Neonatal Jaundice

Question 1

Why is neonatal jaundice common?

Answer 1

This is very common and most of the time does not require treatment.

There are 4 main reasons for normal physiological neonatal jaundice

1. Neonatal liver is quite immature and doesn’t have much function so less conjugation
2. A high rate of haemolysis due to shorter RBC lifespan.
3. Absence of gut flora impedes elimination of bilirubin
4. Exclusive breastfeeding as feeding difficulties can lead to dehydration with reduced bilirubin elimination

Question 2

How does physiological jaundice usually spread?

Answer 2

Usually it spreads from head to toe as the levels of bilirubin increase.

Question 3

When is jaundice never normal?

Answer 3

Visible jaundice within 24hours of birth is always abnormal

Question 4

What can cause jaundice within 24 hours of birth?

Answer 4

• Congenital infection and sepsis – TORCH (toxoplasmosis, other (Hep B, syphilis and HIV), Rubella, cytomegalovirus and HSV
• Haemolytic disease of the new-born – confirm with +ve direct coomb’s test
• ABO incompatibility (IgG so 50% occurs in first child)
• Red cell abnormality e.g. spherocytosis, G6PD deficiency

Question 5

If jaundice hasn’t faded after 14 days (or 21 if preterm) what causes should be considered?

Answer 5

Unconjugated
• Breastfeeding – reduced supply of milk causes less bilirubin to be secreted to the gut
• Infective – TORCH and sepsis
• Metabolic/Endocrine – Hypothyroidism, Galactosaemia, Alpha 1 anti-trypsin
• Haemolytic anaemia
• Pyloric stenosis (high GI obstruction)
• Genetic – Cystic fibrosis, trisomy 21 and 18

Conjugated
• Structural - Biliary atresia
• Hepatitis

Question 6

How can bilirubin levels be monitored at the bed side in the neonate?

Answer 6

Bilirubin levels can be monitored transcutaneously using a device called a bilirubinometer. This will need recalibrating in non-Caucasians. Cannot be used in neonates under 24 hours.

Question 7

What features would suggest a neonate should be admitted for treatment for jaundice?

Answer 7

Features of encephalopathy
If jaundice occurs within the first 7 days of birth
If gestational age is less than 35 weeks
The neonate is also unwell
Pale stools and dark urine
Prolonged jaundice of more than 21 days if gestational age is <37 weeks or 14 days if more
Transcutaneous monitoring show significantly elevated levels of bilirubin

Question 8

How should neonatal jaundice be investigated if suspected to be non physiological?

Answer 8

FBC, blood film and culture
Blood group
TORCH screen
Direct coomb’s test
Liver function test and Thyroid function tests
Test for G6PD deficiency
Test for levels of conjugated and unconjugated bilirubin if diagnosis uncertain
Check Guthrie results as CF and Congenital hypothyroidism can cause jaundice

Question 9

What complications occur as a result of jaundice?

Answer 9

Kernicterus is the main issue with prolonged neonatal jaundice. This is an acute encephalopathy causes by high levels of bilirubin in the blood. Long term this can cause deafness, reduced IQ and athetoid movements (slow convoluted movements).

Question 10

What are the features of a neonate that may be developing kernicterus?

Answer 10

Lethargy, poor feeding, hypertonicity, opisthotonos (severe whole body hyperextension), shrill cry and chronic encephalopathy.

Question 11

How is management of Jaundice decided?

Answer 11

Management is dependent on comparison of serum bilirubin levels with the NICE graph which indicates whether phototherapy or exchange transfusion is needed. This graph is altered by gestational age. Treatment should continue until the levels are well below the treatment line.

Question 12

What two treatments are used for neonatal jaundice?

Answer 12

Phototherapy
Light energy used to break down bilirubin in the blood. Breast feeding should be kept to a minimum to maximise time under the light. Side effects include – labile temperature, eye damage (protection required), diarrhoea, separation from mother and fluid loss.

Exchange transfusion 
Warmed blood (37degrees) given by umbilical vein with removal from umbilical artery. 

Surgical may be needed for biliary atresia

Question 13

What is biliary atresia?

Answer 13

Biliary atresia involves obliteration or discontinuity within the extrahepatic biliary system, which results in an obstruction in the flow of bile. The pathogenesis of biliary atresia is unclear, however, infectious agents, congenital malformations and retained toxins within the bile are all contributing factors. Occurs more often in girls.

Question 14

What are the different types of biliary atresia?

Answer 14

Type 1: The proximal ducts are patent, however, the common duct is obliterated
Type 2: There is atresia of the cystic duct and cystic structures are found in the porta hepatis
Type 3: There is atresia of the left and right ducts to the level of the porta hepatis, this occurs in >90% of cases of biliary atresia

Question 15

How does biliary atresia typically present?

Answer 15

Otherwise healthy baby becomes jaundiced (or physiological jaundice continues beyond 2 weeks), with dark urine and pale stools and appetite and growth disturbances at around the 3rd week of life. Splenomegaly becomes palpable at 3-4weeks and the liver may become hard and enlarged.

Question 16

How should a baby with suspected biliary atresia be investigated?

Answer 16

Serum bilirubin – total bilirubin may be normal but conjugated will be raised
LFTs and bile acids and aminotransferases – raised
Serum alpha 1 antitrypsin (can cause neonatal cholestasis)
Sweat chloride test (cystic fibrosis can involve biliary tree)
USS of the biliary tree and liver - gall bladder shrunken and triangular cord sign
Percutaneous liver biopsy with intraoperative cholangioscopy.

Question 17

How is biliary atresia managed?

Answer 17

Early surgery is key with creation of distinct tracts and anastomosis – kasai portoenterosomy where part of the small intestine is used to form a conduit. If left too late liver damage may become too severe and a transplant is required.

Likely to need a liver transplant later in life anyway