SURGICAL JAUNDICE

BY NUR FADILAH AZHANI

BT MOHAMMAD

JAUNDICE
DEFINITION:
Yellowish pigmentation of the skin,
the conjunctival membrane over
sclera and other mucous membranes
caused by hyperbilirubinemia
Normal total serum bilirubin values :
0.2-1.2 mg/dL
Jaundice may be clinically
recognizable until levels are
>3mg/dL

BILIRUBIN METABOLISM

Reticuloendothelial system

CAUSES OF JAUNDICE

UNCONJUGATED BILIRUBIN

CONJUGATED BILIRUBIN

-breastmilk jaundice
-hemolysis
coombs positive
-rhesus incompatibility
-ABO incompatibility
coombs negative
-red cell membrane defects
(eg.
Sphero/elliptocytosis)
-red cell enzyme defects (eg.
G6PD deficiency,pyruvate kinase
defeciency)
-hemoglobinopathy
-sepsis
-DIC
-increases enterohepatic
circulation
pyloric stenosis
intestinal obstruction
-decrease conjugation
-crigler-Najjaar syndrome
-Gilberts disease

decreased excretion
(conjugated)
-OBSTRUCTION
Biliary atresia
Choledochal cyst
Spontaneous bile duct
perforation
Hepatoblastoma,
hemangioma,neuroblastoma
-INFECTION
Septicemia
TORCHES
Hepatitis
Viral
-INHERITED/METABOLIC
Alpha 1 antitrypsin deficency
Alagilles syndrome
Cystic fibrosis
-TOXIC/DRUGS
Fetal alcohol syndrome

OBSTRUCTIVE
JAUNDICE, BILIARY
ATRESIA,
CHOLEDOCHOCAL

Lab values differentiating

jaundice

BILIARY ATRESIA

NORMAL ANATOMY OF BILE

DUCT

BILIARY ATRESIA
Obliteration or discontinuity of the
extrahepatic biliary system, resulting
in obstruction to bile flow
The most common cause of
prolonged jaundice in infancy

TYPES

Clinical features

 The cardinal signs and symptoms of

biliary atresia are jaundice, claycolored stools,urine dark brown,
hepatomegaly
for the first few months : active, growth
usually normal for the first few months
anemia, malnutrition, and growth
retardation develop gradually because
of malabsorption of fat-soluble vitamins
jaundice that persists beyond 2 weeks
should no longer be considered
physiologic

Investigations
Clinical Findings and Examination for Diagnosis of
Biliary Atresia
Routine Examinations
Color of stool
Consistency of the liver
Conventional liver function tests, including test for glutamyl transpeptidase
Special Examinations
Hepatitis A, B, C serologic studies
TORCHES titers
1-Antitrypsin level
Serum lipoprotein-X
Serum bile acid

Conrmation of patency of
extrahepatic bile ducts
Duodenal fluid aspiration
Ultrasonography
Hepatobiliary scintigraphy
Endoscopic retrograde
cholangiopancreatography Near-infrared
reflectance spectroscopy
Needle biopsy of the liver for
histopathologic studies
Laparoscopy Surgical cholangiography

LFT
Serum bilirubin (total and direct)
Conjugated hyperbilirubinemia defined as any
level exceeding 2mg/dL or 20% of total
bilirubin
Direct fraction : 50-60% of total serum bilirubin

ALP, GGT,serum aminotransferases

Does not discriminate between other causes of
neonatal cholestasis
GGT and ALP elevated in biliary obstructive
conditions
Aminotransferases not helpful in establishing
dx

 Serum lipoprotein-X
-is positive in all patients with biliary atresia,
although it also is positive in 20% to 40% of
patients with neonatal hepatitis

Confirmation of patency of
extrahepatic bile ducts
DUODENAL FLUID ASPIRATION
is recommended as an easy, noninvasive, and rapid
test because biliary atresia can be excluded if
typical yellow bilirubin-stained fluid is aspirated

ULTRASONOGRAPHY
should be performed in all jaundiced infants
will exclude other surgical causes of jaundice such
as choledochal cyst and inspissated bile
In biliary atresia, the intrahepatic ducts are not
dilated on ultrasonography because they are
affected by the inflammatory process.
In biliary atresia, the gallbladder is small, shrunken,
and noncontractile and there is increased
echogenicity of the liver

 Hepatobiliary scintigraphy with

technetium-labeled agents
is widely used for differentiating biliary
atresia from other cholestatic diseases
In biliary atresia, nucleotide uptake by
hepatocytes is rapid but excretion into the
bowel is absent
In hepatocellular jaundice, isotope uptake
is delayed owing to parenchymal disease
and excretion into the intestine may or may
not be demonstrated.

 LIVER BIOPSY
the most reliable test for establishing
the diagnosis

TREATMENT

KASAI PROCEDURE
KASAI OPERATION : named after the
surgeon Morio Kasai
Most effective in infants younger than
3months old as they usually have not
developed permanent liver damage
yet

PICTURE KASAI

removes the damaged

bile ducts, and brings up a
loop of intestine to
replace them
(PORTOENTEROSTOMY)

As a result, bile flows

straight to the small
intestine

ROUX-EN- Y
CONNECTION

COMPLICATION AFTER KASAI

Cholangitis
Portal hypertension
Hypersplenism
Fat soluble vitamin defeciency
vitamin E-peripheral neuropathy
vitamin D-rickets
vitamin A-visual defects
vitamin K-coagulation defects

PROGNOSIS
No bile drainage (10%)
Bile drainage (90%)
1/3 fail severe liver disease
1/3 inderterminate moderate liver
disease
1/3 cured minimal liver disease

LIVER TRANSPLANTATION
Biliary atresia is the most common
indication for liver transplantation in
children
Infants
whose jaundice is not cleared after
portoenterostomy
or those with complications associated
with end-stage chronic liver disease
despite successful portoenterostomy

CHOLEDOCHAL CYST

 A Choledochal cyst is a rare

congenital swelling of the hepatic or
bile duct
Can be intrahepatic, extrahepatic or
both

TODANI'S CHOLEDOCHAL
CYST CLASSIFICATION

Clinical features
Infants :
-jaundice and pale coloured stools : may
prompt workup for biliary atresia
-palpable mass in the right upper quadrant,
with hepatomegaly
Older children (classic triad):
-intermittent attacks of colicky abdominal pain
(primary bouts of pancreatitis)
-jaundice
-palpable mass in the right upper quadrant

Workup
No laboratory studies are specific for
the diagnosis
FBC : elevated TWC (increased
neutrophils) if presence of cholangitis
LFT : elevated aminotransferases and
ALP are non specific for choledochal
cyst
Serum amylase and lipase : both may
be elevated in presence of pancreatitis
Serum biochemistry : deranged if pt is
vomiting

IMAGING
Abdominal ultrasonography
The best screening method in patients
who are suspected of having
choledochal cyst
can diagnose during antenatal
ultrasonography
Ultrasonography clearly
demonstrates IHBD dilatation and the
state of the liver parenchyma

Endoscopic retrograde cholangiopancreatography

(ERCP)
can accurately visualize the configuration of the
pancreaticobiliary ductal system in fine detail
However, it is invasive ,unsuitable for repeated use and is
contraindicated during acute pancreatitis.
Magnetic resonance cholangiopancreatography
(MRCP)
can provide excellent visualization of the
pancreaticobiliary ducts, allowing detection of narrowing,
dilatation, and filling defects with medium to high degrees
of accuracy
noninvasive
However, in children younger than 3 years, MRCP may not
visualize the pancreaticobiliary ductal system because of
the small caliber

TREATMENT
If patient present with pancreatitis or
cholangitis :
Treat supportively prior to definitive
operative management

DEFINITIVE TREATMENT:
Complete resection of the cyst with
reconstruction of the biliary tract
association with the development of
cholangiocarcinoma