JAUNDICE

• Jaundice is present during the first week of life in 60% of

term infants and 80% of preterm infants.
• There is usually an accumulation of unconjugated
bilirubin, but there may also be deposition of direct
bilirubin.

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• During fetal life, the placenta is the principal route of elimination of unconjugated
bilirubin.
• In the newborn infant, bilirubin is conjugated through the activity of glucuronyl
transferase.
• In the conjugated form, bilirubin is water soluble, which results in its excretion into
the biliary system and then into the GI tract.
• Newborns have a relatively high level of circulating hemoglobin and relative
immaturity of the conjugating machinery.
• This results in a transient accumulation of bilirubin in the tissues, which is
manifested as jaundice.
• Physiologic jaundice is evident by the second or third day of life and usually resolves
within approximately 5 to 7 days.
• By definition, jaundice that persists beyond 2 weeks is considered pathologic, also in
the first 24 hrs..

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• Pathologic jaundice may be due to biliary obstruction, increased
hemoglobin load, or liver dysfunction.
• The work-up of the jaundiced infant therefore should include a
search for the following possibilities:
• (a) obstructive disorders, including biliary atresia, choledochal cyst,
and inspissated bile syndrome;
• (b) hematologic disorders, including ABO incompatibility, Rh
incompatibility, and spherocytosis;
• (c) metabolic disorders, including alpha1-antitrypsin deficiency,
galactosemia, and pyruvate kinase deficiency; and
• (d) congenital infection, including syphilis and rubella

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 Biliary Atresia

• PATHOGENESIS
• Biliary atresia is a rare disease associated with significant
morbidity and mortality.
• This disease is characterized by a fibro proliferative
obliteration of the biliary tree that progresses toward hepatic
fibrosis, cirrhosis, and end-stage liver failure.
• The incidence of this disease is approximately 1 in 5000 to 1
in 12,000.
• The etiology of biliary atresia is likely multifactorial.

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 CLINICAL PRESENTATION
• Infants with biliary atresia present with jaundice at birth or shortly
thereafter.
• The diagnosis of biliary atresia is frequently not entertained by
pediatricians, in part because physiologic jaundice of the newborn is
so common and biliary atresia is so uncommon.
• For this reason, a delay in diagnosis is not unusual.
• However, infants with biliary atresia characteristically have acholic,
pale gray stools, secondary to obstructed bile flow.
• With further passage of time, these infants manifest progressive
failure to thrive and, if untreated, develop stigmata of liver failure and
portal hypertension, particularly splenomegaly and esophageal
varices.
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 Inspissated Bile Syndrome
• The term inspissated bile syndrome is applied to patients with
normal biliary tracts who have persistent obstructive jaundice.
• Increased viscosity of bile and obstruction of the canaliculi are
implicated as causes.
• The condition has been seen in infants receiving parenteral
nutrition, but it is also encountered in patients with disorders
associated with hemolysis and in patients with cystic fibrosis.
• In some instances, no etiologic factors can be defined.
• Cholangiography is both diagnostic and therapeutic in
inspissated bile syndrome.

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 Choledochal Cyst
• CLASSIFICATION
• The term choledochal cyst refers to a spectrum of congenital biliary
tract disorders that were previously grouped under the name
idiopathic dilatation of the common bile duct.
• Based on the classification system proposed by Alonso-Lej, five
types of choledochal cyst are described.
• Type I cysts are characterized by fusiform dilatation of the bile duct.
This type is the most common and is found in 80 to 90% of cases.
• Type II choledochal cysts appear as an isolated diverticulum
protruding from the wall of the common bile duct.
• The cyst may be joined to the common bile duct by a narrow stalk.

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• Type III choledochal cysts arise from the intraduodenal portion of the common bile
duct and are also known as choledochoceles.
• Type IVA cysts consist of multiple dilatations of the intrahepatic and extrahepatic bile
ducts.
• Type IVB choledochal cysts are multiple dilatations involving only the extrahepatic
bile ducts.
• Type V cysts (Caroli's disease) consist of multiple dilatations limited to the
intrahepatic bile ducts.
• Choledochal cyst is most appropriately considered the predominant feature in a
constellation of pathologic abnormalities that can occur within the pancreatobiliary
system.
• Frequently associated with choledochal cyst is an anomalous junction of the
pancreatic and common bile ducts.
• The etiology of choledochal cyst is controversial.

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 CLINICAL PRESENTATION
• Choledochal cyst is more common in females than in males (4:1).
• Typically these cysts present in children beyond the toddler age group.
• The classic symptom triad consists of
– abdominal pain,
– mass, and
– jaundice.
• However, this complex is actually encountered in fewer than half of patients.
• The more usual presentation is that of episodic abdominal pain, often recurring over the
course of months or years and generally associated with only minimal jaundice that may
escape detection.
• If the disorder is left undiagnosed, patients may develop cholangitis or pancreatitis.
• Cholangitis may lead to the development of cirrhosis and portal hypertension.
• Choledochal cyst can present in the newborn period, with symptoms very similar to those of
biliary atresia.
• Often neonates have an abdominal mass at presentation.

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 DIAGNOSIS
• Choledochal cyst is frequently diagnosed in the fetus during screening
prenatal ultrasonography.
• In the older child or adolescent, abdominal ultrasonography may reveal a
cystic structure arising from the biliary tree.
• CT will confirm the diagnosis.
• These studies show the dimensions of the cyst and define its relationship
to the vascular structures in the porta hepatis, as well as the intrahepatic
ductal configuration.
• Endoscopic retrograde cholangiopancreatography is reserved for cases
in which confusion remains regarding the diagnosis after evaluation by
less invasive imaging modalities.
• Magnetic resonance cholangiopancreatography may provide a more
detailed depiction of the anatomy of the cyst.

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 TREATMENT
• The cyst wall is composed of fibrous tissue and is devoid of
mucosal lining.
• As a result, the treatment of choledochal cyst is surgical
excision followed by biliary-enteric reconstruction.
• There is no role for internal drainage by cystenterostomy,
which leaves the cyst wall intact and leads to the inevitable
development of cholangitis.
• Rarely, choledochal cyst can lead to the development of a
biliary tract malignancy.
• This provides a further rationale for complete cyst excision.

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• The prognosis for children who have undergone complete
excision of choledochal cyst is excellent.
• Complications include
– anastomotic stricture,
– cholangitis, and
– intrahepatic stone formation.
• These complications may develop a long time after
surgery has been completed.

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