Neonatal Jaundice

Neonatal Jaundice

jaundice

Jaundice in the newborn is a relatively common presentation, typically seen on postnatal wards and presenting to paediatric units in the first couple of weeks of life.

It is often managed in concert with midwifery teams both in hospital and in the community, and generally doesn’t present to General Practice, though it is an important presentation to remain aware of.

Broadly speaking, neonatal jaundice can be split into two types, with typical neonatal jaundice being predominantly unconjugated.

Types of Jaundice

Briefly, neonatal jaundice can be pathological or physiological.

Physiological

The most common cause of jaundice in the first two weeks of life is physiological. A combination of factors contributes to the development of jaundice in newborn infants:

  • Establishing feeding and breast feeding
  • Supressed liver enzymes
  • Foetal-adult haemoglobin switch
Pathological

In contrast to physiological jaundice, pathological newborn jaundice has a much wider variety of causes and requires casting a wider diagnostic net. Significant causes to be aware of include:

  • Sepsis
  • Haemolytic disease of the newborn
  • Biliary Atresia (a potentially fatal cause of conjugated jaundice in a neonate)

This article will focus primarily on physiological jaundice as a primary condition and the investigation and management thereof. It will cover some elements relating to pathological jaundice, but generally other causes will be covered in their own article.

Approach

The approach to assessment of jaundice in a newborn remains the same initially regardless of cause. A thorough history and examination are key, with a few points to focus on:

  • Age:
    • Jaundice requiring treatment in a baby less than 24 hours old is more than likely to be pathological. This should raise your index of suspicion for sepsis or haemolytic disease. Most guidelines would recommend screening and treating for suspected sepsis in a jaundiced baby less than 24 hours old.
    • Incidence of physiological jaundice tends to peak around day 3-5 of life.
    • Jaundice lasting more than 14 days (or 21 in preterm infants born at <37 weeks gestation) is said to be prolonged jaundice. This necessitates additional investigations.
  • General appearance: does the baby look jaundiced, particularly with blanched skin?
    • This can be difficult to assess in non-Caucasian babies and is a bias that often goes overlooked in areas of Caucasian majority.
    • Scleral icterus (yellowing of the sclera) can be a subtle but important sign.
    • Some babies may appear plethoric rather than jaundiced.
    • Generally speaking, visual assessment of jaundice does not correlate well with laboratory values.
  • Hydration: is the baby well hydrated?
    • In your history, are they having wet and dirty nappies, and how many? A typical infant should be having somewhere in the region of 6-8 wet nappies a day.
    • Also ask about the colour of urine, in conjugated jaundice babies can have pale stools and dark urine which is a concerning feature
    • On examination are their mucous membranes moist? Is their fontanelle normotensive or sunken? Are their eyes sunken and what is their skin turgor like? Are they warm and well perfused?
  • Feeding: a thorough feeding history is really important.
    • Method of feeding: breastfeeding, bottle feeding, mixed feeding. This can help to guide your further assessment. It is very easy to evaluate the feeds of a bottle-fed baby and a little more complicated for babies that are exclusively breast fed.
    • Frequency of feeds: some babies will feed every 2 hours, some will feed every 4 hours, each baby is different. A good ballpark to aim for is 3 hourly feeds.
    • Feeding readiness: does the baby show signs of wanting to feed? Are they waking regularly for feeds or do the parents need to wake them and if so, do they struggle to wake them? A baby that is showing lots of feeding cues is more reassuring than a baby that isn’t waking for feeds.
DayDaily Volume3 hourly Volume
060ml/kg/day7.5 ml/kg/3 hours
190ml/kg/day11.25 ml/kg/3 hours
2120ml/kg/day15 ml/kg/3 hours
3150ml/kg/dayml/kg/3 hours
  • Amounts: is the baby taking enough volume. Generally, a stepwise increase in volumes occurs over the first few days of life, starting at 60ml/kg/day on the day of birth and increasing by 30ml/kg/day up to 150ml/kg/day. Work out what the baby should be having per day and per feed and compare that to their actual intake.
    • Quality of feeds: in breastfed babies, assessing the quality of feeds may be easier than the volume. A good breastfeed can be anywhere from 5 to 30 minutes but generally a baby will need to be awake and actively feeding for the majority of this time. Babies that feed for longer may not be feeding effectively, or there may not be a good enough supply.
  • Stool: are they passing normal stool for their age?
    • Babies should generally pass tar-like black or dark green meconium in the first 24 hours, followed by a shift to yellow seedy stool over the next few days.
    • Pale and chalky stool is a cause for concern as it may indicate biliary atresia and requires further investigation into conjugated jaundice.

Following the initial assessment, the further investigation is reasonably simple:

  • Bilirubin levels:
    • Early measurements should be 4-6 hours after the initiating management and when levels are rising. Once levels are stable and falling, this can stretch to between 6 to 12 hourly. Plot these against a gestation appropriate bilirubin chart. *(It is usually a good idea to have an independent check on any bilirubin plotting.)
    • You need the date and time of birth to accurately plot a bilirubin on a chart. Also be aware that these charts are gestation dependent and there are different treatment thresholds for different gestational ages up to 38 weeks
    • There is a lovely app you can download for your phone call biliapp which allows you to plot bilirubins if you don’t have access to a bilichart on shift.
    • Bilirubin levels not meeting the threshold for treatment but coming close should be repeated in a timely manner. Generally, if the level is within 50umol of the treatment threshold it should be repeated in 18-24 hours depending on risk factors such as exclusive breastfeeding or siblings who had jaundice requiring treatment.
    • Transcutaneous bilirubin measurements may be used in some places. These machines are often calibrated for specific skin types and we would caution against overreliance on TCB levels. TCB should not be used in babies less than 24 hours old or below 35 weeks gestation.
    • Conjugated fraction is typically checked in jaundice that is ongoing at 14 days of life (prolonged jaundice). If the conjugated fraction is raised, this is suggestive of a cause other than physiological jaundice. Prolonged jaundice is covered separately. You should also request a conjugated fraction if there is a history of pale stools or dark urine
  • Full blood count:
    • FBC or CBC will help to assess for anaemia and give a baseline haemoglobin. Haematocrit/PCV can often be raised in newborn babies and this is a cause of jaundice. If the haematocrit is significantly high (e.g. > 0.65) the baby may require blood sugar monitoring and IV fluids but this would need discussing with a paediatrician
    • Raised white cell count may indicate infection.
  • Blood group and Direct Antiglobulin Test/Direct Coombs Test (these are the same): these tests will help to distinguish haemolytic causes of jaundice including ABO and Rhesus incompatibility.
    • Cross checking with Mum’s blood group and antibodies is also important, especially with positive DAT. There are plenty of other antibody systems beyond ABO and Rh that you may come across with varying clinical significance.
  • Additional investigations may include:
    • CRP, blood , urine, and CSF cultures if infection is suspected.
    • Liver profile or even ultrasound if hepatic cause is suspected.
    • G6PD in certain populations if deficiency is suspected.
jaundice chart
NICE neonatal jaundice chart for a term baby

Management is fairly simple too:

  • Phototherapy: the primary treatment for neonatal jaundice in the first two weeks of life.
    • Once started, bilirubin levels should be monitored regularly depending on the severity of the jaundice and trend of the level.
    • In order to stop phototherapy, it is recommended to wait until levels at >50umol/L below the treatment threshold (commonly referred to as “5 boxes below” when looking at treatment monographs – beware, box size may vary!) This is because once treatment is stopped the bilirubin can “rebound” back up.
    • Once phototherapy has been stopped, a rebound bilirubin level should be measured after 12-18 hours, though this may be done earlier or multiple times if there is significant concern.
    • Eye protection and thermoregulation are points to consider during phototherapy as infants need to be well exposed to light. Brief breaks for feeding and care should be encouraged but be mindful that phototherapy only works if the baby is under the light. Some parents may require gentle persuasion to keep their baby under phototherapy lamps in order to ensure that treatment is effective. Bear in mind that infants may be unsettled, some babies don’t like to lie exposed, and the urge for parents to pick them up and cuddle them is normal
    • Sunlight is not an adequate substitute for phototherapy lamps
  • Feeding plan: calculating feeds and giving a good feeding plan is important too.
    • Depending on how old the infant is you can work out 3 hourly volumes and encourage keeping a feeding diary. For babies that are struggling to feed, they may benefit from NG feeds.
    • For breastfeeding babies, they may benefit from top up feeds. (giving formula feeds after a breastfeed to ensure they are getting adequate amounts)

You may notice that at points in the article and on treatment graphs a timeframe of 14 days or the first 2 weeks of life has been given. Outside of this time, phototherapy is generally no longer required for treatment of jaundice in term infants. At this point, investigation and management moves toward prolonged jaundice. Many localities will have prolonged jaundice pathways. See separate information on prolonged jaundice.

Kernicterus

Kernicterus is the reason that we treat jaundice. It is the build up of bilirubin deposits in the brain and can cause lifelong disability and death. If not prevented, there is no current treatment. It occurs due to the immaturity of the blood-brain barrier, allowing bilirubin to cross into the brain when there are high levels in the blood.

Infants with mild physiological jaundice are at low risk of kernicterus, infants with pathological causes such as severe haemolytic disease are at much higher risk. Bilirubin levels rising at >8.5umol/L/hour or bilirubin levels >340umol/L are both features suggestive of a high risk of kernicterus.

In babies that are at a high risk of kernicterus, admission to a neonatal unit may be required to provide intensive phototherapy, intravenous immunoglobulins, and potentially exchange transfusion.

Exchange transfusion: take all the blood out, put new blood back in. This process is generally done manually and is both time consuming and monotonous. Usually done for infants with severe haemolytic disease of the newborn. Carries a variety of risks including transfusion reactions and thrombosis. As well as severe jaundice, other indications for exchange include sickle cell disease as both prevention and treatment of severe consequences such as stroke or acute chest syndrome.

Clinically, kernicterus can be divided into Acute Bilirubin Encephalopathy (ABE) and Chronic Bilirubin Encephalopathy (CBE). In ABE there may be a wide range of presenting encephalopathic symptoms with general neurological abnormalities including dystonia, altered consciousness, and seizures. CBE is characterised by motor dysfunction, hearing loss, and visual disturbances. This is due to the deposition of bilirubin in the basal ganglia and the auditory and oculomotor nuclei of the brainstem. A major clinical manifestation of kernicterus is dystonic cerebral palsy.

References:

Jaundice in newborn babies under 28 days [NICE CG98]

Written by Dr Joseph Wood Paediatric ST2

Edited by Dr Rebecca Evans Paediatric ST3

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