Paediatric Orthopaedic Problems of the Legs Pt 1 – Greater love hath no colleague than to give guidance rather than a guideline



Last month, the paediatric orthopaedic surgeons at Sheffield Children's Hospital sent out a call for the Primary Care community to ask them questions about common presentations. Unsurprisingly many of the questions were regarding things to do with growing legs.  Although there seems to be a guideline for everything, there is none for such problems.  While it would be possible to make such a guideline, referral pathways vary and guidelines do not always apply to every child.

It is a common concern: Are my child's legs normal?  Much of the time, children’s legs seem to be a 'funny shape'.  The vast majority of the time, these legs are normal.  This is the problem with paediatrics though: for everything that is normal or benign, there is an evil twin.  A relatively rare problem that is neither normal nor benign.  These complex problems usually have a considerable overlap with the simple ones and so can be difficult to spot.

To break with convention, we've put together some guidance which should apply across all the common presentations of growing legs.  That way, clinicians have a framework that allows them to make an assessment, rather than a rigid decision tool that tells people what to do and what not to do.  How refreshing!

The first thing to cover, is what is normal in growing legs.  As a general rule, things start to point out, then in and then straighten up as a child grows into their adult body.  

Fig 1. From birth, children's knees will tend to go into varus and then valgus before becoming a normal adult shape.  Bandy legs (also called bow legs) are therefore expected in a child under the age of three and knock knees are considered normal until roughly 8 years old.  Genu valgum may persist into adolescence without any need for intervention.

The other simple rule of thumb is that normal legs are symmetrical, function normally and are not associated with any other abnormality.  This applies to pretty much every scenario - Genu valgum, genu varus, in-toeing, flat feet, hypermobility, "growing pains" and tiptoe walking.

In general terms, the following presentations are normal unless there is reason to think otherwise (see red flags above):
  • Bow legs (genu varus), in a child up to the age of 2 years
  • Knock knees (genu valgum), in a child up to the age of 3 years or up to the age of 10 if resolving
  • In-toeing gait (also called pigeon toe) up to the age of about 9
  • Flat feet on weight bearing
  • Tip toe walking in toddlers
  • Hypermobility

The lovely thing about all of them is that the history and examination required is usually brief.  It is rare to need to refer or investigate.  What's wrong with  this child's legs?  Usually nothing, but more on that in the next GPpaedsTips post.

Many thanks to the paediatric orthopaedic team at Sheffield Children's Hospital for taking the time to answer questions and to turn their expertise into guidance.  At a time when Primary Care Guidelines are often written by Secondary Care clinicians, I find it most refreshing that someone is willing to give their time to share insights and provide general guidance which facilitates rather than dictates management in Primary Care.

Edward Snelson
The Hitchcock of Free Open Access Medical Education
@sailordoctor

Disclaimer:  All of the above is based on a standard number of legs.  For any variation on two legs, discuss with your local orthopaedic surgeon or possibly a vet.


Paediatric Sepsis – the Facts, the Myths, How We Got Here and Where We Need to Go Next

(Note that there is a call for questions about orthopaedic problems at the end - please read and respond)

You may be wondering if you're the only one confused about what's happening with paediatric sepsis.  You're not.  I work in paediatric emergency medicine and I just about get it.  Just.

I am frequently asked questions about what people (in both Primary and Secondary Care) should be doing now with regards to sepsis.  Everything seems to be changing so quickly that it is difficult to keep up.  As intelligent clinicians, we like to understand the reasons for change as well as what changes are occurring.  In the case of sepsis, research evidence is only part of the explanation for the changes that are happening.  That doesn't seem very reasonable but there are valid reasons.  As you're the one having to change your practice, it's probably time that you received a full and honest explanation for why you should do this in the absence of robust evidence.

Sepsis has always been with us.  We might have changed but sepsis has always been the same - a dysfunctional response to infection, manifesting as an exaggerated response to the illness or as organ dysfunction.

Some things have changed in our understanding of sepsis, while other aspects remain frustratingly beyond comprehension.  What we know and what we don't know are key elements to understanding the evolving approach to paediatric sepsis.

What we know:
  • Sepsis carries a high morbidity and mortality
  • Early treatment with antibiotics reduces morbidity and mortality
  • Undertreatment of sepsis increases morbidity and mortality
That's about it for what we know.  It's a short list.

What we don't know:
  • We don't have a good definition of sepsis in children.  The Third International Consensus Definitions for Sepsis and Septic Shock (1) gave a definition of "life-threatening organ dysfunction caused by a dysregulated host response to infection."  This is in many ways spot on but at the same time suitably subjective so as not to be specific when it comes to a clinical decision.  The other commonly used definition of SIRS (systemic inflammatory response syndrome) is very specific (if you have an abnormal temperature and tachycardia, you fulfil the SIRS criteria) and has the opposite problem.  It is very prescriptive and very often wrong.  Hot, tachycardic children are not hard to come by.  A few of these will have sepsis but most will not.
  • We don't have a good way of deciding if a child has sepsis.  Various approaches have been tried.  Decision tools have always been hampered by poor sensitivity and specificity.  No blood test or combination of blood tests has been found to be reliable either in the ruling in or ruling out of sepsis in children.  If there was a reliable method we would all be using it. Telling the difference between a febrile viral child and a child with early sepsis is as much of a challenge now as it was in years past.
Image taken from http://rolobotrambles.com/ with kind permission from Damian Roland.
  • We don't know the impact of the various paediatric decision tools that have been introduced in recent years.  In an attempt to improve the recognition of sepsis, there have been many campaigns, guidelines and decision tools landing in your inbox over the past few years.  Driven by the fact that sepsis is often diagnosed late and that the consequences can be fatal, these initiatives inevitably push for increased diagnosis, using a lower threshold. Increased diagnosis can only be through a lower threshold at the moment, since we have failed to find a way to improve sensitivity and specificity together.  What nobody knows is whether this move to a lower threshold is having a positive or a negative effect.  If for every case that is diagnosed earlier, 1000 have unnecessary treatment (totally made up numbers of course), is the overall effect good or bad?
  • We don't know how many cases of sepsis can be diagnosed earlier.  The main problem of course is that retrospective analysis of cases of paediatric sepsis are inherently flawed.  The outcome is known, therefore all tachycardia becomes evidence of sepsis, despite the fact that other causes are possible.  Most children who present with sepsis will have had one or more contacts with a clinician in the days preceding their diagnosis of sepsis.  In some cases the symptoms will have been due to a viral infection which preceded the sepsis and in other cases the symptoms may have been due to the developing sepsis.  It is rarely possible to be certain which of these was the case.

It is therefore with a large number of uncertainties that we go forward.  There is no doubt that we are getting better at treating sepsis, but recognising it remains an area that we have a serious amount of work to do.

So why have we changed practice in the absence of evidence?  The answer is that, although we are all coming at the problem from a different point of view and with different agendas, we all want to do this better.  With the evidence pointing towards a benefit from early recognition and more aggressive treatment, changes are afoot to try to achieve these outcomes.  We need to alter our perspective and awareness of the problem of sepsis even if we do not yet have the answer to the question of how to better recognise it in children.

How should I change my practice?

Taking Damian Roland's sepsis spectrum and simplifying it, there are three clinical scenarios that I think are generally true by the end of consultation with an ill child.

Scenario 1 - the child with a febrile illness who has demonstrated their wellness to the point that sepsis is extremely unlikely

For these children, we should no longer be talking in terms of them having 'just a virus.'  They have a virus and they have no signs of sepsis, however, they are now in a group that is at risk of developing sepsis.  Worse still, if sepsis should develop, the signs might not be recognised as they will be attributed to the viral illness.

What should now be standard practice is a safety-netting conversation at the close of the consultation which makes clear what the red flags are and when to seek further assessment or advice.  Parents should feel that they are empowered to seek this without fear of being seen as over anxious people.

Scenario 3 - the child who appears to be seriously unwell

If you work outside of a centre which has acute paediatrics, get them there.  If you are the person at the other end, make sure that the child is seen quickly, a decision is made to treat ASAP and that there is no delay to treatment with a full Sepsis 6 bundle as appropriate.  These children might have had multiple cannulation attempts over a long period of time in the past, but now it's time to get out that drill.

Scenario 2 - all the children in between 1 and 3

In between those two set pieces is where we make the big decisions and make them very carefully.  As mentioned above, there is no good decision tool available, no test and no easy answer.  Decision making in these circumstances is complicated (2) and the explanation of how these decisions are made cannot be oversimplified.

What can be simplified are the guiding principle for assessing these children who occupy the uncertain zone.
  • Gut feel is an important part of the decision making process.  This means that you should listen to your gut feel and to the anxieties of the parents and your colleagues.
  • Persistence of abnormal signs is concerning.  While signs (heart rate, level of activity) fluctuate significantly during an uncomplicated viral illness, children with a significant infection tend to be persistently unwell.  Persistent tachycardia, tachypnoea, lethargy or other signs of illness should be taken very seriously.
  • No one factor will give you the answer.  Making these decisions would be a lot easier if there was a solid rule.  Can you send someone home with a pyrexia? Often.  Can you send someone home with tachycardia? Sometimes.  
  • You can only work with the information that you have.  What do I do if it is 2am, the child is asleep and is suitably grumpy when woken?  In this situation, the doctor-patient banter that I rely on to rule out sepsis is not available to me, so I am forced to look at all the other information that I have.   Admittedly, I look at it very carefully before making a decision.  I can only work with what I've got though and I'm not going to keep the child till morning just to tick the "I've seen them smile and run around" box.
  • The sepsis assessment never ends.  If the initial assessment leaves uncertainty, there are several options including referral, observation and discharge, depending on the degree of suspicion.  In all of these cases, we need to be clear about the need to take persistent, worsening or new symptoms seriously. 

Early sepsis is difficult to detect, so we need to use every tool available to us to make sure that the diagnosis is made at the earliest possible opportunity.  Until we have something better, creating that opportunity means as many clinical assessments as needed depending on the scenario.

What has changed over the past few years?  Hopefully we have.  We now better recognise the importance of early recognition of and immediate treatment of sepsis.  What we shouldn't forget is that while we have set rules for ourselves that achieve the latter, we haven't yet found a way to consistently do the former.  So that leaves us with a need to do what we can to make the best clinical decision possible for each of our patients, while being patient with the academics who will hopefully one day come up with a magic test.

Edward Snelson
Medical Historian or Historical Medic?
@sailordoctor

Disclaimer - very little research into the actual history of sepsis went into this article.  By little, I may mean none.  Still, the leeches bit is probably true.

The paediatric orthopaedic surgeons from Sheffield Children's Hospital would like to invite questions about children's orthopaedic problems from the Primary Care community. We will be publishing the questions and answers in a GPpaedsTips post in the near future.  If you have anything that has always confused you; if you have a niggling doubt about the need to refer a problem; if you want to know what they do with a certain condition, send me your questions.  You can do this by posting as a comment below, via a twitter message to me or via Facebook.
Please note that this Q&A session is for clinicians only and the only questions that will be entertained are generic questions. In other words, something like "Can the orthopaedic team help a child with Osgood Schlatters or should we just manage them in General Practice?" is perfectly acceptable. "My child has zika virus in their left wrist and I wanted to know..." is not.

References
  1. The Third International Consensus Definitions for Sepsis and Septic Shock (Sepsis-3), JAMA, February 23, 2016
  2. Roland D, Snelson E ‘So why didn’t you think this baby was ill?’ Decision-making in acute paediatrics Archives of Disease in Childhood - Education and Practice Published Online First: 01 March 2018. doi: 10.1136/archdischild-2017-313199




Adding my uncertainty to your uncertainty – how to decide when there are conflicting signs

It’s always nice when a clinical assessment ends with a clear way forward.  If a child presents in the community with a severe exacerbation of asthma, there are clear guidelines for pre-hospital treatment and referral.  There is usually no dilemma in what to do if a child presents with fever and signs of meningitis to Primary Care.  Similarly, it is usually obvious what to do with a child brought with signs of an uncomplicated viral upper respiratory tract infection.

What is more challenging is when a child has conflicting or paradoxical signs and symptoms.  This is especially true when all the evidence points towards an illness that can and should be managed in Primary Care, yet one small piece of information is inconsistent.  What should you do with that?

Let me give an example:
A four year old boy presents with a history of diarrhoea and vomiting for 24 hours.  It is now 6pm.  The history of the illness is typical of a straightforward viral gastroenteritis.  The child looks well and is alert, interactive and surprisingly willing to mobilise when he spots the toys available.  He has wet mucous membranes and has no signs of dehydration.  Abdominal examination is normal as is the rest of his systemic exam.  All physiological observations are normal.  There is an unexpected snag: the parent with him reports that he has not passed urine since he went to sleep the previous night. 
So, with all but one piece of information in favour of a pathway that involves symptomatic management and oral hydration, what should one do?

Should this child be referred to Secondary Care?  I think you will get different answers from different people for this kind of situation.  On one hand the reported lack of urine output is a clear red flag.  On the other hand it is completely at odds with the wellness and clinical hydration of the child.  The child could be referred, but would that change anything?

There are many reasons for referring a child to Secondary Care.  I the initial examples, the reason is for undisputable and necessary management.  If the child is referred in the case of the paradoxical urine output, there may be an assumption that someone in the hospital will be better able to make a decision than the person making the referral.  This may or may not be true.  If it is true, it is probably not because they will have further information that could not have been obtained by the referrer.  It is probably not because the person receiving the referral has some sort of child assessment super power.  They may not even have more confidence in their gut feeling (that the child is fine and can safely be managed as an uncomplicated gastroenteritis) than the referrer.  What they will mostly have is their uncertainty to add to the referrer’s uncertainty.

Let’s say that you do refer this child.  Let’s assume that I see them shortly after you have seen them and that nothing has changed apart from the child having a bit more to drink in the meantime.  I now have the same information that you had and the same dilemma: to go one way or another.  I can do blood tests and consider intravenous fluids, but my gut tells me that this is an unnecessary torture of a child best managed with analgesia and oral fluids.  I could give symptomatic treatment advice and safety-netting advice and send them home but that is difficult to justify in the face of the reported anuria.  I could keep them for observation, but what would be the end point of that or the purpose.  If I am confident enough to do no active management in hospital, why am I keeping them?  Observing moderately unwell children while awaiting an expected move towards wellness is one thing but observing a well hydrated and clinically well child to prove that they are going to pass urine seems to have little value over allowing the same outcome at home.

When faced with conflicting information, it is important to have a way of deciding which information carries more weight and has more validity.  In ill children, it is often useful to categorise according to effort and efficacy. 

Effort is the body’s way of responding to illness.  There is usually some sort of physiological response to illness which is often a way of compensation for an effect of the illness.  Efficacy is everything to do with the end point of how effective the increased effort is.  In other words effort tells you that the child is trying to cope with an illness and efficacy tells you if it is working.  A good example is the happy wheezer.  If an infant with bronchiolitis has an increased respiratory rate, that tells you that there is some effort to compensate for what is happening in the chest.  The smile tells you that this is being effective.

Inevitably, efficacy is more important than effort when it comes to clinical decision making.  It’s not that the compensatory efforts should be ignored, it’s that the effect of that effort is of greater importance. 

So in this case, I would say that whatever is happening with this gastroenteritis, all the evidence that I can see tells me that it is having the desired effect.  The child is well and has no signs of dehydration.  Even if we were to believe the lack of urine output (and to disbelieve is not to disbelieve the parent) then a reduced urine output would be part of an effort to appropriately retain fluid during an illness.  I’ll say that is being quite effective in this case.

Do I have any niggling doubts?  I am certainly not dismissive of the contradictory report of anuria.  I am aware of my own fallibility and would only chose to ignore the lack of history of good urine output if I felt that to do so was in the child’s best interests.  In short, I would love complete certainty but I’m not being given that so I can either make a decision or find another way.  What I don’t want to do is a test or treatment that I don’t believe in.

But it isn’t strictly true that I’m no further forward than the referring clinician.  After all, I’ve got their uncertainty to add to my own.  If I’m lucky, it will be clear from the referral that when you referred this child for further assessment, you were very happy with the hydration and general wellness of the child.  You, like I am now, were perplexed more than concerned by the lack of history of passage of urine.

I have no super-powers and no particular clinical skills that you don’t have.  I am also lacking a 100% certainty about this case, but it’s time to make a decision.  Adding my certainty to your certainty means that between us we are 198% certain that there is nothing about this child requiring a blood test or a night on an intravenous infusion.  Let’s go with that then shall we?
There’s nothing wrong with referring a child for a further opinion when there is some uncertainty about the best management.  It is particularly helpful to know what the opinion is along with everything else in the referral.  That way I know that my uncertainty is the same, which in itself is useful information.

There’s also nothing wrong with trusting your assessment, in the knowledge that in secondary care we may well have little to add other than another appraisal of the same paradox.  Of course there is always the phone call to discuss the uncertainty.  How much fun would that be?

Edward Snelson
Medical astrologist
@sailordoctor

Disclaimer: I'm not even certain what I'm disclaiming.