Em first 12 months of life by GP Academy

Independent learning program for GPs

Unit 466/467 January/February 2011

First 12 months of life

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Independent learning program for GPs

Medical Editors Kath O’Connor Jo-Anne Zappia

First 12 months of life

Editor Nicole Kouros

Unit 466/467 January/February 2011

Production Coordinator Morgan Liotta

From the editor

Case 1

Samuel’s head is an odd shape

Case 2

Mandy’s baby won’t stop crying

Case 3

Ariel was born at 25 weeks gestation

Case 4

Does Olivia have a food allergy?

Case 5

Josh has had a fit

Case 6

Baby Emily is wheezy

References

Resources

Category 2 QI&CPD activity

Senior Graphic Designer Jason Farrugia Graphic Designer Beverly Jongue Authors Harriet Hiscock Geoff Solarsh Julee Oei Preena Uppal Katie Allen Dean Tey Mike Starr Reviewer Marie-Louise Dick Subscriptions Call the Subscription Coordinator for all enquiries on 03 8699 0495 or email check@racgp.org.au. Published by

The five domains of general practice Communication skills and the patient-doctor relationship Applied professional knowledge and skills Population health and the context of general practice Professional and ethical role Organisational and legal dimensions

The Royal Australian College of General Practitioners College House, 1 Palmerston Crescent South Melbourne, Victoria 3205, Australia Telephone 03 8699 0414 Facsimile 03 8699 0400 www.racgp.org.au ACN 000 223 807 ABN 34 000 223 807 ISSN 0812-9630 © The Royal Australian College of General Practitioners 2011. All rights reserved. The opinions expressed in check are not necessarily those of the RACGP. Please address all letters concerning content to the medical editor. Printed by Printgraphics Pty Ltd, 14 Hardner Road, Mount Waverley, Victoria 3149 Telephone 03 9562 9600.

FROM THE EDITOR

check First 12 months of life

This unit of check looks at the first 12 months of life and provides clinical scenarios relating to abnormally shaped heads; crying in infants; assessment and management of the premature infant in the first 12 months of life; gastrointestinal symptoms; and diagnosis of food allergy, febrile convulsions and the wheezy infant. The first 12 months in a child’s life can be a rewarding and joyful time for parents and families, however, illness and significant challenges and difficulties may also occur. General practitioners play an important role in diagnosing and managing the illnesses that can arise during the first 12 months of life, as well as in providing information and support about illness and normal behaviour during this period of a child’s life. The authors of this unit bring a wealth of clinical, research and teaching experience to the topic. The authors are: t Harriet Hiscock MBBS, FRACP, MD, GradDipEpiBiostat, consultant paediatrician and Postdoctoral Research Fellow at the Centre for Community Child Health, The Royal Children’s Hospital Murdoch Childrens Research Institute, Victoria. Her clinical and research interests include prevention and early intervention for infant sleep and colic and associated maternal depression, and sleep and mental health problems in children t Geoff Solarsh MBBCh, DCH, DipAdEd, FCP, FRACP, consultant paediatrician, Bendigo Health, Victoria and Director, Bendigo Regional Clinical School, School of Rural Health, Monash University, Victoria. His clinical, teaching and research interests include general paediatrics, community based medical education and primary healthcare systems t Julee Oei MBBS MD FRACP, neonatologist, The Royal Hospital for Women, and senior lecturer, University of New South Wales, New South Wales. Her clinical and research interests include the care of the drug exposed newborn infant, oxygen for the resuscitation of preterm infants, drug use in pregnancy and long term health outcomes of neonatal intensive care graduates t Preena Uppal MBBS, MD, neonatal registrar, The Royal Hospital for Women, New South Wales. Her clinical interests include the care of the preterm infant and long term neurodevelopmental outcomes of neonatal intensive care graduates t Katie Allen MBBS, BMedSc, FRACP, PhD, consultant allergist/gastroenterologist, The Royal Children’s Hospital, Victoria. Her clinical and research interests include food allergies and coeliac disease t Dean Tey MBBS, FRACP, paediatric allergist and immunologist, The Royal Children’s Hospital, Victoria and Epworth Hospital, Victoria. His clinical and research interests include food allergy, eczema, allergic rhinitis, asthma, urticaria and anaphylaxis t Mike Starr MBBS, FRACP, paediatrician, infectious diseases physician and consultant in emergency medicine, and Director of Paediatric Physician Training, The Royal Children’s Hospital, Victoria. His clinical and research interests include tropical and travel medicine, perinatal infections and emergency department presentations of infectious diseases. The learning objectives of this unit are to: t understand the possible causes of abnormally shaped heads in infants, and assess and manage these patients appropriately t understand the natural history of crying in infants and provide appropriate management advice to parents who are concerned their baby may be crying excessively t display increased confidence in the assessment and management of premature infants in the first 12 months of life t recognise the important causes of gastrointestinal upset in infants and assess and manage these patients appropriately t recognise the clinical features of a typical febrile convulsion and manage this condition appropriately t understand the possible causes of wheezing in infants and assess and manage these patients appropriately. We hope that this unit will assist you to confidently assess and manage infants in the first 12 months of life. Finally, this unit marks the beginning of a new year of check units and a new triennium for the RACGP’s QI&CPD unit. Over the past 3 years, only a handful of participants completed the Category 1 activity associated with check. As a result, the Category 1 activity will no longer be available from January 2011. The RACGP apologises for any inconvenience caused by this change. Participants can still qualify for 6 Category 2 points by completing the unit and answering the 10 multiple choice questions online at gplearning (see page 23 for further instructions). Wishing you a happy and safe new year. Best wishes

Kath O’Connor Medical Editor

CASE 1

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CASE 1 SAMUEL’S HEAD IS AN ODD SHAPE Samuel, aged 9 months, is brought in by his mother Sally who is concerned that his head is oddly shaped. Samuel was born at term by normal vaginal delivery after an uneventful pregnancy. His Apgar scores were 8 and 10 at 1 and 10 minutes respectively, and mother and child were discharged together after 2 days in hospital. He was followed for the first 3 months of life by a maternal and child health nurse who commented at all visits that his growth and development were normal. He smiled at 6 weeks, sat without support at 6 months and has just started crawling. Sally first noted that Samuel’s head was abnormally shaped when he was 4 months old. At the time a friend reassured her that he would grow out of it but she is now concerned as it has not improved and she thinks it may be getting worse. Sally wants to know if Samuel’s head shape is normal and whether anything can be done about it. On examination Samuel is happy, alert and interactive. He weighs 9.7 kg. Multiple head circumference measurements since birth indicate that his head is growing along the 25th percentile for age. Samuel’s head shape viewed from above is shown in Figure 1. There are no obvious dysmorphic features besides the skull asymmetry shown in this photograph. His developmental assessment is appropriate for age and neurological, cardiorespiratory and abdominal examinations are normal.

QUESTION 1 What is the descriptive term for a head of this shape?

QUESTION 2 List the two most likely causes for this head shape.

QUESTION 3 What underlying developmental mechanisms could be responsible for this head shape?

QUESTION 4 Which of the two causes outlined in your answer to Question 2 is more likely and why?

Figure 1. Samuel’s head shape viewed from above

CASE 1

check First 12 months of life

QUESTION 5

QUESTION 8

What additional history or examination findings would help you to distinguish between these two causes?

What additional underlying cause must you now consider? What is its significance and how would you confirm this clinically?

QUESTION 9 QUESTION 6

What is the definitive diagnosis?

What investigations, if any, would you perform in Samuel?

QUESTION 10 What is your management plan?

FURTHER INFORMATION Samuel’s skull X-ray shows bilateral patency of the lambdoid sutures. QUESTION 7 What implications, if any, does this head shape have for Samuel’s future physical and cognitive development?

CASE 1 ANSWERS

ANSWER 1 The term for the head shape shown in this image is plagiocephaly. It describes an asymmetric cranium and is derived from the Greek words plagio (oblique, twisted or slanted) and kephale (head). FURTHER INFORMATION On observation of Samuel’s face and head from the front, it is noted that his head is tilted to the right and his face is turned to the left.

ANSWER 2 The most likely causes for a skull of this shape are: t deformational (or positional) plagiocephaly t craniosynostosis (unilateral lambdoidal plagiocephaly).

CASE 1

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ANSWER 3

Side view

Deformational plagiocephaly Deformational plagiocephaly refers to a flattening of the occiput with resulting changes to the craniofacial skeleton. It begins in the postnatal period. It has been recognised as an entity for many years, but there has been a steep increase in its incidence since the recommendation by paediatricians that babies should be placed to sleep in the supine position to minimise the risk of sudden infant death syndrome (SIDS).1 When placed supine infants assume a position of comfort that most likely corresponds to their previous intrauterine lie. Since babies are unable to lift their heads or turn across the midline until 3 months of age there will be consistent pressure on one side of the occiput, more frequently on the right. Once established, this adopted sleep position is quite difficult to change and results in ongoing deformational forces that maintain, and in time, aggravate this asymmetry. The main consequence of deformational plagiocephaly is cosmetic. Craniosynostosis Craniosynostosis is defined as premature closure of skull sutures (Figure 2) during development, resulting in deformities of the skull. The initiating event in craniosynostosis is the premature closure of one or more of the skull sutures. In some cases this may result in secondary restriction of brain growth because of a decrease in volume of the cranial vault. Invariably, this condition has significant cosmetic effects. As craniosynostosis involves selective closure of particular sutures, it results in recognisable patterns of skull deformity that reflect failed cranial growth in specific planes. As a general principle, suture fusion results in a failure of growth at right angles to the affected suture line (Table 1). Early fusion of the sagittal suture causes failure of lateral growth but ongoing growth from front to back, resulting in temporal narrowing and compensatory elongation of the skull in the anteroposterior diameter (this is called scaphocephaly). Bilateral fusion of the coronal sutures, on the other hand, results in a skull that is shortened in the anteroposterior diameter but widened in the lateral diameter (this is called brachycephaly). There are variations of bilateral coronal fusion in the extent to which skull growth is restricted in width and height, and that probably reflect the timing of fusion at different stages of skull development (these are called acrocephaly, oxycephaly and turricephaly). When the metopic suture alone is fused, the forehead is noted to be narrow and triangular with a prominent ridge (this is called trigonocephaly). Finally, the generic term plagiocephaly is also used for forms of craniosynostosis in which asymmetric skull shapes are generated by unilateral fusion of the coronal or lambdoidal sutures. ANSWER 4 Deformational plagiocephaly is by far the most likely diagnosis. It is a much more common condition than craniosynostosis and the shape of Samuelâ&#x20AC;&#x2122;s head when viewed from above is typical of deformational plagiocephaly. The hallmark of deformational plagiocephaly is positional moulding, where the skull takes on a parallelogram shape when viewed from

Coronal suture Lambdoid suture Sphenoidal fontanelle Maxilla

Squamosal suture Mastoid fontanelle

Mandible

Top view Frontal bone Parietal bone

Metopic suture Anterior fontanelle Sagittal suture

Posterior fontanelle Occipital bone Figure 2. Infant skull showing position of sutures. Adapted from and reproduced with permission from A.D.A.M. Images online. Available at www.adamimages.com

Table 1. Descriptive terms for skull shapes in infants Descriptive term

Suture/s involved

Shape

Acrocephaly

Bilateral coronal

Skull height greater anteriorly, slanting downwards posteriorly

Brachycephaly

Bilateral coronal

Wide, taller, skull shortened in anteroposterior diameter

Oxycephaly

Bilateral coronal

Taller skull, shortened in both width and anteroposterior diameter

Turricephaly

Bilateral coronal

Tall skull

Plagiocephaly

Unilateral coronal or lambdoidal

Asymmetrical skull

Scaphocephaly

Sagittal

Anteroposterior elongation with bitemporal narrowing

Trigonocephaly

Metopic

Narrow triangular ridged forehead

above (Figure 3). There is associated unilateral flattening of the occiput and anterior displacement of the ipsilateral craniofacial features, including the ipsilateral forehead and ear and posterior displacement of the contralateral forehead. Certain forms of craniosynostosis with unilateral suture fusion may look quite similar to deformational plagiocephaly at first glance. In this case the form of craniosynostosis to consider in the differential diagnosis is unilateral lambdoidal craniosynostosis, in which unilateral flattening of the occiput is a consequence of fusion of a single lambdoid suture (Figure 3). Flattening of the occiput may have a similar appearance in unilateral lambdoidal craniosynostosis to that in deformational plagiocephaly. However, in craniosynostosis the ipsilateral craniofacial structures are displaced posteriorly rather than anteriorly, and the skull, when viewed from above, has the shape of a rhomboid rather than a parallelogram. In other forms of craniosynostosis, such as sagittal suture fusion, bilateral coronal suture fusion and metopic suture fusion, there are very typical and quite different patterns of skull asymmetry (Table 1).

CASE 1

check First 12 months of life

ANSWER 8 Positional molding (A)

Contralateral occipital bossing

Ipsilateral ear displaced anteriorly Flattening

Unilateral lambdoid synostosis (B)

Parietal Flattening bossing

Ipsilateral ear displaced posteriorly (variable)

Ipsilateral occipitomastoid bossing

Figure 3. Shape of infant skull (viewed from above) in deformational plagiocephaly (A – parallelogram) and unilateral lambdoidal synostosis (B – rhomboid). Reproduced with permission from Elsevier Ltd

ANSWER 5 In unilateral lambdoidal craniosynostosis, there are seldom any other dysmorphic or syndromic features and no clinically significant restriction of brain growth, so there may be little to distinguish between these two conditions on history. An important distinguishing feature on examination, beyond the skull shape itself, is the absence of a clearly palpable lambdoid suture on the affected side in the case of craniosynostosis. However, in some cases, this suture may be quite difficult to feel. ANSWER 6 When there is remaining doubt, skull radiography should be requested to determine whether the sutures remain patent or not. Signs of premature fusion include loss of visualisation of the suture or part of it, perisutural sclerosis and bony bridging. Importantly, X-rays are often unreliable in the first 3 months of life when low mineralisation of the skull makes it difficult to visualise the presence of sutural fusion.2 ANSWER 7 The skull shape, X-ray findings and overall clinical picture strongly suggest deformational plagiocephaly. In Samuel’s case, there is no increased risk for neurodevelopmental problems but there is clearly a risk for long term cosmetic problems. FEEDBACK In single suture craniosynostosis, focal neurological deficits and raised intracranial pressure are rare unless the synostosis is severe, although there is growing evidence that a significant number of children with single suture fusion have mild to moderate neurobehavioural impairment despite normal intelligence.3

These observations suggest that Samuel may have congenital torticollis (ie. a congenital shortening of his right sternocleidomastoid [SCM]muscle). Deformational plagiocephaly is commonly associated with torticollis as a result of shortening of the SCM muscle on the opposite side to the direction in which the head is consistently turned. This is considered to be a developmental defect from the persistence of this head position in utero, causing a secondary stiffness and shortening of the muscle that continues into the postnatal period, rather than true atrophy or fibrosis. The contracted SCM muscle acts to tilt the head to the same side and rotate it to the opposite side. In deformational plagiocephaly, the head is tilted on the opposite side to, and rotated in the same direction as, the flattened occiput. In some cases there may also be a tumour (benign lump) in the belly of the shortened SCM muscle (this is not present in Samuel’s case). The clear relationship between a flattened occiput on the left, a head that is tilted to the right and a face that is perpetually turned to the left, suggests that the deformational plagiocephaly is directly related to the torticollis in this infant. ANSWER 9 Samuel’s final diagnosis is deformational plagiocephaly associated with congenital torticollis. ANSWER 10 Management begins with prevention of further deformity. The earlier this is instituted, the greater the likelihood of success. Although the supine sleeping position should continue to be promoted for its established value in preventing SIDS, prone positioning or ‘tummy time’ for the awake observed infant can be safely recommended to prevent the development of a persistent head position. Additionally, attempts should be made to reposition the baby’s head during sleep by placing a rolled-up receiving blanket to offload pressure from the affected side of the occiput and by positioning the cot so that the infant is forced to lie on the unaffected side in order to receive auditory and visual stimuli from toys, mobiles and other activity in the room. During feeding, parents are encouraged to cradle the baby’s head so that pressure is removed from the affected side of the head. If torticollis is present, gentle neck exercises should be recommended with each nappy change for a duration of 5–6 months. Each position should be held for 10 seconds and repeated 3 times each side. These exercises include: t gently rotating the baby’s head bringing the chin toward the shoulder t tilting the head toward the ipsilateral shoulder t bringing the chin toward the chest. Infants who fail conservative management and have significant cosmetic deformation can be referred, preferably before the age of 6 months, to a craniofacial unit in a large paediatric centre for external cranioplasty with an orthotic device or moulding helmet to assist with the reshaping of the skull. A physiotherapist may assist with exercises in the case of torticollis. Early referral to a craniofacial unit would also be needed in the less likely case of craniosynostosis.

CASE 2

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CASE 2 MANDY’S BABY WON’T STOP CRYING

QUESTION 1 What is the natural history of crying in infants?

Mandy presents with her son Tom, aged 7 weeks. Tom is her second child with her partner Ravi. Mandy says Tom has been crying excessively for the last 4 weeks. He cries for most of the day, but it is worse in the evening. When he cries, he arches his back, goes red in the face and sometimes makes gulping sounds. He does not vomit. She has tried picking him up, herbal drops, a chiropractor and is thinking about changing her diet as nothing is working. He is fully breastfed but Mandy thinks she may not have enough milk. Tom has 3–4 soft bowel actions per day. They are dark green in colour with no blood or mucous and they are not frothy. He has three naps per day that last around 60 minutes each. He naps in his cot or in the car or in a rocker in the living room. She cannot tell when he is tired as he seems to cry ‘all the time’. Overnight, he sleeps in his own cot in his parents’ room. His mother feeds him off to sleep for his day and night sleeps. He falls asleep around 11.00 pm and wakes twice overnight. After a feed he generally settles back to sleep okay. He wakes at 7.00 am.

QUESTION 2 What proportion of infants will have a medical cause for prolonged (ie. greater than 1 week) unsoothable crying?

During her pregnancy with Tom, Mandy had morning sickness well into the second trimester. Tom was born by vacuum extraction at term plus 1 week. Apgar scores were 9 at 1 minute and 9 again at 5 minutes. His birth weight was 3.1 kg. There were no neonatal complications. Tom has smiled once, fixes and follows his mother around the room with his eyes, and startles to loud noises. Mandy says he is very alert compared with her daughter as a baby. There is no family history of food allergies or atopy. Mandy was depressed as an adolescent, this was managed with counselling. Ravi works the evening shift in a café and Mandy is on maternity leave from her administration job. Her family live interstate and Ravi’s family live overseas. Their daughter is 3 years of age. She is getting upset by Tom’s crying and all the attention he is receiving so she has started hitting Tom.

QUESTION 3 What other factors commonly contribute to prolonged infant crying?

On examination, Tom is crying but stops as you begin to talk with him and make eye contact. His weight and length are on the 10th percentile. He has good subcutaneous fat deposition. His examination is otherwise unremarkable. His mother appears exhausted but says she still enjoys him when he is not crying.

CASE 2

QUESTION 4 What causes infant colic?

check First 12 months of life

FURTHER INFORMATION On review, Mandy says Tom is sleeping more and crying less. The diary confirms this. However, Mandy says she now feels utterly exhausted and is no longer enjoying Tom. QUESTION 7 What maternal comorbidity commonly exists with infant colic and how can you screen for it?

FURTHER INFORMATION Mandy says she has heard that parenting style can influence how much a baby cries. She asks whether a routine based approach, where the baby is fed every 3–4 hours and placed to sleep in their own cot or basinet, is better than holding the baby most of the time and feeding on demand. QUESTION 5 Can parenting style or culture influence the nature of crying?

QUESTION 6 How will you manage Tom?

CASE 2 ANSWERS ANSWER 1 All infants cry. Some crying is easy to soothe (eg. with a feed or a sleep) but some is not. Problem or unsoothable crying often begins around age 3 weeks, peaks between 6–8 weeks of life and resolves by 12–16 weeks. ‘Colic’ is a name given to unsoothable crying that lasts for more than 3 hours per day, for more than 3 days per week, for at least 1 week (Wessel’s modified criteria).4 Crying bouts tend to occur in the late afternoon and evening but can take place any time. Crying beyond age 3 months is more concerning, it may indicate an underlying medical cause and/or suboptimal parent-infant relationship. Figure 4 shows the crying curve from the Period of PURPLE Crying website, an evidenced based, parent support website (see Resources). The crying curve shows the duration of crying over 24 hours for ‘high criers’ that would often be called ‘colicky’ babies. The acronym PURPLE describes the characteristics of the crying – Peak of crying at 2 months, Unexpected, Resists soothing, Pain-like face, Long lasting and Evening peak. ANSWER 2 Medical problems account for less than 10% of causes of prolonged crying.5 Medical causes include food intolerances (usually non-immunoglobulin E (IgE) mediated cow’s milk protein intolerance) and possibly lactose intolerance and gastro-oesophageal reflux (GOR). However, in a large study (n=151) of irritable babies, there was no correlation between GOR as measured by pH monitoring and crying.6 In another study of 70 infants, GOR measured by pH monitoring was predicted by frequent vomiting only.7 ‘Silent reflux’ whereby milk is thought to come up into the back of the infant’s throat and cause crying but not vomiting, was not observed. In a separate double-blind crossover study of 30 irritable infants, crying time did not differ between placebo and omeprazole despite effective acid suppression with the latter.8

CASE 2

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growth may plateau or even cross percentile lines on a growth chart. Tom is growing well so hunger is an unlikely cause of his crying.

Curves of early infant crying 2 weeks to 4–5 months 5–6 hours

ANSWER 4 The cause of infant colic is unknown and is likely to be multifactorial. For most babies, problem crying is part of a normal spectrum whereby babies who have not yet learned to ‘self soothe’ and regulate their own crying become persistent criers. They are often described by their parents as very alert and easily startled or upset. They may have a delay in their neuromaturation, ie. a delay in their ability to self soothe once they start crying.

High crier

Length of time crying

Average crier Low crier

20–30 minutes

2 weeks

2 months

4–5 months

Figure 4. The normal crying curve from the Period of PURPLE Crying website. Reproduced with permission from Professor Ronald G Barr

ANSWER 3 Two of the most common causes of crying in infants are tiredness and hunger. On average, babies sleep for 16.5 hours per day at birth. This falls to 14 hours by age 2–3 months. If babies are awake during the day and happy, they are unlikely to need more sleep. Generally, a baby of 6 weeks becomes tired after being awake for 1.5 hours, while a baby of 3 months becomes tired after being awake for 2 hours.9 Tom is getting at the most 9.5 hours of sleep out of 24 hours, so is sleep deprived. Infants who are hungry tend to feed more often and may not settle for long daytime naps (ie. naps that last at least 1 hour). Their

ANSWER 5 There does not appear to be any cultural difference in how much babies cry. The normal crying curve shown in Figure 4 is applicable to babies all over the world.10 Parents who hold their baby for 18 hours per day or more and feed them on demand report less fussing from their babies in the first few months of life compared with parents who are more routine based. However, the amount of unsoothable crying is the same for the two parenting styles.11 ANSWER 6 Figure 5 provides a flowchart for the assessment and management of persistent crying. A medical cause is unlikely in Tom. Recommended management for a baby with unsoothable crying if a medical cause of crying is unlikely includes the following: t inform parents that their baby is physically well and developing normally. Parents report that one of the most useful things is learning that their baby is medically okay12

Diagnostic and management flowchart for infant irritability Persistent irritability

Daily vomiting or explosive diarrhoea or diarrhoea with mucus/blood

History and examination

Medical cause unlikely

Yes

Consider

Yes Consider t lactose intolerance (explosive diarrhoea) t cow’s milk allergy (blood/mucus diarrhoea)

Yes

Diarrhoea and/or poor weight gain

Faecal-reducing substances >0.25%, and faecal pH <7.0

Vomiting

Yes

t t t t

discuss normal sleep and crying discuss settling techniques maximise parental support arrange regular follow up

t cow’s milk allergy (especially if atopic disease present) t gastro-oesophageal reflux

Trial of cow’s-milk-free formula or cow’s-milk-free maternal diet

Trial of lactose-free formula or lactase-treated breast milk Figure 5. Diagnostic and management flowchart for infant irritability.9 Reproduced with permission from The Medical Journal of Australia

CASE 2

check First 12 months of life

t plot the baby’s growth and show the parents the growth chart to reassure them their baby is thriving

t maximise supports for the family (eg. ask the local maternal and child health nurse service to do a home visit to show parents how to settle their baby, suggest parents think about getting some support looking after their baby from family, friends or a baby sitter)

t explain the natural history of crying t show the family the normal crying curve from the Period of PURPLE Crying website

t give the mother permission to rest completely once per day when the baby is asleep

t if the baby is sleep deprived:

t ask parents to keep a baby ‘cry/sleep’ diary (Figure 6)

– cue the family into the baby’s tired signs (eg. frowning, clenching hands and feet; jerking arms and legs; and grizzling and crying)

t organise follow up within 1–2 weeks and review the diary. Are there any patterns to the crying such as just before feed time or after the baby has been awake for a prolonged period of time?

– maximise day sleeps, aiming for 3–4 naps per day, preferably in the same room with a consistent settling strategy

t if the crying is not improving, reconsider underlying medical causes and family issues including postnatal depression (mother and father), domestic violence, relationship problems, drug and alcohol problems, financial or housing stress.

– aim for a bedtime between 7.00 and 8.00 pm t if the baby is hungry: – ask about the mother’s milk supply or amount of formula where relevant

ANSWER 7 The most important maternal comorbidity to consider here is postnatal depression. Postnatal depression affects 10–15% of Australian mothers in the first year postpartum12 but this rises to 45% in mothers of infants with sleep/cry problems.13 Postnatal depression can also affect partners. Consider screening all mothers who present with infant crying for postnatal depression, eg. by using the 10-item Edinburgh Postnatal Depression Scale14 (see Resources). Scores of more than 12 are indicative of postnatal depression in mothers.

– consider referral to a lactation consultant to assess the mother’s milk supply and breast feeding technique t discuss the baby’s need for interaction and play. For some families, a ‘campaign’ to soothe the baby and avert a crying episode takes over. For such families, it may be more useful to ‘go with the flow’ and encourage the mother to have some enjoyable time with her baby t offer some practical settling tips even though they may not always work (eg. taking the baby for a walk, putting the baby in a sling or giving the baby a warm, deep bath) Cry/sleep diary

When your child is asleep

When your child is awake

V When baby is awake and crying

F To indicate feed

Day Date Events/notes Mn 1am 2am 3am 4am 5am 6am 7am 8am 9am 10am 11am Md 1pm 2pm 3pm 4pm 5pm 6pm 7pm 8pm 9pm 10pm 11pm Mn Mon 19/5

Figure 6. An example of a baby behaviour diary. Reproduced with permission from the Unsettled Babies Clinic, The Royal Children’s Hospital, Victoria

CASE 3

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CASE 3 ARIEL WAS BORN AT 25 WEEKS GESTATION Ariel, aged 6 months, is the first child to Deborah and Marcus and was born at 25 weeks gestation weighing 700 g (Figure 7 ). Deborah and Marcus have noticed that he is having difficulty meeting his milestones. He does not roll over or sit unsupported and has difficulty sucking on a bottle. Nappies are hard to change because Ariel’s legs are very stiff. Deborah says that her pregnancy with Ariel was unremarkable until she was admitted with preterm labour at 25 weeks. There is no history of alcohol, nicotine or illicit drug use. Deborah says she was given a dose of steroids and something to stop the contractions but the labour progressed and she delivered Ariel vaginally a few hours later. You start to read the discharge summary from the hospital and glean the following facts about Ariel’s first days of life. t Amniotic fluid clear and Apgar scores 7 at 1 minute and 8 at 5 minutes t Resuscitation with drying and warmth and elective nasal continuous positive airways pressure (CPAP) at 7 cm H2O and 0.3 FiO2 t Transfer to special care nursery. Admission observations: – peripheral axillary temperature 36.5°C – heart rate 145 beats per minute – respiratory rate 70 breaths per minute t Day 1. Within 1 hour of arrival in special care nursery, development of audible grunting, subcostal retractions and nasal flaring with increasing acrocyanosis, oxygen

saturations 88% (right wrist) on CPAP. Examination unremarkable with clear lung fields and no audible murmurs on auscultation. Blood sugar level 2.0 mmol/L (bedside testing). Intravenous infusion of 7–8 mg/kg per hour of glucose started, electively intubated at 2 hours old and two doses of exogenous surfactant given in the first day of life. Chest X-ray showed low volume lungs, diffuse reticulogranular patterns and air bronchograms in the lung fields (Figure 8 ). Arterial blood gases showed hypercapnia (paCO2 >45 mmHg), acidosis (pH <7.2) and hypoxemia (paO2 <50 mmHg). Haemoglobin level was 15 g/L. Diagnosis of the cause of respiratory distress was hyaline membrane disease (HMD) or respiratory distress syndrome t Day 2. Responded well to surfactant therapy and extubated after 24 hours. Required reintubation 6 hours later because of a profound apnoeic episode. Haemoglobin level was 8 g/L. Head ultrasound showed bilateral intraventricular haemorrhages with ventricular dilatation (Figure 9 ) t Day 3–45 weeks corrected gestation. Progressive ultrasounds showed resolution of the haemorrhages but cystic lesions developed at 36 weeks corrected gestation (Figure 10). After the subsequent reintubation on day 2, ventilatory support needed for a further 3 weeks. Chest X-ray consistent with chronic lung disease (Figure 11). Required dexamethasone at age 4 weeks in order to be extubated. Remained on CPAP for many weeks and was finally weaned onto nasal cannula oxygen at age 39 weeks corrected gestation. Diagnosis chronic lung disease (CLD). Discharged at 45 weeks corrected gestation on 0.5 L nasal cannula oxygen, which is still required.

QUESTION 1 What causes hyaline membrane disease (HMD)?

Figure 7. Ariel soon after his birth at 25 weeks gestation

CASE 3

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QUESTION 2 Why is normoglycaemia important for preterm infants?

Figure 8. Ariel’s Chest X-ray demonstrating the features of HMD including low volume lung fields and air bronchograms

QUESTION 3 What is the likely cause of Ariel’s presenting problems today?

Figure 9. Ariel’s head ultrasound showing bilateral intraventricular haemorrhages with ventricular dilatation

QUESTION 4

Figure 10. Ariel’s head ultrasound showing periventricular leukomalacia, cystic lesions adjacent to the ventricular margins

Figure 11. Ariel’s chest X-ray demonstrating the features of CLD or bronchopulmonary dysplasia. These include hyperexpanded lung fields and areas of collapse and consolidation

What is your management plan for the further assessment and management of Ariel’s presenting symptoms? How will you explain the diagnosis to Deborah and Marcus?

CASE 3

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QUESTION 5 What types of treatment and care is Ariel likely to need as he grows up?

QUESTION 6 What is chronic lung disease (CLD)?

QUESTION 7 When should Ariel be immunised? Are there any contraindications to immunisation?

QUESTION 8 Should Ariel receive prophylactic immunisation against respiratory syncytial virus (RSV)? If so, when?

CASE 3 ANSWERS ANSWER 1 Hyaline membrane disease is caused by surfactant deficiency. Surfactant is vital to decrease airway surface tension and is not manufactured, stored or released in adequate amounts by the type II alveolar cells until approximately 34 weeks gestation.15 Prenatal administration of steroids (betamethasone) to the mother at least 24 hours before birth hastens surfactant synthesis and release, and significantly decreases the incidence and severity of HMD.16,17 Prenatal steroids also decrease the severity and incidence of other complications of prematurity such as necrotising enterocolitis and intraventricular haemorrhage. Surfactant replacement therapy via the endotracheal tube has also markedly decreased the severity and complications (eg. air leaks) caused by HMD. ANSWER 2 When treating preterm infants, neonatal paediatricians now aim for a higher blood sugar level (a minimum of around 2–2.5 mmol/L) than they did in the past, as cerebral compromise is now increasingly noted at what was previously thought to be ‘adequate’ blood sugar levels. Normoglycaemia is vital for cerebral metabolism and premature infants are at higher risk of hypoglycaemia and its effects because of lower brown fat stores and a higher need for glucose.18 For instance, exposure to cold, and nursing an infant in an environment outside their neutral thermal zone (the temperature range where energy expenditure is least) will increase cold stress and risk of hypoglycaemia.19 ANSWER 3 It is likely that Ariel has cerebral palsy, a static encephalopathy that can result from many causes. The most likely aetiology in Ariel’s case is ventricular haemorrhage with the development of cystic lesions (Figure 10 ). Ariel’s apnoeic episode and significant decrease in haemoglobin levels are clues to this diagnosis. In some cases, ventricular haemorrhage results in death or ventricular cerebrospinal fluid obstruction, leading to hydrocephalus and the need for surgical intervention with shunts. Having a shunt can increase morbidity as children may require shunt changes due to infection and growth. The cystic changes evident on Ariel’s ultrasound at 36 weeks gestation represent cavitation of hypoxic brain matter and are most common in the watershed areas of the brain around the ventricles. It takes about 4–6 weeks for the lesions to manifest themselves on ultrasound and the cysts sometimes coalesce so may not be identified on later imaging. ANSWER 4 Ariel should initially be referred to a general paediatrician if there is concern about his neurodevelopmental progress. This will be a frightening time for the parents and it is worthwhile explaining that Ariel will need further comprehensive assessment by a team specialised in caring for infants with problems such as this. Early

CASE 3

diagnosis and treatment of problems are paramount to optimising the outcome for Ariel because necessary therapies can then be started as soon as possible. A high risk infant such as Ariel should have already been linked into formal follow up programs coordinated by the perinatal centre of birth, however, there may be occasions when the chain of follow up may need to be reinstated. The general paediatrician will then most likely refer Ariel to a coordinated follow up clinic in the closest children’s or tertiary perinatal hospital where there are multidisciplinary teams to assess Ariel, including developmental paediatricians, occupational therapists, physiotherapists, speech pathologists. The general paediatrician is an essential cog of this referral system as they are required to coordinate Ariel’s follow up care. ANSWER 5 A child with cerebral palsy is likely to have problems with feeding, physical growth and development, as well as with learning disabilities, vision and hearing. Children with cerebral palsy are also at a greater risk of abuse and neglect, probably because of parental stress. The management of a child with cerebral palsy requires multidisciplinary coordination between many specialties, such as physicians, occupational and physical therapists, speech pathologists, social workers, educators and developmental psychologists.20,21 Ariel’s care may involve:22–25 t provision of assistance with feeding, dressing, bathing and playing in ways that limit the effects of abnormal muscle tone t stretching exercises to prevent the development of contractures t provision of adaptive equipment, such as walkers, poles, standing frames and motorised wheelchairs t use of symbols, talking typewriters and specially adapted computers to enhance communication skills t identification and management of behavioural problems. These issues may substantially interfere with the development of the child and referral to a psychologist or psychiatrist may be necessary t ophthalmological reviews to assess for and treat problems such as strabismus, nystagmus and optic atrophy t medications to treat spasticity, including dantrolene sodium, benzodiazepines, baclofen and botulinum toxin. These are best administered under the guidance of a paediatric rehabilitation specialist t advice and support from social services. It is important to consider financial limitations, especially if one parent has to forgo employment to care for the child or there is a need for expensive modifications to transportation or household structures. ANSWER 6 Chronic lung disease is a syndrome of chronic respiratory insufficiency predominantly affecting preterm infants. These infants continue to require supplemental oxygen and/or ventilatory support

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beyond a corrected age of 36 weeks gestation. Chest X-rays often show areas of collapse, consolidation and hyperexpansion. Infants with CLD are more at risk of reactive airways disease and respiratory infections. Chronic lung disease may also be associated with poor physical and neurological development. The rehospitalisation rate for respiratory illness of patients with CLD during the first 2 years of life is approximately twice that of infants of a similar gestation without CLD, and ongoing respiratory symptoms such as tachypnoea, rib retraction, dyspnoea, cough and wheezing may occur for years.26 ANSWER 7 Ariel should be immunised according to the recommended schedule for term infants at the usual chronological age (see Resources). Consider the influenza vaccine and prophylaxis against RSV (see Answer 8 ). ANSWER 8 Respiratory syncytial virus infection can cause acute respiratory distress syndrome (ARDS) which is associated with significant morbidity and mortality. Children with CLD, cyanotic congenital heart disease, cystic fibrosis, immune suppression and those of indigenous origin in all races are particularly at risk. Respiratory syncytial virus infection has also been associated with the development of chronic obstructive airways diseases such as asthma. Palivizumab, a monoclonal antibody directed against RSV, has been shown to decrease the risk of hospitalisation of children in developed countries.27,28 Palivizumab is given during the RSV season, which is during the winter months in temperate areas or during the rainy season in tropical areas. It should be considered especially in preterm survivors who continue to require home oxygen. Extremely premature infants with bronchopulmonary dysplasia or those younger than 28 weeks who have had protracted oxygen therapy in the nursery, derive the maximum economic benefit from RSV prophylaxis.29 However, palivizumab is relatively expensive at AUD$795 per 50 mg vial and AUD$1456 per 100 mg, and infants require 15 mg/kg intramuscular every month during the RSV season for up to 5 months for prophylaxis.30 Parents also need to be educated about simple measures such as frequent hand washing which may be effective in preventing the spread of RSV.

CASE 4

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CASE 4 DOES OLIVIA HAVE A FOOD ALLERGY? Olivia, a girl aged 8 months, is brought in by her mother Anita with intermittent vomiting and loose stools. Olivia was born by normal vaginal delivery at 38 weeks gestation. Anita says she showed some irritability during the first few weeks of life and that she vomited after most feeds. However, she continued to gain weight. At that that time she attended for review and your colleague recommended regular monitoring of her weight and height by the maternal and child health nurse and prompt review of any changes. Until 6 months of age, Olivia was exclusively breastfed, with her mother on an unrestricted diet which included cow’s milk. At 6 months, Olivia was weaned from the breast onto cow’s milk formula and solids were introduced including fruit, vegetables, rice and wheat. She subsequently developed increasing loose bowel actions and poor weight gain. The vomiting has settled but the irritability has continued. There is no recent history of acute gastroenteritis. Family history includes maternal coeliac disease and atopy and a sister with eczema. You examine Olivia and find that both her weight and length are on the 25th percentile for her age. There were no other abnormalities on physical examiniation. According to her ‘blue book’, at birth she was on the 50th percentile for both weight and length.

QUESTION 1 What differential diagnoses would you consider given the above case history?

QUESTION 2 What will you do next?

FURTHER INFORMATION Olivia’s symptoms of diarrhoea and irritability resolved on replacing cow’s milk with soy formula and recurred upon rechallenge with cow’s milk. Her test results revealed: t negative antideaminated gliadin peptide IgG and antitissue transglutaminase IgA (coeliac serology) t negative coeliac genetic susceptibility test (HLA DQ2,8) t faecal pH 4.5 (reference range: 5.0–5.5 in infants) t stool positive for reducing substances. QUESTION 3 What is the diagnosis and how will you manage Olivia now?

CASE 4

CASE 4 ANSWERS

ANSWER 1 The important differential diagnoses to consider in Olivia are outlined below. Coeliac disease This is important to consider as Olivia’s symptoms deteriorated at a time when wheat was introduced into the diet. Coeliac disease is a chronic, autoimmune enteropathy of the small intestine occurring in genetically predisposed individuals, characterised by lifelong intolerance to gluten and related prolamins.31 Patients classically present with chronic diarrhoea, anorexia, abdominal distension, weight loss, vomiting, irritability and lethargy. Suspicion should be increased in those with a family history and in children with coexistent disorders including type 1 diabetes mellitus, Down syndrome and Turner syndrome. Lactose intolerance This results from lactose malabsorption, which is associated with symptoms of bloating and abdominal cramps. Primary lactase deficiency which occurs from birth is rare and presents in the first few weeks of life with severe diarrhoea. Secondary lactose intolerance can be either temporary or permanent. The temporary form occurs following damage to the gastrointestinal mucosa from a variety of aetiologies including acute viral gastroenteritis, giardiasis, coeliac disease and non-IgE mediated cow’s milk allergy and is reversible upon correction of the underlying pathology. Irreversible secondary lactose intolerance occurs most commonly in those descended from Asian and African populations. Approximately 20% of Hispanic, Asian, and black children younger than 5 years of age have evidence of lactase deficiency and lactose malabsorption.32 Non-IgE mediated cow’s milk allergy This is important to consider as Olivia’s symptoms deteriorated at a time when cow’s milk formula was introduced at age 6 months. Another clue is the early symptoms of irritability when breastfed while the mother was still ingesting cow’s milk in her diet. Non-IgE mediated cow’s milk allergy is distinct from IgE mediated cow’s milk allergy both in terms of underlying mechanism of disease and presenting symptoms.33 The former is associated with delayed symptoms (sometimes hours after ingestion) referrable to the gastrointestinal tract (including diarrhoea and vomiting or rectal bleeding). By contrast, IgE mediated cow’s milk allergy presents with an acute allergic response (usually within 30 minutes of ingestion) to small amounts of dairy (usually less than 20 mL) with symptoms that include generalised urticaria, facial angioedema, vomiting and in severe cases respiratory or cardiovascular compromise consistent with anaphylaxis. An IgE mediated cow’s milk allergy can be confirmed in those with a convincing history by measuring serum IgE antibodies to cow’s milk (CAP FEIA test).

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FEEDBACK This child’s initial presentation may have been secondary to mild gastro-oesophageal reflux disease (GORD). This occurs when gastric contents reflux into the oesophagus or oropharynx secondary to transient relaxation of the lower oesophageal sphincter.34 In infants, symptoms usually become apparent in the first few months of life, peaking at age 4 months, before resolving by 2 years of age. Initial management strategies for GORD can be instituted. These include upright positioning of the infant after feeds and thickening of feeds. Suspected GORD that is resistant to these initial measures should be referred to a paediatric gastroenterologist for consideration of further investigations, including intraoesophageal pH monitoring and gastroscopy. A trial of proton pump inhibitor therapy in infants with GORD can be considered, although randomised controlled trials supporting its use in paediatrics is still lacking.34 Gastro-oesophageal reflux disease can also be a presenting symptom of non-IgE mediated cow’s milk protein allergy (see Answer 1 ). ANSWER 2 It is important to rule out coeliac disease in this infant by ordering coeliac serology. Coeliac serology has a high sensitivity and specificity and should be performed while the child is regularly ingesting wheat (equivalent to 1–2 slices of bread per day). Given the suggestive symptoms and positive family history, it is worth ordering a coeliac genetic susceptibility test (HLA DQ2,8). The definitive diagnosis of coeliac disease is made via small bowel biopsy. To exclude lactose intolerance, check the stool for reducing substances and faecal pH (which may be decreased due to the formation of volatile fatty acids following carbohydrate malabsorption). The diagnosis of non-IgE mediated cow’s milk allergy can be easily established via a strict 2 week elimination diet of cow’s milk (and dairy replacement with soy formula or a hypoallergenic extensively hydrolysed formula if intolerant to the former35) followed by a rechallenge with cow’s milk. ANSWER 3 The presenting symptoms are consistent with non-IgE cow’s milk allergy with secondary reversible lactose intolerance. Olivia should be started on a soy formula and soy products in the place of cow’s milk and have her weight monitored regularly. She should be challenged to cow’s milk at home every 6 months and monitored for recurrence of symptoms. FEEDBACK Olivia regained percentiles and at 2 years of age she was tolerant to a cow’s milk challenge. She is now on an unrestricted diet. In approximately 50% of children with cow’s milk allergy, the condition will resolve by age 2.

CASE 5

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CASE 5 JOSH HAS HAD A FIT Josh, a boy 3 years of age, is brought in to see you at 4.00 pm on a Friday by his mother, Jane. Jane is very distressed because she says Josh had a fit at home, and this has never happened before. Josh looks flushed, but alert. Jane says Josh was fine this morning and went to child care as usual. When Jane picked him up, the carer reported that he’d played happily enough and had a snack, but had ‘not been quite himself’. At 3.00 pm, he was sitting at the table at home doing some drawing, when he suddenly stopped drawing. Jane says his eyes seemed to become glazed and roll back, and his arms and legs started shaking. This lasted for 90 seconds, and then he became limp, and Jane lay him down on his bed. He slept for 10 minutes, and then woke up crying. Jane says he is now ‘pretty much back to his normal self’, but is a bit quieter than usual. Josh is a previously well, developmentally normal boy who has had all of his routine immunisations. There is no family history of epilepsy. On examination, Josh is sitting quietly in Jane’s lap. He is alert and cooperative and his cheeks are flushed. He has a tympanic temperature of 38°C, is well hydrated, has no neck stiffness, and he’s not bothered when you shine a torch in his eyes. His pupils are normal and there is no focal neurology. He has some clear coryza, bilateral cervical lymphadenopathy and a red throat with large inflamed tonsils and no exudate. Both tympanic membranes are injected. His chest is clear.

QUESTION 3 What investigations are required at this stage?

QUESTION 4 What treatment is required?

QUESTION 5 What will you recommend to Jane regarding fever control?

QUESTION 1 What is the likely cause of Josh’s seizure?

QUESTION 6 What is the likelihood of Josh having further seizures with this illness and with subsequent illnesses? What is the risk of him having an afebrile convulsion? QUESTION 2 What is the likelihood that Josh has meningitis?

CASE 5

QUESTION 7 What follow up is required? Should an electroencephalogram (EEG) or imaging be arranged?

CASE 5 ANSWERS ANSWER 1 Josh has had a typical simple febrile convulsion in the setting of presumed viral pharyngitis/tonsillitis. A simple febrile convulsion is a brief (less than 10 minutes duration) generalised convulsion in a febrile (more than 38°C) child aged between 6 months and 6 years, with no previous afebrile seizures, no progressive neurological condition and no signs of a central nervous system infection. Febrile convulsions are common, occurring in 3% of healthy children between the ages of 6 months and 6 years. They are usually associated with a simple viral infection. The onset of the convulsion may be sudden with little evidence of preceding illness, as was the case in Josh. ANSWER 2 Meningitis should be considered in any child who presents with fever and fitting. The risk of bacterial meningitis in a child who has fever and a convulsion lasting less than 10 minutes is between 0.5 and 2%. The risk is higher for those with prolonged convulsions, rising to 17% for convulsions that last longer than 30 minutes. Based on his clinical presentation it is very unlikely that Josh has meningitis. He is relatively well, is alert and cooperative and has no neck stiffness. ANSWER 3 In any child who has had a seizure, it is important to check a blood sugar level as hypoglycaemia is a readily treatable cause of seizures. Apart from this, investigations should be directed at determining the cause of the fever. In Josh, the focus is almost certainly viral pharyngitis/tonsillitis, and no further investigations are required. If there is no clear focus, check urine for an infection and consider blood tests and a lumbar puncture if the child is unwell. A lumbar puncture is strongly recommended in all children aged younger than 6 months, and in older children who have prolonged seizures or any clinical signs suggestive of meningitis. ANSWER 4 Josh needs no treatment apart from supportive care. He has a viral illness, and so does not need antibiotics. His parents will need

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education about febrile convulsions, including first aid management and long term expectations. ANSWER 5 In general, fever does not require treatment, particularly if the aim is simply to lower the temperature. Paracetamol or ibuprofen can be used to alleviate pain or discomfort, which in Josh’s case may be associated with tonsillitis or perhaps myalgia or arthralgia. With respect to febrile convulsions, paracetamol has not been shown to reduce the risk of further febrile convulsions. Indeed, no measure to control fever has been shown to do so. ANSWER 6 Repeated convulsions during the same illness occur in about 10–15% of children. There are usually no serious implications of these but these children should be reassessed in hospital as a period of observation may be required. The recurrence rate for febrile convulsions in subsequent illnesses depends on the age of the child. The younger the child at the time of the initial convulsion, the greater the risk of a further febrile convulsion (age 1 year: 50%; age 2 years: 30%). At 3 years of age, Josh’s risk of recurrent febrile convulsions is approximately 20%. The risk of future afebrile convulsions (epilepsy) is increased in a child with: t a family history of epilepsy t any neurodevelopmental problem t atypical febrile convulsions (prolonged or focal). Without these risk factors (as in Josh’s case), the risk of subsequent epilepsy is approximately 1% (similar to the population risk). With one risk factor, the risk is 2%, with more than one risk factor it is 10%. ANSWER 7 Febrile convulsions are frightening for parents, and follow up for further education and reassurance is advisable. However, there is no requirement for specific assessment, nor are any investigations indicated. Investigation of seizures in children is only warranted following multiple afebrile convulsions or in any seizure with complicating features such as duration longer than 10 minutes, focal symptoms, or focal signs on neurological examination following the seizure. Table 2 lists suggested investigation of seizures in children.

Table 2. Suggested investigation of seizures in children Seizure type

Investigation required

Simple febrile convulsion

Nil

First generalised afebrile seizure

Nil

Subsequent afebrile seizure

EEG

Any seizure (including febrile convulsion) with focal features

Urgent neuroimaging (CT or MRI)

Any seizure with focal features on EEG

Consider neuroimaging (CT or MRI)

CASE 6

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CASE 6 BABY EMILY IS WHEEZY

QUESTION 3 What treatment will you offer?

Emily, age 8 months, is brought to see you by her parents. She has had a ‘cold’ for a couple of days, with a fever and runny nose. Today she’s woken up with a cough and rapid breathing. Yesterday, she didn’t eat as well as she usually does. She refused to eat breakfast this morning, and she only drank half a bottle of formula. She has had a normal number of wet nappies. Emily’s brother, who is 3 years of age, has had a cold for the last week. His asthma played up during the illness, and he needed regular salbutamol for 3 days. On examination, Emily is miserable. Her (tympanic) temperature is 37.8°C, oxygen saturation 96% in room air and respiratory rate 50 breaths per minute. Capillary refill time is 2 seconds. She does not have any increased work of breathing but has a cough and audible wheeze and clear coryza. Both tympanic membranes are slightly red, as is her throat. She has some tiny cervical lymph nodes bilaterally. Examination of her chest reveals bilateral wheeze and crackles, but air entry is normal.

QUESTION 4 What will you suggest regarding Emily’s feeds?

QUESTION 1 What is the most likely diagnosis? FURTHER INFORMATION You explain the diagnosis and recommended management to Emily’s mother, and arrange to review her the following morning. On review, Emily is still unwell. She has had no solids and has had only half of her usual formula intake in the last 24 hours. She is still slightly febrile and has a respiratory rate of 60 breaths per minute. She has some intercostal retraction. Her chest has widespread wheeze and crackles. QUESTION 5 What is the most appropriate management now? QUESTION 2 What investigations, if any, would you order to confirm the diagnosis?

CASE 6

CASE 6 ANSWERS ANSWER 1 The most likely diagnosis is bronchiolitis. Bronchopneumonia is a possible differential diagnosis, though less likely. Bronchopneumonia is usually caused by a virus, and distinguishing it from bronchiolitis is not terribly important from a management point of view. The more difficult issue is whether or not this could be asthma. With a family history of asthma, it is possible that this is Emily’s first episode. However, a confident diagnosis of asthma can generally only be made if Emily has further similar episodes over time. ANSWER 2 There are no investigations necessary for infants with this clinical picture. A routine chest X-ray is not required for children with a typical clinical picture of bronchiolitis. A chest X-ray would typically demonstrate hyperinflation, peribronchial thickening, and often patchy areas of consolidation and collapse, but it is not diagnostic and does not influence management. A nasopharyngeal aspirate might identify the causative virus, but once again, is not routinely indicated, as it does not influence management. Respiratory syncytial virus is the most common pathogen (85%), but other organisms can cause bronchiolitis, including parainfluenza 3 and adenovirus. ANSWER 3 There is no specific treatment for bronchiolitis. Treatment is supportive and aimed at the complications (hypoxia and dehydration). t If the infant has oxygen saturations less than 92%, oxygen may be required t If there are signs of dehydration (prolonged capillary refill time, decreased tissue turgor or recent loss of weight) and adequate hydration can’t be maintained with oral fluids, nasogastric or intravenous fluids may be required t Paracetamol may be given for general malaise or discomfort t Other options – the question of whether to give a trial of salbutamol is difficult. This may be Emily’s first episode of asthma, especially given her family history, however, salbutamol is often ineffective in infants as they have little bronchial wall smooth muscle. There is no proven clinical benefit of salbutamol for bronchiolitis. Indeed, salbutamol may be associated with desaturation in infants with bronchiolitis.36 Other treatments, such as nebulised adrenaline and oral steroids, have been studied, but should not be used routinely. ANSWER 4 Emily has coryza and probably has some nasal obstruction. She also has some lower respiratory tract obstruction with tachypnoea, and may not be able to manage a full bottle. To ensure that she maintains her hydration, she should be offered smaller, more frequent feeds.

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Saline nose drops may be used to try and alleviate the blocked nose, particularly before feeds. These can be prepared by the parents using one-quarter of a teaspoon of salt in one-half of a cup of lukewarm water, and introduced using a dropper or small syringe. ANSWER 5 Emily now has moderately severe bronchiolitis and most likely needs admission to hospital. She should be sent to a (paediatric) emergency department for assessment. Indications for admission may include any one of: t poor feeding t lethargy t marked respiratory distress t oxygen saturation ≤90% t age <6 weeks t underlying cardiorespiratory disease.

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Turk AE, McCarthy JG, Thorne CH, et al. The ‘back to sleep campaign’ and deformational plagiocephaly: is there cause for concern? J Craniofac Surg 1996;7:12–8.

Kotrikova B, Krempien R, Frier K, et al. Diagnostic imaging in the management of craniosynostoses. Eur Radiol 2007;17:1968–78.

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Armstrong K, Previtera N, McCallum RN. Medicalizing normality? Management of irritability in infants. J Paediatr Child Health 2000;36:301–5.

Heine JB, Meehan RG, Catto-Smith AG, et al. Effect of antireflux medication, placebo and infant mental health intervention on persistent crying: a randomized clinical trial. J Paediatr Child Health 2006;42:49–58.

Heine RG, Jaquiery A, Lubitz L, et al. Role of gastro-oesophageal reflux in infant irritability. Arch Dis Child 1995:73;121–5, doi:10.1136/ adc.73.2.121.

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10. Barr RG, Konner M, Bakeman R, et al. Crying in Kung San infants: a test of the cultural specificity hypothesis. Dev Med Child Neurol 1991;33:601–10. 11. St James-Roberts I, Alvarez M, Csipke E, et al. Infant crying and sleeping in London, Copenhagen and when parents adopt a ‘proximal’ form of care. Pediatrics 2006;117:e1146–55, doi:10.1542/peds.2005– 2387. 12. Buist AE, Austin MV, Hayes B, et al. Postnatal mental health of women giving birth in Australia 2002–2004. Findings from the beyondblue National Postnatal Depression Program. Aust N Z J Psychiatry 2008;42:66–73. 13. Smart J, Hiscock H. Early infant cry and sleep problems: a pilot study of impact on parental wellbeing and parent-endorsed strategies for management. J Paediatr Child Health 2007;43:284–90, doi:10.1111 /j.1440–1754.2007.01060.

REFERENCES

22. Brunstrom JE. Clinical considerations on cerebral palsy and spasticity. J Child Neurol 2001;16:10–5. 23. Butler C, Darrah J. Effects of neurodevelopmental treatment (NDT) for cerebral palsy: an AACPDM evidence report. Dev Med Child Neurol 2001;43:778–90. 24. Yap R, Majnemer A, Benaroch T, et al. Determinants of responsiveness to botulinum toxin, casting, and bracing in the treatment of spastic equinus in children with cerebral palsy. Dev Med Child Neurol 2010;52:186–93. 25. Parkes J, McCullough N, Madden A, et al. The health of children with cerebral palsy and stress in their parents. J Adv Nurs 2009;65:2311–23. 26. Bhandari A, Panitch HB. Pulmonary outcomes in bronchopulmonary dysplasia. Semin Perinatol 2006;30:219–26. 27. Laughon M, O’Shea MT, Allred EN, et al. Chronic lung disease and developmental delay at 2 years of age in children born before 28 weeks’ gestation. Pediatrics 2009;124:637–48. 28. Doyle LW, Ehrenkranz RA, Halliday HL. Dexamethasone treatment after the first week of life for bronchopulmonary dysplasia in preterm infants: a systematic review. Neonatology 2010;98:289–296. 29. Numa A. Outcome of respiratory syncytial virus infection and a costbenefit analysis of prophylaxis. J Paediatr Child Health 2000;36:422–7. 30. Priyadarshi A, Jaffe A, Walls T, et al. Strategies for reducing the burden of respiratory syncytial virus in high-risk infants. Pediatr Health 2009;3:391–406. 31. Polanco I. Celiac disease. J Pediatr Gastroenterol Nutr [Review] 2008;47(Suppl 1):S3–6. 32. Heyman MB. Committee on lactose intolerance in infants, children, and adolescents. Pediatrics [Review] 2006;118:1279–86. 33. Allen KJ, Davidson GP, Day AS, et al. Management of cow’s milk protein allergy in infants and young children: an expert panel perspective. J Paediatr Child Health 2009;21:481–6. 34. Colletti RB, Di Lorenzo C, Colletti RB, et al. Overview of pediatric gastroesophageal reflux disease and proton pump inhibitor therapy. J Pediatr Gastroenterol Nutr 2003;37(Suppl 1):S7–S11. 35. Kemp AS, Hill DJ, Allen KJ, et al. Guidelines for the use of infant formulae for the treatment of cow’s milk protein allergy: an Australian consensus panel opinion. MJA 2008;188:109–12. 36. Ho L, Collis G, Landau LI, et al. Effect of salbutamol on oxygen saturation in bronchiolitis. Arch Dis Child 1991;66:1061–4.

14. Cox JL, Holden JM, Sagovsky R. Detection of postnatal depression: development of a 10-item Edinburgh Postnatal Depression Scale. Br J Psychiatry 1987;150:782–6. 15. Avery ME, Mead J. Surface properties in relation to atelectasis and hyaline membrane disease. AMA J Dis Child 1959;97(Part 1):517–23. 16. Ramanthan R. Surfactant therapy in preterm infants with respiratory distress syndrome and in near term or term newborns with acute RDS. J Perinatol 2006;26:S51–S56. 17. Crowley P, Chalmers I, Keirse MNJC. The effects of corticosteroid administration before preterm delivery: an overview of the evidence from controlled trials. BJOG 1990;97:11–25. 18. Koh TH. Glucose and the newborn baby: sweet justice? J Paediatr Child Health 1996;32:281–4. 19. Bell EF, Gray JC, Weinstein MR, et al. The effects of thermal environment on heat balance and insensible water loss in low-birthweight infants. J Pediatr 1980;96(Part 1):452–9. 20. Shevell MI. Current understandings and challenges in the management of cerebral palsy. Minerva Pediatr 2009;61:399–413. 21. Johnston MV. Cerebral palsy. In: Kliegman RM, Behrman RE, Jenson HB, et al, editors. Nelson textbook of pediatrics. 18th edn. Philadelphia, PA: WB Saunders, 2007;2494–6.

RESOURCES

PATIENT INFORMATION Information to help parents with the day-to-day decisions of raising children is available at www.raisingchildren.net.au. Information for parents about understanding and managing periods of increased infant crying is available at www.purplecrying.info. Information, resources and support for women suffering from perinatal depression and anxiety is available from the Post and Antenatal Depression Association at www.panda.org.au or 1300 726 306. Patient information on gastrointestinal illness in children including coeliac disease, infant colic and diarrhoea is available from The Gut Foundation at www.gut.nsw.edu.au. Information for parents on febrile convulsions is available from The Royal Children’s Hospital, Melbourne at www.wch.org.au/kidsinfo/ handout/index.php?doc_id=3722 and www.wch.org.au/kidsinfo/ factsheets.cfm?doc_id=9452. Information for parents on fever in children is available from The Royal Children’s Hospital, Melbourne at www.wch.org.au/kidsinfo/ factsheets.cfm?doc_id=5200. Information for parents on bronchiolitis is available from The Royal Children‘s Hospital, Melbourne at www.rch.org.au/kidsinfo/handout/ index.php?doc_id=3717. The book Baby Love by Robyn Barker, Pan Macmillan, 2009, provides up-to-date information for parents on the first 12 months of life. PROFESSIONAL INFORMATION Information on vaccination of preterm infants is available at www. health.gov.au/internet/immunise/publishing.nsf/Content/Handbookspecialrisk232. The Edinburgh Postnatal Depression Scale is available at www. beyondblue.org.au/index.aspx?link_id=103.885 and www. blackdoginstitute.org.au/docs/CliniciansdownloadableEdinburgh.pdf. The Royal Children’s Hospital, Melbourne’s Clinical Practice Guidelines on febrile convulsions are available at www.wch.org.au/ clinicalguide/cpg.cfm?doc_id=5132. The Royal Children’s Hospital, Melbourne’s Clinical Practice Guidelines on bronchiolitis are available at www.rch.org.au/ clinicalguide/cpg.cfm?doc_id=5157. Fetveit A. Assessment of febrile seizures in children. Eur J Pediatr 2008;167:17–7 is a useful article about febrile seizures in children. It is available at www.springerlink.com/content/ h71750333k231843. Zorc JJ and Hall CB. Bronchiolitis: recent evidence on diagnosis and management. Pediatrics 2010;125:342–9 is a useful article about bronchiolitis. It is available at http://pediatrics.aappublications.org/ cgi/content/abstract/peds.2009–2092v1.

check First 12 months of life

Comprehensive information on postnatal depression in women in Australia and New Zealand is available from Buist AE, Austin MV, Hayes BA. Postnatal mental health of women giving birth in Australia 2002–2004. Findings from the beyondblue National Postnatal Depression Program. This article can be found in the Australian and New Zealand Journal of Psychiatry 2008;42:66–73, or is available at http://informahealthcare.com/doi/ pdf/10.1080/00048670701732749. FURTHER READING Williams H. Lumps, bumps and funny shaped heads. Archives of Disease in Childhood Education and Practice 2008;93:120–8. Zitelli BJ, Davis HW, editors. Atlas of pediatric physical diagnosis. 5th edn. Philadelphia: Mosby Elsevier, 2007.

CHECK CATEGORY 2 QI&CPD ACTIVITY

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FIRST 12 MONTHS OF LIFE In order to qualify for 6 Category 2 points for the QI&CPD activity associated with this unit:

QUESTION 2 Your examination of baby James also reveals the presence of torticollis without a tumour. What is the initial management of torticollis?

t SFBE BOE DPNQMFUF UIF VOJU PG check in hardcopy or online at the gplearning website at www.gplearning. com.au, and

A. Refer the baby for a neck brace

t MPH POUP UIF gplearning website at www.gplearning. com.au and answer the following 10 multiple choice questions (MCQs) online

C. Prevent further deformity by careful positioning and recommend exercises for 5â&#x20AC;&#x201C;6 months to stretch the affected muscle

t DPNQMFUF UIF POMJOF FWBMVBUJPO If you are not an RACGP member, please contact the gplearning helpdesk on 1800 284 789 to register in the first instance. You will be provided with a username and password that will allow you access to the test. The expected time to complete this activity is 3 hours. Please note t 'SPN +BOVBSZ UIFSF XJMM OP MPOHFS CF B Category 1 activity (ALM) associated with check units. This decision was made due to a lack of interest in this activity. The RACGP apologises for any inconvenience caused by this change t %P OPU TFOE BOTXFST UP UIF .$2T JOUP UIF check office. This activity can only be completed online at www.gplearning.com.au. If you have any queries or technical issues accessing the test online, please contact the gplearning helpdesk on 1800 284 789.

QUESTION 1 Wendy brings in baby James, age 2 months; she is concerned about the shape of his head. On examination his head has a parallelogram shape when viewed from above, but no other dysmorphic features are evident. His developmental and general examination is normal. You confidently make a diagnosis of deformational plagiocephaly. Your next step is to: A. refer James to a developmental paediatrician for assessment

B. Administer botox injections into the affected sternocleidomastoid muscle

D. Refer for surgery E. Recommend the baby be put down to sleep in the prone position. QUESTION 3 You see baby Anna, age 4 months for routine immunisations. Her mother, Karen, who has no other children, tells you that Anna is fully breastfed and is growing normally according to the percentile charts completed by the maternal and child health nurse. Karen mentions that Anna is harder to settle for naps at her usual time than she was when younger, she stays awake for several hours at a time and cries a lot. The most likely explanation is: A. Karenâ&#x20AC;&#x2122;s milk supply has dropped and the baby is hungry B. Anna is learning how to manipulate Karen C. Anna is becoming overtired as she is staying awake for too long D. Anna has developed reflux E. Anna needs to start solids. QUESTION 4 Karenâ&#x20AC;&#x2122;s partner, Ian attends the following week and says that Anna is now settling more quickly after dropping one of her daytime naps, but he is concerned about Karen, who seems to lack confidence, cries easily and is irritable all the time. Her appetite is variable and she doesnâ&#x20AC;&#x2122;t seem to shower as often as she used to. He is finding that he doesnâ&#x20AC;&#x2122;t want to come home from work at the end of the day as he never knows what kind of chaos he is likely to find. What is the possible diagnosis in this situation? A. Karen has postnatal depression B. Karen is sleep deprived

B. organise a skull X-ray

C. Ian misses his old way of life and is becoming depressed

C. reassure Wendy that this problem is not sinister and can be helped by careful positioning of James while he is sleeping to avoid further pressure on the affected side of his head

D. The couple is having difficulty adjusting to life as parents E. Any of the above.

D. arrange paediatric orthopaedic review to look for other bony abnormalities E. recommend James be fitted with a moulding helmet.

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QUESTION 5 Melissa visits you with baby Alison, who was born at 25 weeks gestation after her mother went into preterm labour. Despite all efforts to stop the progress of the labour, and administration of steroids to Melissa during labour, Alison was born with respiratory difficulties and continues to need home oxygen at the chronological age of 6 months. Melissa is concerned about vaccinating her baby as she is so small. Vaccinations should be given:

QUESTION 8 Neuroimaging and/or electroencephalogram (EEG) may be recommended following a febrile convulsion if:

A. according to the recommended schedule for term infants at the usual chronological age

E. all of the above.

B. according to the recommended schedule for term infants with age adjusted for prematurity

QUESTION 9 You see baby Eloise, age 8 months, with her parents. Eloise’s brother has a cold which has set off his asthma. Eloise has had a bit of a cold for 2 days, with clear coryza, flushed cheeks and mild fever. Overnight she has developed a cough and rapid breathing, and today she is having trouble taking her breastfeeds due to a blocked nose. She is also refusing most solid food and drinks from a cup. Her nappies are still wet, although not as wet as usual. On examination she looks unwell, with a borderline temperature and respiratory rate of 50 breaths per minute at rest. Capillary refill time is 2 seconds. Her chest movements appear normal but she has an audible wheeze, slightly red tympanic membranes and very small cervical lymph nodes. Chest examination reveals bilateral wheeze and crackles but normal air entry. Which of the following is true?

C. only when Alison no longer needs oxygen D. with extra caution as Alison’s immune system is likely to be immature E. only to full term infants. QUESTION 6 Emily, aged 8 months, is brought in by her mother who has come to see you with some questions after a recent paediatrician appointment in which Emily was diagnosed with non-IgE cow’s milk allergy. Which of the following is true of cow’s milk allergy? A. It is always a lifelong condition B. Non-IgE mediated cow’s milk allergy presents with generalised urticaria, facial angioedema, vomiting and anaphylaxis in severe cases

A. this is not the first febrile convulsion B. focal signs were noted during the convulsion C. the convulsion lasted longer than 5 minutes D. the convulsion occurred at a temperature of only 38.5°C

A. It is important to distinguish between a diagnosis of bronchiolitis and bronchopneumonia B. Eloise should be fed smaller, more frequent feeds

C. The non-IgE mediated form can be identified by a 2 week elimination diet followed by a rechallenge

C. Milk should be withheld as it will increase the amount of mucus in her chest

D. Heating of cow’s milk destroys the allergenic protein

D. Paracetamol should not be given unless her temperature reaches 39°C

E. Cow’s milk consumed by a breastfeeding mother cannot affect her infant. QUESTION 7 Beth presents with her 2 year old son Benjamin 1 hour after an episode in which Benjamin, while playing quietly on the floor, fell down and started shaking all over for about 1 minute. He then slept for 10 minutes before waking but remained very quiet. Your examination reveals a temperature of 38°C, good hydration, signs of an upper respiratory tract infection, no neck stiffness and no light sensitivity. His neurological examination is normal. Which of the following statements is true following a diagnosis of simple febrile convulsion? A. An urgent lumbar puncture is needed to exclude bacterial meningitis B. Benjamin needs antibiotics to reduce the risk of further seizures C. Beth should give Benjamin regular paracetamol to keep his temperature down D. Benjamin has a 30% chance of a having febrile convulsion in a subsequent illness E. It is not necessary to check Benjamin’s blood sugar level.

E. A diagnosis of asthma should be made. QUESTION 10 On review the following day Eloise’s condition has deteriorated. Which of the following is not an indication for paediatric emergency department review? A. Hyperactivity B. Marked respiratory distress C. Age of less than 6 weeks D. Underlying cardiorespiratory disease E. Signs of dehydration.

6 reasons to be an RACGP member Advocacy Education Credibility e-health Collegiality Lifestyle Regular participation in continuing professional development is important in assisting GPs to maintain and improve the quality of care provided to patients. The RACGP has designed a membership package for Australian registered GPs that makes continuing professional development even easier and more cost effective for members. It includes free access to the RACGP QI&CPD Program*, check Program, gplearning online education and much more. For greater convenience, many of these services are delivered online.Â If you prefer face-to-face education, our Australia-wide workshops and annual conference, provide the perfect opportunity for hands-on professional development.

Visit www.racgp.org.au/ membership or call 1800 331 626 today. * not currently included in the membership package for retired, resident or intern members

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