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.

VP shunts

Cite this article as:
Angharad Griffiths. VP shunts, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.25996

Rhiannon is a 2 year old with spina bifida who had a VP shunt inserted during the first month.  It was revised because of a “blockage” at 18 months.  She has not been herself for the last 24hours; more lethargic than usual- especially this morning when she woke up where she also felt hot.  She vomited in the car on the way to the Emergency Department.  She has had previous urinary tract infections.

Rhiannon was afebrile on presentation in ED.  In triage, she was observed as being awake and drinking from a bottle but wasn’t perturbed by having her observations taken or finger-prick glucose.  Overall she was a little quiet in the ED and her mum was concerned that this was absolutely different from her baseline.  Her urine microscopy was not concerning.  She had a mild neutrophilia and a normal CRP.  Subcutaneous examination of her shunt, from her skull to the right clavicle was normal.  She vomited once in the ED.

 

This is a common presentation and one that can be challenging to manage. Missing a shunt problem can have catastrophic consequences. This post will take you through some pearls and pitfalls of managing children with VP shunts presenting to the ED.

 

What is a VP shunt?

A ventriculoperitoneal (VP) shunt is a medical device used to drain fluid via a pressure gradient, away from the brain for conditions of excessive cerebrospinal fluid (CSF).  The intention is to shunt fluid away and avoid excessive pressure on the brain.  It is one of the commonest performed neurosurgical procedures and is the treatment of choice for the vast majority of patients with hydrocephalus.

 

Shunts drain according to the differential pressure gradient between the ventricle and the tip of the distal catheter.  The ventricular end of the catheter is inserted through a burr hole in the right parietooccipital region and the valve often sits behind the right ear.  The distal portion is subcutaneously tunneled down into the abdomen where it’s positioned inside the peritoneal cavity.

The diagnosis of raised intracranial pressure in children with VP shunts is challenging.  The symptoms are non-specific and the commonest causes often benign.  Rhiannon could easily have a simple viral illness or her symptoms could be associated with rising intracranial pressure. Missing a shunt malfunction in these patients can be catastrophic.

 

Physiology of CSF circulation and drainage

  • CSF  is produced at a rate of approximately 20ml/h in children and adults. In normal circumstances, in a normal adult, CSF is recycled three times a day. The normal adult volumes of CSF (approximately 150mls) are reached by age 5.  The volume in a neonate can be estimated at  2ml/kg.
  • Around 50% of CSF is created by the choroid plexus of the lateral, third and fourth ventricles.  The rest of the CSF arises from the extracellular fluid of the brain.  CSF travels out of the foramen of Lushka and foramen of Magendie (at the level of the fourth ventricle), and heads through to the subarachnoid spaces, along the spinal cord to the cerebral hemispheres.  Over the cerebral hemispheres, the CSF is reabsorbed by arachnoid villi and then into the venous sinuses which drain into the jugular (internal) veins.
  • Intracranial pressure (ICP) rises when CSF production exceeds absorption.
  • Hydrocephalus is the consequence of the excessive accumulation of CSF. This could be from disruptions in formation, flow or absorption.
  • As hydrocephalus worsens and intracranial pressure increases, the temporal and frontal horns dilate, sometimes asymmetrically; and there’s elevation of the corpus callosum and stretching of white matter tracts.

 

Lateral = Lushka — Median = Magendie

 

Shunting CSF is an effective way to avoid the neurological damage that ensues if the build-up of CSF is left untreated.

Three shunts types are mainly used to shunt CSF: Ventriculoperitoneal (VP), ventriculopleural (VPL), and ventriculoatrial (VA). By far, the commonest are VP shunts.

 

Reasons for shunt placement

There is a myriad of reasons as to why a VP shunt should be placed.  They can be categorized into congenital or acquired causes.

 

Having a VP shunt in itself is a cause for concern for patients and caregivers.  Common areas for concern in paediatric patients include:

  • Flying and travel: there is no evidence that flying is dangerous but patients have concern over access to neurosurgical help should they need it. Shunt alert cards displaying the type of shunt are available.  The need for augmented travel insurance is also an area of concern.
  • Sports: Contact sports such as boxing are contra-indicated, and the US paediatric neurosurgeons named wrestling and football (soccer) as the commonest sports with adverse events.  Neurosurgeons in the UK are advising that football and rugby wearing a skullcap are acceptable.  You can still go scuba diving.
  • Future employment: in the UK, the Royal Air Force, Royal Navy and Police Force are the only services that preclude entry.
  • Programmable shunts and magnets: Background magnetic fields of household objects such as microwaves etc are safe as are walk-through metal detectors.  Post MRI check is advised with some programmable shunts but all are MRI safe.  The iPad2 has a strong magnetic field and can re-program some shunt valves, thus important to keep them at safe distances.
  • Shunt length: reassurance that placing sufficient length inside the abdomen will suffice and allow for growth.
  • Weight: Obesity is a risk factor for failure of VP shunts and dislodgement.

 

Shunt related complications

Failure rates are quoted as 30-40% at 1 year and 50% at 2 years in the paediatric cohort. A patient can expect to have 2-3 shunt revisions over the course of 20 years and the median time to shunt failure is just 1 and a half years. Paediatric revisions are more commonplace than adult revisions.

 

Risk factors for shunt failure include:

  • Younger patients (<6months), particularly neonates
  • Complex comorbidities, for example, cardiac, myelomeningocele, IVH’s, tumour and post-meningitic hydrocephalus, spinal dysraphism, and congenital hydrocephalus
  • Prior shunt failure and short time intervals between revisions
  • Male sex
  • Low socioeconomic status

 

Causes of shunt failure

Obstruction, blockage or occlusion

The commonest cause of shunt malfunction is proximal occlusion. There’s no clear data on whether programmable or non-programmable shunts are less likely to occlude.

It’s hypothesized that on insertion, the lumen can be obstructed with brain parenchyma from the cerebral cortex before reaching the ventricle, or choroid plexus when inserted near to the foramen of Monroe. It can also be occluded with blood following choroid plexus haemorrhage along with other cellular matter such as macrophages, giant cells, connective tissue, fibrin networks, debris, neoplastic cells.

Distal catheter blockage tends to occur later and when it occurs, it should raise the suspicion for an intra-abdominal pseudocyst or adhesions which may affect future peritoneal catheter placements.

Shunt infection

Shunt infection is the second most common reason for malfunction. The data is mixed, particularly as some older papers use data from before the time of antibiotic-impregnated catheters, skewing the data. Risk factors include young age (including neonates), post-op CSF leak, previous shunt infections, and the presence of a gastrostomy tube.

The vast majority of shunt infections are acute.  Far fewer late-onset infections have been reported. They can be attributed, mostly, to peritonitis, abdominal pseudocyst, bowel perforation, and haematogenous inoculation.

Shunt infection is associated with an increased risk of seizure disorder, decreased intellectual performance, and a two-fold increase in long term mortality. Re-infection occurs in one-quarter of children.

The proportion of shunt infections falls off rapidly after the first several months following implantation. The vast majority occur during the first 6 months. Children will present with signs of shunt failure, as well as systemic infection.  Fever is common and if we were to sample CSF (which is not often done in the ED), the presence of >10% neutrophils in the ventricular fluid is highly specific and sensitive of infection.

The commonest organisms are skin flora, including Staphylococcus epidermidis, Staph. aureus and gram-negative rods.  Infections with Staph. aureus and epidermidis are associated with an earlier onset as they are skin commensals. Infections with Staph. aureus are associated with a significantly increased likelihood of subsequent shunt infection.

Shunt fracture

This is often a late complication and almost always occurs along the distal portion between the valve and peritoneum.  With age, fibrous tissue becomes calcified and does not slide freely within the subcutaneous tissue then the tubing can crack.  This is more likely to happen in the neck where most movement occurs.

Tension can also form along areas of calcification causing tethering and stretching as the child grows.  It is important to note that early shunt fracture can occur and this could be a consequence of trauma to the tubing during surgery.

CSF can still drain resulting in an insidious duration of symptoms, clouding and confusing the diagnostic process.

Shunt series radiographs should always be sought in this cohort, though frequently fractures and disconnections are incidental findings during surveillance exams.

VP shunt fracture. Reused with permission from Education and Practice, Archives of Disease of Childhood

 

Shunt disconnections

Catheters are generally multi-component and hubbed together so disconnections can occur soon after surgery. The disconnection impedes the flow of CSF and it may still leak.  The onset of these symptoms may be slow.  Disconnections can happen at either the proximal or distal aspect of the valve.

Case courtesy of Dr Adam Eid Ramsey, Radiopaedia.org. From the case rID: 71794

 

Abdominal pseudocyst

This is a rare complication of VP shunts and is usually a late complication occurring years after initial placement. A pseudocysts is a fluid-filled sac that collects at the distal tip of the catheter.  It is thought that they form because of inflammation or due to abdominal adhesions.  It can present with abdominal pain or distention with, or without, a palpable abdominal mass.  Neurological symptoms occur when there is elevated ICP.

Shunt migration

The proximal or distal catheter tip may migrate.  With growth, the proximal catheter can withdraw from the ventricle (extremely rare), or the distal catheter can shift away from the peritoneum.  The distal tubing can become tethered and cause traction on some of the components causing a disconnection.  Distal migration occurs as the child grows.

 

Over-drainage

It is possible that a VP shunt can over-drain as well and ‘under-drain’.  With rapid over-drainage, the dura can be stressed and subdural haematomas and/or extra-axial fluid collections can form.

A slit ventricular syndrome occurs when gravitational forces exert a siphoning effect on the ventricles.  This effect is generally amplified by pressure.

 

Clinical presentation of shunt malfunction

Children with a blocked shunt can present with a myriad of symptoms including:

  • headache
  • nausea
  • vomiting
  • fever
  • irritability
  • abnormal level of consciousness

Infants and older children may present differently.

Infants

  • difficulty feeding
  • bulging fontanelle

Older children may present more specifically with

  • Nausea, somnolence, lethargy, cognitive difficulties, or eye pain.

 

Predictably, fever is commoner in children with shunt infections. Those with shunts because of myelomeningoceles may present with symptoms such as:-

  • weakness,
  • difficulty walking
  • bowel/bladder dysfunction
  • lower cranial nerve palsies.

Children present with these symptoms all the time to the ED. They are clearly not specific to a shunt problem.  As a consequence, diagnosing shunt malfunction on clinical grounds alone is incredibly difficult. Patients with shunt fracture or disconnection can present with a slow onset of symptoms.  They may have pain/tenderness localized to the area of fracture/disconnection or an area of calcification of an area of fluctuant swelling.

 

Diagnosis, evaluation, and imaging

The diagnosis of a shunt malfunction requires a combination of CT, shunt series radiographs, and occasionally (though seldom in the ED), CSF sampling.

A CT is likely to show an increase in ventricular size and occasionally, periventricular lucency representing oedema.  There may be increasing ventricular size on cross-sectional imaging but up to 15% will have “such profound alterations on brain compliance that their ventricles will not enlarge in the face of shunt failure and increased ICP”.  Ventricular size doesn’t appear to reach a plateau until approximately 14months after placement of the shunt (regardless of type implanted).

A lumbar puncture (LP) may demonstrate increased opening pressures, but not always.  It is also used for evidence of infection.  This not performed commonly in the ED in the context of possible shunt malfunction.

Shunt series (SS) radiographs are used to check the overall course of the catheter, looking for disconnection or disruption.  The series will not show obstructions, only damage to the catheter. It can rarely demonstrate complications such as a CSF pseudocyst (abnormal separation of bowel loops near the catheter tip) but shouldn’t be relied upon for this.

The number of radiographs needed varies according to the size of the child.  It is usually 3-4 radiographs, including two views of the skull and the continuous trajectory of the shunt tubing down the neck, chest, and then looping into the abdomen.

If a series is performed after the scan, theoretically a 2 view skull radiographs can be eliminated, provided that the chest x-ray includes the base of the neck. Unnecessary radiation may then be avoided.

The use of ultrasound is an area of ongoing research and has been largely unvalidated in children with VP shunts.

 

No clear cause for Rhiannon’s symptoms was found following a thorough examination.  A CT was performed because of concern over shunt failure. Her ventricles were noted to be slightly larger than a CT performed previously.  Shunt series radiographs showed continuous, non-kinked tubing.  She was admitted under the care of the Neurosurgeons and her shunt was revised.  No physical reason for shunt obstruction was found.

 

Selected references

Mansson PK, Johansson S, Ziebell M, Juhler M. Forty years of shunt surgery at Rigshospitalet, Denmark: A retrospective study comparing past and present rates and causes of revision and infection. BMJ Open. 2017;7(1).

Berry JG, Hall MA, Ph D. A multi-institutional 5 year analysis of Initial and multiple ventricular shunt revisions in children. Neurosurgery. 2008;62(2):445–54.

Pople IK. Hydrocephalus and shunts: What the neurologist should know. Neurol Pract. 2002;73(1).

Paff M, Alexandru-Abrams D, Muhonen M, Loudon W. Ventriculoperitoneal shunt complications: A review. Interdiscip Neurosurg Adv Tech Case Manag. 2018;13(June 2017):66–70.

Gonzalez DO, Mahida JB, Asti L, Ambeba EJ, Kenney B, Governale L, et al. Predictors of Ventriculoperitoneal Shunt Failure in Children Undergoing Initial Placement or Revision. Pediatr Neurosurg. 2016;52(1):6–12.

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Simon TD, Butler J, Whitlock KB, Browd SR, Holubkov R, Kestle JRW, et al. Risk factors for first cerebrospinal fluid shunt infection: Findings from a multi-center prospective cohort study. J Pediatr [Internet]. 2014;164(6):1462-1468.e2. Available from: https://dx.doi.org/10.1016/j.jpeds.2014.02.013

Buster BE, Bonney PA, Cheema AA, Glenn CA, Conner AK, Safavi-Abbasi S, et al. Proximal ventricular shunt malfunctions in children: Factors associated with failure. J Clin Neurosci [Internet]. 2016;24:94–8. Available from: https://dx.doi.org/10.1016/j.jocn.2015.08.024

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Tuli S, O’Hayon B, Drake J, Clarke M, Kestle J. Change in ventricular size and effect of ventricular catheter placement in pediatric patients with shunted hydrocephalus. Neurosurgery. 1999;45(6):1329–35.

DeFlorio RM, Shah CC. Techniques that decrease or eliminate ionizing radiation for evaluation of ventricular shunts in children with hydrocephalus. Semin Ultrasound, CT MRI [Internet]. 2014;35(4):365–73. Available from: https://dx.doi.org/10.1053/j.sult.2014.05.002

Smyth MD, Narayan P, Tubbs RS, Leonard JR, Park TS, Loukas M, et al. Cumulative diagnostic radiation exposure in children with ventriculoperitoneal shunts: A review. Child’s Nerv Syst. 2008;24(4):493–7.

An approach to the floppy infant

Cite this article as:
Taryn Miller. An approach to the floppy infant, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.25882

You are a junior doctor doing a rotation in neonates. Your registrar asks you to assess a 2-day old baby who was found to be hypotonic on their baby check. They ask you your approach to assessing the “floppy infant”. Luckily, you have a stepwise approach to answer this question ready!

 

Step 1- Definition and terminology

What does the term floppy mean?

The word floppy can be used to mean:

  1. A decrease in muscle tone (hypotonia)
  2. A decrease in muscle power (weakness)
  3. Ligamentous laxity and an increased range of joint mobility

What does the term hypotonia mean?

It is defined as “resistance to passive movement around the joint

It’s assessed in two ways by clinicians 

  • Phasic tone: assessed by the response of the muscle to a rapid stretch (tendon reflexes)
  • Postural tone: measured by the response of the muscle to a sustained low-intensity stretch (maintaining posture against gravity = significant head lag on pull-to-sit, ragdoll posture on ventral suspension, slipping through the hands when the infant is held under their arms).

With that in mind, you go on to start your approach

 

Step 2 – A focused history

Discuss with mother and review the notes focusing in on specific risk factors that could give you a clue to the diagnosis

  • Antenatal history – Reduced fetal movements, polyhydramnios, breech presentation 
  • Family history – Muscle disease, stillbirth or consanguinity
  • Birth History – Labour, delivery, resuscitation, Apgar score and cord gases
  • History since delivery- Respiratory effort, feeding history, level of alertness, level of spontaneous activity and character of cry

 

Step 3 – Examination and clinical clues

As always, your examination should start with a top to toe assessment of the baby using an A-E approach. Specific to the floppy baby is your neurological examination.

Some clinical clues that may further help you:-

  • Poor swallowing ability as indicated by drooling and oropharyngeal pooling of secretions
  • The cry!!  Infants with consistent respiratory weakness have a weak cry
  • Paradoxical breathing pattern – intercostal muscles paralyzed with intact diaphragm

It is important to determine whether the hypotonia is central (upper motor neuron) or peripheral (lower motor neuron).

*open mouth with tented upper lip, poor seal when sucking, lack of facial expressions, ptosis

TIP- Examine the baby with mum in a familiar environment to increase the likelihood of the baby being alert but not unsettled or crying.

Remember that in the neonatal period, central causes account for two-thirds of all cases, with hypoxic ischaemic encephalopathy being the most common.

Now you have narrowed down the likely lesion type let’s think of some aetiologies. Time to think back to the corticospinal tract that you learned all those years ago in medical school to help you.

 

 

Step 4 – Investigations

So what next? Let us decide which investigations we think are appropriate according to our central or peripheral causes.

Central hypotonia

1st line to consider

  • Serum (Ca, Glucose, U&Es, Mg, PO4, LFTs, VBG, Lactate and ammonia)
  • Septic screen
  • Plasma AA
  • Urine organic acids
  • Cranial Ultrasound
  • Microarray CCG

2nd line to consider

  • MRI
  • EEG
  • Urine sample
  • Congenital viral infections

Peripheral hypotonia

1st line to consider

  • CK,
  • DNA for Muscular Dystrophy
  • Genetic testing for SMA
  • EDTA for Prader Willi
  • CXR
  • Echo
  • Microarray CCG

2nd line to consider

  • Neurology services for EMG/ NCS, muscle biopsy

 

Step 5 – Formulating a management plan

Management plans will differ from case to case but should include a multi-disciplinary team approach.

  • Hypotonia can cause a loss of airway control and diminished breathing effort therefore some babies will need:
    • Resuscitation at birth
    • Assistance in maintaining airway
    • Ongoing respiratory support
  • Regular physiotherapy: stretches aimed at the prevention of contractures, positioning.
  • Occupational therapy: important to facilitate activities of daily living
  • Vigorous treatment of respiratory infections, including annual influenza vaccination
  • Feeding strategies – Nasogastric tube or gastrotomy
  • Management of gastro-oesophageal reflux.
  • Evaluation and treatment of cardiac dysfunction
  • Parental counseling

Later:

  • Prevention and correction of scoliosis with orthopaedic input
  • Consideration to the ethical appropriateness of & considerations to the ethical appropriateness of CPR in the event of acute respiratory arrest
  • Follow up of general development and stimulation of learning.

Please note that with advances in treatment of SMA and potential gene therapy in DMD, early diagnosis is important. Initiation of early treatment is recommended for individuals with infantile-onset (Type 1) and pre-symptomatic SMA.

 

Selected references

Ahmed MI, Iqbal M, Hussain N. A structured approach to the assessment of a floppy neonate. J Pediatr Neurosci. 2016;11(1):2-6. doi:10.4103/1817-1745.181250

Leyenaar J, Camfield P, Camfield C. A schematic approach to hypotonia in infancy. Paediatr Child Health. 2005;10(7):397-400. doi:10.1093/pch/10.7.397

https://ggnc.azurewebsites.net/ggc-paediatric-guidelines/ggc-guidelines/neonatology/evaluation-of-the-floppy-infant/

Guillain-Barre Syndrome

Cite this article as:
Aoife Fox. Guillain-Barre Syndrome, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.25051

A 2-year-old girl, Amy, attends the emergency department. Her father says that for the last 24 hours she has been refusing to walk. Prior to this, she was running amuck without difficulty. In the ED, you notice that she is now having difficulty crawling. She has no significant medical history but did have fever along with a runny nose and cough 2 weeks prior to her attendance which her parents managed with paracetamol at home.

 

What is Guillain-Barré Syndrome?

Guillain-Barré Syndrome (GBS) is a group of acute immune-mediated polyneuropathies.  It most commonly presents as an acute monophasic, paralyzing illness provoked by a preceding infection.

It is the most common cause of acute flaccid paralysis in children. The annual incidence is 0.34 to 1.34 cases per 100,000 in under the 18s, which makes it less common in children than in adults. It rarely occurs in children younger than 2 years, but when it does affect younger children because GBS is way off our radars, this can make it really tricky to diagnose. Boys are affected more often than girls.

 

What causes it?

It’s thought that an immune response cross-reacts with the myelin or axon of peripheral nerves due to molecular mimicry. Similar peptide sequences between the body’s own peptides and foreign peptides sometimes cause the immune system to get confused and attack its own tissues.  You probably knew that myasthenia gravis is due to auto-antibodies against the acetylcholine receptor but did you know that the receptor shares a 7 amino acid sequence with HSV, the herpes simplex virus? It’s thought that exposure to HSV may be the precipitant for myasthenia gravis.

Approximate 2/3 of patients give a history of an antecedent respiratory tract or GI infection. Campylobacter infection is the most commonly identified precipitant and can be demonstrated in as many as 30% of cases. Other infectious precipitants include CMV, EBV, Mycoplasma pneumoniae, and influenza-like illnesses.

Other suggested triggers include immunization, although there is no clear causal relationship several cases suggest an association, as well as one with trauma and surgery.

What about Guillain Barre and COVID?

There have been several case studies reporting GBS associated with SARS-CoV-2 during the COVID-19 pandemic. Given the small number of cases, it is unclear whether severe neurological deficits are typical features of COVID-19 associated GBS. An answer to the diagnostic conundrum of whether the respiratory compromise in COVID19-associated-GBS is due to coronavirus or muscle weakness is yet to be answered.

 

How can I recognize it?

GBS classically begins with paraesthesia in the extremities – fingers and toes –  followed by lower extremity symmetric, or modestly asymmetric, weakness that ascends up the body. In severe cases, the muscles of respiration are affected, in about 10-20% of children.

Cranial neuropathy can also occur, most commonly affecting facial nerves, causing bilateral facial weakness.

Autonomic dysfunction occurs in approximately half of children with GBS: cardiac dysrhythmias, orthostatic hypotension, hypertension, paralytic ileus, bladder dysfunction, sweating.

Physical exam typically reveals:

  • Symmetric weakness
  • Diminished or absent reflexes
  • Gait abnormalities
  • Sensory symptoms include pain, paraesthesia (reflecting nerve irritability)

Generally, children have shorter clinical courses and more complete recoveries in comparison to adults. A child’s function typically deteriorates for 2-4 weeks followed by a slow return of function over the coming weeks to months.

 

On examination, you find a quiet child who is otherwise acting appropriately. She is afebrile and the rest of her vitals are within normal limits. No bruises or rashes are observed on her skin and there is no evidence of trauma. Cardiovascular, respiratory, abdominal and ENT exams are unremarkable. Her extremities are warm and well perfused with normal pulses. There is no bony tenderness or deformities on palpation of her limbs. On neurological examination, she is moving all 4 limbs spontaneously. However, she will not bear weight or stand. Both her lower limbs are weak on exam. Her grip strength is reduced and when given a toy, it falls. Both upper and lower extremity reflexes are absent.

 

Subtypes

GBS most commonly presents in the classical way above: a mixed motor and sensory polyneuropathy with lower limb pain and ascending weakness. This is the classic Acute Inflammatory Demyelinating  Polyradiculopathy (AIDP), which accounts for 85 to 90% of cases in the developed world. But, there are a few other subtypes of GBS you should be aware of.

Acute Motor Axonal Neuropathy (AMAN), is a purely motor from of GBS, occurring mainly in Asia, Central and South America and associated with a preceding Campylobacter infection. Its clinical features are similar to AIDP, but respiratory failure is more common.

Acute Motor-Sensory Axonal Neuropathy (AMSAN) is similar to AMAN but with more sensory symptoms. The course tends to be prolonged and severe but is pretty uncommon in children.

Miller-Fisher syndrome is characterized by an external ophthalmoplegia, ataxia and muscle weakness with areflexia. It affects adults more commonly than children but should definitely be on your radar in a child presenting with cranial nerve and lower limb neurology.

 

How can it be diagnosed?

The initial diagnosis of GBS is based on the history and clinical exam – be suspicious of a child with lower limb weakness, weak reflexes and a preceding illness. Use investigations to confirm your suspicion.

CSF

CSF protein above 45mg/dL with a normal WCC count is present in 50-66% of patients in the first week after symptoms onset and ≥75% of patients in the third week. This disconnect between protein and white cells is called albuminocytologic dissociation.

Gadolinium-enhanced MRI of Spine

MRI will show contrast enhancement of the spinal nerve roots, cauda equina or cranial nerve roots. These changes aren’t specific to the GBS, but can be helpful in the correct clinical setting.

Nerve conduction studies

This is the most specific and sensitive test available for GBS, abnormal in up to 90% of cases. The test can be technically difficult in small children.

Antibodies

Antibodies against GQ1b (the ganglioside component of a nerve) are present in the vast majority of patients with Miller-Fisher syndrome.

 

In the emergency department, you send baseline bloods (FBC, U&E, LFTs and CRP) which are all normal and organize a CT head under sedation which is unremarkable. After getting consent from her parents you perform a lumbar puncture. The CSF appears clear. It has no red blood cells, 2 white blood cells and CSF glucose is within the normal limits but her protein is mildly elevated. No organisms were seen on gram stain and cultures had no growth after 5-days. You refer her to the neurology team for further investigation.

 

What else could it be?

The differential diagnosis of GBS is long.

 

Brain

  • Bilateral strokes
  • Acute disseminated encephalomyelitis
  • Acute cerebellar ataxia syndrome
  • Psychogenic symptoms

Spine

  • Anterior spinal artery syndrome
  • Compressive myelopathy
  • Transverse myelitis
  • Poliomyelitis
  • Infectious causes of acute myelitis

Peripheral nervous system

  • Chronic inflammatory demyelinating polyneuropathy
  • Critical illness polyneuropathy
  • Infection-related radiculitis (e.g. HIV, CMV, Lyme disease)
  • Thiamine deficiency
  • Toxins: biologic toxins (diphtheria), heavy metals (arsenic)
  • Vasculitis
  • Metabolic and electrolyte disorders (e.g. hypoglycaemia, hypophosphatemia)

Neuromuscular junction

  • Botulism
  • Myasthenia gravis
  • Neuromuscular blocking agents

Muscle

  • Acute inflammatory myopathies (e.g. dermatomyositis, polymyositis)
  • Acute viral myositis
  • Acute rhabdomyolysis
  • Critical illness myopathy
  • Metabolic myopathies (e.g. hypokalaemia, hyperkalaemia)
  • Mitochondrial myopathies

 

What is the treatment?

The mainstay of treatment is supportive management including close monitoring of motor, autonomic and respiratory function as well as pain management and prevention of immobility complications, such as pressure ulcers. ICU admission for mechanical ventilation will be required in 10-20% of kids. This is more likely to be needed in children with:

  • rapidly increasing weakness,
  • bulbar dysfunction,
  • bilateral facial weakness or

In addition IV immunoglobulin (IVIG) and plasmapheresis (plasma exchange) can be used in children with severe, progressive GBS (i.e. worsening respiratory status or need for mechanical ventilation, rapidly progressing weakness, inability to walk unaided or significant bulbar weakness).

IVIG  is typically preferred to plasmapheresis in children due to its better safety record and ease of administration

Plasmapheresis can be useful in bigger children where technically it is more feasible to perform. However, there are no reliable studies to suggest one has better efficacy than the other in children.

 

During her admission, Amy has a Gadolinium-enhanced MRI of the spine and nerve conduction studies which are consistent with the acute inflammatory demyelinating polyradiculopathy (ADIP) subtype of GBS. She is given IVIG. She does not develop any respiratory complications. On discharge after three weeks, her weakness is greatly improved and completely resolves over the next two months.

 

Bottom line

  • Clinical examination is key – do not forget to examine reflexes!
  • Always ask about recent viral illnesses.
  • GBS is the most common cause of acute flaccid paralysis in children and 10-20% will require mechanical ventilation.

 

Selected references

Bloch SA, Akhavan M, Avarello J. Weakness and the Inability to Ambulate in a 14-Month-Old Female: A Case Report and Concise Review of Guillain-Barre Syndrome. Case Rep Emerg Med [Internet]. 2013 [cited 2020 Apr 11];2013. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3572648/

 Yuki N, Hartung H-P. Guillain–Barré Syndrome. New England Journal of Medicine [Internet]. 2012 Jun 14 [cited 2020 Apr 5];366(24):2294–304. Available from: https://doi.org/10.1056/NEJMra1114525

Rudant J, Dupont A, Mikaeloff Y, Bolgert F, Coste J, Weill A. Surgery and risk of Guillain-Barré syndrome: A French nationwide epidemiologic study. Neurology. 2018 25;91(13):e1220–7.

Hicks CW, Kay B, Worley SE, Moodley M. A clinical picture of Guillain-Barré syndrome in children in the United States. J Child Neurol. 2010 Dec;25(12):1504–10.

Dimachkie MM, Barohn RJ. Guillain-Barré Syndrome and Variants. Neurol Clin [Internet]. 2013 May [cited 2020 Apr 5];31(2):491–510. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3939842/

Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barré syndrome. The Lancet [Internet]. 2016 Aug 13 [cited 2020 Apr 5];388(10045):717–27. Available from: https://www.sciencedirect.com/science/article/pii/S0140673616003391

Ryan MM. Pediatric Guillain-Barré syndrome. Curr Opin Pediatr. 2013 Dec;25(6):689–93.

Hughes RAC, Wijdicks EFM, Barohn R, Benson E, Cornblath DR, Hahn AF, et al. Practice parameter: Immunotherapy for Guillain–Barré syndrome. Neurology. 2003 Sep 23;61(6):736.

Headaches Module

Cite this article as:
Anna McCorquodale. Headaches Module, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.27380
TopicHeadaches
AuthorAnna McCorquodale + Arie Fisher
Duration1-2 hours
Equipment requiredNone
  • Basics (10 mins)
  • Main session: (2 x 15 minute) case discussions covering the key points and evidence
  • Advanced session: (2 x 20 minutes) case discussions covering grey areas, diagnostic dilemmas; advanced management and escalation
  • Sim scenario (30-60 mins)
  • Quiz (10 mins)
  • Infographic sharing (5 mins): 5 take home learning points

We also recommend printing/sharing a copy of your local guideline.

https://www.headsmart.org.uk/symptoms/sam-animation/ (2 min video) headsmart website contains around 20 min of reading around symptoms which could suggest an intracranial tumour

https://pemplaybook.org/podcast/pediatric-headache-some-relief-for-all/ (30 mins)
A good podcast including risk stratification, diagnosis and management of headache 

https://www.nice.org.uk
Quality standard 42 and clinical guideline 150

https://pemcincinnati.com/blog/headaches-in-the-pediatric-ed/

https://www.pemcincinnati.com/podcasts/?p=89

This is a lecture and a podcasts from PEM Currents which talks succinctly about the emergency management of migraine in ED and the likely treatment outcomes.

https://www.researchgate.net/publication/327473427_Cognitive_bias_in_clinical_medicine

https://miro.medium.com/max/800/1*yN2Xhv-M5PPerWzDVNt3sw.jpeg

Lecture by a US doctor specialising in headaches. Great review over the first 18 min of both the history and examination red flags and where she considers imaging. The second half is a question and answer session which is less useful.

(30 min video)

This is a lecture from a 2016 conference. It is pretty involved in the chronic management of headaches in children so quite advanced for the majority of attendees but might interest paediatricians who run follow up with outpatient clinics.

Headaches are common in children with 75% of children having experienced a headache by age 15. Our primary jobs in emergency are to provide effective symptom relief and filter out the headaches that are more likely to be concerning in their origin. The aetiology of headaches relies enormously on the history and, even in sinister causes, there are often few or no examination findings.

The most common cause of headache in children is a viral infection and the most worrying an intracranial space occupying lesion. Happily the latter is rare.

A good history is key:

  1. Onset, gradual or sudden?
  2. Location & severity, these questions will be incredibly age dependent. Younger children find localisation of pain difficult.
  3. Duration of the problem? How many times has this been reviewed and by who. Is there a lead professional. Be aware of cognitive bias created at this point by knowing what labels others have used. Here there should also be some investigation into what treatment strategies have been tried previously.
  4. Timing and associated symptoms? Compare a morning headache with some nausea/vomiting to one which worsens during the day and is relieved with sleep.
  5. Background medical history
  6. Family history sp of migraines. The presence or absence of this can be incredibly important when establishing aetiology. High prevalence of migraines running in families.

Full examination is necessary but here it should be stressed that abnormal neurological findings are rare in children with headaches. Where they exist there is a clear reason to pursue investigation by imaging, however, their absence cannot be wholly reassuring.

A 12 year old boy is brought to ED with a headache. He does not ordinarily suffer from headaches but today came home from school with a throbbing headache on the right side of his head. It is now 8pm and there has been no change. He has never been seen for headaches before but had a number of attendances for abdominal pain between the ages of 5 & 9 years.

On examination  you see an afebrile child, holding his head with his eyes closed. His neurology is otherwise normal (GCS 14 M 6 V 5 E 3).

What additional information would you consider important in the history?

What would be your next management steps?

Would you discharge this child?

With the additional history what are we trying to establish? Is this a primary or secondary headache. Here the presence of a family history of migraine can contribute to a likely ‘primary headache’ history. We also need to be clear about whether there is any possible infectious aetiology. How do we distinguish between a viral infection with a headache and meningitis? 

Clinical features suggestive of meningitis in children: a systematic review of prospective data, Curtis et al, Pediatrics 2010 – Having either a bulging fontanelle or neck stiffness (older child) increases the likelihood of meningitis by 8 fold

Without either of the above treating symptoms and re-evaluating seems reasonable.

  • The ongoing management of this child is based around clinical judgement of the underlying cause. There aren’t any features of space-occupying lesion (SOL) so it seems reasonable to proceed with either simple analgesia (with an antiemetic if migraine seems likely).

Treatment of pediatric migraine headaches: a randomized, double-blind trial of prochlorperazine versus ketorolac, Brousseau et al, Annuls of Emergency Medicine 2004

  • Migraine symptoms more optimally treated with analgesia with additional antiemetic with >90% resolution of symptoms at 3 hours compared to either analgesia alone (55% symptom resolution) or antiemetic alone (85% resolution)
  • https://bpna.org.uk/uploads/pet/chat-pre-course-june-2017.pdf Offer combination therapy with an oral triptan and an NSAID,or an oral triptan and paracetamol,for acute treatment of migraine with or without aura.
  • https://www.rch.org.au/clinicalguide/guideline_index/Headache/ Acute migraine management on presentation to ED to consider is Chlorpromazine 0.25mg/kg (max 12.5mg) IV over at least 30 minutes with 10 – 20mL/kg sodium chloride 0.9% (max 1L); may cause hypotension, monitor BP.

A 2 year old girl is brought to your emergency with a headache. She has been unsettled at night and wakes slapping the back of her head. She has been seen on four previous occasions over a 5 month period with similar presentations (varying grades of staff involved including a paediatric consultant).

The first presentation was related to the appearance of her molars, an MRI was booked but cancelled as symptoms had resolved when the molars erupted. Subsequent attendances have been documented as teething.

The parents history corroborates the above. She is waking at night with increasing frequency and have come today because it has been worse over the past week. Full neurological examination is normal and she is developing normally. There are some areas of white bulging on the lower gums.

Are there any features here which suggest additional investigation is necessary?

  • If so, what would you plan?
  • If not, how do you proceed

What non-medical features of this cases should we be aware of?

  • There are red flags in this history, however, due to the ages of the child these are difficult to clearly elicit through the history. Waking at night holding the occiput would seem unusual and a primary headache at this age is less likely.
  • Given that clinical examination findings are unlikely even in sinister diagnoses, we should endeavour to find those red flags embedded in the history.
  • There are no neurological examination findings and the behaviour change is not sustained so here evidence would suggest that, although imaging is required, it should be of the most optimal type ie MRI

Children with headache suspected of having a brain tumor: a cost-effectiveness analysis of diagnostic strategies, Medina et al, Pediatrics 2001

  • Here is an optimal time to discuss cognitive bias. Many people had seen this child before, it is easy to ‘plan’ an MRI in a teaching session on headaches but in reality, if your consultant had seen and discharged this child with a diagnosis of teething how would you actually feel?
  • Much has been written on cognitive bias in business and it has been extrapolated to clinical medicine.  It could be useful to explore way we can individually become more aware of this as a process in day to day practice. (www.researchgate.net/publication/327473427_Cognitive_bias_in_clinical_medicine)

A 13 year old boy presents with a headache. He has been seen on four previous occasions spanning your hospital and another local emergency department over a 6wk period. His mother is particularly distressed by the headaches as she has previously lost a child. The boy’s mother clearly voices her anxieties and feels that things are worsening. This morning she reports witnessing an episode of vomiting with some ‘shaky walking’.

It is clear during your assessment that the boy is less concerned than his mother about these headaches. Neurological examination is normal while seated as he fears this will bring the headache back on lying down.

How do you proceed, is any further information required?

What investigations are indicated?

In practical terms this history and examination will need elucidating from both the mother and child independently being sympathetic to the overlying anxieties this mother is carrying from her deceased child. In saying this, there are already red flags appearing here:

  • Worsening headaches
  • Morning vomiting
  • Shaky walking (might indicated cerebellar signs)
  • Refusal to move to a supine posture

Again, we have a normal neurological examination (aside from the whole examination being conducted in a seated position).

How is imaging arranged within your department? This boy does require neuroimaging, but what would happen, CT or MRI? Does a refusal to lie down constitute enough information for a non-contrast CT head in emergency?

As you are deciding to neuroimage this child, you may wish to discuss:

  • Process of gaining CT at different times of day
  • Who reports this
  • If there are abnormalities on the imaging suggestive of raised intracranial pressure, what are the local arrangements to discuss this and where will definitive treatment be arranged?

A non-contrast CT head was undertaken which showed an obstructive posterior fossa mass requiring intervention by neurosurgery. The child required urgent transfer to a different unit for definitive treatment. Some further discussion points could be:

  • How is a time critical transfer arranged in you department?
  • What staff should go and what skills set would be required?
  • What would you prepare for if you were transferring this child (not ventilated, with a 6 week history of headaches but whom you now know has significant hydrocephalus)?

A 14 year old girl arrives immediately following an ophthalmology appointment for a general paediatric review. She has been suffering from mild headaches which have been controlled with simple analgesia for 2 months. These last from 1-4 hours, usually after school and have not worsened over this period. In the past 4 weeks she has become more aware of intermittent visual changes. She sees flashes of colour or ‘lego bricks’ which fall across her vision. This occurs daily, usually in the afternoon. Her ophthalmology appointment was unremarkable, including fundoscopy.

She is afebrile and lucid with no headache currently. Full neurological examination including co-ordination is normal.

Does this girl fit criteria for additional investigations?

What would you do?

You are intending to discharge her, what follow up should be arranged?

We refer back to:

Children with headache suspected of having a brain tumor: a cost-effectiveness analysis of diagnostic strategies, Medina et al, Pediatrics 2001

as an evidence base for imaging. She would not meet the criteria for urgent imaging. Consensus opinion from the American Academy of Neurology would suggest neuroimaging should be considered on the basis of new headaches with some features suggestive of neurological dysfunction. With a normal neurological examination, which here includes fundoscopy by an ophthalmologist, time is on our side so if imaging is pursued this should be MRI. Here it might be pertinent to consider other investigative strategies:

  • Would blood tests offer additional value? If not in this particular case then might they help in the context of low grade fever?
  • Would you consider an EEG?

In this case headaches aren’t really the major feature as they are easily controlled with analgesia. These do not sound epileptic – episodes are too frequent to not have a non-visual epileptic manifestation by now and the associated headache is not severe so an EEG may not be helpful. In childhood, migraine can encompass many other features and headache may not be the most prominent. Where patients are being discharged counselling/advice cannot be underestimated. A headache (or symptom in this case) diary can be a fast track to diagnosis in the outpatient setting and can easily be started from an emergency setting. Taking the time to talk about what your number one diagnosis is, and what environmental strategies might help and what features should prompt a further review, prior to discharge is vital.

A 10 year old boy presents with a 2 day history of a headache. He was referred by the GP to rule out meningitis. He appears uncomfortable but is alert and cooperative. The pain is throbbing and bilateral with a degree of photophobia. There is nausea but no vomiting. He is coryzal and has a temperature of 37.8C. The heart rate is 105 BPM and oxygen saturation rate 99% in room air. Neurological examination does not reveal any abnormalities and he has no problem lying flat for the exam. There is no meningism. On systemic examination there is only mild costophrenic tenderness.

What is the next best step in the management of this patient?

If a urinalysis is requested it shows microscopic haematuria and microscopic proteinuria but no pyuria.

What is the next step?

Participants may become fixated on headache characteristics at this point and may wish to ask additional questions about the character, timing and intensity and about associated symptoms. But these are vague and unspecific in this case. There is a heavy clue in the systemic findings including a mild tachycardia, low grade pyrexia and costophrenic tenderness. This is where the focus should shift towards investigating the cause of all of the patient’s findings, not just the headache.

The blood pressure was intentionally omitted from the vignette, an omission which also occurs frequently in real life. If it is requested it’s revealed to be 161/102. If it is not requested the vignette can continue with the patient developing seizures, which constitutes hypertensive emergency. The cause appears to be renal and the history, clinical findings and urinalysis are more in keeping with glomerulonephritis then acute infection. The most likely is IgA nephropathy. The management is behind the scope of this discussion – starting an anti-hypertensive and consulting a renal service is appropriate in the first instance. Here it is demonstrated that headache can be a sign of systemic illness and a thorough history and exam is always required including a full set of vital signs.

A 12 year old girl presenting with a 2 month history of headache. The pain is throbbing, bilateral, worse at night and is accompanied by nausea. She is anxious as the headache is now affecting her sleep. The GP started her on Amitryptiline but she stopped it due to daytime somnolence. She has a history of chronic abdominal pain. There is a family history of essential hypertension and diabetes. She has long been bullied about her weight. Her BMI is >99th centile.

Her fundscopy examination is shown.

Are there any red flags in the history?

What is the most likely diagnosis and the differential diagnosis?

What are the most important aspects of the exam?

This patient has symptoms which could suggest increased intracranial pressure. Given the history idiopathic intracranial hypertension is probably most likely but other causes like mass or syringomyelia must be considered. If these are ruled out than a primary headache disorder is most likely. Participants will likely list important aspect of the neurological examination. Visual fields and extraocular movements are of particular importance to screen for complications of ICP and a thorough screen for lateralizing signs to outrule mass. Ultimately this patient will need a scan prior to lumbar puncture but these findings will determine urgency.

A bedside fundoscopy can be used as a test for papilloedema but is it really possible? Most children are not fully cooperative and most ED are equipped with direct ophthalmoscopes which give a very small field of view (a panoptic ophthalmoscope is better). Additionally, the exam is usually undilated, adding another layer of difficulty. Overall a reliable ophthalmoscopy under these conditions requires significant expertise, so it should not be relied on unless a specialist is available. If the history is concerning, than the child should be worked up.

Which of the following is not a sign of raised intracranial pressure when co-existing with a headache?

A: Increasing head circumference in <1 year old

B: Vomiting

C: Behavioural change/irritability

D: Fever

E: Waking from sleep with pain

The correct answer is D.

Fever may suggest meningism but not raised ICP. All the others are concerning features of raised ICP.

What is the investigation of choice in headaches with clinical neurological signs?

A: MRI

B: Non-contrast CT

C: EEG

D: Bloods including infection markers/clotting profile

The correct answer is B.

Children who present with headaches and clear neurological signs are the cases where an in department non-contrast CT is indicated. Where there are no clinical findings MRI is the preferred imaging modality.

In paediatric migraine, what is the most effective single treatment for children presenting to emergency?

A: Analgesia

B: Rest and reassess

C: Modify environmental factors

D: Antiemetic

E: Keeping a headache diary

The correct answer is D.

All of the above have a role in the treatment of migraine, however, in the acute setting evidence points to antiemetics are most effective in symptom relief. Analgesia and antiemetics together are even more beneficial. Modification of environmental factors, including rest/exercise/diet and keeping a diary of symptoms will not help acutely but hand some control to the patient in the long term management of symptoms.

https://www.headsmart.org.uk/

Clinical features suggestive of meningitis in children: a systematic review of prospective data, Curtis et al, Pediatrics 2010

Treatment of pediatric migraine headaches: a randomized, double-blind trial of prochlorperazine versus ketorolac, Brousseau et al, Annuls of Emergency Medicine 2004

https://bpna.org.uk/uploads/pet/chat-pre-course-june-2017.pdf

https://www.rch.org.au/clinicalguide/guideline_index/Headache/ www.researchgate.net/publication/327473427_Cognitive_bias_in_clinical_medicine

Children with headache suspected of having a brain tumor: a cost-effectiveness analysis of diagnostic strategies, Medina et al, Pediatrics 2001

https://pemplaybook.org/podcast/pediatric-headache-some-relief-for-all/

https://pemcincinnati.com/blog/headaches-in-the-pediatric-ed/

https://dontforgetthebubbles.com/non-specific-symptoms-in-the-emergency-department-are-you-headsmart/



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ConSEPT and EcLiPSE – Levetiracetam versus Phenytoin for Status Epilepticus

Cite this article as:
Roland D, Davis T. ConSEPT and EcLiPSE – Levetiracetam versus Phenytoin for Status Epilepticus, Don't Forget the Bubbles, 2019. Available at:
https://doi.org/10.31440/DFTB.18696

This week sees the publication of two hugely anticipated randomised controlled trials both published in the Lancet simultaneously which DFTB have been given exclusive access to.  

An approach to irritability and pain in the severely neurologically impaired child.

Cite this article as:
Henry Goldstein. An approach to irritability and pain in the severely neurologically impaired child., Don't Forget the Bubbles, 2019. Available at:
https://doi.org/10.31440/DFTB.18178

Logan is a 6yo who is presented to ED by his mother, one Tuesday evening as “just not himself“. Logan is well known to your local paediatric team for management of his GMFCS 5 spastic quadriplegic cerebral palsy. He has a long list of comorbidities, frequent hospital attendance and multiple unplanned admissions for, variously, aspiration pneumonia, seizures or irritability ?cause.

Delayed presentation of head injuries – should we be worried?

Cite this article as:
Tessa Davis. Delayed presentation of head injuries – should we be worried?, Don't Forget the Bubbles, 2019. Available at:
https://doi.org/10.31440/DFTB.17874

We have a clear algorithm for when to CT children who present with head injuries immediately after the injury. But, when children present more than 24 hours after an injury, we aren’t really sure what is best practice. This paper, by the PREDICT group, look at the rates of traumatic brain injury in this patient group.

Borland M,  Dalziel SR, Phillips N, Lyttle M, Bressan S, Oakley E, Hearps SJC, Kochar A, Furyk J, Cheek J, Neutze J, Gilhotra Y, Dalton S, Babl F. Delayed Presentations to Emergency Departments of Children With Head Injury: A PREDICT Study, Annals of Emergency Medicine, DOI: https://doi.org/10.1016/j.annemergmed.2018.11.035

Why is this study needed?

We have a range of decision rules to help guide us for children presenting immediately after a head injury. PECARN and CATCH clinical decision rules specifically exclude children who present with a head injury more than 24 hours after the injury. CHALICE doesn’t specifically exclude this group, but there is no published data on this group of patients.

What we worry about is missing a traumatic brain injury, and in particular one that will need surgical intervention. If a child presents after the initial 24 hours, are they more likely to have a traumatic brain injury and should we therefore have a lower threshold to CT scan these patients?

This is a really common dilemma in Paediatric Emergency, and in my own experience, most people have a lower threshold for scanning children presenting late with head injury concerns because of the lack of guidance and evidence in this group.

The authors’ aim is to look at the prevalence of traumatic brain injury in this group, and to identify any factors in these patients that would make a traumatic brain injury more likely.

Who were the patients?

This was a secondary analysis of an existing cohort – the Australian Paediatric Head Injury Study cohort. This was children with a head injury who presented to one of ten paediatric EDs in Australia/New Zealand over a 3.5 year period.

For this secondary analysis, the cohort was split into those presenting within 24 hours, and those presenting later than 24 hours after the head injury. 5% of the cohort presented >24 hours after the injury.

Children were excluded if they had GCS<14, and were also excluded for representations of the same injury.

The original APHIRST cohort included 20,137 head injury presentations.

352 were excluded due to GSC<14 and 20 were excluded due to unknown time to presentation.

Of the 19,765 left, 981 children presented >24 hours after the injury.

Definitions

Traumatic brain injury on CT (TBI) – intracranial haemorrhage or contusion, cerebral oedema, traumatic infarction, diffuse axonal injury, shearing injury, sigmoid sinus thrombosis, signs of brain herniation, midline shift, diastasis of the skull, pneumocephalus, and depressed skull fracture.

Clinically important traumatic brain injury (cTBI) – death, intubation >24 hours, neurosurgery, or a traumatic brain injury-related admission to hospital of two or more nights.

What were the authors looking at?

The paper examined any associations between a delay in presentation and the mechanism of injury.

It also looked at the injury characteristics and demographics for patients presenting within and after 24 hours of the injury.

Who presented more than 24 hours after a head injury?

Those presenting >24 hours after the injury were significantly more likely to have had a non-frontal scalp haematoma, headache, vomiting, and assault with non-accidental injury concerns.

Loss of consciousness and amnesia were more likely to present within 24 hours of the injury.

Were the late presentations more likely to have a head CT and a brain injury than those presenting within 24 hours?

203 of the 981 patients had a head CT in the late group. This is 20.6% compared to 7.9% in the early presentations.

37 of these children had a TBI on head CT. This is 3.8% compared to 1.2% in the early presentations group. The most common injuries were a depressed skull fracture, intracranial haemorrhage, or contusions.

Eight children had a cTBI (0.8% – which is the same as in the early group) and two required neurosurgical intervention (also not significantly higher than in the early group).

Who were the eight children with clinically important traumatic brain injuries?

The children ranged from six months to 15 years.

  • Five of them had a low-level fall (<1 m) – one of these required neurosurgical intervention
  • One was struck by a high speed object
  • One sustained a blunt injury with a bat during sport – required neurosurgical intervention
  • One fell out of bed more than two days earlier

Of note in the late group…

No children with amnesia had a traumatic brain injury on head CT

Suspicion of a depressed skull fracture and a non-frontal scalp haematoma were significantly associated with a cTBI

No children with loss of consciousness had a cTBI

What can we take from this?

There may be many reasons why our scanning rate in delayed head injury presentations is so much higher – including the lack of previously existing evidence, and our clinical concern that a TBI is more likely if the symptoms are persisting.

The authors conclude that presenting >24 hours after the injury (with a GSC>14), significantly increases the risk of a TBI. Suspicion of depressed skull fracture or a non-frontal scalp haematoma increase the risk of TBI and cTBI in this group.

Commentary from Damian Roland:

This is a useful sub-analysis of a very good research data set prepared by the PREDICT group which has good face validity and is likely to be externally reproducible in other developed nations.

The question I ask myself when reviewing head injury patients with a ‘delayed’ presentation is ‘why are you delayed?’. The sheer size of this data set is testament to the fact that lots of children present to Emergency Departments because of parental concern following a fall or blunt trauma. If a parent chooses not to present initially it’s usually because they thought the injury was not that significant (not a very high bar to reach usually!) and symptoms have evolved or perhaps the initial circumstances weren’t clear or un-witnessed. For the former case this ‘evolution’ of disease is (not surprisingly) significant. The ‘delayed’ group more likely to demonstrate relevant pathology because the symptoms that pathology were producing were becoming more apparent. For the latter “historical’ muddying is either sinister (note the relationship with non-accidental injury concerns) or perhaps critical information which may have resulted in earlier attendance has been missed.

It is important to note that while the post 24 hour group demonstrated increased risks for many features and outcomes, the absolute numbers are still low. Just because you present 24 hours down the line doesn’t mean do a CT. Just think that bit more carefully than if the child had presented straight after the injury. As this same group have also recently shown, our individual decision making capacity is probably just as good as any rule so we can still trust our own clinical judgement

.

The Higher Tech Kid in the ED

Cite this article as:
Pascoe, E. The Higher Tech Kid in the ED, Don't Forget the Bubbles, 2018. Available at:
https://dontforgetthebubbles.com/higher-tech-kid-ed/

This month’s Podcast of the Month is from the Pediatric Emergency Playbook.

In a 30 minute podcast Tim Horeczko (Emergency Physician, and Educator) demystifies vagus nerve stimulators, intrathecal pumps, and ventricular assist devices. Through clinical cases, he provides some useful tips and tricks for what to do when the ‘higher tech kid’ presents to ED with a malfunctioning device.

What’s the emergency treatment for baclofen overdose when you can’t turn the pump off?

If you only switch on one podcast this month, make it this one.

Listen to the podcast.

Spina bifida – prognosis

Cite this article as:
Lydia Garside. Spina bifida – prognosis, Don't Forget the Bubbles, 2015. Available at:
https://doi.org/10.31440/DFTB.6980

This series is covering all you need to know about spina bifida. In the previous posts we looked at issues around diagnosis and antenatal counselling. This week, we look at the prognosis for patients with spina bifida.

Executive functioning is always affected in patients with hydrocephalus even when shunting is not required. Specific deficits and extent of deficits varies from patient to patient.

Executive functioning is organising and planning, problem solving, motivation, and multiskilling. It is the last learning skill to develop, and the first to be lost.

How can we help with learning?

For preschool children:

  • They should be encouraged to develop age appropriate skills – facilitate rather than “do”
  • Encourage thinking by extending sentences e.g. ‘What happened next?
  • Enable simple choices
  • Preschool is excellent preparation for school – should be strongly recommended

For school-aged children:

  • Structured day, regular routines
  • Didactic teaching, lots of repetition, revision
  • Concrete reinforcement
  • Prepare for the day
  • Anticipate change
  • May need to assist with socialisation

In the classroom:

  • Structured learning
  • Reduce stimulus/business of classroom
  • Reduce distractions
  • Simplify the tasks into steps
  • Make sure child understands the task
  • May need help to begin, organise the task
  • Memory – need to store information in an organised fashion

Survival of patients with spina bifida

One third die before 5 years of age

One quarter die before 40 years of age from:

  •   Epilepsy
  •   Pulmonary embolus
  •   Acute hydrocephalus
  •   Acute renal sepsis

If there is a thoracic level spinal lesion – only 17% survive to age 40 years.

47% die due to potentially reversible causes:

  • Renal failure – this is preventable as UTI – 7.2% (normal population 0.5%)
  • Osteomyelitis and skin breakdown
  • Pneumonia
  • Pressure ulcers
  • Hypertension

Children with spina bifida have complex needs and will need to see several different specialists regularly throughout their lives. Medical surveillance is key. Education is also imperative, initially for parents and then later as the child grows so they can self-manage as adults. Given the complex care, children are best served in specialist rehabilitation spina bifida clinics with immediate access to  allied health and different medical and surgical specialities as required.

Spina bifida is a life-long condition and how we treat children with spina bifida will have a huge impact on their adult health.

You can check out the rest of the series here:-

Spina bifida – fertility

Cite this article as:
Lydia Garside. Spina bifida – fertility, Don't Forget the Bubbles, 2015. Available at:
https://doi.org/10.31440/DFTB.6977

This series is covering all you need to know about spina bifida. In the previous posts we looked at issues around diagnosis and antenatal counselling. In this post, we look at fertility problems associated with spina bifida.

Folate is essential for rapidly dividing cells, and problems with folate metabolism can lead to miscarriage. Spina bifida risk is not increased for women who have families in later life.

Spina bifida 4 – bladder and bowel management

Cite this article as:
Lydia Garside. Spina bifida 4 – bladder and bowel management, Don't Forget the Bubbles, 2015. Available at:
https://doi.org/10.31440/DFTB.6975

This series is covering all you need to know about spina bifida. In the previous posts we looked at issues around diagnosis and antenatal counselling. In this post, we look at the bladder and bowel problems associated with spina bifida.