Gen Paeds, ED Flashcards

Question

Turricephaly - general

Answer 1

Premature fusion of coronal + sphenofrontal + frontal ethmoidal sutures • Cone shaped head

Answer 2

• Cloverleaf shaped head – prominent temporal bones

Answer 3

* 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 1. Skull deformity (brachycephaly – bilateral coronal suture fusion, tall forehead) 2. Midface hypoplasia (prominent nose & forehead, maxillary hypoplasia) + OSA 3. Mild hypotelorism, proptosis 4. NORMAL hands and feet ii. Neuro 1. Hydrocephalus 2. Visual problems (optic atrophy, proptosis) 2. Apert syndrome b. FGFR2 mutation c. Clinical manifestations i. Skeletal 1. Irregular craniosynostosis (brachycephaly, plagiocephaly - coronal) 2. Premature fusion of coronal, sagittal, lambdoid sutures 3. Midface hypoplasia + OSA 4. Exophthalmos but less severe than Crouzon 5. Symmetrical syndactyly (fusion of 2nd/3rd/4th digits – single nail 2nd-4th digits) ii. Neuro 1. Hydrocephalus 2. Intellectual disability 3. Pfeiffer syndrome b. Rarely FGFR1 c. Clinical manifestations i. Skeletal 1. Skull deformity (scaphocephaly + brachiocephaly) 2. Midface hypoplasia (flat face, shallow orbits) 3. Hypertelorism, prominent eyes, mild exophthalmos, small nose 4. Partial syndactyly – webbing fingers/toes 5. Deviated broad and short thumb/big toe 4. 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

Answer 4

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

Answer 5

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 4. Investigations a. No diagnostic test b. Consider other causes of syncope – ECG c. FBE + Fe studies 5. Treatment a. Fe supplementation i. Indications 1. Anaemic 2. 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

Answer 6

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

Answer 7

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

Answer 8

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

Answer 9

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

Answer 10

Sits unsupported Rolls supine to prone Transfers – crossing midline Raking Reaches for object Feeds self Holds bottle Begins to recognize strangers Babbles

Answer 11

Crawls/stands/cruises Comes to sit from lying Crude pincer Object permanence Waves Peek a boo Mama/dada

Answer 12

Walks alone Stands alone Refined pincer Bangs objects together Imitates eg. clapping Mama/dada Words Jargoning (polysyllabic) 1 command

Answer 13

Walks backwards Runs Scribble 2 blocks Spoon Indicates wants

Answer 14

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

Answer 15

Rides on toy without pedals Line 6 blocks Pages, doors, undress Parallel play 50-200 words Pronouns 2/4 clear 2 commands

Answer 16

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

Answer 17

One foot Hop Stairs without support Square Cross Person – 3 parts ``` 1500 words Full name Colours Songs 4/4 clear ```

Answer 18

Skips Heel to toe Leaps Triangle Shoe laces Spreads with knife

Answer 19

* 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

Answer 20

* 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

Answer 21

* 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

Answer 22

* 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

Answer 23

* 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

Answer 24

* 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

Answer 25

* 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

Answer 26

``` 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 ```

Answer 27

* 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

Answer 28

• 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

Answer 29

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

Answer 30

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

Answer 31

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 5. 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

Answer 32

6. Assessment a. History i. Prenatal 1. Genetic (T21, Fr X) 2. Toxin (FAS) 3. Infection (TORCHS) ii. Perinatal 1. Neurological insult (prematurity, HIE) iii. Postnatal 1. ABI (infection, trauma) 2. 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 8. 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 10. 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

Answer 33

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

Answer 34

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

Answer 35

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

Answer 36

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 1. Minor physical anomalies – eg. simian crease, low-set ears 2. Disturbance of growth – microcephaly, macrocephaly, extremes of stature or weight 3. Abnormal skin lesions – multiple depigmented or pigmented naevi 4. Malformations or abnormal findings in several organ symptoms 5. 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 7. 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

Answer 37

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

Answer 38

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

Answer 39

- 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.

Answer 40

• 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

Answer 41

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

Answer 42

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

Answer 43

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 2. 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

Answer 44

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 1. MacArthur Bates Communicative Behaviour Scales (CSBS) – Infant/Toddler Checklist 2. Ages and Stages Questionnaire (ASQ) 3. Receptive-Expressive Emergent Language Scale (REEL-3) 4. Brigance Infant Toddler Screen (BITS) 5. Ward Infant Language Screen b. Screening Assessments i. Renfrew Action Picture Test 1. Short, easily administered and scored screening tool for expressive language and semantic skills 2. 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) 1. Time = 30-60 minutes 2. Initial diagnosis of the language disorder a. Core, receptive and expressive language index standard scores b. Normative mean 3. 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)

Answer 45

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 1. Speech 2. Other allied health iii. Financial support/ funding access (carers allowance, medicare plans, funding for school, NDIS) 13. 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

Answer 46

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.

Answer 47

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

Answer 48

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

Answer 49

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

Answer 50

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

Answer 51

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

Answer 52

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

Answer 53

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’

Answer 54

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)

Answer 55

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

Answer 56

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

Answer 57

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 1. Adequate ventilation 2. Sufficient cerebral perfusion 3. Maintaining normal metabolic status 4. Controlling seizures 5. Treating underlying cause for encephalopathy ii. Therapeutic hypothermia 6. 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

Answer 58

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

Answer 59

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

Answer 60

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)

Answer 61

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

Answer 62

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 ```

Answer 63

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

Answer 64

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

Answer 65

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 ```

Answer 66

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

Answer 67

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

Answer 68

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

Answer 69

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%

Answer 70

1. Complications of spasticity a. Hip displacement b. Scoliosis c. Contractures + deformity 2. Hip a. Surveillance i. ALL children with CP require surveillance 1. Hip displacement common 2. 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 1. Salvage surgery 2. Preventative surgery – adductor muscle release, aims to halt progression 3. Bony surgery – VDRO a. Femoral osteotomy +/- acetabular reconstruction c. Maintains stable, enlocated hips in the long-term with minimal risk of recurrence 3. 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 4. Osteoporosis a. Non-weight bearing b. Monitoring should start at 3 years and choose as indicated

Answer 71

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

Answer 72

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

Answer 73

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

Answer 74

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

Answer 75

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

Answer 76

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

Answer 77

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 1. Tonsillectomy 2. Adenoidectomy 3. Tongue reduction 4. Jaw distraction

Answer 78

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

Answer 79

• 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

Answer 80

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

Answer 81

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)

Answer 82

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

Answer 83

* 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.

Answer 84

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

Answer 85

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)

Answer 86

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

Answer 87

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

Answer 88

* 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

Answer 89

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

Answer 90

* 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

Answer 91

* 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

Answer 92

* 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

Answer 93

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

Answer 94

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.

Answer 95

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.

Answer 96

• 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

Answer 97

``` 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

Answer 98

* 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

Answer 99

* 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

Answer 100

``` 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

Answer 101

SBP (50th centile) = Age x2 +85 Lower limit of normal for neonates: MAP gestational age in weeks

Answer 102

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

Answer 103

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

Answer 104

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

Answer 105

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 1. Trauma is the most often associated with a reversible cause of PEA – severe hypovolaemia, tension pneumothorax, and pericardial tamponade 2. PEA is also seen in hypothermic patients, electrolyte derangement (hypocalcaemia) 3. Massive PE 2. 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 3. 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)

Answer 106

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 1. Assess rhythm 2. If VF/pVT continue with sequence 3. If asystole change to asystole/PEA sequence 4. 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 3. 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)

Answer 107

* 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

Answer 108

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 1. Synchronized DC cardioversion starting at 2 Jkg – can be increased (synchronised for haemodynamically stable patients with a pulse, cf APLS) 2. Child who is responsive to pain should be anaesthetized or sedated first 3. 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

Answer 109

• 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

Answer 110

• 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

Answer 111

• 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

Answer 112

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

Answer 113

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 6. 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

Answer 114

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

Answer 115

= 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 ```

Answer 116

1. Key points a. Classification i. Scalds = generally involve water at below boiling point and contact for <4 seconds 1. 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 2. Primary a. Airway i. Airway may be compromised either because of inhalational injury or scalds ii. Indicators of inhalational injury 1. History of exposure to smoke in confined space 2. Deposits around the mouth and nose 3. Carbonaceous sputum 4. 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

Answer 117

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

Answer 118

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

Answer 119

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

Answer 120

• 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

Answer 121

* 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

Answer 122

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

Answer 123

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

Answer 124

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

Answer 125

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 1. Conscious = 5 back blows then 5 chest thrusts 2. 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

Answer 126

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

Answer 127

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

Answer 128

• 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

Answer 129

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

Answer 130

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

Answer 131

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

Answer 132

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

Answer 133

• 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)

Answer 134

• 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

Answer 135

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

Answer 136

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

Answer 137

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

Answer 138

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%

Answer 139

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

Answer 140

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)

Answer 141

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

Answer 142

8. Complications a. Acute = hours-days i. Re-bleeding ii. Acute hydrocephalus = due to blockage in CSF resorption iii. Vasospasm resulting in infarction 1. Onset 4-14 days post SAH, peak at 6-8 days 2. Clinical features (delayed ischaemic deficit) = confusion, decreased LOC, focal deficit (speech/ motor) 3. Risk factors = large amount of blood on CT, smoking, increased age, HTN 4. 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) 9. 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

Answer 143

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

Answer 144

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 ```

Answer 145

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

Answer 146

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

Answer 147

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

Answer 148

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

Answer 149

``` "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

Answer 150

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

Answer 151

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

Answer 152

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 3. 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

Answer 153

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) 1. 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 2. 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 3. 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 1. Review outline of each vertebra in turn 2. Spaces between the facet joints and the gaps between adjacent spinous processes should be similar 3. Joint between the odontoid peg and the anterior arch of the atlas should be <5mm iv. Cartilage and soft tissue 1. Abnormal widening of the pre vertebral soft tissues may indicate a haematoma due to C-spine injury 2. 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 1. 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

Answer 154

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

Answer 155

* 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

Answer 156

* 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

Answer 157

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

Answer 158

``` 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

Answer 159

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

Answer 160

``` 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

Answer 161

* 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

Answer 162

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

Answer 163

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

Answer 164

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 1. 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 1. FBE, UEC, LFT (ALP) 2. CMP, vitamin D ii. Second line tests 1. Mg, copper 2. PTH 3. Syphilis serology 4. Urine metabolic screen 5. Inflammatory markers 6. Genetic tests for osteogenesis imperfecta – COL1A1, COL1A2

Answer 165

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 1. UEC, LFT, calcium 2. 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

Answer 166

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

Answer 167

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

Answer 168

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

Answer 169

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

Answer 170

* 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

Answer 171

Physostigmine

Answer 172

Flumenazil

Answer 173

Glucagon Isoprenaline (nonselective beta adrenoreceptor analog) Noradrenaline

Answer 174

Calcium | Insulin/glucose

Answer 175

Desferrioxamine Charcoal does not bind iron

Answer 176

Octreotide

Answer 177

N-acetyl cysteine

Answer 178

Sodium bicarbonate

Answer 179

Beta blocker e.g. labetalol

Answer 180

100% oxygen +/- hyperbaric

Answer 181

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

Answer 182

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 ```

Answer 183

``` 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

Answer 184

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)

Answer 185

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 ```

Answer 186

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

Answer 187

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)

Answer 188

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

Answer 189

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.

Answer 190

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

Answer 191

``` "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 ```

Answer 192

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

Answer 193

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

Answer 194

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

Answer 195

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

Answer 196

``` 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

Answer 197

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

Answer 198

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

Answer 199

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

Answer 200

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

Answer 201

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

Answer 202

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

Answer 203

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

Answer 204

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

Answer 205

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 1. QRS widening 2. RAD 3. Tall R wave in aVR 4. 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 8. 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 1. Hypotension resistant to fluid resuscitation 2. 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

Answer 206

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

Answer 207

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 1. 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

Answer 208

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

Answer 209

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

Answer 210

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

Answer 211

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)

Answer 212

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)

Answer 213

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

Answer 214

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

Answer 215

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 ```

Answer 216

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

Answer 217

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

Answer 218

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

Answer 219

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

Answer 220

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

Answer 221

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

Answer 222

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 1. Binds to iron moiety of haem: with 240x the affinity of O2 2. 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 1. 10-15% of CO is (reversibly) bound to myoglobin, cytochromes and NAPHH 2. Results in impaired oxidative phosphorylation in mitochondria 3. In the heart, mitochondrial dysfunction due to CO can lead to myocardial stunning despite adequate O2 delivery

Answer 223

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

Answer 224

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

Answer 225

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 4. Suspect if a. Smoke inhalation b. Carbonaceous sputum c. Neurological dysfunction d. Metabolic acidosis on ABG with lactate >8 5. 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

Answer 226

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 6. 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 7. 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 8. Sequelae a. Delayed onset parkinsonism due to basal ganglia damage (sensitive to cyanide)

Answer 227

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

Answer 228

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

Answer 229

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

Answer 230

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

Answer 231

• 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

Answer 232

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

Answer 233

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 0. 02 to 0.05% Decreased inhibition, diminished fine motor coordination 0. 05% to 0.10 % Impaired judgement; impaired coordination 0. 10% to 0.15% Difficulty with gait and balance 0. 15% to 0.25% Lethargy; difficulty sitting upright without assistance 0. 30% Coma in the non-habituated drinker 0. 40% Respiratory depression

Answer 234

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

Answer 235

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

Answer 236

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

Answer 237

``` 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 ```

Answer 238

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

Answer 239

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

Answer 240

 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

Answer 241

 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

Answer 242

• 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

Answer 243

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

Answer 244

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

Answer 245

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

Answer 246

``` 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 ```

Answer 247

* 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

Answer 248

``` • 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 ```

Answer 249

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.