1
Q
What are the features of respiratory distress in children?
A
- Tachypnoea
- Tachycardia
- Nasal flaring
- Use of accessory muscles
- Intercostal, subcostal, suprasternal recessions
- Head retraction
- Inability to feed
Severe:
- Cyanosis
- Tiring due to increased work of breathing
- Reduced conscious level
- Oxygen saturation <92% despite oxygen therapy
2
Q
Describe the physiology of stridor and wheeze
A
Narrowing of the airway due to inflammation…
- upper airway narrowing results in increased effort and added respiratory noises during inspiration, such as stridor (harsh, single note)
- lower airway narrowing results in increased effort and added respiratory noises during expiration, such as crepitations and wheeze
3
Q
What are the causes of stridor?
A
- Most common: viral laryngeal, tracheal, bronchial infections (e.g. croup)
- Epiglottitis
- Bacterial tracheitis
- Foreign body/trauma
- Anaphylaxis (allergic laryngeal angioedema)
- Severe lymph node swelling (tuberculosis, infections mononucleosis, malignancy)
- Inhalation of smoke/hot fumes in fire
4
Q
Define croup
A
Inflammation of the larynx and/or trachea due to viral infections (parainfluenza, RSV, rhinovirus), most common in ages 6 months to 3 years
5
Q
What are the clinical features of croup?
A
- Coryzal prodrome over 12-48 hours
- Low grade fever
- Barking cough
- Stridor
- Hoarse voice
- Increased work of breathing
6
Q
What is the management of croup?
A
Mild illness:
- supportive care
- oral dexamethasone
- parent education
Moderate-severe illness (e.g. recessions and stridor at rest, significant distress):
- admission to hospital
- oral dexamethasone
- oxygen
- nebulised budesonide
Severe obstruction:
- nebulised epinephrine
- intubation and ventilation
7
Q
Define epiglottitis
A
Rare but life-threatening inflammation of the epiglottis and surrounding structures leading to rapid airway obstruction, usually caused by Haemophilus influenza B (or strep pneumoniae), occurring in any age but typically 2-7 years old
8
Q
What are the clinical features of epiglottitis?
A
- Very unwell looking (with rapid progression)
- High fever
- Sore throat and painful swallowing, leading to drooling
- Stridor
- Muffled ‘hot potato’ voice
- Respiratory distress (difficulty breathing, tachypnoea, using accessory muscles)
- Tripod positioning
9
Q
What is the management of epiglottitis?
A
- Do not examine the throat (may precipitate total obstruction)
- Lateral neck X-ray: thumb sign
- Airway management: intubation or tracheostomy
- Antibiotics: e.g. ceftriaxone
- Supportive care: fluids, analgesia, antipyretics
10
Q
Describe laryngomalacia
A
- Congenital anomaly of the larynx, characterised by inward collapse of the supraglottic structures during inspiration
- Causes a partial airway obstruction and chronic intermittent stridor, with no respiratory distress
- Symptoms peak at around 6 months, and often resolve by 18-24 months
- Most cases are self-limiting, surgical intervention (e.g. tracheostomy, laryngoplasty) if severe
11
Q
Describe bacterial tracheitis
A
- Similar presentation to epiglottitis with high fever, looks very ill, rapidly progressing airway obstruction, with copious thick airway secretions
- Typically caused by staphylococcus aureus
- Management includes IV antibiotics, and intubation and ventilation if required
12
Q
What are the causes of wheeze?
A
- Bronchiolitis
- Viral episodic wheeze
- Multiple trigger wheeze
- Asthma
- Anaphylaxis
- Foreign object inhalation
- Cystic fibrosis
- Bronchopulmonary dysplasia
13
Q
Define bronchiolitis
A
Inflammation and infection of the small airway (bronchioles), very common during winter months in under 1 year olds, most common cause is RSV (or rhinovirus, adenovirus)
14
Q
What are the clinical features of bronchiolitis?
A
- Coryzal prodrome
- Mild fever
- Dry cough
- Wheeze (may also have fine inspiratory crackles)
- Increased work of breathing (tachypnoea, recessions, use of accessory muscles
- Feeding difficulties
15
Q
What is the management of bronchiolitis?
A
Mild:
- supportive care at home
- ensure adequate hydration
- nasal suction
Moderate-severe:
- hospital admission
- oxygen
- fluid and feeding support
- further respiratory support (high-flow oxygen, CPAP, ventilation)
- prophylaxis of RSV using monoclonal antibody given to high-risk preterm infants
16
Q
What features of bronchiolitis require immediate hospital admission?
A
- Aged under 3 months
- Apnoea
- Severe respiratory distress (grunting, severe recessions, RR >70)
- Consistent oxygen >92%
- Central cyanosis
- Less than 50% of normal intake
- Clinical dehydration
- Difficulty managing at home
17
Q
Describe viral induced wheeze
A
- Common problem in pre-school age group, usually resolving by age 5
- Wheeze occurs only during viral illnesses, due to smaller airways being prone to narrowing
- Managed with supportive care, SABAs (e.g. salbutamol), and maybe oral prednisolone
18
Q
Describe multiple trigger wheeze
A
- Wheeze in pre-school age group that is triggered by viral infections, allergens, cold, dust, exercise
- Helpful diagnosis where a formal diagnosis of asthma is not yet justified (many are diagnosed with asthma later)
- May benefit from asthma therapy (e.g. regular ICS + SABA as needed, +/- LTRA)
19
Q
Describe the pathophysiology of asthma
A
- Variable and reversible airflow restriction, caused by smooth muscle constriction, bronchial inflammation (IgE dependent, mediators released form mast cells) , and mucous production
- Airway hyper-responsiveness to inhaled stimuli, causing exaggerated airway narrowing
20
Q
What are the typical triggers for asthma?
A
- Viral infections
- Exercise
- Allergens (animals, house dust mites, pollen, moulds, food)
- Cold or damp air
- Air pollution
- Tobacco smoke
- Strong emotions
21
Q
What are the features of asthma?
A
- Cough (worse at night, non-productive)
- Shortness of breath (on exertion or exposure to triggers)
- Wheeze (widespread, expiratory, polyphonic)
- Harrison’s sulci (depressions at base of thorax associated with muscular insertion of diaphragm, seen in chronic obstructive airway disease during childhood)
- Symptoms present outside of viral illnesses, and respond to bronchodilators
- Personal or family history of atopy
22
Q
What is the management of asthma in children aged 5-11?
A
Initially, offer twice daily low-dose ISC and SABA as needed, then add in…
- MART regimen (maintenance and reliever therapy, ICS + LABA) : start with low dose, then moderate dose
- Conventional regimen: trial LTRA for 8-12 weeks (stop if ineffective), switch regular to twice-daily ICS+LABA +/- LTRA (start low dose, the moderate dose)
- refer to specialist if still uncontrolled
23
Q
What is the management of asthma in children aged 12 and older?
A
- AIR (anti-inflammatory reliever) therapy: ICS+LABA
- MART (maintenance and reliever) therapy: low dose, then high dose)
- Check FeNO and eosinophil count, specialist referral if raised
- Add LTRA or LAMA, or both if still uncontrolled
- Specialist referral
24
Q
Describe the pharmacology of asthma therapies
A
- Beta-2 adrenergic receptor agonists act on adrenaline receptors to dilate the bronchioles and reverse bronchoconstriction, which can be short-acting (SABA, e.g. salbutamol), or long-acting (LABA, e.g. formoterol)
- Inhaled corticosteroids (ICS) reduce inflammation and reactivity of the airways (e.g. beclometasone)
- Long-acting muscarinic antagonists (LAMA) block acetylcholine receptors to dilate the bronchioles and reverse bronchoconstriction (e.g. tiotropium)
- Leukotriene receptor antagonists (LTRA) block the effects of leukotrienes to prevent inflammation, bronchoconstriction, and mucus secretion
25
What are the features and management of a moderate acute asthma attack?
Features:
- able to talk
- oxygen saturations > 92%
- peak flow > 50% of best
- respiratory rate <40 for 2-5 years, <30 for 5+
- heart rate <140 for 2-5 years, <125 for 5+
Management:
- reassurance to child and parents
- SABA via spacer 2-4 puffs every 2 mins up to 10 puffs
- oral prednisolone (3-5 days)
- monitor response
26
What are the features and management of severe acute asthma?
Features:
- incomplete sentences or too breathless to talk/feed
- oxygen saturations < 92%
- peak flow 33-50% of best
- respiratory rate >40 for 2-5 years, >30 for 5+
- heart rate >140 for 2-5 years, >125 for 5+
- recessions or use of accessory neck muscles
Management:
- high flow oxygen
- nebulised or inhaled salbutamol, repeat as required
- oral prednisolone, or IV hydrocortisone (continue for 3-5 days)
- consider nebulised ipratropium, IV salbutamol, aminophylline, or magnesium sulphate
27
What are the features and management of life-threatening acute asthma?
Features:
- silent chest
- cyanosis
- poor respiratory effort
- altered consciousness
- agitation or confusion
- hypotension
- peak flow <33% of best
- oxygen saturation <92%
- normal paCO2
Management:
- high flow oxygen
- nebulised SABA, repeated as required
- oral prednisolone, or IV hydrocortisone
- nebulised ipratropium
- consider IV salbutamol, aminophylline, or magnesium
- discuss with HDU/ICU, may need ventilation
28
What are the most common causes of pneumonia?
- Newborn: organisms from mothers genital tract e.g. group B strep, gram-negative bacilli
- Viral: RSV, parainfluenza, influenza
- Bacterial: strep. pneumoniae (most common), H. influenzae, strep. pyogenes, staph. aureus, Mycoplasma, chlamydia pneumoniae
- In cystic fibrosis: staph. aureus, Pseudomonas aeruginosa
- In immunocompromised: Pneumocystis jirovecii, CMV
29
What are the features of pneumonia?
- High fever
- Productive cough
- Increase work of breathing (tachypnoea, recessions, etc.)
- Low oxygen saturations
- Cyanosis
- Tachycardia
- Hypotension
- Drowsiness or confusion
- Focal coarse crackles and dull percussion on examination
30
What investigations are needed for pneumonia?
- Chest x-ray: consolidation
- Blood tests: raised WBCs and CRP
- Sputum sample: for viral PCR and bacterial culture
- Blood cultures: if suspecting sepsis
31
What is the management of pneumonia?
- Managed at home or in hospital (if oxygen <92%, apnoea/grunting, inadequate fluid intake)
- Antibiotics: amoxicillin +/- clarithromycin (covers mycoplasma), or co-amoxiclav (in cystic fibrosis, to cover staph. aureus)
- Supportive care: oxygen, fluids, etc.
32
What are the causes of recurrent chest infections?
- Cystic fibrosis
- Primary ciliary dyskinesia
- Bronchiectasis
- Immunodeficiency
- Neurological disease
- Congenital heart disease
33
What does the term upper respiratory infection include?
The most common presentation is a combination of these conditions...
- common cold (coryza)
- sore throat (pharyngitis, tonsilitis)
- acute otitis media
- sinusitis (uncommon)
- cough (secondary to postnasal drip, or attempts to clear secretions)
34
What are the causes of a persistent or recurrent cough?
- Recurrent respiratory infections
- Following specific infections (e.g. pertussis, RSV, mycoplasma - can last up to 8 weeks)
- Asthma (associated with wheeze)
- Persistent lobar collapse following acute infection
- Persistent bacterial bronchitis
- Suppurative lung diseases (e.g. cystic fibrosis, ciliary dyskinesia)
- Recurrent aspiration (due to GORD, or swallowing problems)
- Inhaled foreign body
- Smoking (active or passive)
- Tuberculosis
- Habit cough
35
What are the complications of upper respiratory tract infections?
- Difficulty feeding (blocked nose obstructs breathing)
- Febrile seizures
- Acute exacerbations of asthma
- Hospital admission (rare, if feeding/hydration is inadequate)
36
Describe a common cold (coryza)
- Most common infection in childhood
- Presentation: nasal discharge and blockage, cough, sore throat, fever
- Pathogens: rhinovirus, coronavirus, RSV
- Treatment: self-limiting, simple analgesia for pain, antibiotics have o benefit
37
Describe tonsilitis
- Common pathogens are viral, or group A strep or strop pneumoniae
- Presentation: fever, reduced oral intake, headache, abdominal pain, cervical lymphadenopathy
- Examination: red, inflamed, enlarged tonsils with or without exudates
- Management: supportive care, antibiotics if indicated (penicillin V or clarithromycin), admission if immunocompromised, respiratory distress, or evidence of peritonsillar abscess
38
Describe the feverPAIN score and Centor criteria
FeverPAIN: score of 2-3 = 30-40%, and 4-5 = 62-65% chance of bacterial cause...
- Fever: present in last 24 hours
- P: pus on tonsils
- A: attends within 3 days
- I: inflamed tonsils
- N: no cough or coryza
Centor criteria: score of 3 or more = 40-60% chance of bacterial tonsilitis...
- Fever over 38
- Tonsillar exudates
- Absence of cough
- Tender cervical lymph nodes
39
What are the indications for tonsillectomy?
- 7 or more episodes in 1 year
- 5 episodes per year for 2 years
- 3 episodes per year for 3 years
- Recurrent tonsillar abscesses
- Enlarged tonsils causing difficulty breathing, swallowing, or snoring
40
Define acute otitis media
- Common infection of the middle ear, affecting all ages but typically under 4s
- Pathogens: viruses (RSV, influenza, rhinovirus), bacteria (strep. pneumoniae, H. influenza, Moraxella catarrhalis)
41
What are the clinical features of acute otitis media?
- Ear pain
- Reduced hearing
- Fever
- Coryza
- Sore throat
- Discharge (in tympanic membrane perforation)
- Non-specific illness (e.g. vomiting, lethargy, poor feeding)
Examination:
- Bulging tympanic membrane
- Opacification or erythema of the tympanic membrane (loss of light reflection)
- Perforation with discharge
42
What is the management of acute otitis media
- Most cases resolve spontaneously with supportive care and no antibiotics
- Consider antibiotics (amoxicillin, immediate or delayed) if severe symptoms (e.g. bilateral or with discharge), systemically unwell, or immunocompromised
- Admit if under 3 months with a fever above 38, or under 6 months if above 39
43
Describe otitis media with effusion
- Collection of fluid within the middle ear, due to Eustachian tube dysfunction, causing hearing loss
- Risk factors: under 3 years old, winter months, atopic conditions, craniofacial abnormalities (cleft palate, Down syndrome), bottle feeding, passive smoking
- May contribute to speech and language delay, behavioural problems, balance issues
- Examination: dull or retracted tympanic membrane with visible air bubbles or fluid level
- Management: conservative, usually self resolves, may require hearing aids, or grommets to drain fluid
44
Describe bronchiectasis
- Localised or generalised permanent dilatation of the airways secondary to chronic infection, inflammation, or long-term conditions (e.g. COPD, cystic fibrosis, primary ciliary dyskinesia)
- Results in a persistent productive cough with large volumes of sputum, dyspnoea, and wheeze/crackles on examination
- Management: postural drainage, airway clearance techniques (physio) antibiotics for exacerbation, bronchodilators, surgical management (resection or transplant)
45
Define cystic fibrosis
Autosomal recessive disorder caused by CFTR gene mutations, primarily affecting the respiratory and gastrointestinal systems, screened for in newborns, with average life expectancy of 40, most common in Caucasians (incidence = 1 in 2500, carriers = 1 in 25)
46
Describe the pathophysiology of cystic fibrosis
- Defective cystic fibrosis transmembrane conductance regulator (CFTR) protein, due to a number of different possible gene mutation on chromosome 7
- The CFTR protein regulates ion transport across epithelial cells, and malfunction results in retention of chloride, sodium and water in the cells and thickened mucus
- In the airways, this causes retention of mucopurulent secretions, chronic infection, progressive lung damage
- In the intestine, thick viscid mucus can result in meconium ileus in some infants or obstruction in older children
- In the pancreas, ducts become blocked by thick secretions, leading to pancreatic enzyme deficiency and malabsorption
- In the liver, blockage of the biliary ducts can cause liver disease such as biliary cirrhosis
- In the reproductive system: males can be infertile due to absence of the vas deferens, and females can have thickened cervical mucus, also contributing to infertility
- Abnormal function of the sweat glands results in excessive concentrations of sodium and chloride in the sweat, causing dehydration and electrolyte imbalances
47
What are the clinical features of cystic fibrosis?
Respiratory:
- persistent cough
- wheezing and dyspnoea
- recurrent infections
- nasal polyps and chronic sinusitis
- finger clubbing
GI:
- meconium ileus
- pancreatic insufficiency
- distal intestinal obstruction
- biliary cirrhosis
- GORD
- malabsorption of vitamin D causing osteoporosis
Endocrine:
- delayed growth and pubertal development
- diabetes
Reproductive:
- male infertility
- female subfertility
48
What investigations are needed for cystic fibrosis?
- Newborn screening: raised immunoreactive trypsin warrants further testing
- Sweat chloride test: levels >60 are diagnostic
- Genetic testing: confirms diagnosis and prognosis
- Regular sputum cultures: for monitoring, and guides antibiotic therapy
49
What are the common microbial colonisers in cystic fibrosis?
- Staphylococcus aureus
- Haemophilus influenza
- Klebsiella pneumoniae
- Pseudomonas aeruginosa (affects 25%, increases morbidity, must avoid others with CF, treated with nebulised colistimethate and oral ciprofloxacin)
50
What is the management of cystic fibrosis?
Respiratory:
- chest physio
- regular exercise
- mucolytics
- bronchodilators
- anti-inflammatories
- nebulised hypertonic saline
- antibiotics (prophylactic and for exacerbations)
- nebulised Dornase alfa (breaks down DNA in secretions)
Nutritional:
- high calorie diet
- pancreatic enzyme replacement
- vitamin supplements
Complications:
- insulin
- assisted reproductive technologies
- lung transplant
- liver transplant
CFTR modulators:
- available for specific criteria are met
- e.g. ivacaftor, lumacaftor
51
Describe primary ciliary dyskinesia
- Autosomal recessive condition affecting the structure or function of cilia lining the respiratory tract, leading to impaired mucociliary clearance
- Clinical features: recurrent respiratory infections, bronchiectasis, sinusitis, subfertility, situs inversus (major organs in mirror position, in 50%)
- Diagnosis: microscopy of ciliated epithelium sample, family history (consanguinity)
- Treatment: regular chest physio, antibiotics, ENT
52
Describe tuberculosis
- Risk factors: immunosuppression, malnutrition, poverty, foreign travel
- Features: fever, persistent cough, dyspnoea, reduced appetite
- Investigations: tuberculin skin test, interferon-gamma release assay, chest x-ray (upper lobe infiltrates, hilar lymphadenopathy)
- Management: rifampicin, isoniazid (6 months), pyrazinamide, ethambutol (2 months)
53
Describe the circulatory changes at birth
- In antenatal circulation, the left atrium is at low pressure and the right atrium is at higher pressure, meaning that blood flows through the foramen ovale across the septum
- With the first breaths, resistance to pulmonary blood flow falls and the volume of blooding flowing to the lungs increases 6 fold
- Meanwhile the volume of blood returning to the right atrium falls as the placenta is excluded
- This change in pressure difference causes the flap valve of the foramen ovale to close
- The ductus arteriosus (which connects the pulmonary artery to the aorta in the foetus) will also close within the first few hours or days of life
54
What are the most common congenital heart lesions?
Left-to-right shunts (breathless):
- ventricular septal defect
- persistent ductus arteriosus
- atrial septal defect
Right-to-left shunts (blue):
- tetralogy of Fallot
- transposition of the great arteries
Common mixing (breathless and blue):
- atrioventricular septal defect
Outflow obstruction in a well child (asymptomatic with murmur):
- pulmonary stenosis
- aortic stenosis
Outflow obstruction in a sick neonate (collapsed with shock):
- coarctation of the aorta
55
What are the common causes of congenital heart diseases?
Maternal disorders...
- rubella infection = peripheral pulmonary stenosis, PDA
- systemic lupus erythematosus = complete heart block
- diabetes mellitus = incidence increased overall
Maternal drugs...
- warfarin therapy = pulmonary valve stenosis , PDA
- fetal alcohol syndrome = ASD, VSD, tetralogy of Fallot
Chromosomal abnormality...
- Down syndrome (trisomy 21) = atrioventricular septal defect, VSD
- Edwards syndrome (trisomy 18) = complex
- Patau syndrome (trisomy 13) = complex
- Turners syndrome = aortic valve stenosis, coarctation of aorta
- Noonan syndrome = hypertrophic cardiomyopathy, ASD, pulmonary valve stenosis
- Duchenne muscular dystrophy = cardiomyopathy
56
What are the three main types of atrial septal defect?
- Ostium secundum ASD: most common, defect in the centre of the atrial septum, arises from enlarged foramen ovale
- Ostium primum ASD (partial atrioventricular septal defect): intraarterial communication between the bottom end of atrial septum and atrioventricular valve
- Sinus venous ASD: defects located near the inflow of the superior or inferior vena cava
57
What are the clinical features of atrial septal defects?
- Asymptomatic (common)
- Shortness of breath
- Difficulty feeding
- Poor weight gain
- Stroke (emboli from right side of heart bypasses lungs through ASD and travels to brain)
- Right sided heart failure and pulmonary hypertension
- Recurrent chest infection/wheeze
- Arrhythmias/palpitations in older children/adults
- Ejection systolic murmur (heard at upper left sternal edge, due to increased flow across pulmonary valve due to left-to-right shunt)
- Fixed and widely split second heart sound (due to closure of the aortic and pulmonary valves at different times)
58
What is the management of atrial septal defects?
- Confirm diagnosis with echocardiogram
- Active monitoring (for small defects)
- Medical management heart failure and arrhythmias
- Percutaneous transvenous catheter closure
- Surgical correction in open heart surgery
59
Describe ventricular septal defects
- Account for 30% of all congenital heart disease, associated with Down's and Turner's syndrome
- Defects can be anywhere in the ventricular septum (peri-membranous or muscular)
- Categorised as small and large (less than/more than the diameter of the aortic valve, roughly 3mm)
60
What are the clinical features of a ventricular septal defect?
- Asymptomatic if small
- Pan-systolic murmur (at lower left sternal edge)
- Breathlessness
- Poor feeding
- Heart failure
- Faltering growth
- Recurrent chest infections
- No or soft pansystolic murmur
- Apical mid-diastolic murmur
- Loud pulmonary second sound
61
What is the management of ventricular septal defects?
- Confirm diagnosis with echocardiogram
- Active monitoring (for small defects)
- Medical management of heart failure
- Percutaneous transvenous catheter closure
- Surgical correction with open heart surgery
- Prophylaxis against endocarditis for some dental/surgical procedures
62
Describe patent ductus arteriosus
- Failure of the closing of ductus arteriosus which should occur after 1 month of expected delivery date
- Blood flows from the aorta to the pulmonary artery (left to right) following the fall in levels of prostaglandin E2 after birth
- In preterm infants, PDA is due to prematurity not congenital heart disease
- May be related to genetic conditions (e.g. Down's syndrome) or maternal condition (e.g. rubella)
63
What are the clinical features of patent ductus arteriosus?
- Continuous "machinery" murmur beneath the left clavicle
- Shortness of breath
- Difficulty feeding
- Poor weight gain
- Recurrent lower respiratory infection
- Wise pulse pressure
- Large volume, bounding, collapsing pulse
64
What is the management of patent ductus arteriosus?
- Confirm diagnosis with echocardiogram
- Medical closure with ibuprofen or indomethacin (inhibits prostaglandin)
- Mechanical closure with transcatheter procedure or open surgery
65
Describe Eisenmenger syndrome
- Reversal of a left-to-right shunt in a congenital heart defect (ASD, VSD, PDA) due to pulmonary hypertension
- Over time, high pulmonary blood flow causes thickened pulmonary artery walls which increases resistance, so that pulmonary pressure rises above arterial pressure, so that eventually blood flow is shunted right-to-left
- Features: cyanosis, dyspnoea, clubbing, oedema, raised JVP, right ventricular heave, murmur due to underlying defect
- Management: sildenafil for pulmonary hypertension, diuretics for oedema, anticoagulation for thrombosis, antibiotics for endocarditis, definitive cure is heart-lung transplant
66
What are the anatomical features of tetralogy of Fallot?
- Large ventricular septal defect
- Overring of the aorta
- Pulmonary stenosis
- Right ventricular hypertrophy
67
What are the clinical features of tetralogy of Fallot?
- Most are diagnosed antenatally
- Cyanosis
- Collapse
- Finger clubbing
- Poor feeding and weight gain
- Harsh ejection systolic murmur at left sternal edge
- Hypercyanotic (tet) spells (rapidly increasing cyanosis, severe hypoxia, irritability, breathlessness)
68
What is the management of tetralogy of Fallot?
- In neonates: prostaglandin E1 infusion to maintain ductus arteriosus
- Shunt to create connection between systemic artery and pulmonary artery (temporary solution)
- Definitive cure: surgical repair around 6 months old
- Tet spells treated with oxygen, beta-blockers, fluids
69
Describe transposition of the great arteries
- Swapped attachments of the aorta and pulmonary artery
- This forms two parallel circulations which would be incompatible with life
- Often associated with other anomalies (e.g. VSD, ASD, PDA), so mixing of oxygenated blood does occur
70
What are the clinical features of transposition of the great arteries?
- Often diagnosed antenatally, with normal foetal development
- Cyanosis (may be profound, or less severe/delayed if there is more mixing of blood)
- After initial compensation with PDA, develop respiratory distress, and poor feeding and weight gain
- Second heart sound often loud and single
- 'Egg on the side' appearance on x-ray
71
What is the management of transposition of the great arteries?
- Prostaglandin E2 infusion: maintains ductus arteriosus
- Balloon septostomy: catheter inserted into foramen ovale to allow mixing between atria (temporary measure)
- Arterial switch operation: performed in first few days of life (definitive cure)
72
Describe coarctation of the aorta
- Narrowing of the aorta, usually at the insertion of the ductus arteriosus
- Results in left ventricular outflow obstruction, increased proximal pressure and decreased distal pressure
- Associated with Turner's syndrome
73
What are the clinical features of coarctation of the aorta?
- Examination on the first day of life is usually normal, before the duct closes
- Neonates may present after duct closes with acute circulatory collapse
- Reduced femoral pulses
- Difference in blood pressure between upper and lower limbs
- Systolic murmur loudest below left scapula or clavicle
- Other signs: heart failure, dyspnoea, poor feeding
74
What is the management of coarctation of the aorta?
- Prostaglandin infusion (in duct-dependent neonates)
- Mild cases: no intervention needed
- Severe cases: early surgical intervention
75
Describe aortic stenosis
- Narrowing of the aortic valve, restricting blood flow from the left ventricle
- May be associated with abnormal number of valve leaflets (commonly bicuspid)
- Asymptomatic if mild, btu can cause fatigue, dyspnoea, dizziness, syncope (worse on exertion)
- Signs: ejection systolic murmur over right sternal edge radiating to carotids
- Management: percutaneous balloon valvuloplasty, or valve replacement
76
Describe pulmonary valve stenosis
- Narrowing of the pulmonary valve, due to thickening or fusion of the valve leaflets
- Associated with tetralogy of Fallot, William and Noonan syndrome
- Can be asymptomatic, or cause fatigue on exertion, dyspnoea, dizziness, syncope
- Signs ejection systolic murmur over left sternal edge, widely split second heart sound
- Management: percutaneous balloon valvuloplasty, or valve replacement
77
Describe Ebstein's anomaly
- Low insertion of the tricuspid valve, resulting in a large atrium and small ventricle (atrialisation of the right ventricle)
- May be caused by lithium exposure in-utero
- Associated with ASD, PDA, and Wolff-Parkinson White syndrome
- Presentation: cyanosis, heart failure, arrythmias (SVT), tricuspid regurgitation
- Management: medical therapy for heart failure and arrhythmias, surgical repair or replacement of tricuspid valve
78
What are some causes of acute abdominal pain?
- Urinary tract infection
- Pyelonephritis
- Gastroenteritis
- Appendicitis
- Mesenteric adenitis
- Intussusception
- Bowel obstruction
- Testicular torsion
- Ectopic pregnancy
- Ovarian cyst torsion/rupture
- Diabetic ketoacidosis
79
What are some causes of chronic abdominal pain?
- Functional abdominal pain
- Constipation
- GORD
- Peptic ulcers
- Infantile colic
- Abdominal migraine
- Coeliac disease
- IBD
- IBS
- Cystic fibrosis
- Sickle cell
- Tumours (neuroblastoma, Wilms tumour)
- Gynaecological pain (dysmenorrhoea, Mittelschmerz)
80
What are some common causes of vomiting?
- Feeding problems
- GORD
- Gastroenteritis
- Cow's milk protein intolerance/allergy
- Pyloric stenosis
- Intestinal volvulus
- Intussusception
- Appendicitis
- Constipation
- DKA
- UTI
- Raised intracranial pressure
- Migraine
- Meningitis
- Bulimia nervosa
- Pregnancy
81
What are the red flag features of vomiting?
- Bile-stained vomit (intestinal obstruction)
- Haematemesis (oesophagitis, peptic ulcers, oral/nasal bleeding)
- Projectile vomit in neonate (pyloric stenosis)
- Abdominal tenderness (surgical abdomen)
- Abdominal distension (intestinal obstruction)
- Hepatosplenomegaly (chronic liver disease, inborn error of metabolism)
- Blood in stool (intussusception, bacterial gastroenteritis, IBD)
- Severe dehydration/shock (systemic infection, DKA, severe gastroenteritis)
- Bulging fontanelle or seizures (raised intracranial pressure)
- Faltering growth (GORD, coeliac disease, chronic GI conditions)
82
Define GORD
Involuntary passage of gastric contents into the oesophagus, due to...
- immature lower oesophageal sphincter
- shorter oesophagus
- slower gastric emptying
- liquid diet and supine positioning
83
What are the clinical features of GORD?
- Frequent regurgitation
- Irritability/distress during or after feeds
- Back arching during feeds
- Persistent feeding difficulties (e.g. refusal, gagging)
- Chronic cough
- Hoarseness
- Epigastric pain
- Faltering growth or weight loss
84
What is the management of GORD?
Practical advice:
- smaller frequent feeds
- keeping baby upright after feeds
- breastfeeding advice
Medical:
- gaviscon (first line if breast fed)
- thickened formulas (first line if formula fed)
- PP or H2 receptor antagonist
85
Describe pyloric stenosis
- Hypertrophy of the pylorus muscle, causing gastric outlet obstruction and non-bilious vomiting
- Typically presents before 3 months, more common in males
86
What are the clinical features of pyloric stenosis?
- Projectile non-bilious vomiting (immediately after feeding)
- Poor weight gain
- Dehydration
- Hypochloraemic, hypokalaemic metabolic alkalosis
- 'Olive-like' mass felt in upper abdomen
- Visible gastric peristalsis
87
What is the management of pyloric stenosis?
- Confirm diagnosis with abdominal ultrasound (target sign)
- Correct fluid and electrolyte imbalances
- Surgical correction: laparoscopic pyloromyotomy
88
Describe intussusception
- Invagination or telescoping of the bowel, commonly the ileum passing into the caecum
- Results bowel obstruction (most common cause in infants)
- Peak ages of 3 months to 2 years, more common in males
- No underlying cause but may be due to viral infection leading to enlargement of Peyer's patches, or Meckel diverticulum
89
What are the clinical features of intussusception?
- Severe colicky abdominal pain (drawing up of legs during episodes, lethargy)
- Vomiting (progresses to bilious)
- Palpable sausage-shaped mass
- Red current jelly stool (mixture of blood and mucus)
- Abdominal distention
- Constipation
90
What is the management of intussusception?
- Confirm diagnosis with ultrasound (target/donut sign)
- Enema with contrast, air, or water can both diagnose and treat
- Surgical intervention if unsuccessful or complications occur (peritonitis, perforation)
91
Define Meckel diverticulum
- Common congenital abnormality of the small intestine, caused by remanence of the vitello-intestinal duct
- Present in 2%, peak in ages 2-5 years, more common in males
- Most often contain ectopic gastric or pancreatic mucosa which secretes acids and cause ulceration
92
What are the clinical features of Meckel diverticulum?
- Most are asymptomatic
- Abdominal pain (like appendicitis)
- Rectal bleeding (minimal, or large-volume requiring transfusion)
- Intestinal obstruction, volvulus, or intussusception
93
What is the management of Meckel diverticulum?
- Confirm diagnosis with technetium scan (radioactive uptake of ectopic gastric mucosa)
- Monitoring if asymptomatic
- Surgical intervention (diverticulectomy, or segmental bowel resection)
94
Describe malrotation and volvulus
- Malrotation is a congenital abnormality of the midgut, caused by failure of the intestine to 'rotate' into the correct position during foetal life
- A volvulus results from fibrous bands which tether the caecum to the right upper quadrant, allowing the gut to twist and cause obstruction
- Presentation: colicky abdominal pain, bilious vomiting, abdominal distention
- Management: X-ray (coffee bean sign) or CT (whirl sign), surgical correction
95
Describe abdominal migraine
- Paroxysmal episodes of intense periumbilical or diffuse abdominal pain lasting between 1 hour and 3 days
- Pain interfering with normal activities, and associated with anorexia, nausea, vomiting, headaches, photophobia, pallor, and aura
- Often personal or family history of migraine, with similar triggers (stress, fatigue, travel) and relieving factors (rest, sleep) to classic migraines
- Management: low stimulus environment, paracetamol, ibuprofen, triptans, beta-blockers
96
Define Hirschsprung's disease
- The absence of parasympathetic ganglion cells from the myenteric and sub-mucosal plexuses the large bowel, resulting in narrow and contracted segment
- The abnormal bowel extends from the rectum for a variable distance proximally, ending in a normal distal colon
- More common in boys, associated with Down's syndrome
97
What are the clinical features of Hirschsprung's disease
Neonatal:
- failure to pass meconium
- abdominal distention
- repeated vomiting
Older infants and children:
- chronic constipation
- overflow incontinence
- poor weight gain
- recurrent vomiting
Enterocolitis:
- abdominal pain and distention
- fever
- offensive/bloody diarrhoea
- can lead to sepsis, necrosis, perforation
98
What is the management of Hirschsprung's disease?
- Diagnosis: rectal biopsy (aganglionic)
- Acute management of obstruction: IV fluids, NG tube decompression
- Antibiotics for enterocolitis
- Surgical removal (and anastomosis with normal bowel)
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