Wrist Examination & Pathology Module

Cite this article as:
Segn Nedd. Wrist Examination & Pathology Module, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.30095
TopicWrist Examination and Pathology
AuthorSegn Nedd
Duration2 hrs
Equipment requiredSplints, soft bandages, plaster of paris sets

  • Basics (10 minutes)
  • Main Session (2 x15 minutes) case discussions covering key points and evidence
  • Advanced  Session ( 2 x 20 minutes) case discussions covering diagnostic dilemma, advanced management
  • Sim scenario – (30 minutes) 
  • Quiz (5 minutes)
  • Infographic sharing (5 minutes): 5 take home learning points

(From TeachMeAnatomy and LITFL)

The wrist is a common place for injuries in children often occurring following a Fall Onto an OutStretched Hand (FOOSH). The wrist joint connects the hand to the forearm. It is made up of the radius and 8 carpal bones. Although commonly included, the ulna is not technically part of the wrist joint. The ulna articulates with the radius just proximal to the wrist at the radio-ulnar joint. It is separated from the carpal bones by a fibrocartilaginous ligament (articular disk). The wrist joint is a synovial joint. It therefore has a capsule. Its internal membrane secretes synovial fluid to lubricate the joint. 

When describing injuries of the wrist (and hand) for documentation or referral purposes it is important to know the terminology widely in use in order to convey an accurate description to others. Injuries present on the palmar surface would be described as Palmar or Volar. Injuries on the back of the hand are dorsal. The proximal part of the wrist is more towards the forearm, whereas the distal end is towards the fingers. The thumb lies on the radial side and the little is the ulnar side. 

Anatomy: (from Radiopedia, NYSORA & teachme anatomy)

In order to understand what you are examining and the associated pathologies that need to be considered it is important to have knowledge of the underlying structures that form the wrist. The wrist and hand have a complex anatomy with bony structures surrounded by a matrix of soft tissues including, muscles, tendons and ligaments. It additionally has an intricate blood and nerve supply. We will focus on the structures most important when assessing paediatric wrists in the emergency department.

Bones:

The radius is on the side of the thumb, the ulna on the side of the little finger. A good mnemonic to remember the position of the carpal bones is to describe them starting from the base layer thumb to little finger, followed by the top layer little finger to thumb.

So             Long     To                 Pinky,     Here       Comes    The            Thumb

Scaphoid, Lunate, Triquestrum, Pisiform, Hamate, Capitate, Trapezoid, Trapezium

Ligaments: (from Radiopedia)

There are multiple ligaments of the wrist. These play a vital role in the stability of the wrist joint. They are specifically important in holding the carpal bones together. Those most clinically important in wrist joint stability are labelled as above. Ligaments of the wrist are not visible on X-ray and to be fully examined are best assessed with a dedicated wrist MRI. However, increases in the spacing between bones on plain X-rays can indicate a ligament injury with clinical correlation. 

The Nervous System: (from NYSORA)

The ulnar, median, and radial nerves innervate the hand. The course of these nerves traverse the wrist. They therefore have the potential to be damaged following wrist injuries. The median, anterior interosseous nerve (a branch of the median) and the ulnar nerve specifically although rare can be compromised following wrist fractures. The nerves of the wrist and hand also have an important role in functionality of the wrist (and hand). The radial nerve facilitates extension of the wrist and metacarpophalangeal joints. The ulnar nerve facilitates movement of the small muscles of the hand. The median nerve supports finger extension and anterior interosseous branch enables thumb flexion at the interphalangeal joint and flexion of the index finger at the distal interphalangeal joint.

The corresponding dermatomal innervation of the wrist and hand is illustrated below.

Vasculature: (from teachmeanatomy)

Arising from bifurcations of the brachial artery in the cubital fossa are the radial and ulnar arteries (and their branches) to supply blood to the forearm, wrist and hand. These two arteries merge in the hand forming the superficial palmar and the deep palmar arch. The radial artery supplies the posterolateral aspect of the forearm and is important in contributing to the blood supply of the carpal bones. The ulnar artery supplies the anteromedial aspect of the forearm. It mostly supplies blood to the elbow joint, but its branches do however help supply some of the deeper structures in the forearm.

Examination: From Geeky Medics 

The look, feel, move & function approach is generally used to examine the hand and wrist. Always offer analgesia prior to your examination of a child with an injury.

As functions involve both areas they are often examined together

Look
1Perform general inspection
2Inspect the dorsum of the hands
3Inspect the palms of the hands and elbow

Careful note should be taken to ensure that full inspection is undertaken. This may identify any bruising, overlying skin changes, swelling or deformity. Remember also to always examine the joint above and the joint below.

Feel
1Asses and compare temperature of wrist and small joints of hand
2Palpate radial and ulnar pulse & check capillary refill
3Palpate thenar and hypothenar eminence
4Asses median nerve sensation
5Asses ulnar nerve sensation
6Asses radial nerve sensation
7Perform MCP squeeze
8Bimanually palpate hand and finger joints
9Palpate anatomical snuff box
10Bimanually palpate the wrist joints

It is important not to miss any neurovascular compromise when examining the wrist and hand. Findings to suggest compromise may include colour change, coolness to touch, prolonged capillary refill time and altered sensation.

Move
1Assess finger extension
2Assess finger flexion
3Assess active wrist extension
4Assess active wrist flexion
5Assess wrist/finger extension against resistance (radial nerve)
6Assess index finger ABduction against resistance (ulnar nerve)
7Assess thumb ABduction against resistance median nerve)

Where possible movements should be actively undertaken by the patient. Take notice of any movements that are undertaken with difficulty or cause pain in undertaking.  

Function
1Assess power grip
2Assess pincer grip
3Assess picking up small objects
4Supination and pronation- twisting key movement or ‘turning the key’

An 8 year old boy is brought to ED with his father. He had been outside roller-skating but fell over onto the concrete patio within the last hour. He is complaining of pain in his wrist and has difficulty moving it. An x-ray was done following triage: and it’s a buckle fracture

What would be your approach to examining his injury?

What type of fracture do you suspect and how would you differentiate on x-ray?

What type of immobilisation would you use?

Historic studies have shown that radial tenderness, focal swelling, or an abnormal supination/pronation were the clinical signs most often associated with correctly identifying children who had  wrist fractures. In 2016, in a multicenter study by Slaar et al. a clinical decision tool known as the Amsterdam paediatric wrist rules was created for use in children presenting with wrist trauma to determine clinically whether a radiograph was required or not.  

The prediction model had high sensitivity and moderate specificity of 95.9% and 37.3%, respectively. It was calculated that through using this model there would be a 22% absolute reduction of radiographic examinations. Although not perfect, the use of the paediatric Amsterdam wrist rules may therefore be a useful aide memoir in facilitating clinicians to rationalise which children who present with wrist trauma to x-ray.

The clinical prediction model used eight variables to analyse those at risk of any wrist fracture. These were increasing age; sex (if male), swelling of the wrist; swelling of the anatomical snuffbox; visible deformation; distal radius tenderness on palpation; pain on radial deviation and painful axial compression of the thumb. The more of these factors that were present resulted in the increased probability of a fracture. Painful axial compression of the thumb however decreased the probability of a fracture.

This study also further analysed those with distal radius fractures. Children with increasing age, swelling of the wrist, visible deformation, distal radius tender to palpation, pain on palmar flexion, pain on supination and or painful radioulnar ballottement test were more likely to have distal radius fractures. However, pain on ulnar deviation was found to decrease the likelihood of a distal radial fracture.

Always ensure adequate analgesia is given when first assessing an injury. Ensure that the examination is systematic. It is best to use the look, feel, move, function process when examining the wrist. As with any orthopaedic examination however it is always important to also assess and assess the join above and below the affected area.

Look – for any deformity, swelling, bruising, colour change or overlying lacerations

Feel – assess for radial tenderness, remember to assess for any signs of neurovascular compromise and check sensation in the forearm and hand. Neurovascular compromise is rare in distal radius fractures but can occur in greenstick fractures.

Move – making tasks quick and easy to reproduce will assist in making identification of pathologies easier when assessing children. A combination of movements described by Dawson can be used to assess motor and neurological function in the hand and wrist. This can be done by starting a game of rock, paper, scissors. The addition of the O.K sign and also encouraging pronation and supination by “turning the key”, turning the door handle” or “turning the lightbulb” will allow easy testing of wrist and hand movement and functionality

Types of distal radius and ulna fractures

(from DFTB and Radiopaedia)

Buckle fractures are common in children especially in the 5-10 year old age range. Following a fall (often onto an outstretched hand) the force is transmitted from the carpus to the distal radius and as this is the point of least resistance fractures occur. Fractures are also often around the dorsal cortex of distal radius.

Greenstick fractures are incomplete fractures of the long bones in children. They are usually only seen in those under 10 years of age. The integrity of the bone cortex is breached on the convex side. The concave surface remains intact. It resembles the break that occurs when a young green branch of a tree is bent and breaks incompletely. One side snaps whilst the other side is still intact.

Buckle/torus and greenstick fractures are often discussed together as they have similarities; they are unique to children due to their softer compressible bones. However they also have clear differences. 

Buckle/torus fractures:

  • In buckle fractures only one side of the bone is affected, strictly speaking both sides are affected in a torus fracture. However the terms are often used interchangeably
  • In buckle/torus fractures the bone cortex crumples/buckles but does not crack
  • Buckle/torus fractures are caused by longitudinal force through a long bone often following a fall a FOOSH
  • Buckling of the bone occurs due to paediatric bone softness

Greenstick fractures:

  • In greenstick fractures there is a clear cortex breach but only on one side of the bone
  • There may also be some degree of angulation
  • There may be visible deformity in greenstick fracture where often not present in a buckle fracture

More difficult to recognise distal radius fracture features on lateral wrist x-rays include:

  • A crinkle, or any irregularity of the cortex of the dorsal aspect of the distal radius
  • In an impacted and undisplaced fracture, the only abnormality may be a very slight increase in the density of the radial metaphysis and/or loss of the normal palmar tilt of the radial articular surface

What is the normal volar tilt of the radial articular surface?

In a lateral view of the distal forearm

The distal radius, the lunate and the capitate articulate with each other and lie in a straight line, like an apple in a cup sitting on a saucer.

The radius holds the lunate (cup) and the cup contains the capitate (apple)

The articular surface of the radius has a palmar tilt and is usually about 10 degrees with a normal range of 10-25 degrees

Controversies in management:

Rest, support and analgesia are the mainstay of treatment for buckle fractures. Buckle fractures often heal well without complication. There is however much variance in how these are treated in different departments. Removable splints are widely used for up to 3 weeks in children old enough to keep them on (hard casts may be required in younger children). There is however uncertainty as to whether immobilisation is actually really needed or if early mobilisation to reduce stiffness is preferable. The FOrearm fracture Recovery in Children Evaluation (FORCE) Study is currently in its final stages. It will evaluate outcomes (pain, functional improvement and complications) between encouragement of use of the wrist, an optional bandage, and a point of contact for any ongoing concern versus hard splints use and local hospital outpatient fracture follow up (https://force.octru.ox.ac.uk/).

Buckle fractures often heal well with minimal complication. There is however a risk of refracture. General advice includes avoidance of sports for three to six weeks and contact sports for 6 weeks post injury. You should also refer to your local guideline on the management of buckle fractures.

A 12 year old girl is brought to ED with her mother. She was jumping on her trampoline but fell out. She had immediate pain and has not been able to use her left hand since.  Her mum gave her some paracetamol and ibuprofen prior to arrival. An  x-ray was then done and is as follows:

What does this fracture show?

How  would you further classify this type of fracture?

How would you manage these fractures?

Mum asks you if she should let her 6 year old daughter use the trampoline. What is your advice?

(from Royal Children’s Hospital Melbourne)

In contrast to adult bones, children’s bones are still developing. They have cartilaginous discs which separate the epiphysis from the metaphysis of long bone. This area is called the growth plate (physis). Physeal injuries are very common in children and can account for up to 15-30% of all bony injuries. Physeal injuries occur most commonly in the pre-adolescent growth spurt age.

Physeal fractures are classified by the Salter-Harris classification. A Type II fracture is the most common type. Distal radial physeal fractures are uncommon in children younger than five years. The most common mechanism of injury is a fall on an outstretched hand. Extension of the wrist at the time of injury causes the distal fragment to be displaced dorsally (posteriorly). Commonly this also causes an associated ulna fracture (greenstick, physeal or styloid).

Always give appropriate analgesia prior to assessment and x-ray. Ensure both AP and lateral views are undertaken of the wrist AND distal forearm.

See the Salter-Harris Classification below;

(from Royal Children’s Hospital Melbourne & First10EM)

Type II is the commonest type of Salter-harris fracture and accounts for around 75% of physeal fractures. Reduction is required for distal radial physeal fractures that are angulated >20 degrees. Salter-Harris type I and II injuries rarely cause growth problems. The risk of growth arrest is higher in type III-V injuries. The risk of physeal arrest is rare in young children but the risk is higher if the child is near the end of growth. It is therefore increasingly important to correct any angulation in adolescents especially where there is less than two years of growth remaining.

For type I and II injuries, closed reduction may be required. A fracture clinic review is required within five days with x-ray. RICE advice and instruction to monitor for any swelling should be given. This as there is a risk of early compartment syndrome due to restriction by the cast.

Urgent orthopaedic review is required when there is an open fracture, a fracture is associated with neurovascular compromise (most notably in the median nerve distribution), any Salter-Harris type III and IV (lower or through) fracture is seen. Additionally referral should be made when there is an associated fracture in the same upper limb or if there is any difficulty achieving acceptable reduction. This should only be attempted with adequate supervision or clinical competence. However, for children presenting with distal radial physeal fractures after 5 days since the injury closed reduction should not be attempted and specialist input from the appropriate orthopaedic team must be arranged.

After any reduction or immobilisation of a fracture, repeat x-rays should be undertaken.

(from RSPA and GPPaedsTips)

Trampolines are thoroughly enjoyed by children of all ages. Especially in current times many families have invested in trampolines for their gardens. However injuries sustained whilst playing on trampolines contribute greatly to injury presentations in the children’s emergency departments.  There are however steps that can be taken to try and minimise the chances of injuries occurring. The first advice would be that children should take turns to bounce. 60% of injuries have been found to have occurred when more than one person is on the trampoline. Often it is the smallest (lightest) person who is (five times) more likely to be injured. If they are not alone they should be of similar age and size. 

The Royal Society for the Prevention of Accidents suggests that Trampolining is only suitable for children over six years of age when they can are sufficiently developed to control their bouncing. Adult supervision cannot prevent all injuries but may prevent children engaging in dangerous practices. Additionally having some formal training such as joining a local trampolining club to learn new skills will help children who are keen to learn how to attempt advanced trampolining skills and tricks safely.

A 14 year old boy was skateboarding and dismounted, landing on his outstretched hand. He had significant pain in his wrist around the distal radius. Following analgesia in the emergency department an x-ray was undertaken. No fracture was identified and he was reassured that he had sustained a soft tissue injury. He was discharged with RICE advice. 

Four weeks later he is still in pain – the original x-ray is re-reviewed – a scaphoid fracture is seen.

What are thoughts surrounding soft tissue injuries in children and how should they be defined and managed ?

Are there any other pathologies you should consider when x-rays appear normal

Many times when there are no overt fractures on an x-ray we conclude that the patient has a “soft tissue” injury or  sprain. A scaphoid series (not wrist views) should be requested when there is ‘snuffbox tenderness’. Even when bony pathology cannot be identified on X-rays it is important to consider that the muscles, ligaments and tendons of the wrist, if damaged, can have a significant impact on a child’s ability to undertake daily activities, especially if the injury is to their dominant hand. As with any injury presenting to the emergency department pain should always be assessed and managed. Fractures through the waist (middle) of the scaphoid jeopardise the blood supply of the proximal fragment. If the patient is managed incorrectly then non-union, delayed union or avascular necrosis of the proximal fragment may result.

Elvey et al. conducted a single centre study of MRI studies of children who had presented in the two weeks prior with wrist injuries. X-rays undertaken at the time had not identified any fracture. Although a small study of 57 cases, over 75% of cases had a positive finding on MRI. There were no cases at all of isolated soft-tissue injury. Occult fractures and bony contusions (focal oedema and haemorrhages which occur following microfracture) accounted collectively for almost 70% of the pathologies seen on MRIs in these children. Following MRI almost ⅓ of cases required additional further management changes.  This study raised questions about the best modality and timing of imaging in children presenting with wrist pain following an injury. Fracture lines may not be apparent in children on initial X-rays and may only become visible weeks later following callus formation. Alternative imaging options considered have included ultrasound, CT and nuclear medicine scintigraphy. The cost-effectiveness, time constraints, risk-benefit analysis of radiation exposure and operator feasibility in the emergency setting is however difficult to justify. Additionally, some of these modalities have excellent sensitivity, but low specificity and operator-dependency. 

It is important to remember that even when no injury is seen on X-ray wrist injuries often classified as sprains can have clinical sequelae. At 5–6 weeks in the Elvey et al. study children who had had occult cortical fractures typically had resolution of their pain. However, those who had bone contusions typically had continued pain on palpation. 

Carpal instability injuries: (From LITFL)

Some non-fracture pathologies are visible on x-ray but sometimes missed. Scapholunate injuries include scapholunate dissociation which is caused by damage of the ligament between the scaphoid and lunate bones. It is very uncommon in children but may occur in adolescent age groups. These will often be very painful.

The carpal bones on a normal plain X-ray are evenly spaced. Where there is a scapholunate dissociation there will be a large gap (>3mm) between scaphoid and lunate bones. This is also known as the Terry Thomas sign (named after a famed comedian who had a large gap between his two front teeth). 

Other ligament bands may tear between carpal bones causing carpal instability. These can lead to the following 4 stages of pathology; perilunate dislocation, perilunate dislocation with triquetrum dislocation and lunate dislocation. Injuries of these ligaments can cause long term damage including chronic pain and arthritis. PA views with help with Terry Thomas sign. Lateral views are most useful in helping to identify any misalignment and potential dislocation of the other carpal bones (mostly lunate and perilunate dislocations).

A lunate dislocation can be a devastating injury. There is loss of articulation between the lunate and radial head and lunate and capitate. This injury would be excruciatingly painful. However most importantly there is also a high risk of median nerve damage as the dislocated lunate bone causes pressure on the median nerve which would usually run freely through the carpal tunnel. This can cause an acute carpal tunnel syndrome. Due to pain it should be hard to miss but needs urgent management.

Scaphoid fractures (from LITFL and pedemmorsels)

A scaphoid fracture is uncommon in 4-11 year olds as ossification centres appear to be protective against scaphoid fractures. However, the scaphoid bone is the most easily broken carpal bone and is easily broken in the adolescent age group. The fracture occurs via transference of force onto the scaphoid following FOOSH where the wrist deviates radially during impact. It is a more common injury following extreme sports. Where a scaphoid fracture has occured X-ray a lucency will be apparent running through the scaphoid bone.

The scaphoid is positioned beneath the anatomical snuff box. On examination it is important to check for pain here.Tenderness of the Scaphoid Tubercle (on the volar aspect), pain with radial deviation, pain on axial loading to the thumb and pain with active wrist range of motion may also point to this diagnosis. It is important not to miss scaphoid fractures. The reason being is that the scaphoid bone is at high risk of non union and avascular necrosis if fractured and left untreated. 

Although simplistic to attribute all blood supply to the scaphoid from the radial artery a fracture especially at the distal end of the scaphoid has been associated with compromise in the blood supply to the rest of the scaphoid. Complications of missed scaphoid fractures can be bone growth arrest and chronic pain. 

Controversy is however present as to whether surgical treatment is preferential to conservative management. A recent systematic review of randomised controlled trials surrounding this question in 2018 by Al-Ajmi et al suggested that surgical management of minimally or non-displaced scaphoid fractures resulted in better functional outcomes than conservative management. However, the findings were not significantly strong enough to make concrete conclusions. It is however generally accepted that scaphoid fractures which are unstable due to being at the proximal pole, having displacement > 1 mm, those with associated carpal bone dislocation and those with significant angulation or clinical deformity will need referral to orthopaedics and surgical intervention.

Scaphoid fractures in children are generally believed to heal well. Casting of non-displaced, acute fractures leads to high rates of scaphoid union. Where there is clinical suspicion of a scaphoid fracture but uncertain or negative x-ray findings generally early immobilisation is initiated by application of a thumb spica splint or cast with follow-up imaging 2 weeks later.  Casting may however need to be applied for 3 months or more. The videos on thumb splints and hard cast spica’s from Don’t Forget the Bubbles and Orthofilms can be used for demonstration and to assist in practical sessions. (Please see the simulation section for full details). 

Conversely Porter et al suggested that symptomatic treatment is sufficient with those who have normal x-rays. This paper advocated using a removable splint with follow-up only arranged if symptoms do not improve. This was however a single centre study and advocated for a larger multicentered prospective clinical trial on this matter.

If in doubt and there is high clinical suspicion of a scaphoid fracture it is not unreasonable to consider application of a thumb spica cast with a plan to bring back the child for review in 2 weeks. 

A 13 year old girl is brought to ED following a fall from a tree she has significant pain, swelling and deformity of the distal shaft of the radius. Analgesia is given and she is taken to x-ray:

Case courtesy of Radswiki, Radiopaedia.org. From the case rID: 12221

What type of fracture has occurred?

How would you manage this fracture?

(from the Royal Children’s Hospital Melbourne)

Distal radius fractures can be classified according to:

  • Presence of displacement (whether they are displaced or nondisplaced)
  • Bone involvement (an isolated radius fracture only or if both radius and ulna are involved)
  • Fracture type
    • Buckle and greenstick fractures: – see previous sections for further information 
    • Complete fractures:  These fractures extend through both cortices of the radius. Most complete metaphyseal fractures of the distal radius also involve the ulna with either an associated complete fracture, greenstick fracture, or bowing deformity.

From RCH Clinical Guidelines: distal radius fractures

With complete fractures generally if there is clear deformity on examination, reduction is likely to be indicated. Acceptable angulations of the distal radius fracture are dependent on the age of the child. Coronal plane angulation (seen on AP view) has a poorer prognosis as it does not remodel as well as angulation in the sagittal plane (seen on lateral views).

As a rule of thumb, if the deformity is clinically visible, reduction (non-operative or operative) may be indicated. Acceptable angulations are dependent on the age of the child.

In the 0 – 5 year age group, an acceptable angulation for a distal radius metaphyseal fracture is < 20 degrees.

Acceptable angulations in the 5 – 10 year old group is < 15 degrees.

Acceptable angulations in the 10 – 15 year old group is < 10 degrees.

Where no reduction is required for a complete distal radius fracture a below elbow plaster of paris back slab should be applied, fracture clinic follow-up arranged within a week and a cast may be required for up to 6 weeks.

Those needing closed reduction may be able to be undertaken in the emergency department using local anesthetic or procedural sedation. However this should only be attempted with adequate supervision or clinical competence. These children will also need a hard cast applied but with extra moulding in the opposite direction to any angulation.

Following any reduction of a fracture X-rays should be taken. Angles of the fracture should be within the same parameters for acceptable angulation. An orthopaedics referral should be made for any child presenting with an open fracture, signs of neurovascular compromise or if there is over 10 degrees of angulation of the fractured segments. In children where there has been difficulty in reduction (be that due to ED team inexperience or difficulty in procedure) and those with an associated fracture in the same or opposite limb an orthopaedics referral must also be made. 

A Galeazzi fracture-dislocation is one such instance where an urgent orthopaedic team referral should be made. Galeazzi fracture-dislocations are often missed and may be difficult to recognise. It is however really important that a Galeazzi fracture is identified as it must be repositioned prior to casting. If there is an isolated radius fracture, always examine the distal radioulnar joint (also known as DRUJ) on x-ray.

A Galeazzi fracture-dislocation is a fracture of the distal third of the shaft of the radius with a disruption to the DRUJ. This by ulnar displacement which can occur in the volar or dorsal direction. True Galeazzi fractures are very uncommon in children but can occur after a FOOSH with forearm rotation. However, the Galeazzi equivalent is more common and is when there is a distal radius fracture with an associated distal ulna physeal fracture. There is however no disruption of the DRUJ.

Most Galeazzi-equivalent fractures can often be managed with closed reduction in children. However, in some adolescents especially where there is a true Galeazzi fracture-dislocation then open or percutaneous fixation to stabilise the distal radioulnar joint after reduction may be required. Children with Galeazzi-type fractures should be placed in an above elbow cast following any manipulation.

The majority of these fractures will do well.  However outcomes can be poor if there is a delay in diagnosis and or the fracture of radius has been immobilised without  correct alignment of the ulnar dislocation or inadequate support such as a below-elbow cast. Nerve injury is uncommon but there have been some case reports of ulnar nerve injury. Neurovascular status must therefore be carefully examined and assessed. This however does usually resolve with observation.

Depending on the experience of the learners in your group please choose and adapt the following practical elements

  1. Role play hand examinations in pairs. Identifying techniques to follow and signs to exclude. Can be done in OSCE format. 
  2. Removable casts – demonstration by facilitator of rigid casts and soft bandages available in your department alternatively videos from below could be used prior to a learner practice session:
  3. Plaster of Paris hard cast application- demonstration by facilitators or alternatively videos from below be used prior to a learner practice session:

Which of the following is false?

A: A buckle fracture occurs due to longitudinal force along long bone

B: Greenstick fractures do not have any breach in the bone cortex

C: A buckle fracture will have an intact cortex

D: A torus fracture is always circumferential

B: Greenstick

Greenstick, torus and buckle fractures occur due to longitudinal forces exerted along a long bone. Whilst generally used interchangeably with a buckle fractur, a torus fracture actually involves both sides of the bone whereas a buckle fracture generally involves on side. What differentiates a torus or buckle fracture from a greenstick fracture is that there is a breech in the cortex of the bone in greenstick fractures. The cortex itself remains intact in buckle and torus fractures. 

Which nerve is most likely to be affected by a lunate dislocation?

A: Radial nerve 

B: Ulnar nerve

C: Median nerve

D: All of the above

Answer C

The ulnar, median, and radial nerves innervate the hand. The course of these nerves all traverse the wrist. They therefore have the potential to be damaged following wrist injuries. It is important to assess neurovascular status in wrist and hand injuries. However, the median nerve runs through the middle of the palmar side of the hand through the carpal tunnel. This can cause an acute carpal tunnel syndrome. Radial nerve damage may be associated with supracondylar and humeral shaft fractures, whilst Ulnar nerve damage although rare may be seen supracondylar and Galeazzi-type fractures. 

Which statement is true?

A: A Galeazzi fracture-dislocation is one in which the radius is fractured and also dislocated from the radioulnar joint.

B: Scaphoid fractures always require surgical intervention

C: The ulnar nerve may be affected following a Galeazzi fracture-dislocation

D: Bayonet apposition is when the two portions of a fracture are aligned end to end with some angulation

Answer C

A Galeazzi fracture-dislocation is where the distal third of the shaft of the radius is fractured. There is also a disruption to the distal radioulnar joint. The ulna (not radius) bone is displaced. This can occur in the volar or dorsal direction. The Galeazzi equivalent fracture-dislocation is more common and is when there is a distal radius fracture with an associated distal ulna physeal fracture. The ulnar nerve may be affected in a Galeazzi fracture dislocation as it is entrapped by the ulna displacement. Bayonet apposition is a terminology used to describe fractured bone portions which are aligned side by side and not end to end. Depending on the patient’s age and degree of angulation it may be acceptable to leave a fracture in the Bayonet position to heal. Scaphoid fractures may be managed conservatively, however unstable fractures (due to being at the proximal pole, those having displacement >1mm, those with associated carpal bone dislocation and those with significant angulation or clinical deformity will need referral to orthopaedics and are more likely to need surgical intervention. 

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  2. https://teachmeanatomy.info/upper-limb/joints/wrist-joint/
  3. https://teachmeanatomy.info/upper-limb/vessels/arteries/
  4. https://www.nysora.com/techniques/upper-extremity/wrist/wrist-block/
  5. https://geekymedics.com/hand-examination/
  6. https://radiopaedia.org/cases/buckle-fracture
  7. Slaar A, Walenkamp MM, Bentohami A, et al. A clinical decision rule for the use of plain radiography in children after acute wrist injury: development and external validation of the Amsterdam Pediatric Wrist Rules. Pediatr Radiol. 2016;46(1):50‐60. doi:10.1007/s00247-015-3436-3
  8. Davidson AW. Rock-paper-scissors. Injury. 2003;34(1):61‐63. doi:10.1016/s0020-1383(02)00102-x
  9. https://www.peminfographics.com/infographics/rock-paper-scissors-ok
  10. https://force.octru.ox.ac.uk/
  11. https://www.rch.org.au/fracture-education/growth_plate_injuries/Physeal_growth_plate_injuries/
  12. https://www.rch.org.au/clinicalguide/guideline_index/fractures/Distal_radius_and_or_ulna_metaphyseal_fractures_Emergency_Department_setting/
  13. https://first10em.com/ebm-lecture-handout-6-salter-harris-1-injuries/
  14. https://www.researchgate.net/figure/Salter-Harris-type-III-fracture-in-a-15-year-old-male-patient-Frontal-radiograph-of-the_fig11_260716406
  15. Elvey M, Patel S, Avisar E, White WJ, Sorene E. Defining occult injuries of the distal forearm and wrist in children. J Child Orthop. 2016;10(3):227‐233. doi:10.1007/s11832-016-0735-7
  16. http://gppaedstips.blogspot.com/search/label/Injury
  17. https://www.rospa.com/leisure-safety/Advice/Trampoline
  18. Little, Jason & Klionsky, Nina & Chaturvedi, Abhishek & Soral, Aditya & Chaturvedi, Apeksha. (2014). Pediatric Distal Forearm and Wrist Injury: An Imaging Review. Radiographics : a review publication of the Radiological Society of North America, Inc. 34. 472-90. 10.1148/rg.342135073. 
  19. https://litfl.com/bscc/clinical-anatomy/hand-and-wrist-injuries/
  20. https://pedemmorsels.com/scaphoid-fracture/
  21. Shaterian A1, Santos PJF1, Lee CJ1, Evans GRD1, Leis A1. Management Modalities and Outcomes Following Acute Scaphoid Fractures in Children: A Quantitative Review and Meta-Analysis. Hand (N Y). 2019 May;14(3):305-310. PMID: 29078712.
  22. Porter J, Porter R, Chan KJ. Scaphoid Fractures in Children: Do We Need to X-ray? A Retrospective Chart Review of 144 Wrists. Pediatr Emerg Care. 2018 Mar 12. PMID: 29538268.
  23. https://www.orthobullets.com/hand/6034/scaphoid-fracture
  24. Al-Ajmi TA, Al-Faryan KH, Al-Kanaan NF, et al. A Systematic Review and Meta-analysis of Randomized Controlled Trials Comparing Surgical versus Conservative Treatments for Acute Undisplaced or Minimally-Displaced Scaphoid Fractures. Clin Orthop Surg. 2018;10(1):64‐73. doi:10.4055/cios.2018.10.1.64
  25. https://www.rch.org.au/clinicalguide/guideline_index/fractures/Galeazzi_fracturedislocations_Emergency_Department_setting/
  26. https://www.paediatricpearls.co.uk/wp-content/uploads/minor-injuries-series-wrist.pdf
  27. Clinical Anatomy –hand, wrist (palmar aspect/flexors) Armando Husudungan https://www.youtube.com/watch?v=3aIHxXqKzcU 
  28. http://www.emdocs.net/hand-expedited-examination-key-points-regarding-ed-diagnoses/

Please download our Facilitator and Learner guides

Cervical Spine Injuries Module

Cite this article as:
Ronán Murphy. Cervical Spine Injuries Module, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.29843
TopicCervical spine injury
AuthorRonán Murphy
DurationUp to 2 hours
Equipment requiredComputer with projector for imaging
  • Introduction and Basics: (10 mins) pre-reading, glossary of terms, anatomical considerations
  • Main session: (2 x 15 minutes) case discussions covering the key points and evidence
  • Advanced session: (2 x 20 minutes) case discussions covering diagnostic dilemmas; advanced management and escalation
  • Sim scenario (30-60 mins)
  • Quiz (10 mins)
  • Infographic sharing (5 mins): 5 take home learning points

EMS – Emergency Medical Services

CSI – Cervical Spine Injury

SCIWORA – Spinal cord injury without radiological abnormality

XR – X-radiography

ED – Emergency Department

NEXUS – National Emergency X-radiography utilization study

PECARN – Paediatric Emergency Care Applied Research Network

GCS – Glasgow Coma Scale

MILS – Manual in line stabilization

MVC – Motor Vehicle Collision 

ATV – All terrain vehicles

NPV – Negative Predictive Value

PEM – Paediatric Emergency Medicine

TBI – Traumatic Brain Injury

NICE – National Institute for Health and Care Excellence, United Kingdom

MRI- Magnetic Resonance Imaging

The incidence of cervical spine injury is low and represents only 1-2% of all paediatric major trauma (1). 

The cervical spine is overrepresented as the region where more than half of all paediatric spinal injuries occur and the main reason for this is the relatively larger head size leading to the fulcrum of flexion being in the cervical column (2-4). Other features also make it susceptible to injury: ligamentous laxity, incompletely ossified vertebrae and more horizontally orientated facet joints (5). 

The incidence of ligamentous injury is thought to be higher in younger, non-communicative children under 3 years of age (6).

The precise location of injury in the cervical spine can be variable across the age range (7-9).

Four injury patterns are common in children with cervical spine trauma:

  • Fracture
  • Subluxation with fracture
  • Subluxation without fracture
  • SCIWORA

Dislocations or subluxations are more common in upper cervical spine injuries and associated with greater morbidity (9). To compare with adults, children are over twice as likely to suffer atlanto-axial injury (8).

Weaker musculature and underdeveloped interlocking bony processes contribute to the subluxation/dislocation and SCIWORA (spinal cord injury without radiological abnormality) type injury patterns we see in children (10). SCIWORA refers to CT and plain film. A lesion may be detected on MRI.

SCIWORA is the presence of myelopathy as a result of trauma with no evidence of fracture or ligamentous instability on imaging. The mechanism relates to the flexibility of the paediatric spine being greater than that of the spinal cord which becomes damaged as it is stretched beyond its limits. Neurological signs or symptoms even if transient must be elicited in the history to make this diagnosis (2). 

Manage the cases below as you would in your own setting using local guidelines and procedures to make this whole exercise as realistic as possible and also to stimulate further analysis and discussion. 

A 13 year old boy arrives in your ED. He came off his bicycle at speed whilst engaged in a downhill mountain racing contest. He was wearing a helmet and protective clothing but hit his head against a tree as he landed. He did not lose consciousness. He describes immediate onset neck pain which now persists. Volunteer ambulance services were supervising the event and treated his pain with paracetamol and ibuprofen whilst preparing him for transfer in full spinal precautions. He is brought into your ED strapped to a spinal stretcher with a hard cervical collar in place as well as head blocks and tape. On handover it is noted that he felt a weird sensation in his right arm at the time of the event which lasted perhaps 3-5 mins and has not returned. The crew found his neck to be diffusely tender on examination.

What features in history and on examination are we concerned about regarding the potential for cervical spine injury?

How do we take handover of these patients and protect them whilst we work up their injury?

Predisposing vulnerability to bony or ligamentous failure: Down Syndrome(T21), Rheumatoid Arthritis, Rickets, Osteogenesis Imperfecta, Ehlers-Danlos Syndrome, Achondroplasia, Marfans Syndrome, Renal osteodystrophy, Klippel Feil disease, Morquio Syndrome, Grisel syndrome.

A high risk MVC is one of the following: Head on collision, rollover, patient ejected from the vehicle, death of another passenger occurred, speed of collision over 88kph (55mph).

Distracting injury is vaguely defined by NEXUS to include burns and long bone fractures etc. It can be refined to mean any substantial injury of the upper torso due to proximity to the cervical spine (14).

As described above (11), the Viccelilo study (2001) looked at the performance of NEXUS in the paediatric subgroup. In 2017 however, Cochrane (2017) found there was conflicting evidence to support use of NEXUS in children and called for additional well designed studies with larger sample sizes to better evaluate this population (15). 

Some points to consider regarding immobilization: 

Where a cervical spine injury is suspected, appropriate immobilization must be achieved. 

Ask the co-operative child to lie still. Apply gentle manual in line stabilisation (MILS) and give lots of encouragement (age appropriate) to minimise movement. The neck should reside neutrally or in a position of comfort for the child. Bear in mind that babies may require a pad or similar thoracic elevation device laid onto the trauma mattress to elevate the torso and preserve neutral alignment of spine due to their relatively larger heads. This will prevent any forced flexion occurring.

While providing MILS and reassurance, we must address pain as a matter of urgency. Immobilisation may increase leverage on the neck in a sore and struggling child.  Once deemed safe to do so (and rapport has been established where applicable), radiolucent blocks and straps can be applied to free up that team member from the task of MILS. As well as helping limit movement, blocks also serve as a communication tool / visual reminder to the team that we are worried about this spine and to handle with care.

Transfer: If coming in by EMS, transfer using scoop from ambulance mattress to radiolucent ED trauma mattress. During transferring manoeuvres, the team leader should ensure that the minimal number of movements and gentle max 30 degree tilts are all that’s needed to get them on and off a scoop stretcher. The leader should also ensure that all team members understand their role and are given adequate prompts prior to any movements being performed “ready, steady, move”. Use the opportunity allowed by tilting to complete your standard assessment of the spine (and the patients back for other injuries or relevant findings). Document appropriately. Sometimes children 6 years old and above (not possible to put on below this age) may come in via EMS wearing cervical collars. These are removed to assess the cervical spine in ED while MILS is being applied and hard collars should not be placed back on as they can have a number of negative effects, particularly with prolonged use (16).Two piece collars are different and are often recommended by spinal specialists as a treatment modality for stable fractures or as a bridge to definitive management. 

A 10 month old girl who was a back seat occupant in a rearward facing baby seat is involved in a head on RTC at 30kph (18mph). The incident occurred in a housing estate. She presents with her Mother who was the restrained driver for review at a mixed Adult and Paediatric ED. You have already assessed and cleared Mum from any serious injury. You now examine her baby who is crawling away from you on the bed saying “mama”. 

Discuss how we need to adapt our assessment to suit younger children. 

Are we worried about this baby and do we want imaging? 

Outside of the factors looked at in the large studies, are there any other items which we should consider important in history and on physical examination for all children? 

The studies we have looked at so far don’t have many children under the age of three years old. We will now explore some which do:

Expert Consensus on factors which are suspicious for CSI (18-21):

  • persistent neck pain
  • child or parent feeds like the child has an abnormal head position
  • difficulty with neck movement
  • fall >1m or 6 steps of stairs or fall from greater than body height
  • hyperextension injury, acceleration-deceleration injury involving the head or clothes-lining (blunt trauma to the head/neck by a stationary object while the patient is in motion)
  • bicycle collision, pedestrian versus bicycle, accidents involving motorised recreational vehicles, horse riding accidents.
  • current or transient neurological symptoms (motor or sensory): weakness, paraesthesia, lightning or burning sensation down the spine or radiating to an extremity
  • neurological symptoms related to neck movement.
  • torticollis or abnormal head position
  • posterior midline cervical tenderness
  • substantial injury to the chest, abdomen or pelvis (one that is life-threatening, warrants inpatient observation or surgery)
  • physical signs of neck trauma such as ecchymosis, abrasion, deformity, swelling, tenderness
  • limited cervical range of motion
  • significant trauma to head or face
  • inconsolable child

Where the child does not have any concerning features on history or examination, we can look at some low risk factors which offer us some reassurance:

  • simple rear end MVC
  • comfortable in sitting position in ED
  • ambulatory at any time since injury
  • no midline cervical tenderness
  • presenting with delayed onset neck pain

In the awake and alert patient with no neurological signs or symptoms who has neck pain or unspecified neck tenderness, NICE Clinical Guideline 176 permits the search for any one low risk factor from the above list.

If one is found, further clinical assessment of the neck, beyond the initial assessment and palpation is performed. 

This comprises asking the patient to perform 45 degree bilateral active neck rotations. If this is tolerated the cervical spine can be cleared clinically. If not, the patient gets imaged. 

Persistent neck pain/tenderness in the posterior midline with normal clinical examination otherwise and normal conventional radiographs requires further evaluation. The same applies even if the patient presents sub acutely (18). This will vary between institutions but may take the form of a referral to the orthopaedic or spinal service followed by MRI or CT. 

Beware the “trivial” injury, especially in younger children. Correlate history with clinical examination. Remember these patients can be more difficult to assess and there exists limited evidence to guide their management. There are cases in children 9 months to 6 years of falls less than 5ft, out of bed, down steps, running, somersaulting which have led to C1-2 subluxations, rotatory subluxation, C2 pedicle fractures, odontoid fractures and neural arch C2 fractures. Examination findings were torticollis, neck pain, limited range of motion neck, refusal to move neck any one or combination of the above on exam but never just neck pain (22).

Beware also of markings on the child’s body from seatbelts or other age appropriate restraint systems. These may indicate extreme flexion of the cervical spine has occurred especially in head on collision (23). 

As always, consider the potential for non-accidental injury in your differential. 

Involve a senior early in the assessment and management of all suspected cervical spine injury children. 

Interpretation of imaging (where it is necessary) and patient reassessment: 

As we have discussed above, plain films are the main imaging tool used to assess the cervical spine in the ED. These must be interpreted by a senior physician due to the inherent challenges involved. 

If imaging is adequate and shows no abnormality in an alert and cooperative patient (or if imaging was not required in the absence of concerning features), reassess for resolution of neck pain post analgesia. Check 45 degrees of neck rotation either side of the midline. If normal, ask if you are satisfied that there isn’t any persisting clinical concern. If they examine well and there is no residual concern, many institutions and the NICE guidelines declare that the spine is now clear. This final clearance step should always be performed with a senior present.

An 11 year old boy is involved in a single vehicle RTA as a front seat passenger restrained in a booster seat. The vehicle slid at 120kph (75mph) and spun out of control demolishing a fence at the side of the dual carriageway and impacting a treeline before being propelled back onto the road. There is extensive damage to the four door saloon and all airbags deployed. The boy’s father was driving at the time and they self extricated by kicking out a front door which was slightly wedged by the distorted frame. They stood by the side of the road awaiting help to arrive. The father states he is fine apart from a few scrapes from broken glass and declines further assessment by Paramedics. The son complains that he now notices neck pain on moving his head backward and forward. EMS reviewed and decided to treat him with full spinal precautions. He arrives in your ED and is assessed as having no evidence of bruising or deformity of the neck and no midline bony tenderness. He has a normal neurological examination. His neck pain is persistent despite ibuprofen and paracetamol given by EMS en route. He is reluctant to move it much.

Do you want to image this child and if so, what imaging do you want to perform?

The sensitivity of two or more radiographic views for detecting cervical spine injury has been reported to be in the region of 85-94%, whereas CT ranges from 81-100%. These figures reflect the unique anatomical challenges inherent in interpreting images in this patient cohort, particularly those under the age of 8 years old. In adults, by comparison, CT sensitivity sits around 97-100%. This makes plain films a higher yield modality in the paediatric population, backed up by CT where plain film findings are abnormal or ambiguous (24).

To obtain the optimal sensitivity from plain film we need two or more views. The odontoid view is technically difficult to obtain in children less than 5 years old and may not yield much diagnostic information which can’t be obtained on antero-posterior and lateral. 

Many paediatric radiologists do not routinely obtain odontoid views in children younger than 5 years and many more stop after the first attempt is unsuccessful. The fracture that can only be assessed on the odontoid view is the Jefferson fracture (an eponym for a burst fracture of C1) and this occurs with axial loading mechanisms (uncommon below this age). Usually there is an associated head injury which would require neuroimaging (including upper cervical spine in CT) (25).  

In infants and young children, fractures of the dens tend to involve the subdental synchondrosis (naturally weak area of C2), from flexion mechanisms. The resulting anterior tilt of the dens is normally visible on lateral views. 

Adopting a pragmatic approach based on the child’s age and clinical status allowing them to obey commands and open their mouth is the best course of action (26). Safety is paramount and this view can be dangerous as some movement is required of the patient (10).

It is common practice now to minimize the exposure of children, parents and staff to radiation. The neck is a radiosensitive anatomical area and the thyroid gland receives a 100-200 fold higher radiation dose with CT than with the standard three view plain film series. This extrapolates to a 2x higher mean excess risk of thyroid cancer for patients 0-4years (27). 

To help put that into perspective, in the Republic of Ireland the incidence of thyroid cancer is 3.61/100,000 at baseline (28).

Ionizing radiation imparts a small but real risk of malignancy at the population level. This impact is greater on paediatric patients (29). 

It takes time and a greater degree of patient cooperation to obtain plain films.  As explored above, plain films can have lower sensitivity than CT so require a reliable history and physical examination to back them up as a diagnostic tool. CT has a superior ability to detect critical paediatric cervical spine injury in higher risk trauma patients because it provides more anatomical detail (30, 31). 

Indications for CT imaging (1, 13, 18, 20):

  • peripheral focal neurological signs or symptoms including paraesthesia in upper/lower limb(s)
  • patient is intubated/respiratory failure (severe TBI or C3/4/5 injury damaging phrenic nerve)
  • GCS <13 on initial assessment, pointing to TBI
  • head or multi-system trauma undergoing CT
  • signs of substantial head injury (e.g. signs of base of skull fracture)
  • where an urgent diagnosis is required e.g. for theatre
  • plain films are technically difficult or inadequate
  • strong clinical suspicion persists in spine of normal plain films e.g. symptomatic with head first axial loading as mechanism
  • plain films demonstrate a bony injury

The use of MRI will vary between institutions. 

In patients who have neurological signs on examination, MRI should be the primary modality wherever possible (26). It is often used as a follow on investigation from CT in the intubated sick trauma patient who is unconscious and difficult to assess from a neurological perspective clinically. They are now stabilized enough either via Intensive Care or Surgical input to undergo an MRI.

Younger children with isolated neck injury who are difficult to assess and in whom we are concerned due history and physical examination may also be suitable candidates for MRI (32) post normal plain XR instead of going to CT. Bear in mind that many children will require a general anaesthetic to tolerate this imaging modality as it takes longer than CT or XR to perform.  

A 2 year old girl presents with her father after landing awkwardly post a fall down the last two steps of stairs in her home yesterday evening. She has been starting to walk up and down the stairs and is always supervised. Last night, she complained of some pain which responded to the paracetamol syrup given to her by Dad. She slept well but since this morning, she has been holding her neck strangely and prefers to lie down.

You are the senior registrar on duty and one of your colleagues asks for your review. She is lying in a position of comfort on her left side. When you go to examine her she sits up and clings to her father crying and making it clear that she does not want to be examined, saying bye-bye. She has a torticollis to the left and is moving all limbs. Analgesia was given and plain films were obtained. These looked normal to you and the patient was reviewed and looks more comfortable now, although the torticollis persists.

Should we be concerned? Outline your steps in this patient’s management.

Torticollis in the setting of trauma, even in the absence of neurological signs or symptoms is concerning. 

There is an association between torticollis and cervical spine injury, particularly rotatory subluxation of C1 on C2 (Atlanto axial rotatory subluxation or fixation as it is sometimes termed), however it can also be seen in other patterns of cervical spine injury too (34-38). 

Typically, in trauma, the ipsilateral sternocleidomastoid muscle is in spasm. This differs from torticollis from other causes (benign paroxysmal torticollis, cervical lymphadenitis, cervical spine/cord tumours, posterior fossa tumours) where the contralateral sternocleidomastoid is in spasm (37). 

Despite the fact this little girl’s neurological assessment remained normal, it was decided to proceed to MRI under general anaesthesia. This demonstrated atlanto-axial rotatory subluxation. 

This case emphasises the concerning nature of traumatic torticollis, even in the absence of neurological signs or symptoms.

Injuries sustained by mechanism (33):

Hyper-flexionHyper-extensionAxial Load
Flexion teardropHyperextension dislocationBurst fracture (If occurs to C1 the eponym of Jefferson applies)
Bilateral facet dislocationExtension teardrop
Unilateral facet dislocationHangman’s fracture  (C2 Pedicles)
Anterior subluxation
Wedge fracture
Spinous process fracture

Falls from elevation, MVCs, being struck by motor vehicles while walking or riding and blunt blows to head and neck are more likely to result in axial (C2 and above) CSIs. 

Sports related cervical spine injuries are more likely to result in injuries to the sub-axial (below C2) region or SCIWORA. Children involved in diving and motor sports (All-terrain-vehicles and motorcycles) are more likely to sustain sub-axial cervical spine injuries (8).

What percentage of paediatric spinal injuries are located in the cervical region?

A: 12%

B: 40%

C: 50% or more

D: 2%

C: 50% or more

The cervical spine is overrepresented as the region where more than half of all paediatric spinal injuries occur and the main reason for this is the relatively larger head size leading to the fulcrum of flexion being in the cervical column.

Which of the following mechanisms in history are concerning for a cervical spine injury?

A: Motor Vehicle Collision at a speed of above 30kph (18mph)

B: Diving into a pool

C: Fall from greater than body height

D: Transient neurological symptoms

E: All of the above

E: All of the above

Expert consensus on factors which are suspicious for a CSI include: persistent neck pain, child or parent feeds like the child has an abnormal head position, difficulty with neck movement, fall >1m or 6 steps of stairs or fall from greater than body height, hyperextension injury, acceleration-deceleration injury involving the head or clothes-lining (blunt trauma to the head/neck by a stationary object while the patient is in motion), bicycle collision, pedestrian versus bicycle, accidents involving motorized recreational vehicles, horse riding accidents, current or transient neurological symptoms (motor or sensory): weakness, paraesthesia, lightning or burning sensation down the spine or radiating to an extremity, neurological symptoms related to neck movement.

Plain films are not sensitive enough in children to be our first choice in most circumstances where imaging of the cervical spine is deemed necessary.

A: True

B: False

B: False

The sensitivity of two or more radiographic views for detecting cervical spine injury has been reported to be in the region of 85-94%, whereas CT ranges from 81-100%. These figures reflect the unique anatomical challenges inherent in interpreting images in this patient cohort, particularly those under the age of 8 years old. In adults, by comparison, CT sensitivity sits around 97-100%. This makes plain films a higher yield modality in the paediatric population, backed up by CT where plain film findings are abnormal or ambiguous.

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4. Davies J, Cross S, Evanson J. Radiological assessment of paediatric cervical spine injury in blunt trauma: the potential impact of new NICE guidelines on the use of CT. Clin Radiol. 2016;71(9):844-53.

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11. Viccellio P, Simon H, Pressman BD, Shah MN, Mower WR, Hoffman JR. A prospective multicenter study of cervical spine injury in children. Pediatrics. 2001;108(2):E20. 

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13. Leonard JC, Browne LR, Ahmad FA, Schwartz H, Wallendorf M, Leonard JR, et al. Cervical Spine Injury Risk Factors in Children With Blunt Trauma. Pediatrics. 2019;144(1).

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22. Schwartz GR, Wright SW, Fein JA, Sugarman J, Pasternack J, Salhanick S. Pediatric cervical spine injury sustained in falls from low heights. Ann Emerg Med. 1997;30(3):249-52.

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30. Brockmeyer DL, Ragel BT, Kestle JR. The pediatric cervical spine instability study. A pilot study assessing the prognostic value of four imaging modalities in clearing the cervical spine for children with severe traumatic injuries. Childs Nerv Syst. 2012;28(5):699-705.

31. Tat ST, Mejia MJ, Freishtat RJ. Imaging, clearance, and controversies in pediatric cervical spine trauma. Pediatr Emerg Care. 2014;30(12):911-5; quiz 6-8.

32. Booth TN. Cervical spine evaluation in pediatric trauma. AJR Am J Roentgenol. 2012;198(5):W417-25.

33. Easter JS, Barkin R, Rosen CL, Ban K. Cervical Spine Injuries in Children, Part I: Mechanism of Injury, Clinical Presentation, and Imaging. Journal of Emergency Medicine. 2011;41(2):142-50.

34.      Schwartz GR, Wright SW, Fein JA, Sugarman J, Pasternack J, Salhanick S. Pediatric cervical spine injury sustained in falls from low heights. Ann Emerg Med. 1997;30(3):249-52.

35.      Brown P, Munigangaiah S, Davidson N, Bruce C, Trivedi J. A review of paediatric cervical spinal trauma. Orthopaedics & Trauma. 2018;32(5):288-92.

36.      Leonard JC, Kuppermann N, Olsen C, Babcock-Cimpello L, Brown K, Mahajan P, et al. Factors associated with cervical spine injury in children after blunt trauma. Annals of emergency medicine. 2011;58(2):145-55.

37.      Copley PC, Tilliridou V, Kirby A, Jones J, Kandasamy J. Management of cervical spine trauma in children. European journal of trauma and emergency surgery : official publication of the European Trauma Society. 2019;45(5):777-89.

38.      Klimo P, Jr., Ware ML, Gupta N, Brockmeyer D. Cervical spine trauma in the pediatric patient. Neurosurgery Clinics of North America. 2007;18(4):599-620.


Please download our Facilitator and Learner guides

Non-Traumatic MSK Injuries Module

Cite this article as:
Stephen Gilmartin. Non-Traumatic MSK Injuries Module, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.30012
TopicNon-traumatic MSK injuries
AuthorStephen Gilmartin
DurationUp to 2 hours
Equipment requiredAV to project x-ray images
  • 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
  • Quiz (10 mins)
  • Infographic sharing (5 mins): 5 take home learning points

Expectation is for the learners to have understood the basics before the session.

Anatomy video: https://www.youtube.com/watch?v=kkHRRu6q2_o

Assessment tips: http://pmm-int.boxmodelstaging.co.uk/file.aspx?id=865

If possible or for further resource

Apophysitis, avulsions, Spondolysis: http://pemplaybook.org/podcast/pediatric-sports-injuries/

Rheumatology: https://dontforgetthebubbles.com/podcast/paediatric-rheumatology-jane-munro-dftb19/

  • Differential diagnosis of non-traumatic pain
  • Diagnoses not to miss
  • How to diagnose some of the common causes of non-traumatic MSK pain.
  • How to treat these causes
  • What diagnosis can cause long term morbidity
  • When to seek prompt speciality help.

Non traumatic pain is a common presentation in children and one which has a wide differential.  Lower limbs are most commonly involved. The potential diagnoses range from benign and self-limiting to life and limb threatening. 

There is often a history of an innocuous traumatic event which has prompted the attendance for assessment, but this often has little to do with the underlying diagnosis.

Clinicians should have a standardised approach to history and examination of non-traumatic MSK pain to ensure no diagnoses are missed

The underlying cause varies between age groups as children become susceptible to specific conditions as they progress through childhood.

Common causes

DiagnosisLocationAgeHistoryExamX-ray changes
Apophysitis Any apophysisF 10-14/M12-16Gradual onset
Pain worse on activity
Eases after rest
Point tenderness over apophysis
With or without swelling
Sclerosis and fragmentation
Osteochondrosis Joints: Commonly elbow/hip/foot4-18 dependent on siteGradual onset
Pain worse on activity
Eases after rest
Mild swelling
Stiff and painful joint
Irregular growth of epiphysis
Sclerosis
Fragmentation
Osteochondritis dissecans Commonly knee and ankle>10Gradual/sudden onset
Pain worse on activity
Associated intermittent swelling
Swollen joint in acute phase
Tender joint line
Lucency about the cortical surface 
May be occult
Osteomyelitis Commonly in areas of high bone turnover such as metaphysis/epiphysisAny ageGradual onset
Pyrexia 
Point tenderness
Pyrexia
Limp
Soft tissue swelling
Local osteopenia
Bony lysis or cortical loss
Periosteal reaction
Spondylolysis Lumbar spineAdolescentsGradual onset
History of repetitive activity involving back extension
Pain on extensions and rotation of lumbar spineLimited compared to CT
Scotty dog sign: oblique view, break in pars interarticularis can have appearance of collar on dog
Avulsion fractures Any tendon/ligament attachmentAdolescentsSudden onset
History of pain following sudden muscle contraction
Swelling and bruising if superficial
Pain and weakness with resisted movement 
Widening of open apophysis
With or without displacement and angulation
Patellofemoral pain Anterior kneeAdolescentsGradual onset
Worse on running/jumping and ascending stairs
Commonly in young girls
Weak quadriceps
Altered tracking of patella
Pain on flexion of knee
Normal
Slipper upper femoral epiphysis Hip/Knee10-16Gradual/sudden limp
May be non-weight bearing
Limp
Reduced ROM of hip
Out toeing
Forced external rotation on hip flexion
Displacement of epiphysis from physis.
Inflammatory arthritis Any jointAny ageGradual onset
May have multiple joints involved
Associated systemic symptoms
Swelling of one or more joints
Stiffness
Systemic features
Soft tissue swelling
Osteopenia
Loss of joint space
Joint subluxation
Irregular growth
Malignancy Commonly in areas of high bone turnover such as metaphysis/epiphysisAny ageGradual onset
Systemic symptoms
May have pyrexia
May have swelling and tenderness
Lethargy
Pallor
Bone destruction
Irregular borders
Wide zone of transition 
Septic arthritis Any jointAny ageGradual onset
Red hot swollen joint
Pyrexia
Non weight bearing
Swollen, erythematous painful joint
Pyrexia
Normal in early stages

One of the main priorities in patients presenting with atraumatic MSK pain is to seek out red flag symptoms and signs in order to rule out sinister diagnosis.

Red flag symptoms and signs include:

  • Weight loss
  • Night sweats
  • Pyrexia
  • Nocturnal pain
  • Non weightbearing
  • Rash
  • Eye pain

A standardised assessment should be performed depending on the body area/areas involved.  Look for any swelling, bruising, erythema or cellulitis.  Determine if there is any point tenderness which may give indicate a possible fracture.  Assess joint function for range of motion (passive / active / resisted), weight bearing status, additional stiffness and stability.

Always ask yourself, is the story really atraumatic?  Has there been any possibility of non-accidental injury?

Some level of investigation is required for each of the potential diagnoses. You may make the diagnosis of apophysitis based on an accurate history and exam alone but even then, one study found that 5% of management plans were altered following baseline x-ray in patients with Sever’s disease (apophysitis of the calcaneus).

A low threshold for a baseline x-ray is sensible in all paediatric patients presenting to secondary care with atraumatic MSK pain.  They can be helpful to identify bony lesions, signs of osteomyelitis and unexpected fractures.

Inflammatory markers are useful when the patient’s presentation is concerning an infective or inflammatory process.

Marie is a 12-year-old girl who presents to you complaining of anterior knee pain.  She is an active volleyball player and is trying hard to make her school team.  The pain is getting worse over the past month and is now affecting her ability to train. She denies any trauma.

What are your differential diagnoses?

What factors in the history and exam would you like to elicit in order to narrow the diagnosis?

You feel she has apophysitis of her tibial tuberosity. 

What is the pathophysiology of apophysitis?

Can you name any other common sites affected by apophysitis?

What investigations would you like to perform?

What is your treatment plan?

  • Patello-femoral pain
  • Apophysitis of tibial tuberosity (Osgood Schlatter disease) or inferior pole of patella (Sinding-Larsen-Johansson)
  • Osteochondritis dissecans
  • Arthritis
  • Malignancy
  • Osteomyelitis
  • Hip pathology

Apophysitis has a typical history of gradual onset localised pain in a child from 10-16 years of age.  Pain is exacerbated by activity and initially improves with rest.  The typical patient is highly active and may be overtraining.  Examination will typically reveal point tenderness over the area involved with possibly some mild swelling.

Important factors to exclude are pyrexia, trauma, weight loss and systemic symptoms.

An apophysis is an area of bony growth in children separate to ossification centres.  It is the site of tendon or ligament attachment and fuses with the bone as the body matures. Rapid growth and repetitive movements combined with relative bone weakness, cause increased traction forces at the point of tendon attachment.  This leads to micro-separation and bone fragmentation which is known as apophysitis.  This clinically presents as an insidious onset focal pain, worsened by activity and eased by rest.  There may be point tenderness and swelling on exam. 

Apophysitis commonly affects:

  • Tibial tuberosity: Osgood Schlatter disease
  • Inferior pole of the patella: Sinding-Larsen-Johansson disease
  • Calcaneal tuberosity: Sever’s disease
  • Medial vondyle of elbow: Little leaguers’ elbow

Apophysitis is described as a clinical diagnosis and as such patients do not require any investigations.

Despite this, it is reasonable with the above differentials in mind to perform a baseline x-ray in all patients presenting with possible apophysitis.  This is especially important if the patient presentation or clinical course is atypical.

One study found that 5% of management plans were altered following baseline x-ray in patients with Sever’s disease.

Knee x-ray of a 12-year-old female volleyball player.  She is presenting with progressive pain over her tibial tuberosity.  Her pain is exacerbated by jumping.  The x-ray shows fragmentation of apophysis with overlying soft tissue swelling.  Some isolated fragmentation can be normal at the tibial tuberosity.

Case courtesy of Dr Hani Salam, Radiopaedia.org, rID: 9740

https://radiopaedia.org/articles/osgood-schlatter-disease?lang=gb

Plain ankle radiograph of an 11-year-old male basketball player complaining of heel pain. There is increased density of the calcaneal apophysis, typical for ages between 7 and 14 years. There is loss of fat/soft tissue planes in the region of the retrocalcaneal bursa in keeping with acute inflammation. This may be seen in the context of the clinical diagnosis of Sever’s disease.

https://radiopaedia.org/cases/sever-disease-5

Case courtesy of Dr Dinesh Brand, Radiopaedia.org, rID: 60324

  • There is sparse evidence looking at appropriate type and length of treatment for apophysitis. This has led to guidance on treatment being expert opinion only.
  • Traditional treatment plans have involved activity cessation until symptoms free with a gradual return.
  • Most of the research that is available focuses on Osgood Schlatter and Severs disease. 
  • One RCT looking at management of Osgood Schlatter disease looked at the effectiveness of dextrose injections vs steroids injection vs normal therapy.  This study showed small benefits of dextrose injections.  But this is unlikely to be a sensible treatment plan due to possible adverse effects in a self-limiting condition
  • There have been recent developments looking at active treatment pathways.  These are moving away from total rest and sport cessation. Instead the aim is to move towards active and monitored treatment plans. Rathleff et al (2020) have a good infographic describing different treatment options for Osgood Schlatter’s.
  • There is some weak evidence that treatment of Sever’s disease with heel raises can improve symptoms when compared to physiotherapy or doing nothing.
  • The type of heel raise does not need to be customised, but whatever is comfortable and available to the patient.
  • Principles can be adopted to all forms of apophysitis with the main aims of treatment being
    1. Altering current activity to prevent worsening symptoms
    2. Stretching and strengthening programmes as appropriate
    3. Cross training
    4. Graduated return to sporting activity
    5. Prevention of recurrence
  • Although apophysitis is self-limiting.  One study found that up to 40% of patients will continue to suffer from intermittent pain even 2 years after diagnosis.  This pain might not necessarily prevent a return to sporting activities.

Exercise programmes https://bjsm.bmj.com/pages/wp-content/uploads/sites/17/2019/06/OSD-table.pdf

Good podcast for extended learning https://soundcloud.com/bmjpodcasts/osgood-schlatter-not-the-self-limiting-condition-we-once-thought-episode-384

Katie is a 9-year-old complaining of left foot pain.  The pain has been getting worse over the past month and she is now beginning to develop some stiffness.  She is a keen athlete and trains five times per week.  She denies any trauma and is systemically well.

What are some of the differential diagnosis?

What changes do you see on the x-ray?

What is the diagnosis?

What is the pathophysiology?

What other sites can be affected?

What is your treatment plan for this patient?

  • Stress fracture
  • Arthritis
  • Osteochondrosis
  • Apophysitis
  • Arthritis
  • Osteomyelitis
  • Malignancy
  • Retained foreign body

Flattening and sclerosis of the navicular bone. Mild soft tissue swelling. No fracture seen.

Kohler disease: osteochondrosis of the navicular bone.

Typical x-ray Findings of osteochondrosis:

Early: Potentially normal

Initial radiological findings

  • Irregular epiphyseal growth
  • Flattening of the epiphysis
  • Soft tissue swelling

Radiological findings as disease progresses

  • Sclerosis
  • Fragmentation
  • Joint destruction
  • Osteochondrosis is often described as idiopathic osteonecrosis.
  • It is a disorder of bone growth primarily involving the ossification centres at the epiphysis.
  • It leads to altered bone and cartilage formation beyond the growth plate.
  • There are some links showing genetic factors and high activity levels can increase a person’s risk of developing osteochondrosis.
  • You should always ensure the osteonecrosis is not from a secondary cause such as sickle cell disease or leukaemia.
Common Location   Eponymous nameAge of onset
Femoral headLegg-Calve-Perthes (Perthes)4-8
CapitellumPanner10-16
CapitellumKohler4-10
Head of metatarsals (2nd most commonly) Freiberg13-18

Treatment in this case will involve activity modification.  This will entail reduced overall activity but and specifically avoiding activity which stresses the foot.  Immobilisation in a walking boot may be beneficial if there is significant pain or inability to weight bear comfortably.

  • Osteochondrosis is self-limiting and the bone will eventually revascularise. 
  • The goal of therapy is to facilitate maximal revascularisation while minimising long term affects.

Three broad treatment strategies exist for osteochondrosis

Conservative: This will involve modified activity to ensure no further stress is placed on the area involved. A physio programme can help to strengthen the area and improve joint function.  This approach is suitable for patients with minimal symptoms and early changes of disease progression on x-ray.

Immobilisation:  Immobilisation can be beneficial for patients with significant pain or more advanced changes on x-ray.  This may be in the form of a cast, walking boot or splint depending on the area involved.  This needs to be weighed up against the risk of worsening joint stiffness.

Surgery:  It is very rare, if ever, that patients will require surgery.  When used it is only in advanced stages of disease and when appropriate conservative management has proved ineffective. Surgical options include osteotomy, arthroplasty and physeal drilling.

A 15-year-old girl attends with intermittent pain and swelling to her left knee for the past two months.  She is a keen soccer player but pain on the medial aspect of her knee is affecting her ability to run. She complains that after every game her knee swells and is now taking increasingly longer to subside. On exam she is walking with a limp, her knee is swollen and she has pain to the medial joint line.  Her knee feels stable with all ligaments intact on testing.

 You decide to do an x-ray:

Describe the x-ray findings.

Are you aware of any grading system used for Osteochondritis dissecans?

What investigations should you consider?

Largely normal knee x-ray. Subtle lucency to chondral surface of medial femoral condyle. Findings consistent with osteochondritis dissecans. 

  • Osteochondritis Dissecans is a focal injury disruption of articular cartilage and subchondral bone.
  • It is largely idiopathic, but some theoretical causes are genetics, trauma or vascular phenomenon.
  • There is a juvenile and adult form.  The juvenile version is most common and present in patients between 10 and 16 with open physis.
  • The knee is the most common joint involved with >70% of knee lesions being in the posterolateral aspect of the medial femoral condyle.  The ankle and elbow are other joints that are regularly affected.

Presentation depends on the stage of lesion.  Initially nonspecific pain with or without swelling.  As the process progresses, patients can develop mechanical symptoms such as reduced range of movement (ROM) and locking of joints.

Osteochondritis dessicans grading system:

Clanton Classification of Osteochondritis (Clanton and DeLee)

Type IDepressed osteochondral fracture
Type IIFragment attached by osseous bridge
Type IIIDetached non-displaced fragment
Type IVDisplaced fragment

  • Bilateral x-rays should be obtained as up to 30% of patients can have bilateral lesions.
  • An additional notch view x-ray can help to get a better image of the femoral intercondylar spaces.  This is taken with the patient supine and knee flexed to 40 degrees.
  • MRI is widely used in patients with high suspicion for osteochondritis dissecans or to assess stability of a lesion diagnosed on x-ray. 
  • MRI has superior capabilities for assessing stability of cartilage and subchondral bone when compared to standard radiographs.
  • Arthroscopy is the gold standard for assessing stability and may be used if questions marks remain following MRI

There is a lack of consensus on appropriate type and length of treatment.

The latest American Academy of Orthopaedic surgeons’ guidelines were unable to recommend any conservative treatment regime.

Masquijo and Kothari (2019) illustrate their preferred treatment algorithm in a flowchart 

Modern protocols recommend patients should have a three to 6 months trial of conservative management. 

Conservative management involves

  • Immobilisation phase with minimal weight bearing followed by
  • A phase of partial weightbearing and
  • Lastly a gradual supervised return to activity.

Favourable prognostic factors are:

  • Younger age
  • Open distal femoral physis

Poor prognostic factors include

  • Lesions involving the patella
  • Sclerosis on x-ray

Approximately 50-75% of lesions will heal in 6-12 months following conservative management.

Conservative management is not suitable for displaced or loose fragments.

Surgical treatment

Surgical treatment is preserved for large or unstable lesions, displaced fragments and those not responding to conservative therapy

Three broad surgical options

  • Stimulate growth by retroarticular drilling,
  • Reducing and fixing displaced fragments
  • Osteochondral grafts

Drilling and fixation are usually successful surgical options with minimal complications.  Up to 90% of patients can expect radiographic resolution of lesions.  Unfortunately, grafts are usually seen as salvaging surgeries and outcomes can be variable.

Judith is a 10-year-old girl who is attending with pain and stiffness to bilateral wrists with intermittent swelling to fingers.  She has no history of trauma. You think she may have arthritis.

What will you want to decipher during your history and exam?

Our patient has bilateral wrist, metacarpophalangeal and proximal inter phalangeal joints involvement.  She complains of some morning stiffness but denies any previous medical problems.  She cannot remember any trauma and has not had any temperatures or rashes.

What are your differentials?

You think this patient has Juvenile Idiopathic Arthritis. What is JIA?

What investigations will help with this diagnosis?

What is your chosen treatment for JIA?

Apart from rheumatology who else should see this patient with JIA?

Length of symptoms: This is important as JIA can only be diagnosed once the patient has had symptoms for over 6 weeks without any other cause found.

Effect on activities: Has the patient stopped playing a sport they previously enjoyed.  Is the patient regressing at physical activity or schoolwork such as handwriting?

Pattern of symptoms: Important to elicit if any stiffness or worsening of symptoms in the morning.  Ask about symptoms associated with malignancy such as leukaemia.

Illicit any associated symptoms which may help discover a cause such as multisystem condition (Lupus/Vasculitis/psoriasis) or a reactive arthritis from a satellite infection (UTI/STI)

You should also enquire about symptoms which may indicate complications such as eye pain caused by uveitis or tendon pain caused by enthesitis.

Pattern of joint involvement.

Monoarthropathy: Single joint. Can still be JIA but infection, trauma and malignancy would be higher on your list and need to be consciously out ruled.

Oligoarthritis: Four or fewer joints involved

Polyarthritis: Over four joints involved.

Typical patterns include:

  • Asymmetric, small and large joints and distal interphalangeal joint involvement is typical of psoriatic arthritis.
  • Symmetric, small and large joints is typical of polyarticular JIA.
  • Hip involvement and enthesitis is typical of Enthesitis-Related Arthritis. 
  • Large joint and intermittent / flitting involvement is typical of acute rheumatic fever. 
  • Fever, rash and serositis and later symmetrical involvement of small and large joints (including distal small joints of the hands, ankle or wrist involvement) are typical of systemic JIA.

Examination: 

  • Trauma: Bruising, wound, bleeding, deformity.
  • Infection: Guarding joint and refusing to move/weight bear.  Hot red and swollen.  Temperature
  • Malignancy: Secondary signs of anaemia, thrombocytopenia.  Cachexia in advanced stages.  Unusual swelling i.e. not involving a joint and not in an area typically injured.
  • Rashes: look for signs of psoriasis, vasculitis rashes, rheumatological rashes as seen in lupus, skin changes produced by Kawasaki.
  • Joints: As previously mentioned look for patterns of swelling, record joints involved, assess range of motion and function of joints.
  • Review of systems: Assess for any other signs of systemic disease, distant infection or complications which may give you a clue to the aetiology of the arthropathy.

The most likely diagnosis is JIA but there are also some other reasonable differentials.

  • Psoriatic arthritis
  • Post viral arthritis
  • Rheumatic fever
  • Malignancy
  • Metabolic disease (rickets/osteomalacia)

JIA comprises a group of inflammatory disorders that begins before the 18th birthday and persists for at least 6 weeks and other known conditions are excluded.

There are six main disorders of JIA with their own individual diagnostic criteria

  • Systemic JIA
  • RF-Positive JIA
  • Enthesitis/Spondylitis-related JIA
  • Early onset ANA positive JIA
  • Other JIA: does not meet

For further background on the individual criteria. http://www.jrheum.org/content/46/2/190

No blood or radiological test can definitively make a diagnosis of JIA.  The diagnosis is based on careful clinical assessment, exclusion of other possible causes and aided by blood and radiology.

pGALS screen has the benefit of quickly assessing all joints. pGALS is a standardised musculoskeletal (MSK) basic examination. Free educational resources to demonstrate pGALS are available online. (www.pmmonline.org).

  • CRP and ESR give an indication of total body inflammation.  But you cannot rule out the diagnosis if inflammatory markers are normal.  These markers are more useful in disease monitoring.
  • Antinuclear antibodies (ANA) are positive in roughly 50% of oligoarticular JIA, however positive ANA can be seen in healthy children also.  A positive ANA can indicate a higher risk of uveitis once JIA is diagnosed.
  • HLA-B27 is positive in 27% of patients with JIA and up to 80% of patients with enthesitis related arthropathy.
  • Rheumatoid factor can help with diagnosis of RF-positive JIA.  It also provides a worse prognosis if positive.
  • Radiographs are useful for investigating alternative causes.  They rarely show any changes in the early stages of arthropathy but are important to get a baseline condition of the joints.

Refer early. Treatment should be initiated by a specialist. If you have concerns about JIA, you should discuss with rheumatology.  The dawn of biologic treatment has ensured reduced symptoms, chronic complications and minimised need for systemic steroids.

Ophthalmology.  JIA patients are at risk of losing their eyesight from chronic uveitis and may be asymptomatic despite having eye changes.

Which of these conditions and age of onset do not match?

A: Apophysitis 10-14 F 12-16 M

B: Osteochondrosis 12-18

C: SUFE 10-16

D: Osteochondritis dissecans >10

E: Septic arthritis Any age

B: Osteochondrosis, the usual age of onset is anywhere between 4 and 18.  There have even been some Kohler diseases documented as young as 3.

Which of these anatomical areas do you not commonly see apophysitis?

A: Capitellum

B: Inferior pole of patella

C: Tibial tuberosity

D: Medial condyle of elbow

E: Calcaneal tuberosity

A: Capitellum.  Apophysitis only occurs at sight of apophysis formation.

Which of these statements about osteochondritis dissecans is not true?

A: Osteochondritis is a focal disruption of articular cartilage and subchondral bone

B: Most commonly seen on the medial femoral condyle

C: The aetiology is largely unknown

D: Recovery can take 6-12 months

E: A closed physis is a good prognostic factor

E: An open physis is seen as a good prognostic factor

Which of these is not a radiological finding of osteochondrosis?

A: Sclerosis

B: Fragmentation

C: Flattening of epiphysis 

D: Lytic lesion

E: Irregular epiphyseal growth

D: Lytic lesions.  There may be no radiographic changes in the initial stages of osteochondrosis.  You will then begin to see epiphyseal changes, soft tissue swelling, fragmentation and sclerosis. 

Lytic lesions would help point you towards another possible differential diagnosis depending on its location and characteristics.  Lesions can be caused by infections, malignancy or simple cysts.

Apophysitis:

Brenner, J. S. (2007). Overuse Injuries, Overtraining, and Burnout in Child and Adolescent Athletes. Pediatrics, 119(6), 1242 LP – 1245

Rathleff, M. S., Winiarski, L., Krommes, K., Graven-Nielsen, T., Hölmich, P., Olesen, J. L., … Thorborg, K. (2020). Activity Modification and Knee Strengthening for Osgood-Schlatter Disease: A Prospective Cohort Study. Orthopaedic Journal of Sports Medicine.

James, A. M., Williams, C. M., & Haines, T. P. (2013). “Effectiveness of interventions in reducing pain and maintaining physical activity in children and adolescents with calcaneal apophysitis (Sever’s disease): a systematic review.” Journal of Foot and Ankle Research, 6(1), 16.

Osteochondrosis

Achar, S., & Yamanaka, J. (2019). Apophysitis and Osteochondrosis: Common

Causes of Pain in Growing Bones. American Family Physician, 99(10), 610–

618.

Chan, J. Y., & Young, J. L. (2019). K&#xf6;hler Disease: Avascular Necrosis in the Child. Foot and Ankle Clinics, 24(1), 83–88.

Osteochondritis dissecans

Masquijo, J., & Kothari, A. (2019). Juvenile osteochondritis dissecans (JOCD) of the knee: current

concepts review. EFORT Open Reviews, 4(5), 201–212.

American Academy of Orthopedic Surgeons. Clinical practice guideline on the diagnosis and treatment of osteochondritis dissecans. Rosemont, IL: American Academy of Orthopedic Surgeons, 2010.

JIA

Alberto Martini et al, for the Pediatric Rheumatology International Trials Organization (PRINTO) Toward New Classification Criteria for Juvenile Idiopathic Arthritis: First Steps, Pediatric Rheumatology International Trials Organization International Consensus, The Journal of Rheumatology Feb 2019, 46 (2) 190-197

Jason Palman, Stephanie Shoop-Worrall, Kimme Hyrich, Janet E. McDonagh, Update on the epidemiology, risk factors and disease outcomes of Juvenile idiopathic arthritis, Best Practice & Research Clinical Rheumatology, Volume 32, Issue 2, 2018, Pages 206-222,



Please download our Facilitator and Learner guides

Rapid Sequence Induction and the Difficult Airway Module

Cite this article as:
Robyn Goodier. Rapid Sequence Induction and the Difficult Airway Module, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.30071
TopicRSI and the difficult airway
AuthorRobyn Goodier
DurationUp to 2 hrs
Equipment requiredCan be done without equipment, however for interactivity it would be useful to have different laryngoscopes/ETT/bougie/stylet etc for demonstration purposes.
  • Basics – including airway plans and assessment (30 mins)
  • Main session: (2 x 15 minute) case discussions 
  • Advanced session: (2 x 20 minutes) case discussions covering more controversial settings
  • Sim scenario – optional (30-60 mins)
  • Quiz (10 mins)
  • Infographic sharing (5 mins): 5 take home learning points

Paediatric airway compromise requiring emergency management by rapid sequence induction (RSI) is a rare event in the Emergency Department. However, despite it being rare, it is associated with high mortality and morbidity with an overall death rate of 3.8%, the highest for a critically unwell child. 

Airway securement is a procedure that every critical care physician should be competent in performing. 

RSI is an airway management technique that produces unconsciousness and muscular relaxation for the purposes of intubating and taking control of the emergency airway. The airway is usually intubated and controlled within 3 minutes of paralysis. 

Don’t forget ABC…

A – Airway protection, this can be due to numerous reasons such as burns, penetrating neck injury 

B – Respiratory failure – hypoventilation, severe asthma, hypercarbia 

C- Circulatory collapse – severe sepsis

D – Neurological problems – termination of seizures, need for neuroprotection, GCS <8, C Spine trauma, diaphragmatic paralysis

E-  Everything else! Transportation or facilitation of procedure, for patient safety (e.g. combative patient)

Once you have decided that you need to intubate the child, you should prepare to intubate the child using a local cognitive aid. The Twelve P’s of RSI are a useful way to intubate the child safely and successfully, however please refer to your local guidance. 

To ensure you are correctly prepared, we would advocate the use of an airway checklist. This is a checklist to ensure that all aspects of the RSI have been thought about to mitigate any omissions during the procedure. This is an example airway checklist from Liverpool Hospital, Sydney (Airway Checklist) recommended by the Emergency Care Institute in Australia. 

Please check your hospital for your airway checklist – if you don’t have one, then check the ECI website for a blank version to create your own! They are a great aide memoire for a safe intubation. 

Preparation includes:

Roles allocated
Team Leader
Airway Doctor
Airway Nurse
Procedure Nurse x2 for drug checking
Procedure doctor – usually the drug giver
Scribe 

This is the minimum set up – you may have more but ensure your roles are clearly allocated

Labels on the front of scrubs can help the team know who is responsible for each role. 

Have you considered calling for help? The definition of help will depend on your setting but could include Emergency Consultant, Anaesthetics or ICU. 

Equipment required – remember the mnemonic SOAP ME

Suction – large bore suction (x2 if soiled airway) under the pillow and turned on 

Oxygen (mask and BVM ventilation)

Airway equipment:

  • Bag valve mask with PEEP valve, oxygen on. (Neopuff for infants <10kg may be more effective than BVM)
  • Nasal prongs for apnoeic oxygenation
  • Adjuncts available – specifically Oropharyngeal and nasopharyngeal airway devices (x2). A correctly sized LMA (Laryngeal mask airway) should also be available.
  • Laryngoscope – direct and video -(direct – light checked, video – plugged in and tested)
  • ETT – size up and down also available, cuff tested and lubricated
  • 10ml syringe
  • Tube tie or tape avaliable
  • Ventilator (checked) with a paediatric circuit 
  • Bougie/stylet – (size selected)

Pharmacy:
Patent IV line with fluids available – from bag or flushes drawn up 

Specific RSI medications: Correct doses drawn up, labelled correctly as per local guidelines, order of medications to be given decided before administration 

Monitoring Equipment:
ECG
NIBP on 2 minutely cycles (or arterial line if already inserted)
SpO2 probe with good trace
ETCO2 – attached to the circuit, if ETCO2 is unavailable alternative capnography such as colour capnography should be used.

How do I size my equipment?

ETT 
ETT = age / 4 + 4 (for uncuffed tube)
Age / 4 + 3.5 (for cuffed tube)

Depth of insertion
<1 year insert to 10cm
>1 year age/2 + 12cm 

Laryngoscope 
Miller blades are straight blades which are designed to directly lift the epiglottis

MAC blades are curved to sit in the vallecula to lift the epiglottis indirectly but putting pressure on the glossoepiglottic ligament. 

Miller blades are better for neonates and young infants – up to 1 year then MAC blades are better. Miller are used in this age group due to their large floppy epiglottis and laxity of the ligament. 

< 1 year Miller 00, 0 and 1 

>1 year MAC size 2 and 3 (size 3 usually 5 years upwards)

Please see your local policy for what is available in the area in which you work.

Royal Children’s Hospital Melbourne Airway Recommendations

Remember – you can intubate with a larger blade, but not a smaller one! If in doubt go for the bigger one!

Anatomical differences in paediatric vs adult airways

  1. The airways are smaller! This might sound very obvious, however, this means that there is a lot less room for other things such as secretions, oedema and foreign bodies. Also, external compression can lead to rapid increase in airway resistance 
  2. Larger tongue and adenoids – increases the difficulty in advancing the laryngoscope and visualizing the cords on laryngoscopy. Think of Macroglossia seen in conditions such as Trisomy 21 and Beckwith-Wiedemann syndrome 
  3. Large, floppy epiglottis 
  4. Short trachea (high risk of endobronchial intubation)
  5. Soft structures are at higher risk of airway trauma with repeated attempts causing oedema and further airway narrowing 
  6. Large occiputs – neck flexed causes obstruction
  7. Young children have higher and more anterior tracheal openings than adults (C1 in infants, C7 in adults), therefore visualisation of the glottis is difficult
  8. There is a small cricothyroid membrane so landmarks for surgical airways are more difficult to locate

Positioning in an RSI

  • Infant should be a neutral position
  • Younger child consider a shoulder roll 
  • Older child use an occipital pad 

What are the differences in physiology in intubating children?

The two most important things to consider are:

  1. Oxygen consumption – this is much greater than in an adult counterpart especially when unwell. There is a lower functional reserve capacity and it can cause rapid desaturation during laryngoscopy and intubation despite adequate preoxygenation. 
  2. Horizontal ribs limit the ability to increase tidal volume and ventilation is predominantly diaphragmatic, any air in the stomach may splint the diaphragm and make ventilation difficult. Prompt decompression of the stomach post intubation via NGT or OGT will reduce this splinting and improve ventilation.
    In the <12 month old the NGT can be inserted during the preoxygenation phase of intubation.

Pre-oxygenation should be done for all patients requiring an RSI. The aim is to wash out all of the nitrogen from the lungs and replace it with oxygen, thereby creating a reservoir of oxygen within the lungs. This is especially critical in children due to their propensity to desaturate quickly. 

Pre-oxygenation can be done in a number of ways and will largely depend on the patient’s physiology. All patients (except trauma with suspected base of skull fracture) should have nasal prong oxygen delivering 15L min on in addition to preoxygenation aids.

  • If the patient is awake and spontaneously ventilating well consider 15L oxygen via non- rebreathe mask that it fitted well. Otherwise bag-valve-mask with PEEP valve at 15L oxygen 
  • In the obtunded patient use bag-valve-mask with PEEP valve at 15L oxygen with assisted ventilations for 5 minutes prior to induction. 

Use a LEMON!

 L – Look Externally

  • Body habitus
  • Head and neck anatomy
  • Small mouth 
  • Teeth – overcrowding? Loose? 
  • Jaw abnormalities- micrognathia?
  • Tongue – Macroglossia?

E – Evaluate

  • Mouth opening
  • Thyromental distance

M – Mallampati 

  • Ability to view may be easier in older children who are cooperative 
  • Difficult to do in an emergency situation
  • The higher the number the more difficult the airway is predicted to be

NB This should be done ideally in an upright patient without vocalisation.

https://www.clinicaladvisor.com/home/the-waiting-room/understanding-the-mallampati-score/

O- Obstruction

  • Head and neck abnormalities i.e. cancer, surgeries, laryngectomy 
  • Foreign body
  • Burns
  • Epiglottitis

N – Neck mobility

  • Remember in children to have a more neutral neck position 
  • May have immobilised due to trauma (C Spine collar)


Respiratory

  • This goes with what we had predicted earlier that desaturation occurs quickly in children
  • Those with underlying respiratory disease, or acutely unwell with respiratory distress will desaturate quickly
  • Ensure preoxygenation is given well and that apnoeic oxygenation is maintained 
  • Ensure laryngoscopy time is limited

Cardiovascular

  • Children are sensitive to changes in circulatory volume, they can compensate up to a point by increasing their heart rate but not their stroke volume. They decompensate very quickly
  • Ensure they are adequately fluid resuscitated prior to intubation. Consider concurrent inotropes if there are concerns over pre induction haemodynamic instability.

Disability

  • This is a metabolically stressful situation and children are prone to hypoglycaemia due to their lower glycogen stores in the liver. Ensure BSL checks are done regularly, hypoglycaemia is promptly corrected and that maintenance fluids contain 5% dextrose. 

So, you have everything ready and the team leader asks you for your airway plan, “what is an airway plan?” you ask….

As the airway doctor you should have an airway plan which is verbalised to the entire team so everyone is aware of the expected sequence of events. 

This is an example of an airway plan from Life In the Fast Lane. 

At each plan everyone is aware of the expected outcome and the triggers for moving on to each section. Although this is written for adults, the same is true of paediatrics.

IF YOU PREDICT A DIFFICULT AIRWAY, VERBALISE THAT TO THE TEAM AND HAVE THE DIFFICULT AIRWAY TROLLEY WITH YOU AND THE AIRWAY NURSE TO BE CLEAR ABOUT WHAT YOU WILL NEED!

Difficult airways will be covered in more detail later – however signposting the Vortex website to learners now is helpful. Vortex approach is an approach where there are set triggers meaning you move further down the vortex mental model to prepare for front of neck access. The website has invaluable information regarding CICO packs and an instructional video of paediatric front of neck access.  

Consider atropine – this will be discussed in detail later.

Induction agent of choice – i.e. ketamine, propofol, thiopentone

This will differ with institutions, clinical picture, availability and personal preference

The majority of emergency environments will now prefer ketamine as the induction agent of choice, except for status epilepticus where thiopentone is preferred, however this is site specific. 

In a neonate an induction agent is often not required and it is an opiate based induction using either fentanyl or morphine, remembering that morphine has a longer time of onset. 

Muscle relaxant
Depolarising – e.g. suxamethonium
Non- depolarising e.g Rocuronium, Atracurium etc 

Other medications indicated by presentation e.g. mannitol, adrenaline, midazolam etc 

Post intubation sedation – usually morphine and midazolam, check with the team leader

Perform laryngoscopy

Insert ETT past the vocal cords 

Inflate cuff

  • Attach capnography, end tidal CO2 is gold standard (colourimetry can also be used) 
  • Check for misting of the tube
  • Check for equal air entry and movement of the chest (to ensure not Right main  bronchus intubation)
  • Secure the airway with tape in children, or tie in older children
  • Confirmatory post intubation chest x-ray 

Post intubation care is a large topic on its own and beyond the scope of this session. 

The main considerations post RSI are:

  1. Ensure tube is secured correctly 
  2. NG or OG tube inserted for decompression of the stomach 
  3. Ensure IDC is inserted for drainage of the bladder
  4. Ensure nutrition is addressed (usually ongoing IV fluids in the acute phase) 
  5. Post intubation sedation is running 
  6. Further investigations/procedures/treatments are coordinated with as little disruption to the patient as possible 
  7. Disposition is decided upon

Again, transportation of the critically unwell child is beyond the scope of this teaching session. There are numerous specialist retrieval services that facilitate  interhospital transfers. For any staff member doing transfers within the hospital they should have specialist training.

Robert is a 7 year old boy seen in ED with a cough for 5 days, increasing shortness of breath and fevers. Mum brought him to ED as he was lethargic and breathing quickly. On examination he is lethargic with dry mucous membranes, in respiratory distress with a rate of 45, saturations of 92% on 15L oxygen. He is persistently hypotensive despite 40ml/kg fluids. He is becoming bradycardic and his GCS is now 9. You are worried he is in septic shock with impending respiratory failure and circulatory collapse. You decide to proceed to an emergent RSI.

How can he be optimized physiologically before RSI?

Would you start inotropes?

What is your induction agent of choice for RSI in these haemodynamically compromised children? 

First thing’s first here, this is a very, very sick child – have you called for help? Depending on your setting you will require help from ICU, senior Emergency and Paediatric staff and if not in a tertiary centre from specialist paediatric retrieval services. 

In this setting this child has a high risk of mortality associated with the RSI. Ensure you have optimised and resuscitated as much as possible before the RSI.

Hypotension before intubation is associated with a higher mortality. This child has been fluid resuscitated, therefore you will need pressors to maintain the blood pressure prior to intubation.
In this situation you need to optimize the blood pressure prior to intubation, therefore an adrenaline infusion is the treatment of choice to support the blood pressure during the induction process.

Ketamine is the drug of choice. It exhibits a stimulatory effect on the cardiovascular system and is the least cardiac depressive induction drug available, therefore has the least chance of inducing hypotension. That being said, it is not only the drug that is important but the dose. Smaller amounts of induction agent will be required than a “typical” RSI.

Dosing is usually 1-2 mg/kg, doses of 0.5mg – 1mg/kg would be more appropriate in this setting.

Intubation, Hypotension and Shock • LITFL • CCC Airway
Additional reading – please look at the powerpoint from Dr Chris Nickson 

Jeremy is a 10 year old boy brought in by ambulance after falling off his BMX at a skate park doing a jump without a helmet on. He had a fall from approximately 2 metres onto his head. He had an initial LOC for 2 minutes then was ok, but since then he has had multiple vomits and become drowsy. The ambulance have issued a pre arrival phone call as they are concerned he has a reduced GCS of 8 but no evidence of raised ICP at this stage. The ambulance crew have immobilised his C Spine.

You decide to prepare for an RSI before the child arrives as it seems he will need a secure airway.

How do you do an RSI with a C spine collar on?

His friend tells you they went to McDonalds 2 hours prior to this happening. Would you alter your approach knowing this information? Would you ask for cricoid pressure? 

What is your choice of induction agent and why?

The reason the C Spine collar is on is because of suspected cervical spine trauma, therefore the cervical spine must be protected and avoid hyperextension of the neck during laryngoscopy and intubation. The C Spine collar in children has been contested, with the latest APLS update stating that C spine manual in line stabilisation (MILS) is the preferred option in the conscious patient and that C spine collars can potentially be very distressing for children, fit poorly and therefore a risk/benefit discussion should take place before routinely applying them in children. 

In this case the child has a reduced GCS and a properly fitted, well tolerated collar. Prior to intubation the C spine collar should be removed, however immobilisation should remain in place at all times via MILS. 

The current recommendations of when MILS should be used in general (when C Spine should be thought of) are: 

  • Neck pain or neurological symptoms
  • Altered level of consciousness
  • Blunt injury above the level of the clavicles (significant)

This is aimed to keep the head in a neutral alignment whilst laryngoscopy occurs, to avoid hyperextending the neck. MILS involves a secondary person being tasked with holding the head in neutral alignment, this can either be done facing the intubator and having the hands placed over the side of the head from below, or can be done by crouching beside and underneath the intubator and holding still from above.


Once the airway is intubated the C Spine should be protected with a Philadelphia Collar and sandbags/rolls to ensure ongoing stability is maintained. 

He is likely to have a full stomach or at least food in his stomach which would make him more likely to aspirate, however in an Emergency Situation, not protecting the airway is a larger risk than aspiration. RSI is designed to be a quick induction and reduce the chance of emesis. 

Cricoid pressure was initially thought to help reduce aspiration by blocking the oesophagus, however in children it has been widely contested and not thought to be of benefit. The force required to do cricoid pressure in children is a lot less than in adults; a less trained assistant may cause damage by improperly applied cricoid pressure. It can worsen the view at laryngoscopy and studies have shown that it may only displace the oesophagus laterally and not help with passive aspiration. It can also cause full occlusion of the trachea making intubation impossible.

The short answer is no, you would not change your approach and you would not have routine cricoid pressure. 

Ketamine was previously contraindicated for use in isolated head injury due to the concerns that it raises ICP, however now it is the drug of choice for the head injured child (with the exception of globe injury as ketamine can raise intraocular pressure).

Evidence that it raises ICP was weak. It is advocated for this use now due to its maintenance of haemodynamic stability. 

Haemodynamic stability is very important in traumatic brain injury as hypotension is a major predictor of poor outcomes in TBI, even a single hypotensive event can have deleterious consequences in terms of secondary brain injury. This is a situation where an opiate adjunct would be helpful in ensuring that haemodynamic stability is maintained but so that laryngoscopy does not provoke a hypertensive response. Ketamine activates the sympathetic nervous system, therefore it can result in maintaining cerebral perfusion pressure. Doses should be titrated according to the haemodynamic parameters of the child in front of you; the dosing range is 1 – 2 mg/kg.

Ashleigh is a 2 year old female brought in to you on New Year’s Eve after her sister accidentally let off a firework that exploded in her face.

Ashleigh has obvious burns to her face/neck/chest/upper limbs. When you perform an airway assessment you can hear soft stridor and see burns inside her mouth.

You decide that she has a threatened airway and decide to intubate her. 

Your consultant decides to use suxamethonium as the muscle relaxant of choice. You ask why because you heard it was contraindicated in burns. What is the evidence surrounding use of suxamethonium in burns?

You find yourself in a CICO situation after failed intubation and LMA placement. What is your difficult airway plan for this 2 year old? 

Why is expectant airway management in burns so important?

This is an area that is easy to get confused about. The evidence regarding suxamethonium and burns is that it is safe within the first 24 hours of injury (some evidence states 48 hours) but not for use after 24 hours of injury. After this time it is contraindicated, due to hyperkalaemia (thought to be due to release of potassium from extrajunctional acetylcholine receptors). This potassium release can cause severe hyperkalaemia and lead to cardiac arrest. The important thing to remember is it is contraindicated for 1 year after a burn injury. 

NB The ideal situation for this child is that they are intubated in theatre by an experienced anaesthetist with ENT on standby where there is an option of fibreoptic intubation. This is not available in all institutions. 

Can’t intubate, can’t oxygenate is the worst thing an airway team can hear – but they MUST hear it. The first thing to do is ensure you have said loudly to the team that they are in can’t intubate, can’t oxygenate situation. 

  1. If anaesthetics were not involved earlier, they need to be involved and called now
  2. Consider waking the child up – in this case with airway burns it is prudent to establish access otherwise the airway will be lost later
  3. Front of neck access – the question here is how to puncture the neck – needle or knife?

DAS UK guidelines suggest that children over 8 should have a “scalpel, finger, bougie” technique. Under 8 the cricothyroid membrane is so small that needle jet insufflation should be utilised. Early involvement of ENT and anaesthetics is a must. 


The technique for this as described by DFTB:

  • Extend the neck (making the target as big as possible)
  • Stabilize the larynx with the non-dominant hand
  • Access the cricothyroid membrane with a dedicate 14/16g cannula
  • Aim in a caudal direction
  • Confirm position with aspiration of air into a syringe containing saline
  • Connect to oxygen source
  • Adjustable, pressure limiting device – some departments will have a specific jet insufflation device, other institutions may have to create their own. This can be done by attaching IV tubing to a 3 way tap directly onto the cannula and occluding the 3 way tap to be the breath, proximal end of the tubing can be attached to the oxygen source. Please check your department to see what is available. 
  • 4bar O2 source (hospital oxygen wall meter delivering 10-15L) – matching l/min with age
  • Slowly increase inflation pressure/flow rate to achieve maximal chest rise
  • Maintain upper airway patency to aid expiration

Front of neck access is rarely done, however it is a lifesaving skill that all critical care physicians looking after both adults and children should be able to do. Practice on mannequins and watch videos so that you are able to call upon your knowledge should you ever have to use it!

Airway swelling rapidly increases after the burn and is at risk of airway closure and difficulty intubating the airway later.
Signs of airway burns:

  • History of burn in enclosed space
  • Upper airway oedema (swollen tongue and lips)
  • Sooty sputum (may not be able to assess in a young child that cannot expectorate)
  • Facial burns, singed nasal hair, soot in the mouth
  • Respiratory distress (dyspnoea, stridor, wheeze, hoarse voice)

If any of those are present the airway is at risk and consider intubation of the airway earlier rather than later.

Lily is a 2 month old infant being brought into ED by her mum as she is not feeding well and she has noticed her breathing is abnormal. She has an unremarkable birth history, born at term via NVD, GBS negative, Apgars 9 +9.

She has an older brother Isaac who attends daycare and has a runny nose recently. 

Lily is in respiratory distress with grunting, nasal flaring, recession and head bobbing. You have tried HFNP and CPAP to little avail over the past 3 hours. She is now tiring and is becoming bradypnoeic and bradycardic. To prevent cardiac arrest you decide to intubate this child so proceed to an RSI. 

Does this child need atropine preloading? Do all children need atropine?

Would you use a cuffed or uncuffed ETT?

Would you use a bougie?


There is much debate regarding premedication with atropine prior to RSI. The idea behind atropine as a pre RSI agent is that it increases the heart rate prior to induction to reduce the chance of bradycardia on induction. 

There have been multiple studies which have suggested that atropine is not routinely required for premedication for an RSI and that uncontrolled hypoxia is the largest determinant in bradycardia when compared to the use or not of atropine. 

In this case you could consider atropine given that the patient already has a bradycardia secondary to her respiratory failure, however you could also argue that adrenaline would be a better choice to reverse her bradycardia and improved general perfusion prior to induction. 

This decision would be made with senior decision makers as an RSI in this situation would be high risk due to her already deranged physiological parameters. 

Atropine is not a drug to be given “just in case”, careful consideration needs to be given as it is not without important side effects such as increased temperature with a risk of malignant hyperthermia: at too high a dose it can induce ventricular arrhythmias, at too low a dose it can cause bradycardia. It lowers seizure threshold and increases risk of aspiration by relaxing the lower oesophageal sphincter.

The general rule is no, it is not needed for every RSI, however it should be drawn up and available in the event of a bradycardia. However if you start out bradycardic prior to induction then this needs treatment otherwise there is significant risk of clinical deterioration or cardiac arrest during induction. 

First of all why are we asking this question? In an adult circumstance the answer is always a cuffed ETT, so why is there a choice in paediatrics?

The issue comes from neonates. Cuffed ETTs are thought to cause cuff-related trauma and subglottic stenosis, despite the benefits of a cuffed ETT (better aspiration protection, more accurate ETCO2 detection and lung recruitment). These rates however are much lower than previously thought and the evidence suggests that cuffed tubes are more advantageous. The current APLS guidance is that cuffed ET tubes are advantageous however it requires meticulous attention to size, cuff pressure, and to exact placement to ensure it is in the correct position. 

This will be determined by what is available in the correct size in your department, the correct size is more important than cuffed vs uncuffed. Remember, if you are not in a tertiary centre the tube can always be exchanged if there is an issue. Please check in your department what is the accepted practice and be aware of the availability of these tubes.

A  bougie is a plastic stick which is used to help instrument the airway; it acts as a rigid placeholder for the ETT to be railroaded over the top. In many emergency airways the bougie is the first thing to be called for, however, be aware that it is not small enough for paediatric airways especially neonatal ones. Check and see what your department has – most may have an adult and paediatric bougie with the paediatric bougie being compatible until approximately a size 5 ETT. You do not want to intubate the airway with a bougie and then realise your ETT does not fit!

With the smaller airway especially the neonatal airway then you use a stylet which is small enough for the ETT to fit over the top.

Difficult airway leading to cricothyroidotomy Paediatric Difficult Airway Simulation

More airway learning material Optimus Bonus

The airway assessment mnemonic is named after which fruit? 

A: MANGO – Mallampati/Airway Diameter/Neck/Gnashers/Obstruction

B: APPLE – Airway diameter/Positioning/Palate/Look/Evaluate

C: LEMON – Look/Evaluate/Mallampati/Obstruction/Neck

D: LIME – Look/Incisor distance/Mallampati/Evaluate

C: LEMON

Don’t forget it is a LEMON! Look first, then evaluate, check the mallampati, check for obstructions and lastly look at the neck!

What can you use to help optimise airway anatomy when intubating a small child? 

A: Philly collar

B: Neck/shoulder roll

C: Head of the bed sloping downwards

D: Put the child in the recovery position

B: Neck/shoulder roll

Neck/shoulder roll should be used to due to the large occiput in a child to ensure appropriate position for laryngoscopy.

Which of these is an indication for an RSI in the Emergency Department?

A: Elective surgery for inguinal hernia repair 

B: Suspected airway burns

C: Child with GCS 14 

D: Trauma – isolated leg injury but going to theatre in a few hours

B: Suspected airway burns

Airway burns need to be managed promptly. If possible this should be done in theatre by anaesthetics, but not all centres have this availability therefore it may have to be done in an Emergency Department setting.



Please download our Facilitator and Learner guides

Pneumonia Module

Cite this article as:
Ellis Collins and Michelle Alisio. Pneumonia Module, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.30049
TopicPneumonia
AuthorEllis Collins & Michelle Alisio
Duration1- 2 hrs
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

Khan Academy: What is pneumonia? (9 mins) OR

Khan Academy: Classification of lung diseases. (restrictive, obstructive, ventilation and perfusion lung problems 11mins)

GPpaedstips: Diagnosing a lower respiratory tract infection (LRTI)

LITFL: Pneumonia in the ED

Paediatric clinical examinations- The respiratory system (7mins)

DFTB: Respiratory infections

RCH: Community Acquired Pneumonia

DFTB: The Mire of Mycoplasma

DFTB: POCUS and Pneumonia

ALiEM: Lung Ultrasound for diagnosing pneumonia

Substituting POCUS for CXR Podcast on using lung USS (11 mins)

Pathophysiology and background

According to the WHO pneumonia kills more children than any other illness – more than AIDS, malaria and measles combined. In 2017 pneumonia accounted for 15% of all deaths of children under 5 years old, killing 808 694 children and it accounts for nearly one in five child deaths globally. It should also be noted that pneumonia is one of the leading causes of deaths for children under the age of 5.

Pneumonia is an invasion of the lower respiratory tract, below the larynx by pathogens either by inhalation, aspiration, respiratory epithelium invasion, or hematogenous spread. There are barriers to infection that include anatomical structures (nasal hairs, turbinates, epiglottis, cilia), and humoral and cellular immunity. Once these barriers are breached, infection, either by fomite/droplet spread (mostly viruses) or nasopharyngeal colonization (mostly bacterial), results in inflammation and injury or death of surrounding epithelium and alveoli. This is ultimately accompanied by a migration of inflammatory cells to the site of infection, causing an exudative process, which in turn impairs oxygenation. In the majority of cases, the microbe is not identified, and the most common cause is of viral aetiology.

There are four stages of lobar pneumonia. The first stage occurs within 24 hours and is characterized by alveolar oedema and vascular congestion. Both bacteria and neutrophils are present.

Red hepatization is the second stage, and it has the consistency of the liver. The stage is characterized by neutrophils, red blood cells, and desquamated epithelial cells. Fibrin deposits in the alveoli are common.

The third of the grey hepatization stage occurs 2-3 days later, and the lung appears dark brown. There is an accumulation of hemosiderin and haemolysis of red cells.

The fourth stage is the resolution stage, where the cellular infiltrates are resorbed, and the pulmonary architecture is restored. If the healing is not ideal, then it may lead to parapneumonic effusions and pleural adhesions.

In bronchopneumonia, there is often patch consolidation of one or more lobes. The neutrophilic infiltrate is chiefly around the centre of the bronchi.

The WHO reclassified pneumonia in children into two categories; pneumonia with fast breathing and/or chest in-drawing, which requires home therapy with oral amoxicillin, and severe pneumonia, which is pneumonia with any general danger sign (i.e. hypoxaemia), which requires referral and injectable therapy.

The presentation of children with pneumonia can be very varied and may include cough, fever, tachypnea, and difficulty breathing. Young children may even present with abdominal pain only.

Features from the history and what they might mean

Sign/HistoryImplicationComplication 
Prolonged duration of coughSecondary infection, abscess or empyema formation Longer admission, tertiary referral
ChokingAspiration of FB or foodBronchiole/lower airway obstruction, pneumonitis 
Birth complications- e.g. meconium or prematurityChronic lung disease for the newbornMore susceptible to infections/severe infections
ImmunisationIncomplete immunization/ no immunisationAt risk of acquiring bacterial infections, severe infections or viral complications from measles, chickenpox
Travel and exposureContact with unwell relative, contact with other childrenExposure to different pathogens with travel Contact with older/unwell children, or adults may be exposed to pathogens not yet immunized against, or atypical ones 

Mary is 3 years old and was referred to hospital from the GP with a 2 day history of coryzal symptoms, cough, fever and saturations of 91%. She is not eating but still drinking fluids well. On assessment in triage she is crying; her respiratory rate is 45, saturations are 96% and temperature is 37.8°.

The play therapist distracts her while you examine her chest on mum’s lap. You don’t see any use of accessory muscles or intercostal recessions at rest; you think you heard crackles but it could also be transmitted sounds.

What is the probability that Mary has pneumonia?

Should you do a chest x-ray?

Mary’s mother says the GP frightened her by referring her to hospital. She asks you whether Mary needs antibiotics. Should you prescribe antibiotics?

Mary is a well grown, fully immunised and a previously well child who now displays mild signs and symptoms of pneumonia. She does not need a CXR nor does she need antibiotics. The family requires reassurance that the child is safe, can be managed at home as well as be provided with illness specific information and when to return.

(https://gppaedstips.blogspot.com/2018/11/making-diagnosis-of-lower-respiratory.html)

Children with pneumonia may present with fever, tachypnoea, breathlessness or difficulty in breathing, cough, wheeze or chest pain. They may also present with abdominal pain and/or vomiting and may have headache. Cough and fever are non-specific symptoms and are not grounds for diagnosing LRTI on their own. 

Tachypnoea is also a non-specific sign in children. It may present in fever, when a child cries or is in pain and in many non-respiratory cases. 

Hearing crepitations on auscultation is also a common finding that should not be given too much weight. The infant or child with an upper respiratory tract infection (URTI) will often have crepitations that can be heard in one or more places in the chest.  These may be transmitted sounds or due to secretions. Often, these noises go away or move around if re-examined, especially after a cough. In the absence of abnormal breathing, these crackles are not good evidence for LRTI. Also, auscultation and percussion in infants and small children is difficult. Chests are small and there is always the possibility that the area of abnormality will be missed.

What clinical findings are of value in diagnosing pneumonia?

The Rational Clinical Examination Systematic Review concludes that more important than tachypnoea and auscultatory findings are

Hypoxia (saturations ≤ 96%) and 

Increased work of breathing/abnormal breathing

There are no absolute rules about when to x-ray but we shouldn’t rely on CXRs to make the decision for us. The sensitivity and specificity of a CXR as a way to diagnose pneumonia in children is too poor to justify using radiation when the diagnosis should be made clinically. The BTS guidelines for community acquired pneumonia in children and the Clinical Practice Guidelines by the Pediatric Infectious Diseases Society and the Infectious Diseases Society of America both recommend that CXR is routinely avoided.

Special circumstances where a CXR should be considered include:

Small infants and babies

This age group tend to have a higher probability of serious bacterial infection whenever they present.

The child with complex medical problems

They may not demonstrate abnormal breathing or unwellness in the way that normal children do.

Chronic symptoms in a child that does not appear unwell, red flags (such as weight loss), known exposure to tuberculosis

Daily cough for several weeks should be taken seriously. Underlying causes including bronchiectasis and simply unresolved LRTI may need to be ruled out in which case referral will be necessary. Unilateral findings to evaluate for a foreign body.

Chest radiography should also be done when a child fails to improve clinically after 48-72 hours of appropriate antibiotic therapy, in patients with severe or unexplained respiratory distress, and those who require hospitalisation. 

Severity assessment to direct treatment

A clinical examination cannot distinguish between a viral or bacterial pneumonia, neither can a CXR. More important than distinguishing whether a pneumonia is viral or bacterial is to adopt a severity-based approach to guide your treatment. Even if mild to moderate disease is caused by bacteria, these infections still resolve on their own and antibiotics make little to no difference anyway.There is no single validated severity scoring system to identify children at risk from a severe infection. A global assessment of clinical severity and risk factors is crucial in identifying the child likely to require hospital admission. One key indication for admission to hospital is hypoxaemia. British Thoracic Society Guidelines’ features of severe disease in an infant and older child include oxygen saturations < 92% together with other features of abnormal breathing listed below.

Bringing all these things together shows that there are two key features.  The first of these is abnormal breathing in the context of an unwell child with cough.  The presence of abnormal breathing almost immediately makes it likely that the problem is LRTI, bronchiolitis or viral wheeze.  If there is a wheeze, this largely rules out LRTI. It’s almost that simple.

Safety-netting advice is key.

For the majority of encounters, parents bring their child to medical attention because they are uncertain as to the severity of their child’s illness, and they are frightened. Not because they seek antibiotics. DFTB lists reassurance steps to take in your discussion:

  • Acknowledge their child feels poorly.
  • Acknowledge this is difficult for their child, and for them as parents.
  • Reassure them their child is safe, and there are no ‘red flags’ – remember what we consider severe (physiological derangement) is not the same as parents (behavioural impact).
  • Explain that medical treatment is supportive and offer symptom management.
  • If you need to, confirm antibiotics are neither necessary nor helpful, as it will not speed up recovery and only expose the child to unnecessary risk.
  • Most importantly – provide illness specific information and safety net advice (ideally written information/leaflet).

Life in the Fast Lane – Paediatric CXR (some of the CXR start with CT images)

Martin is an 8 year old fit and healthy young boy who was brought in by his dad with three days of fever, a dry cough, shortness of breath, and abdominal pain, initially seen by the GP and started on amoxicillin. Today he was sent home from school because of breathing difficulties.

On assessment Martin is lying in bed, alert with a tracheal tug, use of accessory muscles, a respiratory rate of 37 breaths per minute, and oxygen saturations of 89% in room air. You also note that Martin has a rash on his lower legs.

Why is Martin not improving on appropriate antibiotics?

How should Martin be investigated and managed?

Perhaps it’s a viral pneumonia

One could consider whether antibiotics were appropriate in the first place. Martin could be dealing with a viral infection, which could explain why there is no change in symptoms. Inappropriate antibiotic prescribing drives antibiotic resistance and drives future medicalised health behaviour.

Perhaps it’s the wrong antibiotics

NICE recommends amoxicillin as the first choice of oral antibiotic for a low severity pneumonia in children and adults less than 18 years of age and high dose oral amoxicillin (30mg/kg TDS) is as effective as IV benzylpenicillin. 

Is Martin allergic to penicillin? Perhaps the amoxicillin has caused the rash and worsening respiratory symptoms, so amoxicillin should be discontinued immediately and replaced with a macrolide. NICE recommends doxycycline or clarithromycin in penicillin allergy.

Perhaps it is the wrong diagnosis

Here we come to the crux of any child that fails to respond to initial treatment: always go back to the drawing board. Retake a detailed history and do a thorough examination. Draw out any red flags, allergies, previous medical history, a significant family history. On examination it is clear that Martin has a severe pneumonia – he is hypoxic with obvious work of breathing and will require oxygen therapy, further work up and admission.

What other differentials would one think about?

Pneumonia can occur at any age but tends to occur in younger children and become less common as they get older.

In neonates respiratory distress can be a sign of underlying pathology and such things as congenital abnormalities, laryngeal injury, pulmonary haemorrhage/birth trauma and these must be considered in the differential.

In older children respiratory distress can be present in asthma, bronchiolitis, chronic anaemia, cystic fibrosis, heart disease, haematological malignancies and even foreign body inhalation.

Also important to consider whether this is a complicated pneumonia (pneumothorax, effusion, empyema) or sepsis.

Some differentials are demonstrated below

Sign/HistoryDifferentialInvestigation
Sudden onset or precipitating trigger of dust/hay/animalAllergy or anaphylaxis
Acute exacerbation of asthma
Trigger/sudden onset more likely asthma/anaphylaxis than pneumonia
If anaphylaxis then IgE levelsPeak flow in Asthma pre and post bronchodilators, response and improvement- more likely asthma over pneumonia
Nocturnal cough or sx of cough and SOB when well (interval symptoms)Undiagnosed or under treated asthmaPeak flow
Fatigue, easy bruising, pallorAnaemia, leukemiaFull blood count with film – low Hb, high WBC or pancytopenia
Failure to thrive in neonate/infantCystic fibrosisSweat test and specialist referral
Feeding difficulties, cyanosis on feedingCongenital cardiac defectECG, CXR, echocardiogram and specialist referral
Hx of sickle cell diseaseAcute chest crisisSevere chest pain and bilateral CXR changes, pain in regions outside of chest, or previous presentations
History of choking, unilateral chest signsForeign body inhalationCXR, bronchoscopy and specialist referral
Previous streptococcal infection, fever, erythema marginatum, carditisRheumatic feverESR, WCC, blood culture, ECG, echocardiogram, antibiotics
Immunocompromised (primary immunodeficiency, HIV)Fungal pneumonia, tuberculosis (if exposure to known contact)Antifungals and anti-tuberculous therapy and specialist referral to Infectious Diseases.

Pneumonias have a variety of classifications, such as community acquired pneumonia (CAP), aspiration pneumonia, hospital acquired pneumonia, and pneumonia classified by age group or causative pathogen. Atypical pneumonia refers predominantly to an uncommon pathogen causing pneumonia. Below is a classification of pneumonia typical for certain age groups of children.

Respiratory tract problems, cough and fever, are the most common presentations to the Paediatric Emergency Department (PED). Most of these children do not have pneumonia, and most who do have pneumonia can be discharged from the PED with oral antibiotics and careful safety netting. 

Refer children under the age of 1 year, if they have comorbidities (i.e. immunodeficiency, cardiac disease), poor oral intake or urine output and most certainly if there is laboured breathing, hypoxaemia and signs of sepsis. RCEMLearning has a simplified (and useful) summary of how to differentiate the common respiratory problems in PED.

There is also fungal pneumonia which in addition to common bacterial and viral pathogens are considered uncommon and opportunistic microorganisms in a ‘poly-microbial mix’ seen mainly in immunocompromised children such as in HIV-exposed or infected children. Pneumocystis jiroveci (PJP) is a common fungal infection of the lung in immunocompromised infants from 2-6 months of age. They present with an acute onset of respiratory distress, minimal/absent chest signs in a child who is HIV exposed or infected. Hypoxaemia and cyanosis are common features in severe disease and CXR shows a range of abnormalities including bilateral perihilar interstitial changes.

Perinatally acquired cytomegalovirus associated pneumonia in HIV infected infants presents as an interstitial pneumonitis with acute hypoxic respiratory failure and tuberculosis in HIV infected children occurs at all ages. The diagnosis is difficult to confirm, one needs to have a high index of suspicion if exposure to a contact has been elicited from the history and a Mantoux test of ≥ 5mm induration is indicative of tuberculosis disease.

Those children with chronic lung diseases such as in immunocompromised children or whose with cystic fibrosis (CF) are typically colonised with uncommon organisms such as Pseudomonas aeroginosa and Klebsiella pneumoniae.

Mycoplasmas are distinguished from other bacteria by their lack of a cell wall, which has implications for its treatment – as most antibiotic classes, which act on the cell wall, will be ineffective in treating Mycoplasma species. While LRTI decreases with age, the prevalence of atypical infections increases, with a median age of about 7. They most commonly present with respiratory symptoms such as pneumonia, however they also have a range of extrapulmonary symptoms. CXR manifestations in this group are also wide and varied as are laboratory findings. Some CXR features can involve reticulonodular patterns confined to one lobe, segmental and lobar consolidations, or diffuse interstitial and bilateral perihilar peribronchial patterns. Below is an example of left lower lobe consolidation complicated by a pleural effusion in a patient with confirmed mycoplasma pneumonia.

Atypical pneumonias, such as those caused my mycoplasma, are generally treated with oral macrolides, fluoroquinolones or tetracycline. There is no need to target extrapulmonary symptoms such as in this case, as it is likely immune mediated but supportive therapy maybe considered. Skin manifestations are the most common of the extra-pulmonary manifestations and range from erythema nodusum (as depicted in the diagram) to Stevens-Johnson Syndrome. These are raised and tender nodules. Part of Martin’s management should include adequate analgesia not only for erythema nodosum but also for his referred abdominal pain.

When considering admission there is no one clinical factor for admission, it is based on a combination of clinical signs, but most importantly on severity of pneumonia. Compliance with medication and parental anxiety can be a valid reason.

Admission does not necessarily need to mean further investigation and can be trial of PO antibiotics in hospital, switching to IV/ambulatory IV if a trial of oral is not tolerated, and importantly supporting the parents.

EmDocs: Paediatric Pneumonia Management Algorithm

Individual risk factors for the child e.g prematurity, immunocompromise, congenital abnormalities or previous complications from CAP must also be considered.

Children who are ex-premature may have chronic lung disease of the newborn and are likely to be more susceptible to severe pneumonias and infections.

The same applies for children with congenital abnormalities and immunocompromised.

It can also be secondary to chemotherapy or as a result of HIV. 

Being immunocompromised may mean they are more likely to require IV antibiotics or a longer period of observation.

Martin has severe mycoplasma pneumonia and requires humidified high flow nasal cannula oxygen (HHNC) therapy to start. He also needs a CXR, so we can make sure we are not dealing with a complicated pneumonia. It’s probably advisable to get intravenous access in case of further deterioration and a set of baseline bloods (FBC, CEU) and a baseline blood gas to determine how well (or poorly) Martin is oxygenating (Pa02). Septic markers are controversial here as they would probably not change the initial management in the paediatric emergency department but seeing that Martin is unwell and needing admission, it would be reasonable in this situation to do a CRP and/or procalcitonin (PCT). If tolerating oral medication, he would continue on oral Azithromycin. Mycoplasma pneumonia’s are usually diagnosed retrospectively so depending on local guidelines a viral pharyngeal polymerase chain reaction (PCR) swab or sputum and/or antibody test to Mycoplasma pneumonia can be done. Martin is admitted to the Paediatric high care isolation ward and PICU is also made aware of Martin’s condition.

Mimi is well known to the department. She has Trisomy 21 and had her VSD repaired at 3 months of age. She is now 10 months old and is brought in with a 2 day history of coryzal symptoms, cough and fever. Today her parents have noticed fast breathing, she is much more lethargic and off food. She is normally a very bright bubbly child.

On examination Mimi is tiring, she is cyanosed with oxygen saturations of 82%.

Which patients are at increased risk of a severe pneumonia?

Should we CPAP ‘trial’ or immediately intubate?

As previously discussed children with other comorbidities or congenital abnormalities are at increased risk of lower respiratory tract infection and complications.

Those with underlying or previous cardiac abnormalities can deteriorate more rapidly with fewer precipitating symptoms.

Similarly, ex-premature infants are at increased risk of severe pneumonia’s (typically RSV pneumonia) and remember the child with complex medical problems may not demonstrate severe clinical signs as would a normal child. One should always have a low threshold for investigating further.

Recognising the child at risk and the deteriorating child early means appropriate early intervention and escalation of care, but sometimes there isn’t the time and a child may need an emergent intubation

It is important to recognise when a child is deteriorating by looking at response to treatments given, work of breathing, RR, SPo2 and general appearance.

In the hypoxic child the simple administration of oxygen may not always be sufficient

This is where continuous positive airway pressure (CPAP) which delivers constant positive end expiratory pressure (PEEP). Normally a mask or nasal prongs are sealed against the nostrils and are connected to a pressure generator and an airflow source. Options are where the mask is connected to a mechanical ventilator, which provides airflow and PEEP. Alternatively an oxygen concentrator or cylinder provides airflow, and the depth of expiratory tubing within a fluid reservoir generates PEEP and this is referred to as bubble CPAP (bCPAP).

There are several studies looking at CPAP particularly in low resource settings and if it reduces mortality in childhood pneumonia. The difficulty in low resource settings (or indeed a small DGH) is access to equipment and a balance of providing highly concentrated/pressurised O2 to a small number of children vs being able to provide low flow to several. Hopefully this is a highly unlikely scenario but was what was recognised in some of the studies conducted to very rural areas.

Generally the studies suggested that CPAP reduced respiratory distress and improved oxygenation, but rate of mortality was unchanged particularly with associated comorbidities.

https://onlinelibrary.wiley.com/doi/full/10.1111/apa.14796

CPAP is useful particularly for respiratory distress regardless of SPO2 and is often better tolerated than a face mask as the nasal prongs are less intrusive and the humidified oxygen less distressing. It can eliminate the need for intubation and along with distraction technique calm a child down. However some models you cannot transfer on easily and this need to be taken into consideration when setting it up (e.g if they are in ED and not a ward)

If a child does not respond to CPAP then the next definitive step is to perform an emergency intubation, or a rapid sequence induction. 

If the child is in respiratory failure then it may be that intubation is the first step.

CPAP is only indicated as a method of pre oxygenation if pre oxygenation is not possible via normal face-mask (but this will take time to set up and may delay intubation)

This podcast discusses some different situations and nuances around RSI

Any child who you are considering CPAP/RSI should have a PICU involvement as this is the area they will need to be transferred to after the interventions.

Ideally PICU should be present at the time of intubation or a paediatric anaesthetist as these will be the best placed clinician to intubate and with a child in respiratory distress the goal is to secure the airway and provide adequate oxygenation as quickly and safely as possible.

A 4 year old child, Hannah, was diagnosed with pneumonia and admitted to the children wards on oxygen and commenced on IV antibiotics. After 48hr of initial therapy her oxygen requirements have increased, and she is still spiking fevers.

You have been called to review Hannah as the nursing staff are concerned that she is febrile again despite paracetamol. Her initial CXR showed a dense left lower lobe consolidation.

Would you repeat a chest x-ray?

Or are their alternative investigations?

Hannah has developed an empyema. Discuss your approach to inserting a chest drain.

Point of care ultrasound is becoming an increasingly utilised tool for clinicians in the emergency field, by specialist and emergency physicians. Several studies have started looking to lung ultrasound for diagnosing pneumonia and this has been expanded into the paediatric cohort.

Several studies have now shown that lung ultrasound (LUS) is as sensitive in diagnosing pneumonia as CXR. However it is noted that this may be user and locality dependent, e.g. clinicians on shift being able to perform and interpret USS, or having access to this modality out of hours.

One meta analysis comparing LUS vs CXR showed that LUS had a sensitivity of 95.5% and specificity of 95.3% whereas CXR had a sensitivity of 86.8% and specificity of 98.2%. We know that CXR is currently the gold standard

Yet some studies have demonstrated LUS may pick up even smaller areas of consolidation that can be missed on CXR. Ultrasound is something that is being used more and more and can be readily taught to physicians to achieve basic competence. Utilising US provides rapid insight into the pathology of the lungs and can identify, monitor and assess changes at regular intervals without the need for repeated CXR. It may be easier to have access to an USS rather than a CXR especially in a critical emergency.

However if LUS is not immediately available then CXR should not be delayed if indicated.

If a child has not responded to antibiotics after 48hr then the clinician must think why and assess what has changed. The incidence of parapneumonic effusion and empyema in children is 3.3 per 100 000 children. If effusion is suspected on CXR then an US must be used to confirm the presence of fluid. All children with effusion/empyema must be admitted for IV antibiotics.

If confirmed on LUS then a CT scan with contrast enhancement can be used as a definitive investigation. Effusions that are enlarging or causing respiratory embarrassment should be considered for invasive intervention. Conservative management alone can be appropriate but can prolong the overall hospital admission. As per the British Thoratic Society (BTS) guidelines for the management of pleural infection in children a chest drain should be considered and placed by an appropriately trained member of staff and with the aid of LUS.

Repeated aspirations are not recommended as they are less efficacious, and more likely to cause distress and involve repeated invasion into the pleural cavity.

Whereas an appropriately placed drain (and not necessarily the biggest!) when inserted under appropriate procedural sedation (or GA) can shorten the illness and resolve the effusion faster. Different types of chest drain are available; one small study compared pigtail with large bore surgical drains and found no significant difference in outcome, but did find that the smaller pigtail drains were better tolerated. If a child has a complicating fibrinopurulent empyema then the drain can also be used to administer intrapleural fibrinolytics e.g. urokinase. This can also allow continued drainage with reduced risk of purulent blockage, and help re-establish normal pleural flow.

Read the DFTB rule of 4s

Then when to remove/ when to clamp?

Clamp the drain for 1 hour once 10 ml/kg are initially removed.

Remove the drain when they no longer swing/bubble and LUS shows resolution of effusion/empyema and importantly the child is clinically improving.

However if the drain stops swinging- check why, has the effusion been drained or has the tube become kinked or blocked, attempts at repositioning or flushing the drain should be undertaken and assessment of the clinical picture.

If the effusion has not drained or the child has not improved then it would be appropriate to refer to the paediatric surgeons for consideration of a replacement drain or potentially a VATS procedure if a particular viscous or loculated effusion remains.

Removal of drains should be based on resolution of effusions and clinical improvement. Antibiotics should be continued for 1-4 weeks after removal, all children should have routine follow up and underlying comorbidities should be considered e.g undiagnosed CF, immunocompromise, malignancy.

Even with effusion/empyema most children should recover without any long-term complications of adverse reduction in lung function.

Optimus Bonus Simulation Package – Paediatric sepsis

This simulation focuses on management of sepsis so would follow on from recognising complications or deteriorations in children with LRTI, recognising shock and when to escalate care.

A 5 year old is brought in with 3 day history of fever, lethargy and complaints of left sided abdominal pain. Normally fit and well, immunisations are up to date and they attend school.

In triage he is noted to have subcostal and intercostal recession, with SpO2 of 90% in air, the triage nurse moves him to a bay and asks for your urgent review.

What on examination/initial investigation would make the diagnosis of pneumonia more likely?

A: Fever and cough

B: Low sats and fever

C: Focal crackles on chest auscultation

D: Hypoxaemia and increased work of breathing

E: Coryzal and increased work of breathing

Answer D

Hypoxaemia and increased work of breathing were most clinically significant in diagnosis of pneumonia. Chest signs can be misleading and it is often difficult to tell upper airway noises from focal signs. Even viral pneumonias can lead to focal signs on auscultation and on chest x-ray. Upper airway noises can be distinguished as they tend to change on positioning/after coughing as upper airway secretions move and are expelled whereas focal signs will be less affected by this. A viral pneumonia may have a history of coryzal symptoms and would be similar to that of bronchiolitis.

On examination the child has consistently reduced air entry at the right, persistently low sats of 91%. CXR shows a right lower lobe pneumonia.

What is the most likely causative pathogen?

A: Streptococcus pneumoniae

B: Staphylococcus aureus

C: Haemophilus influenza (type B)

D: Mycoplasma pneumonia

E: Respiratory Syncytial Virus (RSV)

Answer A

The infective agents that commonly cause pneumonia will vary by age.

Pathogens will vary from neonates, to infants to preschool to school age children, think of the vaccination schedule, maternal swabs in pregnancy and maternal fever in labour and atypical pathogens in immunocompromised children.

Remember atypical e.g. mycoplasma’s become more common in the older child.

Haemophilus influenza B – rates overall are reduced due to vaccinations

You insert an IV cannula and take bloods. Results show a white cell count of 24.3 × 109/L (with neutrophils 92%), a CRP 283 mg/L and a sodium (Na) 126 mmol/L. The rest of his full blood count and renal function are normal.

Which of the following is the most likely cause for his hyponatraemia?

A: Low sodium intake

B: Increased renal excretion

C: Hyponatraemic dehydration

D: Increased sodium dilution

E: High sweat sodium concentrations

Answer D

Hyponatremia has frequently been ascribed to the syndrome of inappropriate antidiuretic hormone (SIADH) in the past, but the existence of this entity in children with pneumonia is now being questioned. SIADH leads to hyponatremia by increasing the total body water causing a dilutional effect.


Please download our Facilitator and Learner guides

A New Way To Teach

Cite this article as:
Team DFTB. A New Way To Teach, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.28420

At DFTB we are very excited to be able to present the DFTB Modules – a set of free, open access teaching modules which are mapped to the UK and Australasian Paediatric Emergency curriculum that you can pick up and run in your own organisation.

This is a project that has been developed by our DFTB Fellows at the Royal London Hospital – Rebecca Paxton, Helena Winstanley, Chris Odedun, and Michelle Alisio. The DFTB Modules would not have been possible without our wonderful community of writers and contributors from around the world who have spent time crafting and reviewing the modules over the past year.

We’ve prioritized flexibility in creating the modules with cases and discussions with both basic and advanced trainees in mind. This way you can adapt them to your learners and existing resources. The first 15 modules have been released and we have another 30 in the pipeline. These will be published over the next few months. We would love to get your feedback or comments at fellows@dontforgetthebubbles.com

Why did we create the project?

The DFTB mission is about taking a “World recognized leadership role in making meaning of information in paediatric medicine, for clinicians“. Our principles are structured around being collaborative, pioneering, community-focused, and evidence-based.

Opportunities for teaching and learning across the curriculum in paediatrics, particularly in paediatric emergency, are variable between hospitals often due to access to useful resources. Whilst there are many fantastic educators in hospitals, many fill clinical roles. This means that their time to prepare for teaching is limited. For trainees, who often rotate from hospital to hospital, having access to structured resources and an opportunity for case-based discussion of a wide range of topics will help strengthen their learning.

By collaborating, as a group of medical professionals across the world, in writing these modules – we are working together as an international community to support thoughtful, evidence-based sessions.

Access the DFTB Modules here

Safeguarding Module

Cite this article as:
Team DFTB. Safeguarding Module, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.27747
TopicSafeguarding
AuthorVictoria Currie
DurationUp to 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
  • Quiz (10 mins)
  • Infographic sharing (5 mins): 5 take-home learning points

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

The Child Protection Companion. Last published December 2017. Available on RCPCH website and Paediatric Care Online

RCPCH: Child Protection Evidence (evidence based resources for clinicians to help inform child protection procedures)

Child Protection Processes: PaediatricFOAMed

DFTB: Skeletal Survey in NAI

St Emlyns: Child Protection

https://www.aliem.com/pem-pearls-child-abuse-case-1/

Safeguarding children is everyone’s responsibility. Abuse of children can come in many forms; physical, sexual and emotional abuse, and neglect.

Children can present in various ways- this teaching tool aims to discuss the terminology surrounding safeguarding, the investigations that are required and processes that occur when a child presents with suspected maltreatment.

Cruelty to children and young people is a criminal offence, and child abuse and neglect can have serious adverse health and social consequences for children and young people.

These include:

Bruising is the most common injury sustained by children who have been physically abused. Paediatricians must have the skills to differentiate abusive bruises from those that arise from everyday activity or unintentional injury

  • Young children who are referred to the paediatric child protection team with suspected physical abuse (PA) frequently have bruises. It is unclear whether there is any difference between the pattern of bruises when PA is confirmed and when PA is excluded.
  • Bruising is the commonest injury seen in physical abuse.
  • The odds of a bruise on the buttocks or genitalia, cheeks, neck, trunk, head, front of thighs, upper arms were significantly greater in children with PA than in children with PA-excluded.
  • Petechiae, linear or bruises with distinct pattern, bruises in clusters, additional injuries or a child known to social services for previous child abuse concerns were significantly more likely in PA.

All professionals working with children have a duty to safeguard their wellbeing. So, if anybody identifies safeguarding concerns, they should raise it with their local Child Protection services.

6-month-old child (Lisa) on a child protection plan presents to ED with coryzal symptoms and fever of 37.8oC. On examination it is felt that Lisa has a viral illness however after exposing her she is  noted to have multiple bruises on their back of differing colors and sizes.

Lisa had been left with her grandmother and grandfather over the weekend as her mother had spent the weekend with her new partner.

What is your next course of action? 

Why are the bruises on this child’s back concerning?

What bruising patterns are more concerning in children presenting to the ED? 

What questions do you need to incorporate into a paediatric history when you are concerned about safeguarding issues? 

ABCDE assessment of Lisa

Two issues here are the viral illness but the concerning multiple bruises. Assessment and examination to determine the viral symptoms and if further medical treatment is needed. Assessment of pain and suitable analgesia if required.

Detailed history with specific questions relating to safeguarding issues. (see below)

On examination you notice that Lisa is mildly coryzal. You note that the clothes Lisa is wearing seem inadequate. It’s a cold day and Lisa  has arrived solely in a baby grow. There is no respiratory distress and the child is cardiovascularly stable. Lisa is alert and active with normal power and tone and a level anterior fontanelle. 

On exposing Lisa you notice multiple bruises. There are some bruises on her back, with further bruises behind Lisa’s ears (they are round and look like fingertip marks). These bruises concern you. You also notice the nappy is sodden and does not look like it has been changed in a while. The car seat Lisa has arrived in is really dirty with crumbs in it. 

You think about the toddler you have just seen prior to reviewing Lisa who was a 3 year old boy that had fallen over with a minor head injury but you had noted multiple bruises on his shin and you now question if you should have been worried by these bruises. 

Children, especially toddlers can often have accidents in the home or at school that can result in bruising. In the ED there are often bruises noted that are not worrying to us as clinicians based on the site of bruise and the child’s developmental age. Bruising in children is common and often not a cause for concern. A polite inquisitive style including asking the child how they attained the injury can often give a plausible and valid reason for the injury. 

Bruises are unusual in babies 6 months or less who are unable to move or crawl. When children become more mobile bruising becomes more common. These bruises are usually <1 cm in diameter, often over the forehead, bony part of the cheek or jaw, or shins. An active baby in the first 18 months might have two or perhaps three of this type of bruise at the same time. 

In older children most accidental bruises are on bony prominences and are often associated with a graze. In children 18m to 3 years facial and forehead bruises are common, however in older children this is less common. In older children bruises of the hands, feet, lower legs- in particular shins are common. Lower back bruises can be seen on  older children but should be a cause for concern in children under the age of three. 

Non-accidental bruises are more likely to be around the mouth and adjacent cheek, neck, eye-socket, ear, chest, abdomen, upper arms, buttocks and upper legs. All these areas are relatively protected.

Concerning bruising patterns (According to NICE guideline 89 Child maltreatment: when to expect child maltreatment <18s)

Suspect child maltreatment if a child or young person has bruising in the shape of a hand, ligature, stick, teeth mark, grip or implement. 

Suspect child maltreatment if there is bruising or petechiae (tiny red or purple spots) that are not caused by a medical condition (for example, a causative coagulation disorder) and if the explanation for the bruising is unsuitable.

 Examples include:  

  • bruising in a child who is not independently mobile  
  • multiple bruises or bruises in clusters  
  • bruises of a similar shape and size  
  • bruises on any non-bony part of the body or face including the eyes, ears and buttocks and back 
  • bruises on the neck that look like attempted strangulation 
  •  bruises on the ankles and wrists that look like ligature marks or holding/restraint marks

Now you have examined Lisa and are happy that the fever is only being caused by a coryzal illness. You prescribe some paracetamol and go on to take a more extensive history from her mother.

Do you need to extend the history- is there any explanation parents can give for the bruising? If there is a mechanism given does it fit the child’s developmental age? Do you need to take a developmental history?

If there is a period of time as in this case when Lisa was left with grandparents, do you need to extend the history to asking other family members about the injury- including siblings who may be too scared of the consequences if they are to admit there was an accident with their brother/ sister? Can this be done over the phone or could you speak to them in person? 

In ALL children presenting to the ED (irrespective of their presenting complaint) it should be commonplace to ask about:

  • The family set up
  • What adults do the children spend time with
  • Who lives in the same household as the child? 
  • Who has parental responsibility? 
  • Do the family have a social worker?
  • Or have they been previously known to social care? 

Important points to be elicited in the context of physical abuse include:

What the injuries are and how they presented
Timing of injuries and preceding events
The explanations given for the injuries and who gave them
Any discrepancy evident in the account
Action taken by parents or carers after the injury was discovered
Previous injuries
Explanation consistent or not with the developmental level of the child.

 Lisa’s mother had noticed some bruising after picking up Lisa from her grandparents yesterday. She was worried about it but did not come immediately to hospital. Lisa’s mother is unsure if her parents may have done this to Lisa. She wants Lisa to be OK but is worried that Lisa will be taken from her. 

Mo is a 3-month-old boy. He has presented to the ED due to family concerns that he is not moving his left leg. Parents are concerned that it looks a bit swollen. 

Mo is normally fit and well. He was born at term by NVD. He lives with his Mother, Father and extended family.

On examination: Mo has normal observations. He has a normal respiratory, cardiovascular, abdominal and neurological examination. On further examination you notice that Mo is reluctant to move his left leg – there looks to be some swelling over the femur. He cries when you examine it. 

You ask more questions- establishing that Mo’s Dad has been away for the last few days at work and Mo has predominantly been with his Mum. Mo has a social worker who was allocated as Mum had disclosed depression and had not wanted to continue with the pregnancy but due to pressure from the extended family had continued with the pregnancy. 

Mo is not yet mobile or rolling. There is no history to suggest how this might have happened. You can’t find any other evidence of injury on examination. You do notice on examination that Mo’s pram has old food in it, his clothes appear dirty. When you are examining him you notice that his nappy is very full and he has some evidence of nappy rash. 

What can some of the more subtle signs be that can alert you to child protection issues?

How do you move forward now? 

You wonder if Mo’s Mum and Dad will agree to all of this and what will happen if they don’t? 

It is important especially when working in a fast paced ED to recognise the more subtle signs of neglect might highlight a cause for concern and a discussion with a Senior colleague. 

Some of these signs may be

  • Child looking unkempt- soiled clothes, pram, dirty fingernails
  • Large full wet nappy (that looks like its been on for a while) the child may have nappy rash- that might suggest nappy has been on for long periods of time
  • Child who is mobile who has been brought out without shoes- an adult wouldn’t come out without shoes on- so why should we expect a child who is walking to do the same?
  • Child not dressed appropriately e.g. in cold weather no coat
  • Poor dental hygiene or dental caries- that would suggest lack of teeth brushing

These signs alone may be the product of a stressed parent who is worried about their child and quickly wants to get to the Emergency Department (a parent rushing out without a coat, or before changing a child who has just spilled food all over themself). However a few of these signs along with a parents behaviour, an unusual injury or a general feeling about the family- may be signs of neglect or even physical abuse. It is important to discuss this with a Senior colleague. 

These signs should be documented in the notes and even if they are the only concerns you have a discussion with social care or with a Health visitor (after discussion with a Senior colleague) can be a good way of sharing information and highlighting the more subtle signs. 

Always discuss the more subtle signs of neglect with a senior colleague.

You decide to give Mo some analgesia and request some X-rays.  X-ray of the left femur shows a mid shaft spiral fracture of the left femur.

The first step is to make sure that Mo has been given adequate analgesia. If required discuss with Trauma and Orthopaedics regarding management of the fracture. 

This is a concerning injury- Mo is non mobile and no history for the injury has been given. 

In house actions

  • Discuss with a Senior colleague in your department – make sure that your Registrar/ Consultant has been informed and knows there are safeguarding concerns
  • Discuss with your local in house safeguarding team (usually present during working hours). There should be a local safeguarding team available usually through your hospital switchboard or intranet. This team should be able to give you advice and tell you the local processes in your hospital/ local area. 
  • Have a read of the local guidelines for the hospital you are working in this may give you an idea of for example who this child should be admitted under if there are acute concerns in ED and the child is not able to be discharged. 

You speak to your consultant in ED who points you in the direction of the hospital intranet page for safeguarding. You speak to the Lead Safeguarding nurse, Brian. He tells you that you need to discuss the matter with the Child Protection Services  . Brian asks you to discuss the matter with both the General Paediatric team and the Trauma and Orthopaedic team once the initial process has been started by the local safeguarding team. Mo needs admission for management of the fracture along with a child protection examination and further investigations.

  • Some children who already have a known Social worker – the social worker can be contacted directly (usually if they present in hours). If they are not available or it is out of hours you may need to speak to the duty or on call Child Protection Services social worker.
  • How you refer to your local Children’s Protection services differs internationally and from region to region. Please ensure you are familiar with the local policy in your area.
  • All services will have a 24 hour accessible referral system, usually by phone in the first instance and then often followed by a written referral by secure email or via on-line web-portal

You wonder – what information should you be expected to provide when you make the referral to the Local Children’s Protection services. ED is really busy- there are lots of patients waiting to be seen- can somebody else complete this referral? 

It’s much easier if you know this before making the call!

  • Name, date of birth and address of the child, parents , siblings and any other household members
  • If the family lives between different households- e.g parents are separated- addresses of all places the child spends time
  • School/ Nursery/ GP name and address
  • Concerns that have lead you to refer the child on this occasion 
  • Have there been any previous concerns that you know about? Previously known to Social Care? Name and number of social worker/ family support worker?
  • Where the child is now and how can they contact you- This is really important if you are going off shift/ the child is moving to a different place from the ED for admission. 
  • They may also like to know if there are any other children in the household who may at present be at risk. 

It is everyone’s responsibility to safeguard children. However the form can be completed by medical or nursing staff. Some trusts will insist that before a child is admitted to a ward this form should be completed ( you know the story so it may be that you are the best person to do this). 

It needs to be clearly handed over to staff if the referral has not been done and why- along with any communication that has already taken place with Social Care. 

After you have made the referral to Social Care they are able to tell you that Mo’s known social worker is actually on duty. Mo’s mother has been very low in mood and the social worker had been having regular contact as they had been concerned she was not coping. The social worker and a member of the police are en route to the hospital to talk to Mo’s parents. There are no other children at home. You inform then Mo is being admitted to the T and O ward- under joint care with General Paediatrics team- who are preparing to perform a full child protection medical examination and further investigations. 

The legislation on holding a child against their parent’s wishes differs internationally.

In most countries the police force are the appropriate first responders to contact when you are concerned that a child may be at risk of harm if they are removed from a place of safety (e.g. hospital). 

In general police powers to hold a child in a place of safety do not override the parent or guardian’s rights to consent (or to refuse consent) to medical investigation/treatment and in most countries a court order is required to override the parent/guardian’s wishes.

You have just seen Eric, a 7 year old boy who, with his siblings, have an allocated social worker. He presented with a two day history of fever and not drinking. On examination you believe he has findings consistent with bacterial tonsillitis. You want to discharge him on oral antibiotics. During your clerking Mum mentions that they have a Social Worker who mum gives you the name and number of. 

Mum has attended during schooltime with all of the children – you notice three of them should be in school. You need to inform the Social Worker about the attendance to ED.

How does a child come to be placed on a ‘Child in need’ or ‘Child Protection plan’?  

Child Protection Processes: The Lowdown (PaediatricFOAMed)

 Each time a referral is made to the Local Child Safeguarding team the team receiving the referral will decide on one of 4 potential outcomes:

1. No further action is required

2. The case is suitable for Early Help (see Chapter 1 in ‘Working Together to Safeguard Children’)

3. An assessment of the family is carried out leading to the child becoming a Child in Need (CIN) under Section 17 of the Children Act 1989

4. The child has sustained or is at risk of significant harm and Child Protection proceedings must be started under Section 47 of the Children Act 1989

Once the referral has been made you should chase the outcome and if you don’t agree challenge it.

Section 47: Local authority should coordinate an investigation where a child has been subject to or at risk of harm. The aim of the meeting is to decide if any action is required to safeguard the child.

If the  threshold has been met for a Section 47 meeting- then a ‘strategy’ meeting should be arranged.  Meeting between social care, police and medical team. A decision will be made if it should be a ‘single’ or ‘joint agency’ between social care and police. 

The role of the Doctor in the strategy meeting is to consider the need for and timing of a medical examination.

The Aim of the Child Protection Plan is to- 

  • Ensure the child is safe and prevent them from suffering further harm
  • Promote the child’s health, welfare and development (this is where we can contribute most to the discussion!)
  • Support the family to protect and promote the child’s welfare, provided this is in the child’s best interests.

The document should specify timescales and allocate professionals to lead on each point of the plan. Crucially, a review conference should be held at regular intervals – first review is at 3 months then at 6 monthly intervals. If all the points of the CP Plan have been achieved, and the child is no longer considered to be at risk of harm, the CP plan can be discontinued. However, if not, or if the child has been on a CP plan for approaching 2 years, a legal planning meeting is held to decide if care proceedings should be started. This may result in the child being taken into care, becoming ‘Looked After’.

You want to inform Social Care about the fact that Eric attended the ED and that his siblings were not in school- it is nearly midnight and you wonder how you can do this – as you are due to be on two weeks of annual leave after today? 

Children attend emergency departments at all times of the day. Often they may be a child who is known to Social care who you need to inform of their attendance but there is nothing that is acutely concerning about the presentation. Some hospitals will have automatic alerts that come up when you see a child who is on a child protection plan. Often the alert says ‘ please inform the social worker of every attendance’. This can be hard when you are seeing the child out of hours. 

Check with your hospital what the system is- sometimes it is enough to document in the notes and there may be a fallback mechanism for a team to contact Social care within working hours. 

There is always an option of calling the out of hours Local Children’s Protections Services to inform them of the attendance and any further information. You are unlikely to get through to the child’s own social worker however you can leave information in a secure way.

You leave a message with the Local Authority Children’s safeguarding team. Who are able to look at the case noted and inform you that the family’s social worker is due to go and visit the following day- so they will leave a note for her of the information you have given. 

Liah is an 8 year old girl who you had seen on your previous shift in ED- she had presented with multiple bruises. You were concerned at the time that she had ITP. You had seen her and sent bloods off before you left – however you handed her over to a colleague as her bloods were not back when you left. You find out when you are back on shift that her results were normal. Liah was admitted under the General Paediatrics team. She is undergoing investigations for suspected non-accidental injury.

What are the investigations that should be performed in a child with suspected non-accidental injury? 

What investigations need to be performed when concerned about child protection? (The Child Protection Manual: RCPCH)

  • Full blood count
  • Coagulation studies (basic and extended)
  • Liver function tests
  • Amylase
  • Bone chemistry and vitamin D/parathyroid hormone
  • Urine and blood toxicology (if appropriate depending on history)
  • Skeletal survey with follow up films
  • Bone scan (done in certain situations)
  • Computed tomography (CT) head scan
  • Magnetic resonance imaging (MRI) brain and spinal cord
  • Ophthalmology examination

You hear from the Medical Team that Liah’s parents initially refused these investigations along with an examination of Liah specifically to look for injuries (Child Protection Medical examination). 

  • Do not be judgemental. You don’t know what happened
  • Speak to the parents in a neutral tone, calmly and kindly
  • Use open body language
  • Explain that you would like to go through the history with them again even though you know they have already been through it with a number of doctors
  • Explain to the parents that the child is the most important thing for you, your role is to find out what has happened and so you are obliged to refer to the Local Children’s Protection Services
  • Many parents will become upset and angry. That’s why it’s important to have another health professional with you. Many, on the other hand, will surprise you if you explain the situation well, by behaving very reasonably
  • Call security if you feel the situation may escalate or if you feel that you and other health professional staff are at risk of harm

If the situation becomes too confrontational and the parents insist on taking the child out of the hospital, you cannot restrain them. Advise them that you will be calling the police

Communication is key in cases of suspected non accidental injury. Open and honest conversation with the family about the need for investigations to check for any underlying conditions that may have caused the bruising. Early open and honest conversation with the family regarding the need for involvement with the Local Children’s Protection Services care social team.

Consent must be gained from parents before investigation or examination.

 You can get consent or authorization from:

  •  a child or young person who has the maturity and understanding to make the decision,
  • a person with parental responsibility if the child or young person does not have the capacity to give consent (it is usually enough to have consent from one person with parental responsibility)
  • the courts – for example, the family courts or the High Court.

When consent is not given

If the child refuses- explore their ideas, concerns and expectations. If they understand and are competent then their decision must be respected- even if it means that forensic evidence is inadequate.

Sometimes a child or young person may refuse consent because they are afraid of the person who is abusing them, or because they are under pressure to refuse. If you suspect this, you should consider the risk of harm to the child or young person and discuss your concerns with your named or designated professional or lead clinician or, if they are not available, an experienced colleague.

If a child or young person refuses, or their parents refuse, to give their consent to a child protection examination that you believe is necessary, and you believe that the child or young person is at immediate risk of harm, you should contact the police and Local Children’s Protection Services, which may take emergency action to protect them.

The medical team informed you that they did eventually get consent for the investigations along with the Child Protection Medical Examination.

(Paediatric FOAM Child protection documentation – where do we start?)

This an extensive history and examination that focuses on history from both the child and parents.  

Try not to use medical jargon- remember there are going to be non-medical professionals reading the report.

 The documentation is usually made up of- 

1.     Medical proforma- most trusts will have their own version. It can act as a prompt to remind you of what questions to ask. Remember to use the child’s own words as much as possible. Consent must be gained, and ideally written consent is best

2.     Growth chart- good practice to document the height and weight especially if there are child protection concerns. For example, neglect may present as a child that is failing to thrive. (available on RCPCH website https://www.rcpch.ac.uk/resources/growth-charts )

3.     Medical Photography- this is an extremely useful resource. Consent again must be gained. This is useful for example in a child with bruising which may change over time. Generally, this requires written consent and needs to be done via the hospital’s medical illustration department. This isn’t always available out of hours- in some hospitals A and E may have a camera that can be used for this purpose (not appropriate to use a phone camera!).

4.     Body mapping- Essential way of documenting examination findings. Dr Gayle Hann (Consultant Paediatrician, North Middlesex Hospital) and Dr Caroline Fertleman (Consultant Paediatrician, Whittington Hospital) have recently published new, more detailed body maps in different age groups to help paediatrician’s make better documentation. These can be found here:

https://www.londonpaediatrics.co.uk/resources

Body mapping can be overwhelming especially if there are lots of areas to draw. Have a colleague with you- draw it as you examine the child. It is useful to examine with a tape measure handy so you can measure the areas. Draw the injury as close to what you can see as possible use terminology like (graze, cut, scar, linear, colour). It makes it easier to number the marks- so it is easier to describe them in your written report- which always must accompany any body map.

Liah disclosed during the child protection examination that she had for the last few months been hit by her older brother. Social care are now involved and with support and her brother no longer being allowed to visit Liah was eventually discharged home with her mother and father. 

 Blood tests have been performed for Zain a 4 month old child who you have seen in ED  He has unexplained bruising- your consultant asks you to request further investigations before the child goes to the ward- what are they?

A: No further investigations required if bloods are normal

B: Ophthalmology review, x-ray or areas that are bruised, CT head

C: Skeletal survey, ophthalmology review, CT/MRI head

The correct answer is C.

What does a ‘Section 47’ mean?

A: This refers to children who have a Police protection order in place- police have the right to remove them to a place of safety for 72 hours- parents still have consent

B: Local authority should coordinate an investigation where a child has been subject to or at risk of harm. The aim of the meeting is to decide if any action is required to safeguard the child.

C: Child is under a child protection plan and should therefore be raised to the Local Authority Children’s care social team

The correct answer is B.

You are asked by your consultant to organize some photographs of a child who has presented with bruising- it is a Sunday and medical illustration is not open- what should you do?

A: With consent from parent use the consultant’s phone

B: Get the parent to take photos on their phone and then get them to send them to your secure work account

C: Try to get the designated camera from another area in the hospital (A&E) if this is not available then do not take the photos and organize for them to be done as soon as medical illustration is available.

The correct answer is C.

NICE Guideline: NG 76 Child Abuse and Neglect Published October 2017.

NICE Guideline: NG 89 Child maltreatment: when to suspect maltreatment in under 18’s. Published 22nd July 2009. Last updated 09th October 2017.

https://www.nice.org.uk/guidance/cg89

https://www.paediatricfoam.com/2018/02/child-protection-documentation-where-do-we-even-start/

https://www.paediatricfoam.com/2018/10/child-protection/

Kemp AM, Maguire SA, Nuttall D, et alBruising in children who are assessed for suspected physical abuseArchives of Disease in Childhood 2014;99:108-113.

Maguire S. Which injuries may indicate child abuse? Archives of disease in childhood – Education & practice edition, 6 December 2010, Vol.95(6), p.170

https://www.gmc-uk.org/ethical-guidance/ethical-guidance-for-doctors/protecting-children-and-young-people/child-protection-examinations

https://pcouk.org/chapter.aspx?sectionid=112958400&bookid=1674

The Child Protection Companion. Last published December 2017. Available on RCPCH website and Paediatric Care Online.

https://www.rcpch.ac.uk/resources/growth-charts

https://www.londonpaediatrics.co.uk/resources



Please download our Facilitator and Learner guides

Common Rashes Module

Cite this article as:
Aoife Fox. Common Rashes Module, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.27731
TopicCommon rashes
AuthorAoife Fox
DurationUp to 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
  • Game
    Quiz (10 mins)
  • Infographic sharing (5 mins): 5 take home learning points

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

Definitions/rash description:

  • Macule: a flat area of colour change <1 cm in size (e.g., viral exanthem [such as measles and rubella], morbilliform drug eruption).
  • Patch: a large macule >1 cm in size (e.g., viral exanthem [such as measles and rubella], morbilliform drug eruption).
  • Papule: a raised area <1 cm in size (e.g., wart).
  • Nodule: a larger papule, >1 cm in size (e.g. nodular prurigo). 
  • Plaque: a flat-topped raised area (a cross between a nodule and a patch; e.g., psoriasis).
  • Vesicle: a small fluid-filled lesion (blister) <0.5 cm in size (e.g. varicella, eczema herpeticum).
  • Bulla: a larger vesicle >0.5 cm (e.g. bullous impetigo).
  • Pustule: a pus-filled lesion (e.g. folliculitis).
  • Wheal: a transient raised papule or plaque caused by dermal oedema (e.g. urticaria)
  • Scale: flakes of stratum corneum (e.g. eczema, psoriasis).
  • Crust: dried serum, blood, or purulent exudate on the skin surface (e.g. impetigo).
  • Erosion: loss of epidermis, heals without scarring (e.g. Stevens-Johnson syndrome, toxic epidermal necrolysis).
  • Ulcer: loss of epidermis and dermis, heals with scarring (e.g. venous ulcer, pyoderma gangrenosum).
  • Excoriation: loss of epidermis following trauma such as scratching (e.g. pruritus).
  • Fissure: a split in the skin (e.g. angular cheilitis, palmoplantar keratoderma).
  • Lichenification: thickening of the skin with accentuation of skin markings (e.g. chronic eczema, lichen simplex chronicus).
  • Purpura: an area of colour change (red or purple) due to bleeding into the skin; does not blanch on pressure (e.g. vasculitis, disseminated intravascular coagulation).
  • Petechia: a pin-point purpuric lesion (e.g.,vasculitis, disseminated intravascular coagulation).
  • Ecchymosis: a larger area of purpura (e.g. vasculitis, disseminated intravascular coagulation).
Some important points to note in history:
  • Where did the rash start?
  • Sequence of the rash?
  • Type of rash?
  • Time of onset and duration?
  • Involvement of palms and soles?
  • Involvement of mucous membranes?
  • Involvement of conjunctiva?
  • Desquamating?
  • Systemic involvement?
  • Associated symptoms – fever/cough/conjunctivitis/runny nose/sore throat/strawberry tongue/itchiness/pain/weakness/headache/lymphadenopathy/swollen extremities/nausea/vomiting/diarrhoea? 
  • Exposures – immunizations/pets/foreign travel/bites (insects/ticks)/recent injury to skin/sexual history/sick contacts?

Common childhood rashes

The terminology for all but fifth disease is not used anymore, however, should anyone be curious here they are:

Also known as..What causes it?When?What rash?Where is the rash?FeverAssociated findings
First diseaseMeaslesParamyxovirusWinter – spring Erythematous, confluent, maculopapularBegins at the hairline spreads inferiorlyHigh feverKoplik spots, cough, coryza and conjunctivitis, Forchheimer spots
Second diseaseScarlet feverStreptococcus pyogenesAutumn – spring Generalised erythema with a sandpaper textureBegins on the face and upper part of trunk and spreads inferiorlyHigh feverPastia lines, Forchheimer spots, strawberry tongue, exudative pharyngitis, abdominal pain, rheumatic fever, circumoral pallor
Third diseaseRubellaRubivirusLate winter – spring Rose-pink, maculopapularSpreads inferiorlySlightly high feverLymphadenopathy, arthralgias, Forchheimer spots
Fourth diseaseThe existence of “fourth disease” is controversial. It was described as a generalised maculopapular rash and desquamation. This exanthema may be staphylococcal scalded skin syndrome
Fifth diseaseSlapped cheekParvovirus B19Winter and spring“Slapped cheek” appearance, lacy reticular rashErythematous cheeks, reticular extremities Slightly high feverRash, waxes and wanes over weeks, arthritis, aplastic crisis
Sixth diseaseRoseola Human herpesvirus 6 and 7SpringRose-pink, maculopapularNeck and trunk High feverLymphadenopathy, febrile seizures, Nagayama spots
Others of note..ChickenpoxHerpes zoster virusLater winter and early springVesicles on erythematous base, crustsBegins on face and trunk and spreads centripetallyHigh feverPruritus
Hand-foot-and-mouthCoxsackie A virusLate summer or early winterElliptical vesicles on an erythematous base, oral vesicle, erosionsMouth, hands and feetHigh feverVesicles on the hands, feet and in the mouth

Forchheimer spots: rose coloured spots on the soft palate that may coalesce into a red blush and extend over the fauces

Koplik spots: clustered white lesions on the buccal mucosa. They are pathognomonic for measles.

Pastia lines: where pink or red lines formed of confluent petechiae are found in the skin creases.

Nagayama spots: erythematous papules on the mucosa of the soft palate and the base of the uvula. You may see these present on the fourth day in two thirds of patients with roseola.

(based on case from RCEM Learning RCEM Learning – Common Childhood Exanthems)

Mark is a 3-year-old boy brought to the ED by his mother with a rash, temperatures and decreased oral intake. His older brother has a similar rash and illness and mum reports that there was an outbreak of chickenpox in the older brother’s school. 

On exam you note a quiet child with a diffuse vesicular rash. On palpation he has generalised lymphadenopathy.

What are the differentials of chicken pox in this case?

What is the incubation period of chicken pox? How long will Mark be infective?

What investigations are necessary?

How would you manage this illness? What treatment would you give?

What patients would you give anti-VZV immunoglobulin to?

Mark’s mum tell you that she has a 2-week old baby at home – what will you do?

What complications of chickenpox can occur?

Mum tells you that the children’s childminder is pregnant what advice do you give?

Discussion point – Do you use NSAIDs?

(Diffuse) disseminated gonococcaemia

(Local) hand, foot and mouth (coxsackievirus)

(Local) herpes zoster

Staphylococcal bacteraemia

DFTB – Exclusion period for infections

The incubation period for chicken pox is usually 10-21 days. The infectivity period starts when symptoms first appear and lasts until all the lesions have crusted over. This is usually around 5-6 days after the onset of the illness, with most crusts disappearing by 20 days.

Chicken pox is a very common illness and the vast majority of cases can be managed symptomatically at home. Prolonged fever >4 days should prompt the suspicion of complications of varicella such as secondary bacterial sepsis. Under these circumstances, patients should be examined carefully with appropriate blood test work up and a chest x-ray, depending on their clinical presentation.

Oral acyclovir has been shown to reduce the effects of chicken pox, for example the number of lesions and duration of fever, if used within 24 hours of the onset of rash in immunocompetent children. Oral acyclovir has not been shown to reduce the incidence of varicella zoster virus pneumonia or other complications when compared to placebo. Cochrane results do not support the widespread use of acyclovir in immunocompetent children.

Pediatric EM Morsels – Chicken Pox

It is used for high risk patients including, immunocompromised children, newborns with maternal Varicella that develops 5 days before to 2 days after delivery, premature babies and hospitalized infants.

Paediatrics Open – Management of varicella in neonates and infants

Asymptomatic newborn in contact with VZV from any infected subject

The mother is proved seropositive:
Very low risk of disease in the baby.
No treatment should be provided.

Observance of the baby at home and encourage parents to come back if any clinical sign or symptom appears in the 2 weeks after contact.

The mother is proved seronegative or refuses testing:
Treat the baby with acyclovir PO 80 mg/kg/day divided into four doses to start 7 days after infective contact and administer during 7 days.

Careful surveillance of the baby during the risk period. Indication and duration of hospitalisation (with airborne and contact precautions) should be discussed in each case depending on child clinical status, parental compliance and social setting. If any doubt, hospitalisation with optimal medical surveillance are warranted during the risk period.

Pneumonia

Bacteraemia

Encephalitis

Bacterial superinfection of skin

Problems may arise where there is a failure to recognise the complications of secondary streptococcal or staphylococcal infections or to appropriately manage high-risk groups. A prolonged fever for more than 4 days in a child with chicken pox, for example, should prompt the suspicion of secondary bacterial complications.

A range of complications including pneumonia, bacteraemia and encephalitis are increasingly being recognised. Neurological complications may occur without a preceding rash.

The incidence of congenital varicella syndrome is low if maternal infection occurs before 20 weeks of gestation. Congenital varicella syndrome is associated with shortened limbs, skin scarring, cataracts and growth retardation.

RCOG – Chickenpox in Pregnancy

The childminder should contact her own GP. She should avoid contact with children until establishing her risk of contracting VZV. 

DFTB – Varicella and NSAIDs

There is a long history of anecdotal evidence associating invasive group A Strep (GAS) complications, or severe skin and soft tissue infections (SSTIs) with exposure to NSAIDs. 

There are currently 5 papers, ranging from 1997 to 2008 which try to answer this question. Almost all the studies used a case-control method to try and answer this question. Where they took a group of children who had varicella and the outcome of interest (invasive GAS infection, severe SSTI), and compared them to a group of children who had varicella and did not get these outcomes, seeing which groups were more likely to have had ibuprofen.

The studies are pretty heterogeneous, so unsurprisingly the results varied. These studies all found an association, but they generally all suffer from the same big problem, which is confounding by indication. It might not be that ibuprofen causes complications, but rather bad varicella needs ibuprofen, and is also more likely to get complications anyway. As the famous saying goes, “Correlation does not equal causation”. The absolute risk increase of GAS complications or SSTIs is 0.00016% in the worst case scenario. 

NICE advises against giving ibuprofen due to the uncertainty but you must risk assess the clinical scenario yourself. 

(Based on a case from the American Academy of dermatology – American Academy of Dermatology – Viral exanthems)

Caleb is a 9-year-old boy who presents for evaluation of fever and rash. His mother noted a fever of 40 °C two days ago. He appeared well and was eating and playing normally, so his mother was not alarmed. After the fever resolved, Caleb developed red rash that progressed rapidly over the past 24 hours.

What is the most likely diagnosis?

What are the differentials?

What is the cause of roseola?

Who gets it?

How is it spread?

What are the signs and symptoms of roseola?

How is it diagnosed?

What is the treatment?

What are the complications from roseola?

Roseola

Measles

Rubella

Erythema infectiosum (fifth disease)

It is caused most commonly by human herpesvirus 6 (HHV-6) and less commonly by human herpesvirus 7 (HHV-7).

Children aged 6 months – 4 years are most typically affected. Most children (86%) will have had roseola by the age of 1 year. It is rarely seen in adults and infection is thought to confer lifelong immunity.

It is spread person-to-person via the saliva of asymptomatic family members. The incubation period is 9-10 days.

It results in an acute febrile illness lasting between 3 and 7 days, which is then followed by the characteristic rash of roseola in around 20% of infected children. 

The prodrome to the rash is a high fever (39-40 °C), palpebral oedema, cervical lymphadenopathy and mild upper respiratory symptoms. The child appears well. As the fever subsites the exanthem appear. This consists of a pink macules and papules surrounded by white halos. It begins on the trunk and spreads to the neck and proximal extremities. Nagayama spots may occur on the soft palate and uvula. The rash is non-itchy, painless and does not blister.

Diagnosis is usually based solely on the characteristic history and physical exam. 

Roseola is usually benign and self-limiting. Rest, maintaining fluid intake and paracetamol for fever is all that is usually required. Treatment may be necessary for atypical cases with complications and immunosuppressed patients. 

Complications are rare in most children.  The most common complication is febrile convulsion that occurs in 5-15% of children.

Acute encephalitis, hepatitis, myocarditis, haemophagocytic syndrome and infectious mononucleosis-like illness occur very rarely. 

Reactivation of HHV-6 with drug exposure can lead to drug-induced hypersensitivity syndrome, which results in fever, rash, pneumonia, hepatitis, bone marrow suppression and encephalitis. 

Simple Febrile Convulsions generally occur in children aged from 6 months to 5 years. They are common affecting 1 in every 20 children. The most common causes are viral URTIs, ear infections, bacterial tonsillitis and UTIs. 

They are generalised seizures, which last less than 15 minutes and occur only once during 24 hours. 

The recurrence risk depends on child’s age at presentation: 1 year old: 50% recurrence;

2 year old: 30% recurrence

Where there are no neurodevelopmental problems and no family history of epilepsy, the subsequent risk of epilepsy is 1% (equivalent to the population risk).

A 5-year-old girl, Emma, attends the ED with after being unwell for the last 3 days. It initially started out with fever, headache and a sore throat. She then developed a rash 24 hours ago. Her parents report that the rash started on her abdomen and spread to the neck and arms and legs and it feels rough to touch. 

On exam she has a sandpaper type rash on her trunk and limbs which is more pronounced in flexures. 

What is the most likely diagnosis?

What other symptoms might Emma have?

What is it caused by?

What are the differential diagnoses?

How is the diagnosis confirmed? What investigations will you do?

What is the treatment? Why do you treat?

Discussion point – Evidence for antibiotic therapy 

What are the possible complications? How can you categorise them?

What advice do you give to Emma’s parents in order to prevent transmission of Scarlet fever?

Is there anything else you need to do?

Scarlet fever 

The symptoms of Scarlet fever start with fever (over 38.3°C), sore throat and general fatigue/headache/nausea. 12-48 hours later a rash appears on the abdomen and spreads to neck and extremities. 

Characteristic features of the rash are a rough texture (like sandpaper) and worse in the skin folds e.g. groin, axilla, neck folds (Pastia’s lines). 

Other symptoms include white coating on tongue which then peels and leaves a ‘strawberry tongue’; flushed red face with perioral pallor and cervical lymphadenopathy. Most symptoms resolve within a week. After the symptoms have resolved it is common to get peeling on the fingers and toes. 

In Emma’s case the other symptoms that you would look for are – cervical lymphadenopathy, white tongue or strawberry tongue and a flushed face with peri-oral pallor. 

Scarlet fever is caused by the bacterium Streptococcus pyogenes (also known as group A streptococcus, or GAS). It can be found on the skin or in the throat, where it is usually unproblematic in asymptomatic carriers – 20% of children are colonized. 

However, certain virulent forms of S. pyogenes carry genes that code for streptococcal superantigens, including pyrogenic exotoxins, which can cause non-invasive infections such as scarlet fever. The typical rash is caused by the exotoxin.

They include measles, glandular fever, slapped cheek infections, other viral pathogens, Kawasaki disease, staphylococcal toxic shock syndrome, and allergic reactions.

The diagnosis is clinical. A throat swab is not routinely recommended, although during specific outbreaks Public Health England might advise this.

Streptococcal antibody tests are used to confirm previous group A streptococcal infection. They may be of value in patients with suspected acute renal failure, acute glomerulonephritis or rheumatic fever.  

Antistreptolysin O (ASO) test is the most commonly available streptococcal antibody test. ASO titres peaks 2-4 weeks after an acute infection and returns to normal over the next 6-12 months. Streptolysin O is produced by almost all strains of S. pyogenes (group A streptococci) and many group C and group G beta-haemolytic streptococci. 

Anti-deoxyribonuclease B (anti-DNase B (ADB)) titres rise after both pharyngeal and skin infections. DNase B is produced by group A streptococci and is therefore more specific than the ASO antibody test.

General guidance for patients may include advice on rest, drinking plenty of fluids, good hygiene measures to minimise the risk of cross-infection, and the use of paracetamol to reduce discomfort and high temperature.

Overall, the evidence base for the management of scarlet fever is limited, and there is a need for more evidence of the benefits and harms of antibiotics.

Public Health England, NICE and the Department of Health in Western Australia recommend treating people with scarlet fever with antibiotics regardless of severity of illness to speed recovery, to reduce the length of time the infection is contagious, and to reduce the risk of complications.

Recommended antibiotic therapy is Penicillin V QDS x 10/7 or azithromycin OD x 5/7 if penicillin allergic. 

Complications of Scarlet Fever are much the same as complications of strep tonsillitis. They are divided into suppurative, and nonsuppurative.

Suppurative complications occur due to the infection spreading and include otitis media; mastoiditis; sinusitis; peritonsillar abscess; meningitis; endocarditis; retropharyngeal abscess; and invasive group A strep (IGAS).

Non-suppurative complications occur later and occur mainly in untreated patients. They are rheumatic fever and post-strep glomerulonephritis.

IGAS is not common in children, but those at increased risk are children with co-morbidities, immunocompromised children, and those with co-existing chickenpox.

(DFTB – Exclusion period for infections)

Children should be excluded from school until they have had 24 hours of antibiotics.

Check if you need to contact the public health authorities – scarlet fever is a notifiable disease in many jurisdictions e.g. England, Wales, Northern Ireland and Western Australia. 

(Based on Life in the Fast Lane case – LITFL – Kawasaki Disease)

Alex, a 4 year-old boy has been brought to the emergency department by his worried parents. He has had fevers for the past 6 days. They are concerned because he is not getting better despite repeated visits to a number of doctors. Each time they were told he had a viral illness.

On examination you note the presence of bilateral conjunctivitis, and erythematous rash on his torso and limbs, a 4 cm tender left-sided cervical lymph node and a diffusely red pharynx.

What is the most likely diagnosis?

How is the diagnosis made?

Who gets this condition?

What are the important differential diagnosis?

What investigations should be performed?

What complications may occur?

What specific treatment is required?

Discussion point – Incomplete Kawasaki Disease: Another child, Sarah, attends the ED with 6 days of fever. On exam you find a strawberry tongue and cervical lymphadenopathy >1.5cm. No other signs of Kawasaki disease are present. What might you consider?

Discussion point – Is there a roll for steroids in Kawasaki disease?

Kawasaki disease, also known as Mucocutaneous Lymph Node Syndrome, this vasculitic disorder was first described by Dr. Tomisaku Kawaski in 1967. It is of uncertain etiology, but may be a post-infectious condition.

The diagnosis is made on the basis of the following clinical criteria (A + B):

A.Fever ≥5 days
B.At least 4 of the 5 following physical examination findings:
1.Bilateral, nonexudative bulbar conjunctival injectionbilateral scleral injection with peri-limbic sparing
2.Oropharyngeal mucous membrane changespharyngeal erythema, red/cracked lips, and a strawberry tongue
3.Cervical lymphadenopathywith at least one node >1.5 cm in diameter
4.Peripheral extremity changesacute phase: diffuse erythema and swelling of the hands and feetconvalescent phase: periungual desquamation (weeks 2 to 3)The diffuse palmar erythema seen in KD is in contrast to the discrete macular lesions of various viral illnesses (e.g., measles) that can sometimes be seen on the palms and soles.
5.A polymorphous generalized rashNon-vesicular and non-bullousThere is no specific rash that is pathognomonic for KD

The manifestations may appear sequentially rather than concurrently. Atypical cases may not meet all the criteria but may still have the same risks of cardiac complications. These 

‘incomplete’ cases occur more often in infants less than 6 months-old — further investigations (see Q5) should be performed if fever of 5 days and 2 or 3 of the other criteria are present.

Kawasaki disease may occur in any child of any age, and even adults in some cases. However, it is more common in:

  • children aged < 5 years
  • child of Asian descent
  • males (RR 1.5)

Diagnosis may be difficult as Kawasaki disease may mimic a number of other conditions:

  • Viral exanthemas including measles
  • Streptococcal disease (e.g. scarlet fever, toxic shock syndrome)
  • Staphylococcal disease (e.g. scalded skin syndrome, toxic shock syndrome)
  • Bilateral cervical lymphadenitis
  • Leptospirosis and rickettsial diseases
  • Stevens-Johnson syndrome and Toxic Epidermal Necrolysis
  • Drug reactions including mercury hypersensitivity reaction
  • Juvenile Chronic Arthritis

Echocardiography —
this is the most important investigation to assess for cardiac complications.
If no abnormalities on presentation the study should be repeated in 4-6 weeks.

Laboratory tests
Rule out other causes:
— ASOT, AntiDNAse B, throat swabs, blood cultures
Non-specific findings seen in Kawasaki disease include:
— FBC: normochromic anaemia and leucocytosis; thrombocytosis (in the 2nd week)
— LFT changes and hypoalbuminemia
— increased CRP and ESR
— Sterile pyuria of ≥10 WBCs per high-power field

Cardiac complications:

  • Carditis during the febrile phase
    — myocarditis with ST-T changes (25%), pericardial effusions (20-40%), valvular dysfunction (1-2%) and cardiac failure (~5%)
  • Coronary vessel abnormalities (occur in 20% of cases if untreated and <5% if treated; peaks at 2-4 weeks)
    — aneurysm formation may lead to fatalities from thrombosis, rupture or ischemia-related dysrhythmia (usually within 6 weeks of onset, but may occur many years later.

Kawasaki disease is a vasculitis that can potentially affect almost any organ, it is commonly associated with:

  • arthritis
  • keratitis and uveitis
  • diarrhoea, vomiting and gallbladder disease
  • coryza and cough

IV immunoglobulin and aspirin

IV immunoglobulin

  • 2g/kg IV over 10 hours
  • ideally start within 10 days of the onset of the illness
  • a second dose may be given if fevers persist

Aspirin

  • 3-5 mg/kg PO daily for 6-8 weeks
    (when laboratory parameters have fully normalised)
  • some advise higher doses of aspirin until the patient is afebrile or 48-72 hours, but others argue this offers no benefit in addition to treatment with IV immunoglobulin.

Despite these therapies 2-4% of cases still go on to develop coronary artery abnormalities. Corticosteroids may be considered in refractory cases, although there is little evidence supporting their use.

Another child, Sarah, attends the ED with 6 days of fever. On exam you find a strawberry tongue and cervical lymphadenopathy >1.5cm. No other signs of Kawasaki disease are present. What might you consider?

Incomplete Kawasaki disease

DFTB – Kawasaki Disease

Very easily missed
Makes up 15-20% of all cases
Patients with incomplete KD, particularly those <6 months of age and older children, may experience significant delays in diagnosis and these children are at high risk of developing coronary artery abnormalities.

Consider KD if:

  • Infants <6 months old with prolonged fever and irritability
  • Infants with prolonged fever and unexplained aseptic meningitis
  • Infants or children with prolonged fever and unexplained or culture-negative shock
  • Infants or children with prolonged fever and cervical lymphadenitis unresponsive to antibiotic therapy
  • Infants or children with prolonged fever and retropharyngeal oroparapharyngeal phlegmon unresponsive to antibiotic therapy

Fever and pyuria in an infant or young child may be diagnosed as a urinary tract infection, with subsequent development of rash, red eyes, and red lips attributed to an antibiotic reaction. Irritability and a culture-negative pleocytosis of the cerebrospinal fluid in an infant with prolonged fever suggestive of aseptic meningitis (or if antibiotics have been given, partially treated meningitis) may cause a diagnosis of KD to be overlooked. Cervical lymphadenitis as the primary clinical manifestation can be misdiagnosed as having bacterial adenitis. Gastrointestinal symptoms are considered for surgical causes, other physical findings of KD can be overlooked.

Toxin-mediated illnesses, such as group A streptococcus infections (e.g. toxic shock syndrome and scarlet fever) can also present with fever, rash, mucous membrane involvement and abnormal extremity findings. Desquamation in Kawasaki disease tends to affect the hands and feet as it does in scarlet fever and toxic shock syndrome; however, in Kawasaki disease, it usually begins in the periungual region. In scarlet fever, the desquamation tends to be diffuse and flaking, whereas in Kawasaki disease it tends to be sheet-like.

BMJ – Kawasaki disease

Children with measles and Kawasaki disease tend to be very irritable and inconsolable. It can be difficult securing the diagnosis of Kawasaki disease as the clinical features may appear sequentially rather than at the same time. In Kawasaki disease there may be presence of erythema and induration at the BCG immunisation site as there is cross reactivity between the heat-shock protein and the T-cells of patients with Kawasaki disease. 

The temperature in measles may exceed 40°C but tends to fall after day 5 of the illness. Koplik spots are not seen in Kawasaki disease and the morbilliform rash of measles begins from the ears and hairline and starts to fade by day 4; after day 7 brownish staining may be seen due to capillary haemorrhage. Desquamation in severely affected cases of measles can occur but is not seen in the hands and feet. In measles, clinical improvement typically begins within 2 days of appearance of the rash.

This table can help distinguish between differentials:

(Cochrane – Using steroids to treat Kawasaki disease)

A Cochrane review published January 2017 concluded “steroids appear to reduce the risk of heart problems after Kawasaki disease without causing any important side effects. They also reduce the length of symptoms (fever and rash), length of hospital stay, and blood markers associated with being unwell. Certain groups, including those based in Asia, those with higher risk scores, and those receiving longer steroid treatment, may have greater benefit from steroid use, especially with decreasing rates of heart problems, but more tests are needed to answer these questions”.

What disease is associated with dermatitis herpetiformis?

A: Herpes

B: Coeliac disease

C: Atopic dermatitis

D: Melanoma

The correct answer is B.

In coeliac disease, there are IgA antibodies against gluten that cross-react with reticulin fibres that anchor the basement membrane to the dermis. Thus, IgA is deposited at the tips of dermal papillae, presenting as grouped pruritic vesicles, papules or bullae. Usually found on elbows.

What is the most common causative agent of erythema multiforme?

A: Penicillin and sulphonamides

B: Systemic lupus erythematosus

C: HSV infection

D: Malignancy

The correct answer is C.

HSV is the most common etiologic agent of EM, which presents as a targetoid rash and bullae. All the other options are also associated with the disorder, but less commonly.

What disorder is characterised by an initial ‘herald patch’ which is then followed by scaly erythematous plaques usually in a ‘Christmas tree’ distribution?

A:    Pityriasis rosea

B:    Herpes

C:    Varicella zoster virus

D:   Erysipelas

The correct answer is A.

Pityriasis rosea classically presents with a salmon coloured solitary patch ‘herald patch’ which enlarges over a few days followed by generalised bilateral and symmetric macules with collarette scale. Pruritus is sometimes present. It itself resolves within 6 – 8 weeks.

What is the infective agent implicated in acne? 

A: Staphylococcus aureus

B: Streptococcus pyogenes

C: Staphylococcus epidermidis

D: Propionibacterium acnes

The correct answer is D.

Propionibacterium acnes infection produces lipases resulting in inflammation and breakdown of sebum, leading to pustule formation.

Which of the following statements about the treatment of measles is correct?

A: No specific antiviral therapy is recommend for immunocompetent patients 

B: Prevention of spread of measles depends on prompt immunization of people at risk of exposure or people already exposed who cannot provide documentation of measles immunity

C: Recommend supportive care with antipyretics, fluids and rest

D: All of the above

The correct answer is D.



Please download our Facilitator and Learner guides

Seizures Module

Cite this article as:
Peter Tormey. Seizures Module, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.27858
TopicSeizures
AuthorPeter Tormey
DurationUp to 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

Quiz (10 mins)

Infographic sharing (5 mins): 5 take home learning points

We also recommend sharing a copy of your local guideline.

Unprovoked seizures are common in children with around 8% having a seizure by 15 years of age

  • Most seizures are brief, self-limiting and generally cease within 5 minutes
  • Convulsive status epilepticus is the most common paediatric neurological emergency worldwide and the 2nd most common reason for PICU admission in the UK.
  • A seizure is the clinical expression of abnormal, excessive, synchronous discharges of neurons residing primarily in the cerebral cortex

Was the seizure a primary event or secondary to something else?

Seizures can be due to an underlying epilepsy or can be acute symptomatic seizures due to:

  • Hyponatraemia
  • Hypoglycaemia
  • Hypocalcaemia
  • High fever
  • Toxin exposure
  • Intracranial bleeding
  • Meningitis

Was this really a seizure or should I consider other differentials?

Tonic clonic activity and incontinence are not specific for seizures so always consider differential diagnoses.

  • Differential diagnosis of a seizure:
    • Vasovagal syncope
    • Blue breath holding spell
    • Reflex anoxic seizure
    • Arrhythmia
    • Non-epileptic paroxysmal event

Seek out clues in the history:

A sudden fright or minor trauma followed by the child turning pale and seizing is suggestive of a reflex anoxic event secondary to a vagal reflex. Hypoxia can induce a short tonic-clonic event that looks like a generalized tonic-clonic seizure but the child will recover quickly.

A history of a temper tantrum crescendo-ing into the child holding their breath, turning blue and then seizing might make you think of a breath holding attack. Again, this child will recover quickly.

Standing in a hot, stuffy room, feeling lightheaded with some visual changes and echoey hearing sounds vasovagal. Compare this to a child who describes palpitations or is exercising before the event; this child could have had an arrhythmia.

A 7-year-old boy called Simon is brought to the ED by his parents. At approximately 7am they were awoken by noises coming from his room. They ran in and noticed that the left side of his face was jerking and he was drooling and making gurgling sounds. He wasn’t responding to them.

The movements stopped after 2 minutes. He was drowsy for a few minutes after and had difficulty talking and expressing himself for 15-20minutes after. They also noticed there was a slight drooping on one side of his mouth for 15-20 minutes.

He has now fully recovered and is bright and alert in ED with GCS 15/15 and a normal neurological exam.

What are some of the key elements of Simon’s past medical history that you must ascertain?

How would you classify his seizure? 

Are there clues in the history as to what specific seizure disorder he may have?

Would you perform any investigations at this point?

Does he need to be admitted? Does he need treatment? What follow up will you arrange?

Any history of hypoxic injury at birth?

Did he have any delay in meeting his developmental milestones?

How does his school performance compare to that of his peers?

Is there any history of similar events? Or unusual behaviours or word-finding difficulties on waking from sleep?

The key here is to determine if Simon is an otherwise well child or if there are details in his medical history, such as developmental delay, that may make him more prone to developing epilepsy

It is also important to determine if he perhaps has had more subtle seizures in the past that may have been missed

This is an opportunity to look at the International League Against Epilepsy infographic.

Simon has had a focal motor seizure with impaired awareness.

Specific seizure disorder: Simon’s seizure would be most in keeping with a clinical diagnosis of benign childhood epilepsy with centrotemporal spikes (BCETS) also known as benign rolandic epilepsy.

BCETS usually presents in early school age children with normal development. The most common seizure type is a focal motor seizure involving the face. There may or may not be impaired awareness. They can also be associated with facial numbness, hypersalivation, drooling, dysphasia and speech arrest. Motor activity in the upper, but not lower, limbs is common.  They may also progress to a generalized tonic-clonic seizure. Approximately 75% of seizures occur at night or on awakening and, therefore, can be easily missed. Patients may have a post-ictal paresis, often of one side of the face which can be concerning for a cerebrovascular accident. 


A blood glucose should be checked. 

Electrolytes are often checked with a first seizure but their utility decreases with patient age and degree of recovery. 5

As this history is strongly in keeping with a diagnosis of BCETS, an EEG is not strictly necessary to confirm the diagnosis, however, your local guideline for first seizure management should be followed.

There is no indication for neuroimaging at present.

Patients generally do not need to be admitted after a first seizure with no red flags:

  • Seizure related to head injury
  • Developmental delay or regression
  • Headache prior to seizure   
  • Bleeding disorder or on anticoagulant medication
  • Drug or alcohol use
  • Focal neurological signs or incomplete recovery
  • Seizure >5 minutes
  • Social concerns e.g. parental coping mechanisms or concerns over parental ability to recognize and seek medical attention if another seizure were to occur

Patients with an uncomplicated first seizure generally do not need to be commenced on treatment. BCETS in particular generally has a benign course and rarely requires treatment. 

All children who have a first seizure episode should be referred for paediatric follow up. This may be General Paediatric or Paediatric Neurology follow up and local referral pathways should be consulted. 

The International League Against Epilepsy have a useful infographic for managing a first seizure:

https://www.ilae.org/patient-care/first-seizure

For more information on managing a 1st afebrile seizure see: 

https://dontforgetthebubbles.com/first-afebrile-seizure/

Emily is a 4-year-old girl brought to ED with episodes of disturbed sleep for the last 3 weeks. This is her 4th visit to ED. She was previously diagnosed with “night terrors” and reassured. Her mum is concerned because the episodes are now occurring each night, having previously been 1-2 per week.

Her mum has videos of the episodes, which she shows you. The events usually occur shortly after going asleep. In the videos Emily wakes from sleep, looks terrified and stares straight ahead. The episodes go on for 2-3minutes. She usually vomits or retches towards the end of the episode. She goes back to sleep after. She is well during the day.

What could be going on here?

What interesting details in the history might lead you towards a specific diagnosis?

What could help differentiate between epileptic and non-epileptic events in this case?

What is the prognosis for these patients?

These episodes sound unusual and their frequency and severity seems more pronounced that what could be put down to normal variance in sleep pattern and arousal. Emily’s symptoms are not likely to be simple night terrors.

Seizures commonly occur in sleep and as a result can be missed or present subtly or without characteristic features.

There are several features in the history that would suggest Panayiotopoulous Syndrome (PS). 

PS is a focal epilepsy that occurs in children aged 1-14 years with a mean age of 5 years. The seizures are usually nocturnal

It is thought PS accounts for 6% of children with epilepsy.

There is a strong association with vomiting (70-85%) of patients. Visual symptoms are also closely related, given the seizures originate in the occipital lobe. Autonomic features can also be seen: pallor, tachycardia, miosis, coughing and hypersalivation.

They may also have head or eye deviation and focal or generalized clonic activity.

The diagnosis of PS is often delayed due to misdiagnosis with other causes of vomiting and autonomic manifestatons e.g encephalitis, migraine, syncope or gastroenteritis.

PS could easily be clinically misdiagnosed as night terrors. Night terrors are dramatic awakenings that usually happen during the first few hours of sleep. They share several characteristics with PS but there are also some subtle differences highlighted in the table below:

Clinical FeaturePSNight Terrors
Usual duration 5-10min++
Occur during the first few hours of sleep++
Autonomic Symptoms e.g tachycardia, tachypnea, sweating+++
Impaired awareness++
Child looks scared++
Starring+++
Running or walking around during episode++
Screaming++
Vomiting++
Thrashing of arms and legs++

Table 1. Clinical characteristics of PS and night terrors

It is not unreasonable to clinically diagnose night terrors if they present with characteristic events, more in keeping with night terrors than PS. However, if there are unusual features, such as vomiting, or exaggerated autonomic symptoms, or in a child who represents, then an alternative diagnosis should be considered. 

An inter-ictal EEG will usually be diagnostic in PS with occipital spikes, which are enhanced in sleep, the characteristic feature. In a child with unusual events occurring in sleep and a normal EEG, a video telemetry EEG may be useful to try and capture and characterise the events and outrule seizures as a possibility.

PS usually has a benign course with spontaneous remission commonly occurring within 2-3 years of onset. 

Seizures are generally infrequent but oxcarbazepine may be required to reduce seizure frequency.

Emma is a 3-year-old girl with a background of refractory epilepsy and developmental delay. Her current medications include levetiracetam, sodium valproate, clobazam and lamotrigine.

She is PEG fed but has been vomiting up her feeds for the last 2 days and mum is unsure if her medications have been staying down.

She normally has up to 20 short seizures per day at home, but this has been increasing in the last 2 days.

You get a pre-alert from the ambulance service: Emma has been having a generalised tonic clonic seizure for 15 minutes. Her mum gave her buccal midazolam at 5 minutes, but it has not had any effect.

The ambulance crew ask you can they repeat the dose of buccal midazolam?

Emma arrives in resus with the seizure ongoing. What is your management plan?

Emma has had two doses of benzodiazepines. What would be your next line agent? Who else should you be calling at this stage?

You decide to suggest 2 papers, the ConSEPT and EcLiPSE papers for your department’s next journal club and to discuss what effect they will have on your department’s practice. One issue you foresee is that a lot of the patients you see are already on maintenance levetiracetam.

Does this preclude children on maintenance levetiracetam from receiving IV levetiracetam in status epilepticus, as is the case with the use of phenytoin in patients who take it as maintenance treatment?

Emma’s seizure terminated with the second line agent and she was admitted under neurology for IV fluids and ongoing management of her seizures until she could tolerate her medications by PEG again. In this case her status epilepticus was likely due to her vomiting up her medications.

Had Emma’s seizure not stopped after the loading dose of phenytoin, what would your next steps be?

There is a risk of respiratory depression with any benzodiazepine. 

A Cochrane review9 in 2018 found that 25/346 (7.2%) patients treated with buccal midazolam experienced respiratory depression. There was no statistically significant difference in risk of respiratory depression between buccal midazolam and other benzodiazepines, administered via various routes. 

The drug information leaflet or Summary of Product Characteristics (SPC) for Buccolam® and Epistatus® recommend that only a single dose be administered at home by a caregiver and that additional doses should only be administered after seeking medical advice and, preferably, under medical supervision.

In this case, it would be reasonable to advise a second dose of buccal midazolam, presuming the paramedics had the necessary equipment and skillset to manage any respiratory depression that may occur. 

Factors that may influence your decision are: the ETA of the ambulance and if the child has a history of respiratory depression with benzodiazepines.

She should be managed as per the APLS guideline (please note this is the Australian APLS guideline and has been updated to include the use of levetiracetam as a second line agent. This has not yet been included in the UK APLS guideline, see discussion below):

At this point you should be informing PICU about the patient and your PEM consultant if you haven’t done so already.

The CONCEPT and ECLIPSE trials were published concurrently in May 2019.

These two studies looked at whether levetiracetam is non-inferior to phenytoin as a second line treatment in the management of convulsive status epilepticus in children.

This question was posed as phenytoin is linked to many adverse events including liver damage, Steven-Johnson syndrome, extravasation and reports of death due to dosing errors. As a result, and because of its biopharmacology, it is a resource-intensive drug to make up in an emergency.

Levetiracetam can be given over 5 minutes (phenytoin takes 20 minutes to infuse), is more compatible with IV fluids, has less drug interactions, and has a lower risk of adverse events.

The infographic below provides a nice summary:

You can find a more detailed summary at: https://dontforgetthebubbles.com/consept-eclipse-status-epilepticus/

It’s important to note that the primary outcomes of the two studies were different:

ConSEPT – The primary outcome was seizure cessation 5 minutes after the drug infusion and where possible the seizure cessation was verified independently via a video recording to reduce observer bias between the two groups.

EcLiPSE – In a key difference to the ConSEPT study the primary outcome was time “from randomisation to cessation of all visible signs of convulsive activity, defined as cessation of all continuous rhythmic clonic activity, as judged by the treating clinician”. As per the inclusion criteria this a very real world pragmatic approach.

The two studies concluded:

ConSEPT – Levetiracetam is not superior to phenytoin as a second line agent for convulsive status epilepticus

EcLiPSE – There is no significant difference between phenytoin and levetiracetam in the second-line treatment of paediatric convulsive status epilepticus for any outcome, including time to seizure cessation

Here is a section of the commentary from the post:

“While there were differences between the study designs, the primary outcome measure of timing being the largest, the fact that both studies found no difference probably means head-to-head there is little difference.

The nature of the statistical analysis means that both groups rightly point out that in their cohorts levetiracetam wasn’t superior in outcomes to phenytoin. A future pooled analysis could still demonstrate a difference, but it seems unlikely that a critical difference will be seen (especially for the safety element).

Given the wealth of evidence on the side effects of phenytoin it is surprising the incident rates were relatively low. Whether in study conditions more care was taken with drawing up and delivering the drug or that previous safety reviews were heterogenous in their inclusion criteria is difficult to know. However, the time to draw up phenytoin, and the background concerns on its potential harm, will lead some to suggest that the switch to levetiracetam is a logical one, regardless of its effectiveness against phenytoin.

The challenge faced by many units is a capacity for PICU beds. Because phenytoin is given over 20 minutes there is time to prepare for airway/anaesthetic intervention if it is unsuccessful in terminating the seizure. The use of levetiracetam may cause some to wonder if they should then try phenytoin either as a stop gap to bed availability or because the time in status now seems ‘shorter’ than normal. These are not statistical issues, these are pragmatic clinical conundrums.

The absence of a clear winner will further fuel this debate meaning it is unlikely in the immediate future we are going to see a change from the ALSG or similar organisations. However, local units may decide, in the clear absence of harm from levetiracetam, that it is a drug they should be adding into their treatment protocols.”

The EcLiPSE trial2 did not report any increase in adverse events in children who were on maintenance leveltiracetam and received a loading dose of IV levetiracetam. The ConSEPT trial excluded all patients who were on maintenance levetiracetam and phenytoin.

The use of phenytoin in status epilepticus in patients who are on maintenance phenytoin is avoided due to its potential cardiovascular side effects. As levetiracetam does not share these side effects and is generally safe and well tolerated it is reasonable to use it in children who are already on maintenance therapy. 

The EcLiPSE trial2 did not report any increase in adverse events in children who were on maintenance leveltiracetam and received a loading dose of IV levetiracetam. The ConSEPT trial excluded all patients who were on maintenance levetiracetam and phenytoin.

The use of phenytoin in status epilepticus in patients who are on maintenance phenytoin is avoided due to its potential cardiovascular side effects. As levetiracetam does not share these side effects and is generally safe and well tolerated it is reasonable to use it in children who are already on maintenance therapy. 

The current APLS guidance in the UK would be to proceed with RSI. As we have discussed above, the Australian APLS guidelines have changed, in view of the results of the ConSEPT and EcLiPSE, to include the use of an additional second line agent prior to proceeding to RSI. As reported in the ConSEPT trial, treatment with one drug and then the other reduced the failure rate by more than 50% at the expense of only an additional 10 minutes. 

For further discussions on advanced seizure management and RSI, the following podcast is recommended:

https://broomedocs.com/2019/06/paediatric-status-epilepticus-debate/

Caroline is a 13-year old girl who presents to ED with a first seizure. Her parents describe a generalised tonic clonic seizure that lasted 20 minutes.

She is an otherwise well girl who is doing well in school. The only concern in her past medical history is that she has been having frequent syncopal episodes for the last 12 months. She has been seen by her GP for this who reassured her that syncopal events were common on her age group and advised her to drink plenty of fluids and try and avoid triggers.

Her neurological exam is normal.

How would you proceed? 

Are there any investigations you could perform in the department to investigate the syncopal episodes she reports?

Is there any link between syncope or arrhythmogenic events and seizures?

Caroline is admitted for further cardiac investigations. She also has an EEG diagnostic for frontal lobe epilepsy which is linkced to ion channel abnormalities.

A blood glucose should be checked. If she has returned to her baseline and there were no red flags with regard to the seizure it would be reasonable to arrange outpatient follow up as per departmental protocol and advise her parents what to do if she should have further seizures.

An ECG and a lying-standing blood pressure should be performed.

Caroline has an ECG performed which has features consistent with Type 1 Brugada syndrome.

Seizures may be triggered by cerebral hypoperfusion due to an arrhythmic event. They can often be treated as a primary seizure and the underlying cardiac abnormality may be missed. Long QT syndrome in particular can present with seizures and almost half of affected patients are initially misdiagnosed and treated for epilepsy before the correct diagnosis is made.

Seizures can also be seen as a primary neurological abnormality, related to the cardiac abnormality. Brugada Syndrome is an autosomal dominant condition characterized by ECG alterations and a predisposition to tachyarrhythmias and sudden death. It is caused by a mutation in the genes SCNA5 and SCN1A. SCN5A codes for the alpha subunit of the voltage-gated sodium channel. As the condition is a channelopathy it can also be associated with epileptic seizures with the channelopathy affecting neuronal pathways. 

Caroline has a ECG which is consistent with features of Type 1 Brugada Syndrome.

There is coved ST segment elevation in V1 and V2 with a negative T wave. This is the only ECG abnormality that is potentially diagnostic and is often referred to as Brugada sign.

For an approach to the paediatric ECG have a look at the following DFTB post:

https://dontforgetthebubbles.com/approaching-the-paediatric-ecg/

For further reading on the specific ECG findings in Brugada Syndrome, please see:

https://litfl.com/brugada-syndrome-ecg-library/

Which symptom is more commonly seen with Panayiotopoulos syndrome than night terrors?

A: Staring

B: Terrified expression

C: Vomiting

D: Thrashing of arms and legs

E: Tachypnoea

70-85% of seizures in PS are associated with vomiting. Vomiting is not usually described in night terrors. The diagnosis of night terrors should be carefully applied to children having events disturbing their sleep that have a strong
association with vomiting.

Which of the following are side effects of phenytoin but not levetiracetam?

A: Mood disturbance

B: Cardiovascular toxicity 

C: Purple glove syndrome

D: Gingival hypertrophy

E: Stevens Johnson syndrome

The correct answers as B, C, & D

Although levetiracetam and phenytoin have several common side effects, cardiovascular toxicity, purple glove syndrome and gingival hypertrophy are more specific to phenytoin and are not generally seen with levetiracetam. Mood
disturbance is one of the most common side effects of levetiracetam. Stevens Johnson syndrome is also reported with levetiracetam.



Which of the following ECG findings are seen in Brugada syndrome:

A: Coved ST segment elevation in V1-3, >2mm

B: Prolonged PR interval

C: Negative T wave

D: Saddleback ST elevation, >2mm

E: LVH voltage criteria

The correct answers are A, C, & D

  • Brugada Type 1 has coved ST segment elevation in V1-3, >2mm, followed by a negative T wave. This is often referred to as Brugada sign.
  • Brugada Type 2 has >2mm of saddleback-shaped ST elevation
  • Brugada Type 3 can have the morphology of type 1 or 2 but with <2mm ST segment
  • elevation
  • Prolonged PR interval and LVH voltage criteria are not characteristic features of Brugada syndrome

1. Clinical Practice Guidelines : Afebrile seizures [Internet]. [cited 2020 Apr 20]. Available from: https://www.rch.org.au/clinicalguide/guideline_index/Afebrile_seizures/

2. Lyttle MD, Rainford NEA, Gamble C, Messahel S, Humphreys A, Hickey H, et al. Levetiracetam versus phenytoin for second-line treatment of paediatric convulsive status epilepticus (EcLiPSE): a multicentre, open-label, randomised trial. Lancet. 2019 May 25;393(10186):2125–34

3. https://www.uptodate.com/contents/seizures-and-epilepsy-in-children-classification-etiology-and-clinical-features?search=seizures%20in%20children&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1

4. https://www.ilae.org/education/infographics

5. https://dontforgetthebubbles.com/first-afebrile-seizure/

6. Michael M, Tsatsou K, Ferrie CD. Panayiotopoulos syndrome: An important childhood autonomic epilepsy to be differentiated from occipital epilepsy and acute non-epileptic disorders. Vol. 32, Brain and Development. Elsevier; 2010. p. 4–9.

7. Ferrie CD, Grünewald RA. Panayiotopoulos syndrome: A common and benign childhood epilepsy. Vol. 357, Lancet. Elsevier Limited; 2001. p. 821–3.

8. Weir E, Gibbs J, Appleton R. Panayiotopoulos syndrome and benign partial epilepsy with centro-temporal spikes: A comparative incidence study. 2018 [cited 2020 May 4]; Available from https://doi.org/10.1016/j.seizure.2018.03.002

9. Mctague A, Martland T, Appleton R. Drug management for acute tonic-clonic convulsions including convulsive status epilepticus in children. Vol. 2018, Cochrane Database of Systematic Reviews. John Wiley and Sons Ltd; 2018.

10. Dalziel SR, Borland ML, Furyk J, Bonisch M, Neutze J, Donath S, et al. Levetiracetam versus phenytoin for second-line treatment of convulsive status epilepticus in children (ConSEPT): an open-label, multicentre, randomised controlled trial. Lancet. 2019 May 25;393(10186):2135–45.

11. Sandorfi G, Clemens B, Csanadi Z. Electrical storm in the brain and in the heart: Epilepsy and Brugada syndrome. Mayo Clin Proc. 2013 Oct 1;88(10):1167–73.

12. Camacho Velásquez JL, Rivero Sanz E, Velazquez Benito A, Mauri Llerda JA. Epilepsy and Brugada syndrome. Neurol (English Ed. 2017 Jan 1;32(1):58–60.



Please download our Facilitator and Learner guides

Wound Management Module

Cite this article as:
Orla Kelly. Wound Management Module, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.27753
TopicWound management
AuthorOrla Kelly
DurationUp to 2 hours
Equipment requiredSkin glue, paper stitches (Steristrips), suture set, sutures, artificial skin or animal product for demonstration, sharps bins
  • Basics (10 mins)
  • Main session: (4 x 15 minute) case discussions covering the key points and evidence
  • Practical stations (30-60 mins)
  • Infographic sharing (5 mins): 5 take home learning points

We also recommend sharing a copy of your local guideline.

The skin is made of three layers: epidermis, dermis and subcutaneous layer. It has many functions including as a barrier to infection and regulation of homeostasis. Wounds in both paediatric and adult populations require similar assessment – haemorrhage control, assessment for underlying structural damage, infection potential and (lastly) scarring.

Highlight the importance of a full thorough examination. Wounds (particularly ones that are bleeding on presentation) have the tendency to be distracting. A full head to toe examination with a careful history taking is essential.

History

The mechanism of injury is the most important determinant of management. A crush injury to a finger will have decidely different outcomes than an incisional wound from a knife.

Crush injury: Is an xray required? A missed open fracture is unacceptable. Where did it occur? Is there potential for a foreign body?

Incisional wound: From what? Knife edge or glass? If glass, an xray is warranted. Risk of damage to underlying nerves tendons and vessels is high, consider the need to refer to local speciality surgical team.

Road rash: These must be cleaned out thoroughly to avoid tattooing. They can often cover large surface area so sedation may be required.

Puncture wound: Again, from what instrument? Is there potential for retained foreign body? Location is important, puncture wounds to the palmar surface of hand or finger runs the risk of infection, particularly flexor tenosynovitis.

Bite wounds: These require thorough washout and antibiotic cover and are not suitable for closure in the ED. Consultation with speciality surgical services is advised, often healing by secondary intention is more appropriate to avoid sealing in deep seated infection.

A 12 year old girl is brought into ED by her mum. As she was making lunch at home, the ring came off a ring-pull can, so she tried to pull open the lid of the can by grasping and pulling the sharp edges. She arrives with a blood soaked tea towel around her hand and is tearful. On examination she has wounds to her thumb, index and middle fingers of her right hand.

What structures require assessment in a wound?

How do you assess neurovascular function in hand injuries?

How do you assess the function of the flexor and extensor tendons?

A strong knowledge of anatomy is essential in the assessment of a wound; its location will indicate the possible structures that may be damaged. For example, on the face, be particularly mindful of the facial nerve, parotid gland and lacrimal ducts.

In the fingers, assessment of radial and ulnar digital nerves and arteries, superficial and deep flexors and extensor tendon is necessary and must be documented.  Don’t forget that tendons move; looking into a wound and seeing an intact tendon does not exclude a tendon injury. Wounds to hands and forearms must be carefully assessed, and if there is any possibility of damage to these structures, plastic surgery referral is recommended.

Another important factor of location is the likelihood of tension across the wound or articulation with a joint. The possiblity of an open joint means referral to orthopaedics for washout.

This is an opportunity to reference some learning from the RCEM learning soft-tissue injuries of the hand post.

The anterior interosseous nerve is a branch of the median nerve and has purely motor function. It innervates flexor pollicis longus, pronator quadratus and the radial half of the flexor digitorum profundus. The ‘OK’ sign tests it: if the O shape is pincer-shaped, rather than O shaped, with extension at the thumb IPJ and index DIPJ, it is abnormal.

The median nerve innervates the LOAF muscles (all of the thumb muscles except adductor pollicis). Although children are often asked to make a fist (the ‘rock’ of rock paper scissors), this also tests the ulnar nerve. To test for the median nerve alone, ask the child to oppose their thumb by touching it to the little finger against resistance.

The ulnar nerve innervates adductor pollicis, the lumbricals, palmar and dorsal interossei. The ‘scissors’ test (finger abduction) tests the dorsal interossei – test by abducting fingers against resistance.

Assess flexor digitorum profundus (FDP) and flexor digitorum superficialis (FDS) function, if there is a wound on the volar (palmar) surface of the hand or finger. Remember ‘P’ for point –FDP is assessed at the distal IPJ.

  • To check FDS function, hold all adjacent fingers in extension and then release the finger you want to assess. Ask the child to flex the free digit at the PIP joint, against resistance. A normal intact FDS can be flexed at the PIP joint on the unrestricted finger.
  • To examine the FDP, hold the middle phalanx in extension and ask the child to flex the DIP joint. A normal intact FDP is indicated by flexion at the DIP joint.

The extensor tendons are assessed by asking the child to extend their finger against resistance.

To test the Extensor Pollicis Longus (EPL), ask the patient to place their hand flat on a table and lift up their thumb against resistance.In young or uncooperative children, looking for normal wrist tenodesis can give clues about whether the flexor and extensor tendons are intact or not. Check out the DFTB finger injuries post for examination pearls.

A 4 year old boy is brought to the ED by his parents. He was playing with his brother in the garden when he tripped and hit his head off the curb. He did not lose consciousness and has not vomited. On examination there is a 6cm horizontal wound with jagged edges and visible contamination on his left forehead.

What techniques can you employ to ensure the child cooperates with assessment of his wound? Discuss non-pharmacological as well as pharmacological.

In order to fully clean, explore and close a wound, the patient must be comfortable.

Distraction techniques such as smart phones, books, bubbles should all be employed. If you are lucky enough to have a play specialist in your department then they should be used as soon as possible!

Topical anaesthetic such as LAT gel is wonderful for anaesthetising wounds, and the adrenaline in it blanches surrounding skin helpfully letting you know when it’s ready. If further anaesthesia is required, local anaesthetic (for example 1% lidocaine) can be used. A few tips to avoid the pain associated with injection: to inject it through the wound edges and not through surrounding intact skin, use a small needle (insulin syringe if only a small amount is needed), buffer it with bicarbonate to raise the pH, and to make sure it’s at room temperature.

If after all the armoury has been depleted, then the patient may need to be sedated, in which case a senior doctor should be involved, local guidelines followed, and consider referral for GA if appropriate.

 Have a look at the DFTB procedural sedation post or watch Deb Shellshear’s procedural sedation talk from DFTB17.

An 8 year old girl is brought to the department by her Dad. She was cycling in front of the house when she fell off her bike. She has a large area of road rash to her left forearm and left knee. It is visibly contaminated with gravel, dirt and plant material.

How will you ensure this wound is cleaned adequately?

How would your management change if the wound was deemed to be very dirty?

What closure options are available to you?

Washout

The mainstay of treatment of wounds is cleaning. Thorough washout with normal saline is recommended for all wounds. Scrubbing with a sponge or soft brush is essential for road rash to avoid tattooing and infection. For other wounds a cannula (without needle) can be fitted to the top of a 20 ml syringe and used to create a type of pressure wash. A polystyrene cup can be placed around the syringe to create a type of splash guard. All debris and underlying haematoma must be removed, if that’s not possible then formal washout in theatre is necessary.

Closure

Wounds can be closed via secondary intention (ie, with no adjuncts), glue, steristrips or sutures. Superficial dermal wounds (more scratches) and puncture or bite wounds are usually more suited to secondary intention healing.

Glue is useful for small wounds (<3cm) with clean easily opposable edges and no tension. As with all wounds, washout is necessary. When the wound is clean and not bleeding, the two edges must be gently pinched together, everting the edges, and glued in place. Be careful when pinching the wound sides together to avoid creating a valley that the glue sticks in – this will widen the scar. Aslo be careful not to glue your gloves to the patient, which is quite easy to do!

Steristrips are useful for similar wounds as glue, but can be longer. Prep the wound edges with tincture of benzoin prior to application of the steris to increase adherence. Place the strip on one side of the wound, and with a ‘pinch and pull’ motion, bring the wound edges together. Leave some space between the strips to allow fluid to escape to avoid infection. If there is a chance that little fingers or drool can get at the wound, some duoderm over the wound will leave the strips impervious.Finally, if the wound is under tension or likely to reopen due to movement, then suturing is recommended – see case 4.

A 12 year old boy is brought in by ambulance after falling off his bike while swerving to avoid a car. He was wearing a helmet and thinks he did not hit his head. He has a painful right ankle and is unable to weight bear. He has a wound to his mid anterolateral shin. It is horizontal, 4cm wide and approximately 1 cm deep. He is unable to weight bear and has pinpoint tenderness at his lateral malleolus X-ray confirms a non displaced isolated distal fibular fracture.

What size suture would you like to use?

What type: absorbable or non-absorbable?

What do you need to ensure before the child leaves the ED?

Suturing is useful for wounds that will be subject to movement and tension. Suture material can be divided into absorbable and non absorbable. The benefits to absorbable sutures is the lack of removal, however they can take time to dissolve, adding to scarring. Therefore they are useful for wounds that are not cosmetically sensitive such as the scalp and wounds under casts. In this case, there is an isolated wound that needs to be closed, and a separate distal fracture (NB not an open fracture). In this case an absorbable suture,  e.g. vicryl rapide, would be most suitable as this patient is likely to be in a cast or boot for a number of weeks.

The two types of sutures that should be demonstrated in the practice stations are deep dermal and simple interrupted. Deep sutures must be absorbable such as vicryl or monocryl, dermal sutures can be absorbable such as vicryl rapide, or non absorbable like prolene or seralon.

  • ALWAYS ascertain the tetanus status of the patient and consult local guidelines.
  • Most simple wounds don’t require antibiotic cover, but particularly contaminated ones and bites certainly do. It’s likely in those situations specialist referral is necessary.
  • Open fractures require prompt referral to orthopaedics and IV antibiotics.
  • Patients and parents must be advised about the warning signs for infection.
  • With regards to scarring – parents must be informed that everyone and every wound scars differently. The main thing is the wound stays clean, infection will certainly worsen cosmetic outcomes. Post initial healing, longitudinal steristripping for a number of weeks followed by pinpoint massage has been thought to improve scarring. The wound should be covered from ultraviolet rays for up to 18 months.

 Description and demonstration of steristrips, glue, different suture materials and equipment.

 Equipment needed:

Skin glue

Prolene, monocryl, vicryl rapide sutures

Suture kit

Sharps bin

https://geekymedics.com/simple-interrupted-suture-osce-guide/

https://dontforgetthebubbles.com/tissue-adhesive/

Demonstration and practice of local anaesthetic injection and simple interrupted sutures using either fake skin or animal skin (if using animal products, ensure hygiene standards are adhered to – yellow bin for disposal of gloves and soft waste).

Equipment needed:

Gloves

Table cover (eg incontinence sheet)

Fake skin or animal skin

Sharps bin

Hazardous waste bin if using animal products

Plastic apron if using animal products

5ml syringes

Dermal needles

Saline

Suture kits

Prolene sutures – sizes 3-0 and 4-0

https://geekymedics.com/simple-interrupted-suture-osce-guide/

This is an opportunity to cover more challenging sutures such as deep dermal sutures and 3 point sutures. It uses the same equipment as Station 2.

See Corner and deep suture videos:

https://litfl.com/own-the-wound/



Please download our Facilitator and Learner guides

Pyrexia of Unknown Origin Module

Cite this article as:
Beatrice Zanetti. Pyrexia of Unknown Origin Module, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.27689
TopicPyrexia of Unknown Origin
AuthorBeatrice Zanetti
DurationUp to 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
  • Quiz (10 mins)
  • Infographic sharing (5 mins): 5 take home learning points

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

To prepare for this session, learners could read the below case report article (20 minutes): 

Wood M, Abinun M. and Foster H., Pyrexia of unknown origin. Archives of Disease in Childhood, Education and Practice, 89 ep 63-69 (2004) 

And/or look at these useful resources from the web: 

https://pedemmorsels.com/fever-of-unknown-origin/ (5 minutes)

https://dontforgetthebubbles.com/recurrent-or-periodic-fevers-investigate-or-reassure/ (10 minutes)

https://dontforgetthebubbles.com/tuberculosis/ (5 minutes)

https://dontforgetthebubbles.com/claire-nourse-tuberculosis-at-dftb17/ (20 minutes)

https://gppaedstips.blogspot.com/search/label/Juvenile%20idiopathic%20arthritis  (10 minutes)

https://www.paediatricfoam.com/?s=kawasaki (10 minutes)

The term pyrexia of unknown origin (PUO) is used when a patient has more than 8 days with fever (temperature> 38°C) without a clinical diagnosis after exhaustive investigations have been carried out (in hospital or in primary care). 

Other more specific PUOs are:

  • nosocomial PUO
  • neutropenic PUO
  • HIV-associated PUO

These 3 have specific risk factors and will not be covered in this session. 

Fever is a sign of an underlying pathology. In PUO, pyrexia is usually the main symptom while other signs may be very subtle. Many times, the underlying disease is a common pathology that is presenting in an atypical or incomplete way.  

Here are 3 main clinical dilemmas for clinicians: 

In the paediatric population, 30% of PUO will not reach a final diagnosis. However, in those cases, PUO is often a self-limited and benign episode. 

When a definitive diagnosis is reached, the majority of causes are related to infectious diseases (38%), followed by connective tissue disorders/autoimmune pathology (13%) and malignancies (6%).

At present, there is not a generic PUO work-up since this wouldn’t be efficient. Remember that more than ¼ of cases are benign and self-limited! 

In many cases, PUO is a consequence of a late diagnosis. Clinical history taking and careful physical examination are crucial to pick up subtle signs and guide the complementary tests and imaging. New signs and symptoms, which weren’t present on initial examination, can appear later on.

In the literature, retrospective studies have shown that when imaging requests are prompted by some examination finding, they are more likely to yield a positive result. 

Here’s a table with possible differential diagnosis based on clinical findings.

Diagnosis based on common clinical findings
Rash
Maculopapular -Purpuric-Erythema Nodosum-Butterfly rashEBV, Kawasaki Disease, SOJIA, Typhoid feverCMV, Endocarditis, Leukaemias, Histiocytosis, VasculitisTB, Ulcerative colitis, Crohn’s disease, Streptococcal infectionSLE, Dermatomyositis
Adenopathies
-Infections-Connective tissue disorders-Malignancies-Other causesCMV, EBV, TB, Bartonella (Cat-scratch disease)Rheumatoid Arthritis (RA)
Lymphomas, Leukaemias, HistiocytosisSarcoidosis and Primary Immunodeficiencies
Splenomegaly
-Infections
-Connective tissue disorders-Malignancies
-Other causes
TB, Bartonella, Malaria, Visceral Leishmaniasis, Endocarditis, Brucelosis, SalmonelosisSLE, RA
Lymphomas, Leukaemias, Histiocytosis, Macrophagic Activation SyndromePrimary immunodeficiencies
Arthritis
-Infections-Connective tissue disorders-Malignancies-Other causesTB, Lyme disease, Brucella, Staphylococcal InfectionSOJIA, RA, SLE, Rheumatic fever
Leukaemias

The speed of the complementary tests will depend on the general appearance of the patient. Empirical treatment with antibiotics can blur the microbiology results resulting in a delayed diagnosis. The empirical use of steroids can mask other pathologies and again delay the diagnosis. 

For the above reasons, clinicians should reserve empirical treatment with antibiotics or steroids to the sick patient based on clinical assessment.

Causes of fever of unknown origin
Infectious: Bacterial infections: Localised: Pyelonephritis, Sinusitis, Mastoiditis, Pneumonia/pleural effusion, Osteomyelitis, Endocarditis, Intravenous catheter infectionAbscesses (intracranial, dental, intestinal, hepatic, pelvic…)
Systemic infections: Tuberculosis, Brucellosis, Bartonella (cat-scratch disease), Leptospirosis, Q fever (Coxiella), Lyme disease, Salmonellosis (Typhoid fever), Tularaemia. 
Viruses: EBV, CMV, Adenovirus, Enterovirus, HIV, Dengue
Fungal: Blastomicosis, Histoplasmosis, Coccidiomicosis
Parasites/protozoos: Malaria, Visceral Leishmaniasis, Toxoplasmosis, Visceral Larva Migrans, Tripanosomiasis
Connective Tissue DisorderKawasaki Systemic Onset of Juvenile Idiopathic Arthritis (SOJIA)Systemic Lupus ErythematousAcute Rheumatic fever
MalignanciesLeukaemia LymphomasSolid tumours (Neuroblastoma)Hemophagocytic SyndromeMyelodysplastic syndromeHistiocytosis 
Other: Drug related feversFabricated illness Inflammatory bowel diseaseCentral origin feverPeriodic feversMetabolic fevers (hyperthyroidism, dehydration)Primary immunodeficiencies

A 14-month-old girl was referred to hospital by GP due to 8 days of fever, non-tender cervical lymphadenopathies (scattered small submandibular, posterior and 1 supraclavicular lymphadenopathies) and mild cough. On examination, the patient has a good general appearance with a mildly red throat and the above described lymphadenopathies. Father is concerned as the child also had a febrile illness the previous week which was labelled as a viral infection. 

Blood tests showed raised WCC (24×109/L) with neutrophilia (18×109/L). Normal lymphocytes (6 x109/L) with a CRP of 30 mg/L. Chest x-ray showed a bilateral bronchial opacification.

The patient was admitted and started on amoxicillin and azithromycin PO. 

Despite 5 days of treatment, the patient is still spiking fevers (see chart below). Blood culture is negative. Clinically stable, cough has now disappeared. You are classifying this patient as PUO. 


What questions do you want to ask the parents? Take a detailed history. 

Why is this patient not getting better despite treatment? 

What investigations can be prompted by clinical findings?

At this point, would you escalate the antibiotic treatment?

In PUO, a detailed clinical history is the most important diagnostic tool that can guide all investigations. Instead of ordering random tests, ask more questions!

When taking the history, consider: 

  • Characteristics of the fever: when did it start, duration and intensity. Note that this child had a previous febrile illness which can be part of the same illness. 
  • Pattern of fever: there are several patterns of fever which can help with the diagnosis. If managed in an outpatient setting, ask the family to do a symptoms diary. From looking at the fever chart, the child has an intermittent fever pattern.
Type of feverCharacteristics of feverPossible causative agent
Intermittent feverSharp febrile peak which goes back to baselineBacterial infections, TB, SOJIA
Remittent feverFever peaks and elevated basal temperatureViral illness, Endocarditis, lymphomas
Sustained feverPersistent fever with minimal variationTyphoid fever, Brucellosis
Recurrent feverPeriods of fevers intercalated with afebrile periodsMalaria, Lymphomas, Borellia
Periodic feverFebrile periods are intercalated with afebrile periods with a predictable pattern of 6 monthsPeriodic fever syndromes 
  • Age of the child: PUO in young children is often caused by infections while in older children and teenagers tends to be caused by a connective tissue disorder or a malignancy.
  • Associated symptoms and signs: headaches, vomiting and diarrhoea, rash, arthralgias, myalgias, bony pain, lymphadenopathies. They can be very subtle therefore a systematic review of all systems is necessary. 
  • Systemic symptoms: fatigue, anorexia, weight loss, sweating.
  • Previous medical history: a history of many bacterial infections can be related to a primary immunodeficiency. The most common primary immunodeficiencies are: 
  1. Common variable immunodeficiency
  2. Chronic granulomatous disease

Usually the immunodeficiencies are associated with complicated infections, failure to thrive, atopic disease or autoimmune disease.

For more information: https://dontforgetthebubbles.com/ent-infections-immunodeficiency/

  • Vaccination history: patient is fully vaccinated as per UK protocol. This includes BCG due to risk factors (Parents are from a country where incidence of TB is > 40/100,000 or more). 
  • Regular medications or any exposure to new medication (think about drug- related fevers).
  • Family History: Ethnic background, consanguinity. Family is from India and there is no consanguinity. 
  • Environmental risk factors: TB contacts, area where family lives, exposure to animals, vectors (mosquitos, ticks…), food intake (unpasteurised dairy products, uncooked meat and fish), international travels (place, malaria prophylaxis and compliance of prophylaxis). 

Patient travelled to India to visit grandparents when she was 9 months old. She was exposed to some mosquito bites.  She lived in an urban area for 3 months. Parents were not aware of any TB contacts.

For international travellers, the following website provides relevant information on potential risks and outbreaks occurring on each country: https://travelhealthpro.org.uk/

When a patient has received a provisional diagnosis plus empirical treatment for at least 48 hours and there is no improvement, clinicians should use a systematic approach to understand the reasons behind the poor response to treatment. 

Here are the four main questions to be answered: 

  1. Is there a problem with the medication? 

The diagnosis is right but the problem is within the treatment. Issues with the treatment could be related to: 

-Drug resistances (MRSA, ESBL bacteria).

This website will provide a map with all antibiotic resistances over the world:  https://resistancemap.cddep.org/AntibioticResistance.php, treatments should, when possible, be guided by microbiological culture results.

-Underdosages of the antibiotics which don’t reach the effective concentration.

-Very virulent bacteria creating a toxin that requires more antibiotics (eg. Staph. aureus PVL) 

-Adherence to treatment (low compliance) 

-Malabsorption of medication (for example vomiting, diarrhoea when taking oral medications)

-The selected antibiotics are not reaching the right place of infection (bone, abscess…)

2. Are we targeting the wrong bug?

Antibiotics are mainly covering for bacteria but the actual infection can be caused by other microbes like viruses, atypical bacteria, TB, parasites and fungal infections. 

3. Is there a problem with the host? 

Consider whether the episode could be only a prolonged febrile syndrome for a common disease due to host problem.  The problem can reside in the immunity (immunodeficiencies), structural problems that can predispose to localized infections (for example a patient with previous abdominal surgery who now has an abscess) or whether the patient has a foreign body or a central catheter that can be the source of the infection.

4. The problem is not infectious: fever can be a sign of a tissue connective disorder, malignancies and other illnesses like a central origin related fever, drug related fever or a factitious illness.

This child has now had at least 12 days of fevers. It could be even longer if we consider that he had a febrile illness labelled as “viral” before this episode. 

On examination, the main clinical sign are the small cervical lymphadenopathies bilaterally with 1 small supraclavicular lymphadenopathy. This clinical finding could prompt the clinician to investigate for others cause: URTI, EBV, CMV, pneumonia, pleural effusion, TB, Bartonella, connective tissue disorders, malignancies, histiocytosis.

After 18 days of intermittent fever, cervical lymphadenopathies and some fatigue, the patient underwent a fine-needle aspiration of the supraclavicular lymphadenopathy. The histology showed a caseating granuloma and the microbiology sample showed acid fast bacilli. TB GeneXpert of the sample and culture were positive for non-resistant Mycobacterium Tuberculosis. Patient was diagnosed with tuberculous cervical lymphadenitis (extrapulmonary TB).

Probably not. Use of antibiotics can delay microbiological diagnosis since the blood cultures’ yield is decreased. If the patient has a good general appearance and fevers are well managed with PRN antipyretics, then clinician can consider withholding the antibiotics until a definitive diagnosis is reached. 

Reaching the definitive diagnosis: 
After 5 days of treatment with amoxicillin and azithromycin and no clinical improvement, basic investigations were repeated and further were added.

Bloods tests
WCC 22 x109/L with neutrophils of 18 x109/L,
Lymphocytes 4 x109/L, CRP 36 mg/L
ESR >60 mm/h
Normal renal and liver function
Blood film normal: No reactive lymphocytes, no lymphoblasts seen
Blood cultures Negative.
CMV Serology: IgM negative, IgG Negative
EBV serology: IgM negative, IgG Negative

Microbiology
Mantoux /TST (Tuberculin skin test): 10 mm induration
(Patient received BCG vaccination)
IGRA (Interferon gamma release assay): Positive
Gastric aspirate for AFB smear: negative 
Gastric aspirate for TB GeneXpert: negative

Imaging
Repeated chest XR: similar features compared to previous one, peri-bronchial shadowing.
Ultrasound of lymph-nodes: several lymph-nodes with nodal matting and surrounding soft tissue oedema. Prominent vascularity in the hilum.

After 18 days of intermittent fever, cervical lymphadenopathies and some fatigue, the patient underwent a fine-needle aspiration of the supraclavicular lymphadenopathy. The histology showed a caseating granuloma and the microbiology sample showed acid fast bacilli. TB GeneXpert of the sample and culture were positive for non-resistant Mycobacterium Tuberculosis. Patient was diagnosed with tuberculous cervical lymphadenitis (extrapulmonary TB).

  • Systemic symptoms 
  • Supraclavicular lymph-node
  • Firm and/or fixed lymph-nodes
  • CXR changes, abnormal Full blood count or increased ESR
  • Adenopathies > 1 cm in a neonate 
  • Suspicion of TB 
  • Persistent lymphadenopathies for more than 4 weeks despite antimicrobial treatment
  • Sometimes in acute lymphadenitis if a patient is not responding after 48 hours of treatment

To note, the patient had received the BCG vaccine. However, it has about 50% efficacy which implies that patients with BCG vaccination can still have tuberculosis. BCG is more effective in preventing children from developing disseminated (Miliary) TB or TB meningitis. She was probably exposed to TB and became infected while in India, subsequently developing the disease over the next few months. 

Contact tracing of family members is mandatory to identify the source case. Usually, children are not very infectious since the majority of cases tend to be paucibacillary (low bacterial load) unless they have lung cavities or extensive lung involvement. 

TB in children often presents in a non-specific way. The typical symptoms are weight loss or failure to gain weight, fever, night sweats and fatigue. When children present with pulmonary TB, this is usually confined within the intrathoracic nodes. Patients may have persistent cough and asymmetrical and persistent wheeze caused by airway compression due to enlarged tuberculous peri-hilar nodes. 

Chest XR can be helpful in the diagnosis of early primary infection by detecting intrathoracic lymph-node enlargement. However, these changes may be subtle as a strong index of suspicion is required. More information on radiological features of paediatric TB can be found on the following link: doi: 10.1101/cshperspect.a017855

Sputum and gastric aspirate mycobacterial cultures have a low diagnostic yield since most children have paucibacillary TB. Recently, diagnostic sensibility for these samples has increased due to the rollout of new molecular techniques (GeneXpert TB PCR).

TST (Mantoux test) and new immunological assays such as IGRAs detect exposure. TST is performed by injecting 0.1ml of tuberculin purified protein derivative (PPD) intradermally into the inner surface of the forearm. The skin reaction produced by the PPD should be read between 48 and 72 hours. The reaction is measured in millimetres of induration, not redness. There are different measures to define a positive result depending on patient background history (for example BCG vaccination) and there are also many causes of false positive and false negative results.For more information (https://www.cdc.gov/tb/publications/factsheets/testing/skintesting.htm).

On the other hand, IGRA is a blood test which measures the body’s immune response (interferon-gamma production) to TB antigens. Our patient had a positive Mantoux test (10 mm) but the result might have been affected by previous BCG vaccination.  However, this result, combined with a positive IGRA, demonstrated that the child had been previously exposed to TB. Unfortunately, neither of these tests can distinguish between latent infection and active disease. 

The patient was treated with Isoniazid (with Pyridoxine), Rifampicin, Ethambutol, and Pyrazinamide for 2 months and Rifampicin and isoniazid for another 4 months. Corticosteroids were not deemed necessary in this case since the lymphadenopathies were not compressing other structures. Empirical treatment of tuberculosis is usually limited to clinical cases where milliary or CNS TB are suspected, as a treatment delay in these cases will often lead to worse outcomes.

3-year-old boy with a 5-day history of fever and loss of appetite presented to the emergency department with his mother as he had been crying all night and refused to put his T-shirt on. No history of trauma reported. On examination, he looked skinny and he was crying when the right arm was moved. Bloods test showed
Hb 9 g/L
WCC 4 x109/L
Neutrophils 1.5 x109/L
Lymphocytes 2.5 x109/L
Platelets 120 x109/L.
CRP 40 mg/L.

Right arm x-ray was normal. The patient was admitted for observation. On the ward, it was noted that he was spiking fevers every night. 

After 3 days of admission, MRI of the right upper limb was performed. MRI showed possible osteomyelitis of the right distal clavicle.  He was diagnosed with acute pyogenic osteomyelitis and was started on ceftriaxone 50mg/kg IV OD. Blood cultures (taken before administration of antibiotics) were negative. Fever settled after 5 days of antibiotics. Patient was discharged home on oral antibiotics for 3 weeks.  

10 days later, the patient was reviewed in the clinic. Mother was worried since the patient had had fevers again over the last 2 days, felt fatigued and was reluctant to walk.

At this stage, what is the differential diagnosis? 

What investigations would you perform? 

What treatment would you give? If you were to suspect an autoinflammatory disease, would you give steroids? 

What is the role of PET-CT in PUO?

Infectious diseases: 

Osteomyelitis: 

Every time a patient presents with reduced range of movement due to a bony pain, osteomyelitis should be considered. In non-verbal children, it can present with irritability and inability to bear weight. It usually affects the metaphysis of the long bones (femur, tibia…). Therefore, the original diagnosis of clavicle osteomyelitis was quite rare. Now that the patient presented again with fever and a new similar problem after receiving adequate antibiotic therapy, another diagnosis should be considered. 

Septic Arthritis: 

It has a similar presentation to osteomyelitis but usually the joint is swollen, red and hot. Ultrasound of the joint can detect joint effusion which can be a sign of septic arthritis. Urgent orthopaedic referral for aspiration +- surgical washout is necessary. 

Connective tissue disorder: 

Transient synovitis: Fever and inability to bear weight can be a common presentation for transient synovitis. In this particular case, the initial diagnosis might have been wrong and the first inflammatory/infectious process could have triggered the production of antibodies causing inflammation over the joint. 

SOJIA (Systemic Onset of Juvenile Idiopathic Arthritis): Usually the joints affected by the arthritis are hot, tender and erythematous. We can suspect this pathology when there are different joints affected at different times. It is usually associated with systemic symptoms (fever and salmon pink rash, splenomegaly, serositis).

Diagnosis is made by elevated ESR, elevated ferritin, absence of antibodies, rheumatoid factor negative and exclusion of malignancy or infectious process.

CRMO (Chronic Recurrent Multifocal Osteomyelitis): this is an idiopathic inflammatory bone disorder with chronic multifocal bone pain. Sometimes systemic symptoms like fever can appear. Clavicle involvement is characteristic of this pathology.

Diagnosis is made by lesion’s biopsy: this will show an inflammatory reaction with no microbiological growth. 

Malignancies: 

Leukaemia: can present with bony pain and fatigue, lethargy and weight loss. Pancytopenia and blast can be seen in the blood film. LDH and uric acid are raised. Definitive diagnosis is reached with the bone marrow aspirate and flow cytometry.

Neuroblastoma: This is a malignancy that usually presents with abdominal mass. Sometimes mass can be found in the thoracic cavity. It appears in children below 5 years. The neuroblasts infiltrate the bone marrow. Therefore, patients can present with bony pain and pancytopenia. 

Diagnosis is reached by abdominal ultrasound and further imaging to evaluate the stage of the disease. Bone marrow aspirate is necessary along urine Vanillylmandelic Acid (VMA). 

Bone tumours (osteosarcoma /Ewing’s Sarcoma): 

Even though bone tumours are much less common than leukaemia and neuroblastoma, the presence of bony pain and prolonged fevers would prompt the diagnosis. LDH is usually elevated with raised calcium. 

X-Ray show bony abnormalities and further imaging with MRI or CT can provide more information. Sometimes biopsy of the lesion is necessary to confirm the diagnosis. 

Bone marrow is recommended in Ewing’s sarcoma. Metastasis and benign bone tumour should also be considered in the differential diagnosis.

Other: Histiocytosis:  this is a systemic illness which can affect bones. Associated symptoms are erythematous skin lesions, oral ulcers, lymphadenopathies, cough, shortness of breath, hepatosplenomegaly, malabsorption. Diagnosis is reached by seeing Langerhans cells in the biopsy of the lesion.

At this point, the patient has had a fever on and off for more than 3 weeks. A provisional diagnosis of osteomyelitis was made based on imaging findings. However, treatment is failing and the patient is now presenting with new symptoms (unable to bear weight). 

Repeated basic investigations:

Full blood count Hb 8.5 g/LWCC 1.0 x109/LLymphocytes 9.0 x109/LPlatelets 100 x109/LBlood cultureNegative
Peripheral blood film NormalUrine cultureNegative
CRP15 mg/LMantoux testNegative
ESR>40 mm/hHIV serologyNegative
Renal functionNormal rangeSickle cell testNegative
Liver function Normal range Chest XR Normal.
LDH900 U/L (240-480)
Uric Acid 12 μmol/L (High)

If after the above investigations, the clinician does not reach a diagnosis, then: 

  • Re-take a good clinical history 
  • Re-assessment of the patient
  • Withhold current medications
  • Do specific imaging (XR/Ultrasound/MRI of the new affected area)
  • Perform immunological studies: rheumatoid factor, ANA and anti-DNA antibodies, Immunoglobulins
  • Perform a bone marrow aspirate and trephine for histology, cytology and microbiology.

In this case, the diagnosis of osteomyelitis was discarded. SOJIA vs Leukaemia were the 2 main differential diagnoses.  Discussion regarding therapeutic steroid treatment for SOJIA was raised. 

Usually in PUO, steroid treatment should be avoided until malignancy is ruled out.  Steroids are used therapeutically in many oncology protocols. The use of steroids in an unconfirmed case of leukaemia can improve symptoms but it can blur the histological picture required for the diagnosis and confuse the staging process. This would lead to a delayed and potentially incorrect treatment. 

It is crucial to perform a bone marrow aspirate before steroid treatment is given, especially if there are symptoms and signs compatible with malignancies. 

In the above case, the full blood count showed mild pancytopenia which can be related to a bone marrow infiltration. The peripheral blood film was normal. Finally, the patient underwent a bone marrow biopsy and this confirmed the diagnosis of T-cell ALL. 

PET-CT is an imaging technique that localises anatomical parts with high metabolic activity, detecting hidden infections, malignancies or any inflammatory foci. 

PET-CT has proven to be useful in patients with PUO who are generally unwell, sick-looking, since early diagnosis is urgent in those patients. Otherwise, PET-CT can be used in those patients who have had extensive investigations done, have not had clinical improvement and still no diagnosis has been reached.

4-year-old boy presented with 5 days of fever, diarrhoea and vomiting and abdominal pain. No relevant past medical history. Fully vaccinated, BCG not included.

Initial blood test showed WCC 24.5 x109/L with neutrophils of 18 x109/L. CRP 139 mg/L. Hb 110 g/L and Platelets of 395 x109/L. He was admitted and started on amoxicillin, gentamicin and metronidazole. Blood cultures were negative and urine culture showed a sterile pyuria (WCC 2250 with no growth). Stool sample was negative. Abdominal ultrasound showed free fluid in the right iliac fossa. On examination, his abdomen was soft with some tenderness in lower quadrants.  He had a second ultrasound which showed findings suggestive of an appendicular mass. A repeated urine sample had 64 WBC and no growth. 

Meanwhile, fevers persisted: on day 7, he was changed to piperacillin-tazobactam and gentamicin. He underwent a laparoscopic appendicectomy on day 8. After operation, he was afebrile for more than 48 hours and antibiotics were stopped. Histological results of the appendix were normal. On day 12 of admission, the patient started again with fever and no focus on examination.

Now that the fever has restarted, and considering the previous history, what investigations would you ask? 

Would you re-start antibiotics? 

Looking at the pattern of fever below, what can you observe? 

Would an echocardiogram help in reaching the final diagnosis?

You should probably start by repeating basic investigations. Results: raised WCC with neutrophilia and thrombocytosis. Hb 101 g/L, WCC 32 x109/L with neutrophils of 24 x109/L, Platelets of 961 x109/L. He had normal renal and liver function.

Infectious diseases investigations: 

Microbiology cultures: Blood cultures were negative, even the prolonged culture for atypical bacteria. Stool sample was negative for viruses, bacteria and parasites. Urine sample became negative (previous sterile pyuria)

Toxoplasma serology:  IgG and IgM negative

CMV serology: IgM positive and IgG positive.  Second sample sent for CMV IgM negative. CMV PCR was negative. The initial positive IgM CMV was considered to be a false positive. IgM positivity in virology/microbiology assays may be non-specific, in patients with autoimmune diseases, cross-reactions.

EBV serology: IgM and IgG negative. 

Blood PCR for EBV, CMV and adenovirus negative.

Respiratory sample PCR: negative.

Lumbar puncture: LP was performed on day 14 of admission: WBC < 3/mm3. RBC <3/mm3. Viral PCR for enterovirus, parechovirus, mumps, VHS1&2 and VVZ negative. Negative culture.

Interferon Gamma Release Assay for TB negative. 

Inflammatory conditions investigations: 
Faecal calprotectin: negative. Since the patient had gastrointestinal symptoms and fever, Inflammatory Bowel Disease (IBD) should be considered as a potential differential diagnosis.

ESR: 50 mm/h.

Ferritin 222 ng/mL: important marker for inflammation. Especially high in Hemophagocytic lymphohistiocytosis /Macrophage Activation Syndrome.

Malignancies: 
Blood film: no atypical cells. Polychromasia and raised platelet count. Neutrophilia.

LDH 446 U/L (high)

Imaging
Day 12 Chest XR: normal
Day 13 Abdominal Ultrasound: normal kidneys and bladder. Normal liver, spleen, gallbladder and bile ducts. No abnormal masses or bowel wall thickening. Trace of fluid in right iliac fossa postoperatively. No fluid collection. 

The patient was clinically stable, so it was decided to wait and hold antibiotic treatment. The patient continued to have daily fevers up to 39°C. On day 14, he had one bilious vomit and became more lethargic therefore antibiotics were restarted (Piperacillin-Tazobactam and gentamicin). The following day, he underwent a Bone marrow aspirate and MRI under general anaesthesia with results as below: 

BMA: Trilineage haematopoiesis. No evidence of abnormal infiltration. No increased haemophagocytic activity. Appearances in keeping with a reactive marrow. Negative for AAFB, both microscopy and culture. 

Abdominal MRI:  There is moderate distention of the proximal small bowel with an apparent jejunal transition point due to ileus, adhesions or oedema from handling.  Some free fluid but no abdominal collections.  No retroperitoneal collection.  No bone marrow abnormality.

On day 16, he did not spike any temperatures.  After 48 hours (on day 18 of admission), he had an evening temperature of 38.5°C.

The patient had 2 episodes when he was apyrexial:

  • the first one between day 9- 11 after antibiotics were escalated (Piperacillin-Tazobactam and Gentamicin (D7)) and after surgery under general anaesthesia (D8).
  • The second afebrile period was on day 16-18 after being re-started on Piperacillin-Tazobactam and Gentamicin (D15) and after he underwent a procedure under general anaesthesia. 

In the first episode, the lack of fever was linked to a good response to antibiotics whereas in the second episode given the fact that a non-infectious condition was highly suspected as a differential diagnosis, the afebrile episode could be linked to the anaesthesia.

A very important investigation to perform in PUO is an echocardiogram to rule out infective endocarditis. In this case, there were no positive cultures or risk factors to point towards an infective endocarditis but it would be useful to rule out this disease. Echocardiography can also help to diagnose Kawasaki disease. In this particular scenario, it would be an incomplete case of KD. 

Reaching the diagnosis: 
On day 19, the patient had an echocardiogram which revealed dilated circumflex artery and an aneurysm of the left anterior descending artery. This finding confirmed the diagnosis of Incomplete Kawasaki. The ophthalmology review showed no pathological findings.  

DIAGNOSTIC CRITERIA FOR KAWASAKI DISEASE
Full case of KawasakiIncomplete case of Kawasaki
Fever (>38°C) every day for 5 days        +At least 4 of the following 5 featuresNon purulent bilateral conjunctivitisCervical lymphadenopathyPolymorphous rashLips/oral mucosa involvementFingers/toes: acute erythema and oedema of palms and soles and then peeling.
Or positive echocardiogram at any time with less than 4 features.
Fevers (>38°C) every day for 3 days+ less than 4 features but diagnosis supported by: Lack of alternative diagnosis (lack to respond to antibiotics, no other pathogen found)High inflammatory markers (high CRP, ESR, NeutrophiliaPresent of other clinical features: Irritability without CNS infectionBCG scar inflammationOther system involvement: CSF pleiocytosis, uveitis, arthritis, gastroenteritis, myocarditis, dysuria, sterile pyuria.

In our particular case: the patient had prolonged fevers with high inflammatory markers (CRP, ESR, Neutrophilia), irritability without CSF infection, sterile pyuria, low albumin, anaemia, thrombocytosis and lack of alternative diagnosis. Furthermore, he had a characteristic echocardiographic finding of Kawasaki Disease.

Patient was started on IVIG and aspirin. Steroids were included in the treatment since the patient already had evolving coronary and or peripheral aneurysm.

For more information on criteria for steroid use in Kawasaki disease, you can read: Eleftheriou D, et al.Managment of Kawasaki disease. Arch Dis Child,99,1 2013 

With regards to the antibiotics, gentamicin was stopped while Piperacillin-tazobactam was continued while evaluating response to IVIG. Piperacillin-tazobactam was stopped after 48 hours. 

Kawasaki disease is rare but early diagnosis is important to avoid cardiological sequelae. Incomplete Kawasaki can present a clinical challenge to diagnose.

You are in an Ethiopian rural hospital. A 7-year-old boy presents to clinic severely malnourished (marasmic type). Mother is complaining of daily fevers for an unknown period of time. 

Patient has cerebral palsy due to an obstructed labour resulting in hypoxic-ischaemic injury. He was in hospital for some time after delivery. He is not vaccinated. He is on phenobarbitone 100mg OD PO for seizures. 

You admit the child to the malnutrition ward and start the appropriate treatment with F-75 Milk. Part of the SAM protocol (Severe Acute Malnutrition) includes a course of at least 7 days with Amoxicillin.  On examination, the patient has a papular rash over hands and groin compatible with scabies but no other clinical findings. On the ward, he spikes a high temperature (39°C) and he is shivering. 

Available investigations at your hospital are performed:

Blood tests: 
Hb 9.1 g/LRenal function and CRP not available in this setting.
WCC 12 x109/L with neutrophils 8 x109/L and lymphocytes 4 x109/LUrine dipstick: leucocytes and nitrates positiveUrine microscopy: many white cells. No culture available.
Platelets 300 x109/LStool: negative for parasites
Blood film: No parasites seen
GGT 61 IU/LHIV antibodies negative
GOT 72 IU/LHepatitis B and C antibodies negative
Bili < 0.5  μmol/L

Based on the above clinical picture and results, what is your differential diagnosis and management? 

Patient was empirically treated but fevers persisted. Given his background of CP and the geographical area, what other infections would you consider?

What other non-infectious causes should be considered? How can you reach the diagnoses in this low-resource-setting?

This is a very challenging patient. Due to their reduced ability to communicate and cognitive impairment, these children are difficult to assess. Furthermore, this patient is malnourished which increases the risk of infections. 

The above results showed a possible UTI which is in keeping with the clinical picture (high fevers, shivering in a patient with high risk of UTI due to his cerebral palsy and poor bladder control). Antibiotics were changed from amoxicillin to amoxicillin-clavulanic to give broader cover for gram negative bacteria (E. Coli, Klebsiella…).

To note, the patient has scabies which is a very common parasitic skin infection that affects mainly the palms and soles and the groin area. If the patient has been scratching over the genital area, it could have triggered a UTI. Furthermore, there are poor hygiene conditions in the area with limited access to water.

The slightly raised GGT and GOT was correlated to the use of phenobarbitone. The hepatitis B and C were negative but the hospital did not have the test for hepatitis A. Nevertheless, the clinical symptoms were not fitting with hepatitis A. 

The patient was treated with co-amoxiclav for 7 days. He initially improved and fevers were spacing in time. However, on day 9 he started again with very high fevers and shivering. He was looking unwell during the fever episodes so he was started on ceftriaxone IV. His baseline temperature was always raised, he had abnormal movements and was irritable. Temperature persisted despite treatment

Another urine sample was requested to rule out a UTI due to a resistant bacterium, since microbiological cultures were not available in the rural hospital. The urine microscopy was negative for WCC and urine dipstick did not show any abnormalities.

Another important differential diagnosis was meningitis. Patient was irritable, had abnormal movements and a fever. The abnormal movements consisted of small twitching of the arms while crying inconsolably. There were considered either shivering or behavioural but there was a lot of discussion if those movements could represent a seizure event. Furthermore, mother was unable to describe the usual seizures that he had at home. The team subsequently realised that there was an error with the regular medications: he was prescribed 100mg of phenobarbitone but mother clarified that at home he was taking 200mg, therefore his daily phenobarbital dose was increased to 200mg OD. To note, the patient did not have any devices (VP shunt) which could increase the risk of infections. In this rural setting, clinicians were not able to perform a lumbar puncture due to lack of laboratory equipment, so the patient was started on ceftriaxone high dose empirically. 

Pneumonia can be a common cause of infection in patients with cerebral palsy since they can have drooling, unsafe swallow prompting for aspiration. Usually, pneumonia in these children can be very silent. In addition, poor nutritional status can increase the risk of severe pneumonia. Patient was not desaturating or with respiratory symptoms but a chest XR was done (in a private clinic) and no lung abnormalities were detected. Furthermore, based on local antimicrobial resistances, the antibiotics he received earlier should have been covered for the most common bacteria causing pneumonia. Gastric aspirate for GeneXpert MTB/Rif was negative. 

Dental infections with abscesses can also present with fever and no other major symptoms. The patient had poor oral hygiene plus the lack of proper tongue movement, drooling and lack of routine dental care made him more prone to these types of infections. These infections are mainly due to anaerobes which should be covered by amoxicillin-clavulanic or ceftriaxone. On examination, no suspicious dental masses were found. 

Viruses can also cause non-specific symptoms. However, they shouldn’t last for very long. He did not have any gastrointestinal symptoms or respiratory symptoms. No palpable lymph-nodes. Unfortunately, in the hospital there were no laboratory diagnoses for viruses.  Full blood count differential was never lymphocytic. 

The most common parasites in this rural area are intestinal parasites (Giardia, Entoaemabeas) and blood parasites (Malaria). Entoaemebas can present with a dysentery which can cause fever. However, our patient did not have any diarrhoea. 

This area has a moderate risk of malaria, especially during the rainy season. Patients with malaria present with very unspecific symptoms: from fever with general malaise or headache and vomiting to seizures, coma and shock. Therefore, any patient with a fever in a tropical setting should prompt investigations for malaria. The most important element in the clinical diagnosis of malaria is a high index of suspicion. 

To reach the laboratory diagnosis, parasites should be seen or detected in blood. Blood film microscopy (thin and thick blood films) is the gold standard for malaria diagnosis, identifies the Plasmodium species and also quantifies the parasitaemia. However, in low resource settings, where microscopy is not always available or reliable, rapid diagnostic tests (RDT) are used to diagnose malaria. The RDTs detect Plasmodium antigens confirming the presence of parasites in the blood but don’t provide any information regarding the species or the parasitaemia. 

Patients with malaria can be classified into severe or non-severe malaria based on clinical and laboratory findings as per the WHO 2015 Malaria Guidelines. This classification is crucial as it will guide treatment. The most important complications of malaria infection in children are cerebral malaria, severe anaemia, respiratory distress due to acidosis and hypoglycaemia. 

Severe Malaria
Clinical findingsLaboratory
-Impaired consciousness/unrousable coma (Glasgow score <11, Blantyre score <3)- More than 2 convulsions in 24 hours- Prostration- Deep breathings/respiratory distress- Shock- Bleeding – Jaundice with parasitaemia > 2% – Severe anaemia with parasitaemia- Acidosis- Hypoglycaemia- Hyperparasitaemia- Haemoglobinuria- Renal impairment

Patients with severe malaria should receive parental antimalarial treatment with Artesunate and supportive management followed by a full course of oral artemisin combination therapy (ACT). Patients with non-severe malaria can be managed with oral antimalarial medication. 

On admission, the initial blood film was negative for haemo-parasites. Repeat blood films and a Malaria RDT (rapid diagnostic test which detects Plasmodium falciparum antigens in blood after 20 minutes) were requested. Repeat Blood film revealed presence of Plasmodium falciparum trophozoites with a parasitaemia of 2%. 

So the patient was diagnosed with Severe Malaria given the suspicion of CNS involvement and started on IV Artesunate. The patient had a good clinical response with resolution of fever and completed a course of oral Artemisin combination treatment (Artemeter Lumefantrine). However, after one week, the fever reappeared. This time, it was a low-grade fever with maximum peaks at 38.5. Repeat blood tests were normal. 

Malignancies: In this case, blood film did not reveal any blasts, chest XR was normal and abdominal ultrasound did not reveal any masses. BMA was not available locally and since the patient was otherwise well, this was not considered necessary. 


Connective tissue disorders: 

SOJIA, AR are very uncommon but still a differential diagnosis of persistent fever. In this setting, no resources were available for auto-antibodies testing, therefore clinical findings are the main way of diagnosing it. Since the patient did not have any rash, arthritis… this diagnosis was not considered. 

Acute Rheumatic Fever (ARF): this condition is quite common in low resource countries due to increased risk of streptococcal tonsillitis due to poor hygiene, overcrowding, poor accessibility to health facilities, fake drugs…  Acute rheumatic fever is an illness caused by an inflammatory reaction to streptococcal infection. It causes an acute, generalised inflammatory response. This illness targets specific parts of the body including the heart, joints, brain and skin. ARF typically leaves no lasting damage to the brain, joints or skin, but can cause persisting heart damage.  Our patient did not meet the Jones’ Criteria of ARF. 

Miscellanea (other possible causes of fever): 

Central origin fever: children affected with cerebral palsy or other neurological disorders relatively often present with chronic intermittent febrile episodes persisting for months. These episodes are not related to any infections but are actually arising from an abnormal thermal regulation resulting from the brain injury.

Hyperthermia from severe dystonia: children with cerebral palsy with dystonia can present with fevers and elevated basal temperature associated with elevated creatinine phosphokinase levels. 

Drug related fever: medications can trigger fevers. Common medications used in cerebral palsy are anticholinergic drugs (e.g. hyoscine) which can provoke unwanted fevers as a side effect. In addition, withdrawal of medications can present with fever (baclofen withdrawal syndrome). 

Lastly, factitious fever is a very challenging diagnosis. Sometimes admissions to hospital and close measurement of fevers plus observation of patient and carer interaction is as important as complementary tests. 

After 2 months of intermittent fever, it finally stopped. Basal temperature was always slightly elevated. Patient was diagnosed with central origin fever.

 The majority of PUOs are caused by: 

A: Malignancy

B: Connective Tissue Disorder 

C: Infections

D: Other diagnosis

E: Unknown diagnosis

The correct answer is C.

Infectious diseases are the main cause of PUO (about 38%), especially in younger children. No diagnosis is reached in 30% of cases but these tend to be benign and self-limited. This is followed by connective tissues disorders (13%) and Other diagnosis (13%). Lastly, malignancies are very uncommon but very important to consider given the severity of the disease.

A patient admitted to your hospital has been spiking fevers every day for 12 days. No other clinical findings are present. What is your next step? 

A: Repeat basic investigations, re-take clinical history, re-examine the patient, perform a Bone marrow aspirate.

B: Repeat basic investigations, re-take clinical history, re-examine the patient and do adequate imaging depending on clinical findings.

C: Perform a PET-CT to localise the pathology.

D: Perform autoimmune studies.

E: Perform a bone marrow aspirate.

The correct answer is B.

In many PUO cases, clinical findings are very subtle and can appear days after the fever. Therefore, re-taking the clinical history and re-examining the patient carefully is key to guide the complementary tests.

A Turkish 5-year-old girl presented with high fevers, profuse night sweating for 21 days.  Clinical detailed history revealed that parents are not consanguineous. She doesn’t have any relevant past medical history. She is fully vaccinated. The whole family was in Turkey for 2 months over the summer holidays. They were living in a farm in rural Turkey where they had goats, cows and chickens. They were drinking fresh milk from the cow. Based on the history, what diagnosis would you consider? 

A: Tuberculosis 

B: Bartonella (Cat-scratch)

C: Brucellosis

D: Toxoplasmosis

E: Lyme disease

The correct answer is B.

Brucellosis is a zoonotic infection caused by ingestion of unpasteurized milk from infected animals. It is also known as the Mediterranean fever. It is caused by a bacterium called Brucella melitensis. The main symptoms are fever, profuse sweating and joint and muscle pain. 

An unaccompanied asylum seeker from Uganda has just arrived in the UK. He refers to being a 12-year-old. He has had fevers for a prolonged time. On examination, he has splenomegaly. Blood tests revealed pancytopenia. Blood film is negative for malaria. HIV and hepatitis B, C negative. He said that in his country many people have these symptoms and they call it Kala-azar. What kind of tropical infection is he referring to? 

A: Visceral Leishmaniasis 

B: Schistosomiasis. 

C: Non falciparum malaria 

D: Visceral Larva Migrans

E: Echinoccocus granulosus

The correct answer is A.

Kala-azar is the local term for Leishmaniasis. This is a parasitic disease spread by the sand-fly. Main symptoms are fever, enlargement of spleen and liver and pancytopenia. Leishmaniasis is the second-largest parasitic killer in the world after malaria. Diagnosis is made by histological finding of amastigotes on spleen aspiration/bone marrow aspiration and RK39 Antigen detection.

A roadmap for fever of unknown origin in children- Rigante, D; Esposito S., International Journal of Immunopathology and Pharmacology. Vol.26 no 2, 315-326 (2013)

Fever in Children and Fever of Unknown Origin- Rajeshwar Dayal, Dipti Agarwal, Indian Journal of Paediatrics, 83 (1): 38-43 (2016)

Pyrexia of unknown origin-Mark Wood, Mario Abinun and Helen Foster. Archives of Disease in Childhood, Education and Practice, 89 ep 63-69 (2004) 

Barbi E, Marzuillo P, Neri E, Naviglio S, Krauss BS. Fever in Children: Pearls and Pitfalls. Children (Basel). 2017;4(9):81. Published 2017 Sep 1. doi:10.3390/children4090081

Antoon J,Peritz D, Parsons M., Skinner A.,Lohr J. Etiology and resource use of fever of unknown origin in Hospitalized children. Hospital Pediatrics, 8 (3).: 135-140(2018)

For malaria: 

https://apps.who.int/iris/bitstream/handle/10665/79317/9789241548526_eng.pdf;jsessionid=AD1DDC86455A8D51D25CFEEADF7E1C75?sequence=1

Website resources: 

https://pedemmorsels.com/fever-of-unknown-origin/

https://dontforgetthebubbles.com/ent-infections-immunodeficiency/

https://dontforgetthebubbles.com/recurrent-or-periodic-fevers-investigate-or-reassure/

https://dontforgetthebubbles.com/tuberculosis/

https://dontforgetthebubbles.com/claire-nourse-tuberculosis-at-dftb17/

https://radiopaedia.org/articles/tuberculous-cervical-lymphadenitis

https://gppaedstips.blogspot.com/search/label/Juvenile%20idiopathic%20arthritis

https://www.paediatricfoam.com/?s=kawasaki

https://gppaedstips.blogspot.com/search?q=kawasaki

https://dontforgetthebubbles.com/josh-francis-rheumatic-heart-disease-at-dftb17/



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The Limping Child Module

Cite this article as:
Team DFTB. The Limping Child Module, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.27391
TopicThe limping child
AuthorHelena Winstanley & Michelle Alisio
DurationUp to 2 hours
Equipment requiredExamination couch (if planning to demonstrate joint examination)
  • 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.

Basic joint anatomy with diagrams of lower limb joints: CHW Anatomy Review

DFTB: Fever and Limp

DFTB: The child with a Limp

Short, basic introduction to the limping child and common differentials:

NHS – Limp in Children

PEM Playbook Podcast on the limping child

A 10 year old boy is brought to ED by his dad who is concerned that his son is limping and has left knee pain. He has complained of pain a few times before – especially after sports (which he dislikes) but seems to be in much more discomfort since coming home from school yesterday.

What are the differential diagnoses in this case?

How would you refine your diagnosis further?

  • The differential is broad at this stage – it includes benign self-limiting conditions such as a minor traumatic soft tissue injury through to more serious conditions such as Perthes disease and Slipped Capital Femoral Epiphysis (SCFE).
  • A detailed history should be elicited to rule out the presence of any red flag symptoms that might suggest systemic illness
    • Night pain
    • Weight loss
    • Night sweats
    • Anorexia/general malaise
  • The child requires a thorough examination including abdomen, groin and the whole of the lower limb to establish the location of the pathology – remember that hip pain can be referred to the knee.
  • X-rays including a pelvis and frog-leg lateral are essential to look for evidence of hip joint integrity.

A thorough history establishes that the child is systemically well but overweight. He has restricted movement in his left hip. You notice that he externally rotates his hip when you try to flex it. This is his x-ray:

  • The x-ray findings suggest a slipped capital femoral epiphysis on the left hand side.
  • It is a Type I Salter Harris injury affecting the growth plate of the upper femur.
  • Triggers include growth spurt (i.e. puberty), obesity and trauma.
  • Initial management involves referral to an orthopaedic specialist
    • Must be kept non-weight bearing
    • Likely to need surgery involve pinning of epiphysis
    • Will need ongoing monitoring due to risk of long term problems e.g.
      • Osteoarthritis
      • Avascular necrosis of the femoral head
      • Slipping of the contralateral side (~20% of cases)

A four year old boy is brought to ED as he is unable to weight-bear. He woke up with a slight limp this morning which has got steadily worse throughout the day and he is now unable to walk at all. He has no recent medical history of note.

Examination is unremarkable other than a moderately restricted range of movement in his right hip. Despite the fact he is systemically well and has had good doses of analgesia he remains unable to weight-bear.

What investigations (if any) would be appropriate at this stage and why?

What is the most likely diagnosis? Can this child go home today? If so, what information would you give to parents and what follow up (if any) would you arrange?

  • X-ray – mandatory to look for evidence of e.g. Perthes
  • Bloods – could be considered as he is still not weight-bearing after analgesia.  
    • He is afebrile and systemically well so will likely be normal
    • May be useful for looking for rare causes of limp e.g. initial presentation of leukaemia
  • US – depending on availability may be useful to look for a joint effusion. In transient synovitis expect to see a simple joint effusion +/- evidence of thickened and inflamed synovium.

X-ray and baseline bloods including inflammatory markers are normal.

  • Most likely to be a transient synovitis
    • Although transient synovitis is more common after a viral illness, it does still occur in children who have been otherwise well recently.
  • As long as you are reasonably confident that this child has transient synovitis it may be possible to discharge him with careful safety-netting advice.
  • If his pain gets worse or he develops a fever his parents should bring him back to ED promptly for further assessment.
  • Some form of review is almost certainly going to be advisable as he is not weight bearing. Depending on local hospital protocol this may be an ED clinic or a paediatric rapid access clinic.

You see a 3 year old child with a two day history of fever. He has woken up with a right sided limp. On examination he is febrile with large, pus covered tonsils, cervical lymphadenopathy and discomfort in the right hip – especially on internal/external rotation. You decide to do bloods and the inflammatory markers come back as raised.

What is the role of tonsillitis in this presentation?

How might you distinguish between a transient synovitis and a septic arthritis in this case?

  • Tonsillitis gives a source for the fever and the raised infection markers
  • It can also act as a source for haematogenous spread of osteomyelitis/septic arthritis
  • Staph aureus is one of the most common causes of both tonsillitis and septic arthritis/osteomyelitis

  • Children with septic arthritis are likely to be in more pain and have a more limited range of movement and are unlikely to weight -bear.
  • They may be systemically unwell.
  • Kocher’s criteria may help clarify your level of suspicion
    • A point is given for each of the four following criteria:
  • Non-weight-bearing on affected side
  • Erythrocyte sedimentation rate >40
  • Fever > 38.5°C
  • White blood cell count >12,000
ScoreLikelihood of septic arthritis
13%
240%
393%
499%
  • Ultrasound of the hip joint may reveal a complicated effusion.
  • The only certain way to know the difference is joint aspiration

A 1 year old girl with a background of sickle cell anaemia attends ED with a history of fevers up to 39°C and diarrhoea for the last two days. Two other family members also have diarrhoea. This morning she is distressed and seems to be in a lot of pain. She is not weight bearing and the family are concerned this could be her first painful crisis.

What are the concerning features in this child – what makes her a high risk patient and why?

What are your next steps in managing this child?

  • Children with sickle cell disease are functionally asplenic due to recurrent micro-infarcts of their spleens.
  • This leaves them at high risk of infection from encapsulated bacteria such as pneumococcus and salmonella.
  • Diarrhoea could leave her dehydrated and provoke a painful crisis – as the family fear.
  • A history of fever and diarrhoea however, must make the clinician suspicious of salmonella infection with the risk of haematogenous spread.
  • She is high risk for systemic bacterial infection so, given her presentation, needs assessing for features of sepsis and shock
  • Meticulous examination of her musculoskeletal system to find a focus for the pain.
  • Initial management is likely to involve fluids, antibiotics and analgesia.

Examination reveals a painful, hot left ankle with some mild swelling. The child screams in pain when you attempt to move it.  

  • Paediatricians
  • Haematologists
  • Radiologists
  • Microbiologists
  • Orthopaedic surgeons
  • Anaesthetists
  • (Critical Care – depending on how unwell she is)

A 10 year old young man, James, with profound learning difficulties presents to the Emergency Department with reduced mobility, a poor appetite and altered behaviour. His mum says he hasn’t fallen or hurt himself recently, but she thinks he is in pain and claims that “my son is not himself”. He is afebrile and systemically well. You notice that James has an abnormal gait as he walks in from the waiting room. You offer him analgesia.

What further information would you like to elicit from the history?

What is a pGALS screen? Practice a pGALS examination with your colleague.

You suspect a hip problem but you are still unsure. What are you going to do next?

Your clinical suspicion and sense of pre-test probability tailors your examination to involve a musculoskeletal and joint exam. With mum’s help, distraction techniques and imaginative play James manages through an incomplete pGALS examination. He doesn’t allow you to complete a comprehensive general examination and mum tells you the last time James had a blood test, the doctor almost got a needle-stick injury.

Despite attempts at a thorough history and examination, you are still unsure what the underlying cause for Jame’s pain is. You cannot safety net and discharge him home without further investigations even though you know taking bloods and performing X-rays will be challenging.

You do bloods and an X-ray which again have been a challenging exercise.

Blood results:
Hb   9.8, WCC  15.6, Plt    299, Na  131, K  haemolysed, Urea  2.4. Creat   15
ESR insufficient
LDH haemolysed

James has presented with atraumatic joint pain.

There is a wide-range of aetiologies but most are benign and self limiting. However nestled among these varied presentations are limb and life-threatening infections and non-accidental injuries. Getting a detailed history from an older child with learning difficulties is going to be challenging, as is getting a history from a preverbal child. One should always approach a scenario with a detailed history; and in this case the main aim would be to tease out red flags with the assistance of the parent/caregiver followed by an examination.

History

A systematic structure to history taking is the SOCRATES pneumonic for a pain focused history.

S: Site. Ask the child where it hurts. Children are more likely than adults to experience referred pain. Remember: knee pain emanates from the hip (in 35% of cases). Also pain from the spine can refer to the lateral thigh.

James rubs his right hip region when you ask him where it hurts.

O: Onset. Is the pain acute or chronic? Acute onset tends to be more concerning and suggests joint/bone infection, trauma, or with acute deterioration of a chronic problem. Chronic pain is more suggestive of an inflammatory process, overuse syndromes or an osseous cause such as SUFE or Legg-Calve-Perthes disease. Malignancy often has a delayed presentation with mild dull pain that may not be activity limiting in the initial stages. Nocturnal pain is a red flag and should always be asked about.

James is unable to answer your questions. Mum thinks James has been unwell since Monday (3 days ago), and says he doesn’t have problems sleeping.

C: Character. Dull ache (deep tissue pathology) or sharp sting (cutaneous involvement) can be helpful.

James is unable to answer both questions to character and radiation.

R: Radiation. Can be difficult to elicit exact origin of pain and radiation, especially in the younger child. Be aware of bilateral limb involvement as this may suggest a more central cause, such as cauda equina.

A: Associations.

Weight-bearing: non-weightbearing can suggest more serious pathology

Mono or polyarticular: polyarticular is generally less concerning and suggests an underlying systemic disease process. However, 8% of septic arthritis cases involve more than one joint and leukaemic infiltrates typically affect multiple joints.

Systemic illness: chronic features such as malaise, fatigue, weight loss could suggest underlying increase in catabolism from a systemic disease such as SLE, JIA, or anaemia from malignancy.

Dermatology: extra-articular features of inflammatory bowel disease (IBD) (eye pain, pyoderma gangrenosum, erythema nodosum, aphthous ulcers). Ask the parents about any new marks on the body and later during the examination look for a Salmon patch of juvenile idiopathic arthritis (JIA). Consider whether this presentation is associated with a recent upper respiratory infection or viral gastroenteritis which may be suggestive of reactive arthritis.

Mum says James is off his food but he hasn’t lost weight.

T: Time course. Note the pain progression over time and response to analgesics.

Mum says James is more comfortable after Ibuprofen which she started giving him 2 days ago.

E: Exacerbating or Relieving factors. Trauma and infection aggravate pain after activity and are relieved by rest. The presence of pain that is relieved by activity is more suggestive of an underlying inflammatory cause. Pain relieved with rest is pain from osteochondrosis in the adolescent experiencing growth spurts.

S: Severity. Severity is subjective but a surrogate of severity is the inability to walk or to tolerate examination with distraction.

Finish off the SOCRATES pain history with a FAST history:

F: Family history for autoimmune disease (IBD, psoriatic arthritis)

A: Adolescent screen. The sexually active teenager could present with joint infection due to gonococcus or joint inflammation due to chlamydia. Similarly ask about IV drug use as this increases the chance for infectious arthritis.

S: Safeguarding. Due attention should be maintained for a history that is not in keeping with the presentation or the child’s developmental age.

T: Travel history

Examination

Look. Inspection is non-invasive, interactive and can be used to establish rapport.

Feel for temperature changes, tender areas, effusions.

Move actively and passively with the aim of gently stressing the ligaments of which the joint is comprised.

It is always important to complete a comprehensive general examination especially in the presence of red flags or suspicion of a multisystem disease.

pGALS is a standardised musculoskeletal (MSK) basic examination. Free educational resources to demonstrate pGALS are available online. (www.pmmonline.org).

pGALS screen has the benefit of quickly assessing all joints, this is important given the sometimes vague presentation of MSK problems and the difficulty of localising the site of joint pathology from the history alone. Frequent practice of pGALS, especially on healthy children, is important to appreciate normal ranges of movement as joint ‘restriction’ – a common finding in JIA can be easily missed especially with symmetrical joint disease.

The x-ray is diagnostic and demonstrates a large pelvic Ewing’s sarcoma.

The blood tests (which were very difficult to obtain from James) reveal an anaemia and leukocytosis. A marginally elevated ESR and LDH may also be evident in Ewing’s sarcoma.

Ewing’s sarcoma typically presents with more systemic disease at presentation and localised pain. At diagnosis, the median duration of symptoms is 3-9months.

Any child or adolescent who’s pain involves any of these characteristics should be investigated to exclude a malignant pathology with at least a plain film X-ray.

–        A painful ‘injury’ which fails to resolve over a reasonable time (over 1 month)

–        Intermittent or persistent localised pain for one month with no history of trauma

–        Night pain that wakes you up from sleep or prevents adequate sleep

–        Fracture where history of force resulting in injury seems insufficient

Ewing’s sarcoma occur more commonly in the pelvic bones, diaphysis of the long bones of the leg, and bones of the chest wall.

At presentation 25% will have metastatic disease to the lung, bone or bone marrow or a combination of these.

Plain X-ray in 2 planes of the painful area remains the first line investigation of choice and is readily available to most clinicians. Ewing’s (as well as an osteosarcoma) can lead to a radiologically significant sign – the Codman triangle. It is a triangular area of new subperiosteal bone that is created when a lesion, often a tumor, raises the periosteum away from the bone. Ewing’s sarcoma traditionally demonstrates osteolysis, detachment of the periosteum and occasional calcification in any associated soft tissue mass.

There are no peripheral blood tests or tumor markers which are diagnostic of bone tumors.

Further imaging of the affected bone is required to adequately determine the true extent of the disease and staging.

Biopsy is the gold standard for histological diagnosis.

Whole body technetium-99m bone scans and CT chest are performed to assess metastatic disease at presentation.

Bone marrow aspirates and trephines are required for Ewing’s sarcoma patients as marrow involvement is a part of the disease process and for some high dose procedures requiring harvest of stem cells from the patient may be part of the treatment.

Referrals to a specialist orthopaedic sarcoma surgeon, paediatric oncologist, radiology services, paediatric surgeon, rehabilitative specialists and physiotherapists to name a few are required.

Important to note that although malignancy is rare, it should be actively excluded. Also, blood tests may be normal even in cases of inflammatory, infectious and neoplastic joint pain and peripheral blood films may not show blast cells in children with leukaemia cells.

Which of the following is NOT part of Kocher’s criteria for assessing the risk of septic arthritis in a limping child?

A: ESR >40

B: Range of movement <50% normal

C: WCC > 12×109

D: Fever >38.5oC

E: Inability to weight bear

The correct answer is B.

Although range of movement can be a useful part of the assessment, it is not part of the official Kocher criteria.

Which of these statements about SCFE/SUFE is TRUE?

A: It is most common in underweight children between the ages of 6-10. It can be a cause of chronic hip pain and is bilateral in approximately 10% of cases.

B: It is a cause of both acute and chronic hip pain, is rarely bilateral and typically occurs in overweight adolescents.

C: It is caused by avascular necrosis of the femoral epiphysis and usually presents as a chronic problem. Radiological detection is improved by requesting a ‘frog-leg lateral’ view.

D: It is most common in children over the age of 10 and can present with acute or chronic pain. It is bilateral in around 20% of cases and radiological detection is improved by a ‘frog-leg lateral’ view.

E: It is a common cause of limp and pain in underweight adolescents and is bilateral in one third of cases. Early detection and referral improves prognosis.

SCFE is most common in boys between the ages of 10-17 with a median age of 13. It is the most common cause of serious hip pathology in adolescents. 50% of them are >95th centile for weight and knee pain is a common initial presentation. Pain and limp may be entirely acute but often presents with an acute on chronic picture with long term low level pain suddenly becoming more acute with a large epiphyseal slip

In a classical antalgic gait, which phase of walking is affected?

A: Strike

B: Contact

C: Stance

D: Propulsion

E: Swing

The correct answer is C.

The stance phase is shortened in an antalgic gait as the patient tries to minimise the amount of time that weight is placed through the painful leg. (https://pemplaybook.org/podcast/please-just-stop-limping/ for diagram of phases of gait and fuller explanation)

Which of the following bacteria is the most likely to cause septic arthritis?

A: E. Coli

B: Streptococcus pneumoniae

C: Haemophilus Influenza B

D: Staphylococcus aureus

E: Salmonella

The correct answer is D.

Although all of the organisms above can cause joint pathology, the most common is Staph aureus. Previous studies suggest it is responsible for over half of all cases of septic arthritis.

Which of the following statements about Perthes disease is FALSE?

A: It is more common in boys than girls

B: It is bilateral in 10% of cases

C: It is defined by avascular necrosis of the femoral head

D: An older age at diagnosis is associated with a more favourable prognosis

E: Physiotherapy, casting and surgery may all form part of the treatment regimen

The correct answer is D.

Perthes disease is five times more common in boys than girls although damage is often more severe in affected girls. It may be bilateral in around 10% of cases and is defined by avascular necrosis of the femoral head. The younger a child is diagnosed the more favourable the prognosis owing to a greater potential for bone remodelling. Children under the age of 6 do especially well. Management is multidisciplinary and physio, casting and surgery may contribute at various stages of the disease process.

A 10 year old presents with an acute onset of right hip and wrist pain following a very minor trip and fall onto the ground. Since the incident she has been unable to bear weight. On examination the right hip and wrist showed no obvious deformity and range of movement were normal after her pain was addressed. During the last 2 years she had not presented to the GP but recently she had complained of poor sleep, leading to daytime somnolence, palpitations, anxiety and sweating. A fall in her weight growth curve was noted after plotting her on a growth chart.

Which statements are FALSE?

A: This is a ‘red herring’ injury commonly reported by patients and their parents and will not require further investigations.

B: For children over the age of 8 years or limping > 7 days an X-ray is an adequate imaging modality as it has a relatively low dose of radiation but is also very likely to show the pathology causing the pain.

C: Blood tests are indicated including thyroid functions because the minor fall is not in proportion to the severity of her symptoms and her unexplained weight loss is of concern

D: Transient synovitis is most common in this age group so cooling, rest and pain relief are adequate therapy.

The correct answers are A and D.

The most common cause for hip pain (in children aged 3-10years) is transient synovitis. Even though this is a benign cause not requiring further treatment, other more severe differential diagnoses need to be considered. A ‘red herring’ injury is commonly reported by patients and their parents but in this case the minor fall was not in proportion to the severity of her symptoms. Similarly, X-ray and blood tests including thyroid functions are indicated due to the red flags of not weight bearing, disproportionate severity of her pain and unexplained weight loss. The indications for a CT scan are limited in this context. In this case, she had a  pathological fracture due to endocrine abnormalities. Hyperthyroidism is rare but a severe disease in children and mostly autoimmune.



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