Conditions Flashcards
What is pneumonia?
Pneumonia is simply an infection of the lung tissue. It causes inflammation of the lung tissue and sputum filling the airways and alveoli. Pneumonia can be seen as consolidation on a chest xray. It can be caused by a bacteria, virus or atypical bacteria such as mycoplasma.
What are the signs and symptoms of pneumonia in children?
-Cough (typically wet and productive)
-High fever (> 38.5ºC)
-Tachypnoea
-Tachycardia
-Increased work of breathing
-Lethargy
-Delirium (acute confusion associated with infection)
Signs
There may be a derangement in basic observations. These can indicate sepsis secondary to the pneumonia:
-Tachypnoea (raised respiratory rate)
-Tachycardia (raised heart rate)
-Hypoxia (low oxygen)
-Hypotension (shock)
-Fever
-Confusion
What are the characteristic chest signs of pneumonia?
-Bronchial breath sounds. These are harsh breath sounds that are equally loud on inspiration and expiration. These are caused by consolidation of the lung tissue around the airway.
-Focal coarse crackles caused by air passing through sputum similar to using a straw to blow into a drink.
-Dullness to percussion due to lung tissue collapse and/or consolidation.
What are the bacterial causes of paediatric pneumonia?
-Streptococcus pneumonia is most common
-Group A strep (e.g. Streptococcus pyogenes)
-Group B strep occurs in pre-vaccinated infants, often contracted during birth as it often colonises the vagina.
-Staphylococcus aureus. This causes typical chest xray findings of pneumatocoeles (round air filled cavities) and consolidations in multiple lobes.
-Haemophilus influenza particularly affects pre-vaccinated or unvaccinated children.
-Mycoplasma pneumonia, an atypical bacteria with extra-pulmonary manifestations (e.g. erythema multiforme).
What are the viral causes of paediatric pneumonia?
-Respiratory syncytial virus (RSV) is the most common viral cause
-Parainfluenza virus
-Influenza virus
How might you investigate pneumonia in a child?
A chest xray is the investigation of choice for diagnosing pneumonia. It is not routinely required, but can be useful if there is diagnostic doubt or in severe or complicated cases.
Viral pneumonia is more common in children <2 and if they have mild symptoms they can generally be discharged without the need for antibiotics.
Sending sputum cultures and throat swabs for bacterial cultures and viral PCR can establish the causative organism and guide treatment. All patients with sepsis should have blood cultures. Capillary blood gas analysis can be helpful in assessing or monitoring respiratory or metabolic acidosis and the blood lactate level in unwell patients.
How is Pneumonia in children managed?
Bacterial pneumonia should be treated with antibiotics according to local guidelines.
Amoxicillin is often used first line. Adding a macrolide (erythromycin, clarithromycin or azithromycin) will cover atypical pneumonia. Macrolides can be used as monotherapy in patients with a penicillin allergy.
IV antibiotics can be used when there is sepsis or a problem with intestinal absorption.
Oxygen is used as required to maintain saturations above 92%.
What investigations might you undertake in a child with recurrent LRTI requiring antibiotics or admission?
It is worth considering further investigations for underlying lung or immune system pathology.
A thorough history (including family history) and examination is needed to assess for reflux, aspiration, neurological disease, heart disease, asthma, cystic fibrosis, primary ciliary dyskinesia and immune deficiency.
The following tests can be done:
-Full blood count to check levels of various white blood cells.
-Chest xray to screen for any structural abnormality in the chest or scarring from the infections.
-Serum immunoglobulins to test for low levels of certain antibody classes indicating selective antibody deficiency.
-Test immunoglobulin G to previous vaccines (i.e. pneumococcus and haemophilus). Some patients are unable to convert IgM to IgG, and therefore cannot form long term immunity to that bug. This is called an immunoglobulin class-switch recombination deficiency.
-Sweat test to check for cystic fibrosis.
-HIV test, especially if mum’s status is unknown or positive.
What are the common congenital heart lesions by presentation?
Left-to-right shunts (breathless)
-Ventricular septal defect (30%)
-Persistent arterial duct (12%)
-Atrial septal defect (7%)
Right-to-left shunts (blue)
-Tetralogy of fallot (5%)
-Transposition of the great arteries (5%)
Common mixing (breathless and blue)
-Atrioventricular septal defect (complete) (2%)
Outflow obstruction in a well child (asymptomatic with a murmur)
-Pulmonary stenosis (7%)
-Aortic stenosis (5%)
Outflow obstruction in a sick neonate (collapsed with shock)
-Coarctation of the aorta (5%)
What are the main circulatory changes at birth?
In the foetus, the left atrial pressure is low, as relatively little blood returns from the lungs. The pressure in the right atrium is higher than in the left, as it receives all the systemic venous return including blood from the placenta. The flap valve of the foramen ovale is held open, blood flows across the atrial septum into the left atrium, and then into the left ventricle, which in turn pumps it to the upper body.
With the first breaths, resistance to pulmonary blood flow falls and the volume of blood flowing through the lungs increases 6-fold. This results in a rise in the left atrial pressure. Meanwhile, the volume of blood returning to the right atrium falls as the placenta is excluded from the circulation. The change in the pressure difference causes the flap valve of the foramen ovale to be closed. The ductus arteriosus will normally close within the first few hours or days. Some babies have duct-dependent circulation. Their clinical condition will deteriorate dramatically when the duct closes.
What are the main causes of congenital heart disease?
- Maternal disorders
-Rubella infection (Peripheral pulmonary stenosis, PDA)
-Lupus (Complete heart block, anti-rho and anti-la antibody)
-Diabetes mellitus - Maternal drugs
-Warfarin therapy (Pulmonary valve stenosis, PDA)
-Fetal alcohol syndrome (ASD, VSD, tetralogy of fallot) - Chromosomal abnormalities
-Down’s syndrome (Atrioventricular septal defect, VSD)
-Edwards syndrome [Trisomy 18]
-Patau syndrome [Trisomy 13]
-Turner syndrome (Aortic valve stenosis, coarctation of the aorta)
-Chromosome 22q11.2 deletion (aortic arch anomalies, tetralogy of fallot, common arterial trunk)
-Williams syndrome (Supravalvular aortic stenosis, peripheral pulmonary stenosis)
-Noonan syndrome (Hypertrophic cardiomyopathy, Atrial septal defect, pulmonary valve stenosis)
In what ways does congenital heart disease present?
-Antenatal cardiac ultrasound diagnoses
-Detection of a heart murmur
-Heart failure
-Shock
-Cyanosis
What is an innocent murmur? What are the hallmarks?
Innocent murmurs can be heard at some time in 30% of children. Hallmarks of an innocent murmur are:
-Asymptomatic
-Soft blowing murmur
-Systolic murmur only, not diastolic
-Left sternal edge
-Normal heart sounds with no added sounds
-No parasternal thrill
-No radiation
During a febrile illness or anaemia, innocent or flow murmurs can often be heard because of increased cardiac output.
Why are not all murmurs found at birth?
Many newborn infants with potential shunts have neither symptoms nor a murmur at birth, as the pulmonary vascular resistance is still high. Therefore, conditions such as a VSD or PDA may only become apparent at several weeks of age when pulmonary vascular resistance falls.
What are the signs and symptoms of heart failure?
-Breathlessness (particularly on feeding or exertion)
-Sweating
-Poor feeding
-Recurrent chest infections
-Poor weight gain or faltering growth
-Tachypnoea
-Tachycardia
-Heart murmur, gallop rhythm
-Enlarged heart
-Hepatomegaly
-Cool peripheries
Signs of right heart failure (ankle oedema, ascites) are rare but may be seen with long-standing rheumatic heart disease or pulmonary hypertension, with tricuspid regurgitation and right atrial dilatation.
What are the causes of heart failurein neonates, infants and older children?
- Neonates: Usually results from the left heart obstruction. Arterial perfusion may be predominantly right to left flow of blood via the arterial duct (duct-dependent systemic circulation). Closure of the duct in these cases rapidly leads to severe acidosis, collapse and death
-Hypoplastic left heart syndrome
-Critical aortic valve stenosis
-Severe coarctation of the aorta
-Interruption of the aortic arch - Infants: After the first week of life, progressive heart failure is most likely due to a left-to-right shunt. As the pulmonary vascular resistance falls, there is a progressive increase in left to right shunt and increasing pulmonary blood flow. This causes pulmonary oedema and breathlessness.
-VSD
-AVSD
-Large persistent PDA - Older children and adolescents: If a left to right shunt is not treated, children may develop Eisenmenger’s syndrome which is irreversibly raised pulmonary vascular resistance resulting from chronic raised pulmonary arterial pressure and flow. Now the shunt is from right to left and the teenager is blue.
-Eisenmenger’s
-Rheumatic heart disease
-Cardiomyopathy
What are the types of cyanosis?
- Peripheral cyanosis: blueness of the hands and feet. May occur when a child is cold or unwell from any cause or with polycythaemia
- Central cyanosis: slate blue colour on the tongue. Associated with a fall in arterial blood oxygen tension. It can only be recognised clinically if the concentration of reduced Hb in the blood exceeds 50g/L so it is less pronounced if the child is anaemic.
What are the causes of cyanosis in a newborn infant with respiratory distress?
- Cardiac disorders - congenital heart disease
- Respiratory disorders - RDS (surfactant deficiency), meconium aspiration, pulmonary hypoplasia
- Persistent pulmonary hypertension - failure of the pulmonary vascular resistance to fall after birth
- Infection - septicaemia from group B streptococcus and other organisms
- Inborn error of metabolism - metabolic acidosis and shock
How is congenital heart disease diagnosed?
CXR
ECG
These are rarely diagnostic but may help to establish abnormality of the cardiovascular system.
Echocardiography
Doppler US
Enables almost all causes of congenital heart disease to be diagnosed.
How is patency of the ductus arteriosus maintained in duct-dependency?
Giving prostaglandin infusions.
Prostaglandin E1 is a substance produced by the ductus that keeps it open.
What are the left-to-right shunts?
-ASDs
-VSDs
-PDA
These causes breathlessness or can be asymptomatic.
What are the two types of ASD?
- Secundum (80%): defect in the centre of the atrial septum and the atrioventricular valves
- Partial AVSD: either primum = interatrial communication between the bottom end of the atrial septum and the atrioventricular valves, or abnormal atrioventricular valves, with a left valve that has three leaflets and tends to leak (regurgitant valve)
What are the clinical features of ASD?
Symptoms:
-None
-Recurrent chest infections/ wheeze
-Arrythmias (in later life)
Signs:
-Ejection systolic murmur (best heard at upper left sternal edge) due to increased flow across the pulmonary valve because of the left to right shunt
-Fixed and widely split second heart sound due to the right ventricular volume being equal in both inspiration and expiration.
-Apical pansystolic murmur from atrioventricular valve regurgitation in partial AVSD
How do you investigate ASDs?
CXR - shows cardiomegaly, enlarged pulmonary arteries and increased pulmonary vascular markings
ECG - May show RBBB in secundum ASD as well as right axis deviation due to right ventricular enlargement.
Echo - Diagnostic as shows the anatomy of the heart.
How are ASDs managed?
Children with significant ASDs significant enough to cause right ventricle dilation will require treatment.
For secundum ASDs this is by cardiac catheterisation with insertion of an occlusion device, but for partial AVSD, surgical correction is required. Treatment is undertaken at about 3 to 5 years of age in order to prevent right heart failure and arrythmias in later life.
What is a ventricular septal defect and where do they happen?
Common, accounting for 30% of all cases of congenital heart disease. There is a defect anywhere in the ventricular septum, perimembranous (adjacent to the tricuspid valve) or muscular (completely surrounded by muscles). They can most conveniently be considered according to the size of the lesion - small (smaller than the aortic valve, up to 3mm) or large (same size or bigger than the aortic valve) VSDs.
What are the clinical features of small and large VSDs?
Small:
-Usually asymptomatic
-Loud pansystolic murmur at lower left sternal edge (loud murmur implies smaller defect)
-Quiet pulmonary second sound
Large:
-Heart failure with breathlessness and faltering growth after 1 week old
-Recurrent chest infections
-Tachypnoea, Tachycardia and enlarged liver from heart failure
-Soft pansystolic murmur or no murmur at all (implying a large defect)
-Apical mid-diastolic murmur (from increased flow over the mitral valve after the blood has circulated through the lungs)
-Loud pulmonary second sound from raised pulmonary arterial pressure
How are VSDs investigated?
CXR - may be normal with a small lesion or show cardiomegaly, enlarged pulmonary arteries, increased pulmonary vascular markings or pulmonary oedema with a large lesion
ECG - may be normal with a small lesion or show biventricular hypertrophy by two months of age with a large lesion
Echocardiography - Demonstrates the precise anatomy of the defect so is diagnostic. Often there is no pulmonary hypertension with a small defect but with a large defect there will be pulmonary hypertension due to high flow.
How are VSDs managed?
Small VSDs: lesions close spontaneously, while the defect is present prevention of bacterial endocarditis is by maintaining good oral hygiene.
Large VSDs: Diuretics are given combined with captopril (ACE-i). Additional calorie input is required. Surgery is usually performed at 3-6 months of age in order to manage heart failure and faltering growth and to prevent permanent lung damage from pulmonary hypertension and high blood flow
What is persistent ductus arteriosus?
The ductus arteriosus connects the pulmonary artery to the descending aorta. In term infants, it normally closes shortly after birth. In PDA it has failed to close by 1 month after the expected date of delivery due to a defect in the constrictor mechanism of the duct.
The flow of blood across a PDA is then from the aorta to the pulmonary artery (i.e. left to right), following the fall in pulmonary vascular resistance after birth. In the preterm infant, the presence of a PDA is not from congenital heart disease but due to prematurity.
What are the clinical features of persistent ductus arteriosus?
Most children present with a continuous murmur
beneath the left clavicle. The murmur continues into diastole because the pressure in the pulmonary artery is lower than that in the aorta throughout
the cardiac cycle. The pulse pressure is increased,
causing a collapsing or bounding pulse. Symptoms
are unusual, but when the duct is large there will be
increased pulmonary blood flow with heart failure and pulmonary hypertension.
How is PDA investigated?
The chest radiograph and ECG are usually normal, but if the PDA is large and symptomatic the features on chest radiograph and ECG are indistinguishable from those seen in a patient with a large VSD (cardiomegaly, enlarged pulmonary arteries, increased pulmonary vascular markings, pulmonary oedema). However, the duct is readily identified on
echocardiography.
How is PDA managed?
Closure is recommended to abolish the lifelong risk
of bacterial endocarditis and of pulmonary vascular
disease. Closure is with a coil or occlusion device
introduced via a cardiac catheter at about 1 year of
age. Occasionally, surgical ligation is
required.
What causes right-to-left shunts (cyanotic heart disease)? How does this generally present?
-Tetralogy of fallot
-Transposition of the great arteries
Presentation is with cyanosis (blue, oxygen saturations <94%, or collapsed), usually in the first week of life.
What test is used to help determine the presence of heart disease in a cyanosed neonate?
The Hyperoxia (Nitrogen washout) test
The infant is place in 100% oxygen (headbox or ventilator) for 10 minutes. If the right radial arterial partial pressure of oxygen (PaO2) from a blood gas remains low (<15 kPa, 113 mmHg) after this time, a diagnosis of ‘cyanotic’ congenital heart disease can be made if lung disease and persistent pulmonary hypertension of the newborn have been excluded. If the PaO2 is over 20kPa, it is not cyanotic heart disease. Blood gas analysis must be performed
as oxygen saturations are not reliable enough in this
range of values.
How are confirmed cyanosed neonates managed?
-Stabilize the airway, breathing, and circulation
(ABC), with artificial ventilation if necessary.
-Start prostaglandin infusion (5ng/kg per min).
Most infants with cyanotic heart disease
presenting in the first few days of life are duct
dependent, i.e. there is reduced mixing between
the pink oxygenated blood returning from the
lungs and the blue deoxygenated blood from the
body. Maintenance of ductal patency is the key to
early survival of these children. Observe
for potential side-effects of prostaglandin –
apnoea, jitteriness and seizures, flushing,
vasodilatation and hypotension.
What are the four features of Tetralogy of fallot?
- Large VSD
- Overriding of the aorta with respect to the
ventricular septum - Subpulmonary stenosis causing right ventricular
outflow tract obstruction - Right ventricular hypertrophy as a result
What are the clinical features of tetralogy of fallot?
Symptoms
Most are diagnosed:
* antenatally or
* following the identification of a murmur in the first
2 months of life. Cyanosis at this stage may not be
obvious, although a few present with severe
cyanosis in the first few days of life.
The classical description of severe cyanosis, hypercyanotic spells and squatting on exercise developing in late infancy, is now rare in developed countries, but still common where access to the necessary paediatric cardiac services is not available. It is important to recognize hypercyanotic spells, as they may lead to myocardial infarction, cerebrovascular accidents and even
death if left untreated. They are characterized by a rapid increase in cyanosis, usually associated with irritability or inconsolable crying because of severe hypoxia and breathlessness and pallor because of tissue acidosis. On auscultation, there is a very short murmur during a spell.
Signs
* Clubbing of the fingers and toes will develop in
older children.
* A loud harsh ejection systolic murmur at the left
sternal edge from day 1 of life. With increasing right ventricular outflow tract obstruction, which is predominantly muscular and below the pulmonary valve the murmur will shorten and cyanosis will increase.
How is tetralogy of fallot diagnosed?
CXR: Relatively small heart, possibly with an uptilted apex (boot shaped) due to right ventricular hypertrophy, more prominent in the older
child. There may be a right-sided aortic arch, but characteristically there is a pulmonary artery ‘bay’, a concavity on the left heart border where the convex-shaped main pulmonary artery and right ventricular outflow tract would normally be profiled. There may also be decreased pulmonary vascular markings reflecting reduced pulmonary blood flow.
ECG: Normal at birth. Right ventricular hypertrophy when older.
Echocardiography: This will demonstrate the cardinal features, but cardiac catheterization may be required to show the detailed anatomy of the coronary arteries.
How is tetralogy of fallot managed?
- Initial management is medical, with definitive
surgery at around 6 months of age. It involves
closing the VSD and relieving right ventricular
outflow tract obstruction, sometimes with an
artificial patch which extends across the
pulmonary valve. - Infants who are very cyanosed in the neonatal
period require a shunt to increase pulmonary
blood flow. This is usually done by surgical
placement of an artificial tube between the
subclavian artery and the pulmonary artery (a
modified Blalock–Taussig shunt), or sometimes by
balloon dilatation of the right ventricular outflow
tract. - Hypercyanotic spells are usually self-limiting and
followed by a period of sleep. If prolonged
(beyond about 15 min), they should be given
prompt treatment, according to need, with:
– sedation and pain relief (morphine is excellent)
– intravenous propranolol (or an α adrenoceptor
agonist), which probably works both as a
peripheral vasoconstrictor and by relieving the
subpulmonary muscular obstruction that is the
cause of reduced pulmonary blood flow
– intravenous volume administration
– bicarbonate to correct acidosis
– muscle paralysis and artificial ventilation in
order to reduce metabolic oxygen demand.
What happens in transposition of the great arteries (TGA)?
The aorta is connected to the right ventricle and the
pulmonary artery is connected to the left ventricle
(discordant ventriculo–arterial connection). The blue
blood is therefore returned to the body and the pink
blood is returned to the lungs. There are
two parallel circulations – unless there is mixing of
blood between them, this condition is incompatible
with life. Fortunately, there are a number of naturally occurring associated anomalies, e.g. VSD, ASD and PDA as well as therapeutic interventions which can achieve this mixing in the short term.
What are the clinical features of transposition of the great arteries (TGA)?
Cyanosis is the predominant symptom. It may be
profound and life-threatening. Presentation is usually on day 2 of life when ductal closure leads to a marked reduction in mixing of the desaturated and saturated blood. Cyanosis will be less severe and presentation delayed if there is more mixing of blood from associated anomalies, e.g. an ASD.
Physical signs:
* Cyanosis is always present.
* The second heart sound is often loud and single.
* Usually no murmur, but may be a systolic murmur
from increased flow or stenosis within the left
ventricular (pulmonary) outflow tract.
How is TGA diagnosed?
CXR: This may reveal the classic findings of a narrow upper mediastinum with an ‘egg on side’ appearance of the cardiac shadow (due to the anteroposterior relationship of the great vessels, narrow vascular pedicle, and hypertrophied right ventricle, respectively). Increased pulmonary vascular markings are common due to increased pulmonary blood flow.
ECG: This is usually normal.
Echocardiography: This is essential to demonstrate the abnormal arterial connections and associated abnormalities
How is TGA managed?
- In the sick cyanosed neonate, the key is to improve
mixing. - Maintaining the patency of the ductus
arteriosus with a prostaglandin infusion is
mandatory. - A balloon atrial septostomy may be a life-saving
procedure, which may need to be performed in
20% of those with transposition of the great
arteries. A catheter with an inflatable balloon at its tip is passed through the umbilical or femoral vein and then on through the right atrium and foramen ovale. The balloon is inflated within the left atrium and then pulled back through the atrial septum. This tears the atrial septum, renders the flap valve of the
foramen ovale incompetent and so allows
mixing of the systemic and pulmonary venous
blood within the atrium. - All patients with transposition of the great arteries
will require surgery, which is usually the arterial
switch procedure in the neonatal period. In this
operation, performed in the first few days of life,
the pulmonary artery and aorta are transected above the arterial valves and switched over. In
addition, the coronary arteries have to be
transferred across to the new aorta.
What is Eisenmenger’s syndrome?
If high pulmonary blood flow due to a large left-to-right shunt or common mixing is not treated at an early stage, then the pulmonary arteries become thick walled and the resistance to flow increases. Gradually, those children that survive become less symptomatic as the shunt decreases. Eventually, at about 10–15 years of age, the shunt reverses and the teenager becomes blue, which is Eisenmenger syndrome. This situation is progressive and the adult will die in right heart failure at a variable age, usually in the fourth or fifth decade of life. Treatment is aimed at prevention of this condition, with early intervention for high pulmonary blood flow.
Transplantation is not easily available although there are now medicines to palliate such pulmonary vascular disease eg inhaled nitric oxide, intravenous magnesium sulfate, oral phosphodiesterase inhibitors including sildenafil, prostacyclin or inhaled iloprost.
Which congenital cardiac conditions cause common mixing, a blue and breathless baby?
-Complete AVSD
-Complex congenital heart disease eg tricuspid atresia
What is complete AVSD and what are the features? How is it diagnosed and managed?
This is most commonly seen in children with Down syndrome. A complete AVSD is a defect in the middle of the heart with a single five-leaflet (common) valve between the atria and ventricles, which stretches across the entire atrioventricular junction and tends to leak. As there is a large defect there is pulmonary
hypertension.
Features of a complete AVSD are:
* presentation on antenatal ultrasound
screening
* cyanosis at birth or heart failure at 2 weeks to 3 weeks of life
* no murmur heard, the lesion being detected on routine echocardiography screening in a newborn baby with Down syndrome
* there is always a superior axis on the ECG
* management is to treat heart failure medically (as for large VSD) and surgical repair at 3 months to 6 months of age.
Which conditions does complex congenital heart disease encompass?
-Tricuspid atresia
-Mitral atresia
-Double inlet left ventricle
-Common arterial trunk (truncus arteriosus)
Their main presenting feature depends on whether cyanosis or heart failure is more predominant. Tricuspid atresia is the most common.
What is tricuspid atresia and what are the clinical features?
In tricuspid atresia, only the left ventricle is effective, the right being small and nonfunctional.
Clinical features:
There is ‘common mixing’ of systemic and pulmonary venous return in the left atrium. Presentation is with cyanosis in the newborn period if duct dependent, or the child may be well at birth and become cyanosed or breathless.
How is tricuspid atresia managed?
Early palliation (as with all the common mixing complex diseases) is performed to maintain a secure supply of blood to the lungs at low pressure, by:
- A Blalock–Taussig shunt insertion (between the subclavian and pulmonary arteries) in children who are severely cyanosed
- Pulmonary artery banding operation to reduce pulmonary blood flow if breathless.
Completely corrective surgery is not possible with most, as there is often only one effective functioning ventricle. Palliation is performed (Glenn or hemi-Fontan operation connecting the superior vena cava to the pulmonary artery after 6 months of age and a Fontan operation to also connect the inferior
vena cava to the pulmonary artery at 3–5 years of age).
Thus the left ventricle drives blood around
the body and systemic venous pressure supplies blood to the lungs. The Fontan operation results in a less than ideal functional outcome, but has the advantages of relieving cyanosis and removing the long-term volume load on the single functional
ventricle.
What are the causes of outflow obstruction in the well child?
These lesions are:
* Aortic stenosis
* Pulmonary stenosis
* Adult-type coarctation of the aorta
What are the clinical features of paediatric aortic stenosis?
The aortic valve leaflets are partly fused together, giving a restrictive exit from the left ventricle. There may be one to three aortic leaflets. Aortic stenosis may not be an isolated lesion. It is often associated with mitral valve stenosis and coarctation of the aorta, and their presence should always be excluded.
Clinical features:
-Most present with an asymptomatic murmur. -Those with severe stenosis may present with reduced exercise tolerance, chest pain on exertion, or syncope.
-In the neonatal period, those with critical aortic stenosis and a duct-dependent systemic circulation may present with severe heart failure leading to shock.
Physical signs
* Small volume, slow rising pulses.
* Carotid thrill (always)
* Ejection systolic murmur maximal at the upper right sternal edge radiating to the neck.
* Delayed and soft aortic second sound.
* Apical ejection click
How is paediatric aortic stenosis investigated and managed?
Investigations
CXR: Normal or prominent left ventricle with post-stenotic dilatation of the ascending aorta.
ECG: There may be left ventricular hypertrophy.
Management
In children, regular clinical and echocardiographic assessment is required in order to assess when to intervene.
Children with symptoms on exercise or who
have a high resting pressure gradient (>64 mmHg) across the aortic valve will undergo balloon valvotomy.
Balloon dilatation in older children is generally safe and uncomplicated, but in neonates this is much more difficult and dangerous.
Most neonates and children with significant
aortic valve stenosis requiring treatment in the first few years of life will eventually require aortic valve replacement. Early treatment is therefore palliative and directed towards delaying this for as long as possible.
What are the clinical features of paediatric pulmonary stenosis?
The pulmonary valve leaflets are partly fused together, giving a restrictive exit from the right ventricle.
Clinical features
Most are asymptomatic. It is diagnosed
clinically. A small number of neonates with critical pulmonary stenosis have a duct-dependent pulmonary circulation and present in the first few days of life with
cyanosis.
Physical signs
* An ejection systolic murmur best heard at the upper left sternal edge; thrill may be present.
* An ejection click best heard at the upper left
sternal edge.
* When severe, there is a prominent right ventricular impulse (heave).
How is paediatric pulmonary stenosis investigated and managed?
CXR: Normal or post-stenotic dilatation of the pulmonary artery.
ECG: Shows evidence of right ventricular hypertrophy (upright T wave in V1).
Management
Most children are asymptomatic and when the pressure gradient across the pulmonary valve becomes markedly increased (>about 64 mmHg), intervention will be required. Transcatheter balloon dilatation is the
treatment of choice in most children.
What are the clinical features of adult-type coarctation of the aorta and how is it investigated and managed?
This uncommon lesion is not duct dependent. It gradually becomes more severe over many
years.
Clinical features
* Asymptomatic.
* Systemic hypertension in the right arm.
* Ejection systolic murmur at upper sternal edge.
* Collaterals heard with continuous murmur at the back.
* Radio-femoral delay. This is due to blood
bypassing the obstruction via collateral vessels in the chest wall and hence the pulse in the legs is delayed.
Investigations
CXR:
* ‘Rib notching’ due to the development of
large collateral intercostal arteries running under the ribs posteriorly to bypass the
obstruction.
* ‘3’ sign, with visible notch in the descending aorta at site of the coarctation.
ECG:
* Left ventricular hypertrophy
Management
When the condition becomes severe, as assessed by echocardiography, a stent may be inserted at cardiac catheter. Sometimes surgical repair is required.
What are the potential causes of outflow obstruction in a sick infant? How do they present and how are they managed in the acute setting?
These lesions include:
* Coarctation of the aorta
* Interruption of the aortic arch
* Hypoplastic left heart syndrome.
Clinical features are:
* In all of these children, they usually present sick with heart failure and shock in the neonatal period, unless diagnosed on antenatal ultrasound
Management is:
* resuscitate (ABC)
* prostaglandin should be commenced at the
earliest opportunity
* referral is made to a cardiac centre for early
surgical intervention.
What are the clinical features of aortic coarctation and how is it investigated and managed?
This is due to arterial duct tissue encircling the aorta just at the point of insertion of the duct. When the duct closes, the aorta also constricts, causing severe obstruction to the left ventricular outflow. This is the most common cause of collapse due to left
outflow obstruction.
Clinical features
Examination on the first day of life is usually normal. The neonates usually present with acute circulatory collapse at 2-days of age when the duct closes.
Physical signs
* A sick baby, with severe heart failure.
* Absent femoral pulses.
* Severe metabolic acidosis.
Investigations
CXR: Cardiomegaly from heart failure and shock.
ECG: Normal.
Management
* resuscitate (ABC)
* prostaglandin should be commenced at the
earliest opportunity
* referral is made to a cardiac centre for early
surgical intervention.
*Surgical repair is performed soon after diagnosis.
What is interruption of the aortic arch? How does it present and how is it managed?
- Uncommon, with no connection between the proximal aorta and distal to the arterial duct, so that the cardiac output is dependent on right-to-left shunt via the duct
- A VSD is usually present.
- Presentation is with shock in the neonatal period .
- Complete correction with closure of the VSD and repair of the aortic arch is usually performed within the first few days of life.
- Association with other conditions (DiGeorge
syndrome – absence of thymus, palatal defects, immunodeficiency and hypocalcaemia, and chromosome 22q11.2 microdeletion).
What are the clinical features of hypoplastic left heart syndrome and how is it investigated and managed?
In this condition there is underdevelopment of the entire left side of the heart. The mitral valve is small or atretic, the left ventricle is diminutive, and there is usually aortic valve atresia. The ascending aorta is very small, and there is almost invariably coarctation
of the aorta.
Clinical features
These children may be detected antenatally at ultrasound screening. This allows for effective counselling and prevents the child from becoming sick after birth.
If they do present after birth, they are the sickest of all neonates presenting with a duct-dependent systemic circulation. There is no flow through the left side of the heart, so ductal constriction leads to profound acidosis
and rapid cardiovascular collapse. There is weakness or absence of all peripheral pulses, in contrast to weak femoral pulses in coarctation of the aorta.
Management
The management of this condition consists of a difficult neonatal operation called the Norwood procedure.
Children who have complex lesions or are small for gestational age undergo hybrid procedures that are a combination of cardiac catheter and surgical operation. This is followed by a further operation (Glenn
or hemi-Fontan) at about 6 months of age and again (Fontan) at about 3 years of age.
What are the common reasons that children will need revision of congenital defect cardiac surgery?
The most common reason for this is replacement of artificial valves and relief of postsurgical suture line stenosis, e.g. re-coarctation or pulmonary artery
stenosis.
What are the childhood arrythmias?
-SVT
-Congenital complete heart block
-Long QT syndrome
Atrial fibrillation, atrial flutter, ectopic atrial tachycardia, ventricular tachycardia and ventricular fibrillation occur in children, but all are rare. They are most often seen in children who have undergone surgery for complex congenital heart disease.
What is SVT and how is it investigated and managed?
This is the most common childhood arrhythmia. The heart rate is rapid, between 250–300 beats/min. It can cause poor cardiac output and pulmonary oedema. It typically presents with symptoms of heart failure in the neonate or young infant. It is a cause of hydrops fetalis and intrauterine death. The term re-entry tachycardia is used because a circuit of conduction is set up, with
premature activation of the atrium via an accessory pathway. There is rarely a structural heart problem, but an echocardiogram should be performed.
Investigation
The ECG will generally show a narrow complex tachycardia of 250–300 beats/min. It may be possible to discern a P wave after the QRS complex due to retrograde activation of the atrium via the accessory pathway. If heart failure is severe, there may be changes suggestive of myocardial ischaemia,
with T-wave inversion in the lateral precordial leads. When in sinus rhythm, a short P–R interval may be discernible.
In the Wolff–Parkinson–White syndrome,
the early antegrade activation of the ventricle via the pathway results in a short P–R interval and a delta wave.
Management
In the severely ill child, prompt restoration of sinus rhythm is the key to improvement. This is achieved by:
* Circulatory and respiratory support – tissue
acidosis is corrected, positive pressure ventilation if required
* Vagal stimulating manoeuvres, e.g. carotid sinus massage or cold ice pack to face, successful in about 80%
* Intravenous adenosine – the treatment of choice. This is safe and effective, inducing atrioventricular block after rapid bolus injection. It terminates the tachycardia by breaking the re-entry circuit that is set up between the atrioventricular node and accessory pathway. It is given incrementally in
increasing doses
* Electrical cardioversion with a synchronized direct current shock (0.5–2 J/kg body weight) if
adenosine fails.
Once sinus rhythm is restored, maintenance therapy will be required, e.g. with flecainide or sotalol. Digoxin can be used on its own when there is no overt pre-excitation
wave (delta wave) on the resting ECG, but propranolol can be added in the presence of pre-excitation. Even though the resting ECG may remain abnormal, 90% of children will have no further attacks after infancy.
Treatment is therefore stopped at 1 year of age. Those who have Wolff–Parkinson–White syndrome need to be assessed to ensure they cannot conduct quickly and this may be undertaken in teenage life, with atrial
pacing. This will reduce the small chance of sudden death in such patients. Those who relapse or are at risk are usually treated with percutaneous radiofrequency ablation or cryoablation of the accessory pathway.
What is congenital complete heart block?
This is a rare condition that is usually related
to the presence of anti-Ro or anti-La antibodies in maternal serum. These mothers will have either manifest or latent connective tissue disorders. Subsequent pregnancies are often affected. This antibody appears
to prevent normal development of the electrical conduction system in the developing heart, with atrophy and fibrosis of the atrioventricular node. It may cause
fetal hydrops, death in utero and heart failure in the neonatal period. However, most remain symptom free for many years, but a few become symptomatic with presyncope or syncope. All children with symptoms
require insertion of an endocardial pacemaker. There are other rare causes of complete heart block.
What are the risks of Long QT syndrome?
Long QT syndrome may be associated with sudden loss of consciousness during exercise, stress or emotion, usually in late childhood. It may be mistakenly diagnosed as epilepsy. If unrecognized, sudden death from ventricular tachycardia may occur. Inheritance is autosomal dominant; there are several phenotypes. It has been associated with erythromycin therapy, electrolyte
disorders and head injury.
It is one of the group of channelopathies caused by specific gene mutations. Abnormalities of the sodium, potassium or calcium channels lead to gain or loss
of function. Anyone with a family history of sudden unexplained death or a history of syncope on exertion should be assessed.
What is syncope and what are the possible causes in children?
Transient loss of consciousness is usually due to syncope, when it is associated with a loss of postural tone with spontaneous recovery. It is caused by a transient impairment of brain oxygen delivery, generally due to impaired cerebral perfusion. This is common in adolescents and is usually benign,
but rarely it is due to cardiac disease, which may be life-threatening.
The causes are:
* Neurally mediated syncope – is in response to a range of provocations and stressors. These may be from just standing up too quickly (a symptom of ‘orthostatic intolerance’), to the sight of blood or needles or to a sudden unexpected pain. There is usually a prodrome of dizziness and light-headed feeling and abnormal vision often with nausea, sweating, or pallor. When associated with jerking movements, it can easily be misdiagnosed as epilepsy. In most episodes there is a maladaptive drop in blood pressure; in a significant minority there is a marked fall in heart rate and in a few there is asystole
* Cardiac syncope – may be arrhythmic, from heart block, supraventricular tachycardia, ventricular tachycardia, e.g. associated with long QT syndrome or structural, associated with aortic stenosis, hypertrophic cardiomyopathy.
Features suggestive of a cardiac cause are:
* Symptoms on exercise – potentially dangerous
* family history of sudden unexplained death
* palpitations.
Check blood pressure and for signs of cardiac disease (murmur, femoral pulses, signs of Marfan syndrome).
Investigate all presenting with transient loss of consciousness with a standard 12-lead ECG, and check the corrected Q-T interval.
What is rheumatic fever and what are the clinical features?
Acute rheumatic fever is a short-lived, multisystem autoimmune response to a preceding infection with group A β-haemolytic streptococcus. The disease mainly affects children aged 5–15 years. It progresses
to chronic rheumatic heart disease in up to 80% of cases.
After a latent interval of 2–6 weeks following a pharyngeal or skin infection, polyarthritis, mild fever and malaise develop.
Jones criteria for diagnosis of rheumatic fever = Two major, or one major and two minor, criteria plus supportive evidence of preceding group A streptococcal infection (markedly raised or rising ASO titre or positive rapid streptococcal antigen test or positive group A streptococcus on throat culture)
Major manifestations:
-Migratory arthritis (80%): Ankles, knees, and wrists, Exquisite tenderness, moderate rednes, and swelling. ‘Flitting’, lasting <1 week in a joint, but migrating to other joints over 1–2 months
-Carditis (50%); Endocarditis - significant murmur, valvular dysfunction.
Myocarditis - may lead to heart failure and death
Pericarditis - pericardial friction rub, pericardial effusion, tamponade
-Sydenham chorea (10%): 2–6 months after the streptococcal infection. Involuntary movements and emotional lability for 3–6 months
-Erythema marginatum (<5%): Uncommon, early manifestation. Rash on trunk and limbs
Pink macules spread outwards, causing pink border with fading centre. Borders may unite to give a maplike outline.
- Subcutaneous nodules (rare): Painless, pea-sized, hard. Mainly on extensor surfaces
Minor manifestations:
Fever
Polyarthralgia
Raised acute-phase reactants: ESR, C-reactive protein, leucocytosis
Prolonged P–R interval on ECG
What is chronic rheumatic heart disease?
The most common form of long-term damage from scarring and fibrosis of the valve tissue of the heart is mitral stenosis. If there have been repeated attacks of rheumatic fever with carditis, this may occur as early as
the second decade of life, but usually symptoms do not develop until early adult life. Although the mitral valve is the most frequently affected, aortic, tricuspid and
rarely, pulmonary valve disease may occur.
How is rheumatic fever treated?
Acute rheumatic fever is usually treated with bed rest and anti-inflammatory agents. While there is evidence of active myocarditis (echocardiographic changes with
a raised erythrocyte sedimentation rate), bed rest and limitation of exercise are essential.
Aspirin is very effective at suppressing the inflammatory response of the joints and heart. It needs to be given in high dosage and serum levels monitored. If the fever and inflammation do not resolve rapidly, corticosteroids may be required.
Symptomatic heart failure is treated with
diuretics and ACE inhibitors, and significant pericardial effusions will require pericardiocentesis.
Anti-streptococcal antibiotics may be given if there is any evidence of persisting infection.
Following resolution of the acute episode, recurrence should be prevented. Monthly injections of benzathine penicillin is the most effective prophylaxis. Alternatively, penicillin can be given orally every day, but it is less effective and compliance may be a problem.
Oral erythromycin can be substituted in those sensitive to penicillin.
Most recommend prophylaxis for either 10 years after the last episode of acute rheumatic fever or until the age of 21 years, whichever is the longer, or lifelong prophylaxis if there is severe valvular disease. The severity of eventual rheumatic valvular disease relates to the number of childhood episodes of rheumatic fever. Symptomatic patients are given medical therapy
to relieve symptoms and disease progression, but surgical intervention with valvular repair or replacement may be required, but carry significant risk.
What are the clinical features of infective endocarditis and how is it diagnosed, treated and prevented?
All children of any age with congenital heart disease (except secundum ASD), including neonates, are at risk of infective endocarditis. The risk is highest when there is a turbulent jet of blood, as with a VSD, coarctation
of the aorta and PDA or if prosthetic material has been inserted at surgery. It may be difficult to diagnose, but should be suspected in any child or adult with a sustained fever, malaise, raised ESR, unexplained anaemia or haematuria. The presence of the classical peripheral stigmata of infective endocarditis should not be relied upon.
Clinical signs
* Fever
* Anaemia and pallor
* Splinter haemorrhages in nailbed
* Clubbing (late)
* Necrotic skin lesions
* Changing cardiac signs
* Splenomegaly
* Neurological signs from cerebral infarction
* Retinal infarcts
* Arthritis/arthralgia
* Haematuria (microscopic).
Diagnosis
Multiple blood cultures should be taken before antibiotics are started. Detailed cross-sectional echocardiography may confirm the diagnosis by identification of vegetations but can never exclude it. The vegetations
consist of fibrin and platelets and contain infecting organisms. Acute-phase reactants are raised and can be useful to monitor response to treatment.
Treatment
The most common causative organism is α-haemolytic streptococcus (Streptococcus viridans). Bacterial endocarditis is usually treated with high-dose penicillin in combination with an aminoglycoside,
giving 6 weeks of intravenous therapy and checking that the serum level of the antibiotic will kill the organism. If there is infected prosthetic material, e.g. prosthetic valves, VSD patches or shunts, there is less chance of complete eradication and surgical removal
may be required.
Prophylaxis
The most important factor in prophylaxis against endocarditis is good dental hygiene that should be strongly encouraged in all children with congenital heart disease along with avoidance of body piercing and tattoos.
Antibiotic prophylaxis is no longer recommended in the UK, but may be required in other countries for:
* dental treatment, however, trivial
* surgery which is likely to be associated with
bacteraemia.
What is dilated cardiomyopathy?
Dilated cardiomyopathy (a large, poorly contracting heart) may be inherited, secondary to metabolic disease or may result from a direct viral infection of the myocardium. It should be suspected in any child with an enlarged heart and heart failure who has previously been well. The diagnosis is readily made on echocardiography. Treatment is symptomatic with diuretics
and ACE inhibitors and carvedilol, a beta-blocker.
The role of steroids and immunoglobulin infusion is controversial.
What cardiac changes may be seen in Kawasaki disease?
This mainly affects children of 6 months to 5 years of age.
An echocardiogram is performed
at diagnosis that may show a pericardial effusion, myocardial disease (poor contractility), endocardial disease (valve regurgitation) or coronary disease with
aneurysm formation, which can be giant (>8 mm in diameter). If the coronary arteries are abnormal, angiography or magnetic resonance imaging will be required.
What are the causes of pulmonary hypertension and how can it be managed?
Causes:
-Pulmonary arterial hypertension
*Idiopathic: sporadic or familial
*Post-tricuspid shunts (e.g. VSD, AVSD, PDA)
*HIV infection
*Persistent pulmonary hypertension of the
newborn
-Pulmonary venous hypertension
*Left-sided heart disease
*Pulmonary vein stenosis or compression
-Pulmonary hypertension with respiratory
disease
*Chronic obstructive lung disease or
bronchopulmonary dysplasia in preterm
infants
*Interstitial lung disease
*Obstructive sleep apnoea or upper airway
obstruction
- Pulmonary thromboembolic disease
-Pulmonary inflammatory or capillary disease.
From the cardiac perspective, most children with pulmonary hypertension (high pulmonary artery pressure, mean >25 mmHg) have a large post-tricuspid shunt with high pulmonary blood flow and low resistance, e.g. VSD, AVSD or PDA. The pressure falls to normal if the defect is corrected by
surgery within 6 months of age. If these children are left untreated, however, the high flow and pressure cause irreversible damage to the pulmonary vascular bed (pulmonary vascular disease), which is not correctable
other than by heart/lung transplantation.
Many medical therapies are now available, which may act on the pulmonary vasculature on the cyclic guanosine monophosphate pathway (e.g. inhaled nitric oxide, intravenous magnesium sulphate and oral phosphodiesterase inhibitors including sildenafil) or on the cyclic adenosine monophosphate pathway (intravenous prostacyclin or inhaled iloprost). In addition, endothelin receptor antagonists are valuable but expensive therapy, e.g. oral bosentan. Anticoagulation is often given with heparin, aspirin or warfarin. These medications allow transplantation to be delayed for many years.
What are the general presentations of respiratory disorders in children?
Presentation of respiratory disorders in children is with:
* upper respiratory tract symptoms of coryza, sore throat, earache, sinusitis or stridor
* lower respiratory tract symptoms of cough,
wheeze and respiratory distress.
What are the signs of moderate and severe respiratory distress in children?
As children, especially infants, have compliant chest walls and poorly developed respiratory muscles, they are particularly susceptible to respiratory failure, and early detection and prevention are the cornerstone of management. Infants may develop signs of respiratory distress from most respiratory disorders. Its features are:
* moderate – tachypnoea, tachycardia, nasal flaring, use of accessory respiratory muscles, intercostal and subcostal recession, head retraction and inability to feed
* severe – cyanosis, tiring because of increased work of breathing, reduced conscious level, oxygen saturation < 92% despite oxygen therapy.
Which children might be particularly susceptible to respiratory failure?
Ex-preterm infants with bronchopulmonary dysplasia, those with haemodynamically significant congenital heart disease or
disorders causing muscle weakness, cystic fibrosis (CF) or immunodeficiency. Susceptibility to specific acute
respiratory infections varies with age.
What is the physiology of Stridor and wheeze?
A review of the respiratory physiology explains why stridor, from extrathoracic airway obstruction in the trachea and larynx, is predominantly inspiratory, and wheeze, from intrathoracic airway narrowing, is predominantly expiratory.
Inspiration is an active process in which the contraction and downward movement of
the diaphragm combines with the upward and outward movement of the ribs to generate a negative pressure in the thoracic cavity, which sucks air into the lungs through the tube of the extrathoracic airways. A gradient of negative pressure is formed from the alveoli to the upper airway. Within the thoracic cavity the airway walls are pulled outwards by the negative intrathoracic
pressure. Above the thoracic inlet, where the external pressure is atmospheric, the negative pressure within the airways leads to a degree of inward collapse during
inspiration.
The reverse happens during expiration,
when the recoil pressure of the chest wall generates a positive intrathoracic pressure and pushes air out from the alveoli to the upper airway, compressing the intrathoracic airways but distending the extrathoracic
airway.
These changes are exaggerated during any form of airway obstruction, since the pressures generated to overcome the obstruction are even higher. As a result, obstruction to the extrathoracic airways
is worse during inspiration (causing stridor), whereas obstruction to the intrathoracic airways is worse during expiration (causing crepitations and wheeze). Narrowing of the airway due to inflammation is a feature of many respiratory pathologies.
Snoring is also inspiratory, but because it is
caused by variable partial upper airway obstruction, it is a rough inspiratory noise (stertor).
What are the common upper respiratory tract infections? What is the general presentation?
Children have a median of five upper respiratory tract infections (URTIs) per year in the first few years of life, but some toddlers and primary school-aged children have as many as 10–12 per year.
Approximately 80% of all respiratory infections involve only the nose, throat,
ears or sinuses. The term URTI embraces a number of different conditions:
* common cold (coryza)
* sore throat (pharyngitis, including tonsillitis)
* acute otitis media
* sinusitis (relatively uncommon).
The most common presentation is a child with a combination of these conditions. Cough may be troublesome and in URTI may be secondary to postnasal drip or attempts to clear upper airway secretions. URTIs may
cause:
* difficulty in feeding in infants as their noses are blocked and this obstructs breathing
* febrile seizures
* acute exacerbations of asthma.
Hospital admission is rarely required but may be necessary if feeding and fluid intake is inadequate.
What are the features and causes of the common cold (coryza)?
This is the most common infection of childhood. Classical features include a clear or mucopurulent nasal discharge and nasal blockage. The most common pathogens are viruses:
–Rhinoviruses (of which there are >100 different serotypes),
-Coronaviruses
-Respiratory syncytial virus (RSV).
Health education to advise parents that colds are self-limiting and have no specific curative treatment may reduce anxiety and save unnecessary visits to doctors. Pain is best treated with paracetamol or ibuprofen. Antibiotics are of no benefit as the common cold is viral in origin and secondary bacterial infection is very uncommon. Cough may persist for up to 4 weeks after a common cold.
What are the features and causes of pharyngitis?
In pharyngitis, the pharynx and soft palate are inflamed and local lymph nodes are enlarged and tender. It is usually due to viral infection (mostly adenoviruses, enteroviruses, as well as rhinoviruses).
In the older child, group A β-haemolytic streptococcus is a common pathogen.
What are the features and causes of tonsillitis?
Tonsillitis is a form of pharyngitis where there
is intense inflammation of the tonsils, often with a purulent exudate. Common pathogens are:
-group A β-haemolytic streptococci
-Epstein–Barr virus (infectious mononucleosis).
It is not possible to distinguish clinically between viral and bacterial causes.
Marked constitutional disturbance, such as headache, apathy and abdominal pain, white tonsillar exudate and cervical lymphadenopathy, is more common with
bacterial infection
How are pharyngitis and tonsillitis treated? What are the potential complications?
Antibiotics (e.g. penicillin V or erythromycin if there is penicillin allergy) are often prescribed for severe pharyngitis and tonsillitis even though only a third are caused by bacteria. They may hasten recovery from streptococcal infection. In order to eradicate the organism completely (and prevent rheumatic fever) 10 days of antibiotic treatment is required for pharyngitis or tonsillitis.
Rarely, in severe cases, children may require
hospital admission for intravenous fluid administration and analgesia if they are unable to swallow solids or liquids. (Amoxicillin is best avoided as it may cause a widespread maculopapular rash if the tonsillitis is due to infectious mononucleosis).
Occasionally, group A streptococcal infection results in scarlet fever, which is most common in children aged 5–12 years. Fever usually precedes the presence of headache and tonsillitis by 2–3 days. The appearance of the rash is variable, although a typical appearance will include a ‘sandpaper-like’ maculopapular rash with flushed cheeks and perioral sparing. The tongue is often white and coated and may be sore or swollen. This is the only childhood exanthema caused by a bacterium, and requires treatment with antibiotics (penicillin V or erythromycin) to prevent complications including acute glomerulonephritis or, very rarely in high-income countries, rheumatic fever.
Why are children more prone to otitis media and what at are the clinical features of acute otitis media?
Most children will have at least one episode of acute
otitis media. This is most common at 6–12 months
of age. Up to 20% will have three or more episodes.
Infants and young children are prone to acute otitis
media because their Eustachian tubes are short,
horizontal, and function poorly.
There is pain in the ear and fever. Every child with a fever must have his/her tympanic membranes examined. In acute otitis media, the tympanic membrane is seen to be bright red and bulging with loss of the normal light reflection. Occasionally, there is acute perforation of the eardrum with pus visible in the external canal.
Which pathogens commonly cause acute otitis media and what are the complications?
Pathogens include viruses, especially RSV and
rhinovirus, and bacteria including pneumococcus, nontypeable Haemophilus influenzae and Moraxella
catarrhalis.
Serious complications are mastoiditis and meningitis, but these are now uncommon. Recurrent infections may lead to glue ear and hearing loss, causing speech and learning difficulties.
How is acute otitis media treated?
Pain should be treated with an analgesic such as paracetamol or ibuprofen. Regular analgesia is more effective than intermittent (as required) and may be needed for up to a week until the acute inflammation has resolved. Most cases of acute otitis media resolve spontaneously.
Antibiotics marginally shorten the duration of pain but have not been shown to reduce the risk of hearing loss. A reasonable approach is to give the parents a prescription but ask them to use it only if the child remains unwell after 2–3 days. Amoxicillin is widely used. Neither decongestants nor antihistamines are beneficial.
What is glue ear? What are the clinical features?
Recurrent ear infections can lead to otitis media
with effusion. Children are usually asymptomatic apart from possible decreased hearing. The eardrum is seen to be dull and retracted, often with a fluid level visible.
Otitis media with effusion is very common between the ages of 2–7 years, with peak incidence between 2.5–5 years of age. It usually resolves spontaneously, but may cause conductive hearing loss as shown on pure tone audiometry (possible if >4 years old) or a flat trace on tympanometry hearing testing in younger children.
How is glue ear managed?
There is no evidence for the longterm benefit from the use of antibiotics, steroids, or decongestants.
Otitis media with effusion is the most common cause of conductive hearing loss in children and can interfere with normal speech development and
result in learning difficulties in school. In such children, insertion of ventilation tubes (grommets) is
often performed, but benefits do not last more than
12 months. In practice, children with recurrent URTIs
and chronic otitis media with effusion (glue ear) that
does not resolve with conservative measures often also undergo grommet insertion. If problems recur after grommet extrusion, then reinsertion of grommets with adjuvant adenoidectomy is often advocated, as there is some evidence that adenoidectomy can offer more long-term benefit.
What is sinusitis? How is it treated?
Infection of the paranasal sinuses may occur with
viral URTIs. Occasionally, there is secondary bacterial
infection, with pain, swelling and tenderness over the cheek from infection of the maxillary sinus. As the frontal sinuses do not develop until late childhood, frontal sinusitis is uncommon in the first decade of life. Antibiotics and analgesia are used for acute sinusitis.
What are the indications for tonsillectomy in children?
The indications for tonsillectomy are controversial,
and must be balanced against the risks of surgery, but include:
* Recurrent severe tonsillitis (as opposed to recurrent URTIs) – tonsillectomy reduces the number of episodes of tonsillitis by a third, e.g. from three to
two per year but is unlikely to benefit mild symptoms
* A peritonsillar abscess (quinsy)
* Obstructive sleep apnoea (the adenoids will also
often be removed).
What are the indications for removal of both tonsils and adenoids?
In young children, the adenoids grow proportionately faster than the airway, so that their effect of narrowing the airway lumen is greatest between ages 2–8 years. They may narrow the posterior nasal space sufficiently to justify
adenoidectomy. Indications for the removal of both
the tonsils and adenoids are controversial but include:
* Recurrent otitis media with effusion with hearing
loss, where it gives a significant long-term
additional benefit
* Obstructive sleep apnoea (an absolute indication).
What is stridor and what are the causes of acute stridor?
Stridor is a harsh, muscial sound due to partial obstruction of the lower portion of the upper airway including the upper trachea and the larynx. By far the most common cause is laryngeal and tracheal infection, where mucosal inflammation and swelling can rapidly cause life-threatening obstruction of the airway in young children.
Common causes:
-Viral laryngotracheobronchitis (‘croup’)
Rare causes:
-Epiglottitis
-Bacterial tracheitis
-Laryngeal or oesophageal foreign body
-Allergic laryngeal angioedema (seen in anaphylaxis
and recurrent croup)
-Inhalation of smoke and hot fumes in fires
-Trauma to the throat
-Retropharyngeal abscess
-Hypocalcaemia
-Severe lymph node swelling (tuberculosis, infectious mononucleosis, malignancy)
-Measles
-Diphtheria
-Psychological – vocal cord dysfunction
What are the signs of upper airway obstruction?
The severity of upper airways obstruction is best
assessed clinically by characteristics of the stridor (none, only on crying, at rest, or biphasic) and the degree of chest retraction (none, only on crying, at rest).
Severe obstruction also leads to increasing respiratory rate, heart rate, and agitation. Central cyanosis, drooling or reduced level of consciousness suggest impending complete airway obstruction. The most reliable objective measure of hypoxaemia is by measuring the oxygen saturation by pulse oximetry, but, in contrast to lung disease, is a late feature of upper airways obstruction.
Total obstruction of the upper airway may be precipitated by examination of the throat using a spatula. One must avoid looking at the throat of a child with upper airways obstruction unless full resuscitation equipment and personnel are at hand.
What are the common causes of Croup and when does it occur?
Viral croup accounts for over 95% of laryngotracheal
infections.
Parainfluenza viruses are the most common cause, but other viruses, such as rhinovirus, RSV and
influenza, can produce a similar clinical picture.
Croup typically occurs from 6 months to 6 years of age but the peak incidence is in the 2nd year of life. It is most common in the autumn.
What are the typical features of croup?
The typical features are coryza and fever followed by:
* Hoarseness due to inflammation of the vocal cords
* A barking cough, like a sea lion, due to tracheal
oedema and collapse
* Harsh stridor
* Variable degree of difficulty breathing with chest
retraction
* The symptoms often start, and are worse, at night.
How is Croup managed?
When the upper airway obstruction is mild, the stridor and chest recession disappear when the child is at rest and the child can usually be managed at home. The parents should observe the child closely for signs of increasing severity. The decision to manage the child at home or in hospital is influenced not only by the severity of the illness but also by the time of day, ease of access to hospital, the child’s age (with a low threshold for admission for those <12 months old due to their narrow airway calibre) and parental understanding and confidence about the disorder.
Oral dexamethasone, oral prednisolone, or nebulized steroids (budesonide) reduce the severity and duration of croup and are first-line therapy for
croup causing chest recession at rest. They have been shown to reduce the need for hospitalization.
In severe upper airways obstruction, nebulized
epinephrine (adrenaline) with oxygen by face mask
provides rapid but transient improvement. The child
must continue to be observed closely for 2–3 hours
after administration as the effects wear off.
Intubation for viral croup has become extremely unusual since the introduction of steroid therapy. Some children have a pattern of recurrent croup, which may be related to atopy.
What is acute epiglottitis and what are the causes?
In acute epiglottitis there is intense swelling of the
epiglottis and surrounding tissues associated with
septicaemia. It is a life-threatening emergency due to
the high risk of respiratory obstruction. It is caused by H. influenzae type b (Hib). In the UK and many other countries, the introduction of universal Hib immunization in infancy has led to more than 99% reduction in the incidence of epiglottitis and other invasive Hib infections.
How can you differentiate between croup and epiglottitis?
- Onset: Days (c) vs hours (e)
- Preceding coryza: present (c) vs not present (e)
- Cough: Severe barking (c) vs absent/slight (e)
- Able to drink: Yes (c) vs No (e)
- Drooling saliva: No (c) vs yes (e)
- Appearance: unwell (c) vs toxic or very ill (e)
- Fever: <38.5 degrees (c) vs >38.5 degrees (e)
- Stridor: Harsh, rasping (c) vs muffled, reluctant to speak (e)
What are the clinical features of acute epiglottitis?
Epiglottitis is most common in children aged 1–6
years but affects all age groups. It is important to
distinguish clinically between epiglottitis and croup as they require quite different treatment.
The onset of epiglottitis is usually very acute with:
* High fever in a very ill, toxic-looking child
* An intensely painful throat that prevents the child
from speaking or swallowing; saliva drools down
the chin
* Soft inspiratory stridor and rapidly increasing
respiratory difficulty over hours
* The child sitting immobile, upright, with an open
mouth to optimize the airway.
In contrast to viral croup, cough is minimal or absent.
How is acute epiglottitis managed?
Attempts to lie the child down or examine the throat with a spatula or perform a lateral neck X-ray to identify a swollen epiglottis and surrounding tissues must not be undertaken as they can precipitate total airway obstruction and death.
If the diagnosis of epiglottitis is suspected, urgent
hospital admission and treatment are required. A
senior anaesthetist, paediatrician, and ENT surgeon should be summoned and treatment initiated without delay. The child should be transferred directly to the intensive care unit or an anaesthetic room, and must be accompanied by senior medical staff in case respiratory obstruction occurs. The
child should be intubated under controlled conditions with a general anaesthetic. Rarely, this is impossible and urgent tracheostomy is life-saving.
Only after the airway is secured should blood be taken for culture and intravenous antibiotics such as cefuroxime started. The tracheal tube can usually be removed after 24 hours and antibiotics given for 3–5 days. With appropriate treatment, most children recover completely within 2–3 days. As with other serious H. influenzae infections, prophylaxis with rifampicin is offered to close household contacts.
What is bacterial tracheitis? What causes it and how is it managed?
Also known as pseudomembranous croup.
This rare but dangerous condition is similar to severe epiglottitis in that the child has a high fever, appears very ill, and has rapidly progressive airways obstruction with copious thick airway secretions. It is typically caused by infection with Staphylococcus aureus. Management is by intravenous antibiotics and intubation and ventilation if required.
What are the non-infectious possible causes of stridor?
When a child with acute stridor presents with atypical features or a poor response to treatment, other causes need to be considered (Box 17.1). If a child has an abrupt onset of stridor without apparent infection, consider anaphylaxis or inhaled foreign body.
Chronic stridor is usually due to a structural problem, either from intrinsic narrowing or collapse of the laryngotracheal airway, e.g. subglottic stenosis, laryngomalacia (floppy larynx), or external compression (e.g. vascular ring, lymph nodes, tumours). Investigations are required to determine the cause.
What causes wheeze?
Acute wheeze is due to a partial obstruction of the
intrathoracic airways. This is from mucosal inflammation and swelling as in bronchiolitis or bronchoconstriction as in asthma or mechanical obstruction (e.g. with foreign body or mucus). It may occur as a combination of all three.
What are the causes of bronchiolitis and what are the clinical features?
RSV is the pathogen in 80%, the remainder are accounted for by parainfluenza virus, rhinovirus, adenovirus, influenza virus, and human metapneumovirus. There is evidence that co-infection with more than one virus, particularly
RSV and human metapneumovirus may lead to a more severe illness.
Coryzal symptoms precede a dry cough and increasing breathlessness. Feeding difficulty associated with increasing dyspnoea is often the reason for admission to hospital. Recurrent apnoea is a serious complication, especially in young infants. Infants born prematurely who develop bronchopulmonary dysplasia or with other underlying lung disease, such as cystic fibrosis,
or have congenital heart disease are most at risk from severe bronchiolitis.
The characteristic findings on examination are:
* Dry wheezy cough
* Tachypnoea and tachycardia
* Subcostal and intercostal recession
* Hyperinflation of the chest
* Fine end-inspiratory crackles
* High-pitched wheezes – expiratory > inspiratory
How is bronchiolitis investigated? When is hospital admission indicated?
Pulse oximetry should be performed on all children
with suspected bronchiolitis. No other investigations
are routinely recommended. In particular, chest X-ray or blood gases are only indicated if respiratory failure is suspected.
Hospital admission is indicated if any of the following are present:
* Apnoea (observed or reported)
* Persistent oxygen saturation of < 90% when
breathing air
* Inadequate oral fluid intake (50–75% of usual
volume)
* Severe respiratory distress – grunting, marked
chest recession, or a respiratory rate over 70
breaths/minute.
How is bronchiolitis managed?
This is supportive. Humidified oxygen is either delivered via nasal cannulae or using a head box; the concentration required is determined by pulse oximetry. The infant is monitored for apnoea.
NB: No evidence for reducing severity or illness duration has been shown from use of mist, nebulized hypertonic saline, antibiotics, corticosteroids or nebulized bronchodilators, such as salbutamol or ipratropium.
Fluids may need to be given by nasogastric tube or intravenously. Assisted ventilation in the form of non-invasive respiratory support with CPAP (continuous positive airway pressure) or else
mechanical ventilation is required in a small percentage of infants admitted to hospital.
RSV is highly infectious, and infection control measures, particularly good hand hygiene, cohort nursing, and gowns and gloves, have been shown to prevent cross-infection to other infants in hospital.
Most infants recover from the acute infection within
2 weeks. However, as many as half will have recurrent episodes of cough and wheeze. Rarely, usually following adenovirus infection, the illness may result in permanent damage to the
airways (bronchiolitis obliterans).
How is bronchiolitis prevented?
A monoclonal antibody to RSV (palivizumab, given
monthly by intramuscular injection) reduces the
number of hospital admissions in high-risk preterm
infants, although 17 babies need to be treated to avoid one admission. Its use is limited by cost and the need for multiple intramuscular injections.
What are the three main patters of wheezing?
- Viral episodic wheezing – wheeze only in response
to viral infections - Multiple trigger wheeze – in response to multiple
triggers and which is more likely to develop into
asthma over time - Asthma.
What is viral episodic wheeze? What are the risk factors?
Most wheezy preschool children have viral episodic wheeze. This is thought to result from small airways being more likely to narrow and obstruct due to inflammation and aberrant immune responses to viral infection. This gives the condition its episodic nature, being triggered by viruses that cause the common cold.
Studies have found that sufferers often have reduced small airway diameter from birth.
Risk factors include maternal smoking during and/or after pregnancy and
prematurity. A family history of asthma or allergy is not a risk factor, but a family history of early viral wheezing is common.
Viral episodic wheezing is more common in males and usually resolves by 5 years of age, presumably from increase in airway size.
What is multiple trigger wheeze?
Some children, both preschool and school aged, have frequent wheeze triggered by many stimuli, not just viruses but also cold air, dust, animal dander and exercise. This has been called multiple-trigger wheeze.
In the preschool age group, where a formal diagnosis of asthma may be
unjustified, this distinction is helpful as many children in this group benefit from asthma preventer therapy and a
significant proportion go on to have asthma.
What is atopic asthma and what is it associated with?
When recurrent wheezing is associated with symptoms between viral infections (interval symptoms) and evidence of allergy to one or more inhaled allergens such as house dust mite, pollens or pets, it is called ‘atopic asthma’. Evidence of allergy may be accompanied by positive skin-prick testing or presence of IgE on blood testing.
Atopic asthma is strongly associated with other atopic diseases such as
eczema, rhinoconjunctivitis and food allergy, and is more common in those with a family history of such diseases.
What are the causes of recurrent or persistent childhood wheeze?
-Viral episodic wheeze
-Multiple trigger wheeze
-Asthma
-Recurrent anaphylaxis (e.g. in food allergy)
-Chronic aspiration
-Cystic fibrosis
-Bronchopulmonary dysplasia
-Bronchiolitis obliterans
-Tracheo-bronchomalacia
What is the pathophysiology of asthma?
Genetic predisposition, atopy, and environmental triggers (Upper respiratory tract infections, Allergens [e.g. house dust mite, grass pollens, pets], Smoking (active or passive), Cold air, Exercise, Emotional upset or anxiety, Chemical irritants [e.g. paint, aerosols]) all lead to:
Bronchial inflammation
With oedema, excessive mucus production, infiltration with cells, eosinophils, mast cells, neutrophils and lymphocytes
There is Bronchial Hyperresponsiveness - exaggerated twitchiness to inhaled stimuli
This leads to airway narrowing with reversible airflow obstruction (eg peak flow variability)
This causes wheeze, cough, breathlessness and chest tightness.
What are the clinical features of asthma?
Asthma should be suspected in any child with wheezing on more than one occasion, particularly if there are
interval symptoms. Asthmatic wheeze is a polyphonic (multiple pitch) noise coming from the airways. It is believed to represent many airways of different sizes vibrating from abnormal narrowing.
Key features associated with a high probability of a child having asthma include:
* Symptoms worse at night and in the early morning
* Symptoms that have nonviral triggers
* Interval symptoms, i.e. symptoms between acute exacerbations
* Personal or family history of an atopic disease
* Positive response to asthma therapy.
Examination of the chest is usually normal between attacks. In long-standing asthma there may be
hyperinflation of the chest, generalized polyphonic expiratory wheeze with a prolonged expiratory phase.
Onset of the disease in early childhood may result in
Harrison’s sulci. Evidence of eczema should be sought, as should examination of the nasal mucosa
for allergic rhinitis.
(The presence of a wet cough or sputum production, finger clubbing or poor growth suggests a condition
characterized by chronic infection such as cystic fibrosis or bronchiectasis)
How is asthma investigated?
In younger children, asthma is usually diagnosed from history and examination alone. Parental description of the symptoms and response to treatment is the cornerstone to diagnosis.
Sometimes, specific investigations are required to confirm the diagnosis, or determine the severity and phenotype in more detail.
-Skin-prick testing for common allergens may be performed to aid the diagnosis of atopy and to identify allergens, which may be acting as triggers.
- If there is uncertainty in the diagnosis or disease severity needs to be monitored, peak expiratory flow rate (PEFR) may be measured or spirometry performed. Peak flow is less sensitive to changes in
airway calibre than spirometry but is portable and therefore helpful for serial measurements. Most children over 5 years of age can use a peak flow meter or undertake spirometry.
Poorly controlled asthma leads to increased variability in peak flow, with both diurnal variability (morning usually lower than evening peak flow) and day-to-day variability.
Spirometry involves measurement of forced expiratory volume in 1 second blowing out as hard and as fast as possible (FEV1). This provides a non-invasive measure of flow through the larger airways (to the bronchioles).
Often, response to a bronchodilator is the most helpful investigation. This can be demonstrated as an improvement in peak flow rate or in FEV1 before and after inhaling a bronchodilator (an improvement of 12% or more confirms bronchodilator reversibility and is characteristic of asthma). Following treatment, this reversibility often reduces or disappears completely.
What bronchodilator therapy is used in asthma?
Inhaled β2-agonists are the most commonly used and most effective bronchodilators.
Short-acting β2-agonists (often called relievers) such as salbutamol or terbutaline have a rapid onset of action (maximum effects after 10–15 min), are effective for 2–4 hours and have few side-effects. They are used “as required” for increased symptoms and in high doses for acute asthma attacks.
By contrast, long-acting β2-agonists (LABAs) such as salmeterol or formoterol are effective for 12 hours. They are not used in acute asthma and should not be used without an inhaled corticosteroid. LABAs are useful in exercise-induced asthma.
Ipratropium bromide, an anticholinergic bronchodilator, is sometimes given to young infants when other bronchodilators are found to be ineffective or in the treatment of severe acute asthma.
What 6 preventer (and add-on) therapies are used in asthma?
- LABAs eg Salmeterol and Formoterol for bronchodilation. (First choice in children over 5 years)
- Inhaled corticosteroids eg Budesonide, Beclometasone, Fluticasone, Mometasone
Inhaled corticosteroids (often called preventers) are the most effective inhaled prophylactic therapy. They
decrease airway inflammation, resulting in decreased symptoms, asthma exacerbations and bronchial hyperactivity. They are often used in conjunction with an inhaled LABA or leukotriene receptor antagonist. - Leukotriene receptor antagonists (LTRA) eg Montelukast as add-on therapy (first choice in children under 5 years but can be used in older children when symptoms are not controlled by the addition of a LABA)
- Methylxanthines eg Theophylline is an alternative add-on therapy , however it has a high incidence of side effects (vomiting, insomnia, headahces, poor concentration) so is not often used in children.
- Oral steroids, prednisolone, usually given on alternate days to
minimize the adverse effect on growth, is required only in severe persistent asthma where other treatment has failed. All children on this therapy must be managed by
a specialist in childhood asthma - Anti-IgE therapy (omalizumab) is an injectable monoclonal antibody that acts against IgE, the natural antibody that mediates allergy. It is used for the treatment of severe atopic asthma, and should also only be administered by a specialist in childhood asthma.
What are the side effects of inhaled corticosteroid therapy in asthma?
They have no clinically significant side-effects when given in low dose, although they can cause mild reduction in height velocity, but this is usually followed by catch-up growth in late childhood. Systemic side effects, including impaired growth, adrenal suppression and altered bone metabolism may be seen when high doses are used. To reduce the risk of unwanted side effects, treatment with inhaled corticosteroids should always be at the lowest dose possible. Treatment for many children is effective at very low doses
What is the definition of complete astma control?
Complete control is defined as the absence of daytime or night-time symptoms, no limit on activities (including exercise), no need for reliever use, normal lung function and no exacerbations (need for hospitalization or oral steroids) in the previous 6 months
What are the steps in asthma treatment starting with step 1 (mild intermittent asthma) up to step 5 (chronic severe asthma requiring continuous or frequent use of oral steroids)?
Treatment increases through the steps and is stepped down when control is good.
STEP 1 (Mild intermittent asthma) = Short acting beta agonist, SABA
STEP 2 (Regular preventer therapy) = Add inhaled corticosteroid (200 micrograms/day or 400 for adolescents) or in those <5 years consider leukotriene receptor antagonist if steroids cannot be used.
STEP 3 (Initial add-on therapy) =
-In <5 years: add LTRA, if poor response increase ICS dose (400 micrograms/day
-In >5 years and young adults: initially add LABA. Assess the response: if good continue, if partial increase the ICS dose (400 or 800 in adolescents), if poor stop the LABA & increase the ICS & consider LTRA and/or slow release theophylline.
STEP 4 (persistent poor control) =
-In <5 years: refere to resp paediatrician
-In 5-12 years: increase ICS dose to 800 micrograms/day
-In adolescents: increase ICS to 1600 and consider LTRA or slow release theophylline. (Issue steroid replacement warning card)
STEP 5 (Continuous or frequent use of oral steroids) =
-In 5-12 years: Maintain ICS at 800. Use lowest possible daily dose of oral steroids to maintain adequate control. Refer.
-In adolescents: Maintain ICS at 1600. Use lowest possible daily dose of oral steroids to maintain adequate control. Refer.
What non-pharmacological measures are important in the treatment of asthma?
Parental smoking cessation
Psychological intervention may be useful in chronic severe asthma
How are children presenting with an acute asthma attack assessed?
With each acute attack, the duration of symptoms, the treatment already given, and the course of previous attacks should be noted.
Determine the severity of the attack based on clinical features (Mild, moderate, severe, life-threatening)
- Increased work of breathing
Chest recession:
* Moderate – some intercostal
recession
* Severe – use of accessory neck
muscles
* Life-threatening – poor respiratory effort
Auscultation:
* Wheeze
* Silent chest – poor air entry from poor expiratory effort or exhaustion in life-threatening - Cardiovascular:
* Tachycardia – varies with age; better guide to severity than respiratory rate but affected by β2-agonists
* Arrythmia, hypotension – life-threatening - Altered consciousness, agitation or
confusion – in life-threatening
Exhaustion – life-threatening - Tongue:
* Cyanosis in life-threatening - Peak flow (% predicted or best or
usual measurement):
* Moderate >50%
* Severe 33–50%
* Life-threatening <33% - O2 saturation:
* Moderate >92%
* Severe or life-threatening <92% - Is there a trigger for the attack?:
* URTI or other viral illness
* Allergen, e.g. animal dander
* Exercise
* Cold air
What are the causes of acute breathlessness in the older child?
- Asthma
- Pneumonia or lower respiratory tract infection
- Foreign body
- Anaphylaxis
- Pneumothorax or pleural effusion
- Metabolic acidosis – diabetic ketoacidosis, inborn error of metabolism, lactic acidosis
- Severe anaemia
- Heart failure
- Panic attacks (hyperventilation)
What are the criteria for admission to hospital in an acute asthma attack?
Children require hospital admission if, after high-dose inhaled bronchodilator therapy, they:
* Have not responded adequately clinically, i.e. there is persisting breathlessness or tachypnoea
* Are becoming exhausted
* Still have a marked reduction in their predicted (or usual best) peak flow rate or FEV1 (<50%)
* Have a reduced oxygen saturation (<92% in air).
A chest X-ray is indicated only if there are unusual features (e.g. asymmetry of chest signs suggesting pneumothorax, lobar collapse) or signs of severe infection. In children, blood gases are only indicated
in life-threatening or refractory cases and often are normal until the child is in extremis.
How is an acute asthma attack managed?
High-dose inhaled bronchodilators, steroids, and oxygen form the foundation of therapy of severe acute asthma.
- As soon as the diagnosis has been made, the child should be given oxygen if the oxygen saturation is <92%.
- All children should be given a β2-bronchodilator, the dose and frequency increasing according to severity of the attack, the child’s age and response to therapy. It should be given via a spacer, as used by the child at home, unless the attack is severe to life-threatening when a nebulizer driven by high-flow oxygen may be indicated.
The addition of nebulized ipratropium
to the initial therapy in severe asthma is beneficial.
- A short course (3–7 days) of oral prednisolone expedites the recovery from moderate or severe
acute asthma.
- Inhaled therapies are not always be successful as the drugs may be delivered in suboptimal doses to areas of the lung that are poorly ventilated. Intravenous therapy therefore has a role in the minority of children who fail to respond adequately to inhaled bronchodilator therapy.
-IV hydrocortisone
-Magnesium sulphate: has the least side-effects and most evidence of benefit, and is increasingly being used as the first choice for intravenous therapy. OR
-Aminophylline: a loading dose is given over 20 minutes, followed by continuous infusion. Seizures,
severe vomiting and fatal cardiac arrhythmias may follow a rapid infusion. If the child is already on oral
theophylline, the loading dose should be omitted. Monitor ECG and blood electrolytes. OR
-Intravenous salbutamol: monitor ECG and blood electrolytes
Occasionally, these measures are insufficient and mechanical ventilation is required.
Other than asthma, viral-induced wheeze, or bronchiolitis, what might cause acute wheeze?
- Atypical pneumonia – although pneumococcal pneumonia rarely causes wheezing, atypical pneumonia caused by Mycoplasma, Chlamydia or
adenovirus can do so. - Foreign body inhalation – abrupt onset of cough followed by wheeze in a previously well child. On passing below the glottis, a foreign body generally impacts in a main or lobar bronchus and may initially cause unilateral wheezing and air trapping.
A chest X-ray performed during expiration will show persistent hyperinflation of the lung distal to
the obstruction. Eventually, airway swelling causes complete obstruction and lobar collapse is seen. - Anaphylaxis – suspect if acute urticaria, facial swelling, stridor, or previous reaction to an allergen.
What pathology do the different types of cough suggest?
- Dry with prolonged expiratory phase: some narrowing of the small-sized to moderate-sized airways
- Barking cough: degree of tracheal inflammation, narrowing or collapse
- Moist: increased mucus secretion or infection in the lower airway
What is Whooping cough (pertussis)? What causes it and what are the typical features?
This is a highly contagious respiratory infection caused by Bordetella pertussis.
- Catarrhal phase: usually a week of coryza
- Paroxysmal phase: paroxysmal
or spasmodic cough followed by a characteristic inspiratory whoop. The spasms of cough are often worse at night and may culminate in vomiting. During a paroxysm, the child goes red or blue in the face, and mucus flows from the nose and mouth. The whoop may be absent in infants, but apnoea is common at this age. Epistaxis and subconjunctival haemorrhages can occur after vigorous coughing. The paroxysmal phase lasts up to 3 months. - Convalescent phase: The symptoms gradually decrease but may persist for many months.
Complications such as pneumonia, seizures and bronchiectasis are uncommon but there is still a significant mortality, particularly in infants who have not yet completed their primary vaccinations at 4 months. Infants and young children suffering severe spasms of cough or cyanotic attacks should be admitted to hospital and isolated from other children.
How is whooping cough (pertussis) diagnosed?
The organism (Bordetella pertussis) can be identified early in the disease
from culture of a pernasal swab, although PCR is more sensitive.
Characteristically, there is a marked lymphocytosis on a blood count.
How is whooping cough (pertussis) managed?
Although macrolide antibiotics (Azithromycin, clarithromycin, erythromycin) eradicate the organism, they decrease symptoms only if started during the catarrhal phase.
Siblings, parents and school contacts may develop a similar cough, and
close contacts should receive macrolide prophylaxis.
Unimmunized infant contacts should be vaccinated. Immunization reduces the risk of developing pertussis and the severity of disease in those affected but does not guarantee protection. The level of protection
declines steadily during childhood.
Reimmunization of mothers during pregnancy reduces the risk of pertussis in her infant during the first few months of life when it is particularly dangerous. It is currently recommended in the UK; this followed the re-emergence of pertussis in infants in the community.
What are the causes of persistent or recurrent cough?
- Recurrent respiratory infections
- Following specific respiratory infections (e.g. pertussis, respiratory syncytial virus, Mycoplasma)
- Asthma
- Persistent lobar collapse following pneumonia
- Suppurative lung diseases (e.g. cystic fibrosis, ciliary dyskinesia or immune deficiency)
- Recurrent aspiration (±gastro-oesophageal reflux)
- Persistent bacterial bronchitis
- Inhaled foreign body
- Cigarette smoking (active or passive)
- Tuberculosis (in a child with severe, persistent cough, TB should be considered)
- Habit cough
- Airway anomalies (e.g. tracheo-bronchomalacia, tracheo-oesophageal fistula)
What are the most common causes of pneumonia (according to age) in newborns, infants and young children and children>5 years?
Viruses are the most common cause in younger children, whereas bacteria are more common in older children,
- Newborns:
-Group B Streptococcus
-Gram-negative enterococci
-Gram negative bacilli (Klebsiella, pseudomonas aeruginosa, E. coli) - Infants and young children
-Respiratory viruses, particularly RSV, - Streptococcus pneumoniae
- H. influenzae
- Bordetella pertussis
- Chlamydia trachomatis
- Staphylococcus aureus (Infrequent bit serious). - Children over 5 years:
- Mycoplasma pneumoniae
- Streptococcus pneumoniae
- Chlamydia pneumoniae
At all ages Mycobacterium tuberculosis should be considered.
What are the clinical features of pneumonia?
Fever, cough and rapid breathing are the most common presenting symptoms. These are usually preceded by a URTI.
Other symptoms include lethargy, poor feeding, and an ‘unwell’ child. Some children do not have a
cough at presentation.
Localized chest, abdominal, or
neck pain is a feature of pleural irritation and suggests bacterial infection.
Examination reveals tachypnoea, nasal flaring and chest indrawing. In contrast to asthma, the most sensitive clinical sign of pneumonia in children is increased respiratory rate, and pneumonia can sometimes be
missed if the respiratory rate is not measured in a febrile child (so-called silent pneumonia).
There may be end-inspiratory coarse crackles over the affected area but the classic signs of consolidation with dullness on percussion, decreased breath sounds and bronchial breathing over the affected area are often absent in young children. Oxygen saturation may be decreased.
How is pneumonia investigated?
A chest X-ray may confirm the diagnosis but cannot reliably differentiate between bacterial and viral pneumonia.
In younger children, a nasopharyngeal aspirate may identify viral causes, but blood tests, including full blood count and acute phase reactants are generally unhelpful in differentiating between a viral and bacterial cause.
In a small proportion of children the pneumonia is associated with a pleural effusion, where there may be blunting of the costophrenic angle on the chest X-ray. Some of these effusions develop into empyema and fibrin strands may form, leading to septations.
How is pneumonia managed?
Most affected children can be managed at home but indications for admission include oxygen saturation <92%, recurrent apnoea, grunting and/or an inability to maintain adequate fluid/feed intake.
General supportive care should
include oxygen for hypoxia and analgesia if there is pain. Intravenous fluids should be given if necessary to
correct dehydration and maintain adequate hydration and sodium balance.
The choice of antibiotic is determined by the child’s age and the severity of illness:
- Newborns require broad spectrum intravenous antibiotics.
- Most older infants can be managed with oral amoxicillin, with broader spectrum antibiotics such as co-amoxiclav reserved for complicated or unresponsive pneumonia.
- For children over 5 years of age, either amoxicillin or an oral macrolide such as erythromycin is the treatment of choice.
There is no advantage in giving intravenous rather than
oral treatment in mild/moderate pneumonia.
What is a common complication of pneumonia in children?
Small parapneumonic effusions occur in up to one third of children with pneumonia and may resolve with
appropriate antibiotics, but persistent fever despite 48 hours of antibiotics suggests a pleural collection which requires drainage. This should be done with ultrasound guidance.
The percutaneous placement of a small-bore chest drain and regular
instillation of a fibrinolytic agent to break down the fibrin strands are usually effective, but more aggressive intervention such as video-assisted thoracoscopic surgery or even thoracotomy and decortication is
sometimes necessary in refractory cases.
What are the two main causes of chronic lung infection and how are they diagnosed and managed?
Any child with a persistent cough that sounds ‘wet’ (i.e. sounds like there is excess sputum in the chest)
or is productive requires further investigations.
- Persistent bacterial bronchitis, where there is persistent inflammation of the lower airways driven by chronic infection, is increasingly recognized as a cause of
chronic wet cough in children. Common organisms are Haemophilus influenzae and Moraxella catarrhalis.
It may be a precursor to bronchiectasis if investigations
and treatment are not instituted.
Bacterial growth from sputum or bronchial lavage is consistent with the diagnosis. Treatment is with a high
dose of antibiotic such as co-amoxiclav, coupled with
physiotherapy.
- Bronchiectasis (permanent dilatation of the bronchi). It may be generalized or restricted to a single lobe.
- Generalized bronchiectasis may be due to cystic fibrosis, primary ciliary dyskinesia, immunodeficiency, or chronic aspiration.
- Focal bronchiectasis is due to previous severe pneumonia, congenital lung abnormality, or obstruction by a foreign body.
A plain chest X-ray may show gross bronchiectasis, but often it is not possible to identify it. It is best identified on a CT scan of the chest. To investigate focal disease, bronchoscopy is usually indicated to exclude a structural cause.
What is Cystic fibrosis and what is the cause?
CF is the most common life-limiting autosomal recessive condition in Caucasians, with an incidence of 1
in 2500 live births and carrier rate of 1 in 25. It is well recognized but less common in other ethnic groups.
Average life expectancy has increased from a few years to the mid-30s, with a projected life expectancy for current newborns into the 40s.
The fundamental problem in CF is a defective protein called the CF transmembrane conductance
regulator (CFTR). This is a cyclic AMP-dependent chloride channel found in the membrane of cells. The gene for CFTR is located on chromosome 7. Over 900 different gene mutations have been discovered that cause a number of distinct defects in CFTR, but by far the most frequent mutation (about 78%) in the UK is
ΔF508.
Identification of the gene mutation involved within a family allows prenatal diagnosis and carrier detection in the wider family. Some genotypes are known to be associated with milder disease and pancreatic sufficiency.
What is the pathophysiology of cystic fibrosis?
CF is a multisystem disorder, which results mainly from abnormal ion transport across epithelial cells. In the
airways this leads to reduction in the airway surface liquid layer and consequent impaired ciliary function
and retention of mucopurulent secretions. Chronic endobronchial infection with specific organisms such
as Pseudomonas aeruginosa ensues.
Defective CFTR also causes dysregulation of inflammation and defence against infection.
In the intestine, thick viscid meconium is produced, leading to meconium ileus in 10–20% of
infants. The pancreatic ducts also become blocked by thick secretions, leading to pancreatic enzyme
deficiency and malabsorption. Abnormal function of the sweat glands results in excessive concentrations of sodium and chloride in the sweat.
What are the clinical features of cystic fibrosis by age (Newborn, Infancy, Young child, Older child and adolescent)?
Newborn:
* Diagnosed through newborn screening
* Meconium ileus
Infancy:
* Prolonged neonatal jaundice
* Growth faltering
* Recurrent chest infections
* Malabsorption, steatorrhoea
Young child:
* Bronchiectasis
* Rectal prolapse
* Nasal polyp
* Sinusitis
Older child and adolescent:
* Allergic bronchopulmonary aspergillosis
* Diabetes mellitus
* Cirrhosis and portal hypertension
* Distal intestinal obstruction (meconium ileus equivalent)
* Pneumothorax or recurrent haemoptysis
* Sterility in males
How is CF screened for in newborns?
Heel prick tests are taken for biochemical screening.
Infants with CF have raised Immunoreactive trypsinogen (IRT). These samples are then screened for common CF gene mutations and infants with two mutations have a sweat test to confirm the diagnosis.
Which bacterial respiratory infections are common in cystic fibrosis and what might these cause?
Chronic infection with specific
bacteria – initially Staphylococcus aureus and Haemophilus influenzae and subsequently with Pseudomonas
aeruginosa or Burkholderia species results from viscid mucus in the smaller airways of the lungs.
This leads to damage of the bronchial wall, bronchiectasis and abscess formation.
What are the common signs of cystic fibrosis on examination?
The child has a persistent, ‘wet’ cough, productive of purulent sputum. On examination there is hyperinflation of the chest due
to air trapping, coarse inspiratory
crepitations, and/or expiratory wheeze. With established disease, there is finger clubbing.
What are the abdominal manifestations of cystic fibrosis?
Over 90% of children with CF have pancreatic exocrine insufficiency (lipase, amylase, and proteases), resulting in maldigestion and malabsorption. Untreated, this leads to faltering growth with frequent
large, pale, and greasy stools (steatorrhoea). Pancreatic
insufficiency can be diagnosed by demonstrating low faecal elastase.
About 10–20% of infants with CF present in the neonatal period with meconium ileus, in which inspissated
meconium causes intestinal obstruction. Typically, there is vomiting, abdominal distension, and failure to pass meconium in the first few days of life. Surgery is usually required, but gastrografin enema may relieve the obstruction.
How is cystic fibrosis diagnosed?
The essential diagnostic procedure is the sweat test, to confirm that the concentration of chloride in sweat is markedly elevated (Cl 60–125 mmol/L in CF, 10–40 mmol/L in normal children).
Sweating is stimulated by applying a low-voltage current to pilocarpine
applied to the skin. The sweat is collected into a special capillary tube or absorbed onto a weighed piece of
filter paper. Diagnostic errors are common if there is an inadequate volume of sweat collected.
Confirmation of diagnosis can be made by testing for gene abnormalities in the CFTR protein.
What is the respiratory management of cystic fibrosis?
Recurrent and persistent bacterial chest infection is the major problem. In younger children, respiratory
monitoring is based on symptoms; older children should have their lung function measured regularly by spirometry. The FEV1 expressed as a percentage predicted for age, sex, and height, is an indicator of
clinical severity and declines with disease progression.
- Physiotherapy:
From diagnosis, children should have physiotherapy at least twice a day, aiming to clear the airways of secretions. In younger children, parents are taught to perform airway clearance at home using chest percussion and postural drainage.
Older patients perform controlled deep breathing exercises and use a variety of physiotherapy devices
for airway clearance. Physical exercise is beneficial and is encouraged. - Antibiotics
Many CF specialists recommend continuous prophylactic oral antibiotics (usually flucloxacillin), with
additional rescue oral antibiotics for any increase in respiratory symptoms or decline in lung function. Persisting symptoms or signs require prompt and vigorous intravenous therapy to limit lung damage, usually administered for 14 days via a PIC (peripherally inserted central) line. Increasingly, parents are taught to
administer courses of intravenous antibiotics at home, to decrease disruption of school and other activities.
Chronic Pseudomonas infection is associated with a more rapid decline in lung function, which is slowed by
the use of daily nebulized antipseudomonal antibiotics (penicillins, cephalosporins, aminoglycosides, carbapenems).
The macrolide antibiotic azithromycin, given regularly, decreases respiratory exacerbations.
If venous access becomes troublesome, implantation of a central venous catheter with
a subcutaneous access port (e.g. Portacath) simplifies venous access, although they require monthly flushing and complications may develop.
- Nebulised saline (and DNase)
Nebulized DNase or hypertonic saline may be helpful to decrease the viscosity of sputum and to increase its
clearance. Regular, nebulized hypertonic saline may decrease the number of respiratory exacerbations. - Bilateral sequential lung transplantation is the only
therapeutic option for end-stage CF lung disease. Outcomes following lung transplantation continue to
improve, with over 50% survival at 10 years.
What is the nutritional management of cystic fibrosis?
Dietary status should be assessed regularly. Pancreatic insufficiency is treated with oral enteric-coated pancreatic replacement therapy taken with all meals and snacks. Dosage is adjusted according to clinical response.
A high-calorie diet is essential, and dietary intake is recommended at 150% of normal. To achieve
this, overnight feeding via a gastrostomy is increasingly used.
Most patients require fat-soluble vitamin supplements.
What are the complications of cystic fibrosis?
Most children with CF survive into adult life. With increasing age come increased complications,
- Diabetes mellitus: due to decreasing pancreatic endocrine function.
- Liver disease: Up to one-third of adolescent patients will have evidence of liver disease with hepatomegaly on liver palpation, abnormal liver function on blood tests, or an abnormal ultrasound.
Regular ursodeoxycholic acid, to improve flow of bile, may be beneficial. Rarely, the liver disease progresses to cirrhosis, portal hypertension, and ultimately liver
failure. Liver transplant is generally very successful in CF-related liver failure. - Distal intestinal obstruction syndrome (DIOS, meconium ileus equivalent): viscid mucofaeculent
material obstructs the bowel. This is usually cleared by a combination of oral laxative agents. - Increasing chest infections, as well as other late respiratory
complications including pneumothorax and life-threatening haemoptysis. There is increasing concern over transmission of virulent strains of Pseudomonasand Burkholderia cepacia between patients, causing rapid decline in lung function. Consequently, patients
are often segregated and advised not to socialise with other people with CF. - Infertility: Females have normal fertility, and unless they have
severe lung disease, tolerate pregnancy well. Males are virtually always infertile due to absence of the vas deferens, although they can father children through intracytoplasmic sperm injection.
What are the features of primary ciliary dyskinesia and how is it investigated and managed?
In primary ciliary dyskinesia there is congenital abnormality in the structure or function of cilia lining the
respiratory tract. This leads to impaired mucociliary clearance. Affected children have recurrent infection of the upper and lower respiratory tracts, which if
untreated may lead to severe bronchiectasis.
They characteristically have a recurrent productive cough, purulent nasal discharge, and chronic ear infections. Since ciliary action is responsible for normal organ situs,
50% have dextrocardia and situs inversus (Kartagener syndrome, where major organs are in mirror position of normal).
The diagnosis is made by examination of the structure and function of the cilia of nasal epithelial
cells brushed from the nose. The cornerstones of management are daily physiotherapy to clear secretions, proactive treatment of infections with antibiotics, and
appropriate ENT follow-up.
What is sleep-disordered breathing and why does it occur?
During REM (rapid eye movement) sleep, the control of breathing becomes unstable and there is relaxation of voluntary muscles in the upper airway and chest. This makes upper airway collapse more likely.
Sleep-disordered breathing occurs either due to
airway obstruction, central hypoventilation or a combination of these.
What are the key features and causes of sleep-disordered breathing?
Key aspects of the history include loud snoring, witnessed pauses in breathing (apnoeas),
restlessness, and disturbed sleep.
Obstructive sleep apnoea leads to excessive daytime
sleepiness or hyperactivity, learning and behaviour
problems, faltering growth, and in severe cases, pulmonary hypertension. In childhood, it is usually due to:
Upper airway obstruction secondary to adenotonsillar hypertrophy.
Predisposing causes of sleep-disordered
breathing are:
- Neuromuscular disease (e.g. Duchenne
muscular dystrophy)
- Craniofacial abnormalities (e.g. Pierre Robin sequence, achondroplasia)
- Dystonia of upper airway muscles (e.g. cerebral palsy)
- Severe obesity.
- Children with Down syndrome have anatomical upper airway restriction as well as hypotonia and
are particularly at risk.
These high-risk groups should be screened for sleep-disordered breathing.
Congenital central hypoventilation syndrome is
a rare congenital condition resulting in disordered
central control of breathing as well as other autonomic dysfunction. In severe cases, life-threatening hypoventilation occurs during sleep, which may result in death in infancy. Long-term ventilation, either continuous or
only during sleep is the mainstay of treatment.
How is sleep disordered breathing investigated?
The most basic assessment is overnight pulse oximetry, which can be performed in the child’s home. The frequency and severity of periods of desaturation can be quantified. Normal oximetry does not exclude the condition, but means that severe physical consequences are unlikely.
Polysomnography is required in more complex cases. This should include monitoring of heart rate, respiratory effort, airflow, CO2 measurement
and video recording. This provides more information
about gas exchange and can distinguish between central and obstructive events.
Sometimes more detailed electrophysiological assessment is needed to assess neurological arousals and sleep staging.
How is sleep-disordered breathing managed?
In children with adenotonsillar hypertrophy, adenotonsillectomy usually dramatically improves their condition.
Before surgery for obstructive sleep apnoea, overnight oximetry should be performed to identify severe hypoxaemia, which may increase the
risk of perioperative complications.
For children with other sleep-related breathing disorders, nasal or face mask continuous positive pressure (CPAP) or BiPAP (bilevel positive airway pressure) to maintain their upper airway may be required at night.
What are the indications for a tracheostomy in children?
- Narrow upper airways:
- Subglottic stenosis
- Laryngeal anomalies (e.g. atresia, haemangiomas, webs)
- Pierre Robin sequence (small jaw and cleft palate)
- Other craniofacial anomalies (e.g. Crouzon disease) - Lower airway anomalies
- Severe tracheobronchomalacia - Long-term ventilation
- Muscle weakness
- Head or spinal injury - Wean from ventilation
- Any prolonged period of ventilation - Airway protection
- To facilitate clearance of secretions
What is the risk with a tracheostomy?
If a child with a tracheostomy develops sudden and
severe breathing difficulties, it may be that the tracheostomy tube is blocked with secretions and needs urgent suction or needs changing immediately. If this does not relieve the difficulty in breathing, respiratory support is given via the tracheostomy tube.
All children with a tracheostomy should have a spare tracheostomy tube with them at all times, and a carer competent to change it.
What is vomiting and what might it be confused with?
Vomiting is the forceful ejection of gastric contents.
Posseting and regurgitation are terms used to describe the non-forceful return of milk, but differ in degree.
Posseting describes the small amounts of milk that
often accompany the return of swallowed air (wind),
whereas regurgitation describes larger, more frequent losses. Posseting occurs in nearly all babies from time to time, whereas regurgitation may indicate the presence of more significant gastro-oesophageal reflux.
Vomiting is the forceful ejection of gastric content
What are the ‘red flag’ clinical features in the vomiting child and what might they suggest?
- Bile-stained vomit
- Intestinal obstruction - Haematemesis
- Oesophagitis
- Peptic ulceration
- Oral/nasal bleeding
- Oesophageal variceal bleeding - Projectile vomiting, in first few weeks of life
- Pyloric stenosis - Vomiting at the end of paroxysmal coughing
- Whooping cough (pertussis) - Abdominal tenderness/abdominal pain on
movement
- Surgical abdomen - Abdominal distension
- Intestinal obstruction
- Strangulated inguinal hernia - Hepatosplenomegaly
-Chronic liver disease
-Inborn error of metabolism - Blood in the stool
-Intussusception
-Bacterial gastroenteritis - Severe dehydration, Shock
- Severe gastroenteritis
- Systemic infection (urinary tract infection, meningitis)
- Diabetic ketoacidosis - Bulging fontanelle or seizures
- Raised intracranial pressure - Faltering growth
- Gastro-oesophageal reflux disease
- Coeliac disease
- Chronic gastrointestinal conditions
What are the possible causes of vomiting in infants?
- Gastro-oesophageal reflux
- Feeding problems
- Infection:
* Gastroenteritis
* Respiratory tract/otitismedia
* Whooping cough (pertussis)
* Urinary tract
* Meningitis - Food allergy and food intolerance
- Eosinophilic oesophagitis
- Intestinal obstruction:
* Plyoric stenosis
* Atresia – duodenal, other sites
* Intussusception
* Malrotation
* Volvulus
* Duplication cysts
* Strangulated inguinalhernia
* Hirschsprung disease - Inborn errors of metabolism
- Congenital adrenal hyperplasia
- Renal failure
What are the possible causes of vomiting in pre-school children?
- Gastroenteritis
- Infection:
* Respiratory tract/otitis media
* Urinary tract
* Meningitis
* Whooping cough (pertussis) - Appendicitis
- Intestinal obstruction:
* Intussusception
* Malrotation
* Volvulus
* Adhesions
* Foreign body – bezoar - Raised intracranial pressure
- Coeliac disease
- Renal failure
- Inborn errors of metabolism
- Torsion of the testis
What are the possible causes of vomiting in school age children and adolescents?
- Gastroenteritis
- Infection
- Pyelonephritis
- Septicaemia
- Meningitis - Peptic ulceration and H. pylori infection
- Appendicitis
- Migraine
- Raised intracranial pressure
- Coeliac disease
- Renal failure
- Diabetic ketoacidosis
- Alcohol/drug ingestion or medications
- Cyclical vomiting syndrome
- Bulimia/anorexia nervosa
- Pregnancy
- Torsion of the testis
What is Gastro-oesophageal reflux and what are the clinical features?
Gastro-oesophageal reflux is the involuntary passage of gastric contents into the oesophagus. It is extremely common in infancy. It is caused by inappropriate relaxation of the lower oesophageal sphincter as a result of functional immaturity. A predominantly fluid diet, a mainly horizontal posture and a short intraabdominal length of oesophagus all contribute. While
common in the 1st year of life, nearly all symptomatic reflux resolves spontaneously by 12 months of age. This is probably due to a combination of maturation
of the lower oesophageal sphincter, assumption of an upright posture and more solids in the diet.
Most infants with gastro-oesophageal reflux have recurrent regurgitation or vomiting but are putting on weight normally and are otherwise well.
Gastro-oesophageal reflux is usually a benign, self-limited condition but when it becomes a significant problem it becomes gastro-oesophageal reflux disease (GORD) and needs treatment.
When is GORD more common?
Gastro-oesophageal reflux disease is more common in:
* Children with cerebral palsy or other neurodevelopmental disorders
* Preterm infants, especially in those with bronchopulmonary dysplasia
* Following surgery for oesophageal atresia or diaphragmatic hernia.
What are the complications of GORD?
- Faltering growth from severe vomiting
- Oesophagitis – haematemesis, discomfort on feeding or heartburn, iron-deficiency anaemia
- Recurrent pulmonary aspiration – recurrent pneumonia, cough or wheeze, apnoea in preterm infants
- Dystonic neck posturing (Sandifer syndrome)
- Apparent life-threatening events
How is gastro-oesophageal reflux investigated?
Gastro-oesophageal reflux is usually diagnosed clinically and no investigations are required. However, they may be indicated if the history is atypical, complications
are present, or there is failure to respond to treatment.
Investigations include:
* 24-hour oesophageal pH monitoring to quantify the degree of acid reflux
* 24-hour impedance monitoring which is available in some centres. Weakly acidic or nonacid reflux,
which may cause disease, is also measured
* Endoscopy with oesophageal biopsies to identify oesophagitis and exclude other causes of vomiting
Contrast studies of the upper gastrointestinal tract may support the diagnosis but are neither sensitive nor specific. They may be required in gastro-oesophageal
disease to exclude underlying anatomical abnormalities in the oesophagus, stomach, and duodenum, and to identify malrotation.
How is Gastro-oesophageal reflux managed?
Uncomplicated gastro-oesophageal reflux has an excellent prognosis and can be managed by parental
reassurance, adding inert thickening agents to feeds (e.g. Carobel), and smaller, more frequent feeds.
Significant gastro-oesophageal reflux disease is managed with acid suppression with either hydrogen
receptor antagonists (e.g. ranitidine) or proton-pump inhibitors (e.g. omeprazole). These drugs reduce the volume of gastric contents and treat acid-related oesophagitis.
If the child fails to respond to these measures, other diagnoses such as
cow’s milk protein allergy should be considered and further investigations performed.
Surgical management is reserved for children with complications unresponsive to intensive medical treatment or oesophageal stricture. A Nissen fundoplication, in which the fundus of the stomach is wrapped around the intra-abdominal oesophagus, is performed either as an abdominal or as a laparoscopic procedure.
