUS LIVER BIOPSY
• infection anywhere in the lungs, the
lining of the chest or abdominal wall, the
biliary tract, or the liver

• benign tumors (angiomas) of the liver,

which consist mostly of enlarged or newly
formed blood vessels and may bleed
heavily

• biliary obstruction (bile may leak from

the biopsy site and cause an infection of
the abdominal cavity)
PERCUTANEOUS LIVER
BIOPSY
•Several cholestatic conditions, including
biliary atresia, may demonstrate an
evolving histopathological pattern

•Accordingly, biopsies are not usually

diagnostic in those younger than 2
weeks, and serial samples, usually at 2-
week intervals, may be required to reach
a definitive diagnosis
HISTOLOGIC FINDINGS
•active inflammation with bile duct
degeneration
•a chronic inflammatory reaction with
proliferation of both ductular and glandular
elements, and fibrosis.

•progressive nature of the disorder is

confirmed by its evolving histological
picture
•evidence of biliary tract obstructive
disease confirmed by liver biopsy findings
determines which infants require
exploratory laparotomy and intraoperative
cholangiography
Portal bile ductular proliferation, bile
plugging, portal-portal fibrosis, and an
acute inflammatory reaction are
characteristic findings in infants with
neonatal cholestasis of an obstructive
etiology
 CLINICAL COURSE
•a progressive, sclerosing, inflammatory
process that affects the extrahepatic
biliary tract, leading to ductular luminal
obliteration and cirrhosis

•Subject to progressive and rapid

development of end-stage liver disease
due to the persistent intrahepatic
inflammatory process

•Prognosis: extremely poor, with death

from liver failure usually occurring within
2 years
 CLINICAL COURSE

•initial opportunity for success in the management

of this disease relies on the EARLY
RECOGNITION of abnormal direct
hyperbilirubinemia

•30% infants undergoing

hepatoportoenterostomy prior to 60 days (8
weeks) of age have a long term successful
outcome and do not require liver transplantation
 CLINICAL COURSE
•Older children and those with
preoperative evidence for bridging
fibrosis seen on liver biopsy
predictably do less well

•Liver transplantation is lifesaving

and associated with an 82% 5-year
survival

•Bile drainage is anticipated when

the operation is carried out early
 CHOLESTASIS
Retention/ Regurgitation Bile Acid delivery to
Proximal Small Intestine
Bile Acids: pruritus
: hepatotoxicity
Bilirubin: jaundice Intraluminal Bile Acid conc.
Cholesterol: xanthomas
: hypercholesterolemia
Trace Elements: copper, etc
Malabsorption

Progressive Liver Disease (Biliary Cirrhosis)

Portal Hypertension: Liver Failure

Hypersplenism, varices, ascites
POST-OPERATIVE COMPLICATIONS
CHOLANGITIS

•Common postoperative complication

•No effective strategy to completely

eliminate this complication

•effectiveness of long-term prophylactic

antibiotics has not been fully resolved
 ASCITES

•Lasts for a few weeks

•If ascites lasts for more than 6

weeks, cirrhosis is likely present and
the infant will probably require a liver
transplant