Author: Meriel Tolhurst-Cleaver / Codes: PAP13 / Published: 14/08/2017
Prolonged jaundice is different from early jaundice, which we discussed in our previous blog, as it is jaundice which persists for 14 days in a baby who was born at term (37 weeks or more gestation), or to 21 days in a pre-term baby born before 37 weeks gestation.
It generally is referred to a paediatric clinic but many of these neonates still rock-up to the ED, so it is good for us to know how to manage them.
Up to 15% of babies have prolonged jaundice, and many will have no underlying pathology. The most common cause of prolonged jaundice is exclusive breastfeeding (breast milk jaundice), and immature neonatal mechanisms and prematurity can also cause benign physiological jaundice after 14 days.
However these are both diagnoses of exclusion and in prolonged jaundice we have to do a bit more digging before we can reassure the parents (and ourselves) that all is well. Most importantly, we have to exclude liver disease and conjugated hyperbilirubinaemia. The most common cause of neonatal liver disease is biliary atresia and the surgical management of this is time critical, i.e. within the first few weeks of life.
Giving some thought to the various causes of prolonged jaundice will allow you to target your history, examination and investigations appropriately. For an exhaustive list, see these papers on unconjugated neonatal hyperbilirubinemia and conjugated.
Where to start
Congratulations on your beautiful little boy/girl/ bundle of joy you must be exhausted, how are you doing?, or similar congratulatory and empathetic phrase is a great way to break the ice with these tired, stressed parents before you launch into the detailed history you need. Then down to the nitty gritty:
- Presenting complaint might sound obvious but is the jaundice the only problem? Are they unwell at all with poor feeding, vomiting, difficulty breathing. Has there been any bleeding or bruising?
- Pregnancy history – any anti-D given? Any maternal drugs? Normal scans?
- Delivery type (thinking about potential for bleeding or bruising), risk factors for sepsis (prolonged rupture of membranes, Group B streptococcus, maternal infection at the time of birth), birth gestation, did they have vitamin K at birth?
- Weight note their birth weight and any recent/current weights (you need to weigh again today if not already done), plot them in the red book and note the centile they are on. Note that most babies have regained their birth weight by day 14
- Feeding – breast, bottle, mixed? If on formula, which, how much and how often?
- Jaundice – when was it noticed, has any treatment already been given?
- Stools and urine – careful questioning about stools, specifically asking parents whether stools are pale or chalky and whether the urine is very dark and stains the nappy (stools in newborns should be green or bright yellow and urine should be straw coloured). Have there been plenty of wet and dirty nappies? Did they pass meconium in the first 24 hours of life?
- Drugs/PMH are they on any medications or have any known medical conditions. Have the Guthrie card (heelprick blood tests) been done on day 5?
- Family history ask about jaundice in the family and, specifically about any blood or liver disorders, cystic fibrosis or metabolic disease.
A set of observations should have been performed at triage, but if not make sure you get them. If the baby appears obviously unwell perform an ABCD assessment, get help and resuscitate as appropriate. Otherwise perform a neonatal top-to-toe examination.
Look for dysmorphic features, jaundice, pallor and signs of bleeding (petechiae or purpura). Does the baby have normal tone, alertness and handling? Note if the baby is floppy or appears encephalopathic (even a newborn baby should have a flat back if held in ventral suspension, they shouldnt droop over your hand, check out this video if you are unsure what is normal tone in a newborn). Assess their hydration status (capillary refill, anterior fontanelle, mucous membranes). Quickly scan the eyes for a present red reflex to exclude cataracts. Examine the chest and pulses for features of congenital heart disease and examine the abdomen for hepatosplenomegaly.
ALWAYS check the nappy to see if you can see any evidence of stool or urine colour for yourself. Let this part of the examination be a reminder for you to ask about stool and urine colour if you havent already.
In prolonged jaundice initial investigations are not too cumbersome. You need to perform a split bilirubin (which should give you a total and a conjugated level), an FBC, a group and Coombs test (also called a Direct antiglobulin test, DAT) and thyroid function tests, all of which can be done from a heelprick blood sample.
You also need to collect a clean-catch urine for culture. Remember to confirm with the parents that the Guthrie screening has been sent at 5 days, and ideally check the result if it is available to you. Before sending the baby home you should see the bilirubin level, FBC, group and Coombs and the urine microscopy. If those are all within normal limits then the baby can go home with a follow-up appointment in clinic to check the thyroid function test and the urine culture.
There isnt any specific management of prolonged neonatal jaundice itself, it doesnt need phototherapy like early jaundice does as the blood brain barrier has matured by around days 10-14 and prevents kernicterus. However, treatment is needed for many of the underlying causes (most urgently the Kasai surgical procedure for biliary atresia) if they are found, and so you essentially need to respond to the results of the investigations you have performed. It goes without saying that if a UTI is found the baby (who is under 3 months of age) would need admitting for IV antibiotics under the General Paediatric team.
The single most important thing to check is the conjugated bilirubin level.
Conjugated hyperbilirubinaemia is defined as a conjugated bilirubin >25micromol/l or if the conjugated fraction is over 20% of the total level. If this is found then a whole heap of tests need to be done, looking for a variety of congenital, liver and metabolic diseases. The common causes are listed below but a full differential diagnosis of conjugated hyperbilirubinaemia and the investigations needed are outlined in this paper. There is also a handy guideline on the management of neonatal conjugated hyperbilirubinaemia from the British Society of Paediatric Gastroenterology, Hepatology and Nutrition that includes an investigation summary sheet to print out and put in the notes. However, these investigation lists are long, and in the ED you realistically want to recognise conjugated hyperbilirubinaemia and refer to the General Paediatric team for admission and further investigation.
An initial prolonged jaundice screen may pop up incidentally. For example, anaemia and low neutrophils are fairly common in neonates and are generally just repeated in a week/few weeks, but its best to consult local guidelines as to when they should be seen again. Some local teams will also repeat another bilirubin in a week or so if the total is over 350, even if it is unconjugated, to ensure it is trending downwards.
Whether the family you are looking after is being admitted or discharged a patient information leaflet is useful to explain what is going on. Most trusts will have one (and Ive included our local one as an example), but the excellent www.yellowalert.org website which has generic leaflets to download on all aspects of neonatal jaundice, including a very useful and comprehensive overview advice leaflet. If you have unfortunately made a diagnosis of conjugated hyperbilirubinaemia, the Childrens Liver Disease website is also an excellent resource for families. It has specific leaflets on biliary atresia and all the other pathologies listed above.