Jaundice

The Surgical Examination of Children Second Edition

Introduction
Jaundice is a common sign in the first 2

weeks of life..
Deep jaundice or the development of

jaundice within 24 h of birth implies that

additional factors such as haemolysis,
infection or inborn metabolic errors may be
present
An unconjugated bilirubin level above 300

mmol/l (or 250 mmol/l in premature infants)

may damage the brain (kernicterus)
The Surgical Examination of
Children Second Edition

 Jaundice that

appears later than,

or persists into,
the third week of
life is not likely to
be benign
physiological
jaundice

The Surgical Examination of

Children Second Edition

 Jaundice at this stage, accompanied by :

pale grey (acholic) stools
dark urine
an elevated serum bilirubin (largely

conjugated bilirubin)
Biliary atresia (or less commonly a

choledochal cyst) causes an obstructive

jaundice in the first months of life

The Surgical Examination of

Children Second Edition

 Severe hepatic damage associated with cholestasis

may show a similar clinical picture.

Neonatal hepatitis of unknown cause or hepatic

damage secondary to septicaemia, viral infections

(e.g. cytomegalovirus) or inborn errors of
metabolism (e.g. galactosaemia)
Jaundice without obstructive features (i.e. normal

stool and urine colour) may follow less severe

hepatic injury without cholestasis or be due to
impaired bilirubin uptake and conjugation (e.g.
hypothyroidism, haemolysis, breast milk jaundice or
rare congenital enzyme defects)
The Surgical Examination of
Children Second Edition

Assessment of a Patient with

Persistent Neonatal Jaundice
When jaundice persists beyond the second

week after birth, biliary atresia must be

considered
Rhesus incompatibility, other forms of

haemolytic disease of the newborn and

congenital infections of the fetus, such as
rubella, herpes, cytomegalovirus, syphilis and
toxoplasmosis, present with jaundice in the
first day or two of life
In the haemolytic diseases, the Coombs test

is positive.
The Surgical Examination of
Children Second Edition

 The common causes of jaundice between the

first day and the first week are produced by

immature enzymes, so-called physiological
jaundice.
The bilirubin is unconjugated and there are

no anti-red cell antibodies (Coombs test

negative).
In the first week, septicaemia with secondary

hepatic dysfunction may also cause jaundice

and will be confirmed when septic screening
tests show a positive blood culture.
The Surgical Examination of
Children Second Edition

The Surgical Examination of

Children Second Edition

 After the first week, jaundice still may be

caused by a number of congenital disorders

which must be excluded.
Hypothyroidism, or neonatal cretinism, may

be difficult to diagnose clinically and is best

excluded by thyroid function screening tests.
The bilirubin is unconjugated because the
maturation of this function of the hepatocytes is
suppressed in the
absence of thyroxin.

The Surgical Examination of

Children Second Edition

 In galactosaemia, hepatic damage is

produced by abnormal sugars in the blood

stream.
The bilirubin may be unconjugated, but there

are non-glucose sugars in the urine, and the

galactose screen test is positive.
In breast milk jaundice, the bilirubin is

unconjugated because of suppression of

glucuronyl transferase by substances in the
milk.
The Surgical Examination of
Children Second Edition

 In

cystic fibrosis, there may be high

concentrations
of
conjugated
bilirubin
secondary to cholestasis. Other features of
cystic fibrosis, such as delayed passage of
meconium or meconium ileus, may be
present. A positive sweat test is diagnostic.

The Surgical Examination of

Children Second Edition

 Biliary atresia presents with signs of outlet

obstruction of bile and high levels of

conjugated bilirubin in a well baby.
The stools are clay- coloured but not always

white since some bile may enter the stools

through the colonic mucosa.
The urine contains a high level of urobilinogen

which gives it a characteristic dark colour.

The baby is jaundiced and has a slightly

enlarged liver and spleen.

The Surgical Examination of
Children Second Edition

The Surgical Examination of

Children Second Edition

The Surgical Examination of

Children Second Edition

Percussion of the right

chest and upper
abdomen will reveal
the upper and lower
borders of the liver at
their margin with the
air-filled lung
superiorly and the gasfilled bowel inferiorly

The Surgical Examination of

Children Second Edition

Initial percussion of the

left upper quadrant
determines an area of
dullness: the edge of
the spleen is then felt
by commencing
palpation in the right
lower quad- rant and
working towards the
left upper quadrant.

The Surgical Examination of

Children Second Edition

 In the first few weeks after birth, it is difficult to distinguish

biliary atresia from persisting neonatal hepatitis. Jaundice with

minor enlargement of the liver and spleen is present in both, and
the only difference may be the higher level of unconjugated
bilirubin in the hepatitis patient.
Since neonatal hepatitis and biliary atresia may be part of a

spectrum of inflammatory disease of the biliary tract, this

similarity in physical presentation is not surprising. It is essential
that any child with apparent neonatal hepatitis be investigated
urgently to determine whether biliary atresia is present.
Investigations include liver biopsy, along with radionuclide liver

function tests and CT scan. If any doubt persists following

investigation, laparoscopy/laparotomy is indicated since early
diagnosis, before sclerosis of the biliary duct has progressed too
far, makes treatment more likely to be successful.
The Surgical Examination of
Children Second Edition

The Surgical Examination of

Children Second Edition

Portal Hypertension
Cirrhosis is the common sequel to biliary atresia and some

inherited metabolic disorders where liver cells are damaged

by accumulation of toxic substances.
Thrombosis of the portal vein secondary to neonatal

umbilical sepsis may also cause portal hypertension.

High pressure in the portal circulation causes dilatation of

collateral veins which connect with the systemic vessels.

These are particularly apparent in the gastrooesophageaI

region, where oesophageal varices may enlarge; in the

umbilical region, where veins in the falciform ligament
communicate with those of the abdominal wall to produce a
caput Medusae; and to a lesser degree, in the inferior
rectal veins where haemorrhoids may be produced
The Surgical Examination of
Children Second Edition

 The child with full-blown

features of cirrhosis and

secondary portal hypertension.
There is wasting and inhibition

of growth, obvious jaundice

and a distended abdomen.
The spleen is palpable, and the

liver may or may not be

palpable depending on
whether its size has decreased
because of the cirrhosis.
The abdomen is distended

because of
hepatosplenomegaly or
associated ascites
The Surgical Examination of
Children Second Edition

The Surgical Examination of

Children Second Edition

 The increase in intra-abdominal

pressure may be manifested by

an umbilical hernia.
Symptoms or signs of dilated

collateral veins include bloodstained vomitus from bleeding

oesophageal varices, or fresh
blood or melaena in the stool
from the haemorrhoidal veins or
major oesophageal variceal
bleeding.
Dilated veins which radiate from

the umbilicus may be visible on

the abdominal wall, the so-called
caput Medusae. These dilated
superficial veins resemble the
arms of an octopus
The Surgical Examination of
Children Second Edition

 Systemic signs of liver

failure are uncommon in

children with hepatic
cirrhosis, although minor
abnormalities, such as
cutaneous telangiectasia
or spider naevi
Spider naevi usually are

present on the upper

half of the body and
appear as tiny red spots
of vessels radiating from
a central arteriole.
The Surgical Examination of
Children Second Edition

Cholelithiasis
Gallstones are an uncommon problem in childhood,

except in adolescence and/or in association with

diseases which cause chronic haemolysis.
Stones caused by accumulation of bile pigments occur

in haemolytic disorders such as thalassaemia and

spherocytosis.
Therefore, the presentation is usually of a child with a

known haemolytic anaemia who suddenly develops

jaundice and abdominal pain.
General examination of the child and local examination

of the abdomen should indicate a gallstone in the bile

duct and prompt appropriate further investigation.
The Surgical Examination of
Children Second Edition

 Cholelithiasis may occur in late childhood and

is an example of a common abnormality of

adults presenting at an earlier age.
Gallstones in this situation are usually

cholesterol stones, rather than pigment

stones, and are related more to diet and
genetic predisposition.
Epigastric pain in a child more than 10 years

of age should alert the examiner to the

possibility of cholelithiasis
The Surgical Examination of
Children Second Edition

 The area where

maximum
tenderness or a
mass can be felt is
in the angle
between the costal
margin and the
right rectus
abdominis muscle.
This can be
demonstrated with
the child supine or
sitting forwards

The Surgical Examination of

Children Second Edition

The Surgical Examination of

Children Second Edition

Thank You

The Surgical Examination of

Children Second Edition