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.

If you enjoyed this post, why not check out our online courses at DFTB Digital

About Dani Hall, Rachael Mitchell and Sarah Davies

Dani Hall, Rachael Mitchell and Sarah DaviesIn 2018, Dani, Rachael and Sarah were asked to give some paediatric case-based teaching in the last session of a European EM conference. Having been warned that by the end of the day audience enthusiasm waned, they set out to make the session interactive, educational and funny. With the help of some clever and somewhat devious friends, Tomas, Bruno, Francesca and Gregory came to life in what later became known as #PEMadventures. The rest, as they say, is history.

Dani Hall, Rachael Mitchell and Sarah Davies
Author: Dani Hall, Rachael Mitchell and Sarah Davies In 2018, Dani, Rachael and Sarah were asked to give some paediatric case-based teaching in the last session of a European EM conference. Having been warned that by the end of the day audience enthusiasm waned, they set out to make the session interactive, educational and funny. With the help of some clever and somewhat devious friends, Tomas, Bruno, Francesca and Gregory came to life in what later became known as #PEMadventures. The rest, as they say, is history.

2 Responses to "PEM Adventures Chapter 1"

  1. Avatar
    Mohammad Osman 4 weeks ago .Reply

    Excellent
    Loved playing
    Waiting for the next one

  2. Avatar
    Andrea mcilroy 3 weeks ago .Reply

    Fantastic can’t wait for the next one.

Leave a Reply