Gen Paeds, ED

Question 1

Crying/Colic - bg

Answer 1

1. Background
   a. Crying is normal physiological behaviour in young infants
   b. Crying starts at 2 weeks of age – peaks at 2 months of age – resolves at 4-5 months
   i. 6 - 8 weeks age = baby cries on average 2 - 3 per 24 hours
   c. Excessive crying is defined as crying >3 hours/day for >3 days/week (Wessel’s criteria)  termed colic (outdated)
   i. Excessive crying of unknown cause
   ii. Infants <3 months
   d. Infants with colic are well and thriving
   e. There is usually no identifiable medical problem
   f. Associated adverse outcomes
   i. Increased risk of PND
   ii. Early weaning
   iii. Multiple formula changes
   iv. Antireflux problems and OTC medication
   v. Shaken baby syndrome
2. Infant sleep
   a. Average sleep requirements
   i. At birth: 16 hours
   ii. At 2 - 3 months: 15 hours
   iii. A 6 week-old baby generally becomes tired after being awake for 1.5 hours
   iv. A 3 month-old baby generally becomes tired after being awake for 2 hours
   b. “Usual” sleep pattersn
   i. First week home = sleep  feed  sleep  feed
   ii. More night sleep from 3 weeks
   iii. Consolidation by 12 weeks – ‘sleeping through’ ie block of sleep lasting 8 hours median age 3 months
   iv. Normal to feed overnight until 6 months of age
   c. Infants sleep cycle
   i. 20-40 minute sleep cycle (vs. 90 minutes in adults)
   ii. How you fall asleep is how you want to go back to sleep – sleep association eg. feeding, rocked, dummy
   iii. Babies have more REM sleep (cf. adults) – sleep is more restless
   d. Infant sleep problems
   i. Night waking, difficulty sleeping, or both – affect 30-45%
   ii. Associated outcomes
3. Double-triple risk of PND
4. Poorer maternal physical functioning
5. Costly to treat

Question 2

Crying/Colic - sx

Answer 2

3. Clinical manifestations
   a. Crying develops in the early weeks of life and peaks around 6-8 weeks of age
   b. Usually worse in late afternoon or evening but may occur at any time
   c. May last several hours
   d. Infant may draw up legs as if in pain, but NO evidence that colic is attributable to an intestinal problem or wind
   e. Usually improves by 3 - 4 months of age
4. The tired baby
   a. Tired signs = jerky movements, frowning, grizzling, crying
   i. Often misread as boredom, hunger
   b. Rough guide
   i. Infants aged 5-6 weeks after 1.5 hours
   ii. Infants aged 12 weeks after 2 hours
   c. Older babies = clumsiness, clinginess, grizzling, crying, demands for constant attention, boredom with toys, fussiness with food
   d. Managing tired baby
   i. Into cot awake
   ii. Pat/stroke until quiet but NOT asleep
   iii. Re-settle at 2 minute intervals in young baby <6 months
   iv. Consider wrapping +/- dummy
5. Red flags
   a. Sudden onset of irritability and crying should not be diagnosed as colic; a specific cause is usually present
   b. The maternal and family psychosocial state must be taken into account
   i. Maternal post-natal depression may be a factor in presentation
   ii. Note that excessive crying is the most proximal risk factor for Shaken Baby Syndrome
   c. Suspect cow milk / soy protein allergy if
   i. Vomiting / blood or mucus in diarrhoea / poor weight gain / family history in first degree relative / signs of atopy (eczema / wheezing) / significant feeding problems (especially worsening with time)
   ii. Gastro-oesophageal reflux is diagnosed
   iii. Lactose malabsorption is diagnosed in formula-fed babies

Question 3

Crying/colic - aetiology

Answer 3

4. Aetiology
   a. Non-organic causes (90%)
   i. Overtired = suspect if the infant’s total sleep duration per 24 hours falls more than an hour short of the “average” for their age
   ii. Hungry = this is more likely if a mother reports her baby has frequent feeds (ie < 3 hourly), poor weight gain and inadequate milk supply
   iii. ‘Difficult’ temperament
   iv. Delay in neuromaturation
5. Infants vary in maturation of self-regulation and self-soothing
6. Infants respond differently to
   a. Internal factors eg. gut spasm
   b. External factors eg. loud noises
   v. Sub-optimal parent-infant relationship
   b. Organic causes (10%)
   i. Allergy – cow’s milk protein, soy protein
   ii. GIT problem – GOR, bowel spasm
   iii. Lactose intolerance
   iv. Neurological – early sign of CP
7. Acute causes of crying
   a. Urinary tract infection
   b. Otitis media
   c. Raised intracranial pressure
   d. Hair tourniquet of fingers / toes
   e. Corneal foreign body / abrasion
   f. Incarcerated inguinal hernia

Question 4

Organic causes of crying/colic - CMPA/soy

Answer 4

a. Food allergy
i. Immediate onset (<2 hours) – vomiting, diarrhoea, rash
ii. Delayed onset (up to 72 hours) – vomiting, diarrhoea
iii. Atopic disease in 50% at presentation
iv. Cause of reflux and oesophagitis

b. Cow’s milk protein/ soy protein allergy
i. Both can be found in human breast milk - goat milk protein is as allergenic as cow milk protein
ii. Usually a manifestation of delayed (non-IgE mediated) reactions
iii. Clinical manifestations
1. Usually colicky crying and 1 or more of
a. Blood or mucous in bowel actions
b. Poor weight gain
c. Eczema
d. Vomiting
e. Family history of 1st degree relative with food allergy
2. Other manifestations
a. Signs of atopy (eczema/wheezing)
b. Significant feeding problems
iv. Management
1. Formula fed
a. Extensively hydrolysed (eg. Apatmil, Pepti Junior, Alfare, Allerpro) - needs consultation with allergist/immunologist/paediatrician/gastroenterologist
b. Amino acid based (eg. Neocate, Elecare)
c. Add golden syrup or vanilla
2. Breast fed
a. Eliminate all cow’s milk product
b. Calcium supplement for mother
3. Trial 2 weeks – if after 2 weeks of extensively hydrolysed then 2 weeks of amino acid formula they do not respond; can cease trial

Question 5

Organic causes crying/colic - reflux

Answer 5

i. Key points
1. No causal relationship between GORD and infant crying and irritability has been demonstrated
2. “Silent reflux” (reflux without vomiting) is an unlikely cause of infant crying
3. The duration of daily crying is unlikely to reflect the severity of gastro-oesophageal reflux
4. GORD may be secondary to cow milk / soy protein intolerance
ii. Clinical manifestations
1. Vomiting >5x per day
2. Feeding difficulty
iii. Management
1. Ranitidine and omeprazole have not been shown to be effective in reducing crying
2. Anti-reflux medication to manage persistent infant irritability is not recommended
3. Side effects
a. Increased risk of pneumonia
b. Gastroenteritis
c. Later fractures – dose response
d. Allergy

Question 6

Organic causes crying/colic - lactose intolerance/overload

Answer 6

i. Not true allergy
ii. Primary lactose intolerance is extremely rare
iii. Clinical manifestations
1. Frothy, watery diarrhoea
2. Perianal excoriation
iv. Cause
1. Breastfed – frequent feeding, frequently changing sides
2. Formula fed – mucosal injury of the GIT secondary to cow milk/ soy protein allergy
v. Diagnosis
1. Faecal reducing substances ≥0.5% and pH < 5.0
2. Confirmed by clinical response to lactose-free formula
3. Lactose hydrogen breath test if available
vi. Management
1. Breastfed = commonly functional lactose overload (high lactose content in foremilk)
a. Empty entire breast – avoid frequently switching sides
b. Feed less frequently (>3 hours)
c. Lactase treated milk (no evidence Cochrane)
d. Lactase tablets (4-12 drops)
2. Formula fed = consider change to lactose free or extensively hydrolysed

Question 7

Colic/crying - ix/rx

Answer 7

8. Investigations - typical hx and ex requires no ix
   a. Stool reducing substances and pH (if indicated)
   b. Acute crying
   i. Urine microscopy and culture
   ii. Fluorescein staining of eyes (if history suggestive)

Management

• Exclude medical cause
• Parental education and reassurance (see RCH guideline for more details)
• Assess parental emotional state and mother-baby relationship, assess for PND

Medication is not indicated, this includes:
Anti-reflux medications — ineffective in reducing crying compared with placebo
Anticholinergic medications — due to risk of serious adverse events eg apnoeas, seizures
Colic mixtures (eg gripe water) – no proven benefit
Simethicone (eg Infacol Wind Drops/Degas Infant Drops) - no effect on crying compared with placebo

There is limited evidence to support probiotic use
Only in exclusively breastfed infants under 3 months, the probiotic Lactobacillus reuteri DSM17938 (BioGaiaTM) has been shown to be effective with excessive crying (colic)
To be given as 5 drops per day orally to the infant for 21 days only
It should not be given to formula-fed infants
The probiotic has not been shown to be effective in both breastfed and formula-fed infants in Victoria, Australia
Probiotic effects are strain-specific; Lactobacillus reuteri DSM17938 is the only probiotic strain with some evidence of efficacy in exclusively breastfed infants with excessive crying (colic)

Formula changes are usually not helpful unless there is proven cow milk allergy. Weaning from breast milk has no benefit
Spinal manipulation is not indicated and has associated risks

Question 8

Tantrums - general

Answer 8

1. Key points
   a. Defined as out of control behavior involving – screaming, stomping, hitting, head banging, falling down, or other displays of frustration
   b. Can include vomiting, aggression, biting
   c. Trigger = frustration, anger, inability to cope with situation
2. Epidemiology
   a. 18 months to 4 years
   b. Peak 2-3 years (50-80% children have regular tantrums)
   c. Lasting 2-5minutes
3. Aetiology
   a. Normal for childhood developmental stage
   b. Must consider – NAI, domestic violence, hearing loss/language delay, behavioural/developmental disorders (Autism), psychiatric conditions, underlying neurological conditions (TBI, Prader Willi)
4. Strategies to prevent
   a. Consistency – toddlers difficulty adapting to different environments, routine/schedules as well as consistent rules/enforcement
   b. Warnings – to familiarize child with rules
   c. Expectations – set reasonable ones for child
   d. Positive reinforcement – for desired behaviors, child feels satisfied and appreciated
   e. Avoid known problems – avoid taking child out when hungry/sleepy
5. Management
   a. Stay cool – the more attention, the more likely it will be repeated
   b. Ignoring – withdraw all attention, consistent amongst care givers. Intervene if others at risk
   c. Distraction – known precipitants
   d. Time outs – only if other methods have failed. Ensure consistent place/room, 1min for each year age.
   e. Verbal reprimand – if child is old enough, discuss what happened and better ways to handle
   f. Punishment – NOT recommended, can increase frequency tantrums and reinforce aggression

Question 9

Poor growth - bg

Answer 9

1. Background
   a. FTT = poor growth
   b. Nutrition is the main driver for a child’s growth
2. Aetiology
   - inadequate intake
   - inadequate absorption (coeliac, liver, CF, diarrhoea, CMPA)
   - excessive caloric utilisation (chronic illness, UTI, CF, CHD, DM, hyperT)
   - psychosocial (low SES, PND, etc)
   - other (genetic/syndromic, IEM, endo)
3. Growth charts
   a. Growth charts
   i. WHO <2 years
   ii. CDC > 2 years
   iii. Condition specific – Turner, Down syndrome, Williams
   b. Serial measurements are needed to assess a child’s growth
   i. Many healthy children grow on centile lines at the top or bottom of the growth chart and many healthy children have small “dips” above or below a particular centile line or growth curve
   ii. Birth weight is not necessarily representative of the genetic potential for future growth
   c. Correct for prematurity (<37 weeks) until 24 months of age
   d. Length and head circumference should also be plotted on growth charts and it is important to take these into account in the overall assessment of a child with poor growth

Expected growth

• 1st 3mo: 150-200g/week
• 3-6mo: 100-150g/week
• 6-12mo: 70-90g/week

Question 10

Poor growth - assessment/hx/ex/ix

Answer 10

a. History
i. Infants
1. Breastfeeding
a. Breast feeding difficulties, timing of feeds and the presence or absence of vomiting.
b. Is the infant “settled” with breast feeds?
c. Mother’s perception of breast milk supply/difficulties.
d. Previous experience of breast feeding.
2. Formula feeding
a. Volumes, changes to formula, dilutions (check scoops to volume of water), vomiting, or diarrhoea
3. Timing of the introduction of solids and the types of foods offered.
a. The parent infant interaction during feeding eg. Force feeding.
b. Are mealtimes pleasant or unpleasant?
c. Does the infant accept solids readily?
ii. Toddlers
1. Mealtime battles, coercive feeding, food refusal
2. Milk volume over 24 hrs
3. Assess parental attitude towards foods and mess
iii. Other
1. Antenatal complications and maternal health
2. Birth weight, length and head circumference
3. Significant intercurrent illnesses coinciding with onset of poor growth
4. Vomiting and diarrhoea
5. Developmental delay, regression or syndromal causes of poor growth
6. Mid-parental height and the family history of childhood weight gain
7. Social
a. Lack of financial resources for food requirements
b. Lack of suitable housing
c. Lack of family/community supports
d. Refugee or recent immigrant background
e. Parental mental health problems
f. Community Services History
g. Failure to attend hospital or community services appointments.
h. Previous history of child protection involvement

b. Examination
i. Does the child appear sick, scrawny, irritable or lethargic?
1. Evidence of loss of muscle bulk and subcutaneous fat stores; especially upper arm, buttocks and thighs
2. Conduct a thorough examination with particular attention to potential underlying diagnoses
3. Look for signs of child abuse and neglect
ii. Observe the child-parent interaction and communication (cues from infant)
iii. In younger infants, consider observing a feed

c. Investigations
i. No investigations are necessary at first
ii. First line investigations
1. FBE, ESR, UEC, LFT
2. Iron studies
3. Calcium, phosphate
4. Thyroid function
5. Blood glucose
6. Urine for microscopy and culture
7. Coeliac screen if on solid feeds containing gluten
8. Stool microscopy and culture
9. Stool for fat globules and fatty acid crystals

Question 11

SIDS - bg

Answer 11

1. Definition
   a. = sudden infant death syndrome
   b. Sudden unexpected death in sleep
   c. 1 month to 12 months
   d. No cause on post mortem
   e. Examination of death at scene not suspicious
2. Epidemiology
   a. 1.3/1000 live births (Australian), 7.5/1000 indigenous
   b. Normal age 2-5 months (95% < 7 months)
   c. Accounts for 45% of infant deaths 1 month – 1 year (post-neonatal mortality)
   d. Winter peaks (viral infections)
   e. Saturday peaks (parental drug use, co-sleeping)
   f. Brainstem abnormality in cardiorespiratory control = asphyxia

Question 12

SIDS - RFs

Answer 12

Biggest RF:

• young maternal age (<20)
• maternal smoking
• late/no prenatal care
• preterm birth and LBW
• prone sleeping
• sleeping on soft surface
• bed sharing
• overheating

NOT a RF:

• neonatal vital signs
• apnoea of prematurity
• recent URTI or immunisation

Risk reduction (uptodate):

• breast feeding
• room sharing
• pacifier use
• fan use
• immunisations

Question 13

SIDS - causes, rx

Answer 13

4. Causes
   a. 85-90% SIDS
   b. Cause more likely if atypical age (<1/12 or >6/12)
   c. Infection (7%)
   d. Cardiovascular disease (2.7%)
   e. Child abuse (2.6%)
   f. Metabolic/genetic disorders
5. Management
   a. Prevent risk factors
   b. Baby on back from birth
   c. Face and head uncovered
   d. Smoke free environment
   e. Safe sleeping environment, minimal sleep overs
   f. Sleep baby in own safe sleeping place in same room as adult carer for first 6-12months
   g. Breastfeed baby if possible
   i. Consider carer training in CPR

Home cardiorespiratory monitoring is NOT recommended (uptodate)

• no benefit
• poor correlation with hospital monitoring (pulse ox etc)
• can be falsely reassuring for parents
• equally can cause more anxiety with false alarms
• apnoea not thought to be the initiating event for SIDS
• infants have died whilst on home CR monitoring

Question 14

Microcephaly - bg

Answer 14

1. Key points
   a. OFC = occipitofrontal circumference (OFC): Standardised charts for age, sex and gestation
2. Definitions
   a. Variable definitions
   b. Generally accepted as >3SD below mean for age and sex (some define as >2SD below)
   c. Further defined
   i. Borderline microcephaly – between 2-3 SD below mean
   ii. Moderate microcephaly – between 3-5 SD below mean
   iii. Severe microcephaly - >=5 SD below mean
3. Pathogenesis
   a. Lack of brain development or abnormal brain development related to a developmental insult during the time-specific period of induction and major cellular migration; thought to result from a reduction in the number of neurons generated during neurogenesis
   i. Forebrain most affected – holoprosencephaly
   b. Injury or insult to a previously normal brain; reduction in the number of dendritic processes and synaptic connections

Question 15

Microcephaly - aetiology, classification

Answer 15

4. Classification
   a. Time of onset
   i. Congenital microcephaly = present at birth or by 36 weeks gestation
   ii. Postnatal microcephaly = failure of normal growth
   b. Aetiology
   i. Primary = no associated malformations and follow a Mendelian pattern of inheritance or are associated with specific genetic syndrome
   ii. Secondary (non-genetic)
   c. Relation to growth parameters
   i. Symmetric = proportionate
   ii. Asymmetric = disproportionate
5. Aetiology
   a. Primary
   i. Genetic = isolated microcephaly (true microcephaly, microcephaly vera)
   ii. Syndromal (T21, T18, cri-du-chat)
   b. Secondary
   i. Environmental
6. Congenital infections = CMV, rubella, toxoplasmosis
7. Meningitis
8. Drug or toxin exposure
9. Other perinatal insult – hypoglycaemia, hypothyroidism, hypopit, hypoadrenalism
10. Anoxia/ischaemia
    ii. Neuroanatomic = NTD, holoprosencephaly, lissencephaly, polymicrogyria
    iii. Metabolic = maternal diabetes, PKU, untreated maternal PKU methylmalonic aciduria, citrullinaemia, neuronal ceroid lipofuscinosis

Question 16

Microcephaly - approach, ix

Answer 16

6. Approach
   a. History
   i. Prenatal history – maternal medical problems (diabetes, epilepsy, PKU, medications etc)
   ii. Birth history
   iii. Weight, length, OFC at birth
   iv. OFC trajectory
   v. History of seizures, developmental history
   vi. Family history consanguinity
   b. Physical examination
   i. Dysmorphology
   ii. OFC, weight + height
   iii. Head shape
7. Anterior fontanelle – closes between 10 and 24 months
8. Eyes – intrauterine infections (chorioretinitis, cataract), metabolic disease (cataract)
9. Oropharynx – single maxillary incisor (holoprosencephaly)
10. Skin
11. Abdomen – hepatosplenomegaly
12. Neurological – at risk for CP developmental
13. Investigations
    a. Genetic testing
    b. Evaluate for TORCH
    c. Evaluate for metabolic or storage disorder
    d. Neuroimaging
    i. MRI = identify structural abnormalities such as lissencephaly, pachygyria, and polymicrogyria
    ii. CT = calcification

Question 17

Macrocephaly - general

Answer 17

1. Key points
   a. Can be caused by increase in size of ANY components of cranium – brain, CSF, blood or bone
   b. Most common causes vary with age of onset
2. Definition
   a. Macrocephaly = OFC >2 SD above mean for age, sex, gestation
   b. Megalencephaly = enlargement of brain parenchyma
3. Aetiology
   - increased brain (anatomic, metabolic)
   - increased CSF (hydrocephalus)
   - increased blood (intracranial haemorrhage)
   - increased bone (thalassaemia, bone dysplasias)
   - increased ICP (idiopathic, infective, inflammation, metabolic, mass lesion)

Early infantile (birth to 6 months)	
        Hydrocephalus 
	Subdural effusion 
	Normal variant (often familial)
Late infantile (6 months to 2 years)	
        Hydrocephalus 
	Dandy-Walker syndrome 
	Subdural effusion 
	Increased ICP
	Primary skeletal cranial dysplasias 
	Megalencephaly 
	Achondroplasia 
	Primary megalencephaly 
Early to late childhood (older than 2 years of age) 	 
        Hydrocephalus 
	Megalencephaly 
	Pseudotumour cerebri 
	Normal variant

Question 18

Craniosynostosis - bg

Answer 18

1. Key points
   a. Premature closing of cranial sutures
   b. Incidence 1/2000
   c. Craniosynostosis can involve single sutures (85%) or multiple sutures (15% - more likely to be assoc with syndrome/genetic defect)
   d. Delayed closure = rickets, hypothyroidism, malnutrition, osteogenesis imperfecta, hydrocephalus, chromosomal anomaly
2. Classification
   a. Primary = due to abnormal skull development; premature closure of one or more sutures
   b. Secondary = failure of brain growth
   i. This leads to restricted growth perpendicular direction to the affected suture
   ii. Compensatory skull growth occurs parallel to suture
3. Aetiology
   a. Idiopathic
   b. Genetic syndrome = 10-20%
   i. Crouzon/ Apert/ Carpenter/ Chotzen/ Pfeiffer/ mutations of fibroblast growth factor receptor
4. Normal development
   a. Cranial bones develop by 5th month of gestation
   b. Normal fontanelle closure
   i. Posterior – 2 months
   ii. Anterior lateral – 3 months
   iii. Posterior lateral – 1 year
   iv. Anterior – 2 years
   c. Normal suture closure
   i. Metopic – 2 months
   ii. Sagittal – 22 months
   iii. Coronal – 24 moths
   d. In the first 2 years of life
   i. Brain volume quadruples
   ii. Brain size reaches 75% of the adult brain

Question 19

Craniosynostosis - sx, rx, cx

Answer 19

5. Clinical manifestations
   a. Prominent bony ridge
   b. Fusion of the suture may be seen in XRs/ CT
   i. Plain films helpful for accurate measurements
   ii. CT usually required to assess underlying brain/ surgical panning
6. Sutures + deformity
   a. Metopic = middle forehead
   i. Trigonocephaly = triangular/pointed forehead
   b. Sagittal = Centre (most common)
   i. Scaphocephaly = long and narrow head
   c. Lambdoid = like lambda = back
   i. Posterior plagiocephaly = unilateral fusion
   d. Coronal = ear to ear
   i. Brachycephaly = bilateral fusion = flat and tall forehead / short and wide head
   ii. Anterior plagiocephaly = unilateral fusion = affect side flat, other side bulges out
7. Management
   a. Surgical correction = usually aim 8-12 months
   i. If one suture involved only , this is purely for cosmetic purposes
   ii. If > 1 suture involved, surgery required to avoid hydrocephalus/ raised ICP
8. Complications
   a. Raised ICP = uncommon, especially if only one affected
   b. Inhibition of brain growth
   c. Impaired; cognition, growth, development, poor feeding, poor weight gain, vision, hearing, speech
   i. Even in single suture craniosynostosis

Question 20

Scaphocephaly - general

Answer 20

Sagittal suture closes prematurely

• Long thin head
• Point on top of head
• Prominent occiput and broad forehead
• Most common
• Sporadic
• M>F
• Does not produce raised ICP or hydrocephalus
• Can occur with obstructed labour

Question 21

Brachycephaly - general

Answer 21

Coronal suture closes prematurely bilaterally

• Both sides forehead flattened

• Apert/ Crouzon disease
• F>M

Question 22

Frontal/anterior plagiocephaly - general

Answer 22

Premature fusion of coronal and sphenofrontal suture (unilateral)

• Affected side flattened forehead
• Elevation of ipsilateral orbit, eyebrow and ear
• Contralateral side prominent forehead

• F>M

Question 23

Occipital/posterior plagiocephaly - general

Answer 23

Most often positional
Can be caused by fusion lambdoid suture

• Occipital flattening, bulging of contralateral forehead
• Ipsilateral ear is inferior

• Positional occipital flattening can mimic posterior plagiocephaly

Question 24

Trigonocephaly - general

Answer 24

Premature fusion of metopic suture

• Ridge down forehead, pointed forehead
• Hypotelorism
• Rare
• Assoc w 19p
• Increased risk of developmental abnormalities of brain

Question 25

Turricephaly - general

Answer 25

Premature fusion of coronal + sphenofrontal + frontal ethmoidal sutures

• Cone shaped head

Question 26

Kleeblattschadel deformity

Answer 26

• Cloverleaf shaped head – prominent temporal bones

Question 27

Syndromes a/w abnormal head shape

Answer 27

• Most AD
• Mutations of FGFR gene
• FGFR1 mutation (chromosome 8) = Pfeiffer syndrome
• FGFR2 = Apert syndrome, Pfeiffer syndrome, Crouzon syndrome

1. Crouzon syndrome
   b. FGFR2 mutation
   d. Clinical manifestations
   i. Skeletal
2. Skull deformity (brachycephaly – bilateral coronal suture fusion, tall forehead)
3. Midface hypoplasia (prominent nose & forehead, maxillary hypoplasia) + OSA
4. Mild hypotelorism, proptosis
5. NORMAL hands and feet
   ii. Neuro
6. Hydrocephalus
7. Visual problems (optic atrophy, proptosis)
8. Apert syndrome
   b. FGFR2 mutation
   c. Clinical manifestations
   i. Skeletal
9. Irregular craniosynostosis (brachycephaly, plagiocephaly - coronal)
10. Premature fusion of coronal, sagittal, lambdoid sutures
11. Midface hypoplasia + OSA
12. Exophthalmos but less severe than Crouzon
13. Symmetrical syndactyly (fusion of 2nd/3rd/4th digits – single nail 2nd-4th digits)
    ii. Neuro
14. Hydrocephalus
15. Intellectual disability
16. Pfeiffer syndrome
    b. Rarely FGFR1
    c. Clinical manifestations
    i. Skeletal
17. Skull deformity (scaphocephaly + brachiocephaly)
18. Midface hypoplasia (flat face, shallow orbits)
19. Hypertelorism, prominent eyes, mild exophthalmos, small nose
20. Partial syndactyly – webbing fingers/toes
21. Deviated broad and short thumb/big toe
22. Carpenter syndrome
    a. AR, rare
    b. Skull deformity (cloverleaf- turricephaly – coronal + other suture fusion)
    c. Prominent metopic ridge
    d. Narrow maxilla with broad depressed nasal bridge
    e. Polydactyly and syndactyly of hands and feet
    f. Intellectual disability common

Question 28

Positional head deformity

Answer 28

Soft shape of infant skull changes shape due to outside forces (in utero, sleep position)

Sutures in tact
Forehead protrudes on SAME side as posterior flattening
Ear anterior displaced on affected side

Rx

• physical therapy
• helmet
• ref to PT

Question 29

Breath holding - general (except classification)

Answer 29

1. Key points
   a. NOT a cause of death, epilepsy, intellectual disability or cerebral damage; EEG unnecessary
   b. Family history common
   c. Caused by bradycardia/ asystole (VERY brief)
2. Clinical manifestation
   a. Occur in children 6 months to 6 years
   b. 80-90% have first episode by 18 months of age
   c. Always has a precipitant – trauma, emotional fright, anger, frustration
   d. Cyanotic more common; pale less common
3. Investigations
   a. No diagnostic test
   b. Consider other causes of syncope – ECG
   c. FBE + Fe studies
4. Treatment
   a. Fe supplementation
   i. Indications
5. Anaemic
6. Iron deficient
   ii. Complete response in 30-50% of children

Iron deficiency anemia is more prevalent in children with breath-holding spells compared with controls and appears to contribute to the occurrence of breath-holding spells and the underlying dysautonomia.

6. Prognosis
   a. Excellent prognosis

Question 30

Breath holding - classification

Answer 30

Pallid
• Trigger – minor injury (often to the head or upper body), upset, tantrum
• Often can be delayed 30s after trigger
• Open mouth as if to cry but no sound, faint/collapse, appear pale, diaphoretic, often followed by increased tone/loss continence/clonus.
• <60s, some confusion afterwards
• Sometimes followed by seizure (rare)
• Caused by cardiac bradycardia

Blue
• Trigger – pain, frustration, reprimand
• Scream, apnea, colour change red/blue, floppy/unconscious, rapid recovery
• Sometimes followed by seizure (rare)
• 15-25% children have multiple episodes daily

Question 31

Milestones (GFSL) - 6 weeks

Answer 31

Raise head
Tight fist, fix and follow to midline
Smiles
Alert to sounds

Question 32

Milestones (GFSL) - 2 months

Answer 32

Lift chest
Hands together, fix and follow past midline, Opens fist
Social smile
Smiles to coos

Question 33

Milestones (GFSL) - 4 months

Answer 33

Rolls prone to supine
Supports on elbows + wrists prone
Minimal head lag

Reaches midline
Hands unfisted
Grasps object

Excited by environment
Works for food and toys

Squeals
Orients to voice

Question 34

Milestones (GFSL) - 6 months

Answer 34

Sits unsupported
Rolls supine to prone

Transfers – crossing midline
Raking
Reaches for object

Feeds self
Holds bottle
Begins to recognize strangers

Babbles

Question 35

Milestones (GFSL) - 9 months

Answer 35

Crawls/stands/cruises
Comes to sit from lying

Crude pincer

Object permanence
Waves
Peek a boo

Mama/dada

Question 36

Milestones (GFSL) - 12 months

Answer 36

Walks alone
Stands alone

Refined pincer
Bangs objects together

Imitates eg. clapping

Mama/dada
Words
Jargoning (polysyllabic)
1 command

Question 37

Milestones (GFSL) - 15 months

Answer 37

Walks backwards
Runs

Scribble
2 blocks
Spoon

Indicates wants

Question 38

Milestones (GFSL) - 18 months

Answer 38

Walks well
Runs, kicks
Throws – 2 hands
Walks up stairs with hand held

Handedness
Scribble
2 blocks

Copies task
Pretend play

5-20 words
2 word sentences
Points to pictures and body parts

Question 39

Milestones (GFSL) - 2 years

Answer 39

Rides on toy without pedals

Line
6 blocks
Pages, doors, undress

Parallel play

50-200 words
Pronouns
2/4 clear
2 commands

Question 40

Milestones (GFSL) - 3 years

Answer 40

Broad jump
Pedals
Alternating steps

Circle
Dressing
Draws person with head + body part

Group play
Knows name, age and gender

1000 words
Grammar
3/4 clear

Question 41

Milestones (GFSL) - 4 years

Answer 41

One foot
Hop
Stairs without support

Square
Cross
Person – 3 parts

1500 words
Full name
Colours
Songs
4/4 clear

Question 42

Milestones (GFSL) - 5 years

Answer 42

Skips
Heel to toe
Leaps

Triangle
Shoe laces
Spreads with knife

Question 43

Object permanence - ages

Answer 43

• 8-12 months: a child will be able to uncover an object he sees being hidden
• 12-18 months: child will be able to uncover an object even if he doesn’t see it being hidden

Question 44

Newborn reflexes - Moro

Answer 44

• The examiner holds the infant supine in his or her arms, then drops the infant’s head slightly but suddenly - this leads to the infant extending and abducting the arms, with the palms open, and sometimes crying
• Alternatively, the examiner may lift the infant’s head off the bed by 1 to 2 inches and allow it to gently drop back; this maneuver elicits a similar response

Appears: 34 to 36 weeks PCA
Disappears: 5 to 6 months

Question 45

Newborn reflexes - asymmetric tonic neck reflex

Answer 45

• With the infant relaxed and lying supine, the examiner rotates the head to one side
• The infant extends the leg or arm on the side towards which the head has been turned, while flexing the arm on the contralateral side (fencing posture)

Appears: 38 to 40 weeks PCA
Disappears: 2 to 3 months

Question 46

Newborn reflexes - trunk incurvation/Galant

Answer 46

• With the infant in a prone position, the examiner strokes or taps along the side of the spine
• The infant twitches his or her hips toward the side of the stimulus

Appears: 38 to 40 weeks PCA
Disappears: 1 to 2 months

Question 47

Newborn reflexes - palmar grasp

Answer 47

• The examiner places a finger in the infant’s open palm
• The infant closes his or her hand around the finger, tightens the grip if the examiner attempts to withdraw the finger.

Appears: 38 to 40 weeks PCA
Disappears: 5 to 6 months

Plantar grasp is the same but remains until 9-10 months

Question 48

Newborn reflexes - rooting

Answer 48

• The examiner strokes the infant’s cheek
• The infant turns the head toward the side that is stroked, and makes sucking motions

Appears: 38 to 40 weeks PCA
Disappears: 2 to 3 months

Question 49

Newborn reflexes - parachute

Answer 49

• The infant is held upright, back to the examiner
• The body is rotated quickly forward (as if falling). The infant reflexively extends the upper extremities towards the ground as if to break a fall.

Appears: 8 to 9 months of age
Persists throughout life

Question 50

Motor milestones (G+F) - red flags

Answer 50

Gross Motor 
•	Reduced movement on one side
•	Poor head control at 4 months
•	Primitive reflexes at 6 months
•	Not sitting at 9 months
•	Not weight bearing at 10 months
•	Unable to walk independently 18months
•	Toe walking at 2 years
•	Clumsiness at 3 years
•	Gross motor is least predictive of a cognitive problem

Fine Motor 
•	Intention tremor
•	Fisting at 4 months
•	Early handedness before 18 months
•	Not reaching at 6 months
•	Not transferring at 9 months
•	No pincer grip at 12 months
•	No stacking at 15 months
•	No feeding self at 18 months
•	No drawing a person at 3 years

Question 51

Milestones - screening

Answer 51

• The following motor skills should be observed in the young child at the specified visit
• These skills are typically acquired at earlier ages, and their absence at these ages signifies delay
• Loss of previously attained motor skills should also raise concern

Common causes of delay: CP, ID, ASD

9 MONTHS

• Rolls to both sides
• Sits well without support
• Demonstrates motor symmetry without established handedness
• Grasps and transfers objects from hand to hand

18 MONTHS

• Sits, stands, and walks independently
• Grasps and manipulates small objects

30 MONTHS

• Evaluate for subtle gross motor, fine motor, speech, and oral motor impairments
• Evaluate for loss of previously attained gross or fine motor skills

4 YEARS

• Evaluate coordination, fine motor, handwriting, gross motor, communication, and feeding abilities
• Address any preschool or child care staff concerns about motor development
• Evaluate for loss of previously attained gross or fine motor skills

Question 52

Psychological development - general

Answer 52

• Bonding – occurs soon after birth and reflects feelings of parents towards newborn = UNIDIRECITONAL

• Attachment – reciprocal feelings between parents and infant develop over first year = BIDIRECTIONAL
o Functional attachment crucial for optimal development
o Parents who respond immediately to crying/fuss show less crying at end of 12 months

• Stanger anxiety – develops 9-18months of age reflects insecurity on separation from care giver.
o Rapid swings from stubborn independence to clinging dependence

• Toddler (2-3 years)– autonomy that allows separation, rapid development of skills in all areas, limit setting essential to balance child’s emerging independence.

• Preschool (4-5years)– readiness for preschool when child autonomous and can separate for hours at a time.
o Preschool assists socialization, language, problem solving skills.

• Adolescence (10-25years) – Early (focus on self/physical changes, and peers), middle (independence, psychosocial development, relationships), late (formal operational thinking)

Piaget’s cognitive developmental theroy:

0-2

• Sensorimotor
• Establishment of mental representation
• Understanding of the world is based on senses and motor skills

2-6

• Preoperational thought
• Symbols, words and numbers
• Egocentric (only own perspective seen)

7- early adolescence

• Concrete operational thought
• Logical operations start

Adolescence and beyond

• Formal operational thought
• Abstract thinking, hypotheticals

Question 53

Developmental delay - primary care screening tools

Answer 53

1. Surveillance = information = Use of developmental milestones
2. Screening = formal screening test (standardized)
   a. Sensitivity/specificity = 70-80%
   b. 20-30% false negatives
   c. Cognitive screening best correlates with receptive language in younger children
   d. Abnormality = referral for full assessment
3. Tests used in primary care
   a. ASQ2: screen development from 1-66 months; 15 minutes, assesses cognition, motor, self-help and language (does NOT include social emotional factors)
   b. Infant-Toddler Checklist for language and communications: 6-24 months, used to screen children during this period
   c. Brigance Early Childhood Screens: screens 0-7 year old
4. Narrow band instruments look specifically for ADHD/ autism
   a. Conners 3rd edition ADHD index
   b. Modified checklist for autism in toddlers
   c. Vanderbilt ADHD diagnostic parent and teacher rating scale

Question 54

Development/IQ assessments - overview

Answer 54

Alphabetical for order of age appropriateness:

• Bailey 1mo-3.5yr (gold standard)
• Griffiths 0-8yrs (better for older children than Bailey)
• McCarthy 2.5-8.5yrs
• Peabody picture test 2.5-4yrs
• WIPPSI (Weschler preschool and primary scale) 2.5-7yrs
• WISC 5-18yrs (Weschler), generates an IQ

Development Screening:

• ASQ (ages/stages) 0-4yrs
• Denver II 0-6, good for language delay
• PEDS (parental) 0-8

IQ Screening

• 0-2: Griffiths
• 1-3: Bailey
• 2-8 McCarthy
• > 2.5 Peabody
• 3-7 WIPPSI
• 6-16 WISC

Question 55

Developmental delay - bg

Answer 55

1. Key points
   a. 10- 15% of children may have some form of developmental delay or disability
   b. 20-30% of children have developmental delay of at least mild-moderate severity that remain undiagnosed unless specific assessment taken – usually diagnosed at school
   c. Most have no identifiable cause such as a syndrome or physical abnormality
   d. Age at presentation
   i. Motor and speech problems present earlier
   ii. Problems affecting receptive language, socialization and cognition present later
2. Definitions
   a. A child younger than 5 (>5 use IQ)
   b. On standardized testing, functional level >2SD below the mean in gross motor, fine motor, language, cognition, psychosocial development
   c. Developmental delay = one domain affected
   d. Global developmental delay = two or more domains affected
   i. Eg. motor + language, language + social
   ii. Includes children with intellectual disability
3. Prevalence
   a. All developmental disabilities – up to 10%
   i. Language concerns most common (12%), then GDD (1-3%), then ASD (<1%)
   b. GDD – 10%
   i. Similar to intellectual disability

Question 56

Developmental delay - assessment

Answer 56

6. Assessment
   a. History
   i. Prenatal
7. Genetic (T21, Fr X)
8. Toxin (FAS)
9. Infection (TORCHS)
   ii. Perinatal
10. Neurological insult (prematurity, HIE)
    iii. Postnatal
11. ABI (infection, trauma)
12. Psychosocial stress (neglect) – COMMON
    b. Physical examination
    i. Dysmorphology
    ii. Growth
    iii. Neurocutaneous signs
    iv. Neurological examination
    c. Developmental assessment
    i. Surveillance
    ii. Screening tools – physician or parent initiated
    iii. Formal multidisciplinary assessment
    d. Syndrome or phenotype
    e. Formulation and differential diagnosis
13. Clinical approach
    a. Elicit parental concerns
    b. Targeted history and physical helps to predict the risk of delay
    c. High risk
    i. Developmental delay
    ii. Dysmorphism
    iii. Chromosomal abnormality
    iv. Hearing, visual, abnormal neurology exam
    d. Moderate risk
    i. VLWB
    ii. Severe pre or perinatal insult
    iii. Low SES, poor support
    iv. Family adversity, mental health, authoritative style
14. Medical evaluation
    a. Try to catch developmental delay early and coordinate access to benefits/ services and further referrals
    b. Aetiological yield proportional to severity of delay and selection of population (45-80%)
    c. Work up done in rational and stepwise manner ? Guided by clinical acumen
    d. Specific diagnosis may help in explanation, management, prognosis, recurrence
    e. Advances in genetic testing and neuroimaging

Question 57

Developmental delay - ix

Answer 57

11. Importance of investigations
    a. Explanation and validation
    b. Management
    i. Parent support groups, advocacy for services, specific therapy
    c. Prognosis
    d. Recurrence risk – genetic counselling
    e. Associated conditions – seizures, CP, vision/hearing deficits, behavioural problems
    f. Stop further investigations
12. Investigations

a. Genetics
i. Molecular karyotype
1. Picks up microdeletion and microduplications
2. ‘Copy number variant’
3. Yield = 10-15% of children who were previously undiagnosed
ii. Exome sequencing (next generation sequencing)
1. Parts of the genome of interested are fully sequenced (rather than looking at deletions or duplications)
iii. Fragile X screening
1. 5% prevalence in GDD
2. Syndrome 1-6/1000 males, half as prevalent in females
a. Females – learning difficulties and anxiety
3. Unstable triplet repeat of FMR1 on X chromosome (symptoms if >200)
4. Boys; girls if suggestive family history

b. MRI
i. Imaging of choice
ii. Consider for certain reasons = abnormal head size, focal seizures, abnormal neuro exam
iii. 13% pickup if no additional features
iv. 25% if additional features
v. Most common findings – HIE, malformations, toxic/metabolic/ non-specific

c. Metabolics
i. Low pick up in GDD (1%) unless target use
ii. Suggestive features
1. Regression
2. Family history
3. Hypotonia
4. Vomiting/ diarrhoea
5. Decompensating during acute illness
iii. Amino acids (plasma), organic acids (urine)
iv. Other = pH, glucose, lactate, pyruvate

d. Other bloods
i. Thyroid function
ii. Iron studies
iii. CK
iv. Lead level – important in children with Pica
v. Vitamin B12
vi. Vitamin D – restricted indications

e. Electrical
i. 4.4% of GDD but provided aetiology only in 0.4%
ii. Careful history important
iii. Acquired epileptiform aphasias
1. Rare but potentially treatable
2. Eg. Landua Kleffner syndrome

Question 58

Developmental delay - rx

Answer 58

13. Management
    a. Early intervention is important
    b. Intervention targeted to symptoms
    i. Eg. Speech therapy, OT, psychology
    c. HOWEVER services are thin on the ground
    d. Paediatricians have an important role in advocacy and follow-up

e. Services
i. Refer to Early Childhood Intervention Services
1. 0 to 6 years
2. Delays in 2 or more areas of development
3. Long waiting list
ii. Transition to NDIS
iii. Community Health Center referral
1. Children with only language delays
2. Wait times generally much shorter
iv. Private therapy
1. Can be expensive
2. Funding
a. Autism or Best Start Funding (including CP, T21 etc) or NDIS
b. Medicare – GP Team Care Plan, Mental Health Plan
c. Private insurance
d. Carer’s allowance
v. Kindergarten/ Childcare can be very helpful
1. Opportunities to socialize
2. Learn routines, cooperation, sharing
3. Kindergarten inclusion support services/ Preschool field officer (DHS)

f. Transitions
i. Support transition into prep
1. Visit school
2. Meet principal
3. Transition programs
ii. Support within school
1. Individual learning plans
2. Extra funding – program for students with disabilities

14. Role of paediatrician
    a. Important role as part of MDT
    b. Recognize the DD
    c. Hear parents’ concerns
    d. Rule out medical causes
    e. Advocate for and coordinate services
    f. Monitor development and well-being over time

Question 59

Intellectual disability - bg, aetiology

Answer 59

1. Definition
   a. Affects up to 2.5% of children
   b. Significantly sub average general intellectual functioning = 2SD below mean intelligence quotient that exists concurrently with
   i. Deficits in adaptive behavior
   ii. Manifests during developmental period
   c. FSIQ (full scale intelligence quotient) <70 = intellectual disability
   d. FSIQ (full scale intelligence quotient) 71 = NO intellectual disability
   e. Classification
   i. Mild = 50-55 to 70 – physical and neurological examination usually normal
   ii. Moderate = 35-50 – often have dysmorphic appearance, a recognized syndrome or other known aetiology for ID
   f. DSM definition of mental retardation = significantly sub-average intellectual function accompanied by limited adaptive function, with onset <18 years
   g. A child with intellectual impairment may have other associated
   i. Developmental delays
   ii. Behavior problems
   iii. Health difficulties
2. Cause
   a. Prenatal
   i. Chromosomal = trisomy 21, fragile X syndrome, velocardiofacial syndrome (22q11- deletion)
   ii. Genetic = tuberous sclerosis, metabolic disorders
   iii. Major structural abnormalities of brain
   iv. Syndromes = Williams, Prader-Willi, Rett
   v. Infections = CMV
   vi. Drugs = alcohol
   vii. Low birth weight increases risk – the lower the birth weight the greater the risk
   viii. Always do a CK and TFT
   b. Perinatal
   i. Infections
   ii. Trauma
   iii. Metabolic abnormalities
   c. Postnatal
   i. Head injury
   ii. Meningitis or encephalitis
   iii. Poisons
   d. Evolving phenotype over time
   i. Rett syndrome
   ii. Prada Willi – detected earlier due to microarray
   iii. Angelman – detected earlier due to microarray
   iv. Velocardiofacial syndrome
   v. Williams syndrome
   vi. Noonan syndrome
   vii. Fragile X syndrome

Question 60

Intellectual disability - presentation

Answer 60

5. History
   a. Detailed birth and prenatal history – drugs, alcohol
   b. Hereditary and family history
   c. Three generation pedigree
6. Examination
   a. Informal
   i. Appearance
   ii. Behavior – eye contact, social engagement, attention, activity level, anxiety
   iii. Play – symbolic, imaginative, repetitive
   iv. Movement – skills, symmetry, quality
   b. Formal
   i. Developmental assessment – strengths + weaknesses
   ii. Minor physical anomalies
   iii. Vision + hearing
   iv. Physical + CNS examinations
7. Minor physical anomalies – eg. simian crease, low-set ears
8. Disturbance of growth – microcephaly, macrocephaly, extremes of stature or weight
9. Abnormal skin lesions – multiple depigmented or pigmented naevi
10. Malformations or abnormal findings in several organ symptoms
11. Behavior characteristics of specific disorders ie. behavioral phenotype
    v. Growth – plot centiles
    c. Other
    i. Video monitoring of posture and gait or behavioural characteristics
    ii. Serial evaluations over several years
12. Differential diagnosis
    a. The differential diagnosis of intellectual impairment includes children:
    i. Who have been severely deprived or abused
    ii. With a progressive neurodegenerative disorder or unrecognized epilepsy
    iii. With severe sensory or specific developmental disorders
    iv. Where the child’s apparent lack of skills is due to cultural differences, mental health disorders, ill health or refusal to participate
    v. Infants with severe movement difficulties

Question 61

Intellectual disability - investigations

Answer 61

a. Genetics
i. Imprinting – Prader Willi an Angelman syndrome
ii. Trinucleotide repeat expansion – Fragile X
iii. Rearrangement of sub-telomeres’ regions recently implicated

b. Consider
i. Chromosome = fragile X, William and Prader-Willi syndromes using DNA probes and FISH for 22q11 deletion
ii. MRI brain
iii. Creatinine phosphokinase in boys (neuromuscular disorder)

c. Metabolic
i. Indications = change with fever, neonatal hypotonia, progressive coarsening of features, loss of skills, recurrent coma, early morning cognitive deficits following fasting
ii. Extremely low yield for unselected metabolic screening
iii. Includes
1. Acid base
2. Plasma amino acids
3. Urinary organic and amino acids
4. Lactate-blood CSF
5. Cholesterol
6. Lysosomal enzyme analysis
7. Plasma and urine carnitine analysis
8. Plasma VLCFA

d. Other
i. TFTs
ii. Mucopolysaccharide screen
iii. Ix for congenital infection = ophthalmological and aetiological examination, maternal/ infant serology and viral culture (CMV)

e. Neuro-imaging
i. Indications
1. Microcephaly
2. Macrocephaly
3. Neurological signs – spasticity, ataxia, dystonia, seizures, loss of psychomotor skills, abnormal reflexes, abnormal cranial contour
ii. Options
1. CT = for cranial synostosis or where intracranial calcification is likely (TS, intrauterine infection) (avoided if possible <2 years due to risk of malignancy)
2. MRI study of choice
3. PET scanning
4. May help date onset of problem – prenatal, perinatal, postnatal

f. Environmental factors
i. Lead and methyl-mercury poisoning
ii. Alcohol
iii. Thalidomide
iv. Valproic acid
v. Polychlorinated biphenyls, dioxins, pesticides and tobacco smoke may be potential neurotoxins

Question 62

Intellectual disability - concomitant problems, rx

Answer 62

9. Concomitant medical problems
   a. Epilepsy 14-45% higher in smore severe ID
   b. Combination of ID and epilepsy is a strong predictor of psychiatric and behavioural problems
   c. Hypothyroidism is common in Down syndrome
   d. Many cancers occur with higher frequency
   e. Visual problems 10x more common cataract and keratoconus also common
   f. Hearing problems 40x more common
   g. Psychiatric disorders very common – pica, self-injurious behaviours, stereotypies and ADHD symptoms
10. Management
    a. Support and info for parents
    b. Referral to and liaison with other practitioners, early intervention, family support and educational services
    c. Child advocacy
    d. Regular assessment of hearing and vision
    e. Investigation for associated anomalies (eg. cardiac and thyroid status with trisomy 21)
    f. Treatment of associated disorders (eg. epilepsy)
    g. Monitoring of development

Question 63

Language - summary of milestones/development and red flags

Answer 63

• 2 year: 100 words, starting to put words together

Development of Expressive Vocabulary 
•	12 months = 2 words + mummy and daddy
•	2 years = 300 words
•	3 years = 1000 words
•	4 years = 2000 words
•	5 years = 6000+ words
•	17 years  36000 to 136000 words 

Reasons for Concern (RCH handbook)

6 months
No response to sound, not cooing, laughing or babbling

12 months
Not localising to sound or babbling
No babbling or babbling contains a low proportion of consonant vowel babble (eg. baba)
Does not understand simple words (eg. no and bye), recognize names of common objects, or respond to simple requests (eg. clap hands) with an action
Does not respond reliably to name by turning head

18 months
No meaningful words except mum dad
Does not understand and hand over objects on request

2 years
Expressive vocab <50 words and no word combinations
Cannot find 2-3 objects on request

3 years
Speech is not understood within the family
Not using simple grammatical structures eg. tense markers
Does not understand concepts such as colour and size

4 years
Speech is not understood outside the family
Not using complex sentences (4-6 words)
Not able to construct simple stories

5 years
Speech is not intelligible
Does not understand abstract words and ideas
Cannot reconstruct a story from a book

How much dose a stranger understand? 1/age i.e. 1/2 at age 2, all at age 4.

Question 64

Speech v Language disorder

Answer 64

• Speech disorder
o Articulation disorders = characterised by substitutions, omissions, additions or distortions of speech that interfere with intelligibility
o Fluency disorders (stuttering) = interruption of flow of speaking
o Voice disorders = pertain to abnormal production of vocal quality, pitch, loudness, resonance and/or duration

• Language disorder
o Impaired comprehension and/or use of spoken, written and/or other symbol systems
o May involve the form (grammar, syntax, morphology), content (vocabulary) and/or function (pragmatic use) of language

Question 65

Language delay - bg, definitions

Answer 65

1. Language
   a. Socially shared code
   b. Best correlates with cognitive ability, receptive»expressive
   c. Aspects of language
   i. Form = syntax, morphology, phonology
   ii. Content = semantics
   iii. Use = pragmatics; social use of language  classically
   d. Classification
   i. Receptive skills/language = ability to understand spoken language
   ii. Expressive skills/language = language production
   iii. Pragmatic skills/language = social use of language
   e. Speech = the sound of the spoken language
   i. Can have a speech problem without a language problem
   ii. Basically a motor impairment eg. stuttering, lisp
2. Speech and language delay
   a. Most common developmental delay
   b. 25% of one year olds have delayed speech
   c. 20% of two year olds have delayed speech
   i. Some of these are ‘late talkers’ who have typical language development but slower than others
   d. 5-8% of five year olds have a speech and language disorder
3. “Late talkers”
   a. Early language delay (2-3 years)
   b. Delayed language acquisition compared with apparent typical development in other areas
   i. Primarily expressive delay
   ii. Completely normal in non-verbal language – good receptive language, desire to communicate
   c. By 24 months late talkers have approximately 20 words in their expressive vocabulary
   i. Typically developing peers have approximately 200 words
   ii. Diagnostic criteria = <50 words in expressive vocab and/or demonstrate no word in combinations (ie. no 2 word utterances)
4. Delay vs disorder
   a. Delay = development proceeds in a typical way but slower than expected
   i. There is an implication that catch up is possible/ expected
   b. Disorder = development is not proceeding in a typical or expected way

Question 66

Language delay - aetiology

Answer 66

5. Aetiology
   a. Primary/secondary
   b. Expressive, receptive, mixed
   c. Word sounds (phonology), modification, grammar, vocab, meanings, pragmatics
6. Causes of language delay
   a. Developmental language delay
   i. Isolated expressive delay
   ii. Mixed expressive receptive
   iii. Oral motor problems
   iv. Receptive worse than expressive (unusual and more difficult to diagnose) – genetic eg. Williams, ASD
   v. Dyspraxia (difficulty with the patterns of movement required to produce words)
   vi. Family history in 33%
   b. Global developmental delay
   c. Autism spectrum disorder
   d. Hearing loss
   e. Other
   i. Neurological/epilepsy
   ii. Behavioural (elective mutism)
   iii. Environmental deprivation

Question 67

Language delay - assessment

Answer 67

7. Factors raising concern about speech and language
   a. Parental concern regarding speech and language development
   b. History of hearing loss
   c. Delay in both receptive and expressive language skills
   d. Concern about other aspects of development and lack of developmental progress
   e. Autistic features
   f. Parental report of regression of babbling or language
   g. Family history of speech and language problems
8. Assessment

a. Language
i. Current language
ii. Development of language
iii. Understanding of language (? Context)
iv. Regression
v. Echolalia vs spontaneous speech
vi. Communicative intent
vii. Non-verbal communication
1. Protoimperative = pointing at what a child wants
2. Protodeclarative = pointing with shared interest
3. Shared attention = shared focus of 2 people on an object
viii. Oro motor function

b. Other features on history
i. Features of a more generalised problem
1. GM and FM delay
2. Social
3. Self help
ii. Features of autism – usually present well before second birthday
1. Abnormal play
2. Obsessions, repetitive interest or behaviour
3. Motor mannerisms
4. Sensory processing difficulties eg. fussy eating

5. LACK OF
   a. Pretend play
   b. Pointing out objects to another person
   c. Social interest
   d. Joint attention
   e. Social play
   f. Response to name when called
   g. Language development is delayed or disordered, there may be unusual social use of language
   h. Language regression may be seen

iii. Family history
1. Language delay/disorder
2. Stutter
3. Autism/Asperger’s
4. Epilepsy
5. Learning difficulties

iv. Social context

Question 68

Language delay - ix

Answer 68

9. Investigations
   a. Audiology
   b. Only if features of wider developmental concerns
   i. Molecular karyotype, fragile X, TFT, CK
   ii. Cognitive assessment
   iii. EEG
   iv. MRI
10. Tools for language assessment
    a. Parent report instruments
    i. Parents are very accurate in reporting on current and emerging behaviours as opposed to giving retrospective accounts of developmental milestones
    ii. Accuracy is greater with a checklist format rather than a free-response or diary method
    iii. Receptive language hard to assess on parental report
    iv. For bilingual families you want information in best language
    v. Examples
11. MacArthur Bates Communicative Behaviour Scales (CSBS) – Infant/Toddler Checklist
12. Ages and Stages Questionnaire (ASQ)
13. Receptive-Expressive Emergent Language Scale (REEL-3)
14. Brigance Infant Toddler Screen (BITS)
15. Ward Infant Language Screen
    b. Screening Assessments
    i. Renfrew Action Picture Test
16. Short, easily administered and scored screening tool for expressive language and semantic skills
17. Does NOT provide information about
    a. Receptive language
    b. Pragmatics
    ii. The Bus Story Narrative Test
    iii. Word Find Vocabulary Test
    c. Standardized Language Assessment – referral to a speech pathologist if required
    i. Clinical Evaluation of Language Fundamentals (CELF4)
18. Time = 30-60 minutes
19. Initial diagnosis of the language disorder
    a. Core, receptive and expressive language index standard scores
    b. Normative mean
20. Determines nature of the language disorder and individual language strengths and weaknesses
    a. Language Structure, Language Content, Language Memory, and Working Memory Index Scores
    ii. Preschool Language Scale (PLS-4)
    iii. Test of Language Development (TOLD)
    iv. Test of Adolescent Language (TOAL)

Question 69

Language delay - rx, prognosis

Answer 69

11. Prognosis
    a. Early language delay is a powerful predictor of later language/learning problems
    b. Early LD associated with
    i. Poorer literacy, learning and education outcomes
    ii. Effects classroom performance and school achievement (poorer grades)
    iii. Difficulties in social use of language
    iv. Impaired peer interactions and peer acceptance
    v. Social, emotional and behavioural problems
    vi. Mental health morbidity
    c. Children with isolated expressive language impairment tend to have positive outcomes
    d. Children with mixed or receptive language impairments, and those with social communication difficulties, are at higher risk
    e. Preschool children with persistent speech and language impairment tend to have
    i. Learning and social difficulties when starting formal schooling
    ii. Increased risk for later literacy and numeracy problems
    iii. Increased rate of emotional and behavioural disorders
12. Management
    a. Key principles
    i. Assess other areas of the child’s development
    ii. Refer to an audiologist to exclude hearing loss
    iii. Refer to a speech pathologist as early as possible
    iv. In cases where regression in language is suspected, refer to paediatrician
    b. Overview
    i. Involvement of family and services – childcare, kinder, school
    ii. Appropriate referrals
13. Speech
14. Other allied health
    iii. Financial support/ funding access (carers allowance, medicare plans, funding for school, NDIS)
15. Treatment
    a. Appear to result in more favourable outcome especially if diagnosed by 5 years
    b. Indirect treatment can be just effective as direct treatment
    c. Referred children may experience lowered parent/teacher expectation and increased family anxiety

Question 70

Semantic pragmatic disorders

Answer 70

a. Individuals with semantic pragmatic disorder have particular trouble understanding the meaning of what others are saying, and they are challenged in using language appropriately to get their needs met and interact with others
b. Pragmatics are able to correlate sounds with meaning
c. Consequences
i. Delayed language
ii. Word search pauses, jargon, category errors, sentence structure errors, pronoun/noun abnormalities
iii. Specific difficulty with understanding
iv. Difficult understanding stories, body language, context, jokes/satire

Uptodate: Pragmatic language refers to the skills needed to select the right words for the situation, so as to have the intended impact upon the listener. Pragmatic language skills include conventions such as taking turns in a conversation and maintaining some eye contact; contingency and topic maintenance (keeping the conversation on the same topic); adjusting the complexity of the language to suit the needs of the listener (eg, simplifying language for a younger audience; explaining terms that might not be familiar to the listener), and using nonverbal strategies such as changes in intonation, changes in facial expression, or gestures to modify the meaning of words or to convey emotion.

Question 71

Speech impairment - articulation disorders

Answer 71

a. Characterised by substitutions, omissions, additions or distortions of speech that interfere with intelligibility
b. Includes:
i. Hearing impairment
ii. Neurological impairment
iii. Apraxias/dyspraxia
iv. Structural disorders

c. Dyspraxia
i. Verbal dyspraxia is when the child cannot voluntarily coordinate their muscles to produce the right speech sounds or words
ii. May be the result of a brain injury
iii. Features
1. Perseveration - get stuck on one word or sound and say it over and over when trying to say something different
2. Difficulty sustaining normal intonation patterns
3. Very limited vocab
4. Speak slowly, lots of pauses, make searching movements with their lips and tongue.

d. Phonological delay
i. People with phonological delay can make a sound correctly but use it in the wrong position in a word or say the wrong word - for example using a d sound instead of a g sound when saying go
ii. These children are typically very difficult to understand
iii. Usually not the result of a brain injury
iv. This child can pronounce words

Question 72

Speech impairment - fluency disorders

Answer 72

2. Fluency disorders (stuttering)
   a. Interruption of flow of speaking
   b. Developmental stuttering begins between the ages of 2 and 5 years
   c. More common in males
   d. High familial incidence

Stuttering

• affects fluency of speech
• strong genetic link (50-75% have affected relative)
• onset NOT a/w anxiety, stress, personality
• features: repeated speech movements, fixed postures which include stretching out a word (prolongation) or not being able to produce any sounds (blocks), superfluous behaviours such as visible tension in the head/neck
• 12% of children stutter at some point by 4 years of age, most recover eventually
• wait up to 12 months before starting treatment (unless distress, parental concern, impacting communication), refer to speech path for LIDCOMBE PROGRAM (best evidence)
• parents should be advised to converse normally with the child and not draw attention to stutter

Question 73

Speech impairment - voice disorders

Answer 73

a. Pertain to abnormal production of vocal quality, pitch, loudness, resonance and/or duration
b. Voice disorders = ulcers, nodules, vocal polyps, cancer, endocrine changes etc
c. Resonance disorders = hypernasality + hyponasality
i. Velopharyngeal insufficiency = absence of normal closure between soft palate (velum) and pharyngeal wall, allowing air to escape into nasal quality

d. Dysphonia
i. Impairment in the ability to produce voice sounds using the vocal organs
ii. It is distinct from dysarthria which means disorders of speech, that is, an impairment in the ability to produce spoken words
iii. The dysphonic voice can be hoarse or excessively breathy, harsh, or rough, but some kind of phonation is still possible
iv. Can be pathological i.e. Vocal nodules or functional - conversion disorder, phonological delay

Question 74

Learning difficulties - bg

Answer 74

1. Key points
   a. Complex group of conditions
   b. Have NORMAL IQ – have another reason apart from intellect that learning is difficult
   c. Often present with behavioural difficulties
   d. Fits the ‘chronic disease model’ – there is no cure
   e. Defined by educational criteria, but contribution of health, developmental, behavioural and environmental factors
   f. Requires multidisciplinary assessment
2. Epidemiology
   a. Learning difficulties represent outcome of constitutional and environmental factors – prevalence 15-20%
   b. Learning disabilities/disorders thought to be neurological in nature – prevalence of 3-5%
   c. Search for neurobiological/physiological correlates disappointing
3. Definition
   a. Multiple definitions
   i. Refers to a gap between ability and achievement
   ii. Refers to discrepancy between verbal and performance scores on cognitive testing
   b. Not a discrete categorical entity, but on several continuums, and varies by type and severity

DSM criteria

• A: difficulties learning and using academic skills in reading, written expression, and maths despite adequate tuition
• B: academic skills substantially and quantifiably below age expectation, interfering with academic performance
• C: learning difficulties manifest in school years, may not be fully manifest until demands exceed limited capabilities
• D: learning difficulties NOT better accounted for by ID, decreased visual/auditory acuity, mental/neurological pathology, ESL, inadequate education instruction
• specifiers: impairments in reading, written, expression, maths

9. Patterns
   a. Reading difficulties = 80%
   b. Usually associated with spelling and writing difficulties
   c. Isolated maths or writing difficulties less common

Question 75

Dyslexia - general/brief

Answer 75

a. Specific learning disability
b. Characterised by difficulties with accurate and/or fluent word recognition and poor spelling and decoding abilities
c. These difficulties typically result from a deficit in the phonological component of language that is often unexpected in relation to other cognitive abilities and the provision of adequate classroom instruction

Question 76

Learning difficulties - comorbidities, correlations

Answer 76

7. Known correlations
   a. Poor phonological awareness
   b. Speech and language delay
   c. Working memory impairment
   d. Poor self-regulation
   e. Behaviour disorders eg. ADHD
8. Comorbidities
   a. Developmental language disorder (specific language impairment) 55-75%
   b. ADHD – other externalizing BPs 10-50%
   c. ASD 5-10%
   d. Motor delay 50%
   e. Emotional disorders
   f. Family dysfunction
   g. Medical conditions eg. Epilepsy, VLBW

Question 77

Readiness for learning/school (5 years) - components

Answer 77

a. Reading = phonological awareness, beginning knowledge of alphabet, auditory memory – plus literacy experiences
i. If a child regularly read to at 6 months onwards – they will learn quicker and better
b. Writing = motor skill, perceptual organisation
c. Maths = one to one correspondence (understand numbers represent objects), ie conceptualise quantity through numbers
d. Behavioural = attention control, integration of auditory and visual input, emotional security, self confidence

e. Experienced preschool teacher is best predictor of ‘school readiness’

Question 78

Learning difficulties - assessment

Answer 78

a. Key features
i. Child specific
ii. Measure strengths and weaknesses
iii. Individual specific management plan
iv. Implies flexible responsive school environment

b. Require
i. Multiple sources of information
ii. Elaboration of history
iii. Physical and neurological examination
iv. Neurodevelopmental assessment
v. Referral for more detailed assessments as appropriate

c. Steps of assessment
i. History
ii. Information from school questionnaire, class reports, previous assessments
iii. Examination – important for reassurance
iv. Child behaviour checklist, parents and teacher
v. Neurodevelopmental assessment
- assess: neuromaturation, gross motor, fine motor, visual motor, temporal-sequential, language (with help of questionnaire), attention and behaviour
d. Environmental factors (poverty, low SES, cultural, language)
e. Constitutional factors (genetics, health, development, gender)

Question 79

Learning difficulties - management

Answer 79

a. Key steps
i. Exclude medical conditions
ii. Interpret biomedical findings
iii. Assessment of developmental status
iv. Match environment to child
v. Coordination and follow-up
vi. Advocacy

b. Plan
i. Acute description leads logically to management
ii. Paediatric role – interpret developmental findings so that appropriate educational strategies can be planned
iii. Refer for cognitive, speech pathology, special education assessment as required
iv. Report to be understood by parents and teachers

Question 80

Cerebral palsy - bg

Answer 80

1. Definition
   a. Persistent, but not unchanging, disorder of movement and posture causing activity limitation, due to a defect or lesion of the developing brain; non-progressive
   - It is accepted that children up to five years, who acquire permanent motor impairment due to non-progressive neurological insults, have cerebral palsy. There are many causes, a wide range of manifestations of the motor disorder and various associated problems.
   - Cerebral palsy is not a single disorder but a group of disorders with diverse implications for children and their families.
   b. The motor disorders of cerebral palsy are accompanied by disturbances in sensation, perception, cognition, communication, and behaviour, by epilepsy and by secondary musculoskeletal problems
   c. NOT a single entity but a term used for a diverse group of disorders, which may relate to events in the prenatal, perinatal or postnatal period
   d. Refers to a group of conditions of variable severity with certain developmental features in common
   e. ALWAYS describe aetiology
   f. No definitive cure
2. Epidemiology
   a. 2/1000 live births – prevalence stable
   b. A child with CP born every 15 hours – 1/500 live babies
   c. Increase in prevalence in the 1980s and 1990s – due to survival of preterm infants + vigorous resuscitation
   i. Prevalence of CP in premature infants is now declining
   d. 60% of children with CP walk without aids
   e. 15% are GMFCSV
3. Risk factors
   a. Pre-term delivery = 30-80 fold increase
   b. IUGR = 10-30 fold increase
   c. Multiple pregnancy = 2 fold increase
   d. IVF = 4 fold increase
   iii. NOTE: HIE - Only 1/4 neonates with neonatal encephalopathy have CP
4. Aetiology
   a. Unknown
   b. Prenatal
   i. Prenatal hypoxia <10%
   c. Postnatal
   i. Postnatal illness 5-10%
   ii. Occurs 28 days to 2 years
   iii. Infection (50%)
   iv. TBI
   v. Stroke

Question 81

Cerebral palsy - prevention, differentials

Answer 81

5. Prevention
   a. Antenatal
   i. Measures to reduce likelihood of preterm birth
   ii. Magnesium sulphate = reduce incidence and severity of CP without affecting mortality
   iii. Delayed cord clamping = may reduce risk of IVH in preterm infants
   b. Postnatal
   i. Supportive measures
6. Adequate ventilation
7. Sufficient cerebral perfusion
8. Maintaining normal metabolic status
9. Controlling seizures
10. Treating underlying cause for encephalopathy
    ii. Therapeutic hypothermia
11. Differential diagnosis
    a. Neurological – hereditary neurodegenerative diseases (SMA), Rett syndrome, tethered spinal cord, myopathy/dystrophy, ataxic disorders (E.g. Neiman-Pick disease)
    b. Metabolic – glutaric aciduria type 1 (dystonia/choreoathetosis), urea cycle disorders (diplegia/quadriplegia)
    c. Endocrine – thyroid dysfunction
    d. Malignancy – intracranial/spinal neoplasm

Question 82

Cerebral palsy - red flags for alternate cause

Answer 82

a. Normal MRI or isolated imaging findings
b. Significant symptoms with unremarkable perinatal history
c. Think again if unremarkable perinatal history
d. Regression  NOT consistent with CP
e. Isolated hypotonia or chorea
f. Rigidity
g. Paraplegia – think of hereditary spastic paraparesis
h. Fluctuating symptoms – worse over the course of the day, symptoms worsening over time, progression
i. Family history – restless legs, mood disorder
j. Atypical phenotype
i. ADCY5 – progressive early onset chorea
ii. GNA01 – severe epilepsy, dyskinesia
iii. Dopamine responsive syndromes
iv. Benign chorea of childhood
k. Genetics
i. 1/3 of children have a genetic abnormality (compared with 2-3% in general population)
ii. Cerebral, cardiac, urinary anomalies and facial clefts common
iii. Increased risk in consanguineous families
iv. Cryptogenic CP – 48% found to have copy number variance of clinical significance; clinically distinct group, dysmorphic features more likely, non-motor comorbidity more likely

Question 83

Cerebral palsy - general presentation

Answer 83

8. Spectrum
   a. Children with cerebral palsy are an extremely heterogenous group and the degree of handicap experienced varies enormously
   b. Approximately 30% are hemiplegic, 25% diplegic and 45% have quadriparesis
   c. Some children have an isolated motor disorder
9. Clinical presentation
   a. Neurobehavioral signs
   i. Excessive docility
   ii. Irritability
   iii. Poor feeding in neonatal period
   iv. Irritably, poor sleep, frequent vomiting
   v. Difficult to handle and cuddle

b. Developmental reflexes
i. Most reflexes related to posture (tonic labyrinthine, tonic neck, Gallant) disappear between 3-6 months of age
ii. Infants with CP – delay in disappearance or exaggeration of a developmental reflex
iii. Obligatory developmental reflex (persists as long as stimulus applied) abnormal at any age

c. Motor tone + posture
i. Tone may be normal, increased or decreased
ii. Persistent or asymmetric fisting abnormal
iii. Abnormal oromotor pattern including tongue retraction and thrust, tonic bite, oral hypersensitivity, grimacing
iv. Poor head control

d. Motor milestones
i. Common delayed milestones
1. Not sitting by 8 months
2. Not walking by 18 months
3. Early asymmetry of hand function <1 year

e. Associated problems
i. Feeding difficulty

Question 84

Cerebral palsy - classification

Answer 84

a. Type of motor disorder = spasticity, dyskinesia (dystonia, athetosis), ataxia
b. Distribution = hemiplegia (35%), diplegia (30%), quadriplegia (35%)
i. Monoplegia = one limb, usually LL
ii. Hemiplegia = unilateral impairment R>L or L>R (usually UL > LL)
iii. Diplegia = impairment of all four limbs, LL>UL
iv. Triplegia = unilateral UL, asymmetric LL
v. Quadriplegia = all four limbs

c. Severity of motor dysfunction
i. Gross Motor Function Classification System
1. Reliable for children with CP <18years – descriptors vary 6-12 and 12-18 years
2. Predictive 1 (mild) to 5 (severe)
3. 60% of children walk without aids
4. Level 1 – walks without restriction, limitations in more advanced gross motor skills
5. Level 2 – walks without devices, limitations walking outdoors and in community
6. Level 3 – walks with mobility device, limitations walking outdoors and community (sit and can get in and out of frame, crutches, walker)
7. Level 4 – self mobility with devices, limitations walking outdoors and community (cannot move in/out of frame, difficult sitting, wheelchair)
8. Level 5 – self mobility severely limited even with use of supporting technology (head and trunk postures absent, dependent on wheelchair)
ii. Functional Mobility score
iii. Manual Ability Classification Scale (MACS) = fine motor
iv. Communication Function Classification System (CFCS)

Question 85

Cerebral palsy - motor subtypes

Answer 85

a. Spastic
i. Injury to UMN of pyramidal tracts or motor cortices
ii. Signs of spastic CP
1. Increased tone
2. Signs of UMN syndrome
3. Brisk deep tendon reflexes
4. Extensor plantar response
5. Clonus
iii. Subtypes
1. Spastic diplegia
2. Spastic hemiplegia
3. Spastic quadriplegia

b. Dyskinetic
i. Basal ganglia lesions which result in involuntary, uncontrolled, recurring movement
ii. Classification
1. Choreoathetosis
a. Chorea = rapid, irregular, unpredictable contractions of individual muscles or small muscle groups that involve the face, bulbar muscles, proximal extremities, and fingers and toes
b. Athetosis = slow, smooth, writhing movements that involve distal muscles
2. Dystonia = involuntary sustained contractions resulting in patterned, twisting movements of the trunk/limbs that may be slow or rapid (hypokinesia and hypotonia)

c. Ataxic
i. Due to cerebellar injury
ii. Loss of muscle coordination with impaired force, rhythm and accuracy
iii. Commonly gait and trunk ataxia, poor balance, past pointing, intention tremor, nystagmus, hypotonia

Question 86

Cerebral palsy - spastic diplegia

Answer 86

13-25%
Aetiology: PVL
Affects: Preterm infants

Infants and young children
• First few months: hypotonia of the lower limbs with delayed functional maturation
• By 6 months: spasticity involving ankle plantar flexors and hip adductors
• Crawling may be combat style

Children >5 years old
•	Contractures of affected muscles
•	LL > UL 
•	Flexion, adduction + IR of hips with contractures of hip flexors + hamstrings 
•	Flexion at elbows + knees 
•	Reduced LL strength + muscle bulk

Question 87

Cerebral palsy - spastic hemiplegia

Answer 87

21-40%
Aetiology: Neonatal stroke, Prenatal circulatory disturbances, Brain maldevelopment
Affects: Term infants with normal BW

Infants/young children
• Motor asymmetry
• Hand dominance <12 months + unable to use both hands in midline
• Unilateral
• Protective reactions (appear at 5-8 months) asymmetric
• Initial low tone + movement on affected side followed by increased tone + reflexes
• Typical posture appears by age two years in most cases

Older children >5yrs
• One side of the body is affected
• Arm > Leg
• UMN posture
• Arm = adducted at the shoulder and flexed at elbow, forearm pronated and wrist + fingers flexed with hands closed
• Leg = hip flexed and adducted, knee and ankle flexed; the foot may remain in the equinovarus or calcaneovalgus position
• Most children also have sensory deficits
• Postural abnormalities more apparent during running/walking
• Independent walking usually occurs at the appropriate age or is only slightly delayed

Question 88

Cerebral palsy - spastic quadriplegia

Answer 88

20-43%
Aetiology: Congenital infection, Cerebral dysgenesis, Perinatal or postnatal evets
Affects: SGA, late preterms

Infants/young children
•	Moderate or severe psychomotor delay
•	Poor head control
•	Spasticity from 2-3 months
•	Scissoring of legs – adduction 
•	9-10 months – unable to flex legs and poor truncal tone	

Older children >5yrs
• All limbs are affected
• UL > than or equal to LL
• Children often are severely handicapped
• Feeding difficulties, chronic respiratory insufficiency, and seizure disorder are common

Question 89

Cerebral palsy - ataxic subtype

Answer 89

4-13%
Aetiology: Early prenatal event, Frequently unknown, Genetic
Affects: Term infants

Infants/young children:
• Hypotonia and incoordination
• Motor milestones and language skills typically are delayed

Children older >5yrs
•	Ataxic movements
•	Widespread disorder of motor function
•	Ataxia usually improves with time
•	Speech typically is slow, jerky, and explosive

Question 90

Cerebral palsy - dyskinetic subtypes (general)

Answer 90

12-14%
Aetiology: Severe perinatal asphyxia, Kernicterus - choreoathetotic CP
Affects: Term infants

Infants/ <2 years 
•	Reduced spontaneous movement 
•	Hypotonia at rest, variable tone with movement or emotion
•	Oromotor incoordination
•	Persistence of primitive reflexes
•	Involuntary grimacing
•	Drooling
•	Delayed psychomotor development
•	Head can be persistently turned

Age 2-3
• Involuntary movements
• Extension patterns in the supine position + flexion with shoulder retraction in prone position
• Head usually is persistently turned to one side

Age >5yrs
• Involuntary movements
• Contractures later in life (not common)
• Variable degree of dysarthria + ID

Question 91

Choreoathetotic CP

Answer 91

Dyskinetic subtype, 12-14% (including dystonic), d/t severe perinatal asphyxia, kernicterus

• Chorea + anthesis - induced or accentuated by emotion or change in posture
• Athetosis is most apparent during reaching
• Stress, excitement, or fever may exacerbate chorea
• Primitive reflexes retained
• Oropharyngeal difficulties occur commonly

Question 92

Dystonic CP

Answer 92

Dyskinetic subtype, 12-14% (including choreoathetotic), d/t severe perinatal asphyxia, kernicterus

• Repetitive, patterned, twisting, and sustained movements of the trunk and limbs that may be either slow or rapid
• Pyramidal signs and anarthria may occur
• “Tension” = sudden involuntary ↑ tone affecting both flexor and extensor muscles, during attempted movement or emotion
• Tendon reflexes = normal or may be difficult to elicit
• Clonus + extensor plantar response absent

Question 93

Cerebral palsy - assessment and ix

Answer 93

1. History
   a. Family history, pregnancy history, maternal disease (eg. thyroid), maternal infection
   b. Drug ingestion, alcohol
   c. Gestational age
   d. Birth history, APGAR, neonatal encephalopathy, duration of NICU/ SCN stay
   i. Identify high risk groups – premature, IUGR, neonatal encephalopathy
   ii. Only 25% of children with neonatal encephalopathy
   e. Concerning features – poor feeding, poor bulbar function, irritability, seizures, fisting, bilateral signs, delay in motor milestones, poor head growth
   f. Developmental history
   g. Age at diagnosis – severe CP can be diagnosed early (<12 months)
2. Examination
   a. General tone – trunk vs limbs
   b. Abnormal tone - hypertonia, hypotonia
   c. Asymmetric tone
   d. Head control, eye movements
   e. Quality of movements, movement disorder – note dystonia can present late
   f. Primitive reflexes
   g. Reduced selective motor control
   h. Hypertonic reflexes
   i. Persistence of primitive reflexes
   j. General Movement Assessment (GMA) – quality of spontaneous movements; absent/abnormal increases risk of CP – higher ‘hit rate’ if combined with MRI
3. Investigations

a. Genetic tests - Karyotype / microarray
i. GATM gene sequencing – AGAT deficiency
ii. SLC6A8 gene sequencing – creatinine transporter deficiency
iii. WBC enzyme testing – Krabbe
iv. Arylsulfatase – MLD

b. TSH (hypothyroid)

c. Metabolic screen
i. Blood = acyl carnitine profile, carnitine, amino acids, cholesterol, copper, caeruloplasmin
ii. CSF = amino acids, glucose (GLUT-1), pyruvate/lactate (PDHD), biogenic amines (Segawa, DTDS, AADC)

d. +/- EEG if indicated

e. MRI brain = abnormal in about 85% of patients with CP
i. Abnormalities of white matter most common – 45%
ii. Grey matter injury associated with perinatal compromise
iii. Not done before 2 years of age
iv. White matter lesions more common in ambulant children
v. Basal ganglia more common in more severe GMFCS
vi. Timing
1. Brain malformations 12-20weeks
2. Periventricular white matter injury 26-34weeks
3. Cortical and subcortical gliosis 36-44weeks

4. Comorbid conditions
   a. 25-80% of all individuals with CP had additional impairments
   b. Impact on function, QOL and longevity
   i. ID 30-65%
   ii. Epilepsy 30-50%
   iii. Speech and language deficit 40%
   iv. Visual impairment 40%
   v. Hearing impairment 5-15%

Question 94

Cerebral palsy - MSK complications

Answer 94

1. Complications of spasticity
   a. Hip displacement
   b. Scoliosis
   c. Contractures + deformity
2. Hip
   a. Surveillance
   i. ALL children with CP require surveillance
3. Hip displacement common
4. GMFCSV dysfunction – almost 100% rate of hip dysplasia
   ii. Initial radiology between 12-24 month
   iii. Frequency based on GMFCSV, radiology, clinical findings
   b. Referral
   i. Migration percentage > 30%, Pain, Other orthopaedic problem
   c. Management
   i. Non-operative, Botox + bracing, Weight bearing assists with hip development
   iv. Surgical
5. Salvage surgery
6. Preventative surgery – adductor muscle release, aims to halt progression
7. Bony surgery – VDRO
   a. Femoral osteotomy +/- acetabular reconstruction
   c. Maintains stable, enlocated hips in the long-term with minimal risk of recurrence
8. Scoliosis
   a. Source of pain
   b. Pressure areas
   c. Respiratory compromise – surgery prevents decline in restrictive lung disease but does not regain function
   d. Management
   i. Bracing
   ii. Botox
   iii. Bipolar surgery – can be done prior to hip development
   iv. Spinal rodding
9. Osteoporosis
   a. Non-weight bearing
   b. Monitoring should start at 3 years and choose as indicated

Question 95

Cerebral palsy - cx: movement disorders - types

Answer 95

a. Dystonia
i. Sustained or intermittent contractions causing twisting and repetitive movement or abnormal posture
ii. Predictable, repeated, superimposed over or part of voluntary movement
iii. Often triggered by purposeful movement

b. Athetosis
i. Slow, continuous, involuntary writhing movement that prevents maintenance of a stable posture
ii. Continuous, smooth, repeated but not sustained

c. Stereotypies
i. Repetitive, simple movements that can be voluntarily suppressed

d. Tremor
i. A rhythmic back and forth or oscillating involuntary movement about a joint axis

e. Myoclonus
i. Sequence of repeated, often non-rhythmic, brief, shock like jerks due to sudden involuntary contraction or relaxation of one or more muscles

f. Chorea
i. Ongoing and random appearing sequence of one or more discrete involuntary movements or movement fragments
ii. Random due to variations in timing, duration, direction and anatomic location

g. Tics
i. Repeated, individually recognizable movements or movement fragments that are almost always briefly suppressible
ii. Associated with an awareness of an urge to perform the movement

h. Hyperkinesis/ dyskinesia
i. Any unwanted or excessive movements seen in children with neurologic disorders
ii. Usually associated with injury to BG, cerebral cortex, cerebellar or other motor pathways

i. Spasticity
i. Isokinetic movement disorder characterised by velocity dependent increased resistance to passive muscle tone
ii. Felt not seen

Question 96

Cerebral palsy - cx: movement disorders, treatment

Answer 96

a. Pharmacological
i. Oral
1. GABA-active medications = baclofen, diazepam
a. AE = sedation, weakness, constipation, nausea, headache, low mood, risk of withdrawal and gradually stop
2. Anti-epileptics = gabapentin, Keppra, topiramate
3. Anti-cholinergic = tetrabenazine, reserpine
4. DOPA-active drugs = levodopa, bromocriptine, pergolide
5. DOPA-antagonist depletors = chlorpromazine
6. Alpha agonists = clonidine, tizanidine
ii. Intrathecal baclofen
1. If severe
2. Improves endurance associated with spasticity (beware withdrawal due to mechanical failures)

b. Non-pharmacological
i. Physiotherapy
ii. Splinting/castings
iii. Orthoses (E.g. AFOs)
iv. Walking aids – brace, crutches, canes, walkers, wheelchairs

c. Surgical
i. Botulinum A toxin for localised spasticity
ii. Selective dorsal rhizotomy – severe spastic diplegia

Question 97

Cerebral palsy - GI cx summary

Answer 97

Poor dentition
Poor salivary control
Dysphagia and GORD
Incontinence and constipation
Feeding and nutrition problems

Question 98

Cerebral palsy - GI cx: poor salivary control

Answer 98

a. Highly associated with ID and epilepsy
b. Prevalence 40%

c. Consequences
i. Respiratory
ii. Aspiration
iii. Sleep disturbance
iv. Skin excoriation
v. Social

d. Contributors
i. Poor dental health
ii. Upper airway issues
iii. Intranasal congestion
iv. Pain
v. Poor control

e. Treatment
i. Referral
1. Dental review
2. Speech therapy
ii. Non-pharm
1. Tone management
2. Positioning
3. Behaviour management
iii. Pharmacological
1. Intranasal steroids – nasal congestion
2. Anticholinergics – benzhexol, glycopyrrolate
3. Anti-muscarinic – scopolamine, hysocine, probantheline
4. Alpha adrenergic agonists – clonidine
5. TCA – amitriptyline
6. All oral medications have side effects – agitation, anxiety, hallucinations, low mood, reduced heat tolerance, poor sleep, constipation, urinary retention, seizures, THICK SALIVA
iv. Surgical
1. Botox injection
a. Effective – reduction in dribble for up to 12 months
b. Complications
i. Problems with swallowing
ii. Changes in saliva consistency
2. Excision of salivary glands
3. Ligation of salivary ducts
4. Relocation of ducts

Question 99

Cerebral palsy - GI cx: dysphagia and GORD

Answer 99

a. Aetiology
i. Oropharyngeal dysphasia
ii. Bulbar dysfunction
iii. Dystonic/dyskinetic oral and tongue movements

b. Consequences
i. Choking + vomiting
ii. Underweight
iii. Increased feeding time, feeding distress + carer burden

c. Treatment
i. GORD
1. Non-pharmacological
a. Positioning
b. Thickened feeds
2. Surgical
a. PEG/GJ feeds – continuous
b. +/- fundoplication
3. Medications
a. Ranitidine
b. Omeprazole + esomeprazole
c. Eradication of H pylori

Question 100

Cerebral palsy - cx: urological

Answer 100

1. Continence
2. Neurogenic bladder
   a. Oxybutinin
   b. Monitoring renal tract/function
3. Undescended testes

Question 101

Cerebral palsy - cx: respiratory

Answer 101

1. Contributors to poor respiratory function
   a. Restrictive lung disease – scoliosis
   b. Neuromuscular weakness
   c. Aspiration – poor saliva control, GORD
   d. Recurrent infections
   e. OSA
   f. Poor sleep
2. Investigations
   a. Studies to assess aspiration/ swallow - videofluoroscopy
   b. CAB when well
   c. Overnight oximetry
   d. Sleep study
3. Treatment
   a. Pharmacological – poor sleep
   i. Melatonin, trimeprazine, chloral hydrate, SSRI, amitriptyline
   b. Aspiration
   i. PPI
   ii. PEG/fundo
   c. Neuromuscular weakness/ restrictive lung disease/ OSA
   i. Supplemental oxygen
   ii. NP airway, CPAP, BiPAP
   d. General
   i. Physiotherapy
   ii. N saline NEB
   iii. Positioning
   iv. Saliva control
   v. Tone management
   vi. Treatment of intercurrent infections
   vii. Surgical – OSA
4. Tonsillectomy
5. Adenoidectomy
6. Tongue reduction
7. Jaw distraction

Question 102

Cerebral palsy - cx: neurological

Answer 102

a. Epilepsy
i. Similar to epilepsy – valproate, carbamazepine, lamotrigine, phenytoin
ii. Diplegia 20%, hemiplegia 50%, quadriplegia 75%

b. Intellectual disability
i. Occur in 50% of individuals with CP
ii. Children benefit from cognitive assessment particularly at school entry
iii. Perceptual problems may also be identified

c. Specific learning disability
d. Perceptual problems

2. Hearing
   a. Hearing deficit = moderate degree found in 7% of children
   b. All children require hearing assessment
3. Visual impairment
   a. Visual problems = found in 45% of children with CP
4. Communication
   a. Receptive or expressive language delays
   b. Dysarthria
   c. May require communication aid such as electronic device
   d. Communication Function Classification System rates a child’s every day communication performance

Question 103

Cerebral palsy - mortality

Answer 103

• Peak in mortality in childhood and adolescence
• Mortality highest in children <15 years of age compared with age matched peers
• Mortality by age group
o Mortality twice population rate at 35 years
o 3% by 5 years, 6% by 10 years, 11% by 20 years
o Median age at death 45-55 years

• Predictors of mortality
o Include = no independent ambulation, severe intellectual impairment, epilepsy, deafness (associated with CMV, kernicterus and long NICU stay), term birth, dyskinesia, quadriplegia, comorbid additive impact on mortality (epilepsy, deafness, severe ID, lack of speech)
o Respiratory causes most common cause of direct death
o Strongest predictor of mortality is no independent ambulation (individuals with severe motor impairment had >30x the risk of mortality)
o Term birth is a risk factor – likely associated more severe motor impairment (likely marker for severe HIE)

• Discovered dead during sleep (DDDS)
o Average age 17 years
o Majority PEG fed – marker of poor bulbar function

Question 104

Salter Harris fracture classification

Answer 104

Although there are more recent and more complex classifications, the Salter-Harris classification is the most widely used and clinically useful approach to classifying and describing physeal injuries in children. About 90% of children with physeal injuries can be classified using the five Salter-Harris classifications from plain x-rays.

S (“Straight across”) – Type I (low risk for growth plate injury), Transverse fracture through the growth plate. Healing is rapid for type I fractures, within 2-3 weeks of injury and problems are rare especially in sites such as the distal radius.

A (“Above”) – Type II, fracture through the physis and the metaphysis (most common type, 75%), Transverse fracture through the growth plate and an oblique or vertical fracture through the metaphysis. Usually easy to reduce.

L (“Lower” or “BeLow”) – Type III, fracture through the physis and the epiphysis, Transverse fracture through the growth plate and a vertical fracture through the epiphysis. Displaced injuries may result in a physeal bar, leading to growth disturbance and joint incongruity, leading to arthritis. Most displaced type III injuries require open reduction internal fixation (ORIF).

T (“Two” or “Through”) – Type IV, fracture through the physis and both the metaphysis and the epiphysis (high risk for growth plate injury), Vertical fracture through all three components, metaphysis, physis and epiphysis. Most displaced type IV injuries require ORIF and long-term follow-up to detect growth disturbance.

E (“End”) or ER (“ERasure of the growth plate”) “cRush” – Type V (high risk for growth plate injury), Compression fracture or crushing of the growth plate. These injuries are almost always diagnosed retrospectively, when a growth arrest has occurred.

Refer to ortho: type III and IV, open #, neurovascular compromise

Question 105

Shoulder dislocations - general

Answer 105

1. Types of shoulder dislocation
   a. Anterior = 95%
   b. Posterior = 5%  electric shock or seizure
   c. Inferior = <1%
2. Complications
   a. Bankart lesion = injury of anterior glenoid labrum
   b. Hill-sachs lesion = cortical depression in posterolateral head of humerus
   c. Rotator cuff tear

d. Axillary nerve injury
i. Course
1. Runs inferiorly to the humeral head and wraps around the surgical neck of the humerus
ii. Muscles innervated
1. Deltoid
2. Teres minor
iii. Motor function
1. Abduction of arm at shoulder beyond first 15 degrees
iv. Sensory
1. Skin over shoulder (miliary badge distribution)

Question 106

Clavicle fractures - general

Answer 106

Location: middle third/midshaft 80%, lateral third 15%, proximal third 5% and a/w dislocation.
• Careful neurological examination should be performed to define potential (but rare) associated brachial plexus injury.
• Vascular assessment of the arm should also be performed as the subclavian artery runs closely opposed to the clavicle in the middle third.

MANAGEMENT

Middle third
• Broad arm sling to support limb for 2 weeks or until comfortable. No evidence to support Figure of 8 bandage or brace
• If age >12 years and shortened >2 cm refer to orthopaedics for opinion
• If <11 years and undisplaced, follow-up by a GP or fracture clinic is usually not required. Repeat x-rays are usually not required
• If displaced or ≥11 years, follow up with GP or fracture clinic in 1 week

Lateral third
• Broad arm sling to support limb for 2 weeks or until comfortable. No evidence to support Figure of 8 bandage or brace
• If displaced, refer to the nearest orthopaedic service on call
• Fracture clinic in 5-7 days with x-ray

Medial third
• If displaced, urgent referral to the nearest orthopaedic on call service
• Follow up to be arranged by orthopaedic service

Question 107

Proximal humerus fractures - general

Answer 107

• Most proximal humeral fractures do not require reduction as remodeling is extremely effective in the proximal humerus
• The usual treatment for this fracture is immobilisation of the shoulder in a sling, body swathe or shoulder immobilizer
• Patients should be seen in the fracture clinic or by an interested GP within 7 days for follow-up with x-rays to assess further displacement

Background
- Proximal humeral fractures represent <5% of all paediatric fractures
- FOOSH
- not commonly a/w NAI
• Infantile proximal injuries are usually transphyseal separation incurred during the birth process
o May not be seen on x-ray as the proximal humeral epiphysis appears at about six months of age.
o Ultrasound may be needed in this setting.

Management
• The proximal physis contributes 80% of the length of the humerus
• Due to the enormous remodeling potential, most of these injuries do not require reduction
• There is no role for attempted reduction in the ED.
• The older child with greater deformity may be treated with closed reduction. This is controversial and there are no agreed figures to guide closed operative reduction.
• Approximate indications are:
o 5-12 years - accept 60 degree angulation and 50% displacement
o >12 years - accept 30 degrees angulation and 30% displacement
• Isolated greater tuberosity fractures with displacement in the adolescent are an exception group in which surgical reduction and fixation is usually required.

Question 108

Humeral shaft fractures - general

Answer 108

Background
• Uncommon – account for 2-5% of all fractures in children
• Transverse and short oblique fractures are generally as a result from direct trauma, whereas spiral fractures are caused by indirect twisting, as with a fall
• Pathological fractures through a humeral simple bone cyst are relatively common after minimal trauma in children over 7 years old
• Humeral shaft fractures are the second most common birth fracture
• Spiral fractures of the humerus in toddler age and younger are strongly linked with non-accidental injury
o Careful history and examination are required to determine the child at risk.

Management
• Reduction is seldom required for humeral shaft fractures
• Fractures will usually “hang out” (i.e. under influence of gravity) to good alignment and apposition using a collar and cuff
• Mid-shaft humeral fractures should be followed up in fracture clinic at 1 week

Question 109

Supracondylar elbow fractures - general

Answer 109

Identifying fracture
• The Gartland type classification is based on the lateral x-ray, identifying where the capitellum sits in relation to a line drawn down the anterior aspect of the humerus - the anterior humeral line.
• In a normal elbow, a line drawn on a lateral view along the anterior surface of the humerus should pass through the middle third of the capitellum
• If it passes through the anterior third of the capitellum or misses the capitellum completely, the fracture is displaced posteriorly.

Complications
• Gartland type III injuries have the highest risk of neurovascular injury -> Volkmann’s ischaemia
o Compartment syndrome followed by the development of Volkmann’s ischaemic contracture

Supracondylar fractures are initially divided into two types, depending on the direction of displacement of the distal fragment:

• Flexion-type (rare) - distal fragment is displaced anteriorly
• Extension-type (98%) - distal fragment is displaced posteriorly

Type 1 (undisplaced)
- ED management
• Immobilisation in an above-elbow backslab in 90 degrees elbow flexion with sling for 3 weeks. The backslab and sling should be worn under clothing (e.g. loose fitting shirt) and not through the sleeve
• TIP: Avoid putting on a short, flimsy backslab. The backslab should extend as high above the elbow as possible (i.e. close to the axilla) and down to the metacarpophalangeal joints (MCP) joints.
- follow up
• Undisplaced fractures can be followed up with the GP in 3 weeks
• Repeat x-ray is not required.

Type 2 (Angulated fracture with intact posterior cortex)
- ED management
• Refer to the nearest orthopaedic on call service for advice
• A gentle reduction can be achieved by an anterior push on the distal fragment as the elbow is flexed to 90 degrees
• Note the exception is type II injuries with coronal plane deformity. These must always be managed by orthopaedics.
- follow up
• Observation overnight
• Fracture clinic within 7 days post-injury with x-ray of distal humerus in backslab

Type 3 (Displaced distal fragment posteriorly, no cortical contact)
- ED management
• Refer to the nearest orthopaedic on call service
• Requires urgent reduction and percutaneous pin fixation
- follow up
• To be organised by orthopaedic service

Indications for prompt consultation (ortho) include:

• Associated absence of pulse or ischaemia
• Open or impending open fracture (large anterior bruise)
• Associated nerve injuries
• Gartland type II & III fractures
• Associated same arm forearm or wrist injury
• Flexion supracondylar fractures
• Unable to achieve or maintain reduction (including if ED is not experienced in fracture reduction, splinting or casting)

Question 110

Forearm fracture dislocations - general

Answer 110

Monteggia fracture-dislocation = dislocation radial head + fracture of ulna

Galeazzi fracture-dislocation = dislocation of distal radioulnar joint + fracture of radial shaft

Both require urgent referral to orthopedics

Question 111

Distal radial fractures - general

Answer 111

0-5 years = <20 deg angulation
5-10 years = <15 deg angulation
10-15 years = <10 deg angulation

Salter Harris 1
- cx: Rarely associated with growth disturbance
- ED management
Undisplaced: Below-elbow plaster backslab or removable splint for 4 weeks
Displaced: Closed reduction and below-elbow plaster backslab for 4 weeks
Reduction is not advisable after ≥5 days of initial injury Undisplaced and displaced:
- follow up: Fracture clinic within 5 days of immobilisation

SH2
- as above

SH3
- cx: Medium risk of growth disturbance
- ED management
Refer to orthopaedics - usually requires open reduction and internal fixation (ORIF)
- f/up: Fracture clinic as per post-operative orders

SH4

• high risk of growth disturbance
• as for SH3

SH5

• high risk of growth disturbance
• rarely seen/diagnosed acutely, usually dx retrospectively
• f/up: fracture clinic as per post op orders

Question 112

SUFE - general

Answer 112

• A child presenting with a chronic SUFE will generally walk with an antalgic gait, out-toeing and some shortening of the affected limb. The child may complain of vague pain in the groin, thigh or knee.
• A very reliable clinical sign of a chronic SUFE, even when mild, is obligatory external rotation of the leg during hip flexion
• Anteroposterior (AP) and frog lateral pelvis x-rays of both hips should be obtained
• All patients with a SUFE or concern for a SUFE should be kept non-weight bearing and referred for an urgent orthopaedic assessment in the ED. The management of SUFE is always surgical.

Classification
• Ability to weight bear
o Stable - the patient is able to weight bear on the affected leg
o Unstable - the patient is unable to weight bear on the affected leg, even with crutches
• Duration of symptoms
o Acute - sudden onset of severe symptoms and inability to weight bear
o Chronic - gradual onset and progression of symptoms for > 3 weeks, without sudden exacerbation
 This is the most common presentation (85% of patients with SUFE)
o Acute on chronic - sudden exacerbation of symptoms due to acute displacement of a chronically slipped epiphysis

Aetiology + Risk Factors
• The aetiology of SUFE is unknown, but biomechanical and biochemical factors play an important role
• SUFE is relatively common and occurs between 0.2 and 10 per 100,000 population
• It is more common in boys (60%) than girls with the mean age at diagnosis being 13.5 years in boys and 12 years in girls
• Approximately 50% of adolescents with SUFE are above the 95th percentile for weight

Complications
• Osteonecrosis - the risk is up to 50% in an unstable SUFE, even with treatment
• Chondrolysis - this can result from the process of the SUFE itself, but more commonly it is from unrecognised screw/pin penetration from surgical stabilisation. The overall incidence of this is approximately 7%
• Osteoarthritis - patients with a moderate or severe SUFE have higher risk of early degenerative joint disease
• Impingement - patients with a severe SUFE have a risk of deformity through the femoral neck when the SUFE is stabilised and healed. This can cause femoral acetabular impingement, and may require further surgical treatment to correct this

Question 113

Hip dislocation - general

Answer 113

Mechanism
• Rare injury
• In older children result from significant trauma, in younger children can occur after minor trauma

Clinical diagnosis
• Patient will be uncomfortable and not want to move the affected leg
• The affected leg will appear shorter than the non-injured side
• With an anterior dislocation, the leg is abducted and externally rotated
• With a posterior dislocation, the leg is usually abducted and internally rotated and the hip in a flexed position

Classification
• Hip dislocations can be described by the direction of the dislocation
o The femoral head can be dislocated posteriorly (most common), anteriorly or very rarely inferiorly
• Hip dislocations can also be described by presence of associated injuries
o Dislocation without fracture (most common)
o Dislocation with fracture, e.g. acetabular, femoral head or femoral neck fracture

Complications
• Avascular necrosis (AVN) of the femoral head
o Most common complication
o This can occur due to disruption of the blood supply to the femoral head as a result of the injury
o The risk is reported between 3-15%
o The risk has been shown to be significantly increased if the hip is not reduced within 6 hours post-injury
• Nerve injury (2-10%)
• Femoral head over growth (coxa magna)
• Osteoarthritis – if acetabular fractures
• Recurrent dislocation and growth arrest

Question 114

Neck of femur fracture - general

Answer 114

• Uncommon in children but have serious consequences
• Generally result from high energy trauma
• Orthopaedic consult required
• Leg is in a shortened and externally rotated position

Complications
• Avascular necrosis (AVN) of the femoral head
o Most common complication following a NOF fracture
o The risk of disruption of the blood supply to the femoral head is dependent on fracture type and age
o The risk for a displaced Delbet type I fracture is up to 100%, for type II fracture is up to 61%, for type III fracture is up to 27% and for type IV fracture is 14%
• Coxa vara (neck shaft angle <120 degrees)
o Second most common complication and has been reported to occur in up to 30% of cases
o This can occur from progressive deformity in initially undisplaced fractures or from loss of reduction in displaced fractures
o More severe coxa vara can cause abductor dysfunction and result in a Trendelenburg gait pattern
• Growth arrest
o Physis of the femoral head contributes to approximately 15% of overall limb length (3-4 mm of growth /year)
o Reports of early physeal closure following NOF fractures vary in incidence of 5-65%
o It is associated with AVN and transphyseal fixation
o Premature physeal closure can result in coxa vara and limb length discrepancy
• Chondrolysis = uncommon but can be associated with osteonecrosis of the femoral head due to disruption of the blood supply to the cartilage of the femoral head
• Infection = uncommon after surgical fixation of femoral neck fractures
o It is reported to occur in less than 1% of cases

Question 115

Femoral shaft fractures - general

Answer 115

• All femoral shaft fractures should be assessed by the nearest orthopaedic on call service to evaluate the need for reduction and type of stabilisation
• Femoral shaft fractures should be put into skin traction (10% of body weight) to help with pain management.
• Represent 1.6% of all paediatric fractures

• Mechanism
o Older children = high energy trauma (e.g. motor vehicle accidents) 90%
o Younger children = usually due to falls
o Children that sustain femur fractures prior to walking age should be screened for non-accidental trauma
 In children <4 years up to 30% of femur fractures are associated with NAI
• All fractures require urgent orthopaedic assessment

Question 116

Tibial fractures - general

Answer 116

• Tibial shaft fractures are the third most common long bone fractures in children and adolescents
• Fractures of the shaft of the tibia results from direct blow or rotational force
• Direct force results in transverse fracture whereas rotational forces result in oblique or spiral fracture
• 30% associated with fibula fracture

Toddler Fracture
• Toddler fractures occur in young ambulatory children (from 9 months to 3 years)
• A toddler’s fracture is a spiral or oblique undisplaced fracture of the distal shaft of the tibia with an intact fibula
• The periosteum remains intact and the bone is stable
• These fractures occur as a result of a twisting injury

MANAGEMENT

Toddler fracture
• Treatment is supportive
• A backslab can be applied (RCH ED experience was no backslabs)
• An above-knee walking cast for 4 weeks is optional
- f/up: fracture clinic w xray 2 weeks

Undisplaced tibial shaft fracture
• No reduction is needed. Above-knee cast for 4-6 weeks (age and healing-dependent)
• Patient would benefit from procedural sedation for application of the cast
- f/up: fracture clinic w xray 1 week

Displaced tibial shaft fracture +/- fibular shaft fracture
• See acceptable reduction parameters
• Closed reduction with above-knee cast for 4-6 weeks (age and healing-dependent), non-weight bearing
• Unstable fractures may require general anaesthesic, manipulation and plaster GAMP) or fixation in theatre
- f/up: fracture clinic w xray 1 week

Question 117

Ankle fractures - general

Answer 117

Classification

• SH
• Due to the asymmetrical closure of the distal tibial physis during early adolescence, transitional fractures can also occur.
• Tillaux fracture - a Salter-Harris type III fracture involving avulsion of the anterolateral corner of the distal tibial epiphysis (the last portion of the physis to close)
• Triplane - a Salter-Harris type IV fracture, which occurs in three planes (sagittal, transverse and coronal)

Isolated undisplaced distal fibula physeal - Salter-Harris type I and II
• Below-knee cast, non-weight bearing
• Fracture clinic within 7-10 days with x-ray

Undisplaced distal tibia physeal
• No reduction required. Immobilise in above-knee cast, non-weight bearing
• For Salter-Harris type III and IV, discuss with orthopaedic on call service whether CT scan is required to confirm that fracture is truly undisplaced
• Fracture clinic within 7 days with x-ray

Displaced distal tibia physeal
• Closed reduction with above-knee cast, non-weight bearing.
• If reduction not anatomic, discuss with orthopaedic on call service
• For Salter-Harris type III and IV, refer to orthopaedic on call service
• If treated with closed reduction, fracture clinic within 5 days
• If treated operatively, to be arranged by orthopaedic service

Tillaux and triplane fracture <2 mm displacement
• No reduction. Above-knee cast, non-weight bearing
• Discuss with orthopaedic on call service whether CT scan is required to confirm that fracture is truly undisplaced
• Fracture clinic within 7 days

Tillaux and triplane fracture >2 mm displacement
• Refer to orthopaedic on call service. Typically requires operative management
• F/up to be arranged by orthopaedic service

Question 118

Klumpke palsy - general

Answer 118

Lower brachial plexus injury

1. Site of injury = Lower trunk of the brachial plexus.
2. Cause of injury = undue abduction of the arm, as in clutching something with the hand after a fall from a height, or sometimes in birth injury
3. Nerve roots involved = mainly T1 and partly C8.
4. Muscles paralysed
   a. Intrinsic muscles of the hand (T1)
   b. Ulnar flexors of the wrist and fingers (C8)
5. Deformity
   a. Claw hand due to the unopposed action of the long flexors and extensors of the fingers
   b. In a claw hand there is hyperextension at the metacarphalangeal joints and flexion at the interphalangeal joints.
6. Disability
   a. Claw hand
   b. Cutaneous anaesthesia and analgesia in a narrow zone along the ulnar border of the forearm and hand
   c. Horner’s syndrome = ptosis, miosis, anhydrosis, enophthalmos and loss of ciliospinal reflex- may be associated.
   i. Injury to sympathetic fibres to the head and neck that leave the spinal cord through nerve T1
7. Vasomotor changes
   a. The skin areas with sensory loss is warmer due to arteriolar dilation
   b. It is also drier due to the absence of sweating as there is loss of sympathetic activity
8. Tropic changes
   a. Long standing case of paralysis leads to dry and scaly skin
   b. The nails crack easily with atrophy of the pulp of fingers.

Question 119

Erb palsy - general

Answer 119

Upper brachial plexus injury

1. Causes of injury
   a. Undue separation of the head from the shoulder, which is commonly encountered in
   i. Birth injury
   ii. Fall on shoulder
   iii. During anaesthesia
2. Nerve roots involved = mainly C5 and partly C6.
3. Muscles paralysed
   a. Mainly biceps, deltoid, brachialis and brachioradialis
   b. Partly supraspinatus, infraspinatus and supinator
4. Deformity
   a. Arm = Hangs by the side, it is adducted and medially rotated
   b. Forearm = Extended and pronated
   c. The deformity is known as “Policeman’s tip hand” or “Porter’s tip hand”
5. Disability
   a. Abduction and lateral rotation of the arm (shoulder)
   b. Flexion and supination of forearm
   c. Biceps and supinator jerks are lost
   d. Sensations are lost over a small area over the lower part of the deltoid.

Question 120

Backpack palsy - general

Answer 120

• Upper brachial plexus disorder
• Clinical features
o Painless arm and/or shoulder weakness (usually unilateral)
o Sensory loss in the same distribution
o Full recovery over subsequent few months

Question 121

Hand injuries / medial and ulnar nerve - general

Answer 121

o	Median nerve
	Lateral two lumbricals 
	Opponens pollicis
	Adductor pollicis brevis
	Flexor pollicis brevis

o Ulna nerve
 All the small muscles except LOAF, dorsal interossei allowing for adduction of fingers
 Flexor carpi ulnaris
 Median aspect of flexor digitorum profundus

Question 122

Facial lacerations - general

Answer 122

• If there is periosteum on view then the deep layers should be closed to prevent a ‘divot’
• Facial lacerations may be repaired with 6/0 or 5/0 sutures
• Interrupted or subcuticular sutures may be used

Question 123

Formulas - weight

Answer 123

• 0-12 moths: Weight = (0.5x age in months) + 4
• 1-5 years: Weight = (2 x age in years) + 8
• 6-12 years: Weight = (3x age in years) + 7

Question 124

Formula - tidal volume

Answer 124

5-7mL/kg

Question 125

Formula - airways

Answer 125

OPA = centre of incisor to angle of mandible 
NPA = lateral edge of nostril to ear tragus

Laryngoscope = blade should measure from middle incisors to angle of the jaw

ETT size

• 3-3.5 <6mo
• 4.0 6-12mo
• > 12mo = age/4 +4

Question 126

Formulas - BP

Answer 126

SBP (50th centile) = Age x2 +85

Lower limit of normal for neonates: MAP gestational age in weeks

Question 127

Formulas - circulation

Answer 127

Adrenaline - 10mcg/kg
Fluids - 20mL/kg NS (can be boluses of 10)
Defib - 4J/kg

Question 128

Cardiac arrest - causes

Answer 128

a. Respiratory – HYPOXIA
i. Number 1 cause
ii. Birth asphyxia, inhalation foreign body
iii. Lower airways – infection, asthma

b. Cardiac/circulatory
i. Rarely primary cardiac disease
ii. More commonly shock – obstructive (tension, tamponade, thrombus), distributive (sepsis, anaphylaxis, neurogenic), hypovolaemic (fluid loss gastroenteritis, burns), cardiogenic

c. Neurological
i. Causing respiratory insufficiency
ii. Poisoning
iii. Convulsion
iv. Head injury
v. Infection with increased ICP

3. Outcomes
   a. Poor
   b. Worse in out of hospital arrest who arrive apneic and pulseless – almost no chance of survival if CPR >20mins

Question 129

APLS - BLS basics

Answer 129

1. Dangers
2. Responsive
3. Send for help
4. Open airway
   a. Head tit/chin lift
   b. Jaw thrust
   c. +/- suction
   d. +/- airway adjunct (nasopharyngeal airway, oropharyngeal airway)
5. Normal breathing? 2 rescue breaths
   a. Look, listen, feel
   b. If still inadequate breathing, 2 rescue breaths (mouth children, nose+mouth infants)
   i. Use bag and mask if in hospital, run high flow oxygen 10L/min
   c. Watch for chest rise and fall, if not present, reposition, if not present ?foreign body
   d. Attach pulse oximetry, monitoring
   e. Preparation for intubation in true cardiac arrest
6. Check for signs of life +/- start CPR (15:2)
   a. Start CPR within 10 seconds if
   i. There are no signs of life
   ii. You are not certain there is a pulse
   iii. There is a slow pulse <60bpm with no signs of circulation and reaction to ventilation
   ii. Infants = hand encircling technique
   iii. Children = heel of one hand over the lower half of the sternum
   c. Ratio
   i. Neonates = 3:1
   ii. Infants and children = 15:2
7. Attach defibrillator
   a. Put on to assess rhythm in ?cardiac arrest
   b. Not often required in children as not primary cardiac arrest
8. Disability
   a. GCS/ AVPU
   b. Posture –decorticate, decerebrate
   c. Pupils – size
9. Exposure
   a. Assess all aspects of body
   b. Temperature
10. Glucose – DEFG

Question 130

APLS - non shockable rhythm

Answer 130

1. Non-Shockable rhythms
   a. Asystole
   i. Most common arrest rhythm in children
   ii. Response of the young heart to prolonged severe hypoxia and acidosis is progressive bradycardia leading to asystole
   iii. ECG – straight line, occasionally P waves are seen
   b. Pulseless electrical activity (PEA)
   i. Absence of signs of life or a palpable pulse despite the presence on the ECG monitor of recognisable complexes that normally produce perfusion
   ii. May be due to identifiable and reversible cause
2. Trauma is the most often associated with a reversible cause of PEA – severe hypovolaemia, tension pneumothorax, and pericardial tamponade
3. PEA is also seen in hypothermic patients, electrolyte derangement (hypocalcaemia)
4. Massive PE
5. Management
   a. Algorithm
   i. Commence and continue CPR
   ii. Adrenaline 10 mcg/kg - IV or IO (tracheal tube if neither available) – give immediately then every 4 minutes (ie. every 2nd pause to assess rhythm)
   b. At intervals of 2 minutes briefly pause in the delivery of chest compression to assess rhythm on the monitor – if asystole remains, continue CPR
   c. If there is an organised rhythm check for signs of life and for a pulse
   d. If there is a return of spontaneous circulation (ROSC), continue post-resuscitation care, increasing ventilation to 12-20 breaths per minute
   e. If there are no signs of life and no pulse continue the protocol
6. Adjuncts
   a. Alkalising agents – routine use has not shown to be of benefit
   b. Calcium – no evidence for routine use in PEA
   c. Atropine – no place in the management of cardiac patients (ONLY used for excessive vagal tone causing bradycardia in a perfusing patient)

Question 131

APLS - shockable rhythms

Answer 131

1. Shockable rhythms
   a. VF and pulseless VT
   b. Less common in children but either can occur in sudden collapse
   c. Cause
   i. Hypothermia
   ii. TCA overdose
   iii. Cardiac disease
   d. Protocol for VF and pulseless VT is the same
2. Management
   a. Monitored setting
   i. In PICU, theatre or cath lab – if immediate identification of VF/ pulseless VT -> asynchronous defib of 4 J/kg should be carried out (3 stacked shocks may be given if onset of shockable rhythm witnessed in monitored setting)
   b. Unmonitored setting
   i. BLS should be started in response to the collapse and the identification of VF/pulseless VT will occur when the cardiac monitor is put in place
   ii. An asynchronous shock of 4 J/kg should be given immediately and CPR immediately resumed without reassessing the rhythm or feeling for a pulse – immediate resumption of CPR is vital because there is a pause between successful defibrillation and the appearance of a rhythm on the monitor
   c. Algorithm
   i. DC shock 4 J/Kg on identification of shockable rhythm
   ii. Continue CPR – pause every 2 minutes
3. Assess rhythm
4. If VF/pVT continue with sequence
5. If asystole change to asystole/PEA sequence
6. If organised electrical activity is seen check for ROSC – if present continue post resuscitation care, if absent continue the asystole/PEA sequence
   iii. Adrenaline (10 mcg/kg IV or IO) after the 2nd DC shock and then every alternate DC shock (ie. every 4 minutes)
   iv. Amiodarone (5 mg/kg IV or IO) after 3rd DC shock only
7. Other points about management
   a. Paediatric paddles for patient <10kg
   b. Intubation should be done as soon as possible along with gaining central access
   c. Consider and correct reversible causes of cardiac arrest
   d. Amiodarone – treatment of choice in shock-resistant VF and pulseless VT
   e. Lignocaine – alternative to amiodarone if unavailable (but inferior)
   f. Magnesium – indicated for children with hypoMg or with polymorphic VT (torsades)

Question 132

APLS - cardiac arrest reversible causes

Answer 132

• During resuscitation must identify and correct any reversible causes
• Two commonest causes of cardiac arrest in children are hypoxia and hypovolaemia
• Reversible causes (4Hs, 4Ts)

 Hypoxia is a prime cause of cardiac arrest in childhood and is key to successful resuscitation
 Hypovolaemia may be significant in arrests associated with trauma, anaphylaxis and sepsis and requires infusion of crystalloid
 Hyperkalaemia , hypokalaemia, hypocalcaemia and other metabolic abnormalities may be suggested by the patient ’s underlying condition (e.g. renal failure), tests taken during the resuscitation or clues given in the ECG
o Intravenous calcium (0.3 ml/kg of 10% calcium gluconate) is indicated in hyperkalaemia, hypocalcaemia and calcium channel blocker overdose
 Hypothermia is associated with drowning incidents and requires particular care: a low reading thermometer must be used to detect it
 Tension pneumothorax and cardiac tamponade are especially associated with PEA and found in trauma cases
 Toxic substances either as a result of accidental or deliberate overdose or from an iatrogenic mistake may require specific antidotes
 Thromboembolic phenomena are much less common in children than in adult

Question 133

Arrhythmias - general

Answer 133

Tachyarrythmias
• Re – entrant congenital conduction pathway abnormality (common).
• Poisoning - TCA results in VT
• Metabolic disturbance – hyperkalaemia results in VT
• After cardiac surgery.
• Cardiomyopathy.
• Long QT syndrome.

Bradyarrythmias
• Pre – terminal event in hypoxia or shock***
• Raised intracranial pressure.
• After conduction pathway damage during cardiac surgery.
• Congenital heart block (rare).
• Long QT syndrome.
• Presentations include:
• History of palpitations (verbal child).
• Poor feeding (pre – verbal child).
• Heart failure or shock
• Poisoning – digoxin, beta blockers

2. Clinical presentation
   a. History of palpitations
   b. Poor feeding
   c. Heart failure or shock
3. Primary assessment + resuscitation
   a. Airway, Breathing (oxygen/ventilation/intubation)
   c. Circulation
   i. Abnormal HR: >220 (infants) and >180 (children) abnormal, <60 abnormal
   d. Resuscitation
   i. If there is shock and HR <60 bpm: CPR, acess, IV bolus
   ii. Ventricular tachycardia
4. Synchronized DC cardioversion starting at 2 Jkg – can be increased (synchronised for haemodynamically stable patients with a pulse, cf APLS)
5. Child who is responsive to pain should be anaesthetized or sedated first
6. If synchronous shocks for VT are ineffectual (because the defibrillator cannot recognize the abnormally shaped QRS complex) then the shocks should be given asynchronously – this is more risky as there is a risk of degeneration into VF or asystole

iii. Supraventricular tachycardia
1. Give IV/IO adenosine 100 mcg/kg to a maximum single dose of 500 mcg/kg
iv. Basic bloods – FBE, renal function, glucose

Question 134

Arrhythmias - bradycardia

Answer 134

• Bradycardia is almost always a pre-terminal finding in patients with respiratory or circulatory insufficiency
o Seen as the final response to profound hypoxia and ischaemia
• Precipitated by vagal stimulation as occurs in tracheal intubation and suctioning
• Importantly also seen in patients with raised ICP
• Reassess ABC
o If hypoxia and shock
 High concentration O2, bag-mask ventilation, intubation and PPV
 Volume expansion
 Adrenaline 10 mcg/kg IV bolus
 Adrenaline 0.05-2 mcg/kg/min IV
o If vagal stimulation
 Adequate ventilation
 Atropine 20 mcg/kg IV/IO – repeat after 5 minutes

Question 135

Arrhythmias - SVT

Answer 135

• Sinus tachycardia (ST) vs SVT
o ST is typically characterised by HR <200 bpm in infants and children, whereas SVT > 220bpm
o P Waves may be difficult to identify in both – if P waves are identifiable they are usually upright in I and aVF in sinus tachycardia, while they are negative in leads II, III and AVF in SVT
o In ST the HR varies beat to beat and is often responsive to stimulation, there is no variation in SVT
o Termination of SVT is abrupt, whereas gradually slows for sinus tachycardia

• Resuscitation
o Vagal simulation
o If shocked (ie. hypotensive, poor peripheral perfusion, impaired mental state)  direct current cardioversion
o If child is not shocked  IV adenosine – 100 mcg/kg  200 mcg/kg  300 mcg/kg – max dose 500 mcg/kg (12 mg)
o If adenosine unsuccessful discuss with paediatric cardiologist
 Propranolol
 IV amiodarone – 5 mg/kg
 Flecainade - 2 mg/kg – note membrane stabiliser but can be proarrhythmic and has negative inotropic effect
o DC cardioversion

Question 136

Arrhythmias - VT with pulse (pulseless VT = APLS)

Answer 136

• Cause
o Congenital heart disease
o Myocarditis or cardiomyopathy
o Poisoning with TCA, procainamide, quinidine
o Renal disease or other cause of hyperkalaemia
o Channelopathies (long QT syndrome, catecholaminergic polymorphic VT)

• Management
o ECG = Look for characteristics of Torsades on ECG = polymorphic VT with QRS complexes that change in amplitude and polarity -> Long QT, drug poisoning (quinine, quinidine, disopyramide, amiodarone, TCA, digoxin)
o Check electrolytes

• Reassess ABC
o Haemodynamically unstable -> synchronized DC cardioversion started at 2J/kg
o Pharmacological therapy
 Amiodarone
 Procainamide
o In cases where ventricular arrhythmia caused by drug toxicity, sedation/anaesthesia and DC shock may be the safest approach
 Use synchronous shocks initially as these are less likely to produce VF
 If synchronous shocks ineffectual, subsequent attempts will have to be asynchronous if the child is in shock
o Torsades – magnesium sulphate in a rapid IV infusion
• NOTE:
o It is important not to delay safe therapeutic intervention for longer than necessary in VT as the rhythm often deteriorates into pulseless VT or VF
o Sometimes wide complex tachycardia can be SVT with BBB and aberrant conduction
 This can be difficult to distinguish
o A safe approach is to always treat of VT

Question 137

Status epilepticus - bg

Answer 137

1. Background
   a. Most convulsions are brief and self-limiting, generally ceasing within 5 – 10 minutes
   i. These seizures do not need immediate management unless they continue beyond this timeframe
   ii. The longer the duration of convulsion the more difficult it is to terminate it
   iii. Convulsions >5 minutes may not stop spontaneously
   b. Generalised convulsive status epilepticus (CSE) = generalised convulsion lasting 30 minutes or longer or when successive convulsions occur that the patient does not recover consciousness between them
   c. Seizures should be treated immediately in the following situations
   i. Child presents actively fitting (cause and duration unknown)
   ii. Known cause warranting more urgent treatment – meningitis, hypoxic injury, trauma, cardiorespiratory compromise
2. Epidemiology
   a. Status epilepticus occurs in 1-5% of patients with epilepsy
   b. Up to 5% of children with febrile seizures present with CSE
3. Cause
   a. Fever (<6 years)
   b. Meningoencephalitis
   c. Epilepsy
   d. Hypoxia
   e. Metabolic abnormalities

Question 138

Status epilepticus - assessment, cx

Answer 138

4. Assessment
   a. Any compromise to ABC
   b. Duration of seizure including pre-hospital period
   c. Significant past history including seizures, neurological comorbidity including VP shunts, renal failure (hypertensive encephalopathy), endocrinopathies (electrolyte disturbance)?
   d. Focal features
   e. Fever? (Febrile convulsion or CNS infection)
   f. Anticonvulsant medications including any acute pre hospital treatment
   g. Previously successful acute anticonvulsant management
   h. Evidence of underlying cause that may require additional specific emergency management
   i. Hypoglycemia
   ii. Electrolyte disturbance including hypocalcemia
   iii. Meningitis
   iv. Drug overdose
   v. Trauma (consider occult head trauma)
   vi. Stroke and intracranial haemorrhage
5. Complications
   a. Status epilepticus has a mortality of 4-5%
   b. Complications of prolonged convulsions = cardiac arrythmias, hypertension, pulmonary oedema, hyperthermia, DIC, and myoglobinaemia
   c. Adverse neurological outcomes (persistent epilepsy, motor deficits, learning and behavioural difficulties) are age dependent, occurring in 6% of those >3 year but 30% of those <1 year

Question 139

Status epilepticus - rx

Answer 139

5. Acute management
   a. In most situations supportive care for 5-10 minutes is appropriate – ensure adequate airway and breathing while awaiting convulsion to stop spontaneously
   b. If seizure persists or the onset has not been witnessed, pursue active management
   i. Support airway and breathing, apply oxygen by mask, monitor.
   ii. Secure IV access, check bedside BSL and send urgent specimen for calcium / electrolytes and venous blood gas. If hypoglycaemia present, correct the low sugar. Give benzodiazepine.
   iii. Repeat benzodiazepine after 5 minutes of continuing seizures.
   iv. If convulsion continues for a further 5 – 10 minutes, commence IV phenytoin or phenobarbitone. If IV access cannot be secured and seizures refractory to benzodiazepines, consider IO access.
   v. Consider pyridoxine (100mg IV) in young infants with seizures refractory to standard anticonvulsants.
   vi. Seek senior assistance if seizure not controlled. Anticipate need to support respiration. Thiopentone or Propofol and rapid sequence induction (RSI) may be required for seizure control.

APLS PATHWAY

Step 1
• If seizure lasting >5 minutes given buccal midazolam (0.5 mg/kg) or rectal diazepam (0.5 mg/kg)
• If IV/IO access is already established give IV/IO lorazepam (0.1 mg/kg)
Step 2
• If convulsion continues for 10 minutes after step 1 – second dose of benzodiazepine
• If step 1 was buccal or rectal – give IV lorazepam (0.1 mg/kg)
• Do NOT give more than 2 doses of a benzodiazepine
Step 3
• Give phenytoin 20 mg/kg IV over 20 minutes
• Consider dose of rectal paraldehyde
• If the child is already receiving phenytoin as part of epilepsy treatment – give phenobarbitone 20 mg/kg IV over 20 minutes instead of phenytoin
• IV levetiracetam and sodium valproate are also used
Step 4
• Check airway, breathing and circulation
• Full set of bloods
• Treat raised ICP
• RSI
• Midazolam infusion

Question 140

Hypertensive crisis - general

Answer 140

= severe hypertension, and divided into emergencies and urgencies

• Urgency: A severe elevation in BP WITHOUT symptoms or evidence of acute target-organ damage
• Emergency: WITH symptoms/end organ damage

Hypertensive emergencies in children most commonly manifest as hypertensive encephalopathy: severe BP elevation with cerebral edema and neurological symptoms of lethargy, coma, and/or seizures.

• Manage convulsions with lorazepam, midazolam or diazepam
• Patients with signs of raised ICP should be managed as per usual
• Goal of treatment = safe reduction in BP to alleviate the presenting symptoms whilst avoiding the optic nerve or neurological damage that may occur with too rapid a reduction
• Typically goal is to bring BP down to the 95th centile for age (or height) over 24-48 hours
• Monitoring of visual acuity and pupils is crucial during this time as lowering the BP may lead to infarction of the optic nerve heads

Labetolol	
- Alpha and beta blocker
- Titratable infusion
- Do not use in patients with fluid overload or acute heart failure 
Sodium nitroprusside 	
- Vasodilator
- Very easy to adjust dose
- Titratable infusion
- Monitor cyanide levels
Nifedipine 	
- May be difficult to control BP if given as a bolus

Question 141

Burns - bg, primary assessment

Answer 141

1. Key points
   a. Classification
   i. Scalds = generally involve water at below boiling point and contact for <4 seconds
2. Scalds that occur with liquids at higher temperature (eg. fat, steam) or in children incapable of minimizing contact (eg. young infants) – result in more serious injuries
   ii. Flame burns = high temperatures result in serious injuries
   b. Two factors determine severity
   i. Temperature
   ii. Duration of contact
   c. Most common cause of death within the first hour following burn injuries is due to smoke inhalation
   i. Smoke filled rooms contain soot particles, hot gases and noxious substances, and are depleted of oxygen – inhalation can result in cardiac arrest
3. Primary
   a. Airway
   i. Airway may be compromised either because of inhalational injury or scalds
   ii. Indicators of inhalational injury
4. History of exposure to smoke in confined space
5. Deposits around the mouth and nose
6. Carbonaceous sputum
7. Symptoms – cough/hoarseness/stridor
   iii. As oedema follows thermal injury the airway can deteriorate rapidly
   iv. Tracheal intubation may be required – can be increasingly difficult as oedema progresses therefore important to perform as soon as possible
   b. Breathing
   i. Circumferential burns to the chest or abdomen (in infants) may cause difficulty breathing
   ii. All children who have suffered significant burns should be given high flow oxygen
   c. Circulation
   i. If early shock is present, consider alternate causes
   ii. IV access should be obtained
   iii. Blood should be taken for blood glucose, carboxyHb level, Hb, UEC and cross matching
   d. Disability
   e. Exposure

Question 142

Burns - secondary assessment

Answer 142

a. Assess the burn
i. Surface area
1. Must use paediatric chart – Lund and Browder
2. Patients palm and adducted fingers cover 1% - can be used to estimate
3. The rule of 9’s cannot be used in children <14 years of age
4. Palm of hand with thumb adducted = 1% TBSA
5. <14years – modification to rule of “9’s”
a. <1 year – head and neck 18%, legs each 14%
b. For each year, one subtracted from head and 0.5% given to each leg
c. Charts available
6. >14years – rule of “9’s”
a. 9% head and neck
b. 9% arm x 2
c. 18% chest, 18% back
d. 18% leg x 2
e. 1% genitals

DEPTH
Superficial (epidermal)	
- Dry, red, Blanches with pressure	
- Painful	
- Heals: 3-6 days
Superficial partial-thickness	
- Blisters. Moist, red, weeping, Blanches with pressure 	
- Painful to temperature and air 	
- Heals: 7-21 days
Deep partial-thickness	
- Blisters (easily unroofed), Wet or waxy dry, Variable colour (patchy to cheesy white to red), Does not blanch with pressure 	
- Perceptive of pressure only	
- Heals: >21 days, Require surgical treatment 
Full thickness	
- Waxy white to leathery grey to charred and black, Dry and inelastic
- No blanching with pressure	
- Deep pressure only	
- Heals: Rare unless surgically treated 
Deeper injury into the muscle/fascia 

b. Special areas
i. Hands or feet = severe functional loss of scarring occurs
ii. Perineal burns = prone to infection and particularly difficult to manage
iii. Circumferential full or partial thickness burns of the limbs or neck may require urgent incision to relieve distal ischaemia = escharotomy

Question 143

Burns - emergency management

Answer 143

a. First aid
i. Remove the heat source – clothes, chemicals, jewellery
ii. Apply cool running water for 20 minutes
iii. Cover the burn with cling film in the interim
iv. Gel pads can be used if running water not available
v. Seek advice for chemical burns – is it acid or alkali

b. Fluids
i. Burns >10% TBSA (not including superficial burns)
ii. This fluid is in ADDITION to maintenance fluids
iii. Parkland formula = % burn x weight (kg) x 3 = total fluid replaced in 24 hours
1. Half of this in the first 8 hours
iv. Crystalloid should be used
v. Urine output – maintain 1 ml/kg/hour (in >15% maintain 2 ml/kg/hour)

c. Other tubes
i. NGT >10% TBSA = aim to start enteral feeds within 6-18 hours
ii. IDC = if perineal burns or resuscitation required

d. Analgesia and antibiotics
i. Analgesia = must be used early and increased during cleaning of the burn, dressings, exposure
ii. Analgesic administration options = oral, inhalants, intranasal, IV
iii. Antibiotics – not unless indicated by other injuries or comorbidities

e. Tetanus status – give if not up to date

f. Dressing
i. Analgesia
ii. Clean the burn with saline
iii. Deroof blisters = to avoid vascular compromise, if impedes dressing, or uncomfortable
1. NOTE: APLS says to leave blisters intact
iv. Temporary (for transfer/ assessment elsewhere) = glad wrap/ bactigras
v. Choices of dressing (face = paraffin, neck/trunk = acticoat)
1. Superficial
a. Non-stick Vaseline gauze/ mepilex
b. Absorbent gauze/ duoderm
2. Partial thickness/ full thickness (silver improves healing)
a. Nano-crystalline silver dressings eg. anticoat/ mepilex
b. Biological eg. Biobrane

Question 144

Burns - complications

Answer 144

a. CO poisoning
i. Burning of organic compounds in a low oxygen environment produces carbon monoxide
ii. Inhalation results in carboxyhaemaglobin – 200x greater affinity for oxygen molecules than Hb
iii. A high level results in cellular hypoxia as oxygen will not be released to the cells
iv. Children who have been in house fires should have carboxy Hb levels measured (NOTE: pulse oximeters show oxygen saturation regardless of Hb concentration)
v. Treatment
1. 5-20% - oxygen  speeds up removal of CO
2. >20% - urgent consideration of hyperbaric oxygen therapy

b. Cyanide
i. Burning of plastics, wool and silk can produce cyanide
ii. Assessment and treatments are complex
iii. Beware cyanide poisoning in a child who presents in a coma or with severe metabolic acidosis
iv. Antidotes are used when blood levels are >3 mg/L

c. TSS
i. Can occur following relatively small burns
ii. Results in significant mortality
iii. Suspect in a child who develops unwell with fever, rash, diarrhoea or vomiting

Question 145

Electrical injury - general

Answer 145

• Only a small percentage of electrical injuries that require hospital attention occur in children
• Alternating current (AC) produces cardiac arrest at lower voltages than does direct current (DC)
• Cardiac arrest is related to the size of the current and duration of exposure – the current is highest when the resistance is low and voltage is high
• Electrocution can be associated with injuries to any system
• Entry and exit wounds should be sought in order to form a picture of possible internal injuries
• Clinical manifestations
o Muscle tetany complicated by fracture
o Cutaneous burns
o Respiratory arrest due to contraction diaphragm/intercostal muscles
o Cardiac arrest
o Late
 Myoglobuinuria
 Delayed arrhythmia
• Management
o Switch off current
o Management follows ABCDE
o Telemetry
o Manage myoglobuinuria – IV fluids, diuretics, sodium bicarbonate

Question 146

Drowning - bg

Answer 146

• Starting BLS as soon as possible is crucial to the outcome after drowning
• Many associated injuries and underlying illnesses may be associated with submersion
• If cervical injury cannot be excluded, stabilisation should be initiated as soon as practical but should not delay rescue and the start of BLS
• Hypothermia should be actively sought and treated
• Prolonged resuscitation may be necessary and decision to stop resuscitation should be taken after all prognostic factors have been considered

1. Epidemiology
   a. Leading cause of accidental death in children <15 years
   b. Infants most commonly die in bathtubs, older children die in swimming pools, ponds and other waterways
   c. 80% preventable
   d. Type of water does not influence outcome
   e. Contaminated water may result in infection/ARDS
2. Pathophysiology
   a. Bradycardia and apnoea occur shortly after submersion due to the diving reflex
   b. As apnoea continues  hypoxia and acidosis  tachycardia and rise in BP
   c. Between 20 seconds and 5 minutes later a breakpoint is reached and breathing occurs
   d. Fluid is inhaled  glottis  laryngeal spasm
   e. Laryngospasm subsides  fluid is aspirated into the lungs  alveolitis + pulmonary edema
   f. Hypoxia by this time is severe and individual loses consciousness
   g. Bradycardia and other dysrhythmias can occur and may be fatal (VF is rare)
   h. Hypoxia is therefore the key pathological process that leads to death and needs to be corrected quickly
   i. Other pathological processes
   i. Hypothermia, Hypovolaemia, Spinal injury

Question 147

Drowning - management

Answer 147

a. Initiation of early BLS reduces mortality drastically and is the most important factor for survival
b. Mouth to nose ventilation may be easier in shallow water
c. If AED is used it is vital to dry the chest first
d. Risk of aspiration is very high and airway should be secured using ETT RSI
e. Oro or NG tube should be inserted
f. Respiratory deterioration can be delayed 4-6 hours after submersion and even children who have initially apparently recovered should be observed for at least 8 hours
i. CXR changes may occur even later

g. Hypothermia
i. Temperature (rectal or oesophageal) should be obtained ASAP and further cooling prevented
ii. Hypothermia is common following drowning and ADVERSELY affects resuscitation attempts unless treated
iii. Arrythmias are more common with hypothermia
1. VF may be refractory at temperature <30 deg - defib should be limited to 3 shocks and inotropic or anti-arrythmic drugs should not be given
2. If unsuccessful the patient should be warmed to above 30 as soon as possible when further defib may be attempted
3. Dose interval for resuscitation drugs is doubled between 30 and 35 deg
4. Resuscitation should be continued until the core temp is at least 32 deg or cannot be raised despite active measures
iv. The temperature is generally allowed to rise 0.25-0.5 deg per hour to reduce haemodynamic instability
v. Most hypothermic patients are hypovolaemic
vi. During rewarming vasodilation occurs resulting in hypotension that requires large quantities of warmed IV fluids while avoiding overfilling and pulmonary oedema
vii. Therapeutic hypothermia (32-34) for 24 hours has been shown to improve neurological outcome in some patients and may be of benefit to children who remain comatose

h. Infection
i. Prophylactic antibiotics have NOT been shown to be helpful but are often given after immersion in contaminated water
ii. Fever is common in first 24 hours but not necessarily a sign of infection
iii. GN especially Pseudomonas aeruginosa and Aspergillus species have been reported

i. Raised ICP
i. Signs of raised ICP may develop due to post-hypoxic injury and should be treated
ii. Aggressive treatment to lower ICP has not been shown to improve prognosis

Question 148

Drowning - prognosis

Answer 148

4. Prognostic factors
   a. Immersion time = >10 minutes very small chance of intact neurological recovery or survival
   b. Time to BLS = starting BLS at scene reduces mortality, delay of >10 minutes associated with poor prognosis
   c. Time to first respiratory effort
   i. If this occurs within 3 minutes of BLS – prognosis is good
   ii. If there has been no respiratory effort after 40 minutes of CPR there is little or no chance of survival unless the child’s respiration has been depressed (eg. hypothermia, medication, alcohol)
   d. Core temperature
   i. Pre-existing hypothermia and rapid cooling protects vital organs and improves the prognosis
   ii. Core temp <33 deg on arrival and water temp <10 deg associated with increased survival
   e. Persisting coma = GCS <5 bad prognosis
   f. Arterial pH = pH <7.1 despite treatment poor prognosis
   g. Arterial blood PO2 = if <60 mmHg despite treatment poor prognosis
5. Outcome
   a. 70% of children survive drowning when BLS is provided at the scene
   b. 40% of children survive without early BLS, even with maximum therapy
   c. Of those who survive having required full CPR 70% will make a complete recovery and 25% will have mild neurological deficit

Question 149

Drowning - hypothermia, rewarming strategies

Answer 149

External rewarming sufficient if core temp >30
If <30, active core rewarming should be added
Extracorporeal warming is preferred in arrest

Active rewarming

• warm IV fluid to 39 degrees
• warm ventilator gas to 42
• gastric or bladder lavage with normal saline 42 deg
• pleural or pericardial lavage
• endovascular warming
• extracorporeal blood rewarming

External warming

• remove cold/wet clothes
• warm blankets
• warm air system
• heating blanket
• infrared radiant lamp

Question 150

Choking - general

Answer 150

1. Key points
   a. Most common in pre-school children
   b. Suspect if
   i. Sudden onset respiratory distress
   ii. Coughing, gagging, stridor
2. Management
   a. If foreign body easily visible then remove it
   i. Take care not to push it further into the airway
   ii. Do NOT perform blind finger sweep of the mouth or upper airway
   b. Physical methods (described below) to clear the airway should only be performed if
   i. The diagnosis of FBAO is clear cut (witnessed or strongly suspected) and ineffective coughing and increasing dyspnoea, loss of consciousness, or apnoea have occur
   ii. Head tilt/jaw thrust have failed to open the airway of an apnoeic child
   c. SUMMARY
   i. Send for help
   ii. If effective cough – encourage coughing
   iii. If ineffective cough
3. Conscious = 5 back blows then 5 chest thrusts
4. Unconscious = open airway, 2 breaths, CPR 15:2
   a. Each time breaths are attempted look in the mouth for FB
   d. Definitive management
   i. If apneic/obstructed – immediate direct laryngoscopy with McGills forceps removal
   ii. If severe resp distress/cough ineffective – urgent laryngoscopy if back blows/chest thrust ineffective
   iii. If lower airways – removal through bronchoscopy
   e. Gaseous induction preferred
   f. Do not do abdominal thrusts EVER

Question 151

FB ingestion - bg

Answer 151

1. Key points
   a. The majority of ingested foreign bodies are low risk – no imaging or intervention
   b. High risk objects are button batteries and magnets – require imaging
   c. Larger sized objects may cause complication, and require surgical review
2. Epidemiology
   a. Highest incidence in 6months – 4 years
   b. Coins account for 70% of ingested foreign bodies
3. High risk
   a. Button batteries if lodged in oesophagus: immediate removal
   i. If they pass to stomach, they are benign
   b. Objects > 6cm long and/or wider than 2.5cm – can get entrapped at pylorus
   c. Magnet + metal object, or > 1 magnet
   d. Lead based objects that don’t pass through stomach -> acute lead absorption
4. Indications for urgent removal
   a. Ingested object is sharp, long (>5cm), remains in oesophagus or stomach
   b. When the ingested object is high-powered magnet or magnets
   c. When a disk battery is in the oesophagus – some cases in the stomach
   d. Signs of airway compromise
   e. Near or complete oesophageal obstruction
   f. Signs or symptoms suggestive of inflammation or intestinal obstruction

Question 152

FB ingestion - ix, intervention

Answer 152

5. Investigations
   a. X-rays can be avoided in asymptomatic child with a reliable history and without significant PMHx
   b. Consider imaging if
   i. Suspected or known button battery, magnets(s) or other high risk radio-opaque object
   ii. Unknown object
   iii. High risk or symptomatic child
   c. Features suspicious of button battery = radio-opaque foreign body, the halo effect double rim, step off on lateral X-ray
   d. NOTE: majority of metallic objects will show up on X-ray except aluminum
6. Interventions
   a. Objects seen or impacted in the oropharynx require urgent ENT evaluation
   b. Button batteries
   i. If lodged in the oesophagus require urgent referral for removal
   ii. Once in the stomach they are benign
   iii. If the battery is >15 mm and has been swallowed by a child <6 years – repeat X-ray should occur within 4 days – if it remains in the stomach removal is recommended
   c. Ingestion of multiple magnets +/- metal require early endoscopic removal
   i. One magnet seen on X-ray may still be high risk, as two may have been swallowed which give the appearance of one
   d. It is rare for sharp objects to penetrate the mucosal wall of the gut, and these require no intervention if the child is otherwise well
   e. Fishbones may lodge in tonsils and require removal
   f. A FB lodged in the lower oesophagus, and where the child is able to swallow saliva successfully, may be observed for 24 hours to ensure that it transits
   i. If it does not pass it will need to be removed
   g. Larger objects (>6 cm long and/or >2.5 cm in diameter) in the stomach require a Gastroenterology or Surgical opinion due to the increased risk of obstruction

Question 153

FB ingestion - button batteries

Answer 153

• Harm due to the ingestion of button batteries is as a result of the electric current discharged from the battery – NOT leakage from the battery
• Lithium ion batteries are highest risk of burns and perforation
• The current causes liquefactive necrosis of tissues due to generation of sodium hydroxide
• Severe burns can occur within 2 hours of contact if lodged in areas such as the oesophagus
• Damage can continue for days to weeks due to residual alkali or weakened tissues
• Complications
o Perforation and mediastinitis
o Distillation in adjacent structures such as major vessels, or the trachea
o Vocal cord paralysis
o Strictures
• >50% are unwitnessed, 10% have symptoms
• Symptoms = vomiting +/- haematemesis, refusal to take anything orally, increased salivation, dysphagia

Question 154

Traumatic brain injury - bg

Answer 154

1. Key points
   a. Head injury is the single most common cause of trauma death in children 1-15 years
   b. Commonly results from road traffic accidents – falls second most common
   c. Falls are the second most common cause of fatal head injury
   d. In infancy, the most common cause is child abuse
   e. Aim is to minimise the primary head injury and prevent evolution of secondary injury
   f. Leading cause of paediatric mortality and disability
   g. Only 4-7% presenting with minor head injury have brain injury on CT
   h. 0.5% require neurosurgery
2. Pathophysiology
   a. Primary TBI = damage incurred as a direct consequence of the impact
   b. Secondary brain injury = further damage to the CNS by secondary insults

Question 155

Traumatic brain injury - primary vs secondary injury

Answer 155

3. Primary damage
   a. Injury to neural tissue
   i. Focal cerebral contusions and lacerations (direct impact and contrecoup)
   ii. Diffuse axonal injury (shearing injury)
   b. Injury to intracranial blood vessels
   i. Extradural haematoma (especially middle meningeal artery)
   ii. Subdural haematoma (especially dural bridging vessels)
   iii. Intracerebral haematoma
   iv. Subarachnoid haemorrhage
4. Secondary damage
   a. Ischaemia from poor cerebral perfusion secondary to raised ICP
   b. Ischaemia secondary to hypotension and anaemia
   c. Hypoxia
   d. Hypoglycaemia and hyperglycaemia
   e. Fever
   f. Convulsions
   g. Later infection

Question 156

Traumatic brain injury - assessment and rx

Answer 156

5. Primary survey + resuscitation
   a. As per usual algorithm
   b. AVPU – rapid assessment of conscious state
   c. Indications for immediate intubation + ventilation
   i. Coma – GCS <8
   ii. Loss of protective laryngeal gag reflexes
   iii. Ventilatory insufficiency
   iv. Spontaneous hyperventilation
   v. Respiratory irregularity
   vi. Significantly deteriorating conscious level
   vii. Unstable facial fractures
   viii. Copious bleeding into mouth
   ix. Seizure
6. Secondary survey
   a. History
   b. Examination
   c. Investigations
   i. Bloods
   ii. CT brain
7. Emergency treatment
   a. Intubation – indications as above
   b. Management of raised ICP

Question 157

Traumatic brain injury - calculators for CTB

Answer 157

Risks

• radiation -> brain malignancy
• Risk of cancer 24% more likely in those with prev CTB
• dose related

PECARN (main one I’ve seen)

• large study, well validated
• risk of clinically important TBI 0.02% if zero of 6 predictors present (GCS 15, no signs base of skull fracture, no signs AMS, no LOC/vomiting, no severe headache or severe mechanism)

CHALICE

• sensitive but not specific
• estimates suggest if used in Aus rate of CTB would double

CATCH

Question 158

Extradural haematoma - general

Answer 158

• Potential space between dura and skull

1. Aetiology = temporal-parietal skull fracture resulting in dural artery rupture
   a. Most commonly due to ruptured middle meningeal artery (MMA)
2. Epidemiology
   a. Young adult, male > female = 4:1
   b. Rare before the age of 2
3. Clinical presentation
   a. Classic sequence
   i. Post-traumatic LOC
   ii. Lucid interval of several hours
   iii. Obtundation, ipsilateral pupillary dilation and coma
   b. Signs and symptoms depend on severity
   i. Headache, nausea + vomiting, amnesia, altered LOC, HTN and respiratory distress (Cushing’s)
   ii. Deterioration can take hours to days
4. Investigations
   a. CT = high density biconvex mass against skull
   i. Limited by suture lines
   ii. Uniform density + sharp margins
5. Treatment
   a. Admission, observation, serial CT
   b. Craniotomy to evacuate clot, follow-up CT
6. Prognosis
   a. Good with prompt management, as brain often not damaged
   b. Worse prognosis if bilateral Babinski or decerebration pre-op
   c. Death usually due to resp arrest from uncal herniation (injury to midbrain)

Question 159

Subdural haematoma - general

Answer 159

• Potential space between dura and arachnoid membranes

1. Definition = rupture/tear of bridging veins that cross the subarachnoid space and empty into the dural venous sinuses (or cerebral laceration); becomes symptomatic within a day or two after a precipitating event
2. Aetiology = trauma (minor head injury)
   - shaken baby syndrome (consider NAI)
3. Risk factors
   a. Trauma
   b. Acceleration-deceleration injury
   c. Anticoagulants
   d. Alcohol = d/t increased risk of injury and increased risk of bleeding
   e. Epilepsy
   f. Cerebral atrophy (age)
   g. Infant head trauma

Question 160

Subdural haematoma - sx

Answer 160

No lucid period
Most common intracranial bleed and most commonly seen in infants

Symptoms 	
•	Confusion or lethargy 
•	Focal neuro symptoms eg. numbness
•	Headache
•	Dizziness
•	Nausea or vomiting
•	Ataxia	

Signs
• Impaired consciousness
• Focal neuro deficit – contralateral hemiparesis or hemiplegia, aphasia
• Bradycardia + hypotension (Cushing’s)
• Seizures
• Unilateral pupillary dilation = late sign of uncal herniation
• Evidence of trauma

Question 161

Subdural haematoma - ix/rx/prog

Answer 161

5. Investigations
   a. CT = hyperdense concave Crescentic mass
   i. Crosses suture lines
   ii. Usually less uniform, less dense, and more diffuse than extradural haematoma
   b. MRI = may be used to detect brain injury; not done initially
6. Treatment
   a. Craniotomy = evacuate haematoma
   b. Prophylactic anti-epileptics = phenytoin
   c. Correction of coagulopathy = stop antiplatelet or anticoagulant agents
   i. Vitamin K, FFP, platelets, protamine (patients on heparin)
   d. Measures to reduce raised ICP
7. Complications
   a. Cerebral herniation
   b. Ischaemic injury = as ICP rises cerebral perfusion pressures may fall
   c. Neurological deficits (d/t above)
   d. Coma
   e. Epilepsy
8. Prognosis = poor overall since brain parenchyma is often injured

Question 162

Subarachnoid haemorrhage - bg

Answer 162

1. Definition = bleeding into the subarachnoid space
   • Blood widely distributes throughout the subarachnoid space
2. Epidemiology
   a. 2-5% of population have unruptured aneurysm; 1.3% rupture per year
   b. 10-15 cases per 100,000 people per year
   c. >40 years old, F>M
3. Risk Factors
   a. Strong
   i. Hypertension
   ii. Smoking
   iii. Family Hx
   iv. PCKD
   b. Weak
   i. Alcohol, cocaine abuse
   ii. Marfan’s syndrome, Ehlers-Danlos syndrome, NF-1, fibromuscular dysplasia

Question 163

Subarachnoid haemorrhage - aetiology

Answer 163

a. Aneurysm (saccular/Berry) = 70%
i. Acquired lesions with defect in medial muscle; contributed to by haemodynamic stress and smoking
ii. Risks
1. >40 years (but 10-15 years younger on average than infarction)
2. F>M
3. Associated atheroma
4. PCKD or fibromuscular dysplasia
iii. Occurs at branch points of major vessels of circle of Willis
1. 90% anterior circulation
iv. Multiple in 25-35% of cases
v. Risk of rupture if >10mm is 50% per year
1. Rupture can occur at any time but in 1/3 of cases it is associated with acute increases in ICP

b. Vascular malformations – as above = 10%
i. Often with a fistula between arterial and venous systems causing high flow through the AVM and high pressure arterialization of draining veins
ii. Can also cause focal epilepsy
iii. Risk of haemorrhage 2-3% per year (20% fatal, 30% cause permanent disability)

c. Blood dyscrasias or anticoagulant use/ not defined = 20%

Question 164

Subarachnoid haemorrhage - sx

Answer 164

Symptoms
• Split-second, overwhelming, very severe thunder-clap headache [may be located posteriorly]
• LOC occurs in 20% of patients
• Headache, drowsiness, restlessness, agitation
• Nausea + vomiting
• Seizure
• Signs of meningism after 3-12 hours
o Photophobia, neck pain (meningeal irritation)
o Low back pain or bilateral radicular pain (migration of blood in CSF down thecal sac + irritation of lumbar roots)

Signs
• Early on, normal or decreased mental status with non-focal neurological examination
• Decreased LOC = raised ICP, ischaemia, seizure
• Intra-ocular haemorrhage detected via fundoscopy

• EARLY = focal neuro signs
o 3rd nerve palsy = ptosis, pupillary dilation, impaired extra-ocular movements [indicates posterior communicating artery aneurysm compressing ipsilateral 3rd CN]
o Motor hemiparesis contralateral to side of bleed

• LATER = positive Kernig’s sign + Brudzinski’s sign (due to meningeal irritation)

• LATER STILL = increased ICP can result in bilateral 6th nerve palsy
• Other = low-grade pyrexia, intra-ocular haemorrhage

Question 165

Subarachnoid haemorrhage - ddx, ix

Answer 165

5. Differential diagnosis
   a. Intracerebral haemorrhage
   b. Pituitary apoplexy (sudden neurologic impairment) d/t haemorrhage/infarct of pituitary gland
   c. Arterial dissection
   d. First episode of migraine/cluster headache
6. Investigations
   a. CT scan without contrast = shows SAH within the first few hours: hyperdense areas in the basal cisterns, major fissures, and sulci
   b. Lumbar puncture = performed if CT scan is normal: blood + xanthochromia (yellowing)
   c. Other tests = FBC, clotting profile, electrolytes, ECG
   d. Cerebral angiography (DSA, CTA, MRA) = look for source of haemorrhage (AVM, Berry aneurysm)

Question 166

Subarachnoid haemorrhage - rx

Answer 166

7. Management

a. Stroke unit
b. Oxygen/ventilation PRN
c. NPO, bed rest, elevate head of bed 30°, minimal exertional stimulation
d. Cardiac rhythm monitor

e. Medical
i. Anti-hypertensives = no conclusive BP target ranges for managing ICH, BP as low as 140mmHg is safe
ii. ICP lowering medical management if necessary
iii. Seizure prophylaxis (phenytoin)
iv. Mild sedation
v. Ca2+ blocker = Nimodipine – vasospasm neuroprotection
f. Reducing risk of re-bleeding = antitussives, stool softeners
g. Sodium replacement = electrolyte imbalance common

h. Management of cause
i. Aneurysm = hemicraniotomy -> clipping or coil embolisation
1. Usually done 3-14 days from the haemorrhage
ii. AVM
1. Conservative
2. Ablated with endovascular treatment = catheter injection of glue
3. Surgery or stereotactic radiotherapy

Question 167

Subarachnoid haemorrhage - cx, prog

Answer 167

8. Complications
   a. Acute = hours-days
   i. Re-bleeding
   ii. Acute hydrocephalus = due to blockage in CSF resorption
   iii. Vasospasm resulting in infarction
9. Onset 4-14 days post SAH, peak at 6-8 days
10. Clinical features (delayed ischaemic deficit) = confusion, decreased LOC, focal deficit (speech/ motor)
11. Risk factors = large amount of blood on CT, smoking, increased age, HTN
12. Management = HHH (hypertension, hypervolemia, haemodilution)
    iv. Cardiac abnormalities = arrythmias
    v. Other = pulmonary oedema, hyperglycaemia, seizure, fever
    b. Long-term = neuropsychiatric problems, death, chronic hydrocephalus (meningeal inflammation and fibrosis resulting in obstruction)
13. Prognosis
    a. Mortality of 50% prior to admission to hospital
    b. Of patients surviving initial bleed, 30% die within 3 months, of these 10-20% die from further bleeding within the first month
    c. Almost half the survivors make a good functional recovery
    d. Prognostic guides = age, quantity of SAH on CT, LOC, clinical condition on admission, presence of pre-existing HT or arterial disease

Question 168

Decreased conscious state - causes

Answer 168

a. Hypoxic ischaemic injury following respiratory or circulatory failure
b. Epileptic seizures

c. Trauma
i. Intracranial haemorrhage
ii. Brain swelling

d. Infections
i. Meningitis
ii. Encephalitis
iii. Cerebral and extracerebral abscesses
iv. Malaria

e. Intoxication

f. Metabolic
i. Renal or hepatic failure
ii. Hypo or hypernatraemia
iii. Hypoglycaemia
iv. Hypothermia
v. Hypercapnia
vi. Inherited metabolic disease

g. Cerebrovascular event
h. Cerebral tumour
i. Hydrocephalus

Question 169

Glasgow coma scale

Answer 169

Verbal is different depending on age

Eyes – out of 4
4 = spontaneous 
3 = to voice
2 = to pain
1 = no response

Verbal – out of 5 (>4 years)
5 = orientated
4 = confused / disorientated
3 = inappropriate words
2 = incomprehensible
1 = no response to pain

Verbal – out of 5 (<4 years)
5 = alert; babbles, coos, words to usual ability
4 = less than usual ability, spontaneous irritable cry
3 = cries only to pain
2 = moans only to pain
1 = no response

Motor – out of 6
6 = obeys commands
5 = localizes to pain
4 = withdraws from pain
3 = decorticate to pain
2 = decerberate to pain
1 = no response

Question 170

Signs of raised ICP

Answer 170

Signs of raised ICP

a. Abnormal oculocephalic reflexes
i. When the head is turned to the right a normal response for the eyes to move away from the head movement; an abnormal response is no (or random) movement
ii. When the head is flexed, a normal response is deviation of the eyes upwards; a loss of conjugate upward gaze is a sign suggestive of raised ICP

b. Abnormal posuture
i. Decorticate = flexed arms, extended legs
ii. Decerebrate = extended arms, extended legs
iii. May need to be elicited by a painful stimulus

c. Abnormal pupillary responses – unilateral or bilateral dilatation suggests raised ICP
d. Abnormal breathing abnormalities
e. Cushing’s triad = bradycardia, hypertension and abnormal breathing pattern (LATE)

f. Other
i. Decreased GCS
ii. Increased HC, prominent scalp veins (<18months)
iii. Papilloedema
iv. CN6 palsy (false lateralizing)
1. Stretching or compression of the nerve along its intracranial course
v. Tonsillar compression – Hypertension, bradycardia, abnormal breathing
vi. Uncal herniation
1. CN3 palsy + unilateral papillary dilation
2. Pupillary dilatation occurs first – parasympathetic fibers are located on the outside of the nerve

Question 171

Raised ICP - rx

Answer 171

a. Consider intubation (indicated GCS <8) – for control of carbon dioxide and sedation (pCO2 aim 35-40mmHg) to result in vasoconstriction
b. Fluid resuscitation to maintain BP for CPP
c. Head up 20d position
d. Mannitol 2.5-5mL/kg 20% solution over 20-30mins (0.5-1g/kg dose)
e. +/- hypertonic saline 4mL/kg 3% solution over 15minutes (Na140-150)
f. +/- Dexamethasone (for oedema surrounding space-occupying lesion)
g. Seizure control – diaz, midaz, lorazepam, phenytoin load if persistent/prolonged
h. CT brain

Question 172

Lumbar puncture - general

Answer 172

Key points
A lumbar puncture (LP) should only be performed after a thorough neurological examination and once all contraindications have been considered
Performing LP should never delay life-saving interventions such as antibiotics
A normal CT scan does not exclude raised intracranial pressure
Careful preparation, adequate analgesia and an experienced assistant are critical to success

Background
LP is performed at or below the L4 level
The conus medullaris finishes near L3 at birth, but at L1-2 by adulthood
The decision to perform LP should generally be discussed with a senior clinician
It is preferable to obtain a CSF specimen prior to antibiotic administration; however, antibiotics must not be unduly delayed in a child with signs of meningitis or sepsis
In a child with fever and purpura, in whom meningococcal infection is suspected, LP may not change the management and may cause deterioration
In term infants, the seated position has been shown to be the best tolerated and to also have the best chance of obtaining CSF
If an LP is unsuccessful on two occasions, refer to a senior colleague, reassess the need for LP and consider image guidance to assist
CT scans are not helpful in most children with meningitis; a normal CT scan does not exclude raised intracranial pressure (ICP) and brain herniation may occur even in the presence of a normal CT scan

Assessment
Indications
Suspected meningitis or encephalitis
Suspected sub-arachnoid haemorrhage with a normal CT
Diagnosis, monitoring and treatment (using intrathecal medications) of a range of haematological, malignant and neurological disorders

Contraindications

Absolute
GCS <8 or deteriorating/fluctuating level of consciousness
Signs of raised intracranial pressure (ICP): diplopia, abnormal pupillary responses, decerebrate or decorticate posture, low HR + elevated BP + irregular respirations, papilloedema
A bulging fontanelle in the absence of other signs of raised ICP is not a contraindication

Relative
Septic shock or haemodynamic compromise
Significant respiratory compromise (eg apnoeas in a baby)
New focal neurological signs or seizures
Seizure within previous 30 min +/- normal conscious level has not returned following a seizure
INR >1.5 or platelets <50 or child on anticoagulant medication

Complications
Failure to obtain a specimen/traumatic bloody tap (common)
Post-dural puncture headache (uncommon) 5-15%
Transient/persistent paraesthesia/numbness (very uncommon)
Respiratory arrest from positioning (rare)
Infection introduced by needle causing meningitis, epidural abscess or osteomyelitis (very rare)
Spinal haematoma (very rare)
Brain herniation (extremely rare in the absence of contraindications above

Question 173

Trauma - primary survey

Answer 173

1. DANGER
2. RESPONSE
3. SEND FOR HELP
4. HAEMORRHAGE
   a. When catastrophic external haemorrhage obvious, treat prior to commencing ABC
   b. Apply direct pressure with dressing, tourniquet, or pack open wound
5. AIRWAYS + C-SPINE
   a. Assess patency - ?vocalizing
   b. Look, listen and feel for breathing
   c. If no evidence of airways patency - jaw thrust (esp if C spine injury), chin lift (not if C spine injury)
   d. +/- Suction under direct vision, +/- airways adjuncts (gadele airways)
   f. Prepare for intubation
   g. Surgical airways if required
   h. If suspect spinal cord injury: immobilisation, hard collar, soft collar
6. BREATHING + MANAGE ACUTE PROBLEM
   a. Effort – RR, WOB (recession, grunt, AMU, nasal flare etc), stridor/wheeze
   b. Efficacy – Auscultation for breath sounds, additional sounds, percuss
   c. Effect – HR (tachycardia, bradycardia), skin colour (pallor, cyanosis), GCS
   d. Pulse oximetry
   e. If ventilating but inadequate = high flow oxygen 15L/min through non-rebreathing mask with reservoir
   f. If inadequate breathing = support with bag-valve-mask ventilation with intermittent PPV and high flow oxygen through
   g. Identify
   i. Airways obstruction – foreign material, internal/external tissue damage
   ii. Pneumothorax – open or tension
   iii. Haemothorax
   iv. Flail chest
   v. Tamponade
7. CHECK PULSE/CPR + HAEMORRHAGE CONTROL
   a. HR and pulse volume (central and peripheral pulses)
   b. CRT
   c. BP (SBP = Age x 2 + 85)
   d. Effect – tachypnea (without WOB), skin (mottled, cold, pale), depressed GCS (agitation and drowsiness), urinary output (decreased)
   e. If inadequate circulation
   i. IV access with 2 x wide bore IVC, IO if necessary
   iii. Bloods including glucose, FBE, coags, UEC, group and cross match, VBG
   iv. 20mL/kg bolus 0.9% N/Saline
   v. Repeat 20mL/kg bolus - If requires >40mL/kg, need blood
8. DISABILITY
   a. AVPU = alert, responds to void, responds to pain, unresponsive
   b. Posture = decorticate, decerebrate
   c. Pupils = size, reactivity
   d. Note breathing pattern or Cushing’s response
   e. Management
   i. If “PU” likely need ventilation
   ii. Manage seizures
   iii. Manage increased ICP
9. EXPOSURE
   a. Assess all aspects of body – rash (urticaria, Petichiae), traumatic bruising
   b. Temperature ?hypothermia ?febrile
10. DON’T EVERY FORGET GLUCOSE
    a. Manage BSL <3mmol/L with 2mL/kg 10% Dex followed by infusion
11. OTHER EXAMINATION
    a. Abdomen
    b. Pelvis
    c. Limbs
12. TRAUMA SERIES XR
    a. Undertaken as part of primary survey
    b. CXR, pelvic XR, +/- spinal XR

Question 174

Trauma - secondary survey

Answer 174

"AMPLE" history
o	Allergies
o	Medications
o	Past medical history
o	Last meal
o	Event
o	Particularly mechanism 

Head to toe, back to front examination
• Vital signs
• Head
o Pupils
o Conscious level
o Scalp lacerations, bruising, depressions/fractures
o Inspect for battles sign, raccoons eyes
o Rhinorrhoea, otorrhoea (CSF leak)
o Otoscopy and ophthalmoscopy (for haemorrhage)

• Face
o Palpate bones for deformity and stability
o Inspect inside mouth, and teeth
o Nose for deviated septum

•	Neck
o	Inspect for swelling or bruising 
o	Palpate midline (remember NEXUS criteria)
o	Palpate for surgical emphysema 
o	Apply collar back on if any concern

•	Chest 
o	Inspect for any bruising or lacerations 
o	Inspect neck veins 
o	Feel for tracheal deviation 
o	Palpate bones for tenderness, crepitus
o	Percuss
o	Listen to breath sounds
o	Listen to heart sounds

•	Abdomen
o	Inspect for bruising and lacerations
o	Observe of movement
o	Palpate for tenderness, rigidity, masses
o	Auscultation for bowel sounds

•	Pelvis
o	Inspect for bruising or deformity
o	Inspect perineum
o	Inspect external urethral meatus
o	Palpate for stability over ASIS (only once)

•	Extremities
o	Inspect for deformity
o	Palpate bones for injury/haematoma
o	Assess joints for crepitus
o	Assess power, sensation 
o	Assess pulses for vascular supply

•	Back 
o	Observe for swelling and bruising 
o	Palpate for tenderness, swelling
o	Assess motor and sensory function
o	Usually no need to do PR unless exclusion of urethral disruption (males), rectal penetration of pelvic bones, or spinal cord

• Consider further investigations based on findings

Question 175

Indications for urgent CTB following trauma

Answer 175

GCS<13 at any point
Focal neurology
Suspected skull fracture
Dangerous mechanism
>3 distinct episodes of vomiting
Amnesia >30 minutes
Seizure

Question 176

Abdominal trauma in children - general

Answer 176

1. Children at more risk
   a. Small size – multiple systems involved
   b. Thin abdominal wall
   c. Ribs pliable and less protective
   d. Liver and spleen take up more abdominal cavity than adults
   e. Diaphragm horizontal
   f. Adult seat belts ill fitting
2. Types of injuries
   a. Solid organ
   i. Spleen
   ii. Liver
   iii. Pancreas (handlebar)
   b. Intestinal injury – mesentery injury, DJ flexure, intestinal rupture
   c. Genitourinary – bladder, kidney, urethra rupture
3. Management
   a. IV access x2 bloods including lipase and cross match
   b. Fluid bolus +/- blood
   c. Tranexamic acid for internal haemorrhage
   d. ALL handlebar injuries warrant paediatric surgical review
4. Imaging
   a. CTAP – gold standard
   b. FAST scan controversial – 66% sensitivity, 95% specificity to detect haemoperitoneum compared to CT
   i. Better sensitivity/ specificity if hypotensive
   c. Laparotomy is rarely required

Question 177

Spinal trauma - bg

Answer 177

1. Background
   a. Rare in children
   b. For any mechanism of injury capable of causing cervical spine damage the cervical spine is presumed to be at high risk
2. Immobilisation
   a. Manual immobilsation = used if child is unconscious, uncooperative or has had significant mechanism of injury that makes it possible to have a spinal injury
   i. Adjuncts can be used – head blocks, sand bags, vacuum mattress
   ii. Spinal boards only used in the short term
   b. Other points
   i. Remove from spinal board on arrival to hospital
   ii. Manual immobilization
   iii. No chin lift, jaw thrust only
   iv. Hard collar
   v. Imaging
   vi. Soft collar if required for longer period

Question 178

C-spine injuries/trauma - general

Answer 178

1. Injuries
   a. Vary based on age
   i. Children < 8 tend to sustain ligamentous and disc injuries at C 1, 2 ,3 - due to relative large mass of head, flexible back and poorly supportive muscle
   ii. Children > 8 years tend to damage C 5, 6, 7
   b. Types of injury seen are:
   i. Subluxation/ dislocation without fracture
   ii. Fracture +/- subluxation or dislocation
   iii. Spinal cord injury without radiographic abnormality (SCIWORA)
2. If X-ray is normal in conscious child with clinical symptoms continue C spine immobilsation
   c. Atlantoaxial rotary subluxation is the most common injury to the cervical spine
   i. Child presents with torticollis following trauma
   ii. CT or MRI may be necessary
3. Clearing cervical spine
   a. No midline cervical tenderness on direct palpation
   b. No focal neurological deficit
   c. Normal alertness
   d. No intoxication
   e. No painful distracting injuries
   f. If above rules apply – clear with examination
   i. No midline tenderness
   ii. No midline pain with left, right rotation
   iii. Full ROM
   g. If above criteria not met = IMAGING indicated
   i. X ray (AP, lateral + odontoid only >5yrs) – if GCS15 and no clinical features of spinal cord injury
   ii. CT imaging – if GCS <15 (certainly <14) or any other clinical features of injury
   iii. MRI imaging – if spinal cord or nerve root compression
4. Imaging
   a. Indicated in all children who cannot have their spine cleared clinically
   b. Cervical spine X-ray
   i. Adequacy
   ii. Alignment (anterior vertebral line, posterior vertebral line, facet line, spinolaminar line - should all be smooth with no steps or angulation, even in pseudosubluxation)
   iii. Bone
5. Review outline of each vertebra in turn
6. Spaces between the facet joints and the gaps between adjacent spinous processes should be similar
7. Joint between the odontoid peg and the anterior arch of the atlas should be <5mm
   iv. Cartilage and soft tissue
8. Abnormal widening of the pre vertebral soft tissues may indicate a haematoma due to C-spine injury
9. Acceptable soft tissue thicknesses:
   a. Above the larynx: <1/3rd of the vertebral body width
   b. Below the larynx (C3-C7): < 1 vertebral body width
   v. Discs
10. height of vertebral discs should be similar from C2/C3-C7
    c. MRI = if plain views abnormal or inadequate
    d. CT = Children with GCS <13 require CT of cervical spine and CTB

Question 179

Pseudosubluxation of C2 on C3 - general

Answer 179

a. Physiological anterior displacement of C2 on C3 in children
b. Common in children <7 years, but often preset in older children
c. Less commonly seen at C3-C4
d. More pronounced in flexion
e. Commonly confused with fracture

f. Radiographic features
i. Pseudosubluxation is based on a normal relationship of the upper cervical spine using Swischuk’s line and the absence of prevertebral soft tissue swelling.
ii. Swischuk’s line is drawn from anterior aspect of posterior arch of C1 to anterior aspect of posterior arch of C3
1. The anterior aspect of posterior arch of C2 should be within 1-2 mm of this line:
a. if deviated less than 2 mm: it is consistent with pseudosubluxation, but this alone is insufficient to rule out a hangman fracture
b. if deviated more than 2 mm: it is indicative of true subluxation

Question 180

Thoracic spine injuries - general

Answer 180

• Injuries to thoracic and lumbar spine are rare in children most common in the multiply injured child
• Most common mechanism of injury is hyperflexion – and the most common radiographic finding is a wedge or beak shaped vertebra resulting from compression
• Most important clinical sign is a sensory level

Question 181

SCIWORA - general

Answer 181

• Spinal Cord Injury Without Obvious Radiological Abnormality
• Occurs when spinal cord injured without accompanying injury to vertebral column
• Cervical spine is affected mort frequently
• Because the upper segments of the cervical spine have the greatest mobility, the upper cervical cord is most susceptible to injury
• Children who are seriously injured should have immobilsation until such time as a full neurological assessment can be carried out
• Normal X-rays do not exclude cord injury – if any doubt MRI should be obtained

Question 182

Shock - bg

Answer 182

1. Key points
   a. Defined as inadequate delivery of oxygen to meet metabolic demands
   b. Leads to anaerobic tissue metabolism and lactic acidosis
   c. Any sign of shock indicates dehydration >10% has occurred
   d. Shock can occur without dehydration (depends on fluid replacement into intravascular space as shock is clinical diagnosis)
   e. Circulating volume
   i. Infant 80mL/kg
   ii. Older child 70mL/kg
   f. Loss of 25% of circulating volume will cause shock unless replaced by fluid in interstitial space
2. Pathophysiology
   a. Compensated – reversible
   i. Neurohormonal mechanisms adequate to maintain vital organ function (brain, heart, kidneys) but decreased flow
   ii. Signs – tachycardia, pallor, decreased CRT, confusion/agitation, decreased urine output
   b. Uncompensated – reversible
   i. Failure of neurohormonal mechanisms resulting in anaerobic metabolism, lactic acidosis, and DIC
   ii. Signs – tachycardia, hypotension, delayed CRT, decreased GCS, olig/anuria, acidotic breathing
   c. Irreversible
   i. Irreversible damage due to cellular damage
   ii. Despite haemodynamic correction, multiple organ failure

Question 183

Shock - types

Answer 183

Hypovolemic (cold)
Haemorrhage
GIT loss – gastroenteritis, stomal losses
Third space – peritonitis
Burns 

Distributive (WARM)
Sepsis
Anaphylaxis
Spinal cord injury (neurogenic)

Cardiogenic (cold)
Arrhythmias
Obstruction – valvular disease
CCF/CM

Obstructive (cold)
Trauma – flail, haemothorax
Pneumothorax
Pulmonary embolus
Tamponade 

Dissociative (cold)
Profound anaemia
Carbon monoxide poisoning
Methaemoglobinaemia

Question 184

Shock - general management

Answer 184

a. Airways
b. Breathing

c. Circulation
i. Gain IV access with 2 wide bore IVCs, consider IO if not successful
ii. Bloods – VBG (including Ca, lactate), glucose, FBE, UEC, LFT, coags, cross match, +/- septic screen including cultures
iii. 20mL/kg N/saline bolus
1. Caution in underlying renal/cardiac disease- consider 10mL/kg
2. Increased ICP- 10mL/kg to increase CPP but not worsen oedema
3. Reassess to look for signs of improvement
iv. Repeat bolus if required
1. After 2 x boluses, need to consider
a. 3rd bolus colloid (4% albumin)
b. Inotropes
i. Cold = dobutamine (myocardial), adrenaline
ii. Warm = dopamine, noradrenaline (peripheral effects)
c. +/- invasive monitoring, RSI to ventilate
v. Strict fluid balance = IDC
vi. Consider antibiotics
1. Most common cause shock therefore if in doubt give
2. Usually cefotaxime/ceftriaxone unless suspect toxic shock (diffuse erythema, high fever, mucous membrane changes including strawberry tongue and cracked lips) when give fluclox/vancomycin + clindamycin

d. Disability
i. Consciousness AVPU
ii. Pupils
iii. Posturing

e. Exposure
i. Ensure full inspection for bruising related to trauma, non-blanching petechial rash of meningococcal sepsis
ii. Take temperature ?fever

f. Glucose

Question 185

Inotropes - general

Answer 185

Vasopressor = causes constriction and increases MAP
Inotropes = both cardiac and vascular effects including cardiac contractility and chronotropy 

Inotropy = contraction of myocardium 
Lusitropy = relaxation of myocardium
Chronotropy = firing of the SA node (heart rate)
Dromotropy = conduction velocity of the AV node 

Noradrenaline

• Vasopressor
• Alpha 1
• Use: Septic shock, Cardiogenic shock, Hypovolaemic shock

Adrenaline

• Vasopressor, Chronotrope
• Alpha (high dose), Moderate beta-1 and beta-2 effects at low dose Anaphylactic shock
• Use: Add on for septic shock

Dopamine

• Dose dependent
• Alpha 1 = high dose, Beta 1 = medium dose, Dopamine 1 = low dose
• Use: 2nd line for septic shock
• ↑ splachnic blood flow

Dobutamine

• Inotrope
• ↑ Cardiac contractility
• Beta 1
• Use: Low output, Cardiogenic shock, 2nd line for septic shock

Milrinone

• Inotrope
• Beta 1
• Use: Refractory cardiogenic shock to increase cardiac contractility
• ↑ HR ↓ BP

Question 186

Sepsis - general bg

Answer 186

• Clinician judgement is the best performing tool for early recognition of paediatric sepsis; vital signs are dynamic and prone to confounding, hypotension is a late sign, and screening blood tests have not been validated
• Initial management includes obtaining intravenous access, sampling for blood culture and venous blood gas, and early administration of empiric intravenous antibiotics
• Fluid resuscitation should be titrated carefully to avoid the harms associated with inadequate and excessive administration (FEAST trial)
• Inotropes and vasopressors may be safely administered via peripheral intravenous cannula in children during initial resuscitation

2. Pathogens
   <3 months: E. coli (GNB), GBS (GPC), Listeria – uncommon (GPB)
   Older children: Neisseria meningitiditis (GNC), Staphyloccocus pnuemoniae (GPC), Staphylococcus aureus (GPC), GAS (GPC), MRSA (GPC)
3. High risk groups
   a. Neonates
   b. Immunocompromised children
   c. Children with central venous access devices
4. Presentation
   a. Cold shock
   i. Characterised by a narrow pulse pressure and prolonged capillary refill
   ii. The underlying haemodynamic abnormality is septic myocardial dysfunction, which is more common in infants and neonates
   b. Warm shock
   i. Characterised by a wide pulse pressure and rapid capillary refill
   ii. The underlying haemodynamic abnormality is vasoplegia (low systemic vascular resistance), which is more common in older children and adolescents

Notes
b. In an unwell child, resuscitation should not be delayed by procedures such as urinalysis and lumbar puncture. These procedures should, however, be performed as soon as possible once the child has been stabilised.
APLS:
• Use cefotaxime (not ceftriaxone) in premature or jaundiced infants
• If vascular access for >48 hours use vancomycin

Question 187

Sepsis - assessment and rx

Answer 187

1. RECOGNITION (0 minutes)
   a. Fever or hypothermia
   b. Tachycardia
   c. Tachypnoea +/- hypoxia
   d. Altered conscious state/ unwell appearance
   e. Hypotension
   f. Classification: cold or warm
2. INTRAVENOUS ACCESS (<15 minutes)
   a. Take blood culure, VBG, blood glucose
   b. Insert IO if cannot insert PIVC in 15 minutes
3. SIMULTANEOUS: ANTIBIOTICS (<30 minutes)
   a. Give antibiotics on cannulation
   b. If no access within 30 minutes give IM
4. SIMULTNEOUS: FLUID RESUSCITATION (<30 minutes)
   a. Initial fluid bolus as 20 ml/kg as a push (Not through pump)
   b. If required give an additional bolus to a maximum total volume of 40 ml/kg
5. INOTROPE/ VASOPRESSOR (<60 minutes)
   a. For persisting circulatory failure after 40 ml/kg
   i. Adrenaline = for cold shock (infants/neonates)
   ii. Noradrenaline = for warm shock (older children/ adolescents)

Antibiotics
Age <1 month = cefotaxime 50 mg/kg + benpen 60 mg/kg
Age >1 month = ceftriaxone or cefotaxime (50 mg/kg) + flucloxacillin (50 mg/kg)
For oncology patients = piperacillin/tazobactam

IV fluids
0.9% normal saline is the default resuscitation fluid

Respiratory Support
Normal conscious state = noninvasive ventilation (HFNC/CPAP/BiPAP)
Altered conscious state = intubation

Question 188

NAI - bg

Answer 188

1. Key points
   a. 4 per 1000
   b. Perpetrators = father, then mother’s boyfriend, then female babysitter then mother
2. Risk factors
   a. Child factors – children with special needs (E.g. DD, CP)
   b. Social factors – poverty
   c. Family factors – substance abuse, single parent, stress, PHx of personal abuse
3. Suspected feature on history
   a. Unexplained injury
   b. Severity of injury incompatible with history
   c. Injury incompatible with developmental age
   d. History changing
   e. Delay in seeking medical care

Question 189

NAI - fractures

Answer 189

1. Fractures suggestive of NAI
   a. Classic metaphyseal lesions (wide region of long bone where growth occurs)
   b. Posterior rib fractures
   c. Fractures of the scapula, sternum, and spinous processes, especially in young children
2. Most common fractures in abused infants
   a. Rib
   b. Metaphyseal
   c. Skull fractures
3. Other points
   a. Multiple fractures in various stages of healing are suggestive of abuse; nevertheless, underlying conditions need to be considered.
   b. Clavicular, femoral, supracondylar humeral, and distal extremity fractures in children older than 2 yr are most likely noninflicted unless they are multiple or accompanied by other signs of abuse.
   c. Few fractures are pathognomonic of abuse; all must be considered in light of the history.
   d. Common ‘suspicious’ bone injuries seen in abused children
4. Investigations
   a. Radiology
   i. X-ray of suspected sites
   ii. Children <3 years = bone scan + skeletal survey
   iii. Children >3 years =bone scans are used only if occult or healing fractures are suspected
5. Bone scan is unlikely to detect an occult fracture that occurred >12 months previously
   iv. CT scan indicated for skull fractures
   b. Blood tests
   i. First line
6. FBE, UEC, LFT (ALP)
7. CMP, vitamin D
   ii. Second line tests
8. Mg, copper
9. PTH
10. Syphilis serology
11. Urine metabolic screen
12. Inflammatory markers
13. Genetic tests for osteogenesis imperfecta – COL1A1, COL1A2

Question 190

NAI - bruises

Answer 190

1. Age of child
   a. Children sustain more bruises as they get older
   b. Uncommon in non-mobile infants (<1%)
   c. 17% of infants who cruise will bruise
   d. 52% of children who are walking have bruise
   e. Non-ambulatory children = RED FLAG
2. Location of bruises
   a. Inflicted
   i. Away from bony prominence
   ii. Face, back, abdomen, arms, buttocks, ears, genitalia, hands and feet
   iii. TEN (children <4) = Torso, Ears, Neck + BUTTOCKS
   b. Accidental
   i. Anterior aspect of body
   ii. Bone prominences eg. forehead, knees, shins
3. Shape or pattern
   a. Fingertip bruising
   i. Often face, limbs, trunk (shaking/squeezing) injury
   ii. Oval or round
   iii. One surface up to 4 bruises, other surface thumb imprint
   iv. Reasonable to assume significant force
   b. Tramline/tram-track bruising
   i. Linear objects – rigid or flexible
   ii. Often ascribed to discipline methods
   iii. ‘Negative imprinting’
   c. Pinch marks
   d. Slap marks
   e. Implement bruising
   i. Outline of objects on the skin
   ii. Ligatures – bruises reflecting texture and size, circumferential or partly circumferential, limbs or neck
   iii. Rope – areas of bruising interspersed with areas of abrasion
   iv. Belt/cord – loops marks, parallel lines of petechiae with central sparing
4. Investigations
   a. Basic investigations
   i. FBE, PT (INR), APTT
   ii. Consider
5. UEC, LFT, calcium
6. Fibrinogen
   b. Extended clotting profile – might be performed when the child has clinical signs that suggest an inherited/congenital coagulopathy or a medical condition that might be associated with bruising
   i. Factor VIII, IX, XI, XIII
   ii. VWF
   iii. Platelet function (PFA 100)
   iv. Lupus anticoagulant
   v. Inflammatory markers

Question 191

NAI - other injuries (fractures and bruises separately)

Answer 191

1. Burns
   a. Estimated that 15-25% due to abuse
   b. Children usually explore with hands and withdraw from hot surfaces quickly
   c. Suggestive of NAI
   i. Location
   ii. Pattern – cigarette, iron, curling iron, light bulb
   iii. Immersion scalds of hands, feet, buttocks
2. Head trauma
   a. Most common cause of death in NAI
   b. Cause = shaking or blunt head trauma
   c. Unilateral or bilateral subdural, diffuse retinal haemorrhage (60-85% of NAI head injury), diffuse brain injury

Question 192

Poisons - decontamination with charcoal

Answer 192

Experience in Geelong ED: only for paracetamol and ?beta blocker

i. Mechanism
1. Capable of binding a number of poisonous substances without being systemically absorbed
2. Reduces enterohepatic circulation
a. Examples = aspirin, barbiturates, theophylline

ii. Does NOT absorb alcohol and iron
iii. Can be given via NG or lavage tube after gastric washout
iv. Usually only if within 1 hour of presentation

v. Dose
1. Dose is at least 10x the estimated dose of poison ingested
2. Children usually given 25-50g
3. RCH: 1-2 g/kg

vi. Risks
1. Aspirated charcoal causes severe lung damage (bronchiolitis obliterans)
2. Airway protection is CRITICAL in child who is not fully conscious or in whom the projected trajectory of the poisoning is likely to result in ACS

vii. Contraindications
1. Patients with altered conscious state
2. Ethanol/glycols
3. Alkalis / corrosives
4. Metals - including Lithium, Iron compounds, potassium
5. Fluoride
6. Cyanide
7. Hydrocarbons
8. Mineral acids - Boric acid

viii. Side effects
1. Constipation
2. Altered fluid and electrolyte balance

Question 193

Poisons - decontamination with emesis, gastric lavage, whole bowel irrigation

Answer 193

b. Emesis
i. Emesis caused by ipecacuanha now rarely used
ii. Must occur within 1 hour of ingestion of the poison
iii. Indications
1. Poisons requiring removal from the body that are not bound by charcoal
2. Children at risk of developing symptoms from the poison they have taken
iv. Contraindications
1. Depressed conscious level
2. Corrosive substance

c. Gastric lavage
i. Rarely required as benefit rarely outweighs the risk
ii. Problems
1. Poor efficacy in preventing absorption when done >60 minutes after ingestion
2. Risk of aspiration pneumonitis in the less than fully conscious patient and to a lesser extent in the conscious child
3. In the conscious young child, it is psychologically traumatic and difficult
iii. Risks
1. Aspiration pneumonitis
2. Water intoxication
3. Intrabronchial instillation of fluid
iv. Contraindications
1. Corrosives, hydrocarbons, petrochemicals
v. Indicated
1. Serious poisoning when a child is already intubated for airway protection and ventilation
2. Child should be in lateral position

d. Whole bowel irrigation
i. Done with polyethylene glycol with electrolytes via NG tube
ii. Indications
1. Delayed presentation
2. Management of slow-release drugs
3. Substances not absorbed by activated charcoal (eg. iron)
4. Substances which are irretrievable by gastric lavage

Question 194

Poisons - general ED management

Answer 194

a. A = airway
b. B = breathing
c. C = circulation

i. Dysrhythmias – Na+ blockade (treat with sodium bicarbonate), potassium channel blockade (treat with magnesium sulphate)
d. D1 = disability
i. Seizures = those due to poisoning are usually generalised
1. Treat with BDZ and barbiturates
2. Phenytoin not recommended
ii. Consider drug induced syndrome – malignant hyperthermia, serotonin syndrome, and neuroleptic malignant syndrome
iii. Check BSL – treat if <4 mmol/L
e. D2 = decontamination
i. Activated charcoal
ii. Emesis
iii. Gastric lavage
iv. Whole bowel irrigation
f. D3 = drug antidotes

g. E1 = ECG
h. E2 = exposure
i. E3 = enhanced elimination
i. Urinary alkalinisation = useful for salicylate toxicity if performed meticulously
ii. Multi dose activated charcoal = if evidence of improved clinical outcome
iii. Dialysis = intermittent high flux HDx removes water soluble toxins
1. Salicylate, toxic alcohols, lithium, theophylline, valproate, barbiturates, MTX

Question 195

Potentially lethal low dose ingestions (1-3 tablets)

Answer 195

• Beta blockers eg propranolol - coma, seizures, Ventricular Tachycardia, hypoglycaemia
• Calcium channel blockers - delayed onset bradycardia, hypotension, conduction defects
• Chloroquine / hydroxychloroquine - rapid onset coma, seizures, cardiovascular collapse
• Ecstasy and other amphetamines - agitation, hypertension, hyperthermia
• Oral hypoglycaemics eg sulphonylureas - hypoglycaemia may be delayed 8 hours
• Tricyclic antidepressants - coma, seizures, hypotension, VT
• Theophylline - seizures, Supraventricular Tachycardia, tachycardia, vomiting

Question 196

Antidotes - anticholinergic syndrome

Answer 196

Physostigmine

Question 197

Antidotes - benzodiazepine

Answer 197

Flumenazil

Question 198

Antidotes - beta blocker

Answer 198

Glucagon
Isoprenaline (nonselective beta adrenoreceptor analog)
Noradrenaline

Question 199

Antidotes - calcium channel blocker

Answer 199

Calcium

Insulin/glucose

Question 200

Antidotes - iron

Answer 200

Desferrioxamine

Charcoal does not bind iron

Question 201

Antidotes - methanol

Answer 201

Ethanol

Question 202

Antidotes - opiates

Answer 202

Naloxone

Question 203

Antidotes - oral hypoglycaemics

Answer 203

Octreotide

Question 204

Antidotes - paracetamol

Answer 204

N-acetyl cysteine

Question 205

Antidotes - tricyclic antidepressants

Answer 205

Sodium bicarbonate

Question 206

Antidotes - warfarin

Answer 206

Vitamin K

Question 207

Antidotes - amphetamines

Answer 207

Beta blocker e.g. labetalol

Question 208

Antidotes - carbon monoxide

Answer 208

100% oxygen +/- hyperbaric

Question 209

Antidotes - heparin

Answer 209

Protamine

Question 210

Serotonin syndrome - background

Answer 210

1. Key points
   a. Serotonin syndrome is the clinical manifestation of excessive central and peripheral serotonergic neurotransmission
   b. It manifests as a wide variety of signs reflecting the triad of CNS, autonomic and neuromuscular dysfunction
   c. There is a spectrum of severity ranging from mild symptoms to a severe life-threating condition
2. Aetiology
   a. On introduction or increase in dose of a single serotonergic drug
   b. Change in therapy from one serotonergic drug to another without adequate washout period
   c. Drug interaction between two serotonergic agents
   d. Interaction between a serotonergic agent and an illicit drug or herbal preparation
   e. Deliberate self-poisoning with serotonergic agents
   f. Accidental ingestion of serotonergic agents
3. Pathogenesis
   a. Neurotransmitter produced from L-tryptophan
   b. Receptors 5-HT1-7
   c. Re-uptake from synaptic cleft, metabolised with neurons by MAO (A >B), or stored in vesicles
   d. Action of medications
   i. ↑ release eg. amphetamines, MDMA
   ii. ↓ re-uptake eg. SSRIs, TCAs, dextromethorphan, St John’s wort
   iii. ↓ metabolism eg. MAOIs
   iv. Direct receptor stimulation eg. sumatriptan
   v. ↑ production eg. L-tryptophan
   vi. Unknown eg. lithium

Question 211

Serotonin syndrome - sx

Answer 211

4. Clinical presentation
   a. Develops within HOURS of commencing the serotonergic agent or interacting drugs
   b. Generalised hyperreflexia is the most IMPORTANT neurological sign; lower limbs usually display more hyperreflexia and clonus than upper limbs
   SHIVERS (shivering, hyperreflexia+myoclonus, increased temperature, vital instability, encephalopathy, restlessness, sweating)

Neuromuscular excitation	
•	Hyperreflexia
•	Clonus
•	Ocular clonus
•	Myoclonus
•	Shivering
•	Tremor
•	Hypertonia or rigidity	

Autonomic effects 	
•	Hyperthermia 
•	Diaphoresis
•	Flushing
•	Mydriasis
•	Tachycardia	

CNS effects
• Agitation
• Anxiety
• Confusion

Mild	
Nausea + vomiting
Tremor
Anxiety
Diaphoresis	

Moderate	
Agitation
Muscle rigidity
Spontaneous or inducible clonus
Ocular clonus
Hyperreflexia	

Severe 
Hyperthermia
Seizures
Respiratory failure
Rhabdomyolysis
Renal failure

Question 212

Serotonin syndrome - drugs

Answer 212

Antidepressants	
MAOIs 
TCAs
SSRIs
SNRIs
Lithium
Bupropion
Tapentadol	

Analgesics 	
Tramadol
Pethidine
Fentanyl
Dextromethorphan	

Drugs of abuse	
MDMA
LSD
Amphetamines
Cocaine	

Anti-emetics
Ondansetron
Metoclopramide

5HT1 agonists
Sumatriptan

Herbs
St John’s Wort
Ginseng
Nutmeg

Other
Valproate
Appetite suppressants

Question 213

Serotonin syndrome - ddx, rx, prevention

Answer 213

DDX

• serotonin toxicity
• neuroleptic malignant syndrome (absence of clonic excitation, bradykinesia, leadpipe rigidity, extrapyramidal side effects ie Parkinsonism)
• CNS infection
• malignant hyperthermia

7. Treatment
   a. Cease inciting agent(s)
   b. If severe = sedation, intubation, paralysed + cooled
   c. Supportive
   i. Hyperthermia = fans, cooling, blanket, lukewarm water
   ii. Rigidity = BDZ, occasional non-depolarizing muscle relaxation
   iii. Rhabdomyolysis = urinary alkalinsation, increased urine output
   iv. Seizures = BDZ
   v. Serotonin receptor antagonist eg. cyproheptadine (periactin)
   d. Summary of pharmacotherapy
   i. Benzodiazepines
   ii. Cyproheptadine
   iii. Propranolol
8. Prevention
   a. Adequate counseling about over the counter medications
   b. Washout period of two weeks between drugs (five weeks for fluoxetine due to long half-life)

Question 214

Neuroleptic malignant syndrome - background, sx

Answer 214

1. Overview
   a. Life threatening complication of antipsychotic treatment (mortality 10-20%) = muscle rigidity + elevated temp
   b. Greatest incidence in young men
   c. It does not always occur immediately after starting antipsychotic treatment, and may be seen after many months or years
   d. ALL antipsychotics have been associated with NMS
   e. Can ONLY develop in association with the use of neuroleptic medication or if the patient has been withdrawn from a dopaminergic drug

FALTER (fever, autonomic instability, leucocytosis+leadpipe rigidity, tremor, elevated CK, rigid muscles)

2. Presentation = develops over a period of hours to days (contrasting serotonin syndrome) -> CLASSIC TETRAD: hyperthermia, EPS, autonomic effects, CNS effects:

Extra-pyramidal side effects	
•	Rigidity
•	Bradykinesia or akinesia
•	Abnormal movement and postures
•	Dysphagia
•	Tremor 	

Autonomic effects	
•	Tachycardia
•	Hypertension
•	Labile BP
•	Diaphoresis
•	Tachypnoea 	

CNS effects
•	Drowsiness
•	Confusion
•	Coma
•	Mutism
•	Incontinence

Question 215

Neuroleptic malignant syndrome - dx, ddx

Answer 215

3. DSM Diagnosis
   a. Severe muscle rigidity
   b. Elevated temp
   c. Other related findings (eg. diaphoresis, incontinence, changes in consciousness ranging from confusion to coma, mutism, elevated or labile BP, elevated CK)
4. Differential diagnosis = encephalitis, heat exhaustion, acute dystonia, serotonin syndrome, DTs, haemorrhagic stroke, sepsis, rhabdomyolysis

Question 216

Neuroleptic malignant syndrome - ix, rx

Answer 216

5. Investigations
   a. CK = elevated
   b. FBE = leuckocytosis
   c. LFTs = elevated transaminases
   d. OTHER – low serum iron, dehydration, metabolic acidosis, pneumonia and other infections, pulmonary emboli, kidney failure
6. Treatment
   a. Cease antipsychotic -> ECT or BDZ are good to use until starting antipsychotic
   b. Supportive; may require ICU admission
   i. Cooling
   ii. Volume replacement
   iii. Treatment of hyperkalaemia
   iv. Severe cases with rigidity involving chest wall = intubation, sedation + paralysis
   v. Sedation = for confusion, agitation
   c. Dantrolene (muscle relaxant) or bromocriptine (dopamine agonist)

Question 217

Differentiating features SS vs NMS

Answer 217

Onset

• SS: more acute in onset and resolution (hours)
• NMS: gradual, days, months

Catatonic symptoms, leucocytosis, rhabdomyolysis
- more consistent with NMS

Myoclonus and tremor -> more c/w SS (NMS more diffuse rigidity)

Reflexes

• SS: increased
• NMS: decreased

Pupils

• SS: dilated
• NMS: normal

Question 218

Anticholinergic syndrome - bg

Answer 218

Reduced cholinergic/parasympathetic activity

1. Key points
   a. Many medications may have anticholinergic effects and interaction between two or more of these medications can lead to symptoms such as an agitated delirium, mydriasis, dry mouth and tachycardia
   b. Serious symptoms include seizures, coma and cardiac conduction abnormalities and resolution of symptoms can be variable - delirium can persist for days following an acute ingestion
   c. Management involves symptomatic treatment and discussion with toxicologist when considering the need for decontamination or anticholinesterase use
2. Background
   a. Anticholinergic syndrome results from competitive antagonism of acetylcholine at central and peripheral muscarinic receptors
   b. Central inhibition leads to an agitated (hyperactive) delirium - typically including confusion, restlessness and picking at imaginary objects - which characterises this toxidrome
   c. Peripheral inhibition is variable - but the symptoms may include: hot, dry skin, flushed appearance, mydriasis, tachycardia, decreased bowel sounds and urinary retention
   d. There is a spectrum of severity ranging from mild symptoms to a life-threatening condition - although seizures, coma and cardiovascular toxicity may not be mediated by muscarinic effects, rather secondary to drug effects at other receptors, as many anticholinergic medications are active at numerous receptors / ion channels.

Question 219

Anticholinergic syndrome - agents

Answer 219

a. Atropine is the prototypical anticholinergic (anti-muscarinic) drug

“Antis”

Anti-histamines Diphenhydramine, Doxylamine, Promethazine, Chlorpheniramine, Cyproheptadine

Anti-tussives Dextromethorphan

Antidepressants Tricyclic antidepressants (Amitriptyline, Imipramine, Doxepin)

Anti-psychotics Chlorpromazine, Droperidol, Haloperidol, Quetiapine, Olanzapine

Anti-convulsants Carbamazepine

Anti-emetics/travel sickness Hyoscine (scopolamine)
Topical ophthalmoplegics Cyclopentolate, Homatropine

Plants Deadly nightshade (Atropa belladonna), jimsonweed, mandrake root, lupin beans, Angel’s Trumpet / Datura (see Figure 1)

Other Oxybutynin, benztropine, glycopyrrolate

Question 220

Anticholinergic syndrome - sx

Answer 220

"Hot as a hare":  	Fever 
 "Red as a beet:"	Flushed skin 
"Blind as a bat":  	Mydriasis = dilated 
"Dry as a bone":  	 Dry mouth, dry eyes and decreased sweating
"Mad as a hatter": 	Delirium 
“Full as a flask”  	Urinary retention 

Generally dose dependent

Mild Anticholinergic Toxicity	
Tachycardia
Flushed face
Mydriasis and blurred vision
Dry mouth and skin
Fever	

Moderate Anticholinergic Toxicity	
Agitated delirium
Urinary retention
Hypertension
Hyperthermia

Severe Anticholinergic Toxicity
CNS depression / coma
Seizures
Cardiac conduction abnormalities (QRS widening and increased QT interval)
Circulatory collapse /  hypotension
Rhabdomyolysis

Question 221

Anticholinergic syndrome - ddx

Answer 221

a. Other toxicological syndromes
i. Serotonin syndrome
ii. Neuroleptic malignant syndrome
iii. Malignant hyperthermia
iv. Salicylate toxicity

b. Non-toxicological causes
i. Encephalitis
ii. Sepsis
iii. Neurotrauma
iv. Post-ictal phenomena
v. Hypoglycaemia
vi. Hyponatraemia
vii. Behavioural disturbance

Question 222

Anticholinergic syndrome - ix, rx

Answer 222

6. Investigations
   a. Screening tests - 12 lead ECG, blood glucose and paracetamol concentration - in deliberate self-poisoning.
   b. Some cough medications may also contain paracetamol - so a concentration should be considered in these accidental ingestions as well.
   c. Consider salicylate concentrations if differential includes salicylate toxicity.
   d. Consider concentrations for specific agents if available - e.g. carbamazepine
   e. Consider UEC and CK
7. Management
   a. Stop any causative agents
   b. Active resuscitation is infrequently required
   c. Hypotension should be addressed initially with a 20ml/kg crystalloid bolus
   d. Treat seizures with benzodiazepines
   e. Treat hypoglycaemia
   f. Treat hyperthermia with cooling measures. Antipyretics are unhelpful.
   g. Agitation / delirium is typically managed with titrated diazepam (0.1mg/kg - to a max of 5-10mg) PO every 30 minutes or every 15 minutes IV until the child is resting but able to be roused
   i. Avoid haloperidol and droperidol, which may worsen anticholinergic effects
   h. Urinary retention may contribute to the agitation. Bladder scan will reveal the need for a urinary catheter
   i. There exists some controversy over the use of physostigmine - a cholinesterase inhibitor - which has been used to reduce delirium in anticholinergic syndrome

Question 223

Paracetamol toxicity - bg

Answer 223

1. Key points
   a. Paracetamol remains the most important single cause of acute fulminant hepatic failure in Western countries
   b. Delaying NAC > 8hrs after a toxic ingestion is associated with a progressive increased risk of liver injury
   c. 5% of patients with significant paracetamol toxicity will develop acute renal injury
   d. Multi-organ failure and death may occur after 3-4 days if the ingested quantity exceeds 150 mg/kg
   e. Smaller dose of paracetamol result in toxicity if
   i. Prior hepatic dysfunction
   ii. Co-ingestion of alcohol or anticonvulsants
2. Pathogenesis
   a. Pharmacokinetics
   i. Rapidly absorbed from small intestine
   ii. Peak concentrations 1-2hours, distribution within 4hrs
   iii. 20% undergoes first pass metabolism in gut wall
   iv. 80% hepatic biotransformation
   b. Liver metabolism
   i. Liver metabolises it to a toxic product – N-acetyl-p-benzoquinone imine  hepatic necrosis unless neutralized by the hepatic antioxidant glutathione
   ii. With increases in paracetamol doses, greater production NAPQI depletes glutathione stores – when glutathione depletion reaches critical level (30%) NAPQI binds to other proteins causing damage to hepatocytes

Question 224

Paracetamol toxicity - doses, pts requiring management

Answer 224

3. Doses
   a. Children aged 0-6
   i. Single ingestion > 200mg/kg (or 10g) within 8 hours
   ii. 150mg/kg (or 6g) per 24hr period over the previous 48 hours
   iii. 100mg/kg (or 4g) per 24hr period for more than 48 hours who also have symptoms (abdominal pain, vomiting)
   b. Risk factors for hepatic injury
   i. Chronic alcohol use
   ii. Use of enzyme-inducing drugs
   iii. Dehydration and prolonged
   iv. Glutathione deficiency from regular panadol dosing
4. Patients requiring management
   a. Acute ingestion of > 200mg/kg
   b. Ingestion of unknown quantity
   c. Repeated supratherapeutic ingestion of > 100mg/kg/day

Question 225

Paracetamol toxicity - stages/sx

Answer 225

Stage I	0.5-24 hours 	
•	May be asymptomatic 
•	Nausea + vomiting
•	Diaphoresis	
•	Pallor
•	Lethargy 	
•	Transaminases often normal – may rise as early as 8-12 hours 

Stage II 24-72 hours
• Symptoms may improve
• As it progresses – develop RUQ pain with liver enlargement and tenderness
• Worsening of hepatic transaminases
• Of patients that develop hepatic injury, 100% have aminotransferase elevation by 36 hours
• Elevations of prothrombin time (PT) and total bilirubin, oliguria, and renal function abnormalities

Stage III	72-96 hours 	
•	Most at risk of death 
•	Systemic symptoms of stage I reappear
•	Develop hepatic encephalopathy  	
•	Liver function abnormalities peak from 72 to 96 hours after ingestion, hyperammonemia, and a bleeding diathesis

Stage IV 4 days to 2 weeks
• Patients who survive stage III enter a recovery phase that usually begins by day four and is complete by seven days after overdose
• When recovery occurs, it is complete; chronic hepatic dysfunction is not a sequela of paracetamol overdose

Question 226

Paracetamol toxicity - rx

Answer 226

a. Gastric lavage if <1 hour after ingestion of significant amount
b. Serum paracetamol concentration at (or as soon as possible after) 4 hours post ingestion
i. Determines the need for N-acetyl cysteine (NAC) administration
ii. NO benefit to measuring <4 hours

c. Special situations
i. Indications to give NAC immediately – decision to continue treating can be made AFTER concentration
1. Presentation > 8 hours since toxic ingestion (>200 mg/kg)
2. Symptoms (RUQ tenderness or pain, N+V)
3. Slow release paracetamol >200 mg/kg or 10g (see below)
ii. Slow release paracetamol
1. NAC should be commenced in any patient who reports ingestion of 200 mg/kg or 10g of sustained release paracetamol
2. If 4 hour level is above treatment line: 24 hour infusion of NAC
3. If 4 hour level is below the line: continue NAC and another paracetamol concentration in 4 hours – can only discontinue if both below the treatment line

d. N-acetylcysteine
i. Effective if given before hepatic necrosis occurs
ii. Infusion (as per protocol…)
1. 150mg/kg over first hour
2. 50mg/kg over next 4 hours
3. 100mg/kg over next 16 hours
iii. Adverse effects = rash, bronchospasm, hypotension
iv. Delaying NAC administration > 8h post ingestion is associated with a progressive increased risk of liver injury
v. Administer if serum paracetamol level (as per chart)
1. >1000μmol/L at 4 hours
2. 500 at 8 hours
3. 80 at 16 hours
4. 40 at 20 hours
5. Serum levels >18 hours are unreliable predictors of risk

e. Blood tests
i. LFT and serum potassium
ii. All patients have elevated aminotransferases (ALT, AST) at 36 hours
iii. Stage II – elevations in PT, total bilirubin, oliguria and renal function abnormalities develop
iv. Paracetamol-induced hepatitis is acute in onset, progresses rapidly, is characterized by marked elevation of plasma aminotransferases (often >3000 IU/L), and is associated with a rising prothrombin time/international normalized ratio

Question 227

Paracetamol toxicity - prognosis

Answer 227

a. Predictors of death/need for transplant
i. Rarely required in children or adolescents
ii. Indicators of consideration of liver transplant
1. Grade III encephalopathy – or any change in mentation
2. Acute kidney injury
3. Progressive increase in INR
4. Degree of ALT elevation is not considered a prognostic factor

b. King’s college criteria for liver transplantation in paracetamol ingestion
i. Arterial pH <7.3 – irrespective of the grade of encephalopathy
OR
ii. Grade III or IV encephalopathy, AND
iii. Prothrombin time >100 seconds, AND
iv. Serum creatinine > 301 umol/L

Question 228

Iron overdose - background

Answer 228

1. Key points
   a. In iron poisoning, the amount of elemental iron ingested determines the risk, not the amount of iron salt
   i. 10-20 mg/kg – toxicity possible
   ii. If >20 mg/kg – toxicity likely
   iii. If >60 mg/kg – serious toxicity
   iv. If >150 mg/kg – fatal
   b. In severe poisonings, often there is a period of latency where symptoms subside before organ failure occurs
   c. Abdominal X-ray may be helpful if tablets have been ingested
   d. Iron poisoning is a clinical diagnosis - serum iron levels are useful in predicting the clinical course of the patient
   e. Consider whole bowel irrigation and desferrioxamine
2. Pathophysiology
   a. Iron is corrosive to the GIT
   b. Exerts its toxic effect in the free ferric state through the creation of free radicals and lipid peroxidation that cause damage to cells
   c. Protective mechanisms (such as binding to transferrin in the blood and to ferritin within cells) are quickly overwhelmed when there is an overdose

Question 229

Iron overdose - sx

Answer 229

a. Initial symptoms
i. Nausea, vomiting, diarrhoea, abdominal pain, hypotension, haematemesis, fever
ii. Gastrointestinal symptoms related to the corrosive nature of iron may occur without systemic toxicity
iii. Lack of symptoms within the first 6 hours makes significant toxicity unlikely

b. 6-24 hours
i. CVS = tachycardia, vasoconstriction, hypotension and shock
ii. Metabolic = metabolic acidosis
iii. Related to fluid shifts from intravascular to extravascular compartments and cellular hypoxia
iv. Usually appear at 6-24 hours and last 12-24 hours

c. Latent period
i. Often have a latent period (6-24 hours) where initial symptoms resolve, before overt systemic toxicity declares

d. >48 hours after ingestion
i. Multi-organ failure = gastrointestinal (ileus, gastric erosion), CNS, cardiovascular, hepatic, renal

e. 4-6 weeks
i. Chronic sequelae = cirrhosis and GI scarring and strictures, pyloric stenosis

Question 230

Iron overdose - investigations

Answer 230

a. Asymptomatic Children
i. If tablet ingestion: AXR = if negative nil further Ix or observation required
ii. If unknown amount or >40 mg/kg ingested: serum iron concentrations 4 hourly until falling

b. All symptomatic children should have the following investigations:
i. AXR (if tablet ingestion) - AXR may also be helpful in evaluating gastrointestinal decontamination after whole bowel irrigation (WBI)
ii. Blood gas (acidosis)
iii. Glucose (hyperglycaemia)
iv. Serum iron concentration
1. Should be performed immediately and repeated 4-6 hours after ingestion since concentration usually peaks at 4-6 hours after ingestion
2. Concentrations taken after 4-6 hours may underestimate toxicity because the iron may have either been distributed into tissues or be bound to ferritin
3. If slow release – repeat level 6-8 hours as absorption may be erratic and delayed
4. Once desferrioxamine is commenced concentrations inaccurate
v. FBE (leukocytosis)
vi. UEC LFTs
vii. Clotting (reversible early coagulopathy and late coagulopathy secondary to hepatic injury)
viii. Blood group and cross-match

Question 231

Iron overdose - rx

Answer 231

a. Resuscitation
i. Supportive treatment to maintain adequate blood pressure and electrolyte balance is essential.
ii. I.V. fluid resuscitation 20 mL/kg for hypovolaemia or hypotension
iii. Potassium and glucose administration as necessary

b. Decontamination
i. Activated charcoal does not bind to iron and is not indicated
ii. Decontamination of choice is whole bowel irrigation (WBI)
1. WBI is indicated if the AXR reveals tablets or capsules ingested and more than 60mg/kg ingested
2. Discuss with a toxicologist for advice before performing WBI
3. Usual protocol is nasogastric colonic lavage solution 30mL/kg/hr until rectal effluent clear
4. WBI is contraindicated if there are signs of bowel obstruction or haemorrhage

c. Ongoing care and monitoring
i. Antidote - Desferrioxamine
1. Chelating agent that forms a water soluble desferrioxamine-iron complex
2. Indications
a. Serum iron concentrations > 90 micromol/L
b. Concentration 60 - 90 micromol/L and tablets visible on AXR or symptomatic
c. Symptoms of altered conscious state, hypotension, tachycardia, tachypnoea, or worsening symptoms irrespective of ingested dose or serum iron concentration
d. NOTE:
i. Do not wait for iron concentration if altered conscious state, shock, severe acidosis (pH <7.1), or worsening symptoms
ii. If serum iron concentration is not readily available, a fall in serum bicarbonate concentration is a reasonable surrogate marker of systemic iron poisoning
3. Dose
a. 15mg/kg/hr intravenous
b. The rate is reduced after four to six hours so that the total intravenous dose does not exceed 80mg/kg/24 hours
4. Duration
a. Significant poisoning usually requires administration for 12-16 hours, however it is recommended to continue desferrioxamine until:
i. The child is asymptomatic
ii. Decontamination complete
iii. Anion-gap acidosis resolved
iv. Serum iron concentration <60 micromol/L
5. Desferrioxamine has been associated with pulmonary toxicity and should be used with caution if indications persist >24 hours
6. Desferrioxamine-iron complex is renally excreted
a. If oliguria or anuria develop, peritoneal dialysis or haemodialysis may become necessary to remove ferrioxamine

d. Discharge criteria
i. If <40mg/kg ingestion and negative AXR (if tablet ingestion) can discharge if asymptomatic 6 hours post ingestion
ii. If ingestion of >40mg/kg, discharge only if remains asymptomatic and serum iron concentration falling and <60micromol/L on two measurements 4 hours apart

Question 232

TCA toxicity - bg

Answer 232

1. Key points
   a. Can be fatal in relatively low doses
   b. One of the most common causes of fatal drug overdose
   c. Narrow therapeutic window
   d. Potential for rapid deterioration
2. Dose
   a. >10 mg/kg life-threatening
   b. >20 mg/kg coma, cardiotoxicity
3. Pathogenesis
   a. Toxic effect through 4 main receptors involving antagonism/inhibition
   i. Central and peripheral acetylcholine receptors
   ii. α adrenergic receptors peripherally
   iii. Noradrenalin and serotonin reuptake
   iv. Fast sodium channels in myocardial cells
4. Pharmacokinetics
   a. Onset
   i. Signs usually within an hour of ingestion and most within 6 hours
   ii. However can have unpredictable absorption and half-life due to the anticholinergic effect causing delayed gastrointestinal transit time
   b. Metabolism + excretion
   i. Following initial metabolism in the liver, TCA metabolites are renally excreted
   ii. Some metabolites have pharmacological activity equal to that of the parent drug eg. Desipramine, metabolite of imipramine

Question 233

TCA toxicity - sx

Answer 233

5. Assessment
   a. All patients with deliberate self-poisoning
   b. Any symptomatic patient
   c. Asymptomatic patients with underlying cardiac or neurological disease
   d. Doses > 5 mg/kg in children should be referred to hospital
   e. Patients with Doses >10-15mg/kg should be intubated, ventilated and be given charcoal
   f. Any patient whose developmental age is inconsistent with accidental poisoning as non-accidental poisoning should be considered.
6. Clinical manifestations

Myocardial sodium channel antagonism
• Reduced cardiac contractility and hypotension
• Widened QRS predisposing to VT and VF
• Prolonged QT

Inhibition of noradrenaline and serotonin re-uptake
• CNS depression/ coma
• Seizures

Anticholinergic 	
•	Sinus tachycardia
•	Vomiting
•	Blurred vision 
•	Ataxia
•	Delirium
•	Urinary retention 
•	Ileus 

Antiadrenergic
• Vasodilatation

Question 234

TCA toxicity - ix, rx

Answer 234

7. Investigations
   a. TCA levels useful if diagnosis in doubt, not useful as a predictor of outcome
   b. ECG on admission and repeated if abnormalities found
   i. ECG abnormalities
8. QRS widening
9. RAD
10. Tall R wave in aVR
11. QT prolongation (due to K+ blockage)
    ii. QRS >100ms associated with seizures
    iii. QRS>160ms associated with ventricular arrhythmias
    c. Paracetamol level in case of co-ingestion
    d. Glucose level if reduced GCS
    e. Blood gas looking for acidosis
12. Management
    a. Supportive
    b. Decontamination
    i. Charcoal generally contraindicated, BUT can be considered within 2 hours
    ii. Those who ingest doses of >10-15 mg/kg should be given charcoal following intubation
    c. Intubate and ventilate = aim for pH 7.50-7.55
    i. Acidosis potentiates toxicity
    d. Specific treatments
    i. If QRS is widened or ventricular arrythmia: alkalinsation with sodium bicarbonate 2 mmol/kg
    ii. Other indications
13. Hypotension resistant to fluid resuscitation
14. Considered if – seizures (results in acidosis) or prolonged intubation (leads to acidosis)
    e. Supportive
    i. Fluid boluses for hypotension
    ii. If > 40 mg/kg then noradrenaline infusion
    iii. If further arrythmias repeat bicarbonate then lignocaine

Question 235

Salicylate poisoning - bg, sx, doses

Answer 235

1. Key points
   a. Symptoms may be minimal initially – severe toxicity not evident until 6-12 hours
   b. Poor correlation between salicylate concentration and toxicity – deterioration may occur with falling serum concentrations due to rising CNS concentrations
   c. Moderate to severe salicylate consider decontamination (charcoal) or enhanced elimination (urinary alkalinsation +/- hemodialysis)
2. Clinical manifestations
   a. GIT = nausea + vomiting
   b. CNS = tinnitus, vertigo, confusion
   c. Respiratory = hyperventilation
   d. Respiratory alkalosis
   e. Metabolic acidosis

Salicylate dosage Clinical effects
<150mg/kg Minimal symptoms
150-300mg/kg Mild-moderate symptoms: Tinnitus, vomiting, hyperventilation
>300mg/kg Severe symptoms: Acidosis, seizures, hyperthermia

Question 236

Salicylate poisoning - ix, rx

Answer 236

3. Investigations
   a. Blood gases may indicate severity of poisoning
   i. Phase 1: Respiratory stimulation - hyperventilation and respiratory alkalosis with alkaluria
   ii. Phase 2: Paradoxical aciduria (pH <6) and respiratory alkalosis.
   iii. Phase 3: Metabolic acidosis & hypokalaemia (± ongoing respiratory alkalosis)
   b. Urea & electrolytes, creatinine
   c. Hypoglycaemia
   d. Serum salicylate concentration
   i. At presentation
   ii. 2-4 hourly if symptomatic or enteric coated preparation, until declining
   iii. Beware: There is a poor correlation between salicylate concentration and toxicity and deterioration may still occur with falling serum concentrations due to rising CNS concentration
4. Management
   a. Standard resuscitation
   i. Apnoea associated with intubation may worsen acidosis and lead to cardiac arrest
   ii. Consider pre-loading with sodium bicarbonate
   iii. If ventilated maintain alkalaemia to prevent redistribution of salicylate into CNS
   iv. Correct fluid and electrolytes
   b. Decontamination = activated charcoal 1 g/kg
   i. May be indicated in massive overdose
   ii. Ideally within 1 hour of ingestion
   c. Enhance elimination + treat acidosis
   i. Correction of acidosis minimizes CNS penetration
   ii. Urinary alkalinsation = IV bicarbonate; requires IDC
   iii. Haemodialysis = considered in moderate to severe salicylate poisoning as urinary alkalinsation can be difficult to achieve and may not be effective
5. Indications:
   a. Urinary alkalinsation not feasible (eg. renal failure, pulmonary edema)
   b. Serum concentration >7.2 mmol/L or rising despite alkalinsation
   c. Severe toxicity = altered mental state, refractory acidaemia or electrolyte imbalance, hyperthermia or renal failure

Question 237

Hydrocarbon poisoning - bg, sx

Answer 237

1. Key points
   a. CNS, respiratory and cardiac effects are of main concern
   b. Activated charcoal is contraindicated in hydrocarbon poisoning
   c. Inhalation injury may manifest up to 6 hrs after exposure
   d. Ingestion of less than 5ml of pure essential oil can lead to significant CNS toxicity in children
   e. Sources = petrol, kerosene, lighter fluid, paraffin, solvents, white spirit, lubricating oil etc
2. Clinical manifestations
   a. Respiratory
   i. Coughing / gagging / choking indicates aspiration
   ii. Wheeze, tachypnoea, hypoxia, haemoptysis and pulmonary oedema are signs of evolving chemical pneumonitis.
   b. Cardiovascular
   i. Dysrhythmias occur early in exposure
   c. CNS
   i. CNS depression, coma and seizures may occur with large acute exposures. Onset is usually within 2 hours
   d. GIT
   i. Nausea, vomiting and diarrhoea
   ii. Excessive burping, heartburn, epigastric pain

Question 238

Hydrocarbon poisoning - ix, rx

Answer 238

3. Investigations
   a. Asymptomatic children with small ingestions do not usually require investigation.
   b. For children with more significant ingestions, or who are symptomatic:
   i. 12 lead ECG & cardiac monitoring for 4 hours
   ii. FBE, UEC, LFTs, VBG
   iii. CXR if respiratory symptoms
   c. For all children with deliberate poisoning, perform further screening for co-ingestants
   i. BSL
   ii. Paracetamol level
4. Management
   a. Resuscitation - supportive
   b. Decontamination
   i. Activated charcoal is SPECIFICALLY CONTRAINDICATED – do not bind hydrocarbons and increases the risk of aspiration

Question 239

Caustic/corrosive substances - bg

Answer 239

1. Key points
   a. A substance is caustic if it is capable of burning or corroding organic tissue by chemical reaction
   i. Acidic OR alkaline in nature
   b. Corrosive ingestion may be life-threatening due to airway compromise or GI perforation, or due to agent-specific systemic toxicity
2. Examples
   a. Sodium hydroxide — detergents, drain and oven cleaners, button batteries
   b. Sodium hypochlorite — bleaches and household cleaners (unintentional ingestion in children is generally benign, dilute solutions less than 150 mL do not cause significant corrosive injury)
   c. Ammonia — metal and jewellery cleaners, anti-rust products
   d. Hydrochloric acid — metal cleaners
   e. Sulfuric acid — drain cleaners, car batteries
   f. Button batteries — injury results from leakage of alkali, local electrical current discharge and direct pressure necrosis
3. Mechanism
   a. Extent depends on: pH, concentration, volume
   b. Acidic agents (pH <2): protein denaturation resulting in COAGULATIVE necrosis
   c. Alkaline agents (usually pH 11.5-12.5): LIQUEFACTIVE necrosis resulting in deep and progressive mucosal burns = MORE DANGEROUS
   d. Other corrosive agents may have reducing, oxidising, denaturing or defatting actions
4. Timing of tissue injury
   a. Day 0 = acute injury
   b. Day 1-7 = inflammation, vascular thrombosis
   c. Day 10-21 = granulation tissue
   i. By 10 days there is formation of granulation tissue and weakening of the oesophageal wall
   ii. During this early period, the oesophagus is increasingly vulnerable to perforation
   d. Day 21+ = fibrosis
   i. Perforation less likely

Question 240

Caustic/corrosive substance ingestion - cx

Answer 240

a. Acute complications:
i. Oral, oesophageal, gastric burns (varying thickness) – most effected = areas of anatomical narrowing (cricopharyngeal, diaphragmatic oesophagus, antrum, pylorus).
ii. Oesophageal, gastric perforation
iii. Shock
iv. Haemorrhage
v. Mediastinitis
vi. Psychiatric behaviour management
vii. Organ failure
viii. Acidosis
ix. External injury from corrosive exposure

b. Chronic complications
i. Larygopharyngo fibrosis with airway incompetence -> chronic aspiration
ii. Oesophageal fibrosis, stricture (30% with grade IIB or III injury), stenosis and pseudodiverticulum
iii. Gastric outlet obstruction
iv. GORD
v. Psychosocial problems
vi. Oesophageal carcinoma (risk increased 1000 times, may occur up to 40 years later)

Question 241

Caustic/corrosive substance ingestion - ix, rx

Answer 241

6. Investigations
   a. AXR and CXR – for perforation
   b. Endoscopy within 24 hours (unless asymptomatic)
7. Management

a. Management based on symptoms
i. Symptomatic
1. Admitted and closely monitored
3. All patients require endoscopy
ii. Asymptomatic patients
1. Observed for several hours to monitor fluid intake and overall symptoms
2. Observation important particularly for powdered caustic agent – delayed effects
3. If the patient remains asymptomatic with normal swallowing AND the suspected substance is of low causticity (eg. household bleach) or if it unclear from the history if there was an ingestion, endoscopy usually unnecessary
4. Endoscopy required if suspected more caustic substance (eg. drain cleaner) even if asymptomatic - symptoms are not predictive in young children

b. Supportive care
i. Keep the patient NBM if symptomatic, pending endoscopic assessment
ii. Do not insert a nasogastric tube until cleared of gastrointestinal injury (e.g. Endoscopy)
iii. Antibiotics if evidence of perforation
iv. No role for corticosteroids (do not reduce strictures, may increase risk of perforation and sepsis)

c. Decontamination
i. Rinse the mouth with water as an immediate first aid measure.
ii. Do not induce vomiting
iii. Do not administer oral fluids
iv. Do not administer activated charcoal – obstruct endoscopy and the small, highly ionized chemicals are poorly absorbed by charcoal
v. Do not attempt ph neutralization – concern about additional damage from heat injury during neutralization process
vi. Do not perform gastric lavage or insert an nasogastric tube (until endoscopy is performed)

Question 242

Alkali agent ingestion - general

Answer 242

1. Alkalis includes = drain cleaners, oven cleaners, automatic dishwashing liquid, laundry detergent, ammonia, Portland cement
2. Key points
   a. pH >11.5 likely to cause significant GI ulceration
   b. Attempt to obtain a container to check the contents and strength
   c. Corrosive potential varies – liquid more likely to cause burns than powders
3. Assessment
   a. Toxicity = exposure may lead to severe burns of GIT, especially oesophagus
   b. Symptoms
   i. May be minimal
   ii. Pain
   iii. N+V, drooling or refusal to eat and drink
   iv. Stridor
4. Management
   a. Activated charcoal is CONTRAINDICATED
   b. If asymptomatic – fluid dilution with 10 ml/kg of water (max 250ml)
   c. If asymptomatic after 4/24 and can eat and drink – discharge
   d. If any symptoms – admit for esophagoscopy

Question 243

Lead poisoning - bg

Answer 243

1. Epidemiology
   a. Elevated levels common in refugee children, particularly those <6 years
   b. Blood lead screening is now recommended for all refugee children (aged 6 months - 16 years) arriving in the United States
   c. Blood lead screening is not part of Australian refugee guidelines, but should be considered in any child with developmental delay, pica, or where there is a history suggesting exposure
2. Exposure
   a. Routes of exposure to lead include contaminated air, water, soil and food
   b. Hand-to-mouth activities in young children increase their risk of lead exposure
   c. Examples
   i. Lead-based paint from imported toys and in older houses
   ii. Lead-contaminated traditional remedies, imported candies/condiments, and fashion accessories

Question 244

Lead poisoning - sx

Answer 244

Low level exposure
• Decreased learning and memory, lowered IQ, cognitive dysfunction
• Behavioural disturbance (more marked in children) - irritability, restlessness, sleeplessness
• Myalgia/paraesthesias
• Fatigue, lethargy
• Abdominal discomfort

Moderate exposure	 
•	Arthralgia
•	Vomiting, weight loss, constipation, abdominal pain
•	Headache
•	Poor concentration
•	Muscle fatigue, tremor	

High level exposure
•	Lead line (blue discoloration) on gum margins
•	Anaemia
•	Paralysis
•	Encephalopathy, seizures, coma, death

Question 245

Lead poisoning - ix, rx

Answer 245

4. Investigations
   a. FBE – microcytic hypochromic anaemia, basophilic stippling, sideroblasts
   b. Whole blood lead concentrations
   - <10mcg/dL normal, >100 lifethreatening
5. Treatment
   a. Prevention of further exposure
   b. Blood level 10-45 mcg/dL and patient well
   i. Supportive therapy (treat associated iron deficiency)
   ii. Recheck concentration at 1 and 3 months to ensure decreasing
   iii. IF not decreasing consider ongoing exposure
   c. Blood level >45 mcg/dL or patient acutely unwell
   i. Supportive therapy
   ii. Consider chelation

Question 246

Ibuprofen poisoning - general

Answer 246

1. Clinical manifestations
   a. GIT = epigastric pain, nausea, vomiting
   b. CNS = drowsiness, lethargy, seizures
2. Severity
   a. <200 mg/kg = asymptomatic
   b. 200-400 mg/kg = mild GI and CNS effects
   c. >400 mg/kg = risk of multi-organ dysfunction
3. Management
   a. Indications for admission
   i. Symptomatic
   ii. Ibuprofen >200 mg/kg
   iii. All intentional overdoses
   iv. Concurrent illnesses
   b. Acute management
   i. Consider paracetamol level
   ii. Activated charcoal NOT indicated
   iii. Ensure adequate hydration after large overdoses to reduce the likelihood of acute renal failure
   iv. Check FBE, UEC, LFT in symptomatic patients or those with concurrent illnesses
   v. Use PPI for epigastric pain

Question 247

Benzodiazepine poisoning - general

Answer 247

1. Clinical manifestations
   a. CNS depression, drowsiness, coma
   b. Respiratory depression
   c. Hypotension
2. Patients requiring observation
   a. Ingestion of >3 times the recommended dose for age
   b. All symptomatic patients
   c. Ingestion of unknown quantity
3. Management
   a. Charcoal is not usually of benefit (due to low order of toxicity)
   b. Flumazenil – not indicated for ingestions, only used on discussion with toxicology

Question 248

Eucalypt/essential oil poisoning - bg

Answer 248

1. Key points
   a. Mucous membrane irritation and gastrointestinal symptoms usually develop first, followed by CNS depression, increasing the risk of aspiration pneumonitis
   b. Specific symptoms occur with specific oils, contact poisons for further information
   c. Aspiration pneumonitis is a risk from both the essential oil and from hydrocarbons or emulsifiers that are added to many preparations
2. Background
   a. Essential oils are a common household product used for medicinal, aromatic, cleaning and other purposes
   b. Concentrations range from 1-20% and volumes of 5-15 ml are likely to cause some degree of toxicity
   c. Essential oils mimic other fat soluble drugs, are well absorbed through mucous membranes and the skin and are excreted unchanged or as hepatic metabolites via lungs, urine, faeces and skin
   d. Dose related toxicity: 5 – 15 mL should be considered a potentially toxic dose in adults, for some essential oils 2-3 mL ingestions have been associated with toxicity in children
   e. Expect mucous membrane irritation and gastrointestinal symptoms with possible CNS depression

Question 249

Eucalypt/essential oil poisoning - assessment/rx

Answer 249

a. Patients requiring assessment
i. All patients with deliberate self-poisoning or significant accidental ingestion
ii. Any symptomatic patient
iii. Dose > 5 mL
iv. Any patient whose developmental age is inconsistent with accidental poisoning as non-accidental poisoning should be considered

c. Investigations
i. Asymptomatic children with small ingestions do not usually require investigation.
ii. Consider:
1. Chest X-ray and blood gas if signs of aspiration pneumonitis
2. UEC and LFTs in patient with significant illness, large ingestions or with clove oil/pennyroyal ingestions.
3. Paracetamol level in all intentional overdoses

d. Acute management
i. Resuscitation
ii. Aspiration/chemical pneumonitis largely managed supportively
iii. Charcoal contraindicated due to risk of aspiration

Question 250

Activated charcoal (RCH) - general

Answer 250

Key points
Activated charcoal has a very limited role in the treatment of childhood poisoning. It should not be used without consultation with a toxicologist
Aspiration of activated charcoal can cause significant morbidity and mortality. Nasogastric tube position must be confirmed on chest X-ray before administering activated charcoal
If indicated, activated charcoal should be administered as soon as possible, usually within 1 to 2 hours of the exposure

Background

• Activated charcoal is very rarely indicated in children. It should only be used in potentially severe poisonings where supportive care and antidote therapy alone would result in a poor outcome, ie where the benefits outweigh the potential risks
• Activated charcoal is a form of carbon that binds to many drugs and toxins, reducing further absorption from the gastrointestinal tract and increasing elimination of some drugs (‘gastrointestinal dialysis’) if multiple doses of charcoal are given

Potential indications:
Amisulpride
Chloroquine, hydroxychloroquine or quinine
Calcium channel blockers, particularly verapamil and diltiazem
Carbamazepine
Colchicine
Beta Blockers
Flecainide
Methotrexate Paraquat/diquat

NOT helpful:
Acid,and Alkalis / corrosives
Cyanide
Ethanol/methanol/glycols
Eucalyptus and Essential Oils
Fluoride
Hydrocarbons
Metals - including Lithium, Iron compounds, potassium, lead
Mineral acids - Boric acid

Adverse Effects:
Respiratory - aspiration, progressive respiratory failure; Death.
Beware oral use with drowsiness, or following the ingestion of substances which could cause rapidly reduced CNS depression or may cause seizures
Faecal discolouration
GI obstruction: by bezoar formation

Question 251

Carbon monoxide poisoning - bg

Answer 251

1. Key points
   a. Carbon monoxide is odourlesss, colourless and tasteless
   b. It is produced from combustion of any carbon containing fuel. Sources; smoke inhalation (most common), heating systems, camp stoves etc
   c. Non-smokers – 3% carboxyhaemoglobin, smokers 10-15%
2. Pathophysiology
   a. Rapidly diffuses across capillaries
   b. Actions
   i. Impaired oxygen offloading
3. Binds to iron moiety of haem: with 240x the affinity of O2
4. Results in allosteric changes in haem protein  reduced ability of the other three O2 binding sites to offload oxygen in the peripheral tissues = i.e. left shift of oxygen dissociation curve
   ii. Impaired peripheral oxygen utilization
5. 10-15% of CO is (reversibly) bound to myoglobin, cytochromes and NAPHH
6. Results in impaired oxidative phosphorylation in mitochondria
7. In the heart, mitochondrial dysfunction due to CO can lead to myocardial stunning despite adequate O2 delivery

Question 252

Carbon monoxide poisoning - sx, cx

Answer 252

3. Clinical
   a. Variable and non-specific – require careful evaluation of mental status, examination often unremarkable
   b. “Cherry red” appearance of lips and skin – but this is insensitive
   c. Mild-mod = constitutional symptoms, malaise, headache, nausea, dizziness, poor feeding
   i. Headache is the most common presenting symptom
   ii. Altered mental state – may be the only presenting symptom in the absence of trauma or burns
   d. Severe = seizures, syncope, coma
   e. Children = may have more subtle and non-specific symptoms
   i. As children have higher oxygen utilisation, young children may develop signs and symptoms of CO poisoning before older children and adults who have the same exposure
4. Complications
   a. Myocardial ischaemia, arrhythmias
   b. Pulmonary oedema
   c. Lactic acidosis
   d. Delayed neuropsychiatric syndrome
   i. Up to 40% within 20 days of significant exposure  cognitive deficit, behaviour change, focal neurological deficits - may persist for 1yr
   ii. Lower incidence in children
   iii. Poor correlation with levels – but often assoc with LOC at time of intoxication

Question 253

Carbon monoxide poisoning - ix, rx

Answer 253

5. Investigations + Diagnosis
   a. Suggestive history + inc carboxy haemoglobin on ABG (note carboxyhaemaglobin levels are imprecise at degree of poisoning)
   b. PULSE OX CANNOT screen for CO poisoning – doesn’t differentiate carboxy haemoglobin/ oxyhemoglobin
   c. ECG in all patients
6. Management
   a. Remove exposure
   b. Oxygen
   i. Apply high flow oxygen to ALL CO poisoned patients regardless of pulse oximetry or arterial PO2
   ii. CO removed in lungs by competitive binding of Hb by O2
   iii. Half-life of CO in room air = 300minutes, high flow O2 = 90minutes, 100% hyperbaric O2 = 30mins
   iv. If level >20% - consider intubation an deliver 100% oxygen via ETT to assist removal of CO
   c. Hyperbaric O2 indications
   i. Indications = patients with COHb >25%, end-organ ischaemia, LOC, pregnancy
   ii. Probably prevents delayed neuropsychiatric syndrome
   iii. In children: do CXR first (exclude congenital anomalies e.g. lobar emphysema), higher risk hypothermia
   d. Cyanide poisoning = consider if smoke inhalation

Question 254

Cyanide poisoning - bg

Answer 254

1. Key points
   a. Cyanide poisoning is lethal unless treated with antidote
2. Exposure
   a. Fire
   b. Industrial exposure
   c. May occur concurrently with carbon monoxide poisoning
3. Suspect if
   a. Smoke inhalation
   b. Carbonaceous sputum
   c. Neurological dysfunction
   d. Metabolic acidosis on ABG with lactate >8
4. Mechanism
   a. Normal aerobic metabolism, ATP is generated from electron transport chain following TCA cycle
   b. Cyanide binds to the ferric ion (FE3+) of cytochrome oxidase a3, inhibiting final enzyme in mitochondrial electron chain = UNABLE TO MAKE ATP
   c. Therefore cell switch to anaerobic metabolism to generate ATP – lactic acidosis and increased anion gap metabolic acidosis
   d. Despite ample oxygen, cells cannot use oxygen to make ATP = hypoxia and ischemia
   e. Metabolism
   i. Main pathway via rhodanese enzyme which gives a sulphar so can be excreted in urine
   ii. Minor pathway is combination with hydroxocobalamin (precursor B12) which means it is excreted in urine

Question 255

Cyanide poisoning - sx, ix, rx

Answer 255

3. Clinical features
   a. CNS – headache, anxiety, confusion, vertigo, seizures, coma
   b. CVS – tachycardia and hypertension, then bradycardia and hypotension, AV block, ventricular arrhythmia
   c. GIT – vomiting, abdominal pain, metallic taste
   d. Skin - flushing (cherry-red), cyanosis (late), irritant dermatitis
   i. No cyanosis until late because venous blood remains oxygenated (unable to use) therefore venous blood bright red
   e. Rhabdomyolysis, renal failure, hepatic necrosis, bright red venules on fundoscopy
4. Investigations
   a. Basic workup – BSL, paracetamol and salicylate levels
   b. Carboxy and methemoglobin levels (rule out)
   c. ECG
   d. VBG – increased anion gap metabolic acidosis
5. Management
   a. ABCDEFG
   b. High flow oxygen (regardless of pulse oximetry reading = INACCURATE)
   c. IVF bolus is shock
   d. Seizure management
   e. Antidote treatment
   i. Hydroxocobalamin 70mg/kg IV
   ii. Alternatives – sodium nitrite, sodium thiosulphate
6. Sequelae
   a. Delayed onset parkinsonism due to basal ganglia damage (sensitive to cyanide)

Question 256

Digoxin poisoning - bg

Answer 256

1. Key points
   a. Digoxin has a narrow therapeutic index
2. Pharmacology
   a. Summary of effect on heart
   i. Increased inotropy (mild effect)
   ii. Increased automaticity
   iii. Negative dromotropy (slowing of av conduction)
   iv. Increased vagal tone
   b. Direct effect
   i. Inhibition of Na/K ATPase on the cell surface
    increased intracellular Na+ and increased extracellular K+
   increased intracellular Ca2+ due to Na+/Ca2+ antiporter
    calcium-mediated inotropy and increased automaticity, as well as negative dromotropy due to decreased intracellular K+
   c. Indirect effect
   i. Increased vagal tone (vagomimetic effect)
3. Toxicokinetics
   a. Absorption – good oral absorption with oral bioavailability of 80% and peak levels at 6 hours
   b. Distribution – 30% protein bound, Vd 10L/kg (higher in the elderly and obese)
   c. Metabolism – minimal hepatic metabolism
   d. Elimination – 60% renal, t ½ of 30-40 h, longer in renal failure

Question 257

Digoxin poisoning - sx, ix, rx

Answer 257

4. Clinical manifestations
   a. GIT = anorexia, nausea, vomiting, diarrhoea abdominal pain
   b. Metabolic = hyperkalaemia (early sign of significant toxicity)
   c. CVS = arrythmias
   i. Enhanced automaticity (eg. flutter, AF) with AV block
   ii. VF, VT
   iii. Bradyarrythmias (conduction delays/ blocks, slow or regularized AF), hypotension, shock
   d. CNS = lethargy, convulsion
5. Investigations
   a. Digoxin level
   b. UEC – potassium level, renal function
   c. ECG - “digitalis effect” on the ECG consists of T wave changes (flattening or inversion), QT interval shortening, scooped ST segments with ST depression in the lateral leads (saggy T wave depression), and increased amplitude of the U waves
6. Treatment
   a. Digoxin specific Fab fragments (digibind)
   b. Treatment of hyperkalaemia

Question 258

Opioid poisoning - general

Answer 258

Key points

a. Recreational, intentional or iatrogenic
b. Diagnosis made on clinical findings
c. Best predictor of opioid poisoning is respiratory depression
d. Normal pupil examination or mydriasis does NOT exclude opioid intoxication

2. Clinical manifestations
   a. Pulmonary = respiratory depression, non-cardiogenic pulmonary oedema
   b. Cardiovascular = orthostatic hypotension, peripheral vasodilation, dysrhythmias
   c. Gastrointestinal = N+V, constipation
   d. Dermatological = flushing, pruritis
   e. Reproductive = amenorrhoea, anovulation
3. Management
   a. Naloxone = competitively binds opioid receptors
   i. Highly lipophilic, rapidly moves into the CNS
   ii. Onset of action of one minute when given intravenously
   iii. Effects of naloxone last for 45-70 minutes
   iv. Dose repeated every 3 minutes until improvement in respiratory depression is noted
   v. Note shorter half-life than most opioids
   vi. Can precipitate withdrawal in addicted individuals

Question 259

Arsenic poisoning - general

Answer 259

1. Key points
   a. Metalloid element
   b. High dose can result in systemic toxicity and death
2. Sources of exposure
   a. Drinking water
   b. Dietary ingestion
   c. Pressure-treated wood
3. Clinical manifestation
   a. Acute
   i. Gastrointestinal symptoms
   ii. Garlic odour of the breath and stool
   iii. Dehydration and hypotension
   iv. Cardiac arrythmias, shock, ARDS, death
   v. Encephalopathy, delirium, coma, seizures
   b. Chronic
   i. Sequelae of acute poisoning, or due to chronic longer-term exposure to lower levels of arsenic
   ii. Latent toxicity (eg. cancer) can occur after exposure has ceased
   iii. MULTISYSTEM effects – skin, neurological, cancer, cardiovascular, liver, endocrine
   iv. Neurological and skin issues are most prominent
4. Treatment
   a. Acute
   i. Decontamination – remove contaminated clothing
   ii. Supportive fluids
   iii. Chelation
   b. Chronic = supportive

Question 260

Mercury poisoning - general

Answer 260

• Clinical manifestations
o Haemorrhagic gastroenteritis
o CV collapse
o Multi-organ dysfunction

• Management
o WBI
o Chelation
o Not recommended
 Induced emesis – due to corrosive effects
 Not well absorbed to activated charcoal

Question 261

Ethanol poisoning - bg

Answer 261

1. Key points
   a. Exploratory ethanol ingestion by children less than 6 years of age typically results in minor symptoms.
   b. However, infants and young children are prone to profound hypoglycaemia, coma, and hypothermia, despite ingesting relatively small amounts of ethanol
2. Pharmacokinetics
   a. Peak BAC occurs 90 minutes after ingestion (although alcohol is often not ingested as a single dose)
   b. The rate at which BAC decreases varies between individuals
   c. In general, through a process of metabolism and elimination in urine
   i. BAC decreases by 0.01% to 0.02% per hour after ingestion

Question 262

Ethanol poisoning - sx

Answer 262

3. Clinical manifestations
   a. Cardiovascular = hypotension and tachycardia may occur as a result of ethanol-induced peripheral vasodilation, or secondary to volume loss
   b. Hypothermia = hypothermia may occur due to vasodilatation and / or prolonged exposure to cold environment
   c. Hypoglycaemia = can cause decreased conscious state.
   d. Dose related toxicity = CNS effects of blood alcohol concentration (BAC) on non-tolerant adults, by BAC
4. 02 to 0.05% Decreased inhibition, diminished fine motor coordination
5. 05% to 0.10 % Impaired judgement; impaired coordination
6. 10% to 0.15% Difficulty with gait and balance
7. 15% to 0.25% Lethargy; difficulty sitting upright without assistance
8. 30% Coma in the non-habituated drinker
9. 40% Respiratory depression

Question 263

Ethanol poisoning - ix, rx

Answer 263

4. Investigations
   a. Low threshold to investigate infants and young children
   b. If only mild symptoms monitor and give frequent CHO containing drinks
5. Acute management
   a. Management of
   i. Hypoglycaemia
   ii. Hypovolaemia
   iii. Hypothermia
   iv. Decreased conscious state
   v. Respiratory depression
   vi. Management of comorbidities

Question 264

Amphetamine ingestion - general

Answer 264

EG: MDMA, ecstacy, (meth)amphetamine

1. Pharmacology
   a. Catecholamine release from presynaptic vesicles and mass release of serotonin

Symptoms:
• Agitation, sweating, tachycardia, hypertension
v. Bruxism, jaw clenching, paraesthesia, meth mouth
vi. Dry mouth, increased psychomotor activity, blurred vision
g. Serotonin causes mydriasis

Medical complications:
•	Severe hyperthermia
•	Rhabdomyolysis,
•	Seizures
•	Intracranial haemorrhage
•	Hyponatraemia
•	Cerebral oedema

Investigations

• 12 lead ECG
• BSL
• Electrolytes & renal function, CK, troponin
• Consider intracranial imaging if any concern regarding vascular dissection

Management
• Safe, low stimulus environment
• Continuous cardiac monitoring
• Benzodiazepines to control agitation

Question 265

Gammahydroybutyrate (GHB) - general

Answer 265

1. Pharmacology
   a. Precursor of GABA
   b. Acts at GHB receptors as GABA agonist

Symptoms
•	CNS & respiratory depression progressing to coma
•	Bradycardia & myoclonic jerks
- hypertension
- coma 3-6 hours	

3. Management - supportive
   a. Gastric lavage and charcoal not recommended as rapidly absorbed and ingestion time never clear
   b. No reversal agents available
   c. Flumazenil and naloxone are ineffective

Expect improvement within 4-6 hours

Question 266

Cannabis - general

Answer 266

Symptoms
•	Mild sedation
•	Euphoria
•	Disinhibition progressing to CNS depression
•	Anxiety & psychotic symptoms
•	Postural hypotension & tachycardia	

MANAGEMENT
Benign symptoms: 
•	Supportive care until asymptomatic 
Anxiety: Diazepam 
Cardiovascular effects: - IV fluids

Question 267

Hallucinogens - general

Answer 267

EG LSD, psilocybin (mushroom), phencyclidine

Central & peripheral anti-cholinergic toxidrome
•	Agitation
•	Delirium
•	Hallucinations
•	Mydriasis
•	Tachycardia
•	Dry flushed skin	

Management
• Supportive care
• Treat delirium with titrated diazepam

Anti-psychotic major tranquilisers (eg haloperidol) may worsen anti-cholinergic symptoms

Question 268

Inhalants/volatile substance abuse - general

Answer 268

EG glue, gasoline, deoderant

Similar effects to alcohol intoxication:
•	Initial euphoria & disinhibition followed by CNS depression 
Acute medical complications:
•	Sustained 'high'
•	Encephalopathy & seizures, 
•	Methaemoglobinaemia
•	Cardiac arrhythmia & SCD	

Treatment
• Treat behavioural disturbance/delirium with diazepam

Question 269

Cocaine - general

Answer 269

 Can be snorted (absorbed from nasal mucosa) -> less addictive but high within 10 seconds
 can be smoked (“free-basing”) -> more addictive, accidental burns occur
→ ↑ mood/ ↑ energy/ ↓ appetite/ ↑ task performance / ↓ sleep
→ but also get paranoid ideation and sympathetic hyperactivity (↑HR/ ↑bp/ ↑ pupils/ ↑ T)
→ increases myocardial oxygen demand + decreased diastolic flow time = ischemia + arrhythmia
 Teratogenic (prematurity, LBW, congenital malformations, dev disoders)
 Users become tolerant and need to increase dose or change ROA
 No withdrawal
Detect – urine up to 48hours
Management – oxygen, telemetry, BZD, aspirin, heparin, cooling. Contraindicated = BB

Question 270

Anabolic steroids - general

Answer 270

 used by athletes to enhance sports performance
 works via myotrophic action at androgen receptors + antagonism of catabolism-mediating corticosteroid receptors
Effect = muscle bulk
Side effects:
 Acne, oily hair, hirsutism
 Gynaecomastia, testicular atrophy, azoospermia
 Uncontrollable rage, depression, mania
 CVS: fluid retention -> often have to take diuretics
Detection – urine

Question 271

Envenomation - gen bg

Answer 271

• For snakebites monitoring should be for at least 12 hours if there is no evidence of envenoming
• For suspected funnel web spider monitor for at least 4 hours post bite; in significant funnel web spider bites envenoming develops 4/24 post bite
• For red back spiders only symptomatic patients require admission
• For marine envenoming the requirement for hospital monitoring is determined by the type of bite/sting and symptomatology
o Severe or life-threatening envenoming generally occurs very early after exposure, within 1 hour or less and always within the first 4 hours
o Exception is stingray wounds to the trunk

Question 272

Snake bites - bg and types

Answer 272

1. Key points
   a. Uncommon in Victoria
   b. Bite site may be evidence by fang marks or scratches
   c. Bites usually not painful
2. Types of snakes
   - Brown: VICC (venom-induced consumptive coagulopathy), TMA 10%, systemic symptoms <50%, collapse 33% and cardiac arrest 5%
   - Tiger: VICC, systemic symptoms COMMON, CVS rare, TMA 5%
   - Red bellied black: Anticoagulant, systemic symptoms COMMON

Question 273

Snake bites - sx, ix

Answer 273

3. Clinical manifestations
   a. Paired fang marks, a single mark or scratch may be present
   b. Headache, N+V
   c. Collapse or confusion followed by partial or complete recovery
   d. Abdominal pain
   e. Blurred vision, diplopia, or drooping eyelids
   f. Difficulty in speaking, swallowing or breathing
   g. Swollen, tender glands in groin or axilla of bitten limb
   h. Limb weakness or paralysis
   i. Respiratory weakness or arrest
4. Investigations
   a. Initial blood tests: coagulation screen (INR, APTT, fibrinogen), FBE and film, CK, EUC, LDH, LFT.
   b. Serial blood tests: INR, APTT, fibrinogen, CK, FBE, EUC.
   c. Role of snake venom detection kit (VDK)
   i. The choice of antivenom is based on the clinical syndrome and local geographical patterns of snake distribution
   ii. Attempted identification of snakes by witnesses should never be relied upon as snakes of different species may have the same colouring or banding
   iii. Snake venom detection kits can be useful but in inexperienced hands they can have significant rates of snake misidentification, false positives and false negatives.
   iv. If there is no apparent bite, a VDK may be done on urine, but never blood.
   v. Most venomous snakebites in Victoria are from brown or tiger snakes, and both may present with an initial coagulopathy on blood testing. As a result, it may be appropriate to administer one vial each of brown and tiger snake antivenom where envenomation is evident and a person experienced in the use and interpretation of a venom detection kit is not immediately available

Question 274

Snake bites - rx, cx

Answer 274

5. Management
   a. Pressure immobilisation bandage
   b. Indications for immediate anti-venom
   i. Any evidence of neurotoxic paralysis – ptosis, ophthalmoplegia, limb weakness, respiratory effects
   ii. Significant coagulopathy – unclottable blood, INR >1.3, or prolonged bleeding from wounds and venipuncture
   iii. History of unconsciousness, collapse, convulsion or cardiac arrest
   iv. Give 1 vial of brown and 1 vial of tiger AV
6. Complications
   a. Coagulopathy is main concern – bleeding should cease within 20-30 minutes of giving AV
   i. Coagulation profile can take 6 hours to normalize
   b. Anticoagulant coagulopathy
   i. APTT 2.5-3xN
   ii. INR mildly elevated
   iii. D-Dimer and fibrinogen normal
   c. Venom induced consumptive coagulopathy (VICC)
   i. Prolonged INR
   ii. Fibrinogen very low
   iii. D-dimer very high 10-100x
   d. FFP only improves time to normalization of coags – no clinical improvement/ time to DC

Question 275

Spider bites - general

Answer 275

Funnel web spider 	
•	Neuro-excitatory envenoming
•	Catecholamine storm effects
•	Pulmonary oedema 	
Treatment: Pressure bandage immobilsation, Funnel web spider AV 

Red back spider	
•	Neuro-excitatory envenoming 
•	Pain (intense, local pain 5-10 minutes post bite)
•	Sweating
•	Hypertension
•	Nausea	
- swelling and piloerection 
Treatment: No first aid is effective, Red back spider AV if severe pain or unwell and not relieved by simple measures

Question 276

Box jellyfish sting - general

Answer 276

• Death within 5 minutes of sting – direct cardiotoxicity
• Can result in arrhythmias
• Delayed reaction in 50%

• Management
o Ice-pack, analgesia
o Liberal amounts of vinegar
o IN cardiac arrest – give antivenom undiluted, Mg sulphate
o Those with severe envenomation but not arrested – 3 ampules, diluted

Question 277

Hypovolaemia/volume assessment

Answer 277

•	Subtle signs of hemorrhagic shock 
o	Tachycardia
o	Narrowed pulse pressure
o	Prolonged capillary refill time
o	Pallor
o	Decreased urine output
o	Altered mental status 

• Significant intraabdominal hemorrhage in children can be masked by their ability to maintain normal systolic blood pressure despite large volume blood loss

• Pathophysiology
o Hypovolaemic shock is characterized by fluid loss and decreased preload
o Tachycardia and an increase in SVR are the initial compensatory responses to maintain cardiac output and systemic blood pressure
o Early in hypovolaemic shock, the BP remains normal because of compensatory increases in HR and peripheral vascular resistance

Signs of dehydration – all not very reliable
o Weight drop (most accurate if premorbid weight available)
o Depressed fontanelle
o Sunken eyes
o Dry mouth
o Decreased skin turgor (this and cap refill are the only ?validated signs)
o Decreased urine output

Mild <4%
Thirst
Usually no other signs

Moderate 4-7%
Delayed CRT >2s
Tachypnea
Mild decreased tissue turgor

Severe >7%
Delayed CRT >3s, mottled skin
Other signs of shock (tachycardia, irritable, decreased GCS, hypotension)
Acidotic breathing
Decreased tissue turgor

Question 278

Calculations for fluid requirements

Answer 278

Calculating fluid requirement

1. +/- Bolus if any signs of shock – 10-20mL/kg 0.9% N/saline
2. Deficit – degree of dehydration (% body weight)
   a. Correct deficit over 24hours
   b. Correct over 48hours in hypernatremia
3. Maintenance fluid requirement – based on weight
4. Ongoing losses

Maintenance
o 4mL/kg/hour for first 10kg (100 mL/kg/day)
o 2mL/kg/hour second 10kg (50mL/kg/day)
o 1mL/kg/hour third 10kg (20mL/kg/day)
Special circumstances
o If at risk of SIADH (lung/CNS infection/post op) use 2/3 maintainence

Ongoing losses
o Usually calculated on basis of previous 4 hours loss – E.g. ileostomy, gastroenteritis, drain output etc.