Paediatrics notes Flashcards
Define stillbirth
Foetus born with no signs of life >/= 24 weeks of pregnancy
Give examples of teratogenic medications
Retinoids
Warfarin
Sodium valproate
Why should eating liver be avoided in pregnancy?
high concentration of vitamin A
Which marker is raised in neural tube defects?
Raised maternal serum alphafetoprotein with spina bifida or anencepahly (but US is now increasingly used)
How to screen for Down syndrome
Risk estimate calculated from age, biochemical markers combined with US screening for nuchal translucency. Aim is to detect >75% with <3% false positive rate. If high risk, chromosome analysis is offered.
In utero treatment of foetal SVT
Digoxin or flecainide (via the mother)
Treatment of rhesus isoimmunisation
In utero foetal blood transfusion directly into umbilical vein
Foetuses at risk can be detected by looking at maternal antibodies
What is perinatal isoimmune thrombocytopenia, and how is it treated?
When anti-platelet antibodies from the mother cross the placenta and cause thrombocytopenia in the foetus.
Can be treated with IVIg.
Effects of pre-eclampsia on pregnancy
May require preterm delivery
Can cause maternal eclampsia or a cerebrovascular accident due to high BP
Associated with placental insufficiency and growth restriction
Placental insufficiency and IUGR
Growth-restricted foetuses require close monitoring
Absence or reversal of blood flow velocity in the umbilical or middle cerebral artery during diastole is associated with increased risk of morbidity from hypoxic damage to the gut or brain, or of intrauterine death
Multiple births are associated with:
Preterm labour (median gestation for twins is 37 weeks)
IUGR
Congenital abnormalities
Twin-twin transfusion syndromes in monochorionic twins
Complicated deliveries
Pregnancy complications associated with poorly controlled maternal diabetes
Polyhydramnios Pre-eclampsia Increased rate of foetal loss Congenital malformations Late unexplained intrauterine death
Women with insulin-dependent diabetes find it harder to maintain good glycaemic control during pregnancy and have higher insulin requirementa
Foetal problems associated with maternal diabetes
Congenital malformations
IUGR
Macrosomia (Maternal hyperglycaemia causes foetal hyperglycaemia. Insulin does not cross the placenta, so the foetus produced its own insulin, promoting growth) –> associated with increased risk of cephalopelvic disproportion, birth asphyxia, shoulder dystocia and brachial plexus injury
Neonatal problems associated with maternal diabetes
Hypoglycaemia (transient due to foetal hyperinsulinaemia)
Respiratory distress syndrome
Hypertrophic cardiomyopathy
Polycythaemia
In what ethnic population is gestational diabetes more common?
Asian and Afro-Caribbean women
Maternal hypothyroidism
In mothers with Graves disease, 1-2% of babies are hyperthyroid, due to circulating TSH
Foetal hyperthyroidism may be noticed by detecting tachycardia on the CTG trace and a foetal goitre may be seen on ultrasound
SLE with anti-phospholipid syndrome is associated with:
Recurrent miscarriage IUGR Pre-eclampsia Placental abruption Preterm delivery
Some infants born to mother with anti-Ro and anti-La antibodies will develop neonatal lupus syndrome (characterised by a self-limiting rash and (rarely) a heart block)
Maternal autoimmune thrombocytopenic purpura
The foetus may become thrombocytopenic because maternal IgG antibodies cross the placenta and damage foetal platelets–> could increase risk of intracranial haemorrhage following birth trauma
infants with severe thrombocytopenia or petechiae at birth should be given IVIg
Clinical features of foetal alcohol syndrome
Growth restriction Characteristics face (saddle-shaped nose, maxillary hypoplasia, absent philtrum between nose and upper lip, short and thin upper lip) Developmental delay Cardiac defects
Risks of cocaine abuse during pregnancy
Placental abruption
Preterm delivery
Cerbral infarction
Risks with therapeutic drugs used during pregnancy
Opioid analgesia- may suppress respiration at birth
Epidrual anaesthesia- may cause maternal pyrexia during labour (which can be difficult to distinguish from a fever due to infective cause)
Sedatives e.g. diazpeam - may cause sedaiton, hypotherma and hypotension in the newborn
Oxytocin and Prostaglandin F2- may cuase hyperstimulation of the uterus leading to foetal hypoxia
IV fluids - may cause neonatal hyponatremia
How is maternal rubella infection confirmed?
Serologically
Triad of rubella infection in the newborn
Cataracts
Deafness
Congenital heart disease (PDA)
Risk and extent of foetal damage depends on gestational age at onset of maternal infection (infection <8 weeks causes cataracts, defaness and congenital heart disease in 80%, 30% foetuses infected at 13-16 weeks have impaired hearing, no consequences after 20 weeks)
Management of rubella in pregnancy
Notify the Health Protection Unit
HPU may also test for Parvovirus B19
There is NO EFFECTIVE TREATMENT for rubella: recommend rest, adequate fluid intake and paracetamol for symptomatic relief
Stay off work and avoid contact with other pregnant women for 6 days after initial development of rash
Once confirmed, refer urgently to obstetrics for risk assessment and counselling
What is the most common congenital infection?
CMV
Effects of CMV infection on neonate
90% are normal at birth and develop normally
5% will have clinical featuers (e.g. hepatosplenomegaly, petechiae) at birth and most of these babies will have neurodevelopmental disabilities, such as sensorineural hearing loss, cerebral palsy, epilepsy and cognitive impairment
5% develop problems later in life mainly sensorineural hearing loss
Effects of CMV infection on mother
Infection of the pregnant woman is usually asymptomatic
Pregnant women are not screened for CMV and there is no vaccine
Management of newborn infants with CMV
IV ganciclovir
OR
Oral valganciclovir
Clinical features of Toxoplasmosis infection in infants
Most infected infants are asymptomatic- at risk of developing chorioretinitis in adulthood
10% will have clinical features: retinopathy (due to acute fundal chorioretinitis), cerebral calcification, hydroecephalus –> these infants usually have long-term neurological disabilities
Management of newborns with toxoplasmosis
1st line: Pyrimethamine + sulfadiazine + calcium folinate
Adjunct: prednisolone
What is the risk of varicella zoster infection in the mother to the infant?
If the mother develops chicken pox in the first half of pregnancy (<20 weeks), there is a <2% risk of the foetus developing severe scarring of the skin and also ocular and neurological damage and digital dysplasia
If the mother develops chicken pox within 5 days before or 2 days after delivery, when the foetus is unprotected by maternal antibodies and viral dose is high, about 25% will develop a vesicular rash and mortality can be as high as 30%
Management of maternal chicken pox infection
Exposed susceptible mothers can be protected with VZIG and treated with aciclovir
Clinical features of congenital syphilis
Characteristic rash on soles of feet and hand and bone lesions
What changes occur intra-extrauterine?
Before birth, the blood vessels to and from the lungs are constricted, so most of the blood from the right side of the heart will pass through the ductus arteriosus into the aorta, and some will flow across the foramen ovale
Shortly before and during labour, lung liquid production is reduced
During descent through the birth canal, the infant’s chest is squeezed and some lung fluid is drained
Several thermal, tactile and hormonal stimuli (mainly an increase in catecholamine) to initiate breathing
The first breath takes place an average of 6 seconds after delivery
Lung expansion occurs due to intrathoracic negative pressure
Regular breathing is usually established after 30 seconds
After a the infant gasps, the rest of the lung fluid is absorbed into the lymphatic and pulmonary circulation
Pulmonary expansion leads to reduced pulmonary resistance and an increase in pulmonary blood flow
The flow of oxygenated blood through the ductus arteriosus leads to closure of the duct
IMPORTANT: in babies born via an elective Caesarean section, their chest would not have been squeezed so it may take longer for the fluid to be drained from the lungs
Primary apnoea
Some infants will not breathe at birth - this may be due to asphyxia (lack of oxygen during labour or delivery)
If a foetus is deprived of oxygen in utero, the foetus will attempt to breathe
BUT, as they are still in utero, this attempt will be unsuccessful - this is called primary apnoea
During the primary apnoea, heart rate is maintained
If oxygen deprivation continues, primary apnoea is followed by irregular gasping and then a second period of apnoea (secondary or terminal apnoea)
During terminal apnoea, the heart rate and blood pressure will fall
If the infant is delivered after the terminal apnoea, they will need help with lung expansion (e.g. positive pressure ventilation or tracheal tube)
The foetus rarely experiences continuous asphyxia except in the case of placental abruption or complete occlusion of umbilical blood flow in a cord prolapse
More commonly, asphyxia during labour and delivery is intermittent (e.g. due to frequent uterine contractions)
Define asymmetrical growth restriction
Weight or abdominal circumference lies on a lower centile than that of the head
When does asymmetrical growth restriction occur?
When the placenta fails to provide adequate nutrition late in the pregnancy but brain growth is relatively spared at the expense of liver glycogen and skin fat
What are the associated disorders with asymmetrical growth restriction?
Utero-placental dysfunction secondary to maternal pre-eclampsia, multiple pregnancy and maternal smoking
These infants tend to put on weight rapidly after birth
Define symmetrical growth restriction
Head circumference is equally reduced
What is suggested by symmetrical growth restriction?
Prolonged period of poor intrauterine growth starting in early pregnancy
Causes of symmetrical growth restriction
Usually due to a small but normal foetus
Could be due to a foetal chromosomal disorder, congenital infection, maternal drug/alcohol abuse or a chronic medical condition
These infants are more likely to be permanently small
What does intrauterine growth restriction predispose infants to?
Intrauterine hypoxia and unexplained intrauterine death
Asphyxia during labour and delivery
After birth, growth restricted babies are liable to:
Hypothermia (Large surface area)
Hypoglycaemia (low fat and glycogen stores)
Hypocalcaemia
Polycythaemia
Potential consequences of large for gestational age babies
> 90th centile for weight for their age
Birth asphyxia from a difficult delivery
Breathing difficulty from an enlarged tongue in Beckwith-Wiedemann syndrome (overgrowth syndrome)
Hypoglycaemia (due to hyperinsulinism)
Polycythaemia
Why are babies given vitamin K at birth?
Prevent haemorrhagic disease of the newborn
Risk factors for DDH
Breech presentation
6x more common in females
Why is early recognition of DDH important?
Splinting in abduction reduced long-term morbidity
Management of DDH
For infants <2 months with normal physical examination without instabililty, observation is recommended with serial examinations and US on monthly basis
Hip abduction orthosis (splint) in Pavlik harness if the dysplasia persists or worsens (serial follow up and plain X-ray evaluation is recommended after 6 months of age)
Breech delivery: if no DDH at neonatal examination, arrange ultrasound scan at 6 weeks
What is the major risk in haemorrhagic disease of the newborn?
Some may suffer from intracranial haemorrhage which could leave them permanently disabled
Why are infants of mothers taking anticonvulsants at increased risk of haemorrhagic disease?
Anticonvulsants impair synthesis of vitamin-K dependent clotting factors
What does the guthrie test screen for?
PKU
Hypothryoidism
Haemoglobinopathies (sickle cell and thalassemia)
CF (measuring serum immunoreactive trypsin, which is raised if there is pancreatic duct obstruction –> DNA analysis also performed to reduced the false-positive rate)
MCAD deficiency
How is hearing screened in newborns?
Evoked Otoacoustic emission: earphone palced over the ear and a sound is emitted which evoked an echo or emission from the ear if cochlear function is normal. IF a normal result is not achieved, move on to:
Automated Auditory Brainstem Response Audiometry: A computer will analyse the EEG waveforms evoked in response to a series of clicks
Hypoxic-ischaemic encephalopathy
In perinatal asphyxia, gas exchange, either placental or pulmonary, is compromised leading to cardiorespiratory depression. This leads to hypoxia, hypercapnia and metabolic acidosis. Reduced cardiac output leads to hypoxic-ischaemic injury to the brain and other organs.
Significant hypoxic events which may lead to HIE in neonates?
Failure of gas exchange across placenta (excessive or prolonged uterine contractions, placental abruption, ruptures uterus)
Interruption of umbilical blood flow (e.g. cord compression including shoulder dystocia, cord prolapse)
Inadequate maternal placental perfusion
Compromised foetus (anaemia, IUGR)
Failure of cardiorespiratory adaptation at birth (failure to breathe)
Clinical features of HIE
Clinical manifestations of of HIE start up to 48 hours after asphyxia and can be graded:
MILD= infant is irritable and responds excessively to stimulation, may have staring eyes, hyperventilation and impaired feeding
MODERATE= infant shows marked abnormalities of tone and movement, cannot feed and may have seizures
SEVERE= No spontaneous movements or response to pain, tone in limbs may fluctuate between hypotonia to hypertonia, seizures are often prolonged and refractor to treatment, multi-organ failure present
NB: the neuronal injury in HIE may be immediate from primary neuronal death or may be delayed from reperfusion injury causing secondary neuronal death
Prognosis of HIE
With mild HIE, full recovery can be expected
Even infants with moderate HIE, who have recovered fully on neurological examination and are feeding normally by 2 weeks of age have an excellent long-term prognosis
Sever HIE has a mortality of 30-40%. Of the survivors, 80% have neurodevelopmental disabilities, particularly cerebral palsy.
If MRI shows significant abnormalities at 4-14 days, there is a very high risk of later cerebral palsy
Although HIE usually occurs antenatally or during delivery/labour, it can occur postnatally or be caused by a neonatal condition (e.g. kernicterus)
What is the difference between a caput succadaneum and cephalhaematoma (both types of extracranial haemorrhage)?
Both are potential injuries sustained during birth
Caput Succedaneum - bruising and oedema of the presenting part extending beyond the margins of the skull bones (resolves within a few days)
Cephalhaematoma - haematoma from bleeding below the periosteum, confined within the margins of the skull sutures. Usually involves the parietal bone (resolves over several weeks)
What is a chignon?
Oedema and bruising from Ventouse delivery
What potential injuries may be sustained during birth?
Extracranial haemorrhage Chignon Bruising Abrasion Forceps marks Subaponeurotic haemorrhage Nerve palsies (brachial nerve palsies result from traction to the brachial plexus nerve roots. This can occur during breech delivery or shoulder dystocia. Upper nerve root (C5+C6) injury results in Erb's palsy- can be accompanied by a phrenic nerve palsy which results in an elevated diaphragm. facial nerve palsies may occur from compression of the facial nerve against the mother's spine. Most palsies will resolve spontaneously) Fractures
Fractures during birth
Clavicle= usually from shoulder dystocia. A snap may be heard during delivery and the infant may show reduced arm movement on the affected side. A lump may be noticed due to callous formation. Prognosis is excellent- no specific treatment needed.
Humerus/Femur= Humerus fractures tend to occur with shoulder dystocia. Femoral fractures more likely with breech deliveries. Infants may show some deformity, reduced movement of the limb and pain on movement. Fractures heal rapidly with immobilisation.
What is respiratory distress syndrome also known as?
Hyaline membrane disease
What causes respiratory distress syndrome?
Surfactant deficiency–> widespread alveolar collapse and inadequate gas exchange
Risk factors for respiratory distress syndrome
Preterm baby
Maternal diabetes
Treatment for respiratory distress syndrome
Glucocorticoids given antenatally to mother if preterm delivery is anticipated
In babies born with RDS, artifical surfactant can be instilled into the lungs directly or with a tracheal tube
Clinical signs of respiratory distress syndrome
Seen within 4 hours of birth
Tachypnoea (>60breaths/minute)
Laboured breathing with chest wall recession (particularly sternal and subcostal indrawing) and nasal flaring
Expiratory grunting (attempting to create positive airway pressure during expiration and maintain functional residual capacity)
Cyanosis if severe
Diffuse granular or ‘ground glass’ appearance of lungs and air bronchogram on CXR. Indistinct heart border/obscured heart border with severe disease.
In RDS, air from overly distended alveoli may track into the interstitium, resulting in pulmonary interstitial emphysema (PIE)
Management (NICE guidelines) of respiratory distress syndrome
Oxygen and ventilation
CPAP or artificial ventilation via a tracheal tube may be necessary
Other options: mechanical ventilation, high-flow humidified oxygen therapy
What is a major risk of ventilation therapy for RDS?
In about 10% of infants ventilated for RDS, air will leak into the pleural cavity and cause a pneumothorax
Clinical signs of neonatal pneumothorax
Increased oxygen demands
Breath sounds and chest expansion reduced on affected side
A pneumothorax can be demonstrated by transillumination
Management of ventilation-induced pneumothorax
Immediate decompression + oxygen therapy + chest drain if tension pneumothorax
To prevent pneumothoraces, infants should be ventilated with the lowest pressures that provide adequate chest movement and blood gases
What can cause pneumothoraces in term infants?
Can occur spontaneously or be secondary to:
Meconium aspiration
Respiratory distress syndrome
Complication of ventilation
Apnoea–> bradycardia –> desaturation
Very common in very low birthweight infants until 32 weeks’ gestation. Can occur when an infant stops breathing over 20-30 seconds or when breathing continues against a closed glottis.
Tend sot be caused by immaturity or central respiratory control and breathing will usually start again after gentle physical stimulation
Caffeine may also help stimulate breathing
CPAP may be needed if apnoeic episodes are frequent
Sinister underlying causes of apnoea in infants
Hypoglycaemia Infection Anaemia Hypoxia Electrolyte disturbances
What can hypothermia cause in the infant?
Increased energy consumption –> hypoxia, hypoglycaemia –> failure to gain weight and increased mortality
Why are preterm infants particularly susceptible to hypothermia?
Large SA:volume ratio
Thin skin that is heat permeable (transepidermal water loss)
Little subcutaneous fat for insulation
Often nursed naked and cannot conserve heat by curling up or generate heat by shivering
Preterm babies are kept in incubators to closely control the temperature and humidity
In which population of infants is shunting of blood across ductus arteriosus from left to right side of heart more common in?
Infants with respiratory distress syndrome
Clinical features of patent ductus arteriosus
Can be asymptomatic
Can cause apnoea and bradycardia
Increased oxygen requirement
Difficulty in weaning infant from artificial ventilation
Bounding pulse (due to increased pulse pressure)
Prominent precordial impulse
Systolic murmur
Echocardiography can be used to assess infant’s circulation
Management of patent ductus arteriosus?
Duct can be closed using:
IV indomethacin
Prostacyclin synthetase inhibitor
Ibuprofen
If pharmacological methods are unsuccessful, surgical ligation or percutaneous catheter device closure may be used
At what gestational age are infants mature enough to suck and swallow milk?
35-36 weeks
Less mature infants will need feeding via an orogastric or nasogastric tube
What needs to be supplemented into the diets of very preterm infants?
Phosphate
May need supplementation of protein, calories and calcium
How is parenteral nutrition administered for very immature or sick infants?
Central venous catheter (e.g. PICC line)
NB: PICC lines carry a significant risk of septicaemia (and other risks such as thrombosis)
NB: cows’ milk formula increases the risk of necrotising enterocolitis
Why do preterm babies have low iron stores/are at risk of iron deficiency?
Iron is usually transferred to the foetus during the last trimester
Iron supplements are usually started at several weeks of age
Why are preterm infants at increased infection risks?
IgG is mainly transferred across the placenta in the last trimester
Additionally, infection in and around the cervix is often a reason for preterm labour
Most infections in preterm infants occur after several days and are nosocomial (e.g. indwelling catheters, artificial ventilation)
How can haemorrhages in the brain of infants be visualised?
Ultrasound scans
Where do brain haemorrhages occur in preterm babies?
Usually occur in the germinal matrix above the caudate nucleus (contains a fragile network of blood vessels)
Risk factors for brain haemorrhages in preterm infants
More common following perinatal asphyxia and in infants with severe RDS
Pneumothorax is a major risk factor for brain haemorrhage
What is the most severe type of brain haemorrhage in a preterm infant?
The MOST SEVERE type of haemorrhage is a unilateral haemorrhagic infarction involving the parenchyma of the brain - usually resulting in hemiplegia
A large intraventricular haemorrhage may impair the drainage and reabsorption of CSF, leading to an accumulation of CSF
This may resolve spontaneously or may progress to hydrocephalus
Hydrocephalus may lead to separation of cranial sutures, a rapid increase in head circumference and the anterior fontanelle to become tense
A ventriculoperitoneal shunt may be required, but symptomatic relief by relieving CSF via a lumbar puncture or ventricular tap may be sufficient
Around 50% of infants with progressive post-haemorrhagic ventricular dilatation have cerebral palsy
Periventricular white matter brain injury may occur following infarction or inflammation and may occur in the absence of haemorrhage
This may resolve spontaneously
However, if cystic lesions become visible on ultrasound 2-4 weeks later, there is definite loss of white matter
The presence of multiple bilateral cysts is called periventricular leukomalacia (PVL) - it is associated with an 80-90% risk of spastic diplegia
Definition of Spastic Diplegia: a form of cerebral palsy that manifests as especially high tightness or stiffness in the muscles of the lower extremities
NOTE: PVL and intraventricular haemorrhage could both occur in the absence of clinical signs
Necrotising enterocolitis associations
Associated with bacterial infection of ischaemic bowel wall
Preterm infants fed with cows’ milk formula are more likely to develop this condition
Clinical features of necrotising enterocolitis
Infant stops tolerating feeds Milk is aspirated from the stomach Vomiting (may be bile-stained) Abdominal distension Rectal bleeding (sometimes)
The infant may develop shock and require artificial ventilation
Characteristic X-ray features:
Distended loops of bowel
Thickening of the bowel wall with intramural gas
Gas in the portal tract
The disease may progress to bowel perforation, which can be detected by X-ray or transillumination of the abdomen
Management of necrotising enterocolitis
Stop oral feeding
Broad-spectrum antibiotics to cover both aerobic and anaerobic organisms
Surgery if bowel perforation/necrosis
Parenteral nutrition is always needed and artificial ventilation and circulatory support are often needed
Consequences of necrotising enterocolitis
Long-term consequences include the development of strictures and malabsorption if extensive bowel resection is necessary
What is retinopathy of prematurity?
Affects developing blood vessels at the junction of the vascular and non-vascularised retina, usually due to uncontrolled use of high concentrations of oxygen
What are the consequences of retinopathy of prematurity?
May progress to retinal detachment, fibrosis and blindness
What is bronchopulmonary dysplasia?
Infants who still have oxygen requirement at a post-menstrual age of 36 weeks are described as having bronchopulmonary dysplasia (or chronic lung disease)
Lung damage occurs as a result of artificial ventilation, oxygen toxicity and infection
CXR may show widespread areas of opacification, sometimes with cystic changes
Subsequent pertussis or RSV infection could lead to respiratory failure
Management of bronchopulmonary dysplasia
Some infants will need prolonged ventilation, but most will be weaned onto CPAP and then additional ambient oxygen
Corticosteroids may facilitate earlier weaning (although there are concerns about abnormal neurodevelopment)
Problems after discharge in preterm infants
Additional iron supplementation needs to be given until 6 months corrected age
Increased risk of: Poor growth Pneumonia/wehezing/asthma Bronchiolitis from RSV infection Bronchopulmonary dysplasia Gastro-oesophageal reflux Complex nutritional and GI disorders (e.g. following necrotising enterocolitis) Inguinal hernias
About 5-10% of very low birthweight babies develop cerebral palsy, but the most common impairments are learning difficulties
Why are neonates predisposed to developing jaundice?
Over 50% of newborns become visibly jaundiced, due to:
Marked physiological release of haemoglobin from the breakdown of red blood cells because of the high Hb concentration at birth
Red cell life span of newborn infants (70 days) is considerably shorter than that of adults (120 days)
Hepatic bilirubin metabolism is less efficient in the first few days of life
Why is it important to recognise neonatal jaundice?
May be a sign of another disease (e.g. haemolytic anaemia)
Unconjugated bilirubin can get deposited in the brain, particularly in the basal ganglia, causing kernicterus
Define kernicterus
Encephalopathy resulting from the deposition of unconjugated bilirubin in the basal ganglia and brainstem nuclei
It may occur when the level of unconjugated bilirubin exceeds the albumin-binding capacity of the blood- free bilirubin is fat-soluble, so it can cross the blood-brain barrier.
Acute clinical manifestations of kernicterus
Lethargy
Poor feeding
In severe cases: Irritability Increased muscle tone causing the baby to lie with an arched back (opisthotonos) Seizures Coma
Potential consequences of kernicterus
Infants who survive may develop choreoathetoid cerebral palsy (due to damage to the basal ganglia), learning difficulties and sensorineural deafness
At what level of bilirubin do babies become clinically jaundiced?
Around 80mcM/L
What is the likely cause of jaundice <24 hours of age?
Usually due to haemolysis:
Rhesus haemolytic disease: affected infants are usually identified antenatally. Severely affected infants will have anaemia, hydrops and hepatosplenomegaly with rapidly developing jaundice
ABO incompatibility: Most ABO antibodies are IgM and do not cross the placenta. However, some O group women have an IgG anti-A-haemolysin in the blood, which can cross the placenta and haemolyse the red cells of a group A infant. Jaundice doesnt tend to be as bad as with rhesus disease and hepatosplenomegaly is absent. Coombs test positive.
G6PD deficiency: Parents of affected infants should be given a list of drugs that they should avoid that may precipitate haemolysis
Spherocytosis
Congenital infection
What are the likely causes of jaundice between 2 days - 2 weeks of age?
Physiological jaundice
Breastmilk jaundice: Unconjugated
Dehydration: Poor milk intake. In some infants, IV fluids may be needed.
Infection: a baby with an infection may develop unconjugated hyperbilirubinaemia from poor fluid intake, haemolysis, reduced hepatic function and an increase in enterohepatic circulaiton.
Bruising and polycythaemia can exacerbate jaundice
Criggler-Najjar syndrome (deficient or absent UGT)
What are the likely causes of jaundiced at >2 weeks age?
Biliary atresia
Neonatal hepatitis syndrome
However, most cases are unconjugated:
breast milk jaundice is most common
Infection
Congenital hypothydroidism
Where does jaundiced usually begin on the body?
Tends to start on the head and face and spread down the trunk and limbs
When should jaundice be checked for in a neonate?
It is recommended that in the UK all babies should be checked clinically for jaundice in the first 72 hours of life. If clinically jaundiced, a transcutaneous measurement should be made.
Why can serial measurements of bilirubin be taken in an infant?
The rate at which bilirubin rises tends to be linear until it plateaus, so serial measurements can be plotted on a chart and used to anticipate the need for treatment
Drugs that displace bilirubin from albumin (e.g. sulphonamides and diazepam) should be avoided in newborn infants
Assessment of neonatal jaundice
Visually inspect baby in natural light Measure bilirubin (use serum bilirubin if jaundice developed in the first 24 hours of life or if the gestational age if <35 weeks. Use transcutaneous bilirubinometer if >35 weeks or with jaundice that develops after first 24 hours- if result >250micromol/L, measure serum bilirubin)
Assess risk of developing kernicterus: increased risk if serum bilirubin >340 in babies >37 weeks gestation, rapidly rising bilirubin >8.5 micromol/L per hour, clinical features of acute bilirubin encephalopathy
Serum bilirubin should be measured every 6 hours until it drops below the treatment threshold or becomes stable/falling
Investigations for neonatal jaundice
Measure haematocrit
Blood group of mother and baby
Coombs- if mother is Rh -ve, find out whether mother received prophylactic anti-D immunoglobulin during pregnancy
Consider:
FBC and blood film (e.g. looking for hereditary spherocytosis)
Blood G6PD levels (consider ethnic origins)
Microbiological cultures of blood, urine and/or CSF (if suspected infection)
Treatment of neonatal jaundice
Phototherapy 1st line (light from blue-green band of visible spectrum converts conjugated bilirubin into harmless water-soluble pigment that is excreted in the urine) –> monitor baby’s temperature during phototherapy and protect the baby’s eyes. Phototherapy can be stopped once serum bilirubin is >50micromol/L below threshold for treatment. Check for rebound hyperbilirubinaemia by measuring serum bilirubin 12-18 hours after stopping phototherapy
Exchange transfusion
IVIG
What is the most common cause of respiratory distress in term infants?
Transient tachypnoea of the newborn, caused by delay in resorption of lung liquid
Usually settles within the first day of life
More common in birth by C-section
In what percentage of babies is meconium passed before birth?
8-20%
Mechanism of action of meconium aspiration
Meconium may be passed in response to foetal hypoxia- asphyxiated infants may start gasping before delivery and aspirate the meconium.
Meconium is a lung irritant and will cause both mechanical obstruction and chemical pneumonitis, and it predisposes to infection
Clinical features of meconium aspiration
Lungs become overinflated and have patches of collapse and consolidation.
There is a high incidence of air leak, leading to pneumothorax and pneumomediastinum
Artificial ventilation is often needed
Infants may develop persistent pulmonary hypertension
Management of meconium aspiration
If normal term infant with meconium-stained amniotic fluid but no history of GBS, observation is recommended
If there are risk factors or laboratory findings that are suggestive of infection, consider antibiotics (IV ampicillin and gentamicin)
Oxygen therapy and non-invasive ventilation (e.g. (CPAP) may be used in more severe cases)
Risk factors for pneumonia in infants
Prolonged rupture of membranes
Chorioamnionitis
Low birthweight
Treatment of pneumonia in infants
Broad-spectrum antibiotics are started early until the results of the infection screen are available
NB: milk aspiration can also cause respiratory symptoms in the newborn
Associations of persistent pulmonary hypertension of the newborn
Birth asphyxia
Meconium aspiration
Septicaemia
RDS
Can occur as a primary disorder
LIFE THREATENING
Clinical features of persistent pulmonary hypertension of the newborn
High pulmonary vascular resistance leads to right-to-left shunting of blood
Cyanosis occurs soon after birth
CXR will show a normal sized heart but there may be some pulmonary oligaemia
What investigation is required in persistent pulmonary hypertension of the newborn, and why?
Urgent echocardiogram to make sure that the infant does not have a congenital cardiac defect
Management of persistent pulmonary hypertension of the newborn
Most infants will require mechanical ventilation and circulatory support
Inhaled nitric oxide is a potent vasodilator and can be beneficial
Sildenafil has also been introduced recently
High-frequency (oscillatory) ventilation may be useful
ECMO (heart and lung bypass for several days) may be used for severe but reversible causes
How are diaphragmatic hernias detected in infants?
Many are diagnosed during antenatal ultrasound screening
Diagnosis is confirmed by X-ray
Presentation of diaphragmatic hernia
Usually presents with failure to respond to resuscitation or respiratory distress
In most cases, there is a left-sided herniation of abdominal contents through the posterolateral foramen of the diaphragm- this will cause the apex beat and heart sounds to be displaced to the right, with poor air entry in the left
Management of congenital diaphragmatic hernia
Once diagnosed, a large NG tube is passed and suction applied to prevent distension of the intrathoracic bowel. Once stabilised, the hernia will be surgically repaired
Potential consequences of diaphragmatic hernias
In most infants, the main consequence is pulmonary hypoplasia- compression by herniated loops of bowel throughout pregnancy prevents lung development in foetus
Hypoplastic lungs are associated with high mortality
Other causes of respiratory distress
Heart failure (Femoral arteries must be palpated in all infants with respiratory distress, as coarctation of the aorta and interrupted aortic arch are important causes of heart failure in newborn infants)
Causes of early onset bacterial infection in infants (<48 hours)
Bacteria will have either ascended from the birth canal or invaded the amniotic fluid
The foetus becomes infected because the foetal lungs are in direct contact with the infected amniotic fluid
These infants will develop pneumonia and secondary septicaemia/bacteraemia
On the other hand, with congenital viral infections and early-onset infection with Listeria moncytogenes, foetal infection is acquired via the placenta following maternal infection
Risk factors for early-onset infection in infants
Prolonged or premature rupture of membranes when chorioamnionitis is clinically evident (e.g. mother having fever during delivery)
Clinical features of early-onset infection in the newborn
Respiratory distress Apnoea Temperature instability Vomiting Poor feeding Abdominal distension Jaundice Neutropenia Shock Irritability Seizures
Investigations for early-onset infection of the newborn
CXR
Septic screen
FBC to check for neutropenia
Blood cultures
NB: Acute phase proteins can take 12-24 hours to rise, so a single normal result doesnt rule out infection
Management of early-onset infection of the newborn
Antibiotics are started immediately without waiting for culture results
IV antibiotics should cover:
Group B Strep
Listeria monocytogenes
Other Gram +ve organisms (usually with benzylpenicillin or amoxicillin)
Gram -ve organisms (usually with an aminoglycoside such as gentamicin)
If CRPs are negative and the infants appears to have recovered initially, antibiotics can be stopped after 48 hours
If the blood culture is positive or there are any neurological signs, CSF must be examined and cultured
Source of late-onset infection of the newborn
Often the infant’s environment
Common in NICU, due to sources of infection such as indwelling catheters, invasive procedures and tracheal tubes
Most common pathogen is coagulase negative staphylococcus (Staph epidermidis)
Suitable regiments for late-onset infection of the newborn
Ampicillin + gentamicin/cefotaxime
Flucloxacillin + gentamicin
If resistant, specific antibiotics (e.g. vancomycin for coagulase-negative staphylococci or enterococci) or broad-spectrum antibiotics (e.g. meropenem) may be needed
NB: The use of prolonged or broad-spectrum antibiotics predisposes to invasive fungal infections (e.g. candidiasis)
Investigations for late-onset infection of the newborn
Serial measurements of CRP to measure response to therapy
Signs of neonatal meningitis
Late signs:
Bulging fontanelle
Hyperextension of the neck and back (opisthotonos)
Treatment of neonatal meningitis
Ampicillin or penicillin and a 3rd generation cephalosporin (eg. cefotaxime)
Complications of neonatal meningitis
Cerebral abscess
Management of sepsis in children
1) Supplemental oxygen
2) Gain IV or IO access and order blood cultures, blood glucose and arterial/venous/capillary gases
3) IV/IO broad-spectrum antibiotics
4) IV fluids (be cautious of fluid overload)
5) Experienced senior clinicians should be involved early
6) Vasoactive inotropic support (e.g. adrenaline) should be considered early- considered if normal parameters are not achieved after >40ml/kg of fluid resus
How will a baby with early-onset sepsis caused by group B streptococcus present?
Respiratory distress
Pneumonia
May also cause septicaemia and meningitis
Risk factors for Group B strep infection in colonised mothers
Preterm labour Prolonged rupture of membranes Maternal fever during labour Maternal chorioamnionitis Previously infected infant
Prophylactic intrapartum antibiotics given IV can prevent this infection
Management of group B strep infection in neonates
Targeted antibiotic therapy (Benzypenicillin or ampicilolin + gentamicin or cefotaxime or ceftriaxone)
Supportive therapy
How is Listeria monocytogenes transmitted?
Transmitted to the mother through food (e.g. unpasteurised milk, soft cheeses, undercooked poultry)
How does LIsteria infection present?
mild influenza-like illness in mother
Can pass to the foetus throuhg the placenta
Meconium staining of the amniotic fluid (unusual in preterm infants) Widespread rash Septicaemia Pneumonia Meningitis
Consequences of maternal infection with Listeria
Spontaneous abortion
Preterm delivery
Foetal/neonatal sepsis
Management of Listeria infection
Amoxicillin or co-trimoxazole (trimethoprim is contraindicated in pregnancy)
If systemic infection: IV ampicillin or benzylpenicillin with gentamicin
Treatment for neonatal conjunctivitis
Cleaning with saline and water is all that is required- will resolve spontaneously
Discharge and redness is suggestive of staph or strep infection and can be treated with a topical ointment (e.g. neomycin)
Gonococcal infection may cause purulent discharge, conjunctival infection and swelling of the eyelids. Discharge should be Gram-stained and cultured. Treatment (usually a 3rd generation cephalosporin) should be started immediately because loss of vision could occur
Chlamydia infection presents with a purulent discharge and swelling of the eyelids. Treated with oral erythromycin for 2 weeks.
Bacterial conjunctivitis treated with opthalmic azithromycin OR erythromycin OR polymyxin
Treatment for umbilical infection
Systemic antibiotics if skin around umbilicus becomes inflamed
Sometimes, the umbilicus can be prevented from involuting bi an umbilical granuloma, which can be removed by applying silver nitrate or by applying a ligature around the stump
How is herpes simplex transmitted from mother to infant?
During passage through an infected birth canal or occasionally by ascending infection
Often the mother does not know that she is infected
More common in preterm infants
Presentation of neonatal herpes simplex infection
Infants present up to 4 weeks of age with:
Localised herpetic lesions on the skin or eye
Or encephalitis
Or disseminated disease
Management of neonatal herpes simplex infection
If the mother is identified as having primary disease or delivery genital herpetic lesions at the time of devliery, C-section is indicated
Aciclovir or valaciclovir can be given prophylactically to the baby during the at-risk period
Hepatitis B
Infants of mothers who are HbsAg positive should receive the hepatitis V vaccination shortly after birth to prevent vertical infection
Babies are at highest risk of becoming chronic carriers when their mothers are e-antigen positive but have no e-antibodies
Management of hepatitis B infection in neonates
Mothers should receive antiviral monotherapy (Tenofovir disoproxil OR lamivudine)
Babies at risk should receive passive immunisation with hepatitis B immunoglobulin AND hepatitis B vaccine
Risk factors for hypoglycaemia in the newborn
Particularly likely within the first 24 hours of babies with: IUGR Preterm Born to mothers with diabetes (infants undergo hyperplasia of the islet cells in the pancreas causing high insulin levels) Large-for-dates Hypothermia Polycythaemia Ill
Hypoglycaemia symptoms in infants
Jitteriness Irritability Apnoea Lethargy Drowsiness Seizures
During the first 24 hours, many asymptomatic babies will have blood glucose below 2.6mmol/L (optimal level for desirable neurodevelopmental outcomes)
Prolonged, symptomatic hypoglycaemia can cause permanent neurological disability
Management of hypoglycaemia in infants
Can be prevented with early and frequent milk feeding
Glucose can be given IV in refractory or severe hypoglycaemia
Glucagon and hydrocortisone can also be given
Neonatal seizures
Seizures are unstimulated and usually appear as repetitive, rhythmic movement of the limbs which persist despite restraint and are often accompanied by eye movements or changes in respiration
What investigations are used to confirm and monitor neonatla seizures?
EEGs
Causes of seizures in neonataes
HIE Cerebral infarction Septicaemia/meningitis Hypoglycaemia Hypo/hypernatraemia Hypocalcaemia Hypomagnesaemia Inborn errors of metabolism Pyridoxine dependency
Whenever seizures are observed, hypoglycaemia and meningitis need to be excluded or treated urgently
Treatment for neonatal seizures
A cerebral ultrasound can be performed to identify haemorrhage or cerebral malformations
Ongoing or repeated seizures will be treated with anticonvulsants
Which artery is usually affected in cerebral infarction in the newborn?
Infarction in the territory of the middle cerebral artery
Cerebral infarction presentation
May present with seizures
Seizures can be focal or generalised
There are no other abnormal clinical features (unlike HIE)
What investigation can help confirm the diagnosis of neonatal stroke?
MRI
Pathophysiology of cerebral infarction of the newborn
Thrombotic (e.g. thromboembolism from placental vessles or inherited thrombophilia)
Prognosis is relatively good (only 20% have hemiparesis or epilepsy)
What causes cleft lip/palate?
May be unilateral or bilateral
Cleft lip results from failure of fusion of the frontonasal and maxillary processes
In bilateral cases, the pre-maxilla becomes anteverted
Cleft palate result from failure of fusion of the palatine processes and the nasal septum
These defects can occur as a part of a syndrome (e.g. chromosomal disorders)
They are associated with maternal anticonvulsant therapy
Associations with cleft lip/palate
Infants are prone to acute otitis media
Management of cleft palate/lip
Specialised feeding advice may be necessary
Watch out for airway problems (e.g. Pierre-Robin sequence)
Pre-surgical lip tapping, oral appliances or pre-surgical nasal alveolar moulding (PNAM) may be needed to narrow the cleft
Definitive repair is by surgery
Associations with Pierre-Robin sequence
Associated with micrognathia (small jaw), posterior displacement of the tongue (glossoptosis) and midline cleft of the soft palate
Consequences of Pierre-Robin Sequence
Can lead to difficulty feeding
As the tongue falls back, there is obstruction to the upper airways which may cause cyanotic episodes
Infant is at risk of failure to thrive
Due to the risk of airway obstruction, infants may need to lie on their front
Eventually, as the mandible grows, these problems resolve
Associations with oesophageal atresia
Usually associated with a trans-oesophageal fistula
Associated with polydramnios during pregnancy
Almost half of babies with oesophageal atresia will have other congenital malformations (e.g. VACTERL: verterbral, anorectal, cariac, trachea-oEsophageal, renal and radial limb)
Presentation of oesophageal atresia
If not diagnosed at birth, it typically presents with persistent salivaiton and drooling
The infant will cough and choke when fed and may have cyanotic episodes
They may aspirate saliva or milk into the lungs from the upper airways, and acid from the stomach
Investigations for oesophageal atresia
Passing a wide-calibre feeding tube through the mouth and checking, using an X-ray, to see if it reaches the stomach
Small bowel obstruction presentation
Persistent vomiting (may be bile-stianed, unless obstruction is procximal to the ampulla of Vater: Abdominal distension is more prominent. High lesions will present soon after birth, whereas lower lesions may take a few days)
Causes of small bowel obstruction
Atresia or stenosis of the duodenum (1/3 have Down syndrome)
Atresia or stenosis of the jejunum or ileum
Malrotation with volvulus (could lead to infarction of the entire midgut)
Meconium ileus (almlost all affected neonates have CF)
Meconium plug (a plug of congealed meconium causes lower intestinal obstruction)
How is a diagnosis of small bowel obstruction made?
Based on clinical features and abdominal X-ray
Treatment of small bowel obstruction
Atresia or stenosis is treated surgically
A meconium plug will usually pass spontaneously
A meconium ileus can be dislodged using Gastrograffin
Causes of large bowel obstruction
Hirschsprung disease - absence of the myenteric nerve plexus in the rectum which may extend along the colon
Rectal atresia - absence of the anus at the normal site
Exomphalos (Omphalocele)
Often diagnosed antenatally
Abdominal contents protrude through the umbilical ring and are covered by a transparent sac (formed by the amniotic membrane and peritoneum)
Often associated with other major congenital abnormalities
Gastroschis
Bowel protrudes through a defect in the anterior abdominal wall adjacent to the umbilicus and there is no covering sac
It is not associated with any other congenital abnormalities
Carries a much greater risk of dehydration and protein loss, so the abdomen of affected infants should be wrapped in several layers of clingfilm
An NG tube is passed and asiprated frequently and an IV dextrose infusion is set up
Colloid support is often required to replace lost protein
Can be corrected with surgery
What is the difference between posseting and regurgitation?
Posseting refers to small amounts of milk that often accompay the return to swallowed air (burp)
Regurgitation describes larger, more frequent losses, and may indicate the presence of more significant gastro-oesophageal reflux
Vomiting is the forceful ejection of gastric contents
Red flag features of a vomiting child
Bile stained vomit= intestinal obstruction
Haematemesis= oesophagitis, peptic ulceration, oral/nasal bleeding, oesophageal variceal bleeding
Projectile vomiting in the first few weeks of life= pyloric stenosis
Vomiting at the end of paroxysmal coughing = whooping cough
Abdominal tenderness/abdominal pain on movement = surgical abdomen
Abdominal distension= intestinal obstruction, including stragulated inguinal hernia
Hepatosplenomegaly= chronic liver disease, inborn error of metabolism
Blood in stool = intussusception, bacterial gastroenteritis
Severe dehydration, shock = severe gastroenteritis, systemic infection (UTI, meningitis), DKA
Bulging fontanelle or seizures = raised ICP
Faltering growth = GOR, coeliac, other chronic GI conditions
At what age do most cases of GOR resolve by?
1 year
Mechanism of infantile gastro-oesophageal reflux
Functional immaturity of lower oesophageal sphincter leading to inappropriate relaxation
Baby factors that contribute to reflux
Predominantly fluid diet
Mainly horizontal posture
Short intra-abdominal length of oesophagus
When is GOR referred to as gastro-oesophageal reflux disease?
When it starts causing significant problems
GORD is more common in:
Cerebral palsy and other neurodevelopmental disorders
Preterm infants (especially those with bronchopulmonary dysplasia)
Following surgery for oesophageal atresia or diaphragmatic hernia
Complications of GORD
Faltering growth from severe vomiting Oesophagitis Recurrent pulmonary aspiration (recurrent pneumonia, cough, wheeze) Dystonic neck posturing Apparent life-threatening events
Investigations for GOR
24 hour oesophageal pH monitoring
24 hour impedance monitoring
Endoscopy with oesophageal biopsies
Upper GI contrast study
Diagnosis usually clinical and no investigations are required. However, the investigations above may be used.
Management of GOR
Referral
SAME DAY referral if haematemesis, melaena or dysphagia present
Arrange assessment by paediatrician if:
Red flag symptoms
Faltering growth
Unexplained distress
Unresponsive to medical therapy
Feeding aversion
Unexplained iron deficiency anaemia
No improvement after 1 year of age
Suspected Sandifer’s syndrome
Refer if there are complications:
Recurrent aspiration pneumonia
Unexplained apnoeas
Unexplained epileptic seizure-like events
Unexplained upper airway inflammation
Dental erosion with neurodisability
Recurrent acute otitis media
Treatment
Reassure
It is very common
Begins early (< 8 weeks) and may be frequent
It usually becomes less frequent with time
Treatment and investigation is not usually needed
Review infant or child if:
Projectile regurgitation
Bile-stained vomit or haematemesis
New concerns (e.g. faltering growth, feeding difficulties)
Persistent, frequent regurgitation beyond the first year of life
What causes pyloric stenosis?
Caused by hypertrophy of the pyloric muscle causing a gastric outlet obstruction
Clinical features of pyloric stenosis
Presents at 2-8 weeks of age, irrespective of gestational age
4x more common in boys
Vomiting (increases in frequency and forcefulness over time, ultimately becoming projectile)
Hunger after vomiting (until dehydration leads to loss of interest in feeding)
Weight loss if presentation is delayed
Hypochloraemic metabolic alkalosis with low plasma sodium and potassium secondary to vomiting stomach contents
How is the diagnosis of pyloric stenosis made?
Gastric peristalsis may be seen as a wave moving from left to right across the abdomen Pyloric mass (feels like an olive) is usually palpable in the RUQ Ultrasound may be useful
NB: if the stomach is distended with air, it will need to be emptied using an NG tube to allow palpation
Management of pyloric stenosis
IV fluid resuscitation:
This is essential to correct the fluid and electrolyte disturbance before surgery
This should be provided at 1.5x maintenance rate with 5% dextrose and 0.45% saline
Definitive treatment is by performing a Ramstedt pyloromyotomy (dividing the hypertrophied muscle down to but not including the mucosa- can be open or laparoscopic)
Causes of crying of sudden-onset in infants
Infection (UTI, middle-ear, meningeal) Pan from an unrecognised fracture Oesophagitis Testicular torsion Severe nappy rash Constipation Teething
Pattern of symptoms in infant colic
Paroxysmal, inconsolable crying or screaming often accompanied by drawing up the knees and passage or excessive flatus takes place several times a day
NB: if severe and persistent, it may be due to a cows’ milk protein allergy
Management of infant colic
Reassure parents that colic is a common problem that should resolve by 6 months of age
Sources of information/support: NHS choices leaflet, health visitor (help with feeding techniques etc…)
Strategies to soothe a crying infant: holidng the baby, gentle motion, white noise
Encourage parents to look after themselves: get support form family and friends, meet other parents at a similar state (NCT), resting, putting the baby in a safe place to give yourself a time out
Insufficient evidence for Infacol and Colief, so do not recommend
What should always be checked in a child presenting with acute abominal pain?
Testes
Hernial orifices
Hip joints
What is the most common surgical cause of abdo pain in children?
Acute appendicitis (although it is v uncommon <3 years)
Symptoms of acute appendicitis
Anorexia
Vomiting
Abdominal pain (initially central and colicky (appendicular midgut colic), but then localising to right iliac fossa (from localised peritoneal inflammation))
Signs of acute appendicitis
Fever
Abdominal pain aggravated by movement
Persistent tenderness wit guarding in the RIF (McBurney’s point tenderness)- NB: with a retrocaecal appendix, localised guarding may be absent
Perforation can occur rapidly in children because their omentum is less well developed and fails to surround the appendix
Investigations for acute appendicitis
No laboratory investigation or imaging is consistently helpful in making a diagnosis
Neutrophilia is not always present on the blood count
WCC and organisms can be seen in the urine because the inflamed appendix may be adjacent to the ureter or the bladder
Ultrasound may support the clinical diagnosis and show complications (e.g. abscess, perforation, appendix mass)
Management of acute appendicitis
SURGICAL EMERGENCY Patient should be nil-by-mouth from the time of diagnosis IV fluids should be started Requires immediate hospital admission Treatment of choice is an appendicectomy
Define non-specific abdominal pain
Refers to abdominal pain that resolves within 24-48 hours
The pain is less severe than acute appendicitis
Often accompanied by an URTI with cervical lymphadenopathy
In some of these children, abdominal signs do not resolve and an appendicectomy is performed
Mesenteric adenitis is often diagnosed in these children as enlarged mesenteric lymph nodes are seen at laparoscopy with a normal appendix
What is intussusception?
Invagination of proximal bowel into a distal segment
Most commonly involves ileum passing into caecum through the ileocaecal valve
What is the peak age of presentation for intussusception?
3 months - 2 years
It is the most common cause of intestinal obstruction in infants after the neonatal period
What is the most serious complication of intussusception?
Stretching and constriction of the mesentery resulting in venous obstruction, causing engorgement and bleeding from the bowel mucosa, fluid loss, and, eventually, bowel perforation, peritonitis and gut necrosis
Requires prompt diagnosis, immediate fluid resuscitation and urgent reduction of the intussusception
Presentation of intussusception
Paroxysmal, severe colicky pain with pallor (during episodes, the child will become pale, especially around the mouth, and draw their legs up. There is recovery in between episodes, but the child may become lethargic)
Refusing feeds
Vomiting (may be bile-stained depending on the location of the intussusception)
Sausage-shaped mass is palpable in the abdomen
Redcurrant jelly stool is a late sign
Abdominal distension and shock (pooling of fluid in the gut, so IV fluid resuscitation is often necessary)
Viral infection leading to enlargement of Peyer’s patches may form the lead point of the intussusception
Other possible lead points include a Meckel’s diverticulum (more common >2 years)
Investigations for intussusception
X-ray may show distended bowel with no gas in the distal colon and rectum
Abdominal ultrasound is useful to confirm the diagnosis and check the response to treatment (target sign)
Management of intussusception
Unless there are signs of peritonitis, reduction by rectal air insufflation, with fluoroscopy guidance, by radiologist (successful 75% of the time- other 25 may need operation)
Clinically stable with no contraindications (peritonitis, perforation, hypovolaemic shock) to contrast enema reduction
Fluid resuscitation
Contrast enema (air or contrast liquid)
Broad-spectrum antibiotics (Clindamycin + gentamicin OR tazocin OR cefoxitin + vancomycin)
2nd line: surgical reduction with broad-spectrum antibiotics
If recurrent intussusception, consider investigating for a pathological lead point (e.g. Meckel’s diverticulum)
Presentation of Meckel’s diverticulum
Most cases are asymptomatic
May present with severe rectal bleeding (neither bright red nor true melaena), causing an acute drop in Hb
Intussusception
Volvulus
Meckel’s diverticulitis (mimics appendicitis)
Investigations for Meckel’s diverticulum
Technetium scan will demonstrate increased uptake by ectopic gastric mucosa in 70% of cases
Management of Meckel’s diverticulum
Asymptomatic= incidental imaging finding- no treatment required. Detected during surgery for other reasons- prophylactic excision
Symptomatic=
Bleeding - excision of diverticulum with blood trasnfusion (if haemodynamically stable)
Obstruction- excision of diverticulum and lysis of adhesions
Perforation/peritonitis- excision of diverticulum or small bowel segmental resection with perioperative antibiotics
Complications of malrotation
During rotation of the small bowel in foetal life, if the mesentery is not fixed at the duodenojejunal flexure or in the ileocaecal region, its base is shorter than normal and is predisposed to volvulus
What are Ladd bands?
Peritoneal bands that may cross the duodenum (often anteriorly)
Main presentations of malrotation
Obstruction
Obstruction with compromised blood supply
Obstruction with bilious vomiting is the usual presentation within the first few days of life, but it can occur later
If there are signs of vascular compromise, an urgent laparotomy is needed
A child presents with dark green vomiting- what do you do?
Urgent upper GI contrast study to assess intestinal rotation
Management of malrotation
Ladd procedure- detorting the bowel and surgical dividing the Ladd bands (the bowel is placed in the non-rotated position with the duodenojejunal flexure on the right and the caecum and appendix on the left- appendix is usually removed to avoid diagnostic confusion in case the child presents again with an acute abdomen)
Antibiotics (cefazolin)
Define recurrent abdominal pain
Pain sufficient to interrupt normal activities and lasts for at least 3 months
Presentation of recurrent abdominal pain
Pain is typically periumbilical and children are generally well
Constipation is a frequent cause of recurrent abdominal pain and should be excluded
May occur as a manifestation of stress
Vicious cycle of anxiety - parents demanding more invasive investigations etc. could worsen the perceived symptoms
Management of recurrent abdominal pain
Aim to identify any serious cause without subjecting the child to unnecessary investigations
Examination includes inspection of the perineum for anal fissures and checking the child’s growth
Urine MC+S is important as UTIs may cause pain without other signs
Abdominal US may be useful to exclude gallstones and pelvic ureteric junction obstruction
Coeliac antibodies and TFTs should be checked (although these are rare causes of recurrent abdominal pain)
IBS and functional dyspepsia are diagnoses of exclusion and should be explained simply to patients (e.g. “sometimes the insides of the intestines become so sensitive that some children can feel the food going around the bends”)
Long-term prognosis of recurrent abdominal pain
Around 50% rapidly become free of symptoms
IN around 25%, symptoms take months to resolve
In around 35%, symptoms continue or return in adulthood as migraine, IBS or functional dyspepsia
Clinical features of abdominal migraine
Often associated with abdominal pain and headaches
The pain is typically in the midline and is associated with vomiting and facial pallor
There is often a personal or family history of migraine
The history tends to describe long periods of no symptoms followed by a shorter period of non-specific abdominal pain and pallor, with or without vomiting
Treatment with anti-migraine medication could help
What may precipitate IBS symptoms?
Intra-abdominal infection
What is important to remember in IBS?
The disease has a significant psycho-social component (stress and anxiety)
There is often a positive family history
Some people with IBS will also have coeliac disease, so coeliac antibodies must always be tested
Symptoms of IBS
Non-specific abdominal pain (often periumbilical and relieved by defecation)
Explosive, loose or mucousy stool
Bloating
Feeling of incomplete defecation
Constipation (often alternating with loose or normal stools)
Management of IBS
Reassure
Encourage patient to identify sources of stress or anxiety in their lifestyle and any foods that may aggravate symptoms
Recommend adequate fluid intake
What is a strong predisposing factor to duodenal ulcers?
H. pylori
Although they are uncommon in children, duodenal ulcers should be considered in those with epigastric pain particularly if:
It wakes them up at night
Pain radiates to the back
History of peptic ulceration in a first-degree relative
How can nodular antral gastritis be identified?
H. pylori causes nodular antral gastritis, which may be associated with abdominal pain and nausea
It can be identified with gastric antral biopsies
Investigations for H.pylori infection
Gastric antral biopsies (if suspected nodular antral gastritis)
CLO test
C-13 breath test (H.pylori produces urease)
Stool antigen
Management of peptic ulceration
In children with suspected peptic ulceration, it should be treated with proton-pump inhibitors
If investigations suggest the presence of an H.pylori infection, eradication therapy should be given (amoxicillin AND metronidazole OR clarithromycin)
If they fail to respond to treatment, an upper GI endoscopy should be performed (if this is normal –> functional dyspepsia is diagnosed- a variant of IBS)
Non-specific symptoms of functional dyspepsia
As well as symptoms of peptic ulceration, children with functional dyspepsia will have other non-specific symptoms such as:
Early satiety
Bloating
Post-prandial vomiting
Delayed gastric emptying (due to gastric dysmotility)
What is eosinophilic oesophagitis?
An inflammatory condition affecting the oesophagus caused by activation of eosinophils within the mucosa and submucosa
Presentation of eosinophilic oesophagitis
Vomiting
Discomfort when swallowing or bolus dysphagia (when food gets stuck)
Asthma
Eczema
Hayfever
How is the diagnosis of eosinophilic oesophagitis made?
Endoscopy:
Linear furrows and trachealisation of the oesophagus may be seen (looks like the trachea with rings protruding into th elumen)
Microscopically, eosinophilic infiltration will be identified
Management of eosinophilic oesophagitis
Oral corticosteroids (fluticasone or viscous budesonide)
What is the most common causative organism for gastroenteritis in developed countries?
Rotavirus- particularly prevalent in winter and early spring
Bacterial causes are less common in developed countries but may be suggested by presence of blood in stools
Campylobacter jejuni is the most common cause of bacterial gastroenteritis in developed countries, and is often associated with severe abdominal pain
Shigella infection
Shigella and some salmonellae produce a dysenteric type of infection, with blood and pus in the stool, pain and tenesmus
Shigella infection may be accompanied by a high fever
Cholera and enterotoxigenic E.coli infection
Associated with profuse, rapidly dehydrating diarrhoea
What is the most serious complication of gastroenteritis?
Dehydration leading to shock
Which children are at increased risk of dehydration secondary to gastroenteritis?
Infants (especially low birthweight): great SA:volume ratio, higher basal fluid requirements, immature renal tubular reabsorption
If >/=6 diarrhoeal stools in the past 24 hours
If vomited >/=3 times in past 24 hours
If unable to tolerate extra fluids
If they have malnutrition
Management of gastroenteritis in children
Assess for features of dehydration and shock, features that may suggest an alternative diagnosis, and severity and possible cause of infection
Consider hospital admission
Give rehydration advice:
Maintenance volumes:
0-10kg= 100ml/kg
10-20kg= 1000ml + 50ml/kg for each kg over 10
20+ kg= 1500ml + 20ml/kg for each kg over 20kg
Modes of rehydration:
<5 years= 50ml/kg for fluid deficit replacement over 4 hours as well as maintenance with oral rehydration solution
5+ years= 200ml ORS after each loose stool
Perform stool sample analysis: if this reveals causative organism, seek specialist advice regarding antibiotic treatment
Give advice on preventing spread and follow-up
Notify local authority if a notifiable disease is diagnosed or suspected (e.g. cholera)
Red flag signs of dehydration
Appears unwell or deteriorating Altered responsiveness e.g. lethargic, irritable Sunken eyes Tachycardia Tachypnoea Reduced skin turgor
What is the most accurate measure of dehydration?
Degree of weight loss during diarrhoeal illness:
No clinically detectable dehydration <5% BW loss
Clinical dehydration 5-10% loss of BW
Shock >10% loss of BW
What is a potential consequence of hyponatraemic dehydration?
When children with diarrhoeal illness drink a large amount of water –> hyponatraemic
This makes water move from the extracellular compartment into the intracellular compartment. The increase in intracellular volume leads to an increase in brain volume, which may result in seizures
The marked extracellular depletion leads to a greater degree of shock per unit of water loss
Causes of hypernatraemic dehydration
High insensible water loss (e.g. fever, or hot/dry environment)
Profuse, low sodium diarrhoea
More difficult to identify clinically, as signs of extracellular fluid depletion are less per unit of fluid loss
Potential consequences of hypernatraemic dehydration
Water is drawn out of the brain–> cerebral shrinkage, which may lead to jittery movements, increased muscle tone, hyperreflexia, altered consciousness, seizures, multiple small cerebral haemorrhages
Transient hyperglycaemia occurs in some patients, but this is self-correcting
When is a stool culture indicated for dehydration?
Child appears septic Blood or mucus in stools Child is immunocompromised Recent foreign travel Diarrhoea has not improved in 7 days Diagnosis is uncertain
If antibiotics are started, a blood culture should be taken
Indications for IV fluids in dehydration
Shock
Deterioration
Persistent vomiting
Treating hypernatraemic dehydration
ORS
If IV fluids are required, a rapid reduction in plasma sodium concentration and osmolality will lead to a shift of water into the cerebral cells and may result in seizures and cerebral oedema, so reduction in plasma sodium should be slow- at least 48 hours, with regular measurement of plasma sodium
Why should antidiarrhoeals/antiemetics not be used in children
Ineffective
May prolong the excretion of bacteria in stools
Can be associated with side-effects
Adds unnecessarily to cost
Focus attention away from oral rehydration
Indications for antibiotics in dehydration
Suspected or confirmed sepsis
Extra-intestinal spread of bacterial infection
Salmonella gastroenteritis if <6 months
Malnourished or immunocompromised children
Specific bacterial or protozoal infections (e.g. C diff associated with pseudomembranous colitis, cholera, shigellosis, giardiasis)
Dehydration may lead to zinc deficiency
SO supplementation may be necessary
Postgastroenteritis syndrome
Occasionally, after an episode of gastroenteritis, the introduction of a normal diet results in a return of the watery diarrhoea
Oral rehydration therapy should be restarted
Manifestations of malabsorption
Abnormal stools
Poor weight gain or faltering growth (not all cases)
Specific nutrient deficiencies, either singly or in combination
True malabsorption stool is difficult to flush and has a potent smell
Define acrodermatitis enterohepatica
Autosomal recessive metabolic disorder characterised by tge malabsorption of zinc, which results in:
Diarrhoea
Inflammatory rash around the mouth and/or anus
Hair loss
Coeliac disease
Enteropathy in which the gliadin fraction of gluten in wheat, barely and rye provoke a damaging immunological response in the proximal small intestine–> massive increase in rate of migration of absorptive cells moving up the villi from the crypts, but this is insufficient to compensate for increased cell loss from villous tips–> villi become shorter and progressively absent–> flat mucosa
Age of presentation of coeliac disease
Depends on the age of introduction of gluten into the diet
Presentation of coeliac disease
Profound malabsorptive syndrome at 8-24 months of age after introduction of wheat into diet Faltering growth Abdominal distension Buttock wasting Abnormal stools General irritability Mild, non-specific GI symptoms Anaemia
Investigations for coeliac disease
Anti-tTG antibodies
Endomysial antibodies
Duodenal biopsy (required to confirm diagnosis)
Catch up of growth following gluten withdrawal from the diet is also required to confirm diagnosis
Management of coeliac disease
All products containing wheat, rye and barley are removed from diet
Monitor body weight, height and BMI to assess for signs of malnutrition
Consider referral to dietician if there are problems with adhering to the diet
Arrange annual review
Non-adherence results in: micronutrient deficiency (especially osteopenia), small increased risk of bowel cancer (especially small bowel lymphoma)
Toddler’s diarrhoea (chronic non-specific diarrhoea)
Most common cause of persistent loose stools in preschool children
Stools vary in consistency, are sometimes well formed, sometimes explosive and loose
Affected children are well and thriving
In some cases, it might result from undiagnosed coeliac disease or excessive ingestion of fruit juice (especially apple juice)
Occasionally caused by temporary cows’ milk allergy after gastroenteritis
Once most probably causes have been excluded, most cases are probably a result of dysmotility of the gut (form of IBS) and fast-transit diarrhoea
These tend to improve with age
