If a third fluid bolus is being considered then it is reasonable to start dopamine.

This
can initially be given through a peripheral line whilst central access is obtained.

A - Permissive hypotension may be used in trauma to minimise haemorrhage,

however this is not a problem in sepsis.

B – Packed cells would be more appropriate in the trauma setting and not in sepsis.

C – Sepsis has resulted in pulmonary oedema which has been exacerbated by the fluid
boluses already given. Inotrope use is indicated, although a further fluid bolus may be
required whilst drawing up the dopamine.

E – Further inotropes such as Dobutamine or Adrenaline may be required if

Dopamine alone has little benefit, however these ideally require central access and so
Dopamine is used first.

Ref: APLS 5th edition

Fanconi anaemia is a rare autosomal recessive condition.

Features and complications:

 AML
 Aplastic anaemia
 Cardiac
 Deafness
 GI and kidney malformations
 Hypogonadism
 Skeletal abnormalities (triangular shaped head, radius and thumb
dysplasia/aplasia)
 Solid tumours (especially head and neck, and gynaecological)

Abetalipoproteinaemia is a rare autosomal recessive lipoprotein synthesis

disorder.

Clinical features:

 Ataxia
 Failure to thrive
 Reinopathy (retinitis pigmentosa)
 Steatorrhoea
Investigations:

 FBC (acanthyocytes)
 raised ALT
 lowered LDL
 lowered VLDL
 lowered Cholesterol
 lowered Triglycerides.

This is a case of acute otitis media (AOM), a middle ear infection, which is usually
self-limiting and antibiotics are not required initially. The patient should be kept as
comfortable as possible with analgesia and antipyretics and generally it is only if
symptoms persist beyond 2 - 3 days that antibiotics may be considered necessary.

Causes of AOM may be viral (RVS, rhinovirus) or bacterial for example

streptococcus pneumoniae) and a bacterial infection may often follow a viral
infection.

Surgical treatments may include tympanocentesis and myringotomy for severe or

recurrent infections.

A 12-year-old boy presents to your emergency department with evidence of a C-spine

injury; he is alert and complaining of pain in his neck. His head has been immobilised
in the midline, by the paramedic crew.

Which of the below mechanisms would make you obtain a CT C-spine within an
hour of arrival?

Your answer was correct

Axial load to head

Ejection from a car
Focal peripheral neurological signs
Quadbike accident
Rollover motor vehicle accident

Explanation
NICE recommends that if a child has sustained a head injury and has focal peripheral
neurological signs, then a head CT within one hour is required. The criteria aims to be
robust so as to avoid unnecessary radiation doses to the thyroid gland.

A – This may be obtained from a diving mechanism. If there are no other risk factors
for CT, then this requires 3 view C-Spine X-rays within an hour.

B – Being thrown out of a motor vehicle is an indication for C-spine X-rays assuming
there are no risk factors for CT present.

D – Accidents involving motorised recreational vehicles such as these, require C-

spine X-rays assuming there are no risk factors for CT present.

E - If there are no other risks factors for CT, then this requires 3 view C-Spine X-rays
within an hour.

Ref:

[Link]

If the parents are unmarried then the father has no legal parental responsibility.
Although the law was changed for births registered in England & Wales from 1st
December 2003 to give parental rights to non-married fathers who appear on the birth
certificate, this will not apply retrospectively – i.e. if a child was registered before 1st
December 2003 the father has no parental responsibility if not married to the mother.
(Please note that different rules may apply in Scotland and N. Ireland see the
following website for details:
[Link]

Therefore, only the mother can consent to procedures on the child. The child can
consent for him or herself once ‘competent’. It is very unlikely that even a 15-year-
old child would be competent to consent to an operation (appreciating the risks of
anaesthesia). An important point is that even if a child can consent to a procedure
when competent (if under 16), legally they cannot refuse one if the person with
parental responsibility consents.

The management of emergencies is essential for exams such as these.

Features of a moderate asthma attack in children older than 2 include:

 Ability to talk in sentences

 SpO2 ≥92%
  PEF ≥50% best or predicted
 Heart rate ≤140/min in children aged 2–5 years, ≤125/min in children >5 years
 Respiratory rate ≤40/min in children aged 2–5 years, ≤30/min in children >5
years

Bupivacaine is a local anaesthetic with a longer duration of action than lidocaine. It is

most often used for spinal anaesthesia. It is not part of anaesthetic induction.

Ketamine, Etomidate, Propofol, Thiopental (thiopentone) and sedative drugs such as

midazolam, fentanyl and morphine are the most common anaesthetic induction
[Link] oxygenation must also be ensured throughout.

Clinical features of Lyme disease:

 Greater risk of transmission with tick feeding >24 hours

Early stages:

 Erythema chronicum migrans (75% occur up to one month after tick bite)
 fever
 malaise
 muscle and joint pains

Complications:

 Affective disorders
 Arthritis
 Cardiac arrhythmias, Carditis
 Iritis
 Meningo-encephalitis, Polyneuropathy
 Sleep disturbance
 Femoral nerve block is a safe and very effective method of pain relief for limb injuries. The
other options are, of course, useful but probably not sufficiently strong for this severity of
injury. Splinting is vital but analgesia should be given first. Intravenous morphine should be
used with caution if there is the possibility of a significant head injury

Acute Disseminated Encephalomyelitis (ADEM):

Epidemiology: Children>adults.

Aetiology/risk factors: 1 to 20 days post viral infection. Can also occur post
vaccination (measles, mumps, rubella).

Clinical features: Acute onset CNS inflammation, Ataxia, Fatigue, Headache,

Nausea Phyramidal signs, Weakness.

Complications: Bowel and bladder involvement, Brown-Séquard syndrome,

Coma, Deafness, Devics syndrome (optic neuritis and transverse myelitis),
Hemiparesis, Optic neuritis, Permanent disability, Seizure, Transverse myelitis.

A brain MRI shows demyelination.

Foetal alcohol syndrome

Epidemiology: Common

Aetiology/risk factors: Excess alcohol consumption during pregnancy (dose

dependent).

Clinical features and complications:

 Acute neonatal alcohol withdrawal

 ASD
 Ataxia
 Epicanthic folds
 IUGR
 Learning disabilities
 Microcephaly
 Narrow palpebral fissures
 Neonatal hypotonia
  PDA
 Ptosis
 Seizures
 Skeletal deformities
 Narrow philtrum
 Thin superior lip
 Urinary tract deformities
 VSD

Hypothermia is common with drowning episodes. Whilst the temperature is < 30

degrees the chances of converting an arrhythmia to a normal sinus rhythm is poor.
Three shocks should be tried and if unsuccessful further shocks should only be given
once the core temperature is > 30 degrees.

A - Drugs should be avoided at < 30 degrees, however 3 shocks can be attempted.

C - Shocks remain at the same frequency as normal, if they are indicated. The interval
between drug doses is doubled for a temperature between 30-35 degrees.

D – 32 degrees is the temperature at which a patient should be warmed to before

stopping resuscitation attempts.

E – Above 35 degrees shocks and drugs can be given as per normal.

Ref: APLS 5th edition

Below 16 years of age, consent of a parent or guardian is required, unless emergency

treatment is necessary (when consent from a person in loco parentis is also
unnecessary) or the child has given consent and the doctor considers that the child is
of sufficient understanding to make an informed decision about medical care
(including oral contraception) and the child refuses to allow the parents to be asked.
The circumstances in which this may occur are very limited as the bar for considering
a child understands the implications of the treatment has been set, by the Courts,
deliberately high.
If a child aged under 16 years refuses surgery it can still be carried out with
appropriate consent, if the doctor believes the child doesn't have sufficient
understanding to make an informed decision or if a court has considered the child's
objection and told the doctor to proceed.

A mother and father have equal parental responsibilities for their legitimate child. If
parents disagree it is probably inappropriate to proceed, although only one parent is
needed for consent. An unmarried father, not named on the birth certificate, has no
intrinsic rights.

You are called to the emergency department as the paediatric SHO on call. A baby
has just been born in the back of the ambulance and is not breathing with a heart rate
of 40. On arrival in resus the baby is placed on a warm resuscitaire and dried.

What is your next step in management?

Your answer was incorrect

Open airway to the neutral position and give 5 inflation breaths

Open airway to the sniffing position and give 5 inflation breaths
Start CPR at a ratio of 3 compressions to 1 breath
Start CPR at a ratio of 15 compressions to 2 breaths
Start CPR at a ratio of 30 compressions to 2 breaths

Explanation
Neonates have large occiputs which can result in their neck flexing and obscuring
their airway. Placing their head in the neutral position opens the airway. As the
majority of neonatal arrests are from respiratory causes, giving 5 inflation breaths
(prolonged breaths over 3 seconds as opposed to faster ventilation breaths) may be
enough to oxygenate blood entering the heart to increase contractility, if not CPR at a
ratio of 3:1 may be required.

B - The sniffing position is used in children above 28 days of life, to open the airway.
In children, rescue breaths are given rather than inflation breaths.

C – This is the correct ratio to use in neonates, however as respiratory causes

predominate in neonatal arrests, inflation breaths are given first through an open
airway. If the Heart rate remains <60 and unresponsive to breaths, CPR may be
started.
D – This ratio is used for children over 28 days of life.

E – This ratio is used in adults and not for neonates or children.

Ref: APLS (6th ed) and NLS (3rd ed)

Dietary advice is always an integral part of the management of constipation. A diet with
adequate fibre, fruit and vegetables, and fluid intake should be encouraged. Sometimes
Movicol (now first line drug treatment for chronic constipation as per NICE guidelines) will
help soften the stools to allow a normal bowel habit to be re-established. Senna would also
.achieve this but can cause stomach cramps and less compliance

Cyclizine works on the medulla oblongata. The other anti-emetics work by different
mechanisms. Cyclizine has anti-muscarinic properties which may cause urinary retention. It
also has sedating anti-histaminic effects. It starts to work in around 30 mins of
.administration and peaks at around 2 hours

There has been a history of trauma and therefore a fracture is a possibility. Toddlers
fractures are undisplaced fractures to the tibia. They occur due to twisting injuries, which
may be relatively minor. The child will be reluctant to weight bear and may have pain over
.the site of the fracture on palpation

The history and examination are compatible with chronic fatigue syndrome (CFS), also
known generally as myalgic encephalomyelitis (ME) or postviral fatigue syndrome. The only
 treatment for which there is some evidence base is graded mobilisation and physiotherapy.
Increased rest is of no proven value and does not improve mobilisation and exercise
tolerance. Home tuition may be advised, but the primary aim is gradual reintegration into
school. Psychological input may be useful but is often resisted by parents who perceive this
as primarily a medical/organic problem. Antidepressants are mainly indicated for an
.associated clinical depression

VSDs are the most common congenital cardiac defect, the second most common
being bicuspid aortic valve. 40% of children with congenital cardiac disease will have
a VSD. Between 70-80% of cases affect the membranous septum. Large defects can
increase pulmonary vascular resistance and eventually lead to a right to left shunt and
Eisenmenger's syndrome.

90% of ventricular septal defects close spontaneously by one year of age

The following article offers further reading around the topic:

[Link]

The C-spine cannot be cleared, this alongside a multi-trauma presentation means a CT

C-spine is indicated rather than X-rays alone. The imaging modality for blunt trauma
to the Chestis CXR, if this shows significant thoracic trauma,a CT chest should be
considered. These recommendations are from the Royal College of Radiologists and
based on injury types in children and trying to maintain as low as reasonably
achievable (ALARA) radiation doses. (1)

A – This child is at risk of C-Spine injury following the mechanism of trauma. He had
a reduced GCS, has undergone a multi-region trauma and is now intubated. NICE
Head injury guidelines therefore recommend using CT. (2)
 Any child who has taken a deliberate overdose, even if no medical treatment is required,
must be seen by a child psychiatrist. It is considered best practice not to discharge until
assessed safe to do so by a child and adolescent psychiatrist. This means that most will be
admitted until assessed, although some may be seen in the emergency department
.depending on the level of psychiatric service provided

Vigorous fluid boluses may result in dislodging early clots and diluting coagulation
factors, exacerbating major haemorrhage. Permissive hypotension is used to prevent
this.

A – Major haemorrhage protocols ensure that blood products are given at the optimal
ratios in a timely manner.

B – Pelvic binders stabilise a fractured pelvis and apply pressure to minimise

bleeding.

C – Use of tourniquets in major arterial bleeds may be life saving.

D – Tranexamic acid is an anti-fibrinolytic drug that is given at a dose of 15mk/kg. A

loading dose is given followed by an infusion.

Ref: APLS 2015; ATLS 9th edition

A star chart for dry nights is a useful tool for managing nocturnal enuresis. Simple
measures such as minimising fluid intake before bed and ensuring the child goes to the
toilet before going to bed are important. Medical interventions are not a good first-line
measure; desmopressin is effective but there is a high relapse rate off treatment.
Imipramine is infrequently used now because there are more effective treatments and it
has serious side-effects. Enuresis clinics provide good support but the majority do not
.accept referrals for children under 7 years old
Sepsis is a condition that is easily missed, particularly in the early stages.

Initial screening for sepsis criteria:

 Core temp <36°C or > 38.5°C

 Inappropriate tachycardia (or bradycardia) for age
 Altered mental status (including sleeping/ irritability/ lethargy/ floppiness
 Reduced peripheral perfusion/ prolonged capillary refill /reduced urine output
or wet nappies

ou know that as he has >10% burns to his body, he will require additional fluids on
top of his maintenance.

Which formula below is correct in calculating the additional fluid?

Percentage burn x weight (kg) x 4

Explanation
This is the amount required in addition to maintenance IV fluids. Half of this will be
given in the first 8 hours. It is a guide to a starting rate, subsequent fluid rates will be
guided by urine output, which needs to be kept at >2ml/kg/hr.

Ref: APLS 5th Edition; ATLS 9th Edition

This 5-year-old had a persistent cough that was characteristically nocturnal, accompanied by
vomiting and with no evidence of any diurnal respiratory symptoms. The cough is related to
the supine posture, especially as there was accompanying vomiting, which was a constant
feature. Therefore in the first instance, the least invasive and most useful investigation is an
ambulatory oesophageal pH study. A barium meal involves radiation, is not physiological and
is indicated only if the possibility of a hiatus hernia is considered. A bronchoscopy and CT
may be indicated if the initial investigations are negative
Over 10% of children experience non-organic pain (abdominal, headache, limbs) and
it is important to exclude organic pathology quickly and to uncover any underlying
stresses.

Non-organic abdominal pain in children typically peaks around the age of 9-10 years.
It can have a diverse frequency of occurrence - from daily to once a month. It is often
peri-umbilical in position and does not radiate. It rarely wakes the child from sleep or
is associated with eating. Episodes can be brought about by stressful times such as
moving house/changing schools.

If there is blood in the stools, an alternative diagnosis should be sought

In APLS teaching, if a BSL is <3 a glucose bolus of 2ml/kg 10% is indicated. This
situation is different for diabetic patients and for neonates. Local guidelines may also
vary.

A - <2.6is the value that defines neonatal hypoglycaemia.

C - <3.5 this level is too high in the absence of diabetes.

D - <4is the value for hypoglycaemia in patients with a background of diabetes.

E - <5 is too high a threshold.

Ref: APLS 5th Edition

The commonest cause of hiccoughs in terminally ill patients is gastric distension.

Gastric reflux, diaphragmatic irritation and renal failure may also be contributory.
William's syndrome

Epidemiology: Rare

Aetiology/risk factors: Genetic/Often sporadic

Clinical features:

 Clinodactyly
 Dental anomalies
 Epicanthic folds
 Flat nasal bridge
 Learning difficulties
 Pectus excavatum

Complications:

 Attention deficit
 Hypercalcaemia
 Hypertension
 Pulmonary artery stenosis
 Pulmonary stenosis
 Supravalvular aortic valve stenosis

The National Institute for Clinical Excellence (NICE) have produced national guidelines for the
management of head injuries. They are a little more complicated in children for a number of
reasons. Children tend to vomit after injury; this may not be a result of a significant brain
injury but a response to the injury. CT (investigation of choice for significant head injuries)
requires a degree of co-operation and lying still, which may be difficult for younger children
(< 5 years). In this patient, where there was loss of consciousness and some post-event
vomiting, a CT scan may be indicated. However, as the child is 3 years old this may be
difficult unless it is performed under general anaesthetic. In these patients admission for
neurological observation is preferable; if there is any decrease in GCS or development of
.focal neurological signs then a CT is definitely indicated
 Around 7% of congenital heart defects are secundum ASDs. 70% of those who have a
secundum ASD are female. It may form part of a Holt-Oram syndrome which includes an ASD
.(or VSD) and triphalangeal thumbs

Remember there may not always be a sinister pathology going on! Childhood absence
epilepsy (petit mal) may have the same presentation. However, in an absence, the child will
not blink on confrontation (e.g. wave of hand in front of eyes). True absences can occur in
.any setting. They can be precipitated by hyperventilation

The description of salaam attacks together with regression of acquired skills should
lead you to the diagnosis of infantile spasms. Infantile spasms are a severe epileptic
encephalopathy, presenting normally between 3 months to 1 year. They are
characterised by runs of flexion and extension spasms. Often associated with a
peculiar cry and mistaken initially for colic. They are important not to miss and to
initiate treatment as soon as possible because development ceases with the onset of
spasms.

The child had a reduced GCS at the scene suggesting head injury. Cushing’s triad for
RICP is: hypertension, bradycardia and irregular breathing. This child is intubated and
so breathing cannot be assessed however with the mechanism of injury, reduced GCS
and evidence of a blown pupil (from herniation compressing the parasympathetic
fibres of the third nerve); RICP is the most likely diagnosis.

A – Hypovolaemia from massive blood loss would be associated with a tachycardia

and hypotension.

B – Neurogenic shock would be suggested by a flaccid paralysis with hypotension

and bradycardia secondary to loss of sympathetic innervation.

C – Local trauma to the eye can cause a fixed pupil, however does not explain the
other signs seen in this case.
E – Traumatic arrhythmia may occur following blunt chest trauma, however does not
explain all the signs as seen in the case above.

Ref: ATLS 9th edition

A 2-month-old baby girl is brought to your surgery as her mother is concerned

about a 1 cm lump situated lateral to her right eyebrow. The lump is firm and
not attached to the skin. Which of the following lumps is the most likely
diagnosis?

External angular dermoid

Explanation
External angular dermoid is by far the most likely diagnosis. External angular
dermoids (dermoid cysts) are embryological remnants that contain dermal and
epidermal tissues. The site of the lump means that the other options are much less
likely. Neurofibromas are uncommon in children this young

NICE recommend CT head scan within 1 hour for children with more than 1 of the
following risk factors:

 Loss of consciousness lasting more than 5 minutes (witnessed)

 Abnormal drowsiness
 Three or more discrete episodes of vomiting
 Dangerous mechanism of injury (high-speed road traffic accident either as
pedestrian, cyclist or vehicle occupant, fall from a height of greater than 3
metres, high-speed injury from a projectile or other object)
 Amnesia (antegrade or retrograde) lasting more than 5 minute

This 6-year-old child has had 3-4 discrete episodes of vomiting and is abnormally
drowsy, hence requires CT head to exclude intracranial bleed requiring neurosurgical
intervention.

A is incorrect as the child requires CT head as the first step prior to a period of
observation.
B is incorrect as the GCS is 14/15 with no history of deteriorating conscious level, the
observations are normal, and no airway problems have been identified; anaesthetic
input is not required prior to CT head.

C is incorrect as there are not currently any clinical features mandating immediate
neurosurgical input, defined by NICE as:

 New, surgically significant abnormalities on imaging.

 Persisting coma (GCS 8 or less) after initial resuscitation.
 Unexplained confusion which persists for more than 4 hours.
 Deterioration in GCS score after admission (greater attention should be paid to
motor response deterioration).
 Progressive focal neurological signs.
 A seizure without full recovery.
 Definite or suspected penetrating injury.
 A cerebrospinal fluid leak.

E is incorrect as there are no signs of raised intracranial pressure except GCS 14/15
(which is more likely explained by concussion than raised intracranial pressure).

Reference: [Link]

What proportion of patients with Tetralogy of Fallot have Di George syndrome?

15%

Di George syndrome affects around 1 in 4000 live births and is caused by a 22q11.2
micro-deletion.

It has a number of potential features including T-cell dysfunction, thymus

maldevelopment, neonatal seizures due to hypocalcaemia, failure to thrive, low-set
ears, orbital hypertelorism, cleft palate and cardiac defects

The underlying principle of the Fontan operation is to establish systemic venous return to
the pulmonary artery without requiring a sub-pulmonary ventricle. Ultimately, the Fontan
.circulation is where one ventricle supports both the systemic and pulmonary circulations
This boy is showing abnormal decorticate posturing suggestive that he has had a
major head injury.

Decorticate posture can be remembered as the ‘mummy posture’ as it resembles

mummys from ancient tombs. It is caused by brain lesions above the red nucleus
impacting the rubriospinal and corticospinal tracts damaging the motor neurone
pathways and muscular control.

Decerebrate posture, also known as extensor posturing is evidenced by arms and legs
straight, toes pointing downward, head and neck arched backwards. Teeth may be
clenched. This occurs as the brain stem is damaged, specifically below the red
nucleus. Progression from decorticate (arms bent) to decerebrate (arms straight) often
occurs during tonsillar brain herniation.

Opisthotonus posturing is evidenced by a rigid and arching back and head thrown
backwards. It may also be seen in brain injury, tetanus and after drowning. It is an
extrapyramidal effect caused by spasm of the axial muscles along the spinal column.

Decorticate, decerebrate and opisthotonic posturing all suggest brain injury and the
patient requires immediate attention. A patient may fluctuate between posturing forms
or show decorticate on one side and decerebrate posturing on the other.

Posturing occurs as the brain is injured and muscle groups act without opposition.

All of the above have been reported in children with cerebral malaria.

Opsoclonus– Myoclonus / Opsoclonus-Myoclonus Syndrome (OMS) is a rare

neurological disorder thought to be autoimmune in origin. It affects 2-3% of children
with neuroblastoma. Symptoms include opsoclonus ‘dancing eyes’ with unpredictable
rapid conjugate eye movements, myoclonus (muscular twitching), cerebellar ataxia,
speech problems, drooling, vomiting and lethargy.

APLS (Advanced Paediatric Life Support) recommendations indicate that tibial interosseus
needle insertion is a quick and easy method of securing venous access in an arrested child.
The other methods are satisfactory but in a collapsed child, may be difficult and time-
.consuming to insert
 The only medical indication for circumcision is balanitis xeroderma obliterans (BXO). A non-
retractile foreskin is not uncommon until puberty. The adhesions may be released with some
.weak steroid cream. Ballooning of the foreskin is not a problem in itself

Tuberous sclerosis – autosomal dominant, 1 in 6,000:

 ‘Ash leaf’ macules (from infancy): depigmented lesions approximately 1–2

cm long
 ‘Shagreen’ patches (from 2 years): areas of roughened skin, usually sacral,
likened to shark skin
 Adenoma sebaceum (from 5 years): 1- to 2-mm papules, usually facial
(butterfly distribution)
 Epilepsy (usually before 2 years)

Neurofibromatosis type 1 – autosomal dominant, 1 in 3,500:

 Café-au-lait spots (> 2 in children under 5 years, > 5 in children over 5 years is
significant)
 Axillary freckling
 Neurofibromata (from 12 years): papules anywhere on the body
 Epilepsy only in 10%

Ataxia telangiectasia – autosomal recessive, a chromosomal repair defect. Affected

children present as late walkers. Ataxia develops in early childhood and is
progressive:

 Conjunctival telangiectasia: develops from 5 years

 Incontinentia pigmenti: X-linked dominant.
 Vesicular stage: neonatal period, linear distribution; resolves by 1 month
 Verrucose stage: 1–4 months, warty lesions appearing mainly on limbs;
resolves by 6 months
 Whorl stage: by 2 years, linear and whorl pattern of hyperpigmentation on
limbs
 Epilepsy in over 30%

Sturge–Weber syndrome – sporadic, 1 in 50,000:

 Naevus in trigeminal distribution with an ipsilateral leptomeningeal

haemangioma
 Intracranial calcification is common, especially in the occipital region
 Seizures develop in early childhood