Jaundice

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)


Jaundice that appears later than, or persists into, the third week of life is not likely to be benign ‘physiological’ jaundice


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


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)


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 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.



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.


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.


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.


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.




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 gas- filled bowel inferiorly


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 quadrant and working towards the left upper quadrant.


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.



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 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 increase in intra-abdominal pressure may be manifested by an umbilical hernia.

Symptoms or signs of dilated collateral veins include blood- stained 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


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.


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.


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 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



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Jaundice

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