Fracture hide and seek

Cite this article as:
Carl van Heyningen and Katie Keaney. Fracture hide and seek, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.32819

Another winters morning. You are freshly vaccinated, caffeinated and ready for another ED shift. Your first patient is a return visit. A 7 year-old who fell onto his shoulder at school a week ago. You read your colleague’s previous assessment. On examination there was no bony tenderness and the x-ray report of the right clavicle was normal. Yet today there’s a lump over the collar bone and he’s no longer using his arm normally. Has something been missed?

X-ray interpretation is a complex human enterprise vulnerable to a wide variety of errors. The extent of missed diagnoses has been estimated to be as high as 15-20% 1,2.

There are two principle types of error:

  • Perceptual errors – those where the abnormality is simply not seen
  • Cognitive errors – where the abnormality is seen but its significance is not appreciated

You might think that such errors can simply be avoided through education, better imaging techniques and training. Yet since the 1960’s, despite doubtless advances in technology and improvements in medical practice, the rate of radiological errors has remained almost unchanged.

So what do we do? Admit defeat? Never!

Instead, let’s journey inwards and analyse these errors, why we make them and how we can improve ourselves and our approach to avoid missing fractures in children with injuries.

Causes of error

Perceptual errors are the most common and are due to many factors including:

  • Clinician fatigue
  • Distractions from colleagues and the working environment – the extrinsic cognitive load
  • High workload
  • Satisfaction of search (spotting one abnormality then failing to look for any more)

There is a reason your friendly radiologist is sat quietly in a dark room with a cup of coffee – a world away from a noisy, busy accident and emergency department. Consider yourself and your environment when reviewing an x-ray. Just as with prescribing, respect reviewing x-rays.

Even with the best conditions, what the eye sees the brain doesn’t always spot. Consider the now infamous Invisible Gorilla experiment that earned Christopher Chabris and Dan Simons an Ig-Nobel Prize in 2004. Participants were asked to watch a video and count the number of times the ball was passed between players. What they failed to notice was the large hairy simian playing the game. The brain failed to recognise what the eyes clearly saw.

The selective attention test

Cognitive errors occur for a whole host of reasons. Some of these include:

  • Lack of knowledge (e.g. how to interpret x-ray findings, ossification centres, etc.).
  • Lack of clinical information (e.g. history or examination)
  • Faulty reasoning (e.g. fracture identified but not cause of pain)
    • True positive, misclassified
  • Complacency (e.g. fracture identified but from separate injury)
    • False positive finding
  • Satisfaction of report (e.g. reliance on radiology report discourages further analysis).
  • Satisfaction of search (e.g. finding one fracture discourages search for another).  

Then there are our own cognitive biases which may also influence our interpretation…

Anchoring bias– early focusing on one feature of the image so neglecting or misinterpreting the rest of the information

I’ve found the distal radius fracture so that is the diagnosis”. The scaphoid fracture is then missed).

Availability bias– recent experience of a diagnosis/presentation makes you more likely to diagnose the same condition

I saw a pulled elbow the other day, it looks the same”. May miss ulnar dislocation.

Confirmation bias– looking for evidence to support your hypothesis and ignoring evidence against

It looks like a simple ankle sprain, I think that X-ray must be fine”. Can miss fractured fibula.

Outcome bias– opting for the diagnosis associated with the best patient outcome/prognosis

If there is a vertebral fracture, we will have to immobilise this child. It probably isn’t that”.

Zebra retreat– history and findings are in keeping with a rare diagnosis but the diagnostician is afraid to confirm this

As Dr Cox says, if you hear hoofbeats look for horses not zebras” …sometimes it’s a zebra!

Finally, no article on medical error would be complete without reference to the good old Swiss Cheese Model. We are but one step in a sequence of events that can either prevent or lead to error. For our example case, consider the following…

Graphic showing swiss cheese model of errors
Errors were made

Can I have some examples please?

Most fractures in children are easy to spot however some may present with subtle findings, especially when they involve the epiphyseal growth plate.

Examples of where most missed fractures occur are shown below:

Common but low risk as well as rare but high risk missed fractures

Many fracture patterns are unique to children. The paediatric skeleton is more elastic, more porous, and has a relatively stronger periosteum. That makes it uniquely vulnerable to torus fractures, buckle fractures, plastic bowing and greenstick fractures. Knowing to look for such subtleties sets paediatric fracture diagnosis apart. That coupled with odd growth plates and ossification centres explains, in part, why fractures are more easily missed in children5.

There is a subtle angled fracture of the distal radius. Compare this with the normal (middle) and healing (right) – taken from Hernandez, J.A., Swischuk, L.E., Yngve, D.A. et al. The angled buckle fracture in pediatrics: a frequently missed fracture. Emergency Radiology 10, 71–75 (2003). 

A subtle angulated fracture of the proximal radius taken from Hernandez, J.A., Swischuk, L.E., Yngve, D.A. et al. The angled buckle fracture in pediatrics: a frequently missed fracture. Emergency Radiology 10, 71–75 (2003). 

Plastic bowing deformity of the left radius and ulna taken from George MP, Bixby S. Frequently Missed Fractures in Pediatric Trauma A Pictorial Review of Plain Film Radiography Radiol Clin North Am 2019 Jul57(4)843-855

Plastic deformity of the radius with upward bowing (arrows) taken from Swischuk, L.E., Hernandez, J.A. Frequently missed fractures in children (value of comparative views). Emerg Radiol 11, 22–28 (2004). 

A subtle greenstick fracture of the distal ulna taken from George MP, Bixby S. Frequently Missed Fractures in Pediatric Trauma A Pictorial Review of Plain Film Radiography Radiol Clin North Am 2019 Jul57(4)843-855

Note the upward plastic deformity of the right clavicle with the left for comparison taken from Swischuk, L.E., Hernandez, J.A. Frequently missed fractures in children (value of comparative views). Emerg Radiol 11, 22–28 (2004). 

The leftmost image shows an obvious spiral fracture. The Toddler’s fracture in the middle image is not apparent until the line of sclerosis appears with healing taken from Swischuk, L.E., Hernandez, J.A. Frequently missed fractures in children (value of comparative views). Emerg Radiol 11, 22–28 (2004). 

A Salter-Harris 1 fracture of the distal radius. Look at the widened growth plate compared with the ulna taken from Jadhav, S.P., Swischuk, L.E. Commonly missed subtle skeletal injuries in children: a pictorial review. Emerg Radiol 15, 391–398 (2008). 

We have seen how even with the benefit of the patient in front of us and the luxury of radiology reports that we are vulnerable to making mistakes. Yes, we need to first know our ischial spine from our olecranon (our arse from our elbow), but we also need to train ourselves in techniques to avoid perceptual and cognitive traps.

So how do we prevent them?

Reducing missed fractures in children

Sadly the evidence is lacking and largely focuses on the performance of radiologists. Approaches centred solely on education and training are insufficient. Slowing down strategies, group decision-making and feedback systems are, as yet, an unproven step in the right direction. Checklists, however, have a growing evidence base in improving performance despite their poor popularity.

Whether or not you are a fan of the ‘Checklist Manifesto’, less controversial are principles around workplace culture and communication. Facing up to errors, avoiding blame and frequently just talking with colleagues (the clinician, the radiographer, the radiologist, the patient) remains incredibly important.

What else?

Systems-level thinking

A growing number of healthcare trusts now implement peer learning systems. Rather than being punitive, such groups create collective opportunities to teach using diagnostic catches as well as misses. At Leicester Royal Infirmary, Education Fellow Sarah Edwards set up one such weekly group teaching session for A&E staff. It gave them the opportunity to review images with the support of a Consultant Radiologist.

Evidence also supports “double-reading” to reduce the misses. At the Royal London Hospital, we are supported by our Radiology colleagues who review all images from our paediatric emergency department within 24 hours. Furthermore, within our ED we foster a culture of learning from each other through openly sharing learning points without risk of embarrassment and most (if not all) x-rays are reviewed by two or more clinicians to share knowledge and experience.

Such principles underpin the Irish National Radiology Quality Improvement (QI) programme. Through standard setting and measuring performance they pursue a cycle of continued quality improvement.

Individual level thinking

Michael Bruno, Vice Chair for Quality and Chief of Emergency Radiology at Penn State University says “there’s a very simple fix for errors of thinking- cognitive biases.… you must force yourself to ask really open-ended questions…. what else, how else, where else could a finding be… force your mind back open again.

To be more technical, lets consider the “dual process theory of reasoning.” In radiology, automatic system 1 processes typically enable immediate pattern recognition. In contrast deliberate system 2 reasoning enables less obvious abnormalities to be detected. Normally there is a dynamic oscillation between these to forms of thinking. The lesson is not to eliminate type 1 processing, which is prone to mental shortcuts and mistakes, but instead to be aware of our own thinking with the ability to deliberately “turn on” our type 2 brain when needed.

This discipline is termed metacognition or meta-awareness. 

For those who find such talk nebulous, there a number of practical steps that come recommended from Andrew J. Degnan (Department of Radiology at Children’s Hospital of Philadelphia).

Maintain a healthy skepticism

Reflect on your diagnostic process, challenge your interpretation forensically and question yourself objectively.

Use a structure or checklist

Structured reports help radiologists. Find your own repeatable techniques and approach each x-ray systematically, including “review areas” that are often overlooked. 

Consider the clinical findings

What is your pre-test (pre-x-ray) probability? How confident were you in your clinical assessment? Is the x-ray a rule-in or rule-out? Marrying up a thorough history and examination with a careful focus on the relevant radiographic area often bears reward.

Injuries that are missed because of failure to image are typically because the injury was poorly localized or because of the presence of other injuries distracted attention from the injured part.”

Mind your environment

Are you fatigued? Have you had a break? Clearing your mind for even a moment can actually improve overall efficiency. A quiet work space. A few minutes away from distraction. These will all empower your type 2 thinking.

Mitigate, mitigate, mitigate

Mistakes happen. Telling parents about uncertainty is critical to them re-presenting if their child’s soft tissue injury or sprain is not improving. Importantly, this is not the same as forgoing responsibility. Yet if your routine practice includes quality safety netting, discussing cases with your friendly radiologist and chasing up on cases you may not prevent mistakes but you might minimize the harm that comes from them.

What happened with our case?

A repeat x-ray was done but again no fracture was evident. Yet to examine there was an un-deniable lump mid-clavicle. In view of persistent pain and continued non-use of the limb (right arm) the child was discussed with the radiologist who agreed upon ultrasound. Ultrasound confirmed early callus formation and a break in the cortex that was not visible on X-ray. The child went home in a sling for outpatient follow up.

Take home messages  

  • Missed fractures are more common in children and not necessarily subtle
  • Know what to look for and how to look for it
  • Process is important, don’t forget history and examination
  • Communicate clearly, speak frequently with your radiographer and radiologist

Selected references

1. Berner ES, Graber ML. Overconfidence as a cause of diagnostic error in medicine. Am J Med 2008;121(5 suppl):S2–S23.

George MP, Bixby S. Frequently Missed Fractures in Pediatric Trauma A Pictorial Review of Plain Film Radiography Radiol Clin North Am 2019 Jul57(4)843-855. – Images 3,5 in carousel

Hernandez, J.A., Swischuk, L.E., Yngve, D.A. et al. The angled buckle fracture in pediatrics: a frequently missed fracture. Emergency Radiology 10, 71–75 (2003) – Images 1,2 in carousel

Jadhav, S.P., Swischuk, L.E. Commonly missed subtle skeletal injuries in children: a pictorial review. Emerg Radiol 15, 391–398 (2008). – Image 8 in carousel

2. Wachter RM. Why diagnostic errors don’t get any respect: and what can be done about them. Health Aff (Millwood) 2010;29(9):1605–1610.

5. Smith J, Tse S, Barrowman N, Bilbao A, (2016). P123: Missed fractures on radiographs in a paediatric emergency department, CJEM, 18 (S1), S119-S119

Swischuk, L.E., Hernandez, J.A. Frequently missed fractures in children (value of comparative views). Emerg Radiol 11, 22–28 (2004). Images 4,6,7 in carousel

Further reading

Brady AP. Error and discrepancy in radiology: inevitable or avoidable?. Insights Imaging. 2017;8(1):171-182. 

Kim YW, Mansfield LT. Fool me twice: delayed diagnoses in radiology with emphasis on perpetuated errors. AJR Am J Roentgenol 2014;202(3):465–470.

Michael A. Bruno, Eric A. Walker, and Hani H. Abujudeh, Understanding and Confronting Our Mistakes: The Epidemiology of Error in Radiology and Strategies for Error Reduction, RadioGraphics 2015 35:6, 1668-1676 

Martino F., Barbuti D., Martino G., Cirillo M. (2012) Missed Fractures in Children. In: Romano L., Pinto A. (eds) Errors in Radiology. Springer, Milano.

Miele V., Galluzzo M., Trinci M. (2012) Missed Fractures in the Emergency Department. In: Romano L., Pinto A. (eds) Errors in Radiology. Springer, Milano.

Wang CC, Linden KL, Otero HJ. Sonographic Evaluation of Fractures in Children. Journal of Diagnostic Medical Sonography. 2017;33(3):200-207.

Challenges in cannulation

Cite this article as:
Vicki Currie. Challenges in cannulation, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.33103

A look at paediatric cannulation. The good, the bad and the seemingly impossible.

We have all been there – coming onto a busy shift and a child who is well known for having ‘difficult’ vascular access needs a cannula.

It can be a heart sink moment when you realise that the team from the previous shift have already tried and failed. You feel your palms begin to sweat as the nursing staff tell you that access was a huge problem on the last admission. The father of the child tells you that you can have ‘just one go’.

But what really affects the chances of success of getting that tricky cannula in? Are there any modifiable factors that make it easier or harder? And how can we feel more confident in paediatric cannulation?

What is the evidence?

There are several factors that have been shown in the literature to negatively impact the success rates of paediatric cannulation:

  • Use of previous central venous access
  • Obesity
  • Attempts in the hand and lower legs
  • Non-black / non-white race
  • Poor cooperation of the child
  • Lack of confidence prior to the procedure

A recent study by Maduemem et al looked at the ‘Challenges Faced by Non- Consultant Hospital Doctors (NCHDs) in Paediatric Peripheral Intravenous Cannulation in Ireland.’ It aimed to evaluate the level of confidence of NCHD’s and looked to identify the factors that positively or negatively impacted confidence. This is a unique piece of research that is one of the first qualitative studies looking at the level of confidence in doctors in peripheral intravenous cannulation (PIVC).

Maduemem, K., Umana, E., Adedokun, C. et al. Challenges Faced by Non-consultant Hospital Doctors in Paediatric Peripheral Intravenous Cannulation in Ireland. SN Compr. Clin. Med. 2021

The team performed a cross-sectional national survey in 12 hospitals in Ireland using paper-based questionnaires. The survey captured data on the respondents’ clinical demographics (primary speciality, number of years postgraduate experience), clinical experience with PIVC (any paediatric clinical experience, number of children cannulated in preceding three months etc), the level of confidence in paediatric PIVC and potential factors influencing confidence in PIVC.

The primary outcome was the level of confidence in cannulation, measured by a five-point Likert scale assessing the overall level of confidence with ‘agree and strongly agree’ determined as a good level of confidence. Secondary outcomes were self-rated success in PIVC, previous experience and the effect of parental presence during the procedure.

The study had 202 respondents (45% response rate). The median number of years postgraduate experience for SHO level was three years (IQR 2-4) and at registrar level seven years (IQR 5-10.5). Interestingly ALL respondents had carried out paediatric cannulation in the preceding three months with 76% performing the procedure at least 10 times during the three-month time frame.  Despite 89% of respondents rating their performance as at least average, less than half (48%) of respondents reported themselves as feeling confident with the procedure.

Only 29% of respondents were reported as feeling confident in attempting PIVC that had been unsuccessful by a colleague. 37% of the cohort felt anxious when asked to perform PIVC in children, unsurprisingly with NCHD’s below registrar level feeling more anxious than their registrar counterparts.

What was driving this anxiety? More than half of the respondents (56%) stated that nursing staff and parental presence were sources of anxiety with 52% preferring to carry out this procedure without parents present.

Specific phrases that were noted by participants to have an adverse effect on confidence before the procedure were phrases which I’m sure the majority of us have heard before:

So what can we do?

Practice, practice and more practice

The study found that levels of confidence increased with seniority so encouraging junior colleagues and supporting them to perform cannulation is key. Including sessions on simulated patient arms to practice venepuncture may be a useful adjunct for clinicians with limited previous exposure.

Think before we speak

The phrases we use prior to performing a procedure can be powerful – not just the ones we say to ourselves but those we utter to colleagues. Feeding back to colleagues that phrases were unhelpful or signposting to the above study, in a polite way, might be a good way to raise awareness of the impact such phrases can have.

We all have seen the effect a ‘fresh set of eyes’ can have on that difficult cannula. So, if you are the person attempting after a colleague has already had a go, then be confident and try to start from fresh.

What about ultrasound?

Ultrasound guidance as an adjunct to PIVC has been shown to increase the success of the first attempt with good training in the use of ultrasound a big factor in first attempt success.

This is not a mandatory or even optional skill in general paediatric training in the UK. Experience is often gained from placements in PICU, ED or time with anaesthetic colleagues. Courses are becoming more frequent . If you have the opportunity or access to learn this skill from a colleague (paediatric or adult trained) it can be extremely useful.

Vein finders (infra-red lights that magically show veins through the skin) and the cold light that can often be found on the neonatal unit (used to look for evidence of pneumothorax) can be useful adjuncts too.

Are there any scores that can predict if a child’s access is going to be difficult?

The Difficult Intravenous Access (DIVA) prediction score is based upon four variables that are proportionally weighted. The variables are: vein palpability, vein visibility, age (infants score higher) and a history of prematurity. A score > 4 equates to a 50% increase in the likelihood of failure rate with first attempt.

But if a child has a high score, what next? Some difficult access pathways have been proposed with the utilisation of ultrasound, early contact with anaesthetic colleagues to help with access and consideration of midline/ PICC/ CVC in children who are particularly difficult. In practice, highlighting children early who have factors that put them at higher risk of being difficult and early escalation to senior colleagues, limiting attempts and utilisation of some of the steps mentioned can be helpful.

Keep things calm and pain free…

Optimisation of the position of the child and parents can help to not just keep the environment a calmer place but can reduce anxieties all round. The classic ‘bear hug’ position with a parent on a chair and the child chest to chest can provide not only comfort but easy access to limbs.

The use of freeze spray or anaesthetic creams on the area you are going to attempt cannulation can help to reduce pain as well as child and parental anxiety.

The use of distraction techniques can also reduce the child’s perception of pain. Singing, a YouTube video, home video on a smartphone or even bubbles can be easily done whilst attempting cannulation.

And if despite all of this you are still unsuccessful then limit yourself to a maximum number of attempts – usual practice is two to three (two attempts usually for more junior colleagues) before you ask for additional help. This ensures that there are still some veins left for that fresh set of eyes to have a look at. It also gives the child, parent and other staff helping a break from the procedure and means you don’t become super task-focused. In a situation where the child is unwell and access just needs to be attained, this is a different matter, and you will hopefully have multiple people around with lots of sets of eyes.

PIVC in children is tough, it is a skill that takes years to get right and still people who have been doing it for years can have a bad day where they just cannot get that cannula in. Keep practising, keep smiling, think about the words you use in relation to the procedure and how they can affect others and don’t forget the bubbles!

References

Bauman M, Braude D, Crandall C. Ultrasound-guidance vs. standard technique in difficult vascular access patients by ED technicians. Am J Emerg Med. 2009;27(2):135–40.

de Negri DC, Avelar AFM, Andreoni S, et al. Predisposing factors for peripheral intravenous puncture failure in children. Rev Latam Enfermagem. 2012;20(6):1072–80.

Larsen P, Eldridge D, Brinkley J, Newton D, Goff D, Hartzog T, et al. Pediatric peripheral intravenous access: does nursing experience and competence really make a difference? J Infus Nurs. 2010;33(4):226–35.

Maduemem, K., Umana, E., Adedokun, C. et al. Challenges Faced by Non-consultant Hospital Doctors in Paediatric Peripheral Intravenous Cannulation in Ireland. SN Compr. Clin. Med. 2021. https://doi.org/10.1007/s42399-021-00881-9

Nafiu OO, Burke C, Cowan A, et al. Comparing peripheral venous access between obese and normal weight children. Pediatr Anaesthesia. 2010;20:172–6.

Petroski A, Frisch A, Joseph N, Carlson JN. Predictors of difficult pediatric intravenous access in a community emergency department. J Vasc Access. 2015;16(6):521–6.

Sou V, McManus C, Mifflin N, Frost SA, Ale J, Alexandrou E. A clinical pathway for the management of difficult venous access. BMC Nurs. 2017 Nov 17;16:64. doi: 10.1186/s12912-017-0261-z.

Vinograd AM, Chen AE, Woodford AL, Fesnak S, Gaines S, Elci OU, et al. Ultrasonographic guidance to improve first-attempt success in children with predicted difficult intravenous access in the emergency department: a randomized controlled trial. Ann Emerg Med. 2019;74(1):19–27.

Yen K, Riegert A, Gorelick MH. Derivation of the DIVA score: a clinical prediction rule for the identification of children with difficult intravenous access. Pediatr Emerg Care. 2008 Mar;24(3):143-7. doi: 10.1097/PEC.0b013e3181666f32.

Urine dipsticks

Cite this article as:
Laura Riddick. Urine dipsticks, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.32596

This post will cover what’s what on a urine dipstick and clarify what it means when “it lights up like a Christmas tree

It’s 3 am and the 4-year-old with fever has finally produced a urine sample. You dip it and it lights up “positive for everything”. You’re sure it’s positive for infection, but what if the pH is 5.5? What does it mean that there is blood and protein in it?

Leucocytes

Surely white blood cells must mean an infection is present? If you have read the NICE UTI guidelines, so you know that is not necessarily the case.

The dipstick tests for leucocyte esterase. This is an enzyme produced by neutrophils and can be a sign of a urinary tract infection (white cells in urine = pyuria). These neutrophils, however, and the enzyme they produce can also be a sign of infection outside of the body such as vulvovaginitis/ They may also be found in the presence of haematuria.

The overall sensitivity for leucocyte esterase is 49 – 79% with a specificity of 79 – 87%. As a result, it can be considered to be suggestive of “possible UTI”, and “probable UTI” if found with a positive Nitrite sample (specificity increased to 99%).

What does this mean/bottom line: If positive and history suggestive (i.e. dysuria or fever) consider UTI and send for culture. If negative, then it is quite unlikely that there is an infection.

Nitrites

Nitrites are the breakdown product by gram-negative organisms such as E.coli. They are a more specific test (93-98%) than leucocytes but their sensitivity is lower (47-49%). The sensitivity is particularly poor as the urine needs to sit in the bladder for a while (at least 4 hours) for it to be positive.

What does this mean/bottom line: If it is positive, it is highly suggestive of infection. If it is negative, then does not necessarily rule out infection and needs correlation with leucocytes and presentation

Blood

Blood (haematuria) can be present for many reasons, so it is important to determine if there is microscopic (dipstick only) or macroscopic (visibly bloody). If blood is seen seen with leucocytes and/or nitrites then you should consider the child to have a UTI. If blood is seen with protein, then glomerulonephritis needs to be considered as a cause.

Causes of haematuria

  • Infection
  • Fever
  • Kidney stones
  • Glomerulonephritis
  • Renal tumour
  • Exercise
  • Trauma
  • Menstruation (doesn’t cause haematuria but will show up on dipstick so don’t forget to ask)

Isolated microscopic haematuria is common and only needs investigation if persistent, but make sure a blood pressure is checked as this is a often missed test. If haematuria is persistent it may need further investigation.

Reasons for further investigation

  • Macroscopic haematuria
  • Proteinuria
  • High blood pressure
  • Clinical oedema or features of fluid overload
  • Persisting microscopic haematuria (>2 occasions over 2-4 weeks apart)

Bottom line: If just microscopic haematuria on dipstick without explanation, then request a repeat sample with GP in 2-4 weeks. Don’t forget to check a blood pressure!

Protein

The body excretes a small amount which is usually not enough to pick up on dipstick.

If the body is “stressed” in illness or infection, it can cause proteinuria, however it is also sign of inflammation or damage within the kidney and so further history and examination is required.

When there is proteinuria of 2+ or more occurs during illness or a UTI, it can be repeated in a couple of weeks to ensure that it does not persist when the patient is well.

If there is no illness or infection, you would need to consider other causes such as glomerulonephritis and nephrotic syndrome, examine for oedema, and send off a protein : creatinine ratio sample.

Bottom line: small amounts can be seen in illness, but large amounts needs review depending on how the patient is

Glucose

This is not usually found in the urine, but small amounts can be detected if the patient is unwell, or is on steroids. If there is a large amount of glucose, consider checking a blood glucose to rule out diabetes, and see if there is any other evidence of kidney problems.

Ketones

A by-product from the breakdown of fat when sugar stores cannot be used. These can be seen in patients who have not been eating, vomiting and in DKA. It is always worth checking the blood glucose in these patients, as its absence in hypoglycaemic patients should alert you to a potential metabolic disorder.

Bottom line: Seen during periods of vomiting or not eating. Always check a blood glucose.

Bilirubin

Excessive bilirubin that is not dealt with in the liver is excreted in urine. Thus the presence of bilirubin in the urine can be seen in conjugated hyperbilirubinaemia, and therefore a feature of liver disease. If the urine dipstick measures urobilirubin then this can be seen normally on a dipstick (normal to 1+). A high urobilirubin could suggest haemolytic disease, as it reflects unconjugated bilirubin.

Bottom line: Bilirubin – not normal. Urobilinogen – normal (in small amounts)

Specific Gravity

This measures how dilute your urine is by comparing the solubility if the urine to water. If <1.005 then the urine is very dilute – do they drink a lot of water? If not the kidney may be unable to concentrate the urine, there it would be wise to consider checking a serum sodium and assess the patient for features of diabetes insipidus.

A high specific gravity means the urine is concentrated, and suggests that the patient may be dehydrated. If they do not appear hydrated, then does the patient appear oedematous? This could suggest systemic disease

A list of causes of high specific gravity

Bottom line: compare to the patients hydration status

pH

The urine pH varies and is usually slightly acidic. It can be influenced by diet and medication. Usually, alkaline urine is a product of vegetarian diets and medication. It can also be present in UTIs caused by urea splitting organisms, such as Proteus and Pseudomonas. It is seen in renal tubule anomalies or if the patient has metabolic alkalosis. Urinary acidosis is seen with high protein diets and can reflect systemic acidosis (for example, DKA, diarrhoea and vomiting)

Bottom line: Not very useful on its own.

Urine dipticks infographics

Selected references

https://litfl.com/dipstick-urinalysis/

https://patient.info/treatment-medication/urine-dipstick-test

Yates A. Urinalysis: how to interpret results. Nursing Times. 2016 Jun 8;112(2):1-3.

https://geekymedics.com/urinalysis-osce-guide/

https://www.mayoclinic.org/tests-procedures/urinalysis/about/pac-20384907

https://www.nice.org.uk/guidance/cg54/chapter/Recommendations#diagnosis

https://www.clinicalguidelines.scot.nhs.uk/nhsggc-paediatric-clinical-guidelines/nhsggc-guidelines/emergency-medicine/haematuria-management-and-investigation-in-paediatrics/

Fernandes DJ, Jaidev M, Castelino DN. Utility of dipstick test (nitrite and leukocyte esterase) and microscopic analysis of urine when compared to culture in the diagnosis of urinary tract infection in children. Int J Contemp Pediatr 2017;5:156-60

Jeng-Daw Tsai, Chun-Chen Lin, Stephan S. Yang, Diagnosis of pediatric urinary tract infections, Urological Science, Volume 27, Issue 3, 2016, Pages 131-134

Tsai JD, Lin CC, Yang SS. Diagnosis of pediatric urinary tract infections. Urological Science. 2016 Sep 1;27(3):131-4.

Sepsis 2020

Cite this article as:
Emma Lim. Sepsis 2020, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.32392

Where do we start?

Fever and suspected sepsis is our bread and butter. This post will take you through a whirlwind 2020 sepsis update. We’ll cover what sepsis is, how to recognize deterioration and the recent management updates in light of the new 2020 International Surviving Sepsis Campaign Guidelines1.

For me, it is all about “What keeps me up at night?” and there are two things I worry about. The first is missing cases of suspected sepsis.  Think back to all those hot, miserable children you sent home over your career and the heart sink you feel when someone says, “Remember that child you sent home yesterday?”.  My second worry is making bad choices; making mistakes about how much fluid to give or which antibiotics to choose or when to start inotropes.

What is sepsis?

Let’s start at the beginning. How do you get sepsis? A bacterial or viral infection causes a systemic, inflammatory response syndrome (SIRS). We are used to seeing children who have a fever and a fast heart rate or respiratory rate and a raised white cell count, for example with bronchiolitis. A certain proportion of those children will go on to get sepsis but not a lot.

Spotting sepsis in the paediatric ED is like a game of Where’s Wally: there are a whole lot of hot febrile children with accompanying hot cross parents. Fever is common but sepsis is rare – at a quick glance they all look like Wally, but, of course, there is actually only one real one and it takes a bit of time and patience to find him. It is the same with all those children with fever: around 55% have self-limiting viral infections, only 7-13% have serious bacterial infection (SBI)2-4 and only 1% have sepsis. The picture’s different in PICU; 10% of PICU admissions are for sepsis. The 2015 SPROUT study5 looked at 569 children in PICU with sepsis (8.2% point prevalence). 40% were caused by respiratory infections and 19% percent by bloodstream infections. A quarter (25%) of them died.

That quote “7-13% of febrile children have a serious bacterial infection” seems high. There are predefined criteria (such as pneumonia, urinary tract infection, meningitis, osteomyelitis, septic arthritis), but in a reductionist sense, sepsis is any infection that makes a child so unwell that they are admitted to hospital for more than 72 hours and need IV antibiotics. But, the need for admission is very subjective and dependent on the experience of the doctor and the parents’ level of concern.  The goal posts are constantly shifting.  Ten years ago, we would admit children with osteoarticular infections for 6 weeks of IV antibiotics. Now they can be in and out within 72 hours (with most of their course given orally). That doesn’t mean the infections have got less severe, it’s just that our treatments have changed.  And is a urinary tract infection over a year of age really a serious infection?  Most will get treated with a short course of oral antibiotics, as will children with pneumonia.  Because that’s a whole other controversy; reporting focal consolidation on a X ray is art not science and has been shown to be famously unreliable in double blind studies.  So if we remove children who have simple pneumonia, urinary tract infections in older children,  skin and soft tissue infections that do not have positive cultures, the number of true SBI is quite a lot less than the quoted 1 in 10.

Unbelievably, there is no good definition of ‘sepsis’ in paediatrics6, so we tend to use the adult Sepsis 3 definition7 which states:

“Sepsis is life threatening organ dysfunction caused by a dysregulated host immune response to infection including renal, respiratory, hepatic dysfunction or metabolic acidosis”. A small proportion of children or young people with sepsis will go into septic shock, where shock is defined as hypotension, or impaired perfusion requiring inotropes with a higher risk of death than sepsis.”

This doesn’t really help us spot sepsis early enough to prevent these children going into shock.  So far, there is no reliable way of pinpointing who these children are. However, there is some exciting news. 2020 has brought us new international evidence-based guidelines for the management of septic shock and sepsis associated organ dysfunction in children; the Surviving Sepsis Campaign.

This has been a huge piece of work by an incredible transatlantic consortium, including Mark Peters (for the horse’s mouth listen to our latest RCPCH Paediatric Sepsis Podcasts). I am going to take you through some of these recommendations, but I think everybody should read it themselves.  The consortium took 3 years and reviewed over 500 papers, but you only have to read this one paper, so go on, make your life easy!  

Spotting sepsis

Recommendation number one. In children who present acutely well, “we suggest implementing systematic screening for timely recognition.”

Take note of the word suggest. This means there is some, but not definitive, evidence. We all recognise systematic screening for sepsis is a huge problem for paediatricians. Most children with a fever have a self-limiting viral infection, and many of these children will have fever, tachycardia and tachypnoea. But most do not have sepsis.  However, if we use the UK-based NICE high-risk ‘Red Flag’ criteria, these children are all flagged as potentially having sepsis. They over-trigger, shown by a 2020 paper by Ruud Nijman which showed that 41% of all febrile children in PED present with warning signs of sepsis3. If you look at this paper in some detail, 50% of children aged 1-2 years triggered the NICE red high-risk category for tachycardia alone. This mirrors data from a local audit from the Great North Children’s Hospital Emergency Department, conducted between April and June of 2017. Of 868 patients, 5% had serious bacterial infections, but 50% triggered NICE high-risk criteria. Sam Romaine from Alderhey Children’s Hospital, and part of Enitan Carrol’s group, looked at 12,241 patients and again, 55% triggered NICE high risk criteria8. For a full critical review of Ruud’s paper, take a look at our Searching for Sepsis post.

The NICE high risk criteria have a very high sensitivity but limited specificity, which means although they ‘over-trigger’, if a child doesn’t have any red flags then they are potentially ‘good to go’, helping inform safe discharge.

Is there a better score?

For a long time, adults have used the Q-SOFA score, a quick sepsis related organ failure assessment. Typically, this adult score has performed poorly in children. Enitan Carroll’s group have looked at a modified Q-SOFA score called the LQ-SOFA score (L for Liverpool), modified to predict critical care admission rather than sepsis. Critical care admission is a more common outcome than sepsis, particularly relevant because this helps us understand which children are at risk of deterioration. The modified score, is made up of four simple, straightforward criteria, including capillary refill, AVPU (that’s Alert, Verbal, only to Pain or Unresponsive), heart rate and respiratory rate, purposefully not including blood pressure, making this quick and easy to use as a screening tool. But what did they find? Carroll’s group compared five different scores that could help us predict sepsis or deterioration: lactate, CRP, adult Q-SOFA, NICE and LQ-SOFA. Lactate performed the least well, CRP and Q-SOFA a little bit better, NICE high-risk criteria better again, but best of all was the LQ-SOFA score. 

This work suggests that there are more sensitive tools out there, but these need to be combined with some way of de-escalating children who trigger because most of these children have a SIRS response from a self-limiting viral infection and not sepsis. De-escalation is usually done by ‘a senior review,’ with the intention of differentiating the hot and bothered child who has a viral infection from early sepsis.

Listen to parents

There are many examples of systematic screening protocols, the best being electronic scores. But they are not perfect.  Most importantly, the good ones listen to parents. Parental concern or health professional concern is particularly important for children with complex medical conditions: neurodisability, recurrent chest infections, those with indwelling lines or fed by gastrostomy. These children often don’t have typical signs and symptoms that health care professionals associate with infections or sepsis, often presenting with nothing more than their parents saying that they’re not well or not quite themselves. These children can be hypothermic (due to hypothalamic dysfunction) and run ‘cold’ so when they get an infection, their temperature may goes up to ‘normal’ (37 degrees), not triggering at all. The presenting signs can be very, very subtle like not tolerating their feed, or vomiting, or they may just be miserable and unhappy. This is why any escalation tool or score must in some way include parental concern. The NICE sepsis guidelines from 2017 tells us to pay particular attention to ‘concerns expressed by parents, families or carers’, for example, changes from usual behaviour.’  We must not underestimate the expertise of parents and we should incorporate them into the team of people caring for their children.

Doctors can be wary of parental concern but if we look at a systematic review of family-initiated escalation of care for the deteriorating patients in hospital, we can see that this wariness is unfounded.  Gill et al 20169 looked at a systematic review of ten articles (all descriptive studies) over ten years evaluating response systems for patients and families; five described a triaged response; five reported systems for families to directly activate the rapid response team. There were a total of 426 family-initiated calls, range 0.17 to 11 per month, with no deaths reported. All calls were deemed to be appropriate and three calls resulted in intensive care unit admissions.”

I believe there is evidence that parents only escalate when they need to.  As one of our parents of a child with a complex medical condition said;

Please listen to us when we say something is not right, we can see subtle changes in children, in our children, in their health and behaviour. That may not be apparent to the casual observer or even health professionals like yourselves and children like them cannot speak for themselves. Therefore, as parents, we have to ensure that we advocate for them in the strongest possible terms. We do not think we are better than the team, nor are we full of our own importance. But we are simply trying to give a voice to our children as they don’t have one of their own.”

What do you do next?

The Surviving Sepsis campaign developed a management algorithm for children, and while it is useful, there’s a lot of information, for many different teams in a small space. Firstly, when you look closely, the lower half (in black) is actually all about management in a Paediatric Intensive Care (PICU) setting -treatment of refractory shock and advanced haemodynamic monitoring. For paediatric emergency physicians, there is a lot that has to happen first! Let’s break it down.

The first thing that the international guidelines asks us to do is get intravenous or intraosseous access. Please only have three tries at getting intravenous access and if this isn’t successful, go straight to intraosseous access. It’s a great safe route and can be much easier to get than intravenous especially in children with complex medical conditions whom may be difficult to cannulate. Although it may feel like using an IO in an awake child will be traumatic , flushing with 0.5mg/kg of 2% lignocaine before you infuse fluids, antibiotics and other drugs, will reduce the pain.

Test, tests, tests

Recommendation number two. Get a blood culture.

This should always be your next priority, as long as it does not delay treatment. Let’s just think for a moment about blood cultures. Blood cultures are old technology. They were developed in the 1950s and have not really changed since. Traditionally, blood cultures are read at 48 hours but often don’t give any definitive answer. The European Union Childhood Life-threatening Infectious Disease Study (EUCLIDS)10 was a prospective, multi-centre, cohort study of 2844 children under 18  with sepsis (or suspected sepsis) or severe focal infections, admitted to 98 hospitals across Europe and incredibly in 50% of patients the causative organism remained unidentified! Alasdair Munroe explains more in his blood culture post.

What we really want is a point of care test, a test that takes less than 60 minutes, that can quickly differentiate between viral and bacterial infections at the child’s bedside11. Andreola et al12 (and more recent studies by Ruud Nijman again) looked at febrile children and infants in Emergency Departments and this is what they found:

White cell counts, we know, are not helpful. A raised white cell count has poor sensitivity and specificity, so while CRP is better and PCT better still there is room for improvement.  All these tests have problems with sensitivity which means there is still going to be a worrying number of falsely negative tests.  We know this, for example, in children with diseases that progress quickly like meningococcaemia or sepsis who can have normal inflammatory markers early on.

However, new tests are on the horizon. The PERFORM/IRIS group published a diagnostic test using a two-transcript host RNA signature that can discriminate between bacterial and viral infections in febrile children (Herberg, JAMA 2016), using gene arrays to demonstrate up or down regulation of protein expression. Sensitivity in the validation group was 100% and specificity 96.4%13.  

But we don’t just want to know if a child has a bacterial or viral infection, we really want a clinical predictor of severity that could tell us which children are going to get very ill.  We have a few tests, but they’re not very specific. We often look at blood gases, looking for a metabolic acidosis. But that is very broad. What about a lactate >2mmol/l? The international guidelines did not recommend the use of lactate as the evidence is lacking, although it can give an idea of the trend and whether a child is getting better or worse and is generally considered to be best practice and is already standard in adult sepsis. But this is in direct contrast to a study by Elliot Long and team published earlier this year14 looking at predictors of organ dysfunction in over 6000 children presenting to the ED with fever. A lactate of 4 or higher was one of the best performing ED predictor of new organ dysfunction, the need for inotropic support and the need for mechanical ventilation. Take a look at Deirdre Philbin’s DFTB review of the study.

More new tests are coming.  For example, interleukin 6 and 10 may be able to predict which children with febrile neutropenia have serious infections and mid regional pro-adrenoedullin (MR pro-ADM) may be a promising biomarker to predict sepsis and septic shock15. So, watch this space!

Antibiotics

Recommendation number three. Start broad-spectrum antibiotics.

Moving on from tests to treatment, we now want to look at recommendation number three, when to start broad-spectrum antibiotics. There is a change in timing here.

In children with septic shock, antimicrobial therapy should be started as soon as possible and within one hour of recognition of sepsis.”  But, in children with suspected sepsis (i.e. organ dysfunction, but not shock), most of the children we see, guidelines suggest starting antimicrobial treatment as soon as possible after evaluation – you have 3 hours not 1 hour16.

This is important, because it gives you a chance to do tests and decide whether the child in front of you has sepsis or just a SIRS response due to a viral infection. This has bigger implications than just saving hospital beds, because we know timely initial empirical antibiotics will save lives, but unnecessary antibiotic use for all children with fevers increases antibiotic side effects, antibiotic resistance and cost.

Antibiotic choice

There are other recommendations around antibiotics. Importantly, the new consensus recommends a broad-spectrum antibiotic therapy with one single drug in normal children, such as  cefotaxime or ceftriaxone or, if they are allergic, meropenem.

As a quick aside, let’s think about penicillin allergy.

It’s important to get a history and to understand what a ‘real’ penicillin allergy is. We see a lot of children who present with a vague story of having been given a couple of doses of penicillin many years ago, who developed a rash and have been labelled as ‘penicillin allergic’.  But doing that in the heat of the moment can be tricky.

Zagursky believes “Avoidance of cephalosporins, when they are the drug of choice in a penicillin-allergic individual, results in significant morbidity that outweighs the low risk of anaphylaxis. We conclude that there is ample evidence to allow the safe use of cephalosporins in patients with isolated confirmed penicillin or amoxicillin allergy”17

Studies have found the risk of crossover between penicillin/cephalosporin reactions is <1%, so using cephalosporins as a first line is safe.  If the child also has cephalosporin sensitivity, they may need a carbapenem like meropenem.  Later, please think about referring these children to your local allergy service for penicillin or cephalosporin de-labelling, which entails having an antibiotic challenge under controlled, safe circumstances.

Moving on… antibiotics in immunocompromised children

The guidelines suggest using empiric multi-drug therapy in children with immunocompromise and those at high risk for multi-drug resistant pathogens. In this case, you might choose piperacillin-tazobactam and, if shock is present, amikacin. You can add teicoplanin if you suspect a line infection, with rigors when flushing the line, or a line site infection, with redness around their exit site, or signs of any soft tissue cellulitis.

The recommendations also cover antimicrobial stewardship. Once the pathogen and sensitivities are available, the guidelines recommend narrowing antimicrobial therapy coverage. This means narrowing down the antibiotic to something specific to the clinical presentation, site of infection, or risk factors.  Ask yourself these questions:

  • Is the child is showing clinical improvement?
  • Can they have their antibiotics at home? (via a paediatric out-patient antibiotic service)
  • Can they switch to oral antibiotics?
  • Can they stop their antibiotics?  If you don’t find any bugs, and the child is well, then the guidelines recommend stopping antimicrobial therapy.

Remember to phone a friend

Infectious disease teams or microbiologists; you never need to make decisions alone. The guidelines recommended daily assessment with clinical laboratory assessment for de-escalation of antimicrobial therapy. Assessment includes a review of the ongoing indication for antibiotics after the first 48 hours and should be guided by results from microbiology, signs of clinical improvement and evidence of reducing inflammatory markers, such as a halving of CRP, or if the child’s fever has settled for more than 24 hours.

Fluids

Moving on from antibiotics to fluids. The Surviving Sepsis Campaign has another paediatric management algorithm for fluid and vasoactive drugs. It’s also quite busy, incorporating the results of the FEAST study18.  It’s split into two, a green side and a blue side. The green side is for children who live in healthcare systems without intensive care, while the blue side is healthcare systems with paediatric intensive care. The change boils down to being more cautious with fluids.  The guidelines recommend 10-20 ml/kg boluses. I suggest giving 10 ml/kg and then reassessing for signs of fluid overload with hepatomegaly and listening for basal crackles suggesting pulmonary oedema, repeating a second or third bolus as needed.  I use 10 ml/kg because it’s the same in sepsis, in neonates and in trauma.

If the child needs more volume, give them more volume; you can repeat 10ml/kg boluses up to 40 ml/kg or more as needed just use smaller aliquots.  Remember there may still be children who need big volumes of fluid early on, and we have PICU readily available and the technology to support children’s circulation and ventilation and ‘dry them out’ later.  There isn’t enough evidence to fluid restrict children with sepsis in the ‘resource rich’ world just yet but trials are ongoing. The Squeeze Canadian Critical Care Group19 has started a study, so watch this space for results.

Which fluids should you choose?

Please use crystalloids not colloids. And although historically we have used 0.9% saline, it is better to choose balanced or buffered solutions such as Ringer’s lactate or Plasmalyte. Too much saline can cause hyperchloremic acidosis.   

Inotropes

There has been a real sea change in our approach with inotropes. As we’re being more cautious with fluid resuscitation, we need to start giving inotropes earlier. After giving 40 to 60 ml/kg have your inotrope lined up ready to go.  There is good evidence that the drug of choice should be adrenaline20.  You can give adrenaline via a peripheral intravenous cannula or an intra osseous cannula safely if you don’t have central access. There have also been studies in adults that showed that peripheral adrenaline is also safe, especially when given for less than four hours or in a diluted dose.

Safety netting

Most of the febrile children we see will be discharged; safe discharge is a big priority because that’s what the majority of hot bothered children need: good advice and home care.  Winters (2017)21 looked at 33,000 children who were discharged from Emergency Departments with abnormal vital signs. 27,000 (80%) of them were discharged with normal vital signs, with only one case of potentially preventable permanent disability (a child who presented with tummy pains and came back with torsion of the testes, unlucky). 5,500 children (16%) were discharged with abnormal vital signs; there were no permanent disability or deaths from this group. So, you can send children home with fevers safely. But, the proviso to this is they need good safety netting on discharge, including both verbal and written information. This is one of the NICE recommendations. Our discharge safety netting leaflet22, which (gives some straightforward, practical information about giving anti-pyretic medication like paracetamol and ibuprofen), works like a ‘parent’s PEWS’ chart. It allows parents to see if their child is OK to stay at home or if they’re at some risk and should contact the GP, go to a walk in centre or call 111-advice line if they haven’t got better in 48 hours.  If the child is on the ‘high risk’ side, we want to see them back in the Paediatric Emergency Department.

In summary…

So, in summary, please screen for sepsis, we should all be doing it. I don’t know the best systems to help you but, ideally, you should have electronic observations, protocols and local guidelines.  Be aware that in the ED the incidence of sepsis is rare and that recent surviving sepsis campaign guidance suggests you can safely observe while you make a decision on treatment. Give antibiotics within 60 minutes in septic shock, but in sepsis with no shock you have three hours. If you are treating use fluid cautiously, with 10-20 ml/kg boluses and frequent reassessments.  Start adrenaline early if appropriate, and this can be given safely, peripherally.  Finally, safety netting is essential.

Thank you very much for reading this right through to the end! If you want to hear more, please have a listen to our Paediatric Sepsis podcast, hosted by the RCPCH.

Selected references

  1. Surviving Sepsis Campaign International Guidelines for the Management of Septic Shock and Sepsis-Associated Organ Dysfunction in Children. Weiss SL et al. Pediatr Crit Care Med. 2020 Feb;21(2):e52-e106. doi: 10.1097/PCC.0000000000002198.PMID: 32032273
  2. Craig JC et al. The accuracy of clinical symptoms and signs for the diagnosis of serious bacterial infection in young febrile children: prospective cohort study of 15 781 febrile illnesses. BMJ. 2010;340:c1594 10.1136/bmj.c1594
  3. Nijman RG et al. Clinical prediction model to aid emergency doctors managing febrile children at risk of serious bacterial infections: diagnostic study. BMJ. 2013;346:f1706 10.1136/bmj.f1706
  4. van de Maat J et al. Antibiotic prescription for febrile children in European emergency departments: a cross-sectional, observational study. Lancet Infect Dis. 2019;19:382–91. 10.1016/S1473-3099(18)30672-8
  5. Weiss SL et al. Sepsis Prevalence, Outcomes, and Therapies (SPROUT) Study Investigators and Pediatric Acute Lung Injury and Sepsis Investigators (PALISI) Network. Global epidemiology of pediatric severe sepsis: the sepsis prevalence, outcomes, and therapies study. Am J Respir Crit Care Med. 2015 May 15;191(10):1147-57. doi: 10.1164/rccm.201412-2323OC. Erratum in: Am J Respir Crit Care Med. 2016 Jan 15;193(2):223-4. PMID: 25734408; PMCID: PMC4451622.
  6. International pediatric sepsis consensus conference: definitions for sepsis and organ dysfunction in pediatrics. Goldstein B, Giroir B, Randolph A; International Consensus Conference on Pediatric Sepsis.Pediatr Crit Care Med. 2005 Jan;6(1):2-8. doi: 10.1097/01.PCC.0000149131.72248.E6. PMID: 15636651 Review
  7. Developing a New Definition and Assessing New Clinical Criteria for Septic Shock: For the Third International Consensus Definitions for Sepsis and Septic Shock (Sepsis-3). Shankar-Hari M et al. Sepsis Definitions Task Force. JAMA. 2016 Feb 23;315(8):775-87. doi: 10.1001/jama.2016.0289. PMID: 26903336
  8. Romaine ST et al. Accuracy of a Modified qSOFA Score for Predicting Critical Care Admission in Febrile Children. Pediatrics. 2020 Oct;146(4):e20200782. doi: 10.1542/peds.2020-0782. PMID: 32978294; PMCID: PMC7786830.
  9. Gill FJ et al. The Impact of Implementation of Family-Initiated Escalation of Care for the Deteriorating Patient in Hospital: A Systematic Review. Worldviews Evid Based Nurs. 2016 Aug;13(4):303-13. doi: 10.1111/wvn.12168. Epub 2016 Jun 3. PMID: 27258792.
  10. Martinón-Torres F et al. EUCLIDS Consortium. Life-threatening infections in children in Europe (the EUCLIDS Project): a prospective cohort study. Lancet Child Adolesc Health. 2018 Jun;2(6):404-414. doi: 10.1016/S2352-4642(18)30113-5. Epub 2018 Apr 28. PMID: 30169282.
  11. Herberg JA et al. IRIS Consortium. Diagnostic Test Accuracy of a 2-Transcript Host RNA Signature for Discriminating Bacterial vs Viral Infection in Febrile Children. JAMA. 2016 Aug 23-30;316(8):835-45. doi: 10.1001/jama.2016.11236. Erratum in: JAMA. 2017 Feb 7;317(5):538. PMID: 27552617; PMCID: PMC5997174.
  12. Andreola, B et al. Procalcitonin and C-Reactive Protein as Diagnostic Markers of Severe Bacterial Infections in Febrile Infants and Children in the Emergency Department, The Pediatric Infectious Disease Journal: August 2007 – Volume 26 – Issue 8 – p 672-677. doi: 10.1097/INF.0b013e31806215e3
  13. Herberg JA et al. Diagnostic Test Accuracy of a 2-Transcript Host RNA Signature for Discriminating Bacterial vs Viral Infection in Febrile Children. JAMA. 2016 Aug 23-30;316(8):835-45. doi: 10.1001/jama.2016.11236. Erratum in: JAMA. 2017 Feb 7;317(5):538. PMID: 27552617; PMCID: PMC5997174.
  14. Long E, Solan T, Stephens DJ, et al. Febrile children in the Emergency Department: Frequency and predictors of poor outcome. Acta Paediatr. 2020; 00: 1– 10 
  15. Xia T, Xu X, Zhao N, Luo Z, Tang Y. Comparison of the diagnostic power of cytokine patterns and procalcitonin for predicting infection among paediatric haematology/oncology patients. Clin Microbiol Infect. 2016 Dec;22(12):996-1001. doi: 10.1016/j.cmi.2016.09.013. Epub 2016 Sep 22. PMID: 27665705.
  16. Elke G et al. SepNet Critical Care Trials Group. The use of mid-regional proadrenomedullin to identify disease severity and treatment response to sepsis – a secondary analysis of a large randomised controlled trial. Crit Care. 2018 Mar 21;22(1):79. doi: 10.1186/s13054-018-2001-5. PMID: 29562917; PMCID: PMC5863464.
  17. Zagursky RJ, Pichichero ME. Cross-reactivity in β-Lactam Allergy. J Allergy Clin Immunol Pract. 2018 Jan-Feb;6(1):72-81.e1. doi: 10.1016/j.jaip.2017.08.027. Epub 2017 Oct 7. PMID: 29017833.
  18. Maitland K et al. FEAST Trial Group. Mortality after fluid bolus in African children with severe infection. N Engl J Med. 2011 Jun 30;364(26):2483-95. doi: 10.1056/NEJMoa1101549. Epub 2011 May 26. PMID: 21615299.
  19. Parker, M.J., Thabane, L., Fox-Robichaud, A. et al. A trial to determine whether septic shock-reversal is quicker in pediatric patients randomized to an early goal-directed fluid-sparing strategy versus usual care (SQUEEZE): study protocol for a pilot randomized controlled trial. Trials 17, 556 (2016). https://doi.org/10.1186/s13063-016-1689-2
  20. Ramaswamy KN, Singhi S, Jayashree M, Bansal A, Nallasamy K. Double-Blind Randomized Clinical Trial Comparing Dopamine and Epinephrine in Pediatric Fluid-Refractory Hypotensive Septic Shock. Pediatr Crit Care Med. 2016 Nov;17(11):e502-e512. doi: 10.1097/PCC.0000000000000954. PMID: 27673385.
  21. Winter J, Waxman MJ, Waterman G, Ata A, Frisch A, Collins KP, King C. Pediatric Patients Discharged from the Emergency Department with Abnormal Vital Signs. West J Emerg Med. 2017 Aug;18(5):878-883. doi: 10.5811/westjem.2017.5.33000. Epub 2017 Jul 19. PMID: 28874940; PMCID: PMC5576624.
  22. Lim E, Mistry RD, Battersby A, Dockerty K, Koshy A, Chopra MN, Carey MC, Latour JM. “How to Recognize if Your Child Is Seriously Ill” During COVID-19 Lockdown: An Evaluation of Parents’ Confidence and Health-Seeking Behaviors. Front Pediatr. 2020 Nov 17;8:580323. doi: 10.3389/fped.2020.580323. PMID: 33313025; PMCID: PMC7707121.

Febrile Infection-Related Epilepsy Syndrome (FIRES)

Cite this article as:
Jessica Archibald and Catherine Murphy. Febrile Infection-Related Epilepsy Syndrome (FIRES), Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.32716

An 8-year-old presents to the emergency department following a first seizure episode. They had a witnessed generalised tonic-clonic seizure that morning lasting approximately 60 seconds and remain post-ictal. They have a history of being non-specifically unwell yesterday with subjective fever, lethargy and a mild headache. They have no significant past medical history and no family history of seizures. The examination is unremarkable. Whilst in the emergency department they have a further two self-terminating generalised tonic-clonic seizures.

Febrile Infection-Related Epilepsy Syndrome (FIRES) is a rare epileptic encephalopathy that results in prolonged refractory status epilepticus in previously well patients.

Presenting Features

FIRES typically presents in children between the age of 3 to 15 years, with intractable status epilepticus, 2 to 10 days post a febrile illness. The preceding illness is most commonly an upper respiratory tract infection or gastroenteritis. Fevers may have resolved prior to the onset of the acute phase of the condition.

The acute phase of the illness is characterised by frequent seizures, rapidly progressing to status epilepticus. Although the seizures are initially focal in nature, they may evolve into secondary generalised seizures. The acute phase can be prolonged, lasting from weeks to months. An association with rash, liver derangement and arrhythmia has been noted in the literature. There is no latency period.

The chronic phase is denoted by refractory epilepsy, resulting in seizures that may cluster every 2 to 4 weeks. This is often associated with severe neurological impairment and cognitive decline.

FIRES had previously been thought to only occur in children, and New-Onset Refractory Status Epilepticus (NORSE) only in adults, however this theory has been disproven. Although FIRES is more prevalent in children, it has been known to also occur in adults. As such, FIRES is now considered a subtype of NORSE, characterised by a preceding febrile illness. It has previously been known as Acute Encephalitis with Refractory, Bepetitive Partial Seizures (AERRPS) and Devastating Epilepsy in School-age Children (DESC).

Aetiology

The aetiology of FIRES in unknown and as such the pathophysiology remains unclear.

One theory is that FIRES is a form of severe infectious encephalitis, but as yet no infectious agent has been identified, and the refractory nature of the seizures is atypical of encephalitis. Another hypothesis suggests FIRES is the result of an immune response, however, there is not enough evidence to support this theory.

A case identifying anti-GABA A receptor antibodies in the CSF of a patient who presented with severe refractory status epilepticus associated with a fever led to speculation that the condition may be autoimmune-mediated. Again this has not been proven and the case may have been an exception rather than a rule.

Other theories include genetic associations and potential links with metabolic disease, but as yet a cause has not been identified.

Diagnosis and Differentials

The diagnosis of FIRES is essentially clinical, as FIRES is a cryptogenic illness. The work up is initially general, and focused on the exclusion of other treatable causes, such as infectious or autoimmune encephalitis.

A detailed history will identify the preceding febrile illness, and would be focussed on the identification of risk factors for other causes for the presentation, including exposure to animals, drugs and toxins, recent foreign travel and immunosuppression.

Blood sampling will be used to identify an infectious cause for the presentation, through full blood count, blood cultures and a screen for atypical infective agents. Lumbar puncture should be performed for CSF sampling in order to investigate bacterial, viral, fungal or autoimmune causes. CSF may show a mildly elevated white cell count in those with FIRES.

EEGs may show a generalised slowing, in keeping with an encephalopathic picture, but do little in the way of distinguishing between other causes of seizures. However, they are useful in guiding treatment and identifying non-convulsive seizures.

Initial MRI imaging is often normal, however, follow up imaging has been associated with devastating changes. Early MRI, in the first weeks of the acute illness, has shown swelling of the mesial temporal structures and increased T2 weighted signal. Follow up MRI, greater than 6 months after onset, may be associated with bilateral mesial temporal atrophy and increased T2 weighted signal. It should be noted that MRI may be normal in 50% of cases.

Differentials to consider are Dravet Syndrome, which presents with a febrile illness associated with status epilepticus, though this tends to present within the first year of life. Also Alper’s Disease, which presents with refractory seizures in previously well children, and is often associated with liver disease.

The patient is loaded with levetiracetam (40mg/kg) as per hospital guidelines, and admitted under paediatrics locally. A CT head is unremarkable and bloods show mild LFT derangement with normal inflammatory markers. They are treated empirically with intravenous cefotaxime and aciclovir. Later that afternoon they develop a fever of 38.3.

The GCS fluctuates between 11 to 13 with no full recovery to baseline until later that evening. Following two focal seizures the next afternoon, they are transferred to the local tertiary centre for further investigation and management.

Initial Management

Initial management involves treating the seizure, and more often status epilepticus. Local hospitals have their own guideline for managing status epilepticus but the first line is typically benzodiazepines (lorazepam, diazepam, midazolam, clonazepam). Second-line treatment is standard anti-convulsants (levetiracetam, phenytoin, phenobarbitone, sodium valproate), however, FIRES does not typically respond to these medications even in high doses.


The seizure pattern in FIRES is often resistant to multiple anti-epileptics. Alternative treatment options have to be sought although there is limited evidence as to the optimal treatment.

Long-term Management

There is limited data on the treatment of FIRES, however, they all conclude the seizures are very difficult to manage and often require polytherapy. Some of the alternative treatment options include drug-induced burst-suppression comas, immunotherapy, a ketogenic diet, vagus nerve stimulation, therapeutic hypothermia and intravenous magnesium sulfate. The most commonly used and researched options are discussed below.

Burst suppression coma

Burst suppression coma induction is viewed as standard care for refractory status epilepticus. If first and second-line treatments fail the next option involves high doses of anti-convulsants along with anaesthetic agents, for example, an infusion of midazolam, barbiturates or propofol. Unfortunately when the anti-convulsants are weaned the seizures tend to reoccur. Prolonged burst suppression coma has been associated with a significantly worse cognitive outcome and poorer prognosis.

Immunotherapy

Immunotherapy has been trialled due to the suspected role of inflammation in the pathogenesis of FIRES. High dose steroids, intravenous immunoglobulin and plasmapheresis have all been used. There is limited evidence to suggest a beneficial role in the management of refractory epilepsy. A large-scale Japanese study described 2 out of 12 patients responding to steroids, although there is not enough evidence to support this as a treatment option. Treatment with immunotherapy is often associated with significant side effects

Anakinra is a recombinant and modified human interleukin-1 receptor antagonist protein. Recent evidence has shown it to be an effective and promising treatment option in patients with FIRES, though relapse has been reported after withdrawal. It has been shown to decrease the duration of mechanical ventilation and hospital length of stay, and possibly seizure reduction. Future studies are required to understand the optimum dosing regime and safety of anakinra.

Ketogenic diet

A ketogenic diet is a high fat, adequate protein and low carbohydrate diet aimed at imitating the body’s fasting state. The body, therefore, metabolises fat for energy. The early introduction of the ketogenic diet has shown to be beneficial in the management of FIRES in uncontrolled trials. It has been suggested that the ketogenic diet may have an anti-inflammatory, as well as an anti-convulsant effect. Some reports suggest it may also have a positive effect on long term cognition. Currently, it is one of the only management options shown to be effective. Future controlled studies are needed to prove this efficacy.

Vagus nerve stimulation

Vagus nerve stimulation (VNS) involves the implantation of an electrode that produces intermittent electrical stimulation into the left cervical vagus nerve. Case reports have found benefit from VNS in the cessation of seizures in patients with refractory status epilepticus and NORSE. There is limited evidence of its use in FIRES.

Long term effects

The prognosis of FIRES is poor. The outcome varies with the length of the acute phase with mortality rates up to 30%. Of those patients who survive there is 66-100% chance that they will have long term cognitive impairment due to damage of the frontal and temporal lobe functions. Survivors with a normal cognitive function will present with a spectrum of learning disabilities, behavioural disorders, memory issues and sensory changes. There is a high risk of recurrent status epilepticus. Unfortunately, only a small proportion of survivors will have no neurologic sequelae.

The patient required a lengthy PICU admission where they were managed with a burst suppression coma, ketogenic diet, high dose steroids and intravenous immunoglobulin.

They were later diagnosed with Febrile-Infection Related Epilepsy Syndrome after extensive investigations, including a normal brain MRI and a lumbar puncture which showed a mildly elevated white cell count but was otherwise unremarkable.

They are currently seizure free on a combination of oral phenobarbitone, perampanel and levetiracetam but have some cognitive sequelae.

References

  1. Fox K, Wells ME, Tennison M, Vaughn B. Febrile Infection-Related Epilepsy Syndrome (FIRES): A literature Review and Case Study. Neurodiagn J. 2017;57(3):224-233. doi: 10.1080/21646821.2017.1355181. PMID: 28898171
  2. Lee H, Chi C. Febrile infection-related epilepsy syndrome (FIRES): therapeutic complications, long-term neurological and Neuro imaging follow-up. Seizure. 2018;56:53-59.
  3. Serino D, Santarone M, Caputo D, Fusco L. Febrile infection-related epilepsy syndrome (FIRES): prevalence, impact and management strategies. Neuropsychiatric Disease and Treatment. 2019;Volume 15:1897-1903.
  4. NORSE (New Onset Refractory Status Epilepticus) and FIRES (Febrile Infection-Related Epilepsy Syndrome) – NORD (National Organization for Rare Disorders) [Internet]. NORD (National Organization for Rare Disorders). 2021 [cited 20 January 2021]. Available from: https://rarediseases.org/rare-diseases/new-onset-refractory-status-epilepticus-norse
  5. Orphanet: Febrile infection related epilepsy syndrome [Internet]. Orpha.net. 2021 [cited 20 January 2021]. Available from: https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=163703
  6. Caputo D, Iorio R, Vigevano F, Fusco L. Febrile infection-related epilepsy syndrome (FIRES) with super-refractory status epilepticus revealing autoimmune encephalitis due to GABA A R antibodies. European Journal of Paediatric Neurology. 2018;22(1):182-185.
  7. Diagnostic Evaluation — NORSE INSTITUTE [Internet]. NORSE INSTITUTE. 2021 [cited 20 January 2021]. Available from: http://www.norseinstitute.org/definitions
  8. Dravet Syndrome – NORD (National Organization for Rare Disorders) [Internet]. NORD (National Organization for Rare Disorders). 2021 [cited 20 January 2021]. Available from: https://rarediseases.org/rare-diseases/dravet-syndrome-spectrum
  9. Alpers Disease – NORD (National Organization for Rare Disorders) [Internet]. NORD (National Organization for Rare Disorders). 2021 [cited 20 January 2021]. Available from: https://rarediseases.org/rare-diseases/alpers-disease
  10. Wheless. J. Treatment of refractory convulsive status epilepticus in children: other therapies. Seminars in Paediatric Neurology (2010) 17 (3) 190-194.
  11. Kramur U et al. Febrile infection-related epilepsy syndrome (FIRES): Pathogenesis, treatment and outcome. Epilepsia (2011) 52: 1956-65.
  12. Gaspard et al. New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES): State of the art and perspectives. Epilepsia (2018). 59 (4) 745-752.
  13. Sakuma et al. 2010. Acute encephalitis with refractory, repetitive partial seizures (AERRPS): a peculiar form of childhood encephalitis. Acta Neurol Scand 121:251–256.
  14. Hon et al. Febrile Infection-Related Epilepsy Syndrome (FIRES): An overview of treatment and recent patents. Recent Patents on Inflammation & Allergy Drug Discovery (2018). 12 (2): 128-135
  15. Maniscalco et al. The off-label use of anakinra in pediatric systemic autoinflammatory diseases. The Advance Musculoskeletal Disease (2020)
  16. Shukla N et al. Anakinra (IL-1 blockade) use in children with suspected FIRES: a single institution experience. Neurol 2018; 90: 346
  17. Lai et al. Anakinra usage in febrile infection related epilepsy syndrome: an international cohort. Annals of Clinical and Translational Neurology (2020). 7(12): 2467 – 2474
  18. Dibue-Adjei et al. 2019. Vagus nerve stimulation in refractory and super-refractory status epilepticus – A systematic review. Brain Stimuatlion. 12 (4) 1101-1110.
  19. Kurukumbi et al. 2019. Vagus nerve stimulation (VNS) in super refractory new onset refractory status epilepticus (NORSE). Case Reports in Neu

Distal femoral physeal fractures

Cite this article as:
Sinead Fox. Distal femoral physeal fractures, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.31852

Marcus is a 13-year-old boy who sustained an injury to his right leg during a rugby match, describing a hyperextension injury at the knee during a tackle. He is brought to your ED via ambulance. He is complaining of a significantly painful right leg which is quite swollen and exquisitely tender at the distal thigh and knee. Marcus is treated using advanced paediatric trauma protocols and no other injuries are identified. The injury to his leg is a closed injury and his distal neurovascular status is intact. An IV cannula is inserted. IV Morphine is administered for pain and an above knee back-slab is applied to splint the injury. An X-ray is suspicious for a Salter Harris type III fracture to his distal femur. Marcus is kept nil by mouth and referred to the orthopaedic team for urgent review.

Epidemiology

Distal femoral physeal injuries are uncommon. They represent approximately 7% of lower extremity injuries in children and less than 1% of all paediatric fractures. Although rare, distal femoral physeal fractures have a high incidence of long-term complications. These injuries must be considered in patients with open physes to avoid misdiagnosis as collateral ligament injuries and minimise the risk of complications.

Anatomy

Bones

The epiphysis of the distal femur is the first epiphysis to ossify and is present at birth. From birth to skeletal maturity the distal femoral physis contributes 70% of the growth of the femur and 37% of the growth of the lower extremity. The distal femoral physis has an average growth of 1.0 cm/year, making it the fastest growing physis. Growth ceases at a mean skeletal age of fourteen years old in girls and sixteen years old in boys. Compared with ligamentous structures, the physis is generally considered weaker within joints of children, therefore most periarticular injuries involve the growth plate.

Muscles

Both heads of the gastrocnemius and plantaris muscles originate just proximal to the physis of the distal femur.

Ligaments

The collateral ligaments of the knee attach distal to the physis at the level of the epiphysis of the distal femur. The ACL and PCL attach to epiphysis at the intercondylar notch and can be injured.

Blood supply

The popliteal artery is an important vessel in this area and can be prone to injury. It is important to undertake a careful neurovascular exam in children with confirmed or suspected distal femur fractures. Although rare, injury to the popliteal artery can result in loss of lower limb viability.

Nerves

The sciatic nerve divides into the peroneal and tibial nerves proximal to the popliteal space. 

Mechanism of injury

Distal femoral physeal injuries can be caused by hyperextension of the knee, with or without varus or valgus strain, or can occur by direct impact to the area. In the days of horse drawn wagons, this injury was coined ‘wagon-wheel injury’ or ‘cartwheel injury’ because it occurred when children attempted to jump onto a moving wagon and the leg became entrapped between the spoke of the moving wheel. Nowadays, most distal femur fractures are as a result of significant trauma such as motor vehicle accidents or sports related trauma. This is especially true for children between the ages of 2-11 years old, however less force is required for physeal disruption in infants and adolescents.

Children with underlying conditions such as neuromuscular disorders, joint contractures, difficult deliveries, or nutritional deficiencies can be more predisposed to distal femur physeal injuries.

Evaluation

Children with distal femur fractures will have significant pain and may be quite anxious, especially if assessment is undertaken in a resus situation. Make sure to follow advanced trauma protocols and ensure the child has had adequate analgesia.

Children with distal femoral physeal fractures generally present with pain, swelling and tenderness to the distal femur or knee and an inability to weight bear. Displaced separation of the distal femoral epiphysis may produce clinical deformity. Abrasions or lacerations of the overlying soft tissues may be a clue to the mechanism of injury or to an open fracture. Children with distal femur fractures may hold the knee in a flexed position due to hamstring muscle spasm. There may be varus or valgus knee instability on exam also.

A careful neurovascular exam of the lower limb including pulses, colour, temperature and motor and sensory status should be undertaken. Swelling in the popliteal space may be a sign of a vascular injury which requires urgent orthopaedic intervention. Although rare, injury to the popliteal artery is most commonly associated with an anterior displacement of the epiphysis or a posterior spike at the fracture site. The use of doppler ultrasound may be helpful in evaluating circulation distal to the injury but one of the most important screening tools for a vascular injury is measurement of the ankle-brachial index ratio.

The ankle-brachial index ratio involves comparing differences in systolic BP between the lower and upper limb. The cuff systolic blood pressure of the ankle should be >90% of the arm’s (brachial) systolic blood pressure. If the ankle’s cuff systolic blood pressure is <80-90% of the arm’s cuff systolic pressure, further investigation with a flow doppler ultrasound or arteriogram may be indicated.

If either the ankle-brachial index ratio or clinical exam suggest a vascular injury, then formal imaging with CT angiography should be carried out as soon as possible and repair of any defect to revascularize the distal limb must be undertaken as soon as possible but certainly within 6 hours of the initial injury. Urgent referral to orthopaedics is essential.

Evaluate the patient for signs and symptoms of compartment syndrome.

Assessing for compartment syndrome – the 5 Ps

  • Pain – the most important indicator.  Often diffuse and progressive, not resolved by analgesia, worsened by passive flexion of the injury.
  • Pallor – assess distal to the injury.  Dusky or cool skin (compared to the other side) or delayed capillary return.
  • Pulse – weak or absent pulse indicates poor perfusion,
  • Paralysis – assess active movement of the toes and foot.  This may cause pain, but the purpose is to assess ability to move.
  • Paraesthesia – ask about pins and needles or a feeling of the leg/foot “falling asleep”.  Assess sensation with light touch or using an object such as a pen lid.

Any concerns about potential compartment syndrome must be escalated to an ED or orthopaedic senior without delay as this is a time-critical situation.

Injury to the peroneal nerve can be caused by anterior or medial displacement of the femoral epiphysis. The nerve can become stretched resulting in neurapraxia. Spontaneous recovery can be expected following reduction or fixation of the fracture. The exception to this is a transected nerve in association with an open injury which requires urgent orthopaedic input.

Testing motor and sensory function of the lower limb

Radiology

Most distal femur fractures can be identified on plain X-ray. AP, lateral and oblique views are recommended. Stress radiographs for suspected physeal injury are no longer routinely performed, MRI or ultrasound have replaced stress views in this setting. CT may be necessary for evaluation of intra-articular extension and to define fracture fragments to plan fixation.

Classification

The standard Salter Harris (SH) classification is used to describe distal femoral physeal fractures.

Salter Harris Type I (SHI)

A SHI fracture is a separation through the distal femoral physis. A non-displaced SHI fracture may be difficult to diagnose on X-Ray. A slight fleck of bone adjacent to the physis, a slight widening of the growth plate, or other irregularities of the physis can indicate a SHI fracture of the distal femur. Sometimes diagnosis is only made or confirmed during follow up when subperiosteal new bone formation along the adjacent metaphysis is identified on subsequent X-rays.

Non-displaced SHI fractures should be suspected when knee tenderness is localised circumferentially to the distal femoral physis. To help guide examation it is helpful to know that when the knee is in extension the waist of the patella overlies the distal femoral physis. Gentle varus/valgus stress to the knee may elicit pain.

Patients who have tenderness of the growth plate and are unable to weight bear should be treated as having a presumptive physeal fracture and should be casted and referred to a fracture clinic for follow up. If in doubt, seek advice by discussing your clinical findings with orthopaedics.

Distal femoral epiphyseal widening. Courtesy of Orthobullets.com

Salter Harris Type II (SHII)

Distal femoral physeal fractures are most commonly SHII fractures. This fracture pattern is characterised by an oblique fracture that extends across the metaphysis of the distal femur. The metaphyseal corner that remains attached to the epiphysis is called a Thurston-Holland fragment.

Salter Harris II fracture of the distal femur. From Orthobullets.com

Salter Harris Type III (SHIII)

A SHIII fracture involves the physis and extends vertically through the epiphysis. These injuries can be produced by valgus stress during sports activities and may have an associated injury to the cruciate ligaments.

Salter Harris Type IV (SHIV)

SHIV fractures of the distal femur are uncommon. This injury pattern involves a fracture that extends vertically through the distal femoral metaphysis, physis and exits through the articular surface of the epiphysis.

Salter Harris Type V (SHV)

SHV fractures occur when the physis is crushed. This injury is rare and similar to SHI fractures in that they are often diagnosed retrospectively, when growth disturbance is observed following injury to the knee.

Management

Non-operative

Non-operative management may be considered in the case of non-displaced fractures. The injured leg is immobilised in a long leg cast for 4-6 weeks. Close clinical follow up by orthopaedics is essential to minimise complications.

Operative: closed technique

Closed reduction and percutaneous fixation followed by casting is used in cases of displaced SHI or SHII fractures. Some SHIII and SHIV fractures may be treated in this manner if anatomical reduction can be achieved. Again, the patient is followed closely post-operatively by orthopaedics to monitor for complications.

Operative: open technique

Open reduction internal fixation (ORIF) is indicated in the case of SHIII and SHIV fractures with weightbearing articular involvement or in the case of irreducible SHI or SHII fractures.

Complications

Growth arrest and arthritis

Any physeal fractures of the distal femur can be complicated by growth arrest. SHI and SH II fractures in other areas of the body usually have a low risk of growth arrest but in the case of distal femoral physeal fractures even minimally displaced SHI and SHII type fractures should be followed closely for physeal injury leading to growth arrest.

A complete growth arrest can lead to limb length discrepancies. A partial growth arrest can lead to angular deformities at the knee. SHIII and SHIV fractures that heal with displacement can produce post-traumatic arthritis because of their joint surface involvement. The risk of these complications can be minimised by maintaining anatomical physeal alignment and close follow up following non-operative and operative treatment.

Popliteal artery injury, peroneal nerve palsy and compartment syndrome

Although rare, vascular injuries can be associated with anterior displacement of the epiphysis or a posterior spike at the fracture site. Some studies have suggested that peroneal nerve palsy is observed in approximately 7.3% of distal femoral physeal fractures and compartment syndrome is noted in approximately 1.3% of these injuries.

Classic metaphyseal lesions

While discussing distal femur fracture patterns, it is important to mention classic metaphyseal lesions (CML). These fractures, also known as ‘bucket handle fractures’ or ‘corner fractures’, are highly specific for non-accidental injury (NAI) in children <1 year old. CMLs are most common in the tibia, femur and proximal humerus and can result from shearing forces applied to these long bones, which causes avulsion of the metaphysis. Shearing forces can be produced by holding a child by their trunk and shaking them causing their limbs to move back and forth. Any fracture in a non-mobile child should raise suspicion for NAI but be vigilant for the subtle findings of CMLs on X-ray. If you have concerns regarding NAI, escalate your concerns to the most senior clinician, contact the relevant social work / safeguarding department and discuss fracture management with the orthopaedic team. Refer to local hospital guidelines to give you an idea of what teams the child should be admitted under and what investigations should be carried out. See these DFTB resources related to child safeguarding for more information: skeletal surveys and NAI and safeguarding module facilitator guide.

Metaphyseal corner fracure of the distal femur. Courtesy of Orthobullets.com

The orthopaedic team review Marcus and a CT of his distal femur and knee is ordered to evaluate the degree of intra-articular extension and to define fracture fragments to plan fixation. Following CT he undergoes open reduction and internal fixation (ORIF) of the SH III fracture to distal femur and he is discharged home after a couple of days in a long leg cast. He will be closely monitored in fracture clinic during his recovery to minimise the risk of complications.

References

Amick A. (2019, Oct 7). Non-Accidental Trauma. [NUEM Blog. Expert Commentary by Riney C]. Retrieved from: http://www.nuemblog.com/blog/nonaccidental-trauma.

Ilharreborde, B., Raquillet, C., Morel, E., Fitoussi, F., Bensahel, H., Penneçot, G.F. and Mazda, K. (2006). Long-term prognosis of Salter–Harris type 2 injuries of the distal femoral physis. Journal of Pediatric Orthopaedics B15(6), pp.433-438.

Kareem, S., Shirley, E. and Skaggs, D. (2020). Distal Femoral Physeal Fractures- Pediatrics. Retrieved from: https://www.orthobullets.com/pediatrics/4020/distal-femoral-physeal-fractures–pediatric

McKenna, S.M., Hamilton, S.W. and Barker, S.L. (2013). Salter Harris fractures of the distal femur: Learning points from two cases compared. Journal of investigative medicine high impact case reports1(3), p.2324709613500238.

Price, C.T (2020). Extra-Articular Injuries of the Knee. Retrieved from: https://teachmeorthopedics.info/extra-articular-injuries-of-the-knee/

Wall, E.J. and May, M.M. (2012). Growth plate fractures of the distal femur. Journal of Pediatric Orthopaedics32, pp.S40-S46.

Fibula fractures

Cite this article as:
Shah Rahman. Fibula fractures, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.31860

Romesh, a 6 year old boy, was playing on some monkey bars at school when he slipped, and landed on his legs, and has been unable to weight bear since. The bars were approximately 1m high, and on examination, positive findings include an area of bruising over the lateral aspect of the right lower leg and marked tenderness on palpation.

Incidence

Isolated fibula shaft fractures are rare. More commonly, they are associated with tibia fractures, or with an ankle fracture affecting the distal fibula.

How might the patient present?

History

The mechanism is key to the injury pattern identified:

  • Direct trauma to the lateral aspect of the lower leg resulting in a transverse or comminuted fracture.
  • Twisting injuries producing a spiral fracture.
  • Repeated stress such as in long-distance runners can cause a fatigue fracture, usually just above the inferior tibiofibular ligament. Think of the cross-country running teenager who usually wouldn’t present but has new lower leg pain or antalgic gait

Examination

  • The normal process of look, feel, move is a good step after an initial history. Always examine the knee and the ankle as well as evaluating for other areas of injury. Gait is a useful assessment, as isolated fractures are likely to be treated conservatively.

Investigations

  • X- ray is the initial imaging modality of choice
  • Point of care ultrasound could be used to confirm the presence of a fracture, but given the risk of other associated bony injuries, patients will still require imaging.
  • Patients with complex injuries involving other bones or joints may warrant cross sectional imaging
  • Does the history match the injury – is there a risk for NAI?

Classification

Fibula fractures are classified by fracture type, whether there is an associated tibial fracture, whether they’re displaced or not and whether they’re open or closed,

  • Displacement i.e. 0-50% displaced, >50% displacement with bony contact, or fully displaced
  • Open/Closed
  • Greenstick type patterns can occur
  • Toddler’s fracture (Spiral fracture of the tibia) may uncommonly have an associated fibula fracture

Treatment

  1. Analgesia
  2. Remove significant contaminants from open wounds and administer antibiotics early
  3. Isolated shaft fractures – treat with either a supportive dressing, a cast or a boot
  4. As the fibula is rarely fractured in isolation, the need for surgical management (such as open reduction and internal fixation) if usually dictated by that of any associated tibial fractures

Potential complications

As with any fracture, union issues (delayed, malunion and non-union) is a risk, made worse if there’s infection. Compartment syndrome is a risk, but is more relevant if there is an associated tibial fracture. Be suspicious of an isolated spiral fracture at the proximal fibula; it may be associated with a distal tibia fracture, called a Maisonneuve fracture. These do poorly with conservative treatment, meaning the ankle must be imaged in those with an apparently isolated fracture of the fibula to prevent a missed tibial fracture. Although rare, these can occur in older adolescents with closed physes.

Ensure associated nerves (common peroneal if the fibular neck is fractured), arterial territory (the anterior tibial pulse) and lateral collateral ligament is intact with normal function. The lateral collateral ligament joins the femur and fibula, so whilst not as important as the other collateral ligaments, if damaged, it has a high co-incidence of stiffness or pain in other areas such as knee, ankle and foot can delay full rehabilitation.

Do not miss…

  • Compartment syndrome
  • Other associated fractures – namely at the ankle and tibia
  • Fibular head dislocation – the mechanism is usually a fall on a flexed knee, and can be managed with closed or open reduction.

And a bit of trivia

Some patients can be born without a fibula (fibula hemimelia). This will be picked up on ultrasound screening or on newborn screening, but may be relevant for those patients who haven’t presented to healthcare or have migrated.

Romesh was given loading doses of paracetamol and ibuprofen, as well as intranasal diamorphine. His lower leg x-ray, which also included ankle views, and his right lower leg shows a minimally rotated spiral distal tibia fracture and proximal fibula fracture – a Maisonneuve. He was taken to the emergency trauma list and managed with open reduction and internal fixation.

References

Emergency Care of Minor Trauma in Children, 1st Edition, Davies F

Lecture Notes Orthopaedics and Fractures, 4th Edition, Duckworth T and Blundell CM

Essential Orthopaedics and Trauma, 5th Edition, Dandy DJ and Edwards D

Uterine (decidual) Casts

Cite this article as:
Tara George. Uterine (decidual) Casts, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.32416

Lucy, 15, arrives in the ED sobbing hysterically clutching a wad of toilet paper. “I thought it was my period… only I had the worst period pains ever. I went to bed with a hot water bottle and it got worse and then… this came out”. She sobs, opening the tissue to show you a fleshy, pale triangular thing. approximately 5cm long.  “What is it? It’s disgusting. Have I got cancer? I’m not pregnant am I?”

Bodily secretions in tissues are rarely a source of delight but are common opening gambits. Vomit, faeces, sputum, vaginal discharge, worms, lice, blood clots and products of conception may be saved up and brought to the doctor to add colour to the history.    They present a challenge as often we don’t want to look. We don’t trust ourselves not to recoil or be disturbed and being presented with a “sample” early on can catch us off guard. It plays havoc with the “history, examination, management plan” structure we like to impose on our consultations.  In presentation terms, though, this is a gem of a presentation. We have an “Idea”, a “Concern” and it won’t be long before we elicit an “Expectation”.  Avoiding the enormous cue as it is thrust into your orbit, whilst tempting, risks dismissing the concerns. This can destroy any fledgling rapport and make the whole encounter even harder.  It is going to be necessary to take a history, but right now we have a distressed teenager, an unidentified object in a tissue and a lot of emotion. It may well be easiest to address this gift up front and just take a look. This is the time address the upset and the fear head on.

The “thing” looks like this:

A uterine or decidual cast occurs when the entire endometrial lining is shed in one piece. They are uncommon but frequently cause distress to the patient and can be extremely painful to pass.  A cast looks almost triangular in shape and if shed whole you can see the contours of the uterine cavity in a sort of fleshy model if you look closely.

Lucy tells you she had a Nexplanon contraceptive implant fitted about 6 weeks ago. She is not currently sexually active.  Her last period started the day before she had her implant fitted. She’s well otherwise with no past medical history. She had some light PV spotting yesterday and this morning but it has been light.  Since she passed the mass her pain has settled completely. Her observations are normal. She is happy to do a pregnancy test which is negative.  She just wants to know what it was, why it happened and if she can go home now.

The vast majority of uterine casts have no identifiable precipitating causes though there are case reports in association with Ectopic Pregnancy and they may be slightly more common in users of Hormonal Contraception though having had a cast is not a contraindication for continued use, nor are recurrent casts likely with continued use. The pain associated with passage of the cast is often severe – remember they are passing a 5cm mass through their cervix.

You reassure Lucy that this is not cancer, that she wasn’t pregnant and that this was a cast.  You explain what a cast is and that it is unlikely to recur.  She goes home much reassured and relieved.

You decide to send the cast to the lab for histology and a few days later a report lands in your in-tray which reads extensively decidualized endometrial tissue with minimal glandular structures lined by low cuboidal epithelium, consistent with a uterine or decidual cast. No chorionic villi were identified.

References

Nunes, R.D. and Pissetti, V.C., 2015. Membranous Dysmenorrhea–Case Report. Obstet Gynecol Cases Rev2, p.042.

Strauss, L., 2018. Fleshy Mass Passed Vaginally by a Young Woman. American family physician98(7), pp.449-450.

Following bronchiolitis guidelines

Cite this article as:
Ben Lawton. Following bronchiolitis guidelines, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.32798

In 2016 our friends at PREDICT produced a robust, evidence-based guideline for the management of bronchiolitis. They assembled a diverse team of experts, decided on the key questions we ask ourselves when managing bronchy babies and then did a deep dive of the literature to provide answers to those questions. You can read the guideline here, or the DFTB summary here but the key messages will be familiar to regular readers of DFTB, namely the list of things that do not help babies under 12 months with bronchiolitis includes salbutamol, chest x-ray, antibiotics, nebulised adrenaline and steroids. In the real world, however, these ineffective treatments continue to be used – so what can we do about that? 

The authors of a new PREDICT study released in JAMA Pediatrics on 12 April 2021 sought to demonstrate whether a group of interventions they developed using theories of behaviour change would be effective in reducing the number of ineffective interventions given to bronchiolitic babies. 

Haskell L, Tavender EJ, Wilson CL, et al. Effectiveness of Targeted Interventions on Treatment of Infants With Bronchiolitis: A Randomized Clinical Trial. JAMA Pediatr. Published online April 12, 2021. doi:10.1001/jamapediatrics.2021.0295

Who did they study? 

This was an international multicentre cluster randomised controlled trial (RCT) involving 26 hospitals in Australia and New Zealand. It is described as a “cluster” RCT as randomisation was by hospital rather than by patient. The randomisation was a bit complicated. It was stratified to make sure secondary and tertiary hospitals from each country were represented in each group. Baseline data was collected from 8003 patient records from the three bronchiolitis seasons prior to the start of the intervention period. A further 3727 charts analysed from the season in which the intervention took place. The data from the three prior seasons were used to ensure baseline similarity between groups and to establish patterns of practice change that were already occurring. In short, this was a big study that ensured representation of both specialist children’s hospitals and mixed general hospitals. 

What did they do? 

Hospitals randomised to the intervention group received a package of interventions based on the Theoretical Domains Framework (TDF), developed following an earlier qualitative study that investigated why we do what we do when managing bronchiolitis infants. The TDF is one of the most commonly used frameworks in implementation science and is considered particularly good at identifying interventions to address barriers and facilitators that influence behaviour change. The package included:

  • Appointing clinical leads from medical and nursing streams in both emergency departments and inpatient paediatric units.
  • The study team meeting with those clinical leads to explore the local practice and any anticipated barriers to change.
  • A one day train-the trainer workshop to ensure clinical leads were comfortable using the educational materials provided to train local staff.
  • An education pack including a PowerPoint with scripted messages specifically designed to promote change, a clinician training video, evidence fact sheets, promotional materials and parent/caregiver information sheets.
  • Monthly audits of the first 20 bronchiolitis patients with the results shared and compared to the best performing hospital.

What about the control group?

Hospitals randomised to the control group were just left to their own devices for the year of the intervention period. They had access to the guidelines and were welcome to share that information as they would in any other circumstances. The intervention package was made available to control hospitals in the season following the study period. 

What did they show? 

The primary outcome was the proportion of infants who complied with all five of the Australasian Bronchiolitis Guideline recommendations known to have no benefit (chest x-ray, salbutamol, steroids, adrenaline, antibiotics). There was an 85.1% compliance rate in the intervention group compared to a 73% compliance rate in the control group. In other words, in hospitals that were part of the intervention group, an average of 85.1% of kids received care in line with the guidelines, compared to only 73% receiving guideline compliant care in control hospitals. This was a significant difference.

Secondary outcomes showed improvement was consistent in both the ED and inpatient phases of care. Unsurprisingly, there was no difference in hospital length of stay or admission rates to ICU. 

The DFTB verdict

On the surface this is a robust, well designed study showing that if we put some thought and some resources into supporting our colleagues in doing the right thing then babies with bronchiolitis will get better care in our hospitals. They won’t leave hospital any quicker and they won’t have a lesser chance of needing ICU but they will be exposed to fewer interventions that will not do them any good and may do them some harm. Dig a little deeper though and the big messages in this paper go way beyond the management of bronchiolitis. The implementation science based interventions used in this study can be adapted to anything, and though they have been shown to be effective in getting us to do the right thing here, we haven’t shown that their efficiency has been optimised yet. Great breakthroughs in novel medical science are exciting but there are huge improvements in care to be gained through getting the best care that we do know about to every patient every time. This paper should serve as fuel for the fires lighting implementation science’s journey from the shadows to the centre stage of improvement in clinical care. 

From the authors

The study’s senior author, Prof Stuart Dalziel gave DFTB the following take: 

“The key finding is that we can do better. By using targeted interventions, based on established behaviour change theories and developed from work looking at why clinicians manage patients with bronchiolitis the way they do, we can improve the management of patients with bronchiolitis such that it is more consistent with evidence based guidelines.

In the field of implementation science (IS) and knowledge translation (KT) a 14% improvement in care is a large change.

Changing clinician behaviour is complicated, this is especially so for de-implementation of medical interventions. Many factors influence clinician behaviour and it is thus perhaps naïve to think that a single intervention can cause a significant change to behaviour. For a number of decades the majority of clinical guidelines for bronchiolitis have emphasised that chest x-ray, antibiotics, epinephrine, corticosteroids and salbutamol are low-value care and not evidence based. Yet despite this consistent messaging from guidelines the use of these interventions has remained considerably higher than what it should be. While the interventions delivered in our study were not unique (site based clinical leads, stake holder meetings, train-the-trainer workshops, targeted clinical education, educational material, and audit and feedback) they were specifically developed, using an established framework for behavioural change, following a qualitative study that determined why clinicians managed bronchiolitis they way they do. This prior study, addressing the barriers and enablers to evidence based care, and the subsequent step wise approach to developing the targeted interventions that we used was critical in achieving the change in clinician behaviour observed in our randomised controlled trial”.

The study’s lead author, Libby Haskell, stated:

“Bronchiolitis is the most common reason for children less than one year of age to be admitted to hospital. We can improve the care of these infants, such that they are receiving less low-value care. In order to de-implement low-value care we need to first understand barriers and enablers of care, and then develop targeted interventions, built on robust behavioural change models, to address these. This approach can be used to improve care for other high volume conditions where we see considerable clinical variation in care and with clearly established clinical guidelines on appropriate management.”

Let us know what you think in the comments below 

Foot and toe injuries

Cite this article as:
Taskin Kadri. Foot and toe injuries, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.32663

A child’s foot is significantly more cartilaginous than an adult foot, making foot fractures an uncommon injury in children. Foot fractures constitute about 5-8% of paediatric fractures. Evaluation and management of a paediatric foot injury requires an understanding of paediatric anatomy, a careful history, clinical examination, and potentially radiography.

Normal anatomy

The foot is anatomically divided into 3 sections: hindfoot, midfoot and forefoot.

The hindfoot includes the talus and calcaneus. The inferior surface of the talus is more prone to avascular necrosis due to its retrograde blood supply. The part of the calcaneus most prone to fracture is its large posterior facet.

The midfoot includes the navicular, cuboid and the three cuneiform bones.

The forefoot includes the metatarsals and phalanges.

Evaluation of foot injuries

History

The following specific enquiries should be made about the injury:

  • The mechanism of injury (high/low velocity, twisting, compression, direct blow)
  • Characteristics of pain (worse at the time of injury vs late onset)
  • Location of pain
  • Consistency and plausibility of the history, excluding concerns about non-accidental injury
  • The effect of the injury on the child (limping, the distance the child can move)
  • The efficacy of pain relief

Examination

Clinical examination should be tailored to the history.

Look

For external skin abrasions or obvious open fractures.

Feel

Palpation of the bones: tarsals, metatarsals, toes and the base of the 5th metatarsal.

Palpation for tenderness along the ligaments: deltoid ligament on medial side and anterior talofibular, calcaneofibular and posterior talofibular ligaments on the lateral side.

Move

The active movement should be followed by passive movement as much as pain allows.

The usual range of movements are: subtalar eversion (15-20°), subtalar inversion (35-40°), forefoot adduction (20°), forefoot abduction (10°), 1st metatarsal phalangeal (MTP) flexion (45°), 1st MTP extension (70-90°) and free motion of lesser toes.

Neurovascular examination

Two pulses: dorsalis pedis and posterior tibial.

Five sensory nerves: saphenous (medial calf and hindfoot), superficial peroneal (dorsum of the foot), deep peroneal (1st dorsal webspace), sural (lateral foot) and posterior tibial nerve (plantar foot and heel).

Imaging

The Ottawa foot rules can be applied to children. The rules have 97-100% sensitivity in paediatric injury investigation. According to the rules, x-rays of the foot are required if the child is unable to weight bear both immediately after the injury and in the ED, plus bony tenderness over the base of 5th metatarsal or the navicular.  

Accessory ossicles

Accessory foot ossicles can cause pain as a result of stress injuries. The navicular ossicles, arise either on the medial side of (os tibiale externum) or on the lateral tubercle of the navicular bone (os trigonum).

Os tibiale externum

This is an ossification centre that arises at the site of the tibialis posterior tendon on the medial side of the navicular bone. It becomes an accessory bone when it fails to fully ossify.  It is present in 4-14% of patients.

The usual presentation is in adolescence when the patient has pain from overuse, especially in athletes.

Os tibiale externum

Examination may reveal pes planus (flat foot). This happens as the tibialis posterior tendon, maintaining the medial longitudinal arch, attaches to the accessory bone rather than the navicular bone.

Investigations include plain films and, occasionally, MRI scan.  

Treatment is initially conservative with orthotics and casting. The ossicle is excised if these measures fail to resolve the pain.

Os trigonum

This is present in 10-25% of the population. It is often associated with heel pain in ballet dancers due to repetitive microtrauma.

Hindfoot fractures

These are rare, constituting only 0.008% of all paediatric fractures. Children usually present after falling from height or after a motor vehicle injury. The talus can be fractured in multiple places including avulsion fracture.

Case courtesy of Assoc Prof Craig Hacking, Radiopaedia.org. From the case rID: 77140

‘Snowboarder’s fracture’ is a fracture of the lateral process of the talus. The mechanism of injury involves dorsiflexion and inversion.

Do not miss: Snowboarder’s fractures are often misdiagnosed as an ankle sprain. If not evident on x-ray, a CT or MRI should be performed if there is clinical suspicion. Think carefully about the mechanism.

Calcaneus fractures typically occur due to axial loading and are frequently associated with vertebral compression fractures. Radiography should include AP, lateral and axial views. The axial view offers a better view of the fracture.

Treatment of talus and calcaneus fractures is dependent on the degree of displacement.

  • Non or minimally displaced avulsion fractures, or extra-articular fractures should be managed in a posterior short leg splint and should be non–weight bearing with crutches for 3 to 4 weeks.
  • Displaced fractures require reduction, by an orthopaedic surgeon, followed by immobilisation with a splint or cast. Severely displaced, comminuted or intra-articular fragments may require ORIF.

Midfoot fractures

These are also rare fractures and usually result from severe blunt injury. Most fractures are avulsion or stress fractures and are associated with other injuries.

Treatment depends on the severity of displacement of the fracture and associated injuries.

  • Non or minimally displaced fractures (the majority of these fractures) can be treated with a walking cast.
  • Displacement of the fracture fragment greater than 2mm should be managed with reduction and stabilisation before immobilisation.

Midfoot fractures have minimal long term sequelae.

Forefoot fractures

Forefoot fractures represent about 60% of paediatric foot fractures and can result from either direct or indirect trauma. They are easily missed: about 41% are missed due to high energy trauma causing other significant injuries.

Lisfranc fracture

Lisfranc fractures occur due to axial loading with forced plantar flexion (commonly seen in bicycle or horseback riders where a foot gets caught in a pedal or stirrup) or with a crush injury.

Clinical examination

  • Tenderness over the dorsum of the foot with swelling and inability to bear weight.
  • Plantar bruising is a consistent sign and if present should raise suspicion of the injury.  

Diagnosis is made with a weight bearing x-rays (as the fracture may not be evident on non-weight bearing views) – AP, lateral and oblique radiographs of the foot. The normal alignment of the foot should have the 2nd metatarsal aligning with the intermediate cuneiform on the dorsoplantar view and the 3rd metatarsal aligning with the lateral cuneiform on the oblique view. The Lisfranc ligament connects the cuneiforms to the 2nd metatarsal. Disruption of this ligament leaves the foot unstable and hence it is an important not to miss this injury. Due to the ligament attachment, there is often associated fracture of the bases of the 1st or 2nd metatarsal in a Lisfranc injury.

https://emergencymedicineireland.com/

Treatment depends on the degree of severity of the injury.

  • Partial tears with < 2mm malalignment require immobilisation or a walking boot for 4-6 weeks. The patient should be referred to orthopaedic surgeons within 3-5 days due to the high rate of late complications.
  • More severe injuries require operative treatment with internal fixation.

There is a high rate of residual pain in children with a Lisfranc injury.

Metatarsal fractures

Metatarsal fractures are associated with athletic activity and are becoming more common. The 5th metatarsal is the most commonly fractured metatarsal in paediatric patients. It can result from twisting, repetitive stress or direct trauma. 1st and 5th metatarsal fractures can be isolated whereas 2nd-4th metatarsal fractures often occur along with other metatarsal fractures. It is a frequently missed fracture on a radiograph.

Children younger than 5 years of age are more likely to be injured by a fall from height and fracture the 1st metatarsal. Older children are more likely to fracture it from falling from a standing position, during sports and tend to fracture the 5th metatarsal.

Pseudo-Jones fracture

A Pseudo-Jones fracture is an avulsion fracture of the base of the 5th metatarsal resulting from a twisting injury of the foot. The examination will reveal focal point tenderness. The patient should be immobilised for 3-4 weeks in a weight-bearing cast.

Treat with a short walking boot or hard sole shoe for 6 weeks. Follow-up with orthopaedic surgeons.

Jones fracture

The Jones fracture is a fracture of the metaphyseal-diaphyseal junction at the base of the 5th metatarsal bone. It is the most common of metatarsal fractures (40%), representing about 25% of all paediatric foot fractures.

Jones fracture (metaphyseal-diaphyseal junction fractures of the 5th metatarsal).

Fractures at or distal to the metaphyseal-diaphyseal junction require 6 weeks in a non–weight bearing cast, with crutches. All patients should be referred to orthopaedic surgeons as there is a high incidence of delayed union of the fracture. Many of these patients will require ORIF subsequently.

The apophysis of the base of the 5th metatarsal appears at age 10 for girls and at age 12 for boys. An unfused apophysis runs longitudinally whereas pseudo-Jones fracture runs transversely.

Normal apophysis of the 5th metarasal (note it runs longitudinally rather than transversely). Case courtesy of Dr Jeremy Jones, Radiopaedia.org. From the case rID: 8802

Toe fractures

Toes fractures are one of the most common fractures in the paediatric population. Phalangeal fractures constitute about 3-7% of all physeal fractures and are usually Salter-Harris I or II injuries. They are more common in boys than girls and are mostly closed in nature.

The patient may present with localised tenderness to the toe, a limp or inability to bear weight. Nail bed bleeding and bleeding from or around the nail fold should prompt the possibility of an open fracture through the nail bed. Alignment, rotation and neurovascular status should be checked.

Fractures of the 2nd-5th toes are usually treated by buddy strapping and weight bearing as much as possible. Healing can take up to 3-4 weeks. A hard-soled shoe or walking boot may be used for patient comfort. Follow-ups with orthopaedic surgeons can cease 3 weeks after the injury. If there is possible injury to physis then follow-up should continue for 1-2 years to detect abnormal growth.

The big toe

The big toe plays an important part in bearing weight. Fractures of the big toe are therefore managed slightly differently.  Salter-Harris III or IV fractures of the proximal phalanx of the hallux are often intra-articular.

Urgent orthopaedic consultation for closed or open reduction for K wiring if:

  • more than one third of the joint surface is involved or
  • displacement is more than 2-3mm

In other cases, toe platform cast or a walking boot is used.

The epiphysis of the proximal phalanx of the 1st toe is sometimes bipartite, simulating a Salter-Harris III fracture. If there is no tenderness on the 1st toe, no treatment is indicated.

Phalangeal open fractures require thorough irrigation and debridement in addition to antibiotics to avoid osteomyelitis.  A nail-bed injury to the germinal matrix will require surgical repair.

Long term complications include growth arrest and angular deformities from physeal injury, degenerative joint disease from intra-articular fractures and osteomyelitis from open fractures.

References

Boutis K: Paediatric metatarsal and toe fractures. Up to Date 2019

Boutis, K., 2021. UpToDate. [online] Uptodate.com. Available at: <https://www.uptodate.com/contents/foot-fractures-other-than-metatarsal-or-phalangeal-in-children> [Accessed 4 April 2021].

Boutis, K., 2021. UpToDate. [online] Uptodate.com. Available at: <https://www.uptodate.com/contents/foot-fractures-other-than-metatarsal-or-phalangeal-in-children> [Accessed 4 April 2021].

Eiff, M. and Hatch, R. Fracture management for primary care and emergency medicine. Elsevier.

Halai, M., Jamal, B., Rea, P., Qureshi, M. and Pillai, A., 2015. Acute fractures of the pediatric foot and ankle. World Journal of Pediatrics, 11(1), pp.14-20.

Horner K and Tavarez M, 2016. Paediatric Ankle and Foot Injuries. Clin Pediatr Emerg Med, 17 pp. 38-52

Juliano, P., 2018. Lateral Talar Process Fractures – FootEducation. [online] FootEducation. Available at: <https://footeducation.com/lateral-talar-process-fractures/> [Accessed 4 April 2021].

Malanga, G. and Ramirez – Del Toro, J., 2008. Common Injuries of the Foot and Ankle in the Child and Adolescent Athlete. Physical Medicine and Rehabilitation Clinics of North America, 19(2), pp.347-371.

Metaizeau, J. and Denis, D., 2019. Update on leg fractures in paediatric patients. Orthopaedics & Traumatology: Surgery & Research, 105(1), pp.S143-S151.

Smit, K. Foot Fractures – Phalanx | Pediatric Orthopaedic Society of North America (POSNA). [online] Posna.org. Available at: <https://posna.org/Physician-Education/Study-Guide/Foot-Fractures-Phalanx> [Accessed 4 April 2021].

Shoulder examination

Cite this article as:
Mark Webb. Shoulder examination, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.31287

Johnny is five. He fell onto his outstretched arm and now is sat in your ED, crying and holding his shoulder adducted. Triage has been ace and given him analgesia so he is adequately comfortable before you examine him.

Joint examinations can be easily remembered by “look, feel, move” and special tests. It’s important that in addition to the joint you’re interested in that you also examine the joint above and below.

Look

  • Deformity
  • Swelling
  • Atrophy: Asymmetry  
  • Wounds
  • Bruising
  • Skin tenting (typically clavicular fractures, whereby the bony fragment is causing pressure on the skin and thought to cause skin necrosis, although this is controversial) 

A chaperone may be needed to expose the joint adequately in older children.

Feel

Feel for warmth, which could indicate septic arthritis.

From the front:

Start medially at sternoclavicular joint

Anatomy of the acromio-clavicular joint

From the back:

  • Scapula: spine, supraspinatus, infraspinatus muscle

Neurovascular assessment:

  • Check for distal pulses: brachial/ radial.
  • Always check the regimental patch for axillary nerve injury and document it.

Move

Assess for range of motion, both active and passive.

Girl flexing and extending at shoulder showing range of movement

Flexion: 180 degrees. Raise arm forward up until they point to the ceiling.

Extension: 45-60 degrees. Stretch the arm out behind them.

Girl showing range of adduction and abduction at the shoulder

ABduction: 150-160 degrees. Put arms out to the side like an aeroplane’s wings and then bring them above their head to point to the ceiling.

ADduction: 30-40 degrees. Put arms out to the side like an aeroplane’s wings and move them in front of their body so they cross over.

Girl showing range of internal and external rotation

External rotation: 90 degrees. Tuck their elbows to their side and swing the hands out.

Internal rotation: 70-90 degrees. Tuck elbows to the side and bring their hands across their tummy.

Scapula winging: Ask the child to push against the wall or your hand. If the scapula wings out this suggests long thoracic nerve pathology.

Some special tests

It is easy to get lost in the number of special tests when examining the shoulder and the trick is to perform those most relevant to the patient in front of you. Many are to test the integrity of the rotator cuff tendons, i.e. Supraspinatus, Infraspinatus, Teres minor and Subscapularis. (SITS)

Girl performing Apley scratch test

“Appley Scratch” test: (1) Ask the child to reach behind their back to touch the inferior border of the opposite scapula (internal rotation and aDDuction) and then (2) reach behind their head to touch the superior angle of the opposite scapula (external rotation an Abduction). A positive test of pain indicates tendinitis of the rotator cuff, usually supraspinatus.

Girl performing empty can test

Empty can test: Ask the child to hold their arm raised parallel to the ground and then point their thumbs towards the ground as if they were holding an empty can (this rotates the shoulder in full internal rotation while in abduction). Then push down on the child’s wrist while asking them to resist. A positive test is pain or weakness, suggestive of supraspinatus tear or suprascapular nerve neuropathy.

Girl performing lift off test

Lift off test: The child stands and places the back of their hand against their back. Put your hand against theirs, palm to palm, and ask them to push against you. A positive test is pain or weakness, indicating subscapularis muscle pathology.

Girl and boy performing scarf test

Scarf test: Ask the child to wrap their arm over the front of their neck reach down over their opposite shoulder towards the scapula (like a scarf). Pain over ACJ when doing this indicates ACJ pathology.

Although the standard approach to limb examination involves a LOOK, FEEL and MOVE (and special tests) structured assessment, in reality, if a young patient has a significant injury, a more pragmatic approach is needed. An X-ray may be warranted before a more thorough exam. This doesn’t mean that you can get away without a documented range of motion exam (even if you explain it is limited by pain) and neurovascular assessment.

Back to Johnny. You noticed a deformity over the middle third of the clavicle, but no skin tenting. He was neurovascularly intact and range of movement only marginally reduced by pain, so you discharged him with a broad arm sling and follow-up (or not) according to your local guidelines.

Selected references

Carson, S., Woolridge, D.P., Colletti, J. and Kilgore, K. (2006) Pediatric upper extremity injuries. Pediatric Clinical North American: 53(1) pp. 41-67

Chambers, P.N., Van Thiel, G.S. and Ferry, S.T. (2015) Clavicle Fracture more than a theoretical risk? A report of 2 Adolescent cases. The American Journal of Orthopedics. 44(10) 

https://fpnotebook.com/Ortho/Exam/ShldrExm.htm [Accessed April 2019]

McFarland, E.G., Garzon-Muvdi, J., Jia, X., Desai, P. and Petersen, S.A. (2010) Clinical and diagnostic tests for shoulder disorders: a critical review. British Journal of Sports Medicine. 44(5) pp. 328-32.

NationwideChildrens.org/Sports-Medicine

https://shouldercomplexgocatsnmu.weebly.com/range-of-motion.html [Accessed April 2019]

Proximal humeral fractures

Cite this article as:
PJ Whooley. Proximal humeral fractures, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.31855

“Slow down!” Joel’s mom shouts at him as he whizzes past on his scooter. Joel turns to answer and doesn’t see the curb which he hits, goes flying and puts out his hand to stop himself. Mom is already running to find him holding his arm.

Proximal humeral fractures are uncommon, accounting for less than 5% of all paediatric fractures. The anatomic characteristics of the proximal humerus can explain the various fracture presentations, complications and outcomes.

Anatomy

Ossification centres

The proximal humeral physis has three ossification centres. Head, lesser tuberosity and greater tuberosity. The capital centres appear at 3 months whereas the two others appear at 1 year of age and fuse between 3 & 5 years to produce tuberosity ossification. By the time the child’s 6, the capital and tuberosity centres fuse into a single proximal epiphyseal centre. At this point it acquires a characteristic ‘tent’ or inverted V shape. This results also in a double contour that can complicate the interpretation of the images. The proximal physis accounts for approximately 80% of the longitudinal growth of the entire bone.

Periosteum

This thick sleeve of periosteum is present along the shaft and limits fracture displacement and promotes healing.

Nerves

The axillary nerve, which supplies the sensory innervation to the regimental badge area, is at potential risk in displaced proximal humeral fractures. However, axillary nerve damage is rare, the majority being only a temporary neuropraxias.

Epidemiology

Proximal humeral fractures show an early modest peak at 10-14 years of age, account for up to 3.5% of all fractures, followed by return to low levels in young adults and then a second increase in later adulthood.

These fractures account for a third of all humeral fractures in neonates and are the second most common birth injury after clavicular fractures.  However, they are still rare, occurring in only 0.03 per 1000 births.

The pattern of injury varies with age. Metaphyseal proximal humeral fractures are proportionately higher in pre-pubertal children, compared to a higher proportion of epiphyseal separation in adolescents.

As with any fracture, be aware of the potential of a non-accidental cause. These fractures, can be associated with physical abuse.

Mechanism of Injury

Indirect

Proximal humeral fractures in non-newborns commonly result from a fall backwards onto an outstretched hand with elbow extended and wrist dorsiflexed or a direct blow to the lateral aspect of the shoulder. Pathological fractures  can occur as the humerus is a common location of bone cysts and other benign lesions. This might occur with minimal trauma.

Birth injuries

The upper arm can be hyperextended or rotated during delivery, occurring more commonly in infants of diabetic mothers and with shoulder dystocia.

Clinical Evaluation

Newborns

Typically, a newborn with a proximal humeral fracture will hold their arm in extension. Consider these fractures if a history of birth trauma is given. If noted, then infection, clavicle fracture, shoulder dislocation and brachial plexus injuries need to be ruled out. These little ones may be irritable, particularly when the upper limb is moved.

Children and adolescents

As with other upper arm fractures, the typical presentation is with pain, dysfunction, bruising and swelling with a painful range of movement. Displaced fractures result in significant anterior swelling and altered shoulder appearance relative to the contralateral side.

A detailed distal neurovascular examination is needed including evaluation of the radial, ulnar, median, axillary and musculoskeletal nerves. Be particularly vigilant for any axillary nerve deficit with decreased sensation over the regimental badge area and loss of the deltoid muscle function (shoulder ABDuction).

Radiology

Proximal humeral fractures are identified on routine AP and axillary views of the humerus. If there is clinical concern of a dislocation then dedicated shoulder views should also be taken. If there is tenderness over the physis and no obvious fracture, a suspicion of a Salter Harris (SH) I fracture can be made. Imaging the contralateral humerus may be helpful to determine if there is any widening of the physis.

Patterns of fracture

There are two variations of proximal humeral fractures: metaphyseal and epiphyseal separation.

  • Metaphyseal fractures (70%) usually occur at the surgical neck, although can also occur at the metaphyseal-diaphyseal junction, typically a transverse or short oblique fracture. These fractures typically occur in 5-12 year olds.
  • Epiphyseal fractures (30%) occur in the under 5s and over 12s. The type of epiphyseal fracture depends on skeletal maturity.

SH I fractures are less common and can be seen at all ages before growth-plate closure, most commonly in <5 year olds.

SH II fractures are the most common type, chiefly in adolescents over the age of 12.

SH III & IV fractures are exceedingly rare.

A metaphyseal fracture at the surgical neck of the humerus
Epiphyseal Salter Harris II fracture of the proximal humerus in a 6 year old

Pathological fractures

40% of pathological fractures involve the proximal humerus. The leading cause is a unicameral bone cyst, as this lesion develops in the proximal humerus in 51% of cases. Other causes are aneurysmal bone cysts, non-ossifying fibromas, fibrous dysplasia and bone malignancies.

Displacement

If a proximal humeral fracture displaces, it usually does so in a varus direction, with the humeral head moving medially and posterior to the shaft. This occurs due to pectoralis major traction pulling the distal segment medially, while the rotator cuff and deltoid pull the proximal component superiorly in a tendency towards flexion and external rotation. Displacement is often absent or minimal in 40% of metaphyseal fractures, but is more common in epiphyseal injuries, occurring in up to 85%.

Proximal humerus fracture in an 11 year old with varus deformity

Classification

The Neer-Horowitz classification is the most frequently used classification system for this type of fracture. It divides the proximal humerus into 4 parts, classifying fracture by the degree of displacement as well as the fracture line, consisting of:

  1. Humeral head
  2. Greater tuberosity
  3. Lesser tuberosity
  4. Humeral shaft

One-part fractures involve 1 – 4 undisplaced parts (<1cm AND <45 degrees)

Two-part fractures account for 20% of proximal humeral fractures, involving 2 – 4 parts, 1 of which is displaced (i.e. >1cm OR >45 degrees)

  1. Surgical neck – most common
  2. Greater tuberosity – often seen with anterior shoulder dislocation. A lower threshold for displacement (>5 mm) has been proposed.
  3. Anatomical neck
  4. Lesser tuberosity

Three-part fractures account for 5% of proximal humeral fractures and involve 3 – 4 parts, 2 of which are displaced (i.e. > 1cm OR > 45 degrees)

  1. Greater tuberosity and shaft displaced with respect to lesser tuberosity and articular surface which remain together.
  2. Lesser tuberosity and shaft are displaced with respect to the greater tuberosity and articular surface which remain together.

Four-part fractures are uncommon, occurring in less than 1% of proximal humeral fractures. They involve more than 4 parts, 3 of which are displaced (i.e. >1 cm OR > 45 degrees with respect to the 4th). Four-part fractures require operative reduction.

Management

Initial treatment

Displaced fractures can be very painful so ensure pain is addressed with adequate analgesia.

The aim of immobilisation is to keep the elbow by the side, flexed to 90 degrees with the forearm against the torso. A simple sling is sufficient plus / minus a swathe for younger ages. Straps and adhesive tape can be used as described by Durrajer. Other options include a shoulder immobiliser or a U-shaped coaptation splint.

Neurovascular status must be checked before and after immobilisation.

Orthopaedic consultation should be obtained if there is:

  • associated shoulder dislocation
  • intra-articular (SH IV) fracture
  • completely displaced fracture in a child over 12 years.
  • associated neurovascular compromise
  • open fracture (rare)
  • evidence of compartment syndrome

Definitive Treatment

Newborns usually have SH I fractures, which have an excellent prognosis. A sling and a swathe is sufficient for up to 4 weeks. The primary role of follow up is to ensure there is no brachial plexus injury.

Children and adolescents with minimally displaced fractures are usually managed with a sling or shoulder immobiliser. Gentle pendulum exercise is started between weeks 2 to 4 post injury and active range of movement at 4 to 6 weeks. We would expect near to normal function by 2 months.

Significantly displaced fractures in children 12 and under should be treated with a U-slab, sling and swathe.

Acceptable angulation

  • < 5 years – any degree is allowed as proximal humeral fractures in young children have excellent remodelling potential.         
  • 5 to 12 years – 40 to 70 degrees of angulation is acceptable
  • >12 years – up to 40 degrees of angulation or 2/3 displacement.

Operative

Fractures in which immobilisation would result in unacceptable alignment are managed with closed reduction +/- fixation. Open reduction and internal fixation (ORIF) is indicated if acceptable reduction is not possible due to soft tissue interposition. Most commonly this is caused by the long head of biceps tendon, but can also be caused by the joint capsule, infolded periosteum and deltoid muscle. ORIF is also indicated in open fractures, compound fractures and intra-articular displacement of the fracture.

Complications

Complicated are rare in children, but when do occur are more common in older children, with shortening of the humerus due to physeal damage. This usually has no functional affect. Radiographic malunion can occur but rarely has any functional affect.

Non accidental injury

And finally, as with any fracture, it is imperative that a mechanism inconsistent with an injury or fracture in an otherwise healthy child should prompt escalation and involve the child protection team.

Joel’s x-ray shows a proximal humeral fracture through the surgical neck, with 20 degrees of angulation. He’s placed in a shoulder immobiliser and followed-up in fracture clinic, where he’s advised to start gentle pendulum exercises after a couple of weeks. Two months later he’s back on his scooter, helmet on, flying down the pavement without a care in the world.

References

LA. Landin. Epidemiology of the children’s fractures. J Pediatric Orthop B. 1997;6(2):79

E.J. Ortiz, M.H. Isler, J.E. Navia, R. Canosa, Pathologic fractures in children. Clin Orthop Relat Res, 432 (2005), pp. 116-126

MW Shrader et al, Proximal humerus and humeral shaft fractures in children. Hand Clin 2007;23(4);431

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