Newborn Presentations

People get worried about newborn babies.  When presented with a baby problem, there are a few basic rules to apply:

Most things that newborns present with are unlikely to have a significant cause.  Some presentations are more concerning that others and while there are no absolutes, it is good to know which things to be more suspicious of.

In many cases, it is possible to significantly change the index of suspicion by knowing the red flags to look out for.

Let's look at a few examples:

Imperfectly shaped head

Since the discovery that putting a baby to sleep on their back dramatically reduces the risk of sudden infant death, there has been a significant rise in the number of babies and infants with asymmetrically shaped heads.

Picture credit: https://commons.wikimedia.org/wiki/File:Plagiocephalie.JPG

The vast majority of babies with this presentation will have plagiocephaly - the result of gravity on a compliant skull.  Plagiocephaly has no apparent risk of harm and requires no intervention.  However, this presentation nicely illustrates the general principles of assessing any presentation in a baby because there is a significant pathology that is rare but significant - craniosynostosis.

So while the head shape is very likely to be nothing to worry about, it is still necessary to look for evidence to support that decision.  The head should be palpated and measured (and plotted), the baby should be given a neurological examination and development should be assessed.

It also follows another rule of newborn presentations: where there is no credible pathology, we should do as much nothing as possible.  We should avoid unnecessary tests and treatments. Remember that the baby is your patient.  Don't do anything to them that does not stand to benefit them directly.

Sticky eyes

While the temptation is to presume that sticky eyes are due to infection, this is rarely the case in newborns.  In most cases the problem is a blocked tear duct.  True eye infections are uncommon and usually quite obvious.  This presentation nicely illustrates the general principles of:

• Assess the problem - Is the conjunctiva red?  Is there periorbital redness and swelling?
• Look at the baby - Do they appear well?
• Decide if there is a significant problem (ophthalmia neonatorum)
• If not, don't do an unnecessary test (swab) or give an unecessary treatment (antibiotic drops)

Excessive crying

Babies cry.  How much is excessive is hugely subjective and open to interpretation.  Although excessive crying in the absence of pathology has been given a name (colic) this scenario demonstrates the principle that we can't always offer a diagnosis.  Calling the problem colic implies that we know what causes the problem and validates an interventional approach.  There is no treatment for excessive crying in the absence of pathology that has a good evidence base.  Sometimes honesty about uncertainty and futility of intervention is the best policy.

Regurgitation of Feeds

Note the use of the word regurgitation.  People often use the word vomiting or even "projectile vomiting" when neither is what is happening.  Babies often bring back some milk after a feed.  This is generally a passive event, as opposed to vomiting, which is what happens when peristalsis works in reverse or against an obstruction such as pyloric stenosis.

By now, you probably know what I'm going to suggest.

• Assess the problem - Is regurgitation the only symptom?  Are there red flag features (see above)?  How long has the problem been occurring?  Has it changed much and if so how rapidly?
• Look at the baby - Do they appear well?  Do they appear well grown?  Plot their growth on a chart.
• Decide if there is a significant problem (e.g. Pyloric Stenosis) or whether they could be in a feed-cry cycle.
• If not, don't do an unecessary test or give an unnecessary treatment.  If you give alginates to every regurgitating baby, you'll double your workload as they come back the next week with constipation and without the original symptom having improved.

Not opening bowels for X number of days

Guess what?  Yes: in most cases, the baby who has not passed a stool for the past few days is usually going through something that is normal in the first few weeks of life.

As with all of the low risk newborn presentations, if the baby looks well, examines normally and is growing and developing normally, they are normal.  If something is significantly wrong, it should manifest itself in the history or examination.  In most cases, we should normalise this presentation rather that give something to treat it.

It can be really difficult to do nothing when faced with a baby and a concerned parent.  However, a careful clinical assessment and evaluation are the most important interventions you can offer.  If you have done that and not come up with something serious, explanation and safety netting are the premium service, not the economy class package.

Umbilical presentations

There are rare and significant pathologies that can affect the umbilicus.  Most of these will present at birth or in a way that alerts the clinician to the fact that something is clearly not right,  The more common presentations and the best approach to each are:

• Umbilical granuloma - leave it alone
• Umbilical hernia - leave it alone
• Umbilical stump being sticky and smelly - leave it alone unless there is evidence of omphalitis

Skin presentations

Lots of things can happen to the skin of a baby in the first few days and weeks.  Most presentations are either normal phenomena (peeling skin), dysfunctional but harmless (erythema toxicum) or problematic but mostly uncomplicated (cradle cap).

Picture credit: Skin Deep - a DTFB project

In each case there are simple questions to be answered

• Peeling skin - Is the baby well?  Is there dermis exposed by the peeling skin (if so then epidermolysis bullosa is a possibile diagnosis)?
• Erythema toxicum - Is the baby well (erythema toxicum is completely harmless)?
• Cradle cap (seborrheaic dermatitis capitis) - Is the baby well?  Does the skin have signs of infection?

Peeling skin and erythema toxicum are best left alone.  Cradle cap can be treated with olive oil in most cases.  Occasionally it can become infected.

Jittery Movements

Sleep myoclunus is a normal phenomenon at all ages.  In babies it can cause people to worry thet their child is having a seizure.  This is partly due to the protective reflexes that they are born with such as the moro reflex.

Here we go again with the standard procedure for a newborn presentation:

• Assess the problem - Are the movemments occuring during or around sleep time?  Is the baby otherwise normal in between episodes?
• Look at the baby - Do they appear well?  Are they developing normally. Measure and plot head circumference.
• Decide if there is a significant problem (e.g. Infantile spasms/ West syndrome)
• If not, don't do an unecessary test or give an unnecessary treatment.  Explain, reassure and give safetynetting advice.

Demedicalising infancy is a good thing to do.  The simple apprach of assess, look and decide will allow you to do that in the majority of cases.  In the rare cases of the discovery of a red flag or atypical presentation, there are always the options of advice or referral.

Edward Snelson
@sailordoctor

Uncomplicated febrile convulsions in children - where and who to diagnose?

This is the first in a series of posts that explore the clinical scenarios that can be managed entirely in a pre-hospital setting but are often or sometimes sent for further assessment.  In each case, I know that some primary care clinicians do fully manage these problems without involving secondary care.  The aim of these posts is to explore the possibility that the condition can be managed without secondary care.

Clinical scenario

A two year old has been managed symptomatically by their parents for what seems to them to be a viral illness.  The child developed a fever this morning which led the parents to give paracetamol.  Shortly afterwards, the child became stiff and then had rhythmic jerking of all four limbs.  This continued for less than a minute.  During the seizure the child was completely unresponsive and went slightly blue.  Their eyes were fixed and staring.

The jerking movements ceased spontaneously and the child was then sleepy for a few minutes, followed by some crying.

In less than an hour, the child is back to their normal self.  They are alert, settled, interactive and mobilising.

If they manage to get to see a primary care clinician, should they be referred to secondary care for further assessment?

While in the majority of such cases parents will present to secondary care, first febrile convulsions can present to General Practice or be seen initially by a paramedic advanced clinical practitioner.  To explore the value of ensuring a secondary care assessment, we need to look at the answers to a few other questions.

What is a febrile convulsion (seizure)?

A febrile convulsion is like an epileptic seizure in every way other than that it is symptomatic of an illness rather than being due to underlying idiopathic epilepsy.  Anyone who doesn't have epilepsy can have a symptomatic seizure (e.g. due to head injury or hypoglycaemia).  Epilepsy is different - the tendency to have seizures without a specific cause.

A febrile convulsion will look the same as an epileptic seizure.  You can presume that a seizure is a febrile convulsion if it fulfils the following criteria:

• Typical age of 1-6 years old
• Child has a febrile illness (the timing of the fever and seizure are unimportant as long as the illness is current)
• Child has no underlying neurological or developmental abnormality
• The seizure is followed by a full return to normal for the child

A febrile convulsion can be atypical, prolonged or focal in which case the possibility of significant pathology is increased and those children should be seen urgently in secondary care.

What causes a febrile convulsion?

The current thinking is that the illness is the thing that somehow causes the seizure.  The idea that the fever itself causes the convulsion was first questioned in an article in ADC in 2003.  Since there is a lack of correlation between the timing of the fever and the seizure, and there is a lack of evidence that antipyretics are preventative, it is likely that the illness causes the seizure and the fever.

Does a febrile seizure indicate serious pathology?

Although medical literature contains list of possible underlying pathology, a true febrile convulsion by definition cannot have an underlying cause.  If a seizure is caused by an underlying CNS infection or other neuropathology, the diagnosis is not a febrile convulsion.  If a child with meningitis has a seizure, the diagnosis is meningitis with seizure.

This is why the return to baseline (i.e. as well as can be expected for an uncomplicated viral illness) is arguably the most important part of the diagnosis.  If, post seizure the child fails to demonstrate their wellness and neurological normality, the seizure may be symptomatic of CNS infection or other abnormality. An atypical, prolonged or focal seizure also changes the index of suspicion greatly.

What tests are needed following a febrile convulsion?

If the diagnosis of febrile convulsion is as above and was a self-limiting generalised tonic-clonic seizure, no tests are required.

So what happens when a child is seen in secondary care following an uncomplicated febrile convulsion?

The history and examination is repeated.  Unless there are specific indications for further tests (clinical suspicion of CNS infection or abnormality) everything else is unnecessary.

Essentially the endpoint in straightforward cases is a history and examination which leads to a diagnosis of febrile convulsion.  So, if the diagnosis is already made, making it again adds absolutely nothing.

Who makes the diagnosis, when and where is more about timing, availability and clinical knowledge/ ability.  If you're the right clinician in the right place at the right time, congratulations.  It's you.

Edward Snelson
@sailordoctor

It’s all about the sequence - Reflex anoxic seizures and breath-holding attacks in children

There are a couple of childhood phenomena that every clinician should know about.  These events are terrifying to parents yet they are safe and not harmful.  When children have a reflex anoxic seizure or a breath holding episode, it will sound alarming to the clinician to whom the child presents.  The extreme nature of the event might suggest a diagnosis of epilepsy or head injury.  It is possible to distinguish these phenomena from their better known counterparts.  The best bit about diagnosing reflex anoxic seizures and breath holding episodes is that no tests are needed – it’s all about the sequence.

Let’s look at two cases:

Case One – Gina

Gina is an 18 month old toddler who has always been fit and well apart from the usual respiratory tract infections that go with the territory of being a young child.  Gina was born by normal delivery, following a healthy pregnancy and was well at birth.

Gina’s parents have attended following an event which occurred this morning.  Gina was playing when her older sister took away her toy.  Gina cried for a minute while her mother negotiated with the older sister to resolve the situation.  Gina’s mother then noticed that Gina’s crying sounded quieter and looked to see that Gina was turning a deep purple colour.  There were no objects that Gina could be choking on.  As her mother went to pick her up, Gina stopped breathing altogether and then went floppy.  While still unresponsive, Gina then started breathing and slowly her colour returned to normal.  As she improved in colour, she became responsive.  Within minutes she seemed completely normal.  Her parents have brought her to find out what happened.

Gina is now looking very cheerful and is exploring the room you are in.  She has a normal cardiorespiratory examination.  Her neurological examination is normal for her age.

What did happen to Gina?

Gina has had a breath-holding attack.  This is a phenomenon in which disordered breathing leads to prolonged expiration and a temporary failure to inhale.  Sometimes these events simply result in a blue episode and then self-resolve.  In some cases, the child may actually stop breathing and collapse.  When this occurs, the normal respiratory drive re-sets and the child recovers as a result.

Case 2 – Tina

Tina is a 2 year old child who is usually fit and well.  Tina has been brought by her parent following an event that has just happened this morning.

Tina was running around and having a lot of fun in her house this morning. She then banged her head on a door handle.  Her mother was there when it happened and there says that Tina started crying immediately.  After just a few seconds of crying Tina, suddenly went pale and collapsed to the floor.  She looked as though she was dead for a few seconds and then she went stiff.  After that she had a few jerking movements and then stopped.  Her colour then improved and she started to make some normal movements.  Tina then slowly returned to being her normal self over about 30 minutes.

When you examine Tina, she is back to normal and trying to climb onto the chair.  She is laughing and interactive.  She has a normal cardiorespiratory examination.  Her neurological examination is normal for her age.

What happened to Tina?

Tina has had a reflex anoxic seizure.  This is another phenomenon seen almost exclusively in young children.  A noxious stimulation (pain or surprise or emotional upset) causes an extreme vagal response.  This leads to hypotension and bradycardia.  Circulation is briefly arrested causing a collapse and the alarming change of colour.  In some cases the episode resolves from this point.  In some cases the sudden loss of cerebral perfusion leads to a seizure, which is usually brief.
Whether a seizure occurs or not, the child will reset and recover.  It is likely that the collapse itself stops the vagal overstimulation.

Breath-holding attacks and reflex anoxic seizures have many features in common with each other.  Both occur in young children.  Both cause colour change, collapse and self-resolve.  Both phenomena are terrifying for a parent to witness.

The way to tell the difference between a breath holding attack, reflex anoxic seizure and other cause of collapse is by listening to the sequence of events.

Telling the difference between a breath-holding attack and a reflex anoxic seizure is not crucial.  The management of the child who has had one is exactly the same.  The most important thing is to tell the difference between these two phenomena and a traumatic or idiopathic seizure.

Following a reflex anoxic seizure (or reflex anoxic spell without seizure) or breath-holding attack, the most important things to do are as follows:

• Examine the child.
• Ensure the child has a normal cardiorespiratory and normal neurological examination.
• Explain the event to the parent.
• Tell the parent that these episodes fix themselves because the child’s breathing and circulation have an automatic restart mechanism that is not affected by the breath holding or reflex anoxic seizure.
• Explain that it is possible that the child may have further episodes.  If this occurs they should allow the child to collapse to a lying position.  Holding the child up delays the return of circulation.

In which case, trust the sequence. It is worth pointing them to a good information source such as the STARS patient information (Reflex anoxic seizures).

If the diagnosis is clear and the examination is normal, there is no need for investigation or follow-up.  Some clinicians will do an ECG but if there is genuine suspicion about an underlying arrythmia, a resting 12 lead ECG is not an adequate test.  If there are suspicious features in the history or examination, a 12 lead ECG should be a stepping stone to further investigations such as a 24 monitor.

In most cases the diagnosis is apparent and the examination is normal.

Edward Snelson
Consequential clinician
@sailordoctor

Disclaimer - If you get a Fibonacci sequence, that's worth a case report.

Paediatric Examination by the Book - (Easter egg- When to measure head circumference in a child and what it means)

You know that book that you had when you were learning how to do a clinical examination?  Even books have a teaching style.  Mine was like an old school maths professor.  It taught me that there is only one correct answer and often only one correct way to get to the answer.  And stop doodling Snelson!

If I had owned a paediatric examination text I imagine it would have been more like a literature teacher.  I'm imagining Robin Williams in Dead Poet's Society.  It would teach me that you're never going to approach the same problem the same way twice, and you will probably even find that the answer changes.  Who would like a chocolate?

Having moved from primary to secondary care environments has been an interesting experience.  I recall my first awakening to the differences between two approaches to examination when many years ago I saw a young person with earache.  I was a newly appointed paediatric trainee, but having recently left General Practice I was well accustomed to such presentations.  For reasons I cannot recall, I discussed the case with a consultant and found myself being asked about the systemic examination.  I then had a genuinely useful discussion about what constitutes a full examination.  I had done a detailed throat, ear and neck examination but had absolutely no idea what this young person's spleen was doing that day.  Discuss...

In paediatrics there is no such thing as a routine examination.  This is for several reasons.  Having a routine only works if the same approach works regardless of age or cooperation of the child.  It doesn't.  I am sometimes asked by medical students whether they should be palpating the trachea or percussing the chest of a child.  The obtuse answer is that you should do these things when they are useful and practical.  Often they are not.  If they are, do it.

In any case, examination is normally tailored to the situation.  If a child presents with a finger injury, I presume that the GMC will let me off if I don't check to see if the child has developed a cardiac complication.  Paediatrics is a speciality that quickly teaches you not to think in terms of routine.  Children may have their own opinions about what is about to happen and you often find yourself asking whether something is worth the battle.

General Practitioners have made an art form of the focused examination.  The way that this works is that by the time an examination takes place, the clinician has heard the history and thought about the possible causes of the symptoms in that patient, given their age and past medical history.  What is examined is made up of a selection of what that clinician needs to support or refute each differential diagnosis.

This way of working is very different to the way that hospital medics tend to approach the same problem.  The default in secondary care tends to be a full systemic examination (if that is a thing) in all cases, injury not withstanding.  Each approach has strengths and weaknesses.




























The best approach is probably a combination of the two.  Having a minimum general examination is of value as is the ability to tailor your examination to the patient and the presentation.  There are some elements of examination which are just not part of most peoples routines.  Whatever is left off the "do it every time" list, you need to know and remember when to do it as part of the focused element of an examination.

Let's look at head circumference as an example of something that is routine for some clinicians in secondary care paediatrics but not for most clinicians in primary care or emergency medicine.

How to measure head circumference

Picture credit: https://ftjmikesouth.wordpress.com/

As long as it is done properly, with the correct equipment, measuring head circumference is easy and a more reliable growth parameter than length in babies.  Like any growth parameter, the recorded value is of little use without context.

Head circumference is usually measured either as part of routine monitoring of growth or as part of the assessment of an infant or toddle who has presented with a problem.  In either case, the interpretation of the measurement needs to be in context of a clinical assessment.  The most important elements of this are feeding history, concerns about growth, developmental assessment and neurological examination.

What constitutes abnormal head circumference?

The Great Ormond Street Guide to head circumference (1) suggests the following criteria as abnormal:

• The child’s head circumference measurement indicates excessive or limited growth. 
• Their head is an abnormal shape or size (eg if the measurement falls outside 99.6th or 0.4th centile on the chart. 
• The head circumference is >2 centile lines above or below their height or length measurement. 

What causes abnormal head circumference?

In many cases, HC outside of the 98th or 2nd centile (or where it is disproportionate) is constitutional.  That is to say that it is genetic but without underlying abnormality.  Just as some people are taller or shorter, some people have bigger or smaller heads.  In these cases, there is no other abnormality (including development) and the measurement usually closely follows a centile line.

When do I need to check a head circumference?

Well, if you work in secondary care paediatrics, you may well find that you are supposed to be checking it on all your patients under a certain age.  If that's not you then these are some of the common indications to check:

• Noticeably large or small head
• Growth problems
• Feeding problems
• Vomiting infants
• Any history of symptoms or event with a possible neurological cause (including BRUE/ALTE)
• Developmental concerns or impairment
• Asymetrical head shape
• Child with congenital abnormality of any kind

Why vomiting infants?  It's rare as anything can be but brain tumours can present in babies as vomiting.  Of course, because it is such rare pathology, it is rarely considered early.  Although it is not going to identify a problem very often, checking HC in these babies is harmless and could help to make a diagnosis earlier.

What about the asymmetrical heads?  Positional plagiocephaly is a benign moulding of the skull which is now very commonly seen in infants.

Picture credit: Gzzz https://commons.wikimedia.org/wiki/File:Plagiocephalie.JPG

There was a sharp rise in the incidence of plagiocephaly after the "Back to Sleep" campaign advised to only allow babies to sleep on their backs until old enough to roll over.  This change had a huge impact on the number of cot deaths but it meant that more babies had flattening of the back of their heads, or an asymmetry caused by a tendency to look to one side.

There has been much debate about plagiocephaly treatment but the majority of experts without conflict of interest agree that this is a benign condition (no neurological effects) which tends to improve, if not always completely resolve, as the infant becomes a toddler.

Benign though it is, positional plagiocephaly is common enough to create a risk that craniosynostosis (plagiocephaly's evil twin) might be missed.  Avoid that pitfall by measuring and monitoring the head circumference.  Also check for a ridged suture and a misshaped or small fontanelle.

After making sure that the shape is not due to craniosynostosis, parents can be advised to

• Give the baby time on their tummy when awake
• Change the position of interesting things around the cot.  Alternatively, place the baby's head at the opposite end of the cot on alternate days.
• Alternate the side the baby is held when feeding and carrying
• Consider using a sling to carry the baby instead of being flat in a pram.

But what about the spleen?  Somehow it just doesn't feature in the assessment of positional deformational plagiocephaly as long as craniosynostosis has been ruled out.  What does the book say?  Well, that is a question worth discussing with your literature teacher.

Edward Snelson
Literally not a teacher
@sailordoctor

Disclaimer: I've just realised- I was better at maths than literature.  Ignore everything I've said.  Clearly I'm in the wrong speciality.

References

1. Head circumference: measuring a child, Great Ormond Street Hospital online, downloaded from https://www.gosh.nhs.uk/health-professionals/clinical-guidelines/head-circumference-measuring-child on 24/7/2018

It's all about the wave - An expert's tip for recognising seizures in children

I was recently picking the brains of a paediatric neurology colleague and discussing how they distinguished epileptic seizures from other events.  I already knew that it wasn't through the black arts of EEG (electroencephalogram) interpretation.  I'm sorry to be the bearer of bad news but EEG has a very poor sensitivity and specificity for epilepsy (1).  By the time an EEG is requested, a diagnosis should already have been made on clinical grounds.  The EEG is then useful as part of deciding how to manage the epilepsy, not to screen for epilepsy.

Why do we front line clinicians need to be able to tell the difference?  There are so many things that children present with that could be seizures but probably are not.  One good example of this is absence seizures.

Absence seizures (no longer called petit mal) account for 10% of epilepsy in children.  The incidence of epilepsy in childhood is not well described but is probably about 1/2000 making absence seizures about 1/20,000.  Staring episodes are very common in children and if these occur frequently, it is understandable that parents or teachers want to know if this is a form of epilepsy.  Not all of these need to be referred.

If a paediatric neurologist makes the diagnosis from the history, then so can any clinician.  All we need is to know what their secret is.  It turns out that they are riding a wave.

The characteristic feature of absence seizure is the abrupt and brief impairment of consciousness with a complete inability to intrude on the episodes.  (2) What this means to the observer is that they will notice a sudden onset and then notice the event suddenly finish.

 Fig 1. If you were to plot the change over time, an inattentive staring episode would be like a sine wave.

Fig 2. The typical abrupt onset and cessation of an absence seizure gives a square wave.

It may just be that simple.

Of course, I am not suggesting that no examination is needed.  There are also other features of absence seizures that may be helpful:

• The episodes occur during any activity and in any environment
• There may be subtle muscle twitches or lip smacking (automations)
• There may be a slight loss of tone leading to slumping of the head or trunk

There are two ways that you can get to see the episodes for yourself.  If they happen frequently then, through the magic of smartphones the parents will be able to capture an episode for you.  There is a better way though, as almost all absence seizures can be provoked through hyperventilation as demonstrated beautifully in this video:

So, I have to give thanks to Tony for telling me about the wave thing.  I find pictures are a great way to help me understand things that I know, when I don't necessarily know them well enough.

Edward Snelson
Wave junky
@sailordoctor

References

1. EEG in the diagnosis, classification, and management of patients with epilepsy, S J M Smith, J Neurol Neurosurg Psychiatry 2005;76:ii2-ii7 doi:10.1136/jnnp.2005.069245
2. Typical absence seizures and their treatment, C P Panayiotopoulos, Arch Dis Child 1999;81:351-355 doi:10.1136/adc.81.4.351