Paediatrics

Question 1

What is sleep-disordered breathing?

Answer 1

Spectrum of upper airway dysfunction during sleep characterised by SNORING and INCREASED RESPIRATORY EFFORT causes by upper airway collapse

Most severe form is obstructive sleep apnoea

Question 2

Paediatric diagnosis of sleep disordered breathing

Answer 2

Habitual loud breathing/snoring
Audible or witnessed pauses
Bizarre positioning
Daytime symptoms - hyperactivity, inattatention

Question 3

Paediatric diagnosis of OSA

Answer 3

Loud snoring i.e heard through door
Daytime somnolence
Poor school performance
Medical hx of GORD, prematurity

IF diagnostic uncertainty options include overnight pulse oximetry at hospital/home
Overnight polysomnography

Question 4

What is the main indication for tonsillectomy in UK

Answer 4

Obstructive sleep-disordered breathing
Paediatric adenotonsillar hypertrophy can contribute to anatomical pharyngeal obstruction and increased upper airway resistance
Peak age is 3-10

Question 5

Overnight bed required for paediatric patients?

Answer 5

Varies between hospitals but generally overnight bed required

Children <3
Comorbidities e.g. obesity, failure to thrive
Severe obstructive sleep apnoea

Question 6

Post-op analgesia for severe OSA

Answer 6

Multimodal analgesia
- Non-opioid analgesics: paracetamol and NSAIDs
- Dexamethasone
- Opioids for rescue (be aware children with OSA have increased sensitivity to opioids)

Question 7

Pre-med doses in paediatrics
- Midazolam
- Ketamine
- Clonidine

Answer 7

Midazolam
ORAL/buccal = 0.5mg/kg (max 20mg) (20-30mins onset)
Intranasal = 0.2mg/kg

Ketamine (avoid in under 2s)
ORAL = 5-10mg/kg (careful salivation)
IM = 5mg/kg

Clonidine
ORAL 4mcg/kg
INTRANASAL 2mcg/kg

Question 8

General “HOW TO” paediatrics for pre-op management

Answer 8

Perform through history, examination, review old hospital charts and relevant ix.
Establish rapport with child and parents

Prescribe 20mg/kg oral paracetamol and 5mg/kg oral ibuprofen

Midazolam pre-medication 0.5mg/kg ORAL/BUCCAL up to 20mg
If concern re: airway or OSA then pulse oximetry in period between midazolam administration and anaesthetic room arrival

Request AMETOP applied to both hands

In preparation for child arriving in anaesthetic room
Prepare and check anaesthetic machines, monitoring, suction
Prepare induction and emergency drugs
Prepare airway equipment

Question 9

Anaesthetic HOW TO - intraoperative, tonsillectomy

Answer 9

Allow for premedication to take effect

Gain IV access using distraction techniques

Ask ODP to apply full AAGBI monitoring and pre-oxygenate patient

Induce anaesthesia with 1mcg/kg fentanyl and 2-3mg/kg propofol with maintanence using sevoflurane with target age adjusted MAC 1

D/w surgeon regarding airway - either flexible LMA or RAE ETT

Administed 0.15mg/kg dexamethasone and 0.1mg/kg ondansetron

Infiltration of tonsilar fossae with local anaesthetic

Question 10

Key intraoperative moments during tonsilectomy

Answer 10

Boyle-Davis gag application - may cause obstruction/dislodgement of LMA.
Ensure inspection of nasopharynx for blood clots by surgeon or under direct vision

Question 11

Risk factors for post-op respiratory complications post tonsillectomy

Answer 11

Children <3
Severe OSA
Failure to thrive
Obesity
RV hypertrophy
Down’s
Craniofacial abnormalities
Chronic lung disease
Sickle cell disease

Question 12

WETFLAG RECITE

Answer 12

Question 13

Name 5 considerations in the post-tonsillectomy bleed case

Answer 13

1. Paediatric patient with heightened anxiety amongst patient and paretns
2. Full stomach with high risk of aspiration
3. Hypovolaemia and anaemia due to blood loss
4. Difficult airway - oedematous, obstructed by blood, recent instrumentation and surgery
5. Recent anaesthesia - residual effects

Question 14

Pre-op key points for post-tonsillectomy bleed

Answer 14

Review key history/examination/investigations
Previous anaesthetic chart
Post-op drugs, what given when
Targeted clinical examination
- HR/CRT/BP/skin colour
- Conscious level
- If time send bloods for Hb, haematocrit, cross-match, VBG

Resucitation with 20ml/kg crystalloid, further boluses to effect, blood x-match and available
Assistance from consultant anaesthetist and senior ODP
TXA 15mg/kg bolus over 10mins

Question 15

Talk through the RSI technique for post tonsillectomy bleed

Answer 15

Supine position
IV access with fluids running, trained assistant, suction x2
Pre-oxygenation and cricoid pressure
Propofol and suxamethonium
Consider ketamine
Intubation with calculated device + one smaller
Orogastric tube to empty stomach removed prior to extubation
Extubate head down, left lateral

Question 16

Talk through Gas induction for post tonsillectomy bleed

Answer 16

Head down, left lateral
Sevoflurane in 100% O2
Intubate when deep during spontaneous ventilation either in left lateral or turn supine
ONLY advantage is maintanence of spontaneous ventilation if intubation is difficult

Question 17

How can you classify paediatric hypovolaemia

Answer 17

Mild <5% volume loss
Moderate 5-10% volume loss
Severe >10% volume loss

Fluid deficit = % volume loss, x weight (in kg) x 10 = fluid in mls

Question 18

What is blood volume in neonate/child/adult

Answer 18

Neonate 85-90ml/kg
Infant 75-80mls/kg
Child 70-75mls/kg
Adult 65-70mls/kg

Question 19

Paediatric weight formulae

Answer 19

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

Question 20

Uncuffed ETT calculation

Answer 20

Age/4 + 4 = uncuffed

Question 21

What is the maintanence fluid calculation in kids

Answer 21

4-2-1 rule
4ml/hr x 1st 10kg + 2ml/hr x 2nd 10kg + 1ml/hr every kg over 20

E.g. 18kg = 56mls/hr

Question 22

How can you classify post-tonsillectomy haemorrhage

Answer 22

Primary
= Occurs within 24hrs of surgery
- Affects 2% adenotonsillectomies
- Caused by inadequate haemostasis during surgery

Secondary
= Occurs more than 24hrs
- Commonly day 5-10
- Detachment of crust from removed tonsil.
- 2% of tonsillectomies
- Risk factors = adults> children, chronic tonsilitis

Question 23

Clinical features of Down’s syndrome

Answer 23

General features:
Upward slanting palpebral fissures, flat occiput
Single palmar crease
Micrognathia
Flat nasal bridge
Obesity in childhood

Resp:
OSA, large tongue and tonsilar hypertrophy
Sublottic and tracheal stenosis (choose smaller tube)
Frequent LRTI

CVS:
Congenital cardiac disease
ASD
VSD
Tetralogy of fallot
Patent Ductus Arteriosus

GI:
Duodenal atresia
Hirschprung’s

CNS:
Developmental delay
Alzheimer’s in old age
Epilepsy

MSK:
Atlanto-axial instability 10-20%
Hypotonicity, hyperlaxity

Endo:
Hypothyroidism

Immune:
Leukemia more common
Sepsis more common

Question 24

Features of fallot’s tetralogy

Answer 24

VSD
Pulmonary Stenosis
RV Hypertrophy
Overiding aorta

Cause of R-L shunt

Question 25

Eisenmenger's syndrome and anaesthesia

Answer 25

R-L shunt becomes apparent after L-R shunt over time E.g. VSD, ASD, PDA Over-time with increased pulmonary hypertension and increased RV pressures the shunt is reversed leading to Dyspnoea, syncope, angina, arrythmias Cyanosis Worsens with anaesthesia as SVR goes down and PVR goes up, worsening shunt Worsens in pregnancy Pulmonary hypertension changes are permanent

Question 26

Neonate definition

Answer 26

birth to 44 weeks post conceptual age

Question 27

Infant definition

Answer 27

44 weeks post conceptual age to 1 year

Question 28

Physiological differences in paediatrics - RESPIRATORY

Answer 28

1. Immature gas exchange surface - Limited number of small alveoli - Prem neonates may be surfactant deficient 2. Inefficient respiratory mechanics - Very compliant chest wall with low FRC - Horizontal movement of ribs 3. High O2 demand with limited physiological reserve - CC exceeds FRC during tidal breathing leading to airway collapse and increased dead-space ventilation - Tidal volume relatively fixed, MV increased by RR - O2 consumption is high at 7ml/kg/min - Gastric distension easily splints diagram 4. Differences in control of respiration - Blunted response to hypercapnoea and hypoxia - Risk of post-operative apnoeas up to 60 weeks post conceptual age 5. OxyHb curve shifted to left due to presence of foetal Hb which means there is less efficient offloading of O2 in peripheral tissues

Question 29

Physiological differences in paediatrics - AIRWAY

Answer 29

Obligatory nasal bridges Short neck Large head with prominent occiput Compressible tracheal cartilages Larynx: - Long U shaped epiglottis - Funnel shaped trachea - High anterior larynx at C3-4 - Narrowest point at cricoid cartilage - Thin, easily damaged mucosa OPTIMAL HEAD POSITION IS IN NEUTRAL POSITION (use shoulder roll)

Question 30

Physiological differences in paediatrics - CVS

Answer 30

Increased cardiac output (150-300mls/kg/min vs 70mls/kg/min) Stiff non compliant ventricles, limited ability to increase SV CO mainly increased by HR increase Blood volume 90ml/kg at birth, 80ml/kg children, 70ml/kg adults Hb 18g/dL

Question 31

Physiological differences in paediatrics - CNS

Answer 31

Immature blood-brain barrier with increased sensitivity to centrally depressant drugs Active vagal reflexes, bradycardia common Higher MAC

Question 32

Physiological differences in paediatrics - RENAL

Answer 32

Increased total body water Immature kidneys unable to handle Na+ and water load Lower rates of renal excretion of drugs Higher extracellular to intracellular compared with adults

Question 33

Physiological differences in paediatrics - GI

Answer 33

Gastric emptying prolonged, incompetent LOS Immature liver with lower hepatic blood flow in neonates Decrease in metabolism of some drugs Decrease in plasma protein production leading to higher conc. of free drug

Question 34

Physiological differences in paediatrics - THERMOREGULATIO

Answer 34

Heat lost rapidly: - High body surface area - Low subcutaneous fat - Limited ability to shiver - High RR Neonates can metabolise brown fat B3 adrenoceptors, but increases o2 consumption

Question 35

Per kg doses paediatric cardiac arrest Adrenaline Amiodarone Defib Atropine

Answer 35

Adrenaline = 10mcg/kg Amiodarone = 5mg/kg Defib = 4J/kg Atropine = 20mcg/kg

Question 36

Perioperative risk of anaesthetising child with an URTI

Answer 36

Airway hyper-activity - Breath holding - Laryngospasm - Bronchospasm - Desaturation

Question 37

What would you include in your assessment of a child with a potential upper respiratory tract infection?

Answer 37

Standard paediatric anaesthetic history including the following: Parental concern over child symptoms Presence of purulent nasal discharge Productive cough and dyspnoea Systemic symptoms - Fever - Malaise - Irritability - Poor appetite Lung auscultation confirming crackles/wheeze

Question 38

If you choose to delay surgery for URTI when should you reschedule?

Answer 38

May last up to 4 weeks Typically 4-6 weeks for re-listing

Question 39

What patient-related and surgery-related factors increase risk of anaesthetising patient with an URTI

Answer 39

Patient-related: - Age <1 yr - Hx of prematurity - Asthma - Snoring/OSA - Parental smoking Surgery-related - Airway surgery - Surgery requiring ETT

Question 40

EXCLUSION CRITERIA - paediatric day case

Answer 40

PATIENT, SOCIAL, ANAESTHETIC/SURGICAL FACTORS PATIENT - Term baby <4 weeks - Pre-term <60 weeks post conceptual age - Significant co-morbidities e.g. sickle cell, inborn errors of metabolism, cardiac/resp disease - Active infection SOCIAL - Poor housing conditions - >1hr journey time to hospital - Inadequate transport - No telephone SURGICAL/ANAESTHETIC - Prolonged procedure - Opening of body cavity - High risk for OSA/apnoea - High risk for haemorrhage - Postop pain unlikely to be managed with orals - Sibling with SIDS

Question 41

What is paediatric emergence delirium?

Answer 41

"A disturbance of a child's awareness or attention to his/her environment 1. with disorientation and perceptual alterations 2. including hypersensitivity to stimuli and 3. hyperactive motor behaviour in immediate post-anaesthesia period"

Question 42

Risk factors for emergence delirium?

Answer 42

Male Sevoflurane/desflurane anaesthesia ENT surgery Preop child anxiety Parental anxiety Child temperament

Question 43

What is the Cravero scale ?

Answer 43

Scale for measuring paediatric emergence 1 = obtunded 2 = asleep but responsive to movement or stimulation 3 = awake and responsive 4 = crying >3mins 5 = thrashing behaviour requiring restraint

Question 44

Non-pharmacological methods for reducing Emergence Delirium

Answer 44

ADVANCE (Anxiety-reduction, Distraction, Video modelling and education, Adding parents, No excessive reassurance, Coaching, and Exposure/shaping) Parent information Short videos Interactive games

Question 45

Pharmacological methods for reducing Emergence Delirium

Answer 45

TIVA to avoid volatile anaesthesia Intra-op opioids Pre-op oral midazolam Dexmetomidine/clonidine/ketamine/dexamethasone

Question 46

Treatment of emergency delirium

Answer 46

Propofol Fentanyl Midazolam ALL delay PACU discharge

Question 47

What is pyloric stenosis

Answer 47

P.S is a gastric outlet obstruction due to hypertrophy of the pyloric smooth muscle Multifactorial causes seen more in first born males It is a medical rather than a surgical emergency

Question 48

pyloric stenosis What is the metabolic abnormality Why does this appear

Answer 48

Hypokalaemic, hypochloraemic, metabolic alkalosis 1. Loss of gastric secretions - HCl, Na, K+ 2. RAAS activation due to dehydration, aldosterone secretion increasing K+ loss 3. Metabolic alkalosis causes shift of K+ intracellularly therefore hypokalaemia

Question 49

pyloric stenosis How is it medically managed

Answer 49

Medical mx. not surgical Correct metabolic abnormalities before surgical Correction of dehydration and metabolic disturbance Fluids = fluid resus, maintanence, ongoing losses Fluid resus with 20mls/kg Sodium Chloride followed by reassessment Fluid maintanence with 150mls/day or 4-2-1 containing NaCl, dextrose, K+ Replace ongoing enteral losses Aim is normal K+, normal pH or mild alkalosis prior to surgery Alkalosis will increase risk of apnoeas

Question 50

Anaesthetic considerations in neonate

Answer 50

Airway management: - Avoid flexion/extension/gastric insufflation - Consider straight blade but can use macintosh - < 2kg 2.0, 2-4kg 3.0, Term 3.5mm, 3m-1year 4.0 uncuffed Meticulous tube checking 1kg 7cm 2kg 8cm 3kg 9cm 4kg 10cm Respiratory management - Lung protective ventilation, use PEEP - Minimise dead space - Apnoea monitoring up to 60 weeks post conceptual age Cardiovascular management - Avoid raising PVR - Maintain preload and afterload Hepatic management - Monitor blood glucose levels - Administer glucose, early return to feeding - Drug doses reflect decreased hepatic clearance Renal management - Replace maintanence and insensible losses - Aim 2mls/kg/hr Neurological management - Adjust doses according to weight, MAC adjustment Thermoregulation - Neutral thermal environment using heating devices, warmed fluids, warmed ambient environment - Monitor temperature

Question 51

What is the genetic pathophysiology in sickle cell

Answer 51

Autosomal recessive inheritance. HbAS (sickle cell trait) HbSS (sickle cell disease) Glutamic acid REPLACED by Valine at 6th position Sickle cell thought to be malaria protective

Question 52

4 major complications of sickle cell disease

Answer 52

Vaso-occlusive crises Aplastic crises Haemolytic Splenic sequestration

Question 53

Clinical features of sickle cell disease

Answer 53

Anaemia Hb 70-80 Bone marrow hyperplasia, frontal bossing Stoke Venous arterial ulcer PUlmonary infarcts and PAH Priapism Avascular necrosis of bone

Question 54

Sickle cell diagnosis

Answer 54

Sickle blood cells on blood film Haemoglobin electrophoresis Sickledex test

Question 55

What triggers RBC sickling

Answer 55

Hypoxaemia (PO2 5-6 kPA in HbSS, 2.5-4 in HbAS) Acidosis Hypothermia Dehydration Infection Tourniquets though evidence limited Deoxy HbS polymerises and precipitates within RBCs leading to Increased viscocity Impaired low Organ infarction

Question 56

What is autism spectrum disorder

Answer 56

Lifelong developmental disorder characterised by triad of impairments Difficulties with social communication Difficulty with social interaction Difficulty with social imagination

Question 57

Principles of management of child wit Austim SD

Answer 57

Pre-op: - Advance notice of child listed - Advance meeting with parent/guardian e.g. preop clinic - Checklist of child's physical/psychological needs - Pre-op height weight - Pre-agreed strategy e.g. regarding premed Day of surgery - First on list - Sip till send - Communication passport - Quiet waiting area - Local anaesthetic cream, sedative pre-med - Communication aided Post-op - Recover in quiet area of PACU with parents in attendance - Faces of pain scale and symbol communication

Question 58

What is ADHD

Answer 58

Neurodevelopmental disorder characteriesed by 1. Innatention 2. Excessive energy bouts 3. Hyper-fixation 4. Impulsivity Difficulty regulating emotions and strong correlation with substance misuse

Question 59

Methylphenidate and anaesthesia

Answer 59

Ritalin is an INDIRECTLY-ACTING sympathomimetic. Omit on day of surgery In cases of emergency surgery - Hypertension and arrythmias may occur - Serotonin syndrome possible when serotinergic drugs taken e.g. fentanyl, pethidine

Question 60

Fasting guidelines in children

Answer 60

Solid food + formula = 6hrs Breast milk = 4hrs Clear fluids = 2hrs Study looking at fasting times demonstrated 12hrs food and 8hrs CF on average, more likely to be uncooperative

Question 61

Metabolic effects of fasting?

Answer 61

1. Primary source of energy initially is HEPATIC GLYCOGENOLYSIS 2. Glycogen stores deplete, hepatic GLUCONEOGENESIS takes place and lipolysis becomes primary energy source 3. Neuroendocrine stress response further depletes stores and increases lipolysis

Question 62

What is cerebral palsy?

Answer 62

It is a group of NEUROLOGICAL disorders that affect a persons ability to move and maintain posture It is caused by ABNORMAL brain development OR NEUROLOGICAL INJURY before, during and shortly after birth

Question 63

How can causes of cerebral palsy be classified?

Answer 63

ANTENATAL - TORCH infections - Fetal alcohol syndrome - Fetal trauma - Congenital disorder INTRAPARTUM - Fetal asphyxia - Maternal haemorrhage - Pre-eclampsia - Prematurity - Low APGAR score POSTPARTUM - Head injury - Meningoencephalitis - Kernicterus - Intracerebral bleed

Question 64

Cerebral palsy classification

Answer 64

Spastic Ataxic Dyskinetic Mixed Distribution could be DIPLEGIC, HEMIPLEGIC, QUADRIPLEGIC

Question 65

Clinical features of cerebral palsy with anaesthetic relevance

Answer 65

Neuromuscular - Spasticity, contractures, scissoring gait Neurology - 70% have learning difficulties - 50% have epilepsy - Communication and speech problems Respiratory - Chronic lung disease --> premature birth, newborn resp distress sx ----> chronic aspiration pneumonitis, impaired swallow, reflux - High rates of scoliosis, restrictive LD and pulmonary HTN - Poor cough, poor nutrition GI - Pseudobulbar palsy and oromotor dysfunction, poor swallow leading to malnutrition - Drooling - TMJ dislocation/spasticity

Question 66

Management of cerebral palsy

Answer 66

Non pharmacological - MDT (PT, OT, speech) Pharmacological - Anticonvulsants - Antispasticity e.g baclofen - Botox - Antisialogogues - Antacids - Prophylactic abx Surgical - Tendon transfer - Tendotomy - Arthrodesis - Gastrostomy

Question 67

Key features of management of cerebral palsy PREOP

Answer 67

Establish good rapport with patient and parent/guardian Thorough hx/exam/ix as multiple co-morbidities Continue all medications perioperatively Avoid sedative pre-meds

Question 68

Key features of management of cerebral palsy INTRAOP

Answer 68

May need gas induction 30 deg head up due to reflux risk Propofol best agent as reduces smooth muscle tone Sux NOT contra-indicated K+ not significant rise despite extra-junct Ach NDMR less potent, shorter duration of action. But smalled Vd ETT used, high reflux risk

Question 69

Key features of management of cerebral palsy POST-OP

Answer 69

Best managed in HDU or ITU Hypothermia, pain/anxiety may precipitate spasms Communication difficulties make treatment of post op pain difficult Multimodal analgesia

Question 70

What is exomphalos

Answer 70

CONGENITAL condition which presents with herniation of abdominal contents through abdominal wall involving gastrointestinal contents covered by peritoneum Less urgent compared with gastroschisis

Question 71

What is gastroschisis

Answer 71

Defect in anterior abdominal wall, causing herniation of abdominal contents Urgent repair as contents exposed

Question 72

Management options for gastroschisis

Answer 72

Primary surgical closure Staged surgical closure Formation of 'silastic pouch' in theatre or NICU

Question 73

Pre-op management of gastroschisis

Answer 73

Fluid resuscitation Care of herniated organs, inspect for rupture Bowel decompression (nasogastric) Temp reg. Management on NICU

Question 74

Post-op management of gastroschisis

Answer 74

Majority return to NICU ventilated Epidural beneficial if prolonged extubation Monitor for resp compromise Monitor for raised abdomonal pressure May require return to OT if signs compromise Prognosis in patients without abnormalities is good

Question 75

Name 4 potential causes of acute abdomen in infancy

Answer 75

Intussusception Malrotation or volvulus Necrotising enterocolitis Hirschsprung enterocolitis

Question 76

What is intussesception

Answer 76

Obstruction caused by invagination/telescoping of bowel usually at terminal ileum Suggestive history includes paroxsms of abdo pain, blood, mucus in stool Signs - Sausage mass on exam - Profound shock - Deteriorate further if bowel perf or ischaemia

Question 77

What are the intraoperative anaesthetic principles of intussception

Answer 77

In addition to general consideration of major surgery in this age group, there are a number of specific considerations Induction - RSI following aspiration of NGT Intra-op - Ongoing fluid resus, monitor response (1ml-2ml/kg) - IV access secure - Analgesia - multimodal + opiate - Temp regulation - Urinary catheter Post-op - May require HDU, PICU

Question 78

Anaesthetic principles of mediastinal mass

Answer 78

Thorough pre-operative assessment CT/MRI/echo Pre-op treatment e.g. steroids, chemo Periop - Antisialogue - Gas induction in lateral or head up tilt - Maintain spont vent - Awake extubation

Question 79

Management of airway compression on induction of anaesthesia

Answer 79

Positioning lateral or prone may shift mass of tumour away from airway Fibreoptic bronchoscop to identify level of obstruction Rigid bronchoscopy may be required

Question 80

What is muscular dystrophy

Answer 80

Group of genetic conditions in which deficiency of dystrophin protein that links myofibrils to cell cytoskeleton Results in progressive muscle weakness 2 most common are Duchenne muscular dystrophy and Becker's

Question 81

3 key features of duchennes

Answer 81

MSK - multiple e.g. pseudohypertrophy, gowers, scoliosis Resp - restrictive lung Cardiac - dilated

Question 82

How common is Duchenne's

Answer 82

1:3500 live births

Question 83

Anaesthetic "how to" for DMF

Answer 83

Pre-op - MDT work up, cardiac ix, PFTs Intra-op - TIVA - Avoid sux (extra junctional hyper-K), small doses of NMBDs, avoid volatiles - Use of NM monitoring Watch for rhabdo with sux and volatiles - Inc. bleed risk Post-op - HDU for cardiac/resp monitoring

Question 84

Important aspects of assessing pain in children

Answer 84

Physiological indicators Behavioural indicators Structured pain scales Paediatric staff Parent views

Question 85

Penile block anatomy

Answer 85

Dorsal nerves of penis S2-S4 Within bucks fascia next to penile vessels Frenulem supplied by dorsal nerves and branch of perineal nerve Triangular space of - Symphysis pubis above - Corpora cavernosa below - Superficial fascia anteriorly Insert at base of penis either side of suspensory ligament 1-2mls of 0.5% bupivicaine in children up to 3kg

Question 86

Conditions with risk of CHD

Answer 86

Downs syndrome CHARGE VACTERL DiGeorge syndrome

Question 87

Pathological murmur?

Answer 87

Symptomatic Timing - diastolic/pansystolic/late systolic Harsh tone Palpable thrill Signs of overload - tacyhpnoea, creps Hepatosplenomegaly Delayed pulses

Question 88

Ix for paediatric congenital cardiac disease

Answer 88

ECG ?Hypertrophy - Under 5, R wave in V1 >1.75, upright T wave - R-wave V5/6 >4mm ?Vent ectopics ECho

Question 89

how can paeds ccd be risk stratified?

Answer 89

High risk - Poor physiological compensation - Complex lesions - Complex surgery - Under 2 Low risk - Well compensated - Simple lesions - Simple surgery

Question 90

What can you say about restraint

Answer 90

Should be proportional Employed for shortest time possible

Question 91

What is congenital diaphragmatic hernia

Answer 91

A condition with a significant mortality (up to 30%) in which there is abnormal closure of the diaphragm allowing abdominal contents into the thorax Associated with lung hypoplasia, pulmonary hypertension and abnormal pulmonary vasculature

Question 92

How can Congenitla Diaphragmatic Hernia be diagnosed and classified

Answer 92

Antenatal or postnatal Antenatal can be treated, balloon occlusion Postnatal cyanosis and CXR diagnostic A B C D A = small defect B = <50% chest wall with absent tissue C = >50% chest wall with absent tissue D = complete abscence of hemidiaphragm

Question 93

Cong Diaph Hernia principles PREOP

Answer 93

Not a surgical emergency Patients should be optimised pre-op Likely to be ventilated, may be requiring nitric for pulmonary htn Nasogastric reduces pulmonary compression 10-20% have cardiac abnormalities - worse prognosis

Question 94

Cong Diaph Hernia principles POSTOP

Answer 94

Open vs. thoracoscopic Potential for marked intraoperative cardioresp instability - Ventilation challenging - Invasvie access required Lung prot ventilation Avoid excessive airway pressures Avoid gastric insufflation

Question 95

What is necrotising enterocolitis

Answer 95

A form of enterocolitis usually seen in premature infants, typically seen after introduction of enteral feeding Associated with high morbidity and mortality

Question 96

Differential diagnosis of NEC

Answer 96

Meconium ileus, malrotation, intussusception

Question 97

What are Bell's stages

Answer 97

For NEC classification Stage 1 (suspected) - mild systemic + mild GI signs Stage 2 (confirmed) - mild systemic + additional GI signs + radiological signs and abnormal labs Stage 3 (advanced) - severe systemic signs, severe GI signs, deranged lab findings e.g. DIC

Question 98

Risk factors for NEC

Answer 98

Prematurity Indomethacin Cyanotic heart disease + Immature immune system Formula feed Infective - viral/bacterial

Question 99

Mx of NEC

Answer 99

Bell's 1 and 2 Medical mx - Bowel rest - Decompression: NG aspiration - Broad spec abx - Supportive Surgical - In 1/3 cases - Laparotomy - resection + stoma formatoin 20% mortality strictures + short gut sx.