PEM Adventures Chapter 1

Cite this article as:
Dani Hall, Rachael Mitchell and Sarah Davies. PEM Adventures Chapter 1, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.29589

Stories are a powerful vehicle for education. Combine a story with some active participation and you have the recipe for some great learning. And so, it’s with great delight, that we bring you Chapter 1 of PEM Adventures. First presented at EuSEM 2018 and then again with some spectacular twists by Dan Lumsden, Paediatric Neurologist extraordinaire, Dani has a particular soft spot for Tomas, a little boy who dreams of being a footballer. Join us on a journey (with an inbuilt time travel machine) in managing Tomas, a little boy with a dream…

Meet Tomas, an 8-year-old boy who dreams of playing professional football. He’s been completely well until an ill-fated shopping trip for some new football boots. At 2 o’clock hours, while trying to persuade his mother that he definitely did need the new Premier League football to add to the collection, he developed sudden onset right-sided facial drooping. His mum bundled him into the car and drove him directly to your ED. You look at your watch: it’s now 3.30 pm.

Your assessment is as follows: Tomas is alert and he seems orientated. He has right sided facial weakness and weakness of both his right arm and leg. He has no obvious sensory changes but is struggling to communicate with you as he has global aphasia.

Suspecting the worse, you have a critical decision to make. But what are you going to do?

You bleep neurology.

And wait…

They don’t answer so you bleep again…

But they still don’t answer…

You bleep a third time…

But they still don’t answer. So you give up and call radiology instead.

You call radiology. And they ask… What imaging do you want?

The radiologist says, “Sure, imaging sounds like a good idea. Let’s do a combination of both a CT brain with CTA to look for blood and clots.

The CT scanner is available at 16:00.

But,” she adds, “if you’d like MR imaging, we could do that at 18:00.

Do you…

Tomas’ CT and CTA shows evidence of an arterial ischaemic stroke with thrombus occluding the middle cerebral artery. There is no intracranial haemorrhage.

It is now 16:15, 2 ¼ hours after the onset of Tomas’ symptoms.

You’re doing great. Close the toggles and move on to the next part of Tomas’ case.

Tomas has an MRI and MRA.

It shows an arterial ischaemic stroke with thrombus occluding the middle cerebral artery.

It is now 18:45, almost 5 hours after symptom onset – something tells you this is a bit too late.

Luckily for you, the inbuilt time machine whizzes you back to make that last decision again.

This time when you’re told you can have an MR and MRA at 18:00 or CT and CTA now you say… “I’ll have a CT and CTA now please.”

The radiologist says “Sure, imaging like a good idea. Let’s do a combination of both an MRI plus MRA to check the brain and look for clots.”

You phone MR. They say they can do the MR at 18:00. The CT scanner, however, is free now.

Do you…

Tomas has an MRI and MRA. It shows an arterial ischaemic stroke with thrombus occluding the middle cerebral artery. It is now 18:45, almost 5 hours after symptom onset – something tells you this is a bit too late.

Let’s travel back in time…

This time when you’re told you can have an MR and MRA at 18:00 or CT and CTA now you say… “I’ll have a CT and CTA now please.”

Tomas has a CT and CTA. It shows evidence of an arterial ischaemic stroke with thrombus occluding the middle cerebral artery. There is no intracranial haemorrhage.

It is now 16:15, 2 ¼ hours after the onset of Tomas’ symptoms.

You’re doing great. Close the toggles and move on to the next part of Tomas’ case.

With a little luck, Tomas has now had neuroimaging and you know he’s had an arterial ischaemic stroke with thrombus occluding the middle cerebral artery without intracranial haemorrhage.

So, what now? You haven’t managed to get hold of a neurologist for love nor money. So do you…

Tomas has supportive care.

Despite physio, OT and lots of assistance at the best neuro-rehabilitation centre, Tomas has a persistent hemiparesis.

You spend your life wishing you’d treated his stroke differently.

So let’s try that choice again.

You prescribe 5mg/kg aspirin.

Tomas has repeat imaging with an MRI and MRA 24 hours later.  His clot has not increased in size but the original clot remains in the middle cerebral artery.

Tomas has a persistent hemiparesis.

He becomes a demon-swimmer and wins Gold in the 2028 Paralympics

However, you spend your life wishing you’d treated his stroke differently.

Why don’t you try that choice again.

You prescribe heparin.

24 hours later Tomas deteriorates, dropping his GCS to 6

Repeat neuroimaging shows a large haemorrhage in the infarcted territory with significant pressure effect.

Let’s go back in time and try that choice again.

You decide to thrombolyse. Tomas will need to go to PICU after thrombolysis but there isn’t a PICU at your hospital.

Do you…

You opt for thrombolysis at the regional centre but will you…

You work fast to mobilise your anaesthetist, ED nurse and emergency kit as quickly as possible.

Tomas arrives at the regional centre at 19:15, 5 ¼ hours after the onset of his symptoms.

It is too late to thrombolyse.

Let’s hop in the time travel machine and go back in time to decide whether to transfer for thrombolysis or thrombolyse in your ED (Hint: you may want to thrombolyse in your own ED as the clock is ticking…)

The retrieval team are mobilised. They collect Tomas from your ED and deliver him safely to the regional centre at 19:15, 5 ¼ hours after the onset of Tomas’ symptoms.

But it’s now too late to thrombolyse.

Let’s hop in the time travel machine and go back in time to decide whether to transfer for thrombolysis or thrombolyse in your ED (Hint: you may want to thrombolyse in your own ED as the clock is ticking…)

Time is critical,” you think to yourself, and tell the team you’re going to thrombolyse in resus.

Tomas is thrombolysed with tissue plasminogen activator (tPA for short) at 18:00, 4 hours after onset of symptoms. His symptoms start to improve.

After intensive neuro-rehab he has no residual neurological deficit.

He grows up to become a professional football player for Bayern Munich, scoring a hat-trick to win the 2028 UEFA champion’s league.

Congratulations! You successfully treated a stroke in childhood. Now close the toggles and read on…

Although you opt for angiographic thrombectomy, the interventional neuroradiologist is on study leave and no-one is able to cover.

You suspect they are actually scared of children.

Either way Tomas can’t have the clot removed. You’re going to have to choose again.

You phone the neurosurgeons and ask them to do a hemicraniectomy.

They ask you to go through all his neurology and review his imaging.

They say “Sorry, but his PedNIHSS isn’t high enough for us to take to theatre.”

You think, “PedNIHSS?” and make a mental note to look it up later.

Let’s try that choice again.

After your shift you do a quick google search to look at the evidence around using tPA in children and you stumble across this paper:

Rivkin, M.J., deVeber, G., Ichord, R.N., Kirton, A., Chan, A.K., Hovinga, C.A., Gill, J.C., Szabo, A., Hill, M.D., Scholz, K. and Amlie-Lefond, C., 2015. Thrombolysis in pediatric stroke study. Stroke. 2015: 46(3); 880-885.

Rivkin’s team were part of a huge multi-state stroke research team in North America. They designed the incredibly well thought out and well put together TIPS (Thrombolysis in Paediatric Stroke) study, to look at (A) safety of and (B) dose of tissue plasminogen activator (tPA) in children presenting with and arterial ischaemic stroke (AIS). They set out to recruit children aged 2 – 17 with acute AIS and PedNIHSS score between 4 – 24 to receive tPA if initiated within 4.5 hours of symptom onset. Centres were given protocols to manage complications such as intracranial haemorrhage, systemic bleeding, hypotension or angioedema.

Sounds good, right?

Well, in principle, yes.  The study opened in April 2012 but closed only 20 months later in December 2013 because only 1 child had been enrolled and they hadn’t actually been treated due to complications following extubation prior to tPA administration. 

93 children had been screened with 43 having confirmed AIS and the other 50 having a stroke mimic such as migraine, seizure or tumour etc. 

Of the 43 children with AIS about half had medical contraindications to tPA (including moyamoya disease & anticoagulation treatment); 10 were outside the treatment window (including 1 who missed the treatment window by 15 minutes due to delay at scanner); some had a PedNIHSS that was too low ; 1 had a PedNIHSS that was too high; and a couple didn’t have arterial occlusion on imaging.

But it wasn’t a total disaster. Preparing for TIPS also led to the development of Paediatric Stroke Networks in North America.  And designing the TIPS study led to consensus guidelines on the management of stroke in children.

These consensus guidelines derived from the TIPS study design have been extrapolated to the 2017 RCPCH Stroke in Childhood guideline, based on expert opinion and the best available evidence. As well as the full guideline, there’s a simple, easy to follow pathway poster that can be grabbed for quick reference whenever a child presents with potential stroke symptoms.

The poster gives a list of potential stroke presentations, from an unexplained persistent drop in GCS, through acute focal neurology (even if resolved), focal seizures, headaches, ataxia, dizziness, speech disturbance and a prompt to consider stroke in children with sickle cell disease.

It includes a simple, easy to follow, Paediatric National Institute of Health Stroke Scale (that PedNIHSS we’ve talked about) a bit like a Glasgow Coma Scale but specific for paediatric stroke.  The PedNIHSS makes up a really important part of the neurological assessment, a way of scoring the severity of the stroke. It is vitally important that the PedNIHSS is calculated because if the score is very low, with a very minimal deficit at the outset, the risk of thrombolysis outweighs the potential benefit. And if the PedNIHSS score is very high, it’s likely that the child has a very large area of brain damage, with a high risk of haemorrhage into that infarcted territory, again making the risk : benefit ratio too risky. The child’s PedNIHSS score guides your subsequent management.

The pathway lists investigations (which must include coagulation profile and group and save, because of that risk of bleeding), monitoring and neuroimaging. Timing of imaging is key. The guideline states that children should be scanned within 1 hour of presentation to the ED. Pragmatically, this is usually CT with CTA (the angiography component to look at the arteries), because organising an MRI with MRA takes longer. But, if you’re in an institution with great access to MR and you can get your imaging within an hour of presentation then it’s definitely worth a discussion with the radiologist.

If a child has a confirmed AIS, what do we do? The guideline offers two either / or treatments: EITHER aspirin 5mg/kg within an hour, as long as there is no parenchymal haemorrhage OR thrombolysis. The guideline suggests that thrombolysis may be considered in children aged 2-8 and could be considered in children over 8 (some careful wording there because extrapolating evidence from adult studies to an 8 year old is easier than to a 2 year old) provided the PedNIHSS is between 4 and 24 and tPA can be administered within 4.5 hours of symptom onset. There must be either MRA evidence of thrombus or normal or only minimally ischaemic changes on CTA (no huge areas of ischaemia, because the risk of bleeding into it is just too high), with or without evidence of thrombus. And as the biggest risk of giving tPA is haemorrhage, there must be no contraindications, such as abnormal clotting, an underlying bleeding disorder, malignancy, hypertension or moyamoya disease.

It’s really important to note that the treatment for AIS is not the same as for a child with a haemorrhagic stroke (these children need urgent discussion with a neurosurgeon for consideration of evacuation) or a child with an ischaemic stroke secondary to sickle cell disease (pick up the phone, call a haematologist and organise an exchange transfusion). Although not included on the poster, the guideline summary and full guideline give indications for surgical and endovascular interventions in stroke, as well as those nuggets for managing stroke in a child with sickle cell disease or haemorrhagic stroke.

And what about thrombectomy? This is a very active area of interest. In the world of adult AIS there has been a big move towards primary clot removal by thrombectomy rather than clot busting with thrombolysis. In the world of paediatric stroke, although there are some published case series and case reports, we don’t have a clear evidence base or national guidance. Yet.

So, what is the take-home from Tomas’ case? Although stroke is rare in children, it does occur. Thrombolysis is a potential management option given the right conditions, as long as it’s given within the 4.5-hour window. So, next time you see a child with stroke-like symptoms, send bloods early, get early neuroimaging with angio, and pull out the RCPCH Stroke in Childhood poster.

With special thanks to Dr Dan Lumsden, Paediatric Neurologist at the Evelina London Children’s Hospital, who inspired the creation of Tomas’ case and presented him so fabulously at the Royal Society of Medicine. Thank you, Dan.

Soft tissue knee injuries

Cite this article as:
Lisa Dann. Soft tissue knee injuries, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.25908

Sam, a 12-year-old boy, presents to your department after a soccer blitz. He was tackled, heard a pop, and now can’t weight bear on his right knee.

As popularity and intensity of children’s sports increases there are increased demands placed on children and adolescents. This has resulted in an increased presentation of children like Sam. They can present with knee pain that is traumatic or atraumatic, acute or chronic. Paediatric patients are particularly vulnerable to overuse injuries involving the physes and apophyses due to their inherent weakness (see post, hyperlink article on fractures around knee).

Along with these there has also been an increase in soft tissue injuries. These are seen more commonly in older children/adolescents as their bones become stronger and are less likely to fracture with age.

History/examination

Important points to note on the history include:

  • If there was clear onset of pain
  • Traumatic or atraumatic
  • Duration of pain
  • Previous injury/surgery
  • Site of the pain (try be as specific as possible)
  • Severity of pain
  • Nocturnal pain
  • Systemic symptoms
  • Associated swelling (intermittent or progressive)
  • Contralateral injuries (may result in abnormal gait placing additional pressure on knee)
  • Hip or back pain

Recalling the anatomy of the knee makes evaluating the site of pain easier.  The following make up the knee and all can be can be injured/inflamed and cause pain.

  1. Bones around knee – femur ends at lateral and medial condyles which articulates with tibial plateau and anteriorly the patella unsheathed in the patellar tendon.
  2. Ligaments – anterior cruciate ligament (ACL), posterior cruciate ligament (PCL), medial and lateral collateral ligaments.
  3. Meniscus – medial and lateral menisci act as shock absorbers and interdigitate into the ACL and PCL for more stability.
  4. Bursae – supra-patellar bursa, infra-patellar bursa, pre-patellar bursa, and pes anserine bursa (medial aspect of knee).
  5. Tendons – quadriceps tendon (inserts into patella), patellar tendon (inserts into tibial tuberosity)
  6. Other – iliotibial band (fibrous support of fascia lata originating at the external lip of iliac crest and inserting into the lateral condyle of the tibia).

Examination in the acute setting is often difficult and may be limited. This is due to swelling, pain and anxiety. Try your best to be as detailed as possible but ensure you note any red flags on examination. These are:

  • Inability to do straight leg raise (extensor mechanism rupture)
  • Ligamentous laxity
  • Catching, locking or giving away (meniscal injury)
  • Inability to fully straighten the knee

After a thorough history and examination you discover he was tackled and the other player’s foot landed on the lateral aspect of his knee. On examination you find a swelling on medial aspect of the knee and laxity of the medial collateral ligament when valgus stress is placed on the right knee.  You clinically diagnose a medial collateral ligament injury. He is placed in a brace and referred to orthopaedic clinic.

Injured ligaments are considered “sprains” and are graded on a severity scale.

  • Grade 1 sprains: The ligament is mildly damaged. It has been slightly stretched, but is still able to help keep the knee joint stable.
  • Grade 2 sprains: The ligament has been stretched to the point where it becomes loose. This is often referred to as a partial tear of the ligament.
  • Grade 3 sprains: This type of sprain is most commonly referred to as a complete tear of the ligament. The ligament has been split into two pieces, and the knee joint is unstable.

Ligament specific examinations:

  • Anterior and posterior drawer tests – asses anterior and posterior cruciate ligament integrity.
  • Lachman test – assesses ACL integrity. Most sensitive test for ACL rupture. Non-dominant hand cups and support knee. Ensure quads and hip flexors relaxed for it to work. Dominant hand grasps proximal tibia, knee flexed at 20-30 degrees. Pull sharply. Tibia shouldn’t move much and should have distinct end point.
  • Posterior sag test – patient supine, hip flexed at 45 degrees and knee at 90 degree. Look at knee from lateral position. If PCL damaged you’ll see tibia sagging posteriorly.
  • Varus and valgus stresses – assess integrity of medial and lateral collateral ligaments. Compare both sides for laxity.

Management:

  • Unstable joints require a thorough examination of neurovascular status, orthopaedic consultation and very close follow up.
  • ACL tears often have poor healing abilities and may require surgical repair if injury is significant.
  • PCL is much better at healing itself than ACL and low grade tears are managed non-operatively with grade 3 or higher needing reconstruction.
  • Collateral ligament injuries have good healing potential so rest, ice, bracing and slow advancement of range of motion is the management primarily undertaken.

A short while later one of Sam’s team mates, Patrick, presents to ED. He was also playing in the soccer blitz. He got sudden knee pain when turning and his knee is now locked. Following assessment you suspect a meniscal injury.

Meniscal injuries

Meniscal injuries can be traumatic or atraumatic. Suspect if the knee is locked, there was a twisting mechanism, a tearing sensation, or an effusion.

Specific examinations include:

  • McMurray test – patient supine, hip and knee flexed at 90 degrees. Non-dominant hand placed over joint line. Dominant hand grasps patient’s heel and internally and externally rotates tibia exerting valgus and varus forces while extending the leg. This helps to grind on either the medial or lateral menisci. Pain, popping or clicking is a positive test.
  • Appley compression test – the patient lies prone with the knee bent at 90 degrees. The examiner rotates the leg externally and internally several times under simultaneous vertical pressure. A painful pop can point to a meniscal injury.

Treatment includes physiotherapy to compensate for the tear but surgical management may ultimately be required. Follow up with orthopaedics is required.

Patrick’s sister was also brought for review. She is 15 years old and has been having intermittent knee pain for the last few months but it gets much worse after she plays sports. She also says it really hurt her after the cinema yesterday. You suspect patellofemoral pain syndrome.

Patellofemoral pain syndrome (PFPS)

The pain is frequently described as anterior but is often poorly localised. It may feel like it’s “under” or “around” the patella. Pain is classically exacerbated by prolonged periods of sitting, use of stairs and squatting (theatre sign). Pain may be present for several weeks, exacerbated by activity and relieved after periods of rest. Frequently there is a deterioration in sports performance or inability to participate prompts patients to seek medical review.

Clinical examination should look for gait abnormalities, increased lumbar lordosis, and any asymmetry in hips or lower extremity. It is not uncommon to have reduced flexibility in the hamstrings or quadriceps.

Clarkes sign – positive in PFPS. Patient supine, knee extended. Grab the superior pole to the patella with thumb and index finger and have the patient activate the quadriceps while you inhibit the patellar movement. This causes grinding of the articular surface between patella and femur. Pain is indicative of PFPS.

Investigations are not routinely required. However, knee radiographs may assist in ruling out other conditions such as osteochondritis dissecans of the knee/patella and stress fractures of the patella. Radiographic imaging in PFPS is not diagnostic. It is necessary to combine any findings with your clinical examination.

Management of this is conservative as it is a self-resolving condition. It typically resolves over weeks to months but has been known to take up to two years for complete resolution of symptoms. Management involves reduction in activity (complete cessation usually not required), ice, rest, anti-inflammatory for pain control (short term use), avoidance of aggravating exercises (e.g. squatting) and some find relief with taping/knee-braces. Exercises that strengthen and increase flexibility of the quadriceps, hamstrings, soleus and gastrocnemius muscles are also recommended.

Further specific examinations and possible causes of non-specific knee pain

  • Ask the patient to tighten knee and palpate the quadriceps tendon at superior pole (tenderness indicates possible tendonitis), straight leg raise (assessing quadriceps strength and integrity).
  • Palpate body of patella for tenderness (Sinding-Larsen syndrome) and then patellar tendon and tibial tuberosity (Osgood-Schlatter syndrome).
  • Palpate the medial side of patella (possible inflamed medial plica band) and also palpate the proximal tibial surface (medial anserine bursa- pain, swelling, tenderness may indicate bursitis).
  • Feel under the patella (tenderness on articular surface could indicate patella-femoral syndrome).
  • Lateral – assess for patellar instability (need quadriceps relaxed and knee flexed at 30 degrees). Apprehension indicates patellar laxity and potentially previous dislocation.
  • Joint line – bend the knee and palpate either side slowly and carefully. Try to localize as much as possible. Tenderness may indicate a meniscal tear.
  • Hamstring muscles: With the knee flexed palpate the hamstring muscles. Laterally is the biceps femoris and medially semi-tendinosus and semi-membranosus. Chronic tightness may be the cause of knee pain.
  • Patellar ballottement- effusion

Bottom line

A thorough history and examination can greatly assist in reaching the diagnosis. A correct diagnosis helps to properly counsel patients and appropriately manage their expectations. Without proper treatment, knee injuries can lead to chronic knee problems, early onset arthritis, injury to surrounding tissues, and prolonged healing times. Missed injuries can also cause recurrent cartilage damage, instability in the knee, and unnecessary time away from physical activity. It is our duty to diagnose these injuries in a timely manner and provide appropriate advice, support and follow up.

Below is a useful table outlining the causes of intrinsic knee pain, separated by site of pain on examination (table 1).

References

Finlayson C. Knee injuries in the young athlete. Pediatr Ann. 2014;

Brooke Pengel K. Common overuse injuries in the young athlete. Pediatr Ann. 2014;

Beck NA, Patel NM, Ganley TJ. The pediatric knee: Current concepts in sports medicine. J Pediatr Orthop Part B. 2014. doi:10.1097/BPB.0b013e3283655c94.

Calmbach WL, Hutchens M. Evaluation of patients presenting with knee pain. Part II Am Physician. 2003.

PEM Playbook Knee Pain podcast https://pemplaybook.org/podcast/knee-pain/

Tillaux fractures

Cite this article as:
Tadgh Moriarty. Tillaux fractures, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.26111

Jenny is a 14-year-old girl who was at soccer training when she had an awkward injury to her left ankle. She was running for the ball when her foot caught in a clump of grass and she externally rotated her leg while her foot remained planted. While the pain was instantaneous, there was very little swelling. Her father has brought her in remarking that ‘it’s probably just a sprain’. But as you call her down to the cubicle to examine her, you think there might be more to it.

Incidence

Tillaux fractures are a type of ‘transitional’ ankle fracture which occur almost exclusively in adolescents. These occur during the unique closure pattern of the distal tibial physis. This closes over an eighteen-month period; first in the middle, then medially and finally laterally. During the closure, this area is vulnerable to these distinctive transitional fractures; the triplane fracture and the Tillaux fracture.

These fractures account for roughly 3% of paediatric ankle fractures. They are seen more commonly in females and tend to occur at slightly different ages depending on gender. It tends to occur later than a triplane fracture; between 12-14 years in girls and 15-18 years in boys.

Mechanism

This Salter-Harris 3 fracture occurs at the anterolateral distal tibial epiphysis. It tends to cause avulsion of the tibial fragment by the tibiofibular ligament; this strong ligament extends from the anterior aspect of the lateral distal tibial epiphysis to the anterior aspect of the fibula. The lack of a fracture through the coronal plane distinguishes this injury from that of a triplane fracture.

This injury pattern occurs usually through a combination of supination and external rotation of the foot in relation to the leg. It usually occurs through low-velocity trauma for example in skateboard accidents or sports with a sliding injury.

Presentation

These injuries are almost exclusively seen in adolescents. Similar to most ankle injuries there will be a history of trauma, symptoms of pain, swelling, and an inability (or painful) weight-bearing.

The clinical exam often reveals localised tenderness to the anterior joint line. This contrasts with a sprain where the tenderness is usually below the joint line. Marked displacement is prevented by the fibula.

Imaging

Do not be misled by lack of swelling – have a low threshold to image injuries which present with an inability to weight bear (at least four steps). When requesting an ankle x-ray AP and lateral views will be included as standard – if you have a high index of suspicion for a Tillaux fracture ask for an oblique or ‘mortise’ view – this can improve your detection by avoiding the obstructed view through the fibula and potentially making the subtle fracture more apparent.

This injury can sometimes have an associated ipsilateral tibial shaft fracture or a proximal fibular injury. During your clinical exam if you illicit tenderness proximal to the ankle, then include a full-length tibia/fibula x-ray with your request.

Does this injury require a CT? Perhaps! See the controversy section below for a more thorough explanation. CT is generally only required by the orthopaedic team to assist with surgical planning.

Image courtesy of Orthobullets.com

Image showing CT scan of same fracture with >2mm displacement courtesy of Orthobullets.com

This injury however often requires a CT scan to assist the orthopaedic team in deciding between conservative and operative management.

A study by Horn et al showed CT as being more sensitive than plain film at detecting fractures with greater than 2mm displacement (the cut-off point adopted for operative fixation).

Treatment

These fractures are important as they involve the weight-bearing surface and can lead to significant morbidity if missed. The treatment of these injuries is not uniform – different methods and cut-offs are described in different case reports and case series. Having that said the current marker for operative versus conservative treatment is the degree of displacement of the fracture fragment.

Those with < 2mm displacement can generally be treated conservatively. This usually involves an above-knee cast for up to 4 weeks (to control the rotational component) followed by either a walker boot or below-knee cast for a further 2-4 weeks. This conservative approach is well documented in having a satisfactory outcome.

Those with displacement >2mm generally require intervention to ensure articular congruity of the joint surface is restored. Intervention can occur in different forms; some may be suitable for a closed reduction under procedural sedation (or general anaesthetic). Different reduction techniques exist however longitudinal traction while the knee is flexed followed by internally rotating a maximally dorsiflexed ankle seems to achieve greater anatomic reduction. A review by Lurie et al concluded those left with a residual gap of more than 2.5mm led to worse functional outcomes. Therefore post-reduction radiological confirmation should show minimal displacement (the figure of <2mm tends to be favoured in the literature) otherwise operative intervention is required. Many orthopaedic surgeons favour CT as the post-reduction imaging modality of choice and then follow this reduction with serial radiographs to confirm maintenance of the reduction over time.

Operative intervention can involve K-wire insertion, use of lag screws or a novel technique involving percutaneously inserted wires with arthroscopic or radiological guidance. This new technique is seen as less invasive and as-effective but is technically complex and demanding. If this injury presents with a neurovascular compromise or critical skin then emergent surgery is indicated. This is, thankfully, rare.

What to tell the patient

Recovery: Both operative and conservative measures tend to require up to 8 weeks of immobilisation followed by a rehab phase. This phase will vary depending on the age of the patient. Most patients have a good outcome with 86% having complete recovery and no sequelae. Very few will have pain or limitation of ankle movement. Late presentation and a non-anatomical reduction will increase the risk of this.

Complications

These are less common than other ankle fractures; delayed or malunion, osteonecrosis of the distal tibial epiphysis, premature growth arrest and compartment syndrome are all very rare occurrences. Early-onset arthritis can occur, those with late presentations or missed fractures are more at risk.

Operative intervention carries the additional (albeit small) risk of physeal damage from direct pressure by blunt instruments and inadvertent damage to the superficial peroneal nerve.

Controversies

Radiological evaluation remains controversial. Plain x-ray usually identifies the transitional fracture, and the degree of displacement. However, CT (ideally with 3D reconstruction) is more accurate in estimating the degree of displacement and fracture separation.

Case courtesy of Dr Yasser Asiri, Radiopaedia.org. From the case rID: 64778

CT can help in identifying the number and position of fragments. The issue of whether CT or MRI alters treatment or prognosis when compared with plain X-ray has not been fully investigated. Limited research has been carried out on whether CT, with its greater accuracy, actually affects treatment or patient outcome. Liporace et al in 2012 found that interobserver and intra-observer agreements about primary treatment plans did not differ significantly between radiography alone and radiography plus CT. This showed that the addition of CT did not actually change the impression about the degree of displacement in each case. This raises the question as to whether CT really alters outcomes despite having perceived greater benefits.

Jenny is found to have significant tenderness about her distal tibia on exam and an x-ray confirms a Tillaux fracture which is minimally displaced. She is placed in an above-knee backslab and referred to the orthopaedic fracture clinic. She is left disappointed that she will miss this season’s matches, but thankfully you didn’t misdiagnose this as a sprain!

References

Orthobullets.com/paediatrics/4028/tillaux-fractures

Wheelers textbook of orthopaedics (updated 2015) Clifford J Wheeless Tintinnali 7th Ed

Tiefenboeck TM, Binder H, Joestil J et al. Displaced juvenile Tillaux fractures: surgical treatment and outcome. Wien Klin Wochenschr. 2017; 129 (5-6):169-175

Rosenbaum AJ, DiPreta JA, Uhl RL. Review of distal tibial epiphysis transitional fractures. Orthopaedics. 2012;35(12):1046-1049

Horn BD, Cristina K, Krug M, Pizzutillo PD, MacEwen GD. Radiologic evaluation of juvenile Tillaux fractures of the distal tibia. J Pediatr Orthopaedics. 2001; 21(2): 162-4

Cooperman DR, Spiegel PG, Laros GS. Tibial fractures involving the ankle in children. The so-called triplane epiphyseal fracture. J Bone Joint Surg Am. 1978 Dec. 60 (8):1040-6

Panagopoulos A, van Niekerk L. Arthroscopic assisted reduction and fixation of a juvenile Tillaux fracture. Knee Surg Sports Traumatol Arthrosc 2007;15:415-417

Manderson EL, Ollivierre CO. Closed anatomic reduction of a juvenile tillaux fracture by dorsiflexion of the ankle. A case report. Clin Orthop Relat Res. 1992 Mar. (276):262-6.

Crawford AH Triplane and Tillaux fractres: is a 2mm residual gap acceptable. J Pediatr Orthop. 2012 Jun;32 Suppl1:S69-73

Schlesinger I, Wedge JH. Percutaneous reduction and fixation of displaced juvenile Tillaux fractures: a new surgical technique. J Pediatr Orthopaedics. 1993;13:389-391

Stefanich RJ, Lozman J. The juvenile fracture of Tillaux. Clin Orthopaedics Relat Res. 1986;210:219-227

Kaya A, Altay T, Ozturk H, Karapinar L. Open reduction and internal fixation in displaced juvenile Tillaux fractures. Injury 2007;38:201-205

Choudhry IK, Wall EJ, Eismann EA. Crawford AH, Wilson I. Functional outcome analysis of triplane and tillaux fractyres after closed reduction and percutaneous fixation. J Pediatr Orthop. 2014;34:139-43

Jennings MM, Layaway P, Schubert JM. Arthroscopic assisted fixation of juvenile intra-articular epiphyseal ankle fractures. J Foot Ankle Surg 2007;46: 376-386

Rockwood and Wilkin’s fractures in children. 6th Edition.2006

Kim JR, Song KH, Song KJ, Lee HS. Treatment outcomes of triplane and Tillaux fractures of the ankle in adolescence. Clin Orthop Surg. 2010 Mar. 2 (1):34-8

Haapamaki VV, Kiuru MJ, Koskinen SK. Ankle and foot injuries: analysis of MDCT findings. AJR Am J Roentgenol. 2004 Sep. 183 (3):615-22

Charlton M, Costello R, Mooney JF et al. Ankle joint biomechanics following transepiphyseal screw fixation of the distal tibia. J Pediatr Orthopaedics. 2005;25: 635-640

Liporace FA, Yoon RS, Kubiak EN, Parisi DM, Koval KJ, Feldman DS, et al. Does adding computed tomography change the diagnosis and treatment of Tillaux and triplane pediatric ankle fractures?. Orthopedics. 2012 Feb 17. 35 (2):e208-12

Lurie B, Van Rysselberghe N, Pennock AT, Upsani VV. Functional outcomes of Tillaux and triplane fractures with 2-5millimetres of intra articluations gap. J Bone Joint Surg Am. 2020;102:679-686

Rapariz AJ, Avocets G, Gonzalez-Herman P, Texas et al. Distal tibial triplane fractures: long term follow up. J Pediatr Orthopaedics. 1996; 16: 113-118.

EMS Feedback

Cite this article as:
Andrew Patton and Andy O'Toole. EMS Feedback, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.29849

Prehospital practitioners have an ever expanding role in managing the acutely unwell and injured patient. Despite this large contribution to patient care, the majority of practitioners find it very challenging to followup or get feedback on their management of the patient.

The recent publication of the NEMSMA position paper regarding bi-directional information sharing between hospitals and EMS agencies sparked debate on Twitter about the challenges of EMS Feedback.

Gunderson, M.R., Florin, A., Price, M. and Reed, J., 2020. NEMSMA Position Statement and White Paper: Process and Outcomes Data Sharing between EMS and Receiving Hospitals. Prehospital Emergency Care, pp.1-7.

What was the paper about?

The NEMSMA Position statement and White Paper focuses on the bi-directional sharing of data between EMS agencies and receiving hospitals. The authors looked at the challenges EMS agencies face getting feedback data regarding patient outcomes, propose best practices for bi-directional data sharing and explore the current barriers to data exchange. 

The paper highlights the importance of receiving feedback and patient outcome data for quality assurance and improvement (QA/QI). Among other things, feedback is necessary for EMS providers to determine if clinical diagnoses in the field were correct, if pre-arrival notifications were effective and if the destination choice was appropriate. 

The authors surmise that with confusing and complicated healthcare law, hospitals can be reluctant to “share information due to consequences of unintentional violations” of healthcare law, and fears of liability, many of which are misconceptions.

They report that…

“Many of the commonly held legal concerns preventing data exchange are misunderstandings and unfounded fears. While all regulations and laws need to be adequately addressed, legal issues should not preclude properly conducted sharing of electronic health records for quality improvement.”

Technology also creates a number of barriers to data sharing, in particular poor interoperability between EMS electronic patient care records (ePCR) and hospital electronic healthcare records (EHR). The absence of a universal patient identification value is another significant obstacle.   

The authors reference information blocking and market competition between hospitals as two of the big political and economic barriers which can be among the most challenging to overcome. 

They conclude by recommending a collaborative effort between EMS agencies and hospitals to develop and implement bilateral data exchange policies which would benefit all stakeholders. 

This paper focuses mainly on data sharing at an organisational level, it is very relevant to the difficulties faced by individual pre-hospital practitioners trying to follow-up on patients they treat at a local level. 

Why is this so important?

As discussed in the paper, feedback is an important part of quality improvement. For individual practitioners, feedback is a vital part of the learning cycle. Feedback is essential for us to learn from our mistakes, and to improve our practice.  To improve any performance, it is necessary to measure it. A practitioner that never follows up on a patient’s outcome will be left assuming that their treatment for the presenting complaint was accurate and warranted. They will likely continue to treat the same presentation in the same way in the future because their experience has never been challenged by facts that could have been discovered during patient follow up. 

Without feedback we could be unconsciously incompetent… We don’t know what we don’t know!

What’s the difficulty?

On an individual level, obtaining feedback and patient follow-up is challenging for EMS crews for a variety of reasons. In a local survey of 98 prehospital practitioners in Dublin, Ireland, only 21% of practitioners reported being able to follow-up interesting cases.

With dynamic deployment of EMS Resources, crews might transport a patient to a hospital and not return to that same hospital during their shift. If a crew does manage to find an opportunity to call back to the hospital, frequently the diagnostic work-up may be incomplete, and a working diagnosis still unclear. EDs are busy environments and, understandably, some practitioners may feel uncomfortable stopping a doctor or nurse to follow-up on a previous patient.

Calling back a few days later has its own complications; often there will be different staff working in the department who may not have been involved in the patient’s care. This method may work for the high-acuity resus presentations, but that ‘child with shortness of breath’ whose physical exam you were unsure of, or the child with a seizure who had a subtle weakness… the chances of the Emergency Department (ED) staff remembering their diagnosis or outcome is slim! 

Phoning the ED or ward is a route explored by many practitioners, but is fraught with increasing difficulty due to reluctance of staff to give out patient information over the phone fearing confidentiality issues. 

So how do we address this challenge?

Focusing specifically on providing feedback to individual pre-hospital practitioners, there are multiple potential ways to provide prehospital practitioners with follow-up information and feedback,  but you need to consider what system will work best for your individual department, ensuring patient confidentiality and data security.

The pre-hospital postbox

St. Vincent’s University Hospital is a tertiary referral hospital in Dublin, Ireland with approximately 60,000 annual attendances. Inspired by Linda Dykes and her team’s PHEM postbox at Ysbyty Gwynedd Emergency Department in Bangor, Wales, we set-up the Pre-Hospital Post Box in St. Vincent’s University Hospital Emergency Department in August 2017. 

We engaged local prehospital clinicians and ED consultants to develop an SOP. A postbox was built and mounted by the carpentry department. Using a template from Bangor, a feedback request form was developed.  Finally, the service was advertised in the emergency department, local Ambulance and Fire Stations and we were open for business. 

Prehospital clinicians seeking feedback on a case complete a form and place it in the post-box. The case notes are reviewed by an EM doctor and feedback is provided by phone call. 

To ensure patient confidentiality, feedback is only provided to practitioners directly involved with the patient care. A triple-check procedure is used to confirm this. The practitioner’s pin number on the request form is verified on the Pre-Hospital Emergency Care Council (PHECC) register and against the patient care record. The listed phone number is also verified through practitioners known to us or the local Ambulance Officer. 

Other hospitals use systems providing feedback via encrypted email accounts or posted letters.We elected to use a phone call system, the primary reason was the anecdotal reports that many of our pre-hospital staff don’t have easy access to work email accounts. We also anticipated that a phone call would be more likely to facilitate a case discussion and allow paramedics to ask questions that might arise during the discussion. 

Challenges with this system?

Providing feedback to prehospital practitioners is a very time-consuming and labour intensive job, particularly in hospital systems where the majority of clinical documentation is still paper-based. In our own system, where handwritten ED notes are scanned, radiology, labs and discharge letters are available on-line, and in-patient notes are handwritten physical charts – we’ve found the average time required to collate details for the feedback request is just 9 minutes, with a feedback phone call averaging 5 minutes per call.

To successfully upscale this would require a team of doctors or a rota based system with allocated non-clinical time to answer requests. Alternatively a digital solution allowing paramedics to access the data themselves, or facilitating the physician managing the case to reply directly would make it more feasible but may generate further challenges. 

The ideal, as discussed in the NEMSMA paper, would be an organisational process, with the automatic provision of discharge summaries and test results by hospitals to EMS agencies which would provide useful organisational data, and subsequent feedback to individual EMS practitioners.

GDPR / Data Protection Considerations

Patient confidentiality and data protection are of utmost importance in an EMS Feedback System. The system implemented needs to have robust mechanisms, such as our triple-check, to ensure that feedback is only provided to healthcare professionals directly involved in the patient’s care. 

It is also important that it is compliant with data protection legislation in your locality, such as General Data Protection Regulations (GDPR) introduced in Europe in 2018.  Our EMS feedback system is an important mechanism for us to review the care and treatment provided to patients and allows us to assist pre-hospital practitioners in evaluating and improving the safety of our pre-hospital services, which is provided for in the “HSE Privacy Notice – Patients & Service Users”

Providing EMS Feedback, in its current form, is a labour intensive process but we believe it is a worthwhile initiative. It is greatly appreciated by Pre-Hospital Practitioners and it enables them to enhance their diagnostic performance and develop their clinical practice.

If you’d like to find out more about how to set up a Pre-Hospital Post Box in your ED, have a look at these resources…

Attachments

References

Patton A, Menzies D. Feedback for pre-hospital practitioners: is there an appetite? Poster session presented at: 2017 Annual Scientific Meeting of the Irish Association for Emergency Medicine; 2017 Oct 19-20; Galway, Ireland.  

Gunderson MR ,Florin A , Price M & Reed J.(2020): NEMSMA Position Statement and White Paper: Process and Outcomes DataSharing between EMS and Receiving Hospitals, Prehospital Emergency Care, https://doi.org/10.1080/10903127.2020.1792017 

Croskerry P. The feedback sanction. Acad Emerg Med. 2000;7:1232-8.

Jenkinson E, Hayman T, Bleetman A. Clinical feedback to ambulance crews: supporting professional development. Emerg Med J. 2009;26:309.

Patton A, Menzies D. Case feedback requests from pre-hospital practitioners – what do they want to know? Meeting Abstracts: London Trauma Conference, London Cardiac Arrest Symposium, London Pre-hospital Care Conference 2018. Scand J Trauma Resusc Emerg Med 27, 66 (2019). https://doi.org/10.1186/s13049-019-0639-x  

Patton A, Menzies D. Feedback for pre-hospital practitioners – a quality improvement initiative. Meeting Abstracts: London Trauma Conference, London Cardiac Arrest Symposium, London Pre-hospital Care Conference 2018. Scand J Trauma Resusc Emerg Med 27, 66 (2019). https://doi.org/10.1186/s13049-019-0639-x   

O’Sullivan J. HSE Privacy Notice – Patients & Service Users v1.2.  2020 Feb, Accessed on-line: https://www.hse.ie/eng/gdpr/hse-data-protection-policy/hse-privacynotice-service-users.pdf 


Metabolic presentations part 2: children and adolescents

Cite this article as:
Taciane Alegra. Metabolic presentations part 2: children and adolescents, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.28592

Neurological symptoms in a healthy teen

Jane, 14 years old, is brought in by ambulance, unconscious after a 20-minute generalized tonic-clonic seizure at home. She’s wheeled into resus, and while she has a cannula inserted, you take a history from her father. You learn that she has been a healthy child who’s never had a seizure before, with no chronic conditions, no history of drug abuse, no acute illness, and no sick contacts. She’s a vegetarian and enjoys dancing. She’s started a new ‘intermittent fasting diet’ and yesterday hadn’t eaten since brunch. She went to bed early and this morning her mother was woken early by strange sounds coming from Jane’s room and found her seizing on the floor. 

Her primary survey shows that she’s maintaining her airway, is tachypnoeic with oxygen saturations of 98% in air and clear lungs, a normal cardiovascular examination and a GCS of 10, with global hyperreflexia.

This adolescent has an acute onset of neurological symptoms. The differential diagnoses are broad, but her symptoms were precipitated by a new diet that required prolonged fasting. This case is a red flag for a metabolic condition! 

The RCPCH Decreased Consciousness (DeCon) guideline lays out an approach to the child with a decreased conscious level, including differentials, investigations and management (take a look at the DeCon poster and summary guidance). 

You send some bloods and, as suggested by the RCPCH DeCon guideline, you include an ammonia.

Some points to remember

Common things are common: sepsis, CNS infections, intoxication (prescribed and recreational drugs), and primary seizure disorders should all be considered here, but extend your differentials to conditions that can be individually rare but are common as a group: metabolic diseases.

All children presenting with a decreased conscious level, regardless of age, should have an ammonia sent as part of their initial investigation in ED… this could be a case of an undiagnosed urea cycle defect. 

In late onset urea cycle defects, acute metabolic encephalopathy develops following metabolic stress precipitated by a rapid increase in nitrogen load from: 

  • infection
  • trauma
  • rapid weight loss and auto-catabolism
  • increase in protein turnover from steroids
  • surgery and childbirth
  • or other precipitants of protein catabolism.

Adolescents and adults with an undiagnosed urea cycle defect may be completely fit and well, but may have chronic symptoms such as headache, cyclical vomiting, behavioural difficulties, psychiatric symptoms or mild learning difficulties.

They may be selective vegetarians, restricting their protein intake.

Between episodes patients are relatively well. However, acute presentations can be fatal or patients may be left with a neurological deficit.

For more information about cycle urea disorders, check out Metabolic presentations part 1.

The take home

Always send an ammonia in any child presenting with an acute encephalopathy or decreased GCS.

Disorders involving energy metabolism

Next up is Liz, a patient with a diagnosed metabolic disorder.

Liz is a 3-year-old girl from the countryside, who is visiting her grandmother in the city. She has had diarrhoea since yesterday and started vomiting last night. In the last 3 hours, she hasn’t been able to tolerate anything orally. There has been no fever or respiratory symptoms and she is passing urine as normal. Her 5-year-old cousin has similar symptoms. 

Her Grandmother informs you that Liz has MCAD deficiency and her emergency plan was tried at home, without success. Liz is not usually treated at your hospital and you don’t have her chart. Unfortunately, Liz’s grandmother didn’t bring the plan to the hospital. 

Liz looks tired and is mildly dehydrated, but smiles at you. Her heart sounds are normal and her chest is clear. She has increased bowel sounds, a soft abnormal with mild diffuse pain on deep palpation and no masses or organomegaly. She’s afebrile but tachycardic at 165, her capillary  refill time is 3 seconds, and her systolic BP is 104mmHg.

You put in a cannula and measure bedside glucose and ketones. Liz has a hypoketotic hypoglycaemia.

What is MCAD deficiency?

Medium-chain acyl-CoA dehydrogenase deficiency (MCADD) is the most common fatty acid oxidation disorder in Caucasians in Northern Europe and the United States. Most children are now diagnosed through newborn screening. In fatty acid oxidation disorders, the body can only partially break down fat.

Let’s recap some basic biochemistry: in prolonged fasting, the body’s normal response is to break down fat to create ketones, as an alternative source of energy. However, children with MCAD deficiency can’t produce large amounts of ketones, so their ketone response is not appropriate to the degree of hypoglycaemia. 

Clinical symptoms in a previously apparently healthy child with MCAD deficiency include hypoketotic hypoglycemia and vomiting that may progress to lethargy, seizures, and coma, triggered by a common illness. Hepatomegaly and liver disease are often present during an acute episode. These children appear well at birth and, if not identified through newborn screening, typically present between 3 and 24 months of age, although presentation even as late as adulthood is possible. The prognosis is excellent once the diagnosis is established and frequent feedings are instituted to avoid any prolonged periods of fasting (Merritt and Chang, 2019).

Children with fatty acid oxidation disorders (medium, long and short chain defects) have typical acylcarnitine patterns. This is one of the reasons acylcarnitines are sent as part of metabolic and hypoglycaemia work-ups.

What is the priority in acute presentations?

Children who have MCADD, like Liz, need extra calories when sick. The most important intervention is to give simple carbohydrates by mouth, such as glucose tablets or sweetened, non-diet beverages, or intravenously if needed to reverse catabolism and sustain anabolism. In Liz’s case, she’s vomiting all oral intake so cannot tolerate oral carbohydrates, so the intravenous route is necessary. 

The key priorities are:

  • Correct hypoglycaemia immediately with 200mg/kg glucose: 2 ml/kg of 10% glucose or 1ml/kg of 20% glucose, over a few minutes. 
  • Treat shock or circulatory compromise with a bolus of 20ml/kg 0.9% sodium chloride. 
  • Give maintenance fluids with potassium once the plasma potassium concentration is known and the child is passing urine. 

Where can you find resources?

The British Inherited Metabolic Disease Group, BIMDG, has specific guidance on their website.

Disorders involving storage of complex molecules

Mike is 12 years old, presenting to the ED with cough and fever. He has been coughing for 10 days, worse progressively in the last 5  and febrile for the last 3 days. He’s been lethargic since yesterday and even when afebrile he looks unwell. His appetite is poor and he has been “sipping some apple juice”. You learn from his mother that he has a condition called Mucopolysaccharidosis (MPS) type I and is receiving treatment with “the enzyme”. Every now and again, “he is chesty and needs to come to hospital”. 

You examine Mike. He’s pink and well hydrated, but looks sick. You notice that he is shorter than an average 12 year old boy, has hand contractures and coarse facial features. 

He has a soft systolic cardiac murmur with good pulse volume. On auscultating his chest you hear creps and rhonchi on the right side. He has mild hepatomegaly and an umbilical hernia. 

His temperature is 37.5ºC, heart rate is132, respiratory rate 30, and oxygen saturations are just 88% in air.

A bit about mucopolysaccharidoses (MPS)

In mucopolysaccharidosis disorders, the body is unable to break down mucopolysaccharide sugar chains. These mucopolysaccharide sugars build up in cells, blood and connective tissue: hence the name, ‘storage disorders’.

In general, most affected people appear healthy at birth and experience a period of normal development, followed by a decline in physical and/or mental function.

As the condition progresses, it may affect appearance; physical abilities; organ function; and, in most cases, cognitive development. 

Most cases are inherited in an autosomal recessive manner, although one specific form (Type II) follows an X-linked pattern of inheritance. 

Specific treatment can be provided via enzyme replacement therapy or haematopoietic stem cell transplantation in the early stages. 

Presently, enzyme replacement therapy is available for MPS I, II and VI and is given as an intravenous infusion either weekly or biweekly, depending on the disease. 

Both enzyme-replacement and haemotopoietic stem cell treatments still have gaps and few clinical trials supporting them. (rarediseases.info; Dornelles et.al, 2014).

What treatment should be started in the ED?

Patients with Mucopolysaccharidosis don’t require any emergency treatment in the ED for their underlying metabolic disease. They are, however, at increased risk of respiratory infections.

Mike is likely to have a community acquired pneumonia and needs to be treated accordingly with oxygen and antibiotics.

References

Adam , HH. Ardinger, RA. Pagon, S. E. Wallis, L. J. H. Bean, K. Stephens, & A. Amemiya (Eds.), GeneReviews® [online book]

Merritt JL,  Chang IJ. Medium-Chain Acyl-Coenzyme A Dehydrogenase Deficiency.  GeneReviews® [online book], June 2019. Available at  https://www.ncbi.nlm.nih.gov/books/NBK1424/

Genetic and Rare Diseases Information Center (GARD) https://rarediseases.info.nih.gov/diseases/7065/mucopolysaccharidosis 

Dornelles AD et al. Enzyme replacement therapy for Mucopolysaccharidosis Type I among patients followed within the MPS Brazil Network. Genet Mol Biol. 2014

Volcano

Managing Gastro-Oesophageal Reflux Disease

Cite this article as:
Sarah Davies. Managing Gastro-Oesophageal Reflux Disease, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.29563

Isobel is a 10 week old, exclusively breast-fed, baby girl. She is brought into the Emergency Department with a history of frequent vomiting and poor weight gain. Her examination is normal, but when you ask Isobel’s exhausted-looking mother to put her to the breast, she becomes fractious and fussy, pulling away, arching her back, and taking very little feed at all.  

What are you going to do? 

At face value, this familiar presentation sounds like gastro-oesophageal reflux disease (GORD), although the differential for a ten-week old with vomiting and weight loss is wide.

Gastro-oesophageal reflux (GOR) is …the effortless retrograde passage of gastric contents into the oesophagus, with or without overt regurgitation. 

It is:

  • Physiological, due to low tone in the immature lower oesophageal sphincter
  • Common, occurring in up to 50% infants under 6m
  • Frequent – can happen up to x6/day

Gastro-oesophageal reflux disease (GORD) can be diagnosed clinically when GOR is accompanied by troublesome symptoms that affect everyday functioning (eg crying, back-arching, food refusal) and may lead to complications (eg failure to thrive).

Alternative diagnoses should be considered when there are additional red flag features (see below) indicative of a different pathology and under these circumstances, investigations should be tailored to rule these in or out.

*Some red flags overlap with symptoms directly related to GORD. The number, duration and severity of these should inform your decision to investigate on a case by case basis

As Isobel has symptoms of GORD with faltering growth you check her head circumference (which is appropriate), dip a urine (which is negative), and send some bloods for a faltering growth screen (although you strongly suspect they will come back as normal). You explain to Isobel’s mother that there is a stepwise approach to the management of GORD starting with non-pharmacological measures.

So, in the absence of red flag symptoms, do I need to prove its GORD?

In short, no. There is no single gold standard test for the diagnosis of GORD, hence the emphasis on clinical diagnosis. 

Invasive testing does have a place, though it is rarely the job of an ED clinician to be considering this. 

Endoscopy is used under the guidance of a Paediatric Gastroenterologist, for infants who fail to respond to optimal medical management. This will diagnose erosions and eosinophilic oesophagitis. 

pH MII (multi-channel intraluminal impedance) monitoring is used in children whose symptoms persist despite optimal medical therapy with normal endoscopy.   For a great explanation of this technique this previous DFTB post on reflux from 2016

Barium is out. Reliable biomarkers don’t yet exist. Scintigraphy, ultrasound and trial of a proton-pump inhibitor (PPI) are not useful in babies. 

OK, so I only need to investigate if I think there may be another cause for the symptom. But what should be my initial approach to treatment?

  • Positional management?
  • Avoiding overfeeding?
  • Thickening feeds?

Positional management – keeping the baby upright after feeds and elevating the head of the cot to sleep – is often advised for reflux. However, a study by Loots and colleagues in 2014 showed that regurgitation was only reduced through the use of side-lying positions which should NEVER be recommended due to the increased risk of SIDS. Head elevation made no difference at all despite some evidence that it can be beneficial in adults. 

And whilst a common-sense approach would support a move to smaller more frequent feedings and keeping a baby upright for 20-30 minutes after a feed, there isn’t any good quality evidence that confirms this. 

Feed thickeners have been shown repeatedly to reduce the frequency of visible regurgitation episodes in babies with reflux and in some studies to decrease cry/fuss behaviour too. They are safe and come highly recommended as a first-line intervention for babies with troublesome reflux. If you are going to advise a thickener for a breastfed infant, it’s important to suggest a carob bean-based product, such as Carobel, because the amylase in breast milk will digest the rice cereal-based thickeners such as Cerelac.  

Acupuncture, probiotics, massage, hypnotherapy have not yet been adequately studied for us to say one way or another if they are of any benefit. And alginates, probably the most familiar to us being Gaviscon? We’ll cover those shortly.

The key thing to remember for any intervention, is to reserve these for your patients with GORD. Happy, thriving, refluxy babies, typically outgrow their symptoms as they transition to solid food and should be left well alone

OK, but what if my patient has tried these already? What should I advise next? 

First, check how long they have persisted with the intervention. 

One of the biggest reasons for the simpler interventions not to help with GORD is that they are not given enough time to make a difference. Having said that, if a tired parent is repeatedly confronted with a grizzly, uncomfortable baby who is refusing to feed, asking them to persevere for two weeks with an intervention they don’t think is helping, may be practically difficult to achieve. 

In the UK, we have a choice of two key guidelines to help us with the next steps in reflux management.  

  1. NICE, last updated 2019

OR

  1. ESPGHAN/NASPGHAN 2018 joint consensus guidelines which are endorsed and recommended by our own BSPGHAN
  • European Society of Paediatric Gastroenterology, Hepatology and Nutrition
  • North American Society of Paediatric Gastroenterology, Hepatology and Nutrition
  • British Society of Paediatric Gastroenterology, Hepatology and Nutrition

Except that these guidelines differ a little on the advice they give for when simple measures don’t help…

NICE recommend a trial of Gaviscon first, and if that doesn’t work 4-8 weeks of a PPI such as omeprazole, and only then suggest a trial of cow’s milk protein exclusion (either through use of a hydrolysed formula or maternal dairy exclusion in breastfed infants) as a last resort, if reflux does not improve after ‘optimal medical management’. 

NASPGHAN/ESPGHAN on the other hand, suggest that ALL infants undergo an initial trial of cow’s milk protein exclusion, and only if this fails do they suggest the use of a PPI or hydrogen receptor antagonist (H2RA) such as Ranitidine. The bottom line is, that no-one has looked at the efficacy of a cow’s milk protein-free diet for symptom relief in babies presenting with reflux as the single symptom of cow’s milk protein intolerance (CMPI).  

The NASPGHAN team argues, that whilst there is no evidence on the topic, there are a number of babies with CMPI manifesting as reflux only who will benefit from this approach. They suggest eliminating cow’s milk protein from an infant’s diet for a minimum of 2 weeks, ideally four. If symptoms resolve and reappear on reintroduction then the diagnosis is clear. 

NASPGHAN then suggest babies who do not respond should be referred to secondary care services and started on a time-limited trial of PPI. 

This is largely so that infants are not left struggling on inadequate therapy for long periods of time, but also because their review found conflicting evidence around the benefit and side effect profile of these medications for young children. 

In six studies looking at PPI versus placebo, four studies showed no difference in regurgitation or other reflux associated symptoms between intervention and control groups. Three studies comparing H2RAs to placebo did show some benefit of the intervention, however, these studies were all in older children with biopsy-proven erosive oesophagitis up to 8 years of age.  Two studies showed endoscopic and histological and clinical features of GORD were reduced with H2RA over placebo, but these were in mixed-age groups including children up to 8 years old.

All studies showed a similar profile of side effects and between drug and placebo arms, however, one study demonstrated an increased rate of infection, in particular lower respiratory tract infection and diarrhoea in the PPI group. 

Given these findings, NASPGHAN cautiously recommends PPI or H2RA therapy in babies who have troublesome reflux despite trying a number of other non-pharmacological management options. 

Their key message is around early referral to secondary care, giving sufficient time for any one intervention to work, and making sure children are appropriately followed up.

So, what should I do? 

Given the somewhat conflicting advice outlined by these two well-respected groups, you could be left feeling unsure how to manage your next case. However, the genuine gap in the evidence market here does mean you are free to exercise your own clinical judgment and tailor your decision making to each individual refluxy baby, whilst empathetically taking on board the thoughts and preferences of the family.  This could, for some babies and parents, be medicine in itself. 

And what about alginates?

Two studies in the large literature review by the NASPGHAN/ESPAGHN group, compare Gavsicon to placebo. They show a reduction in visible regurgitation but no difference in reflux-associated symptoms. Furthermore, infants treated with alginate and then undergoing pH MII for 24 hours, showed no difference in the frequency of regurgitation events between groups. 

Chronic use of alginates causes constipation and poses a theoretical risk of milk-alkali syndrome, which is perhaps why the authors suggest use is limited to short term therapy. NICE do recommend a trial of Gaviscon therapy at an early stage in their pathway, as an alternative to feed thickener, but again on a time-limited basis with a planned review. 

Isobel’s mother had already tried two weeks of feed thickener on recommendation from the GP with no improvement. She was keen to avoid medication if possible so you agreed to a trial of dietary cow’s milk elimination for Mum who would continue to breastfeed and give top-ups with a hydrolysed formula if there was still no weight gain in a week. You gave her a sheet of dietary advice to ensure she maintained her own calcium intake and asked her to see the GP in 2 weeks for a review.  

Take home message

  • The vomiting infant has a wide differential – actively look for red flag features and investigate if you are concerned.
  • Infants with GORD need a management plan; infants with GOR, leave well alone
  • Start simply with an intervention that the family are happy to trial
  • Give time for it to work (up to two weeks)
  • Ensure follow-up for all and onward referral for infants who require acid-suppressive medication 

References

  1. Loots et al. Body positioning and medical therapy for infantile gastroesophageal reflux symptoms. Journal of Pediatric Gastroenterology and Nutrition 2014; 59 (2): 237-243. 
  2. Rosen et al. Pediatric Gastroesophageal Reflux Clinical Practice Guidelines: Joint Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition and the European Society of Pediatric Gastroenterology, Hepatology and Nutrition. JPGN 2018; 66(3): 516-554. 
  3. Winter et al. Efficacy and safety of pantoprazole delayed release granules for oral suspension in a placebo-controlled treatment withdrawal study in infants 1-11 months old with symptomatic GERD. JPGN 2010; 50: 609-618.  
  4. Orenstein et al. Multicenter, double-blind, randomized, placebo-controlled trial assessing the efficacy and safety of proton pump inhibitor lansoprazole in infants with symptoms of gastroesophageal reflux disease. Journal of Pediatrics 2009; 154: 514-520e4. 
  5. Davidson et al. Efficacy and safety of once daily omeprazole for the treatment of gastroesophageal reflux disease in neonatal patients. Journal of Pediatrics 2013; 163: 692-698.e1-2. 
  6. Winter et al. Esomeprazole for the treatment of GERD in infants ages 1-11 months. JPGN 2012; 55: 14-20. 
  7. Hussain et al. Safety and efficacy of delayed release rabeprazole in 1-11 month old infants with symptomatic GERD. JPGN 2014; 58: 226-236. 
  8. Moore et al. Double-blind placebo-controlled trial of omeprazole in irritable infants with gastroesophageal reflux. Journal of Pediatrics 2003; 143: 219-223. 
  9. Cucchiara et al. Cimetidine treatment of reflux oesophagitis in children: an Italian multi-centric study. JPGN 1989; 8: 150-156. 
  10. Orenstein et al. Ranitidine, 75mg, over the counter dose: pharmacokinetic and pharmacodynamic effects in children with symptoms of gastro-oesophageal reflux. Alimentary Pharmacology and Therapeutics 2002; 16: 899-907. 
  11. Simeone et al. Treatment of childhood peptic esophagitis: a double-blind placebo-controlled trial of nizatidine. JPGN 1997; 25: 51-55. 
  12. Miller et al. Comparison of the efficacy and safety of a new aluminium free paediatric alginate preparation and placebo in infants with recurrent gastroesophageal reflux. Current Medicines and Research Opinion 1999; 15: 160-168. 
  13.  Ummarino et al. Effect of magnesium alginate plus simethicone on gastro-oesophageal reflux in infants. JPGN 2015; 60: 230-235.

Ten ‘not to be missed’ paediatric ECGs

Cite this article as:
Jordan Evans, Megan Thomas, Amos Wong and Jeff Morgan. Ten ‘not to be missed’ paediatric ECGs, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.29306

To refresh your memory on how to read paediatric ECGs take a look at Anna McCorquodale’s fantastic article: Approaching the paediatric ECG.

Here we review ten ‘not to be missed’ abnormal ECGs that may be encountered in acute paediatrics. 

#1 Supraventricular Tachycardia (SVT)

What is it?

SVT is a narrow complex tachycardia, the electrical activity originates above the ventricles (‘supraventricular’). SVT is classified based on whether it originates from the atrium or from the AV node. Finding where the position of the P wave is (with respect to the QRS complex) during tachycardia (‘P wave hunting’) is essential for the diagnosis of SVT.

Why does it happen?

It usually occurs due to one of the following mechanisms:

  • An accessory pathway linking the ventricle to the atrium, which impulses can travel along returning into the atria (AVRT, Atrioventricular Re-entrant Tachycardia)
  • A micro re-entrant circuit in the AV node itself (AVNRT, AV node re-entrant tachycardia)
  • An enhanced automatic focus in the atrium which fires impulses out 

These all lead to excessive impulses being conducted to the ventricles.

So what do we see on ECG?

  • A fast narrow complex tachycardia (approx. 150-220 bpm)
  • SVT – The hunt for the missing P wave:  It is a common misconception  in SVT that there are no P waves. Whilst this may appear to be the case, this is because the P wave is in fact hidden elsewhere.  The location of the missing P wave will depend on the type of SVT.
  • Lack of beat to beat variability i.e. you will see on the monitor that the rate stays pretty much constant

Atrioventricular Re-entrant Tachycardia (AVRT)

This is when there is an accessory electrical pathway connecting the ventricles and the atria. This creates a re-entrant circuit, with impulses either being conducted down the AV node and then back up the accessory pathway (orthodromic) or vice versa (antidromic).  You may see a retrograde P wave at the end of the QRS complex. (See #2 for further info on Wolff-Parkinson-White Syndrome, a classic type of AVRT).

Atrioventricular Nodal Re-entrant Tachycardia (AVNRT)

A micro re-entrant circuit forms in, or adjacent to, the AV node itself.  Here, P waves are very hard to find as they are usually buried in the QRS complex. The circuit often stimulates both the atria and ventricles and therefore the P-wave is hidden, buried within the QRS complex. 

AVNRT: N for No P waves!

Permanent junctional reciprocating tachycardia (PJRT)

This is a type of orthodromic AVRT where the concealed accessory pathway is near the coronary sinus. This means it can conduct at a relatively slow rate for a tachycardia. The characteristic of PJRT is Long RP tachycardia where the P wave is inverted in the inferior leads (hence NOT in sinus rhythm!) and the RP interval is longer than PR interval.

PJRT is commonly misdiagnosed as sinus tachycardia.  If PJRT is suspected seek cardiology input as adenosine is often ineffective and therefore needing multiple anti-arrhythmic therapy.

#2 Wolff-Parkinson-White Syndrome (WPW)

What is it?

Wolff-Parkinson-White is a conduction abnormality, where there is an accessory pathway connecting the atria and the ventricles. If this accessory pathway conducts from the atria to the ventricles (anterogradely) then it can be seen on the ECG as ‘pre-excitation’, as the impulse will travel faster down the accessory pathway than the rate-limited AV node.  WPW can lead to SVT (AVRT type).

What do we see on ECG?

A short PR interval (<120ms) is seen.

The most distinguishing feature is a delta wave which appears as a slow upslope between the Q wave and the R wave – with the Q wave being much earlier than usual. This means that the QRS is wide (>100ms).  The delta wave reflects fusion between the accessory pathway and the normal QRS as conducted via the AV node.

#3 Complete Heart Block

What is it?

Complete heart block (also known as ‘Third Degree’ heart block) occurs when an impulse isn’t conducted from the atria to the ventricles, usually due to AV node pathology. This means that whilst the atrial rate is determined by the SA node, the ventricular rate is a ventricular escape rhythm –which is much slower than the rate of the SA node. This means that the ventricles and atria, therefore, contract completely independent of one another. In AVN block a narrow QRS is seen, whereas, in an infranodal block, a wide QRS is seen. The former is more stable as the pacemaker site is more proximal (Bundle of His) so asystole is less likely.

Why does it happen?

Heart block can occur for a variety of reasons in children, but often it is congenital- secondary to either structural disease (i.e congenitally corrected transposition of the great arteries) or maternal antibodies, as seen in neonatal lupus. Congenital heart block associated with underlying structural heart disease has a poorer prognosis.

So what do we see on ECG?

There are regular P waves and regular QRS complexes, but these are completely unrelated to one another.

But what about the other blocks?

#4 Myocarditis

What is it?

As suggested in the name, myocarditis is inflammation of the myocardium. This can occur due to infection (viruses, bacteria, spirochetes, fungi, and other organisms) having a direct toxic effect on the myocardium.  It is important to consider the diagnosis in children who have recently suffered a systemic illness (esp. Coxsackie). Certain drugs may also be responsible (anthracycline chemotherapy and alcohol).  Myocarditis may occur alongside pericarditis. The inflamed myocardium is unable to contract and conduct as well as usual resulting in poor function of the heart.

So what do we see on ECG?

Usually, myocarditis presents with sinus tachycardia and non-specific T-wave and ST-segment changes (e.g. T wave inversion). We may also see:

  • QRS/QT prolongation
  • Low voltage QRS (<5mm in precordial leads)
  • Pathological Q waves
  • Ventricular arrhythmias (can be ectopics or VT)
  • AV block 

#5 Dilated Cardiomyopathy (DCM)

What is it?

Dilated Cardiomyopathy (DCM) is characterized by weak and floppy myocardium.  It may be inherited or develop as a result of myocarditis secondary to infection or drugs.

So what do we see on ECG?

Cardiomyopathies show similar features to myocarditis.  Pathologically it’s a spectrum, the inflammation in myocarditis is the ‘active phase’ leading to muscle damage present in cardiomyopathy. Changes may include:

  • QRS/QT prolongation
  • Low voltage QRS (<5mm in precordial leads)
  • T wave / ST segment changes
  • Pathological Q waves
  • Ventricular arrhythmias (can be ectopics or VT)
  • AV block 

#6 Hypertrophic Cardiomyopathy (HOCM)

What is it?

HOCM is a genetic condition that affects the sarcomeres in the heart causing left ventricular hypertrophy (LVH), which cannot be explained by other causes. It is very important as it’s the most common cause of sudden cardiac death in those <35, and those who have HOCM may require an internal cardiac defibrillator.

So how do we figure out if there is HOCM?

There are many criteria that can be used to describe HOCM, however no one criteria has been determined to be the most reliable (especially in children).

So what do we see on ECG?

Whereas in DCM there are small QRS complexes, in HOCM they are large due to the hypertrophied muscle.  As above with myocarditis and dilated cardiomyopathy: T wave inversion and ST changes indicate unhealthy myocardium. Pathological Q waves may also be seen.

#7 Long QT

What is it?

As the name suggests, this is when the QT interval is prolonged. In order to determine if the QT is prolonged then we need to determine the QTc using Bazetts formula.

Source: litfl.com/bazett-formula/
  • In boys, a prolonged QTc is >450ms
  • In girls a prolonged QTc is >460ms.

The most important tool in trying to determine the cause of a prolonged QT interval is history! Certain features make a congenital cause of a prolonged QT interval much more likely:

  • Syncope (+/- stress)
  • Congenital deafness (suggests LQT5)
  • FHx of sudden cardiac death <30 yr in immediate family
  • FHx of Long QT syndrome
  • Certain medications

What causes it?

There are many causes of a prolonged QT interval, including:

Acquired prolonged QT: This is when a child has a prolonged QT interval secondary to an underlying cause such as:

  • Drugs: antibiotics, antidepressants, antipsychotics, antihistamines, antiarrythmics, antifungals
  • Electrolyte disturbances
  • Hypothermia

Congenital long QT syndrome: This is an inherited channelopathy, where the child has a prolonged QT interval present either at baseline or unmasked by a stimulus.

There are 17 different forms of long QT syndrome (and counting!), which each have a different genetic mutation.

Three main types of Long QT syndrome (LQTS):

NameGeneTriggersFrequencyT waves
LQT1KCNQ1Peak exercise40%Early onset broad based.
LQT2KCNH2Sudden loud noises, swimming, emotion, stress30%Low voltage double bumped ‘bifid’ T wave with notching
LQT3SCN5ARest, sleep10%Late onset T wave  

ECG changes may be seen at rest or the child may need to go through exercise tolerance testing or an adrenaline challenge in order to unmask the prolonged QT interval.

So what do we see on ECG?

Nice one, Sherlock, you guessed it!  The main feature is a prolonged QTc interval.      

Why do we care so much about a prolonged QT interval?

Those who have a prolonged QT interval are at a higher risk of developing VT or Torsades de Pointes and therefore sudden cardiac death. This means it is important to identify these children so that they can receive medical therapy or an ICD if deemed necessary.

In paediatric cardiology, Schwartz criteria is used to determine the likelihood of Long QT syndrome. Whilst we do not reach an ultimate diagnosis in the ED, it is useful to note the risky features.

#8 Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)


What is it?

As the name suggests, it is a polymorphic (i.e. of multiple morphologies) ventricular tachycardia that is stimulated by catecholamines such as adrenaline. It is an inherited condition, which affects ion channels causing altered calcium flux leading to delayed after-depolarisations causing VT. Whilst baseline ECG may be normal, states in which there is a high adrenaline surge can unmask the CPVT (i.e. heavy exercise). If detected, these children need to be sent for further investigation for medical management or potentially an ICD.

So what do we see on ECG?

A polymorphic ventricular tachycardia. There are two types:

  • Normal baseline ECG!
  • Typical polymorphic / bidirectional VT – where both QRS complexes and T wave change in axis.

Differential for bidirectional VT = CPVT, LQTS Type 7 and Digoxin toxicity.

Do not shock CPVT! This may induce ‘electrical storm’ perpetuate the catecholamine release.  Seek advice from your local Paediatric Cardiologist.

#9 Brugada Syndrome

What is it?

This is an inherited channelopathy which affects impulse conduction, causing ventricular tachyarrhythmias and potentially sudden cardiac death. It is more common in males and commonly seen in carbohydrates consuming nations e.g. rice in South East Asia and pasta in the Western population. Interestingly, the same gene that causes LQT3 (see above) causes Brugada (SCN5A), however in the former there is a gain in function, whereas in Brugada there is a loss of function.

#10 Anomalous Left Coronary Artery arising from the Pulmonary Artery (ALCAPA)

What is it?

This is when the left coronary artery is connected to the pulmonary artery instead of the aorta. This means that instead of receiving oxygenated blood, the left side of the myocardium will receive deoxygenated blood. This can lead to myocardial ischaemia, which is initially transient occurring only in periods of increased myocardial demand (feeding, crying). However, as oxygen demand increases, infarction of the anterolateral left ventricular wall can occur.

So what ECG changes do we see?

  • Pathological Q waves (esp. in leads I, aVL and V6) – 50% of kids with Q waves in aVL have ALCAPA!
  • Ischaemic / T wave changes in inferolateral leads (II, III, aVF, V5-6).  Note on ECG below T waves in V5 and V6 are flattened

Maxilla and zygoma injuries

Cite this article as:
Jessie Lynch. Maxilla and zygoma injuries, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.21700

A 2-year-old child called Lucy is brought to your ED by ambulance. She was the right-sided rear seat passenger in a high-speed head-on road traffic collision. The driver of the other car died on impact. She was restrained in a car seat, however, the seatbelt holding the car seat in place had broken and the car seat was thrown forward. She had a 4-minute episode of loss of consciousness. She had a GCS of 14/15 on arrival, she was maintaining her airway and she was haemodynamically stable. She had significant swelling, bruising, and superficial abrasions to the right side of her face, and her right eye was swollen shut.

Facial injuries in children are relatively common. The most common facial injuries encountered in a paediatric population are dental trauma, oral trauma and facial lacerations. Facial fractures, however, are exceedingly rare in this population, with an incidence of <15% in those under the age of 16 years, and only 0.87% – 1% in those under the age of 5 years.

There are a number of factors which make children less prone to facial fractures. These include:

  • Retruded position of the midface relative to the skull
  • Structural stability increased by the presence of tooth buds within maxilla and mandible and lack of sinus pneumatisation
  • A thick layer of adipose tissue coverage
  • More elastic bones and flexible suture lines
  • High level of adult supervision

These factors also make children more prone to greenstick and minimally displaced fractures as opposed to comminuted or complex fractures. These factors become less significant as the child grows older.

History

Common causes of facial fractures include falls, road traffic collisions, sports-related injuries and, less commonly, interpersonal violence. 2.3% of victims of non-accidental injury have facial fractures and the possibility of this should always be taken into account.

Examination

The presence of a midfacial fracture in a child implies that a significant velocity impact has occurred. 40% of children with a midfacial fracture have an associated skull fracture, and associated cervical spine injury is also common. The primary survey should be undertaken following APLS protocols, with particular attention paid to cervical spine immobilisation and airway management. A detailed craniomaxillofacial examination should be performed as part of the secondary survey, after initial stabilisation.

Image from Wikimedia

Fractures of different parts of the face will lead to different clinical signs.

Zygomatic arch and zygomaticomaxillary complex (ZMC) 

The zygomaticomaxillary complex (ZMC) is made up of four parts:

  • lateral orbital wall
  • zygomatic-maxillary junction
  • zygomatic arch
  • orbital floor

An approach to the assessment of ZMC fractures includes:

  • Inspect the orbit. There may be periorbital swelling or ecchymosis, enophthalmos, subconjunctival haemorrhage and diplopia (due to extraocular muscle dysfunction). The orbital examination should also include visual acuity, visual fields and extraocular muscle function.
  • Palpate the facial bones. There may be a palpable depression or step in the infraorbital rim or zygomatic arch as well as tenderness or widening of the frontozygomatic suture.
  • Oral assessment. There may be trismus (lock jaw) and bruising and tenderness of the upper buccal sulcus.
  • Infraorbital nerve assessment, documenting any paraesthesia.

Maxilla

Maxillary fractures are classified according to the Le Fort classification system*

  1. Le Fort I: A horizontal fracture through the floor of maxillary sinuses with the teeth contained within the detached fragment. Only palate moves.
  2. Le Fort II: A fracture which can be one-sided or bilateral fracture through the maxilla extending into the floor of the orbit, the nasal cavity and hard palate. This results in a pyramidal shaped fracture.
  3. Le Fort III: A fracture through the orbits in which the entire maxilla and one or more facial bones, the entire midface, becomes separated from the base of the skull. This is called craniofacial disjunction.

*Rene Le Fort was a French physician at the turn of the 20th century. He discovered that the midface tended to fracture in three different ways when traumatising cadavers in quite gruesome, but scientifically important, ways.

Clinical signs of Le Fort fractures are much the same signs as for ZMC and zygomatic arch fractures but signs are, for the most part, bilateral. Facial asymmetry, flattening or elongation may be evident in older children.

Management

Manage pain with non-pharmacologic and pharmacological measures. Oral and intravenous analgesia may be required but avoid intranasal analgesia in case of fracture.

Investigations

Facial bone x-rays may give you some valuable information, but the caveat is that they can be difficult to interpret in children. If you have a high clinical suspicion of a facial bone fracture, a CT scan is the imaging modality of choice and can be argued to be the cornerstone of investigation for facial bone fractures in children.

A chest x-ray may be indicated to exclude aspiration of foreign bodies or dental fragments.

Specific treatment

All fractures should be discussed with the local maxillofacial service and/or ophthalmology if orbital involvement is present. A formal ophthalmological review should be carried out as early as is feasible in children with any suspected midfacial fracture.

Most greenstick or minimally displaced fractures can be managed conservatively with soft diet, advice not to blow nose or hold nose closed while sneezing, antibiotic prophylaxis and a nasal decongestant.

There is no clear consensus on the best choice of antibiotic for facial fractures, and there is much variety among papers. The most commonly used would appear to be co-amoxiclav, cefuroxime, and clindamycin in penicillin-allergic patients.

Surgical intervention range from an intraoral approach for minimally displaced zygoma fractures to open reduction and internal fixation for comminuted fractures.

Potential complications, including mal/non-union, are less common in paediatric patients than in adults.

The do not miss bits

  • Reduced or lost vision, severe eye pain or proptosis of the globe are all features of retro-orbital haemorrhage, an ophthalmological emergency which requires immediate surgical intervention to avoid permanent blindness.
  • Although facial fractures are rare in children they have the potential to cause significant disruption to future growth, function & cosmesis and thus it is vital that they are recognised.
  • It takes significant velocity to cause a facial fracture in a child, and examination & investigations must be thorough to identify any other potential injuries. Consideration should be given to the possibility of non-accidental injury when assessing a child with a facial fracture.

Lucy’s CT brain & c-spine showed no significant abnormalities. CT facial bones showed a minimally displaced fracture of the frontal process of the right zygoma. She was reviewed by ophthalmology & maxillofacial specialists & was treated conservatively with oral antibiotics with a soft diet until her fracture had healed.

Selected references

Alcalá-Galiano A,  Arribas-García IJ,  Martín-Pérez MA, et al. Paediatric Facial Fractures: Children Are Not Just Small Adults. RadioGraphics. 2008; 28:441-461

Kumaraswamy SV, Madan N, Keerthi R, Singh DS. Paediatric injuries in maxillofacial trauma: a 5 year study. J Maxillofac Oral Surg. 2009; 8(2):150-153

Braun TL, Xue AS, Maricevich RS. Differences in the Management of Paediatric Facial Trauma. Semin Plast Surg. 2017;31:118-122

Cole P, Kaufman Y, Hollier LH. Managing the Pediatric Facial Fracture. Craniomaxillofac Trauma Reconstruction. 2009;2:77-84

Kidd AJ, Beattie TF, Campbell-Hewson G. Facial injury patterns in a UK paediatric population aged under 13 years. Emergency Medicine Journal. 2010;27:603-606

Mundinger GS, Borsuk DE, Okhah Z, et al. Antibiotics and facial fractures: evidence-based recommendations compared with experience-based practice. Craniomaxillofac Trauma Reconstr. 2015;8(1):64–78

The Royal Children’s Hospital Melbourne.The Paediatric Trauma Manual. Maxillofacial Injury. https://www.rch.org.au/trauma-service/manual/maxillofacial-injury/

Post ROSC care

Cite this article as:
Costas Kanaris. Post ROSC care, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.29109

Or some pointers on clinical management following the successful return of spontaneous circulation in children. 

It’s 5:40 am at Bubblesville ED. The red phone rings. The paramedic crew informs you that they are five minutes away with a 7 kg, 6-month-old, previously thriving, baby boy called Tarquin. He has had a witnessed out of hospital cardiac arrest at home and there was no prodromal illness according to the family. He choked during a breastfeed, turned blue, and stopped breathing. He had 5 minutes of CPR by the parents by the time the ambulance arrived and had ROSC by the paramedic team after a further 8 minutes. The rhythm strip was consistent with a PEA arrest. They are hand-ventilating him through an LMA.

This is his capillary gas on arrival in the ED
  1. What are your clinical priorities?
  2. What clinical problems do you anticipate in the immediate post-arrest phase?
  3. Who do you call for help?
  4. What do you do with the family whilst you’re managing the patient?
  5. What investigations do you need?

A systematic, collaborative, well-led approach to advanced paediatric life support can maximize the chances of the clinical team achieving a return of spontaneous circulation in a child that has arrested. We’ve seen the drill be before. We can go through our algorithms expertly and the 5-H’s and 5-T’s roll off the tongue, even under duress. The return of a pulse is heralded as the “hallelujah moment”, almost as if the patient is now healthy and safe and all we have to do is wait for the paediatric critical care retrieval team to arrive.

Whilst traditional APLS teachings are vital for the dissemination of knowledge and it’s application in everyday clinical life, their main focus is on the initial phase of achieving a pulse with very little attention placed on the all-important post-resuscitation phase.  This part of care is crucial, not only if we are to minimize secondary brain injury to the child but also to improve the chances of permanent returns of spontaneous circulation. Good short and long term outcomes rely heavily on how well we manage the post-resuscitation stage. 

There are four phases of cardiac arrest:-

Phase one: Prevention. This is the pre-arrest phase. Child safety and injury prevention strategies are in place to recognize deterioration. Adequate monitoring by using early warning systems and a pro-active approach to management is likely to contribute to avoiding an arrest.

Phase two: No flow arrest. This is a period of cardiac arrest prior to us commencing CPR. Our aim here is to minimize the time it takes to start life support. It is key that we involve the cardiac arrest team quickly, we start chest compressions early, and that we do not delay defibrillation if this is needed. 

Phase three: Low flow resuscitation. This phase describes when CPR is in progress. The aim is to achieve high-quality CPR in order to allow adequate coronary and cerebellar perfusion. Maintaining good ventilation and oxygenation whilst avoiding aggressive over-ventilation is paramount. It is during this phase that we systematically approach and threaten the reversible causes of cardiac arrest. 

Phase four: This is the post-resuscitation phase after ROSC has been achieved. Our aim here is to optimize coronary and cerebral perfusion. Neuroprotection and treatment of arrhythmias as well as treatment of post-cardiac arrest syndrome come under this phase. 

Adult v paediatric arrest: What’s the difference?

Out of hospital cardiac arrests in children >16 years of age are relatively rare – reported at 8-20/100000/ year. The incidence is only comparable to that of the adult population, estimated at 70/100000/year. The incidence of in-hospital paediatric arrests is much higher, with a nearly one-hundred-fold increase, compared to the out-of-hospital incidence for the <16’s. 

Survival, and especially a “good” survival from a neurological perspective still remain poor. Out of hospital survival rates are estimated to be 5 – 12%. Only 0.3 to 4% of those that survive have no long-term neurological insult.

Children have cardiac arrests due to severe respiratory insult or circulatory collapse, in the main. Either can lead to a respiratory arrest coupled with hypoxia, which then results in a cardiac arrest. The overwhelming majority of cardiac arrests present with a non-shockable rhythm.  It is also worth noting that almost half of the paediatric population that have a cardiac arrest have other chronic comorbidities such as respiratory conditions e.g. asthma, congenital cardiac disorders, or neurodisability. 

In the adult population, cardiac arrest is more likely due to long-term comorbidities such as ischaemic heart disease. This contributes to the development of an acute myocardial insult and (usually) a shockable rhythm. Understanding the difference in pathology leading to a cardiac arrest between adults and children is vital. Reversing the cause of the respiratory compromise can make the impalpable pulse palpable, allowing us to perfuse our patient once again. 

The recommended CPR ratio of 15:2 for children aims to provide adequate ventilation for oxygenation as well as satisfactory cardiac compressions to maintain sufficient perfusion of the coronary and cerebral circulation. Adult studies looking at compression-only CPR in patients with VF arrest have shown that success in achieving ROSC is due to pre-existing pre-arrest aortic blood oxygen and pulmonary oxygen stores.  As a mere 14% of cardiac arrests are due to a shockable rhythm, combining ventilation and compressions is vital. 

What is the Post Cardiac Arrest Syndrome (PCAS)? 

PCAS describes the period in which our patients are at the highest risk of developing ventricular arrhythmias and reperfusion injuries after ROSC. This is secondary to prolonged ischaemia then reperfusion of vital organs, primarily the myocardium and central nervous system. Its systemic effects are not dissimilar to those encountered in severe sepsis. There are four stages to PCAS:

  • Immediate post-arrest – First 20 minutes. 
  • Early post-arrest – 20 minutes to 6-12 hours. 
  • Intermediate phase – 6-12 hours up to 72 hours.
  • Recovery phase – From 72h onwards. 

Neuroprotection

Even high-quality closed-chest CPR can only achieve 50% of normal cerebral blood flow at best. It is not a secret that the brain does not tolerate hypoxia or ischaemia, the effects on both of these processes are exponential during a cardiac arrest, the longer the downtime, the worse the neurological hit

The pathophysiological cascade for neurodegeneration following cardiac arrest is complex and multi-factorial. Following a hypoxic or ischaemic period the brain develops cerebral oedema and cerebral hyperaemia. There is impaired cerebral vascular reactivity and like any other organ trying to reperfuse, the post-ischaemic biochemical cascade is activated. All these factors contribute to a secondary brain injury. Of course, the duration of hypoxia will in large part dictate how severe the primary brain injury is and whether the patient is likely to survive or not. Brain injury can manifest as myoclonus, stroke, seizures, coma, or brain death. 

We can minimize the extent of secondary brain injury with simple proactive, neuroprotective measures:

  • Strict normothermia
  • Aggressive seizure prophylaxis
  • Avoiding hypoxia and hyperoxia
  • Tight circulatory monitoring and support
  • Patient position
  • Eucapnia and normoventilation
  • Vigilant glucose monitoring
  • Frequent neurological assessment, especially before the administration of anaesthetic agents and paralysis

Strict normothermia

Therapeutic hypothermia following a cardiac arrest during the intermediate phase (after VF in adults), as well as newborns with birth asphyxia, has shown some correlation with better neurological outcomes and reduced neurodisability.  Similarly, there is strong evidence linking core temperature above 38° with worse neurological outcomes in patients following cardiac arrest. There is a wide variation in practice in relation to therapeutic hypothermia.  Mild hypothermia after paediatric cardiac arrest is in the policy of some PICU’s. Patients are cooled to 33-34°C for 1 – 2 days and are then gradually rewarmed. Paralysis can be used as an adjunct to stop shivering. Temperatures below 32°C should be avoided as they are associated with worse survival, immunosuppression, arrhythmias, coagulopathies, and infections.  The decision to “cool” must be made early and in conjunction with your critical care transport team. You have many tools at your disposal to achieve this such as cold IV fluids, cooling blankets, and catheters. 

What is, and should be, more aggressively targeted is strict normothermia (temperatures between 36-37°C), and depending on local practice hypothermia can be targeted to 33-36°C. Avoidance of pyrexia is crucial. Fever can result in an increased metabolic demand of the brain. This contributes to more ischemic injury and more infarcts as the threshold for ischemia in the injured brain is lower than that of the normal brain. The brain can no longer auto-regulate the mismatch between cerebral blood flow and metabolic demand.

Aggressive seizure prophylaxis

Seizures after paediatric cardiac arrest can occur in up to 47% of cases. 35% of these can lead to refractory status epilepticus.  Whilst CFAM/EEG monitoring is unlikely to be available in your local PED, it is important to have a low threshold to administer a long-acting anti-epileptic or a continuous infusion of a short-acting medicine to prevent/avoid this from happening. Ideally, a continuous infusion of midazolam +/- levetiracetam (less arrhythmogenic than phenytoin but both will work) and standard national guidelines should be followed. 

Clues as to whether a patient is still fitting include:

  • Unexpected changes in the pupillary size (beware of the child that had atropine on induction with the “fixed dilated pupils”).
  • Sudden changes in BP or heart rate.  

If you have given a paralytic for intubation, do not fall into the trap of thinking that the patient is not seizing, only an EEG or CFAM can tell you that. It is better to err on the side of caution.

Avoiding hypoxia and hyperoxia

Avoiding hypoxia and hyperoxia are also key components in minimizing secondary brain injury.  Whilst hypoxia will further exacerbate secondary brain injury, hyperoxia  (PaO2 > 40 kPa) is also be associated with worse survival due to oxygen free-radical formation that can inactivate intracellular enzymes, damage DNA, and destroy lipid membranes. It is reasonable to have high concentration oxygen therapy during the low-flow resuscitation and early post-resuscitation phases (as the commonest causes are respiratory). In the subsequent phases, we should target oxygen saturations between 94 and 96% and be proactive in how we reduce the FiO2 whilst avoiding hypoxia. There is a caveat in cases of severe anaemia or carbon monoxide poisoning. Then it is clinically appropriate for the highest concentration of oxygen to be administered.

Tight circulatory monitoring and support

Inotropic support may also be needed early. A degree of myocardial dysfunction/stunning is expected following CPR. To ensure adequate cerebral perfusion we need to target an age-specific, physiologically normal blood pressure. Both hypo and hypertension can exacerbate secondary brain injury. Because of this, monitoring the blood pressure through an arterial line is preferred. If the local set-up or skillset does not allow for arterial line placement, especially in smaller children, having non invasive blood pressure on 1-2 minute cycles can be a useful proxy.  

The paediatric myocardium is much more resilient than its adult counterpart.  If the arrest is not secondary to congenital heart disease the paediatric heart can regain normal function within 12-24 hours.  During the first 20 minutes following ROSC poor cardiac function is due to profound systemic vasoconstriction and cellular acidosis. We can support the myocardium by supplying adequate fluid resuscitation, targeting normal (age-appropriate) blood pressure and inotropic support. Point of care ultrasound, CVP monitoring, or assessing for hepatomegaly/rales if there is no access to the former, can help us prevent fluid overload

Inotrope choice is usually made with the help of the critical care team and depends on the balance between the need for inotropy and vasoconstriction.  Adrenaline is preferred for inotropy, noradrenaline for vasoconstriction.  Be aware that severe acidosis can cause catecholamine resistance, so giving some bicarbonate if the pH <7 may help your inotropes work better. Routine administration of bicarbonate has not been shown to improve clinical outcomes. There are some special circumstances in which we should consider its use such as cases of hyperkalaemia or hypermagnesaemia and arrests due to tricyclic antidepressant overdose. 

Patient position

The patient position that can achieve optimum cerebral perfusion is with the patient semi-sat up at a 30-45 degree angle.

Eucapnia and normoventilation

Avoidance of hypercapnia or hypocapnia is important in preventing secondary brain injury. It is, therefore, recommended that eucapnia is achieved by targeting a PaCO2 between 4.5 and 5.5 kPa. Hyperventilation can cause hypoxia and increase intracranial pressure due to hyperaemia, it can also cause further cerebral ischemia. As the intrathoracic pressures increase, cardiac venous return is impaired. Since the myocardium is already injured this can have catastrophic effects causing the BP to plummet and subsequently impair cerebral perfusion.

Vigilant glucose monitoring 

Following ROSC, children are also at risk of developing hypoglycemia (glucose <3 mmol/L). There is good evidence to suggest that hypoglycaemia negatively impacts neurological outcome and cause hypoglycaemic seizures, especially in the younger ages. Vigilant glucose monitoring and correction as per APLS guidelines is important. If regular dextrose boluses are needed, consider a continuous glucose infusion. If the patient mounts an adequate stress response, they may become hyperglycaemic.  There is no evidence to suggest that aggressive glucose control with insulin in the non-diabetic patient is beneficial; wait with watchful deliberation and the glucose will usually return to normal levels with no intervention.

Frequent neurological assessment

It is important to frequently assess neurological status frequently after ROSC as this can help us prognosticate. Take the time to do a very quick assessment ideally before the administration of anaesthetic agents and paralysis. Document clearly pupillary size/reactivity, GCS (and its break down) and any respiratory effort or gasping. 

Adjunctive investigations

Following ROSC a number of investigations will be needed to guide diagnosis and therapy. Routine bloods such as renal function, electrolytes, liver function tests, full blood count, and clotting are a basic standard. In cases of lactaemia and/or severe metabolic acidosis ammonia and toxicology is useful. Arterial blood sampling is invaluable to allow quick correction of any electrolyte abnormalities and help titrate ventilation settings and (in part) guide inotropic support. Arterial samples will also help uncover any exposure to carbon monoxide, especially in burns cases. 

From an imaging perspective, a chest X-ray is vital in ascertaining tube positioning and lung pathology as well as cardiac contours in case a congenital or acquired heart disease is suspected. Head CT is obviously useful in cases in keeping with traumatic arrest and NAI but timing of the CT and whether it should take place pre-departure to PICU or after depends largely on local trauma network protocols so should ideally be discussed with the regional trauma team lead and paediatric critical care transport team. 

Children that die or arrest unexpectedly in the UK are subject to a sudden unexpected death in infancy investigation (SUDI) so the appropriate referrals need to be made to the child protection team, police and social care. It is important to clarify that even near-miss cases merit triggering the same SUDI process to ensure that any NAI cases don’t slip through the net. 

Transport pearls

After ROSC the patients will need stabilisation and transfer to PICU for on-going management. Depending on the geographical location of your hospital and the availability of a critical care retrieval service you may have to transfer the patient yourselves or look after them until he/she is retrieved by transport team. A good transport and adequate neuroprotection can be achieved by applying these simple pearls: 

  1. Aggressive temperature monitoring and control between 33°C and 37°C.
  2. Monitor for seizures and pre-empt with long-acting antiepileptic accordingly.
  3. Correct electrolytes and hypoglycaemia and monitor frequently.
  4. Nurse the patient a 45° degree angle.
  5. Aim for a higher end of normal BP and use inotropes to achieve this. If you can’t insert an arterial line, have the NIVBP cycle every couple of minutes. 
  6. In cases of trauma, blood products should be used for volume. In an atraumatic arrest, balanced solution boluses are less harmful than 0.9% saline; don’t forget that you are still likely to need blood products. 
  7. Aim for a pCO2 of 4.5-5.5 kPa; use your continuous EtCO2 monitor to titrate ventilation. 
  8. Vigilant and through history/examination to rule out NAI. Free up a member of the team to do a thorough history from the family, always suspect NAI until proven otherwise especially in children under 6 months. 
  9. Know your anaesthetic drug side-effects (atropine dilates pupils for example so impairs our ability to monitor for seizures). Primum non nocere. 
  10. Intraosseous access can be used instead of a central line, have a low threshold to insert one and do it early.
  11. Have a member of the team check-in with the family every 10-15 minutes to explain what is happening, this is a bad day at work for you but probably the worst day of their lives. 

Conclusion

Achieving ROSC is an important step to give our patients a shot at survival. In some cases, achieving ROSC can only give us enough time to prognosticate and understand that survival is not possible. In some other cases ROSC can be the stepping-stone for a good, meaningful survival with a good quality of life. To achieve that, we must be able to apply good quality post–ROSC care and aggressive, pre-emptive neuroprotection. Learn the PCAS disease process to beat the PCAS disease process.  The APLS algorithm has become the bread and butter of anyone that is involved in paediatric care. Understanding and applying the principles of post-cardiac arrest syndrome is equally vital in improving survival outcomes for our patients. Learn the pearls, use them, teach them and I guarantee that it will make a difference.

Safety Netting

Cite this article as:
Carl van Heyningen. Safety Netting, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.28803

Safety netting is a cornerstone of paediatric practice. 

Children are a vulnerable group. Their condition can deteriorate and improve rapidly. Uncertainty is inherent in paediatric emergency medicine. From the child with a fever to the infant with vomiting, it is up to us to safeguard children from harm.  

Of course, we can’t admit every child to the hospital. Nor should we. The vast majority of patients in A&E return home with reassurance. Easy right? Reassure. Give advice. Send home. Simple?

You’re at the end of a busy shift and you have a train to catch. You’ve put in blood, sweat, and tears and feel happy your last patient has a simple throat infection. You advise fluids, paracetamol for symptoms of headache and neck pain and to come back if worried. The mother is anxious, you give them a leaflet on fever and rush out the door. 

Typically, our focus is on the front door of care not the exit. Discharge care is often overlooked. Even in the best of circumstances, we are under pressure to maintain patient flow especially as our emergency departments begin to become busy again. 

The next morning, coffee in hand, you walk in the department and hear the words that strike fear into the hearts of all that hear them… 

“Do you remember that child you saw yesterday? They’re being admitted to intensive care, it looks like meningitis.”

What could you have done differently? More tests? Not necessarily needed, no? More time? They had been observed and appeared well for several hours. Senior review? You’d seen them with the Consultant and agreed on the diagnosis. Then what? 

Safety netting

Why is it important? 

Acute illness remains one of the most important causes of childhood mortality in the UK. Early illness is notoriously non-specific. Take meningitis. In only half of cases, diagnosis is made at the first presentation. So what do we do? We must educate parents about uncertainty. Discuss the potential for deterioration. Explain the importance of seeking further help if necessary. We must safety net. 

What is it?

The term was first formally described in 1987. Today, it has come to mean “advice about what to do and what to look out for to empower parents and carers to seek help if the child’s condition deteriorates further or if they need more support.”  

What else should it cover? 

In addition to the above, it is critical to cover how they should seek help, what they should expect ahead (the disease course) and when to become worried.

How should it be done? 

Whilst verbal and written formats exemplify current practice, ranging from information leaflets to printed discharge letters, audiovisual and online resources are growing in abundance. Families report wanting this varied range of approaches.

Let’s consider the options. 

Face to Face – individualized, personal but highly variable and time-dependent

TOP TIP – be adaptable (don’t just simply recite the same information each time)

Social, educational, and cultural differences may all necessitate adapting your usual spiel in order to truly achieve understanding. Remember, the parents are in an unfamiliar, often noisy, and stressful environment in addition to feeling worried about their child. Expect them to be distracted a little. 

Leaflets – standardised, quality assured but not necessarily up to date and potentially bland and uninteresting. 

TOP TIP – use leaflets to re-enforce verbal information

When taken home written materials can often act as an aide memoire.  

Audio-visual – engaging and memorable with the potential to overcome literacy and language barriers if well designed, though resource intensive and expensive upfront   

Internet, social media, websites, apps – there are many innovative methods of connecting families with health information. Our responsibility is thus to navigate the clutter, signpost reliable resources, dispel myths and thus champion true evidence based materials. 

TOP TIP – keep the message simple, it can be easy to overwhelm parents with information. 

Remember, many parents may not wish to go on the internet. A few may even not have access to it. 

Why tailor the information? 

As ever, before talking we must first listen. For example, one interview based study found a mother worrying about “their child with cough dying at night through choking on phlegm.” If we do not listen to such fears how can we expect our own advice to be heard. 

Parental priorities

Parents priorities include… 

Emotional distress (addressing this)

Physical symptoms (addressing these)

Information (providing this, particularly reassurance, diagnosis and explanation)

Care (basic care, including food drink and friendliness)

Closure (finding out what’s wrong and, where possible, going home)

An awareness of these priorities can inform our conversations, helping us to better look after our patients from their perspective.

Ok, but what is the reality?

“You don’t actually know how much of that leaflet they’re gonna actually understand, take in, comprehend… going through things step by step, listening, understanding and explaining, I think is more beneficial” (Paediatric ED doctor). 

“It’s very difficult to know ‘cause often they’ll nod their heads and say “yes I understand everything you say” and walk off and they might have no idea what we’ve just said”  (ED staff nurse). 

“If you’ve got a sick child at home and they’re moaning at you, you haven’t got the time to go on the internet… you’ve got a child hanging off your leg going, “Mummy I feel poorly, mummy I want this, mummy I want that”” (Mother).

“My doctor did give like an information leaflet… and I did read through it, because when you’ve got a sheet at least you can find time to do that” (Mother).

So how can we do it best?

After reviewing the literature and FOAMed (see further resources) here are my top tips for giving the very best safety netting advice, enjoy! 

  • Sit down – it has been shown to increase the perception of empathy 
  • Verbalize back concerns – be explicit important conditions have been excluded 
  • Explain things – share reasoning, show your process 
  • Highlight red flags – signs that necessitate reattendance
  • Be specific – ‘If x happens, do y’
  • Reinforce – provide written leaflets
  • Avoid criticism, foster understanding – put yourself in their shoes 
  • Document advice – yes, write down what you said 

And as with all good communication, ensure a quiet, private area and avoid using jargon. 

Finally, directly ask if parents understand and are happy. Don’t assume they are. 

Here is one good example to get you started… 

“…your little guy is likely to continue to have vomiting and diarrhoea. If he remains well in himself, is drinking the amount of fluid we have discussed and is having wet nappies then he is unlikely to become dehydrated. If, however, he becomes drowsy, develops a fever or fails to stay hydrated please call this number and come back to us.” 

We must stop thinking of reattendance as a failure – patients do get worse and some need to return. 

Good quality safety netting means both you and your patients can get a better nights sleep. 

References

Gill P, Goldacre M, Mannt D, Heneghan C, Thomson A, Seagroatt V and Harnden A (2013) ‘Increase in emergency admissions to hospital for children aged under 15 in England, 1999–2010: national database analysis’, Archives of Disease in Childhood 98, 328–34.

Wolfe I, Cass H, Thompson MJ, Craft A, Peile E, Wiegersma PA, Janson S, Chambers T, McKee M: Improving child health services in the UK: insights from Europe and their implications for the NHS reforms. Bmj 2011, 342:d1277.2. 

Thompson MJ, Ninis N, Perera R, Mayon-White R, Phillips C, Bailey L, Harnden A, Mant D, Levin M: Clinical recognition of meningococcal disease in children and adolescents. Lancet 2006, 367(9508):397–403.3. 

Neighbour R. The inner consultation. Lancaster: MTP Press, 1987.

NICE guideline [NG143], Fever in under 5s: assessment and initial management, November 2019

Available at https://www.nice.org.uk/Guidance/Ng143/evidence

Almond S, Mant D, Thompson M: Diagnostic safety-netting. The British journal of general practice: the journal of the Royal College of General Practitioners 2009, 59(568):872–874

Jones CH, Neill S, Lakhanpaul M, et al. Information needs of parents for acute childhood illness: determining what, how, where and when of safety netting using a qualitative exploration with parents and clinicians. BMJ Open 2014;4:e003874.

Neill SJ, Jones CH, Lakhanpaul M, et al. Parent’s information seeking in acute childhood illness: what helps and what hinders decision making? Health Expect 2015;18:3044–56. 

Austin PE, Matlack R, Dunn KA, et al. Discharge instructions: do illustrations help our patients understand them? Ann Emerg Med 1995;25:317–20.

Scullard P, Peacock C, Davies P. Googling children’s health: reliability of medical advice on the internet. Arch Dis Child 2010;95:580–2.

Mackert M, Kahlor L, Tyler D, et al. Designing e-health interventions for low-health-literate culturally diverse parents: addressing the obesity epidemic. Telemed J E Health 2009;15:672–7.

Knight K, van Leeuwen DM, Roland D, et al. YouTube: are parent-uploaded videos of their unwell children a useful source of medical information for other parents? Arch Dis Child 2017;102:910–4.

CS Cornford, M Morgan, L Risdale, Why do Mothers Consult when their Children Cough?, Family Practice, Volume 10, Issue 2, July 1993, Pages 193–196

Body R, Kaide E, Kendal S, et al. Not all suffering is pain: sources of patients’ suffering in the emergency department call for improvements in communication from practitioners, Emergency Medicine Journal 2015;32:15-20.

(15) Jones, C.H., Neill, S., Lakhanpaul, M. et al. The safety netting behaviour of first contact clinicians: a qualitative study. BMC Fam Pract 14, 140 (2013)

Jones, C.H.D., Neill, S., Lakhanpaul, M., Roland, D., Singlehurst-Mooney, H. and Thompson, M., (2014) Information needs of parents for acute childhood illness: determining ‘what, how, where and when’ of safety netting using a qualitative exploration with parents and clinicians. BMJ Open 4 (1). 

Further resources

RCPCH (2015) Facing the Future: Standards for acute general paediatric services. RCPCH.

RCPCH Safe System Framework, resources accessed 19th November 2019,  https://www.rcpch.ac.uk/resources/safe-system-framework-children-risk-deterioration

Dr. Natalie May, MBChB, MPHe, MSc, PGCert Medical Education, FRCEM, FACEM, #CommunicatED 1: Discharge & Safety Netting in ED, available at https://www.stemlynsblog.org/communicated-discharge-safety-netting/

Bruera, Eduardo & Palmer, J Lynn & Pace, Ellen & Zhang, Karen & Willey, Jie & Strasser, Florian & Bennett, Michael. (2007). A randomized, controlled trial of physician posture when breaking bad news to cancer patients. Palliative medicine. 21. 501-5.

Sarah Jarvis, Medico-legal adviser

BSc MBBS MRCGP, Playing it safe – safety netting advice, available at https://mdujournal.themdu.com/issue-archive/issue-4/playing-it-safe—safety-netting-advice

Damian Roland, BMedSci (Hons) MBBS MRCPCH, PhD, TIGHTEN UP YOUR SAFETY NET #WILTW, available at 

https://rolobotrambles.com/tightenyoursafetynet/

Safety netting – a guide for professionals and parents of sick kids from GP Paedtips

Shame. How it affects patients and their relationships with health care professionals. https://abetternhs.wordpress.com/2012/11/16/shame/ 

The febrile infant conundrum

Cite this article as:
Dani Hall. The febrile infant conundrum, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.28850

It’s fair to say that febrile infants can be challenging. Often presenting with insidious symptoms but looking reasonably okay, they may still have life-changing or life-limiting illnesses like sepsis or meningitis. You could argue that we should take the view of eliminating risk, performing septic screens on all febrile babies, and admitting for IV antibiotics until their cultures are returned. The vast majority will have a benign viral illness but at least you can rest assured you didn’t miss a seriously sick infant.

And that’s what we did when I started my paediatric training back when the dinosaurs roamed the earth – every baby under 6 months (yes, you heard it right, 6 months) with a fever got a full septic screen, including lumbar puncture, and was admitted to the ward for at least 48 hours pending cultures. But, from a health economics point of view, this is, let’s just say, perhaps not the best way to allocate healthcare resources.

Over the years, researchers have tried to rationalise our approach to febrile infants. 2013 saw the first NICE fever in under 5s guideline; a year later a group from Spain published the Step by Step approach to identifying young febrile infants at low risk for invasive bacterial infection; and last year, the PECARN group published a clinical prediction rule for febrile infants under 60 days, which had excellent sensitivity and negative predictive values to rule out serious bacterial infections.

Last month, the Spanish group published an article looking at the external validity of the PECARN rule in their dataset.

Velasco R, Gomez B, Benito J, et al. Accuracy of PECARN rule for predicting serious bacterial infection in infants with fever without a source. Archives of Disease in Childhood Published Online First: 19 August 2020

PICO image

Before we plunge into the paper, let’s stop and think about a couple of important definitions here:

Serious bacterial infection (SBI) is used to describe bacteraemia, meningitis and urinary tract infections, also including infections such as pneumonia, skin, bone and joint infections, bacterial gastroenteritis and sometimes ENT infections.

Invasive bacterial infection (IBI) are infections where bacteria are isolated from a normally sterile body fluid, such as blood, CSF, joint, bone etc. An IBI is a type of SBI in a sterile site.

Who did they study?

Velasco’s group looked back at their registry of infants with a fever without source from a busy paediatric ED (> 50,000 presentations a year) in a tertiary hospital. To match the cohort in the PECARN paper, they used the following inclusion and exclusion criteria:

Inclusion: infants younger than 60 days who presented with a recorded fever, or history of recorded fever, of >38 C over an 11 year period between 2007 (when they started measuring procalcitonin) and 2018.

Exclusion: any infants whose history and/or examination pointed towards a focus, whose results didn’t include those used in the PECARN rule (absolute neutrophil count, PCT, urine dip), who didn’t have culture results, who were critically ill on presentation or who had a past history of prematurity, unexplained jaundice, previous antibiotics or other significant past medical history.

What were they looking for?

The group were interested to see how the PECARN rule fared in their dataset by looking at how many infants were predicted to be low-risk and yet had an SBI or IBI to assess the external validity of the rule.

What did they find?

1247 infants were included in this study. Of these, 256 (20.5%) were diagnosed with an SBI, including 38 (3.1%) with an IBI.

Of the 256 infants with an SBI, 26 (10%) were considered low risk by the rule. Of the 38 with an IBI, 5 were considered low risk (13.2%) by the rule. The PECARN rule would have missed 10% of infants with an SBI.

The PECARN rule’s sensitivity dropped from 97.7% in the original study to 89.8% and specificity dropped to from 60% in the original study to 55.5%.

So, how did Velasco’s group calculate the sensitivities and specificities of the PECARN rule for different groups in their dataset? They’ve nicely shown their data in 2 x 2 contingency tables in their figures. This is the data for SBI.

Table of data from Velasco study

So, we can see that sensitivity (those patients testing positive for the SBI as a proportion of all patients who definitely have SBI) = 230 / 256 = 89.8%. This means that 10.2% are falsely negative.

Specificity (those patients who test negative for SBI as a proportion of all of those who don’t have SBI) = 550 / 991 = 55.5%. This means that 44.5% are falsely positive.

What about infants with a really short duration of fever?

When the group looked at infants with a history of less than 6 hours of fever (n=684, a little over half of the cohort), the sensitivity dropped further to 88.6%.

Why did the PECARN rule perform less well in this study?

The authors offer up a number of suggestions, some of which are outlined below.

The populations may be slightly different. Although the authors attempted to exclude ‘critically ill’ infants from this study (as the PECARN study excluded ‘critically ill infants’), a precise definition wasn’t coded in the original Spanish registry. Instead, they excluded infants from this study if they were ‘not well looking’ or admitted to ICU. Because of the way the data was coded, some critically ill infants may have been included in this study’s dataset, skewing the results.

The Spanish database was of febrile infants without a source, excluding babies with respiratory symptoms, which may explain why the rates of SBI and IBI were much higher in this study than the PECARN database of febrile infants. So, although the PECARN rule was highly sensitive in their group of febrile infants, as in this study it may not perform so well in febrile infants without a source.

This study showed that the PECARN rule performed less well in infants with a short duration of fever. Overall, infants in the PECARN study had a longer history of fever at presentation – over a third of the PECARN infants had fever >12 hours compared to 11% in this study. Over half of the infants in this study presented within the first 6 hours. Blood tests are less sensitive in the first few hours of a febrile illness and this may well partially explain why the rule performed less well outside the PECARN dataset.

It’s important not to ignore this study’s limitations. The PECARN dataset recruited infants from multiple centres, while the registry for this study came from only one ED. As this study was a secondary analysis of a dataset, a power calculation wasn’t performed. Generally, a minimum of 100 cases is recommended for validating a model, but only 38 infants in this study had an IBI.

Study bottom line

This study showed that in the Spanish dataset of infants under 60 days with a fever without source, the PECARN rule performed less well than in the original study. This was especially true for infants with a short history of less than 6 hours of fever.

Clinical bottom line by Damian Roland

In Kuppermann et al’s original 2019 study febrile infants 60 days and younger were demonstrated to be at low risk of SBIs using 3 laboratory test results: Urinalysis, Absolute Neutrophil Count (ANC), and serum procalcitonin (PCT) levels. The study was well designed and therefore compelling in providing a framework in which to manage these challenging presentations. However, with respect to knowledge translation, external validity is critical. The availability of PCT is a significant limiting factor to being able to show the PECARN approach could be reproduced internationally. While PCT is used in Europe and Australia, it’s certainly not widespread in the UK where I practice, and then it is only used routinely in a very small number of hospitals. This makes Velasco and colleagues’ work really important as they were able to replicate the requirements of the original study and helps answer an important question: should centres start introducing PCT into their diagnostic pathology panels? The results of this study will be interpreted differently by different observers as ultimately the question is of risk tolerance. Personally, a 10% false-negative rate (if this is indeed the case) for an outcome that could result in long term disability feels uncomfortable. Counselling a parent that they could return home without treatment knowing this would probably be quite challenging. I am not sure many departments would be rushing to buy point of care PCT.

However, there are two very important caveats.  Firstly, is the validation cohort different from my own local cohort? The prevalence of disease has a huge bearing on the accuracy of any test. Knowing the local incidence of SBI and IBI in your own institution is important (but actually getting the numbers is harder than you may think!). It is likely that the PECARN approach may perform more effectively in other centres. Importantly the original paper highlights that implementation may be more effective in the second month of life due to the impact of HSV and other peri-natal infections present at 0-30 days. Secondly, what is the threshold for undertaking the blood tests in the first place? Fever in an infant less than 3 months is an interesting area as it’s one of the very few presentations in which a solitary symptom or sign is independently predictive of risk. Regardless of how the child appears to a health care professional, there is a risk of SBI and IBI (of anywhere between 2-10%) just by having a fever. This does mean that sometimes there is variation in approaches when there is a history of fever rather than a documented fever (for fear of not wanting to do a battery on tests on a neonate who in front of you appears completely well and has normal observations). But more importantly, this has led to an approach where although blood tests are taken, the results are often disregarded as an LP will be done and antibiotics will be given regardless. There are many cultural practices that have evolved around the management of the febrile neonate both within individuals and institutions. While in a study situation these are controlled for, their influence in the real world can not be underestimated and this is why it’s so important we have some pragmatic studies in this area.

This study makes me more determined to define our incidence of SBI locally and work out what impact new approaches to management may have. I think all centres should probably be doing this. However knowing the potential uncertainty in the sensitivity of the PECARN approach means it’s unlikely to be adopted in the immediate future without further validation.  

**post blog addendum 1st September 2020**

While this blog was in post production phase Kuppermann and colleagues have released further data on implementing their original predictive rule. This work has been summarised by Dr. Kuppermann below (click on to go to the original thread) and provides useful context to the discussion about external validity and implementation – DR.

Prehospital analgesia: part 2

Cite this article as:
Joe Mooney + Dani Hall. Prehospital analgesia: part 2, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.27501

You’re in the rapid response vehicle, having just handed over a 2 year old with a femoral fracture. As you clear the hospital, a call comes in: 8 year old, fall from slide, deformed left arm, conscious and breathing. When you arrive in the house you find him lying on the sofa, with bruising and deformity of his left elbow. The paracetamol and ibuprofen given by his mother has not controlled his pain*, so you take out a methoxyflurane inhaler and explain to him to suck in and blow out through ‘the whistle’. After a few breaths he begins to relax.

Methoxyflurane is a fluorinated hydrocarbon, used as an inhaled anaesthetic in the ’60s and early ’70s, until it fell out of favour after case reports describing renal failure at anaesthetic doses. But, when given in small doses, methoxyflurane has excellent analgesic properties, with no nephrotoxic side effects. It has been used extensively in Australia and New Zealand by prehospital clinicians as a self-administered analgesic for short-term pain relief in adults and children. After being licenced in 2015 in the UK and Ireland for the emergency relief of moderate to severe pain in conscious adults with trauma, methoxyflurane was included in the Irish prehospital CPG for EMTs, paramedics and advanced paramedics with permission under the seventh amendment to allow its use in children.

Added as a liquid to a Penthrox® inhaler, methoxyflurane vaporises, to be inhaled on demand. It has revolutionised prehospital pain control due to its quick onset and easy, pain free administration and, because of its light weight, crews can carry it over rough ground easily. Known as ‘the green whistle‘, each 3ml dose is quoted to last between 20 and 30 minutes, but in practice can sometimes last up to 45 minutes or an hour, depending on a child’s respiratory rate and depth and the way in which they self-administer. The Irish prehospital CPGs allow two inhalers to be administered in 24 hours to a patient, so when there’s an extended journey time, methoxyflurane inhalers used back-to-back can provide up to two hours of analgesia, which can be supplemented by the simple analgesics, paracetamol and ibuprofen, or morphine, fentanyl and ketamine, as needed.

But what’s the evidence for methoxyflurane in children?

Pop methoxyflurane in the PubMed search bar, and a lot comes up. It’s safe, it works, but there are surprisingly few randomised controlled trials (RCTs) that include children. A couple of observational studies are noteworthy. An Australian study in the prehospital setting, published in 2006 by Franz Babl and colleagues, describes an observational case series of 105 children, ranging in age from 15 months to 17 years, who received methoxyflurane while by being conveyed to hospital by ambulance. The children’s pain scores dropped from 7.9 to 4.5, with few side effects, although there was a tendency towards deep sedation in the under 5s. The following year Babl’s team published an ED-based observational case series of 14 children aged 6 to 13 years with extremity injuries who received methoxyflurane for painful procedures in the hospital setting. Although methoxyflurane was a useful analgesic agent, Babl’s team found it did not work as well as a procedural analgesic for fracture reduction.

The first double-blind RCT of methoxyflurane in children was published almost two decades ago by Chin et al in 2002. Forty-one children over the age of 5 with upper limb fractures were randomised to receive either methoxyflurane or placebo. Unsurprisingly, methoxyflurane resulted in a lower pain score at 10 minutes than placebo. Adverse events weren’t reported, but the apparent safety and efficacy of methoxyflurane demonstrated in this study paved the way the some bigger and better RCTs.

A better known, and more recent, RCT involving children was the STOP! trial, published in the EMJ in 2014. This randomised, double-blind placebo-controlled trial was conducted at six EDs in the UK. Three hundred patients, 90 between the ages of 12 and 17, with minor trauma (such as burns, fractures, dislocations and lacerations), were randomised to receive either methoxyflurane or saline via an inhaler. In a nifty way to keep the patients, doctors and nurses blinded to which drug was being administered, a drop of methoxyflurane was added to the outside of every inhaler so both drug devices smelled the same. Pain scores dropped significantly lower in the methoxyflurane group, with a median onset of action of 4 minutes. But what about those adolescents? Although 45 12 to 17 year olds were included in each group, their data wasn’t analysed separately, and children under the age of 12 were excluded from the study, so although we can probably assume methoxyflurane works well and is safe in adolescents, more trials would be helpful.

Segue to the Magpie trial, which is currently recruiting in the UK and Ireland via the PERUKI network. This international multi-centre randomised, double-blind placebo-controlled trial is specifically investigating the efficacy and safety of methoxyflurane in children and young people so that its UK license can be extended to include children. Like STOP!, participants are being randomised to either methoxyflurane or placebo (again saline) via an inhaler. To ensure younger children are well represented in the study data, the study team are aiming to recruit higher numbers of 6 to 11 year olds than adolescents, with a recruitment target of 220 children and adolescents in total. We’re awaiting the results eagerly…


*A top tip on top up dosing

This child had been given 500mg of paracetamol and 280mg of ibuprofen by his mother before the crew arrived. He was 8 years old, with an estimated weight of 31kg. Based on Irish CPGs allowing a paracetamol dose of 20mg/kg (620mg) and ibuprofen dose of 10mg/kg (310mg) he was underdosed. It’s important to top-up simple analgesics as part of your approach to pain relief in children.


But what happened to the 8 year old?

You check CSMs (circulation, sensation and movement) before and after applying a splint and transfer him to the ambulance on a stretcher. His pain is very well controlled, and he asks his mother to take a photo for his friends. This sentence is hard for him to say and he gets the giggles. You transfer him uneventfully to hospital where he’s diagnosed with a supracondylar fracture.

Read more about assessing pain, prehospital analgesia in children and the evidence behind intranasal fentanyl in part 1 of the DFTB prehospital analgesia series.

References

Hartshorn, S., & Middleton, P. M. (2019). Efficacy and safety of inhaled low-dose methoxyflurane for acute paediatric pain: A systematic review. Trauma21(2), 94–102. https://doi.org/10.1177/1460408618798391

Babl FE, Jamison SR, Spicer M, Bernard S. Inhaled methoxyflurane as a prehospital analgesic in children. Emerg Med Australas. 2006;18(4):404-410. doi:10.1111/j.1742-6723.2006.00874.x

Babl FE, Barnett P, Palmer G, Oakley E and Davidson A. A pilot study of inhaled methoxyflurane for procedural analgesia in children. Pediatric Anesthesia. 2007;17:148-153. doi:10.1111/j.1460-9592.2006.02037.x

Chin, R, McCaskill, M, Browne, G A randomized controlled trial of inhaled methoxyflurance pain relief in children with upper limb fracture. J Paediatr Child Health 2002; 38: A13–A13.

Coffey F, Wright J, Hartshorn S, et al. STOP!: a randomised, double-blind, placebo-controlled study of the efficacy and safety of methoxyflurane for the treatment of acute pain. EMJ 2014;31:613-618

Hartshorn, S., Barrett, M.J., Lyttle, M.D. et al. Inhaled methoxyflurane (Penthrox®) versus placebo for injury-associated analgesia in children—the MAGPIE trial (MEOF-002): study protocol for a randomised controlled trial. Trials 20, 393 (2019). https://doi.org/10.1186/s13063-019-3511-4