INTRODUCTION

The examiner introduces you to Hannah, who is a 12-month-old girl. You are told that she spent the initial few months of her life in hospital and you are invited to examine her cardiovascular system.

CLINICAL SCENARIO

Hannah sits on her mother’s lap without any oxygen therapy and is not dyspnoeic at rest. You are surprised she is 12 months old as she looks small for her age. Her head appears narrow when viewed face on and a little long in the anteroposterior direction. You ask the mother to remove her T-shirt. As you approach Hannah she begins to cry. You attempt to console her with the small teddy bear attached to your stethoscope. This makes matters worse. You notice that she has multiple small scars on her hands and a small scar on the left side of her chest.

You do manage to feel strong peripheral pulses and there is no evidence of central cyanosis. Useful examination of the chest and precordium is impossible due to Hannah’s crying.

The examiner then asks you to comment on your findings thus far. What do you say?

INTRODUCTION

On entering the room you are told, ‘This is Simon, who is a 16-year-old boy. Please examine his abdomen’.

CLINICAL SCENARIO

On inspection Simon is a thin boy and seems short for his age. He is pale and has a full and plethoric face. Despite his neck and shoulder blades having generous amounts of adipose tissue his extremities look wasted in comparison.

There are no peripheral stigmata of liver disease. He has three abdominal scars: two oblique scars in the left and right hypochondrium and one suprapubic scar. You also notice a small 2 cm scar close to his right clavicle. On palpation you find a left pelvic mass approximately 10 cm in length. It is non-tender, non-mobile and quite firm. He has no hepatosplenomegaly.

What do you think this pelvic mass is?

How do your clinical findings fit together?

What complications of treatment should be monitored?

INTRODUCTION

The examiner says to you, ‘This is Abdi. He is 11 years old and was born in Somalia. He was first treated by the ENT surgeons 5 years ago and since then his mother has been concerned about his face. Please examine his fifth, seventh and eighth cranial nerves using the equipment provided’.

CLINICAL SCENARIO

Abdi sits happily with his mother. On the table next to Abdi there is a piece of cotton wool and a tuning fork. At first glance there is a mild asymmetry to his face. You find no abnormality when you use the cotton wool to assess the sensation of the ophthalmic, maxillary and mandibular branches of the fifth cranial nerve. When you ask Abdi to screw up his eyes, puff out his cheeks and smile there is obvious weakness of the muscles in the left side of his face. He is unable to move his forehead on the left side. You place a vibrating tuning fork on the centre of the Abdi’s head and ask him which side is louder. He tells you the left. You place the vibrating tuning fork next to his right ear and then place the base on the right mastoid process. He tells you it is louder when placed next to the ear. When repeated on the left side he tells you it is louder when placed on the bone, although you had difficulty finding the mastoid process due to scar tissue in this region.

Is there anything else you would like to examine?

How do you present your findings to the examiner?

What do you think may be the cause of these findings?

INTRODUCTION

On entering the room the examiner says to you, ‘This is Anthony, who is six. Have a look at his skin and then listen to his chest’.

CLINICAL SCENARIO

Anthony is an appropriate size for his age. On inspection of Anthony’s skin you see he has dry, erythematous patches of skin covering his trunk, face and the flexor surfaces of his limbs. There are excoriated areas with visible scratch marks.

He is comfortable at rest and there is no evidence of cyanosis. There is no recession or indrawing. He is not barrel chested. You commence your exam by having a look at his hands. You notice no clubbing. The examiner stops you at this point and tells you he asked you to listen to his chest.

Auscultation of the chest is normal with no wheeze. The expiratory phase is normal.

You present your findings as a child with atopic eczema and a normal chest examination.

You are asked if he has Harrison’s sulci. You are now concerned you are wrong but do not feel he has Harrison’s sulci and he definitely does not have any wheeze.

What do you say to the examiner?

You are asked to explain to his mother how to manage the skin complaint. How would you do this?

INTRODUCTION

On entering the room you are told, ‘This is Nicola and her mother. Nicola is 7 years old. I’d like you to have a chat with her and ask her mum any questions you think may be important’.

CLINICAL SCENARIO

At first glance Nicola is a well-grown 7-year-old girl. Nicola talks throughout the examination with great enthusiasm about numerous subjects but with immaturity of content. You suspect she may have learning difficulty and have a good look for any dysmorphic features. She does not have Down’s syndrome and you note blue eyes and small teeth for her age, although her lips are quite full and she has a slightly rounded end to her nose. As she talks it is apparent that she has below average intellect for an average 7-year-old.

What would you like to ask mum?

You are then asked to listen to her heart. She has a central sternotomy scar with keloid scarring. There are no other scars. Her heart sounds are normal.

How do these findings fit together?

What do you think the diagnosis is?

What else would you like to ask mum to aid your diagnosis in light of the cardiac abnormality?

INTRODUCTION

‘This is Helen and her mother. Helen’s mum was concerned about her speech development compared to other children. Before asking mother any questions what do you think of Helen’s developmental age?’

CLINICAL SCENARIO

Helen is sitting at a table on your entrance to the room. She has been given some bricks to play with and she is currently hitting two of them together. She is suspicious of your arrival and looks between you and her mother. You introduce yourself to her mother and then to Helen. She says something incomprehensible to her mother, who smiles at her. You approach the table and ask her name. Although difficult to understand, there is a definite attempt at her name: ‘Eywen’. She then gets up and walks, with no difficulty, to a box of toys. She finds a crayon and starts scribbling in circular motions on a red book. You are not sure she should be doing this so you remove the book, at which point she clearly says ‘No’. Realising time will be of the essence you ask what colour the book is. She frowns and returns to the table. She starts making a tower of bricks with little difficulty. The examiner asks if there is anything you would like to ask her mother. Mother says she has little difficulty in understanding her but the nursery has raised concerns. She appreciates her speech can be difficult to understand. At this points Helen’s five-brick tower crashes to the ground.

How can you assess Helen further?

Where should she be referred?

SETTING

You are the specialist registrar on a general paediatric ward.

SCENARIO

You have reviewed Laura, a 3-year-old who has been admitted for the fifth time this year with acute asthma. You are informed by the GP that no repeat prescriptions have been picked up for Laura in the past 6 months and that Avril, her mother, is a heavy smoker.

TASK

You are meeting with her mother Avril and need to address how she manages her asthma at home and specifically advise her on the risks of smoking.

SETTING

You are the specialist registrar on the neonatal unit.

SCENARIO

You are leading a teaching session for the unit’s medical students on examination of the newborn. You have been provided with a dislocated hip mannequin.

TASK

Instruct Craig, a fourth-year medical student, on the correct technique for the neonatal hip exam. You do not need to provide background epidemiological information about developmental dysplasia of the hip but if you have time you may check Craig’s understanding of what to do if an abnormal hip is discovered. The aim is for Craig to perform a professional, reproducible and effective hip examination on the mannequin in a role-play situation.

INFORMATION

You are the SpR in a general paediatric clinic. You receive the following letter from a local orthopaedic consultant. Please take a relevant history from the patient, Tamsin, and her mother:

After taking a history the examiner asks you:

DIAGNOSIS: PERSISTENT DUCTUS ARTERIOSUS

Every candidate has a fear that they will have little positive (or negative) to say to the examiner because they have been unable to examine the child properly. Even worse, they have examined the child properly and still have nothing to say! There are numerous learning points to remember in this type of station and if you remember a few you should always be able to give a professional answer.

1 APPROACH

As we all know from clinical practice, children of this age can be difficult to examine. They are often clingy and may have stranger anxiety. While examining centres try hard to enrol cooperative children they often get tired or can be unpredictable. It is this random nature that makes children so endearing to paediatricians. You should be comfortable with some distraction techniques for children from 6 months to 5 years. Here are some classics:

Always try to smile and appear unthreatening, despite the stress you are under. Children are unfortunately good at picking up on this. Never carry on regardless if a child becomes very distressed as this will in turn distress the examiner! If a child becomes uncooperative they will often direct you with an alternative plan. Remember there is a tick box in the exam for acknowledging an uncooperative child.

2 OBSERVATION

Before you begin your hands-on exam ask ‘mum’ to remove any clothing. It will be impossible to pass a station if you miss a scar and you cannot claim you have thoroughly looked unless the chest is exposed.

With a child of this age observation is absolutely vital as it should give you enough information to make a sensible list of possible diagnoses. In the above scenario we need to simply stand back and take a look for a minute.

We note that she sits without oxygen and is not cyanosed or dyspnoeic.

We look more carefully and notice she is small for her age, has plagiocephaly and on inspection of the dorsum of her hands has multiple scars, presumably from previous venepuncture.

As mum distracts her and removes her T-shirt you carefully inspect her chest and both axillae. Now you can see a healed left lateral thoracotomy incision. She has had surgery for either:

In the absence of a central sternotomy scar she has not had a BT shunt as she is pink. Remember, a BT shunt is a palliative procedure and will not reverse the cause of the cyanosis. PA banding is used to prepare the vessels for a Fontan procedure but again will not correct the cyanosis until a more definitive repair has been performed. This leaves (given that this is a cardiac station) only a PDA or coarctation repair as possibilities. We know her pulses are normal, although if the coarctation has been repaired then obviously you will be able to feel the femoral pulse. In this situation, if a classical repair has been used then the left radial pulse should be weak. Balloon dilatation of the coarctation is unlikely to show any discernible difference. So theoretically the child may have had an aortic coarctation or a PDA but we know she shows signs of being premature. Therefore, if Hannah became very distressed and uncooperative you would be able to say to the examiner:

In this way you should be able to pass the station without ever having heard a heart sound! Obviously this system is not so helpful if the primary lesion is a ventriculoseptal defect! But to give yourself at least a sporting chance it is vital you use all the clues you are given.

REMINDERS

Remember your caveat for the end of the cardiovascular examination?

CAN YOU …

Describe two common features of persistent ductus arteriosus? See page 164.

DIAGNOSIS: KIDNEY TRANSPLANT

Obviously it is vital to palpate the mass in this station, and only regular practice of feeling abdomens (easily overlooked in favour of the more classic chest exam) will help you do this. However, you need to know what the mass is to make sense of the station. The common pelvic masses – and note pelvic, not abdominal – are:

Simon has signs of a chronic disease (pallor, suboptimal growth, multiple surgery). This situation would fit very well with chronic renal disease and a kidney transplant. Simon had developed chronic renal failure secondary due to vesicoureteric reflux complicated by persistent and undertreated/missed urinary tract infection. He required peritoneal dialysis (hence two hypochondrial scars) and a short period of haemodialysis (hence the healed central line scar close to the clavicle) and was later successfully transplanted with a donor kidney. Easy when you know! You wouldn’t be able to tell the cause of the chronic renal failure in this case but must know the common causes and any potential features to look for.

It is useful to commit a list of investigations to memory as some are specific for renal problems and just saying, ‘FBC, U&Es, CRP’ etc. is unlikely to win you any favours.

Complications of chronic renal failure (or more relevantly features to look for and so avoid deterioration) are split between those of the disease or those of the surgical treatment (i.e., transplant).

Prior to the exam make sure you get to see a few children with renal problems as they are not uncommon in the exam. Be familiar with the appearance and location of peritoneal dialysis catheters and central lines used for haemodialysis. Renal nurses are a mine of useful information in this regard.

Remember, there is a significant psychosocial burden and counselling, family therapy and social service input may have to be sought.

At the end of your abdominal exam remember your caveat: ‘I would like to complete my examination by performing a blood pressure, urinalysis and plotting on a growth chart appropriate for age and sex’.

CAN YOU …

List the investigations you would perform in a child you suspect has chronic renal failure?

Pre-treatment	Treatment	Post-treatment PASS
Hypertension	Control medically: Beta-blocker (care asthma) Furosemide (care potassium) Must involve nephrologists	Dialysis – complications Peritonitis Volume depletion Dialysate leakage Transplant -complications Infection – particularly CMV Must prevent UTI Rejection Immunosuppression
Metabolic acidosis	Sodium citrate solutions (care: sodium may worsen hypertension)
Growth failure	Growth hormone
Renal osteodystrophy	Dietary phosphate restriction Phosphate binders Vitamin D
Uraemic symptoms (anorexia, nausea and lethargy)	Dietary protein restriction
Anaemia	Erythropoietin
Seizures (hypertensive or electrolyte imbalance)
If poor concentrating power:
Polyuria
Salt wasting

REMINDER

DIAGNOSIS: PREVIOUS MASTOIDECTOMY

Severe, untreated otitis media may present with a facial nerve palsy. It is uncommon in the United Kingdom (but not unheard of) due to the use of antibiotics. In fact there is a feeling in some countries that mastoiditis is on 32 the increase as GPs use antibiotics less for otitis media.

Although we rarely use tuning forks in clinical practice it is one of those things that occasionally comes up in the exam and can flummox candidates if you don’t refresh your memory. Tuning fork tests are based on the principle that sound should travel better through air than through bone in the absence of hearing impairment. Weber’s test is performed by placing a vibrating 512 Hz tuning fork on the vertex of the skull. If the patient, when asked, can hear the sound louder on one side compared to the other then this is abnormal. The side the sound is localised to indicates conductive hearing loss on that side or sensory hearing loss on the contralateral side. Rinne’s test involves placing a vibrating fork next to the pinna (testing air conduction) and then behind the pinna on the mastoid process (testing bone conduction). If the sound is heard better through air than through bone then it is said to be Rinne’s positive and vice versa. Rinne’s test is positive in sensorineural hearing loss or normal hearing and negative in conductive hearing loss.

In this case:

One word of warning – and ignore this if you think it will confuse you: with the above findings it is still possible that there is a sensorineural or perceptive defect on the right side. This is because Rinne’s may be positive on the right because of a sensorineural deficit (and not due to normal hearing). The Weber’s lateralises to the left because there is some bone conduction on the left side. It would be unlucky to have had mastoiditis on the left side and have a sensorineural deafness on the right. You should be aware of this if asked, although it is probably best not to volunteer that information.

It is important to be able to quickly examine different cranial nerves. You are unlikely to be expected to perform an examination of all the nerves due to time constraints. Practise, practise, practise, as being slick under pressure is important and the cranial nerve exam is easy to perform on housemates.

Bell’s palsy is an easy condition to bring to the exam. There is some variation in textbooks as to recovery but as a general rule:

You must remember that methylcellulose eye-drops (artificial tears) need to be prescribed and the eye may need to be taped at night. The use of steroids depends on local guidelines but a short 2 mg/kg course is often recommended.

CAN YOU …

Talk through your history and examination of the child you suspect may have a Bell’s palsy?

DIAGNOSIS: ATOPIC ECZEMA

The moral of this story is: stick to your guns! The patient in this case does have atopic eczema and mild asthma. He is currently well, however, and has no clinical signs of asthma. Never make up clinical signs as it will land you in a whole heap of trouble. The examiners want to see safe, reliable doctors and not imaginative ones! They need to be confident that when phoned at 3 o’clock in the morning you have not made up signs to fit a given clinical picture. If you think you may have difficulty saying there are no clinical signs when you think there should be, then you can always acknowledge this:

Each centre will have a variety of patients with a variety of clinical signs. Not all of them will be obvious or even present at the time of the exam. Be prepared for this. Also, make sure that you read the question. In the above question you were asked to listen to the chest (as opposed to examining the respiratory system). Don’t waste the examiner’s time or your own by starting at the hands. The examiner will probably have other tasks he wants you to perform.

You should have a sound understanding of the management of basic paediatric problems that you are likely to see in clinic as an SpR, such as eczema and asthma.

Ezcema

Diagnosis	May present in infancy onwards
	Family or personal history of atopy
Management: non-medical	Switch to a non-biological washing powder
	Trim fi ngernails
	Use mittens in the persistent child
Management: medical	Emollient: Apply generously. Examples include 50:50 paraffin.
	Avoid soap and use bath oils such as Oilatum. Some bath oils include antiseptic, such as Emulsiderm
	Steroid cream: Use sparingly on troublesome areas. Be wary of using too high a strength. 0.5–1% hydrocortisone on the face or on babies.
	Stronger steroid creams include: • Eumovate: Moderately potent • Betnovate: Potent • Dermovate: Very potent

  Creams can be used on the body of older children if only for short periods of time. The fl exures are prone to atrophy with prolonged treatment. Oral steroid (prednisolone) has a role in diffi cult or acute eczema   Topical tacrolimus ointment: A newer treatment. 0.03% cream may be suitable in children where topical steroid has not worked or they are at particular risk of steroid skin atrophy.   Normally initiated by a dermatologist   Wet wraps: Potassium permanganate soaks may be used or ichthammol bandages over steroid and emollient held in place by Tubigrip. These are changed every 1–3 days   Antimicrobials: Systemic antibiotics generally preferred if indicated. Intravenous antibiotics are sometimes indicated in more severe cases of infected eczema. Herpetic eczema is an emergency requiring treatment with acyclovir   Immunosuppressants: These may be required in severe refractory eczema under the guidance of a dermatologist.   Ciclosporin and azathioprine are the more commonly used drugs of choice

DIAGNOSIS: WILLIAMS’ SYNDROME

This question is another example of the need to be versatile. You may be asked to perform small, separate tasks. In this question it is important to be able to interact well with a child and think on your feet. Don’t be thrown by simple questions such as ‘Look at this. What do you think?’ – a difficult skill to revise for but may be practised by asking friends to pretend to be syndromes and feed you snippets of their features until you recognise them. You may not be able to spot the particular feature in the exam but you will start to learn certain patterns of abnormality.

We learn quite quickly from Nicola that she has some intellectual impairment and soft dysmorphic features, so any number of general questions may be appropriate.

We then see that Nicola has evidence of previous cardiac surgery with no residual murmurs. It is impossible to say what surgery she has had, although the absence of thoracotomy scars indicates that a shunting procedure was unnecessary.

So … dysmorphic features; intellectual impairment; cardiac problems. There are a few syndromes that may have this collection of difficulties:

(Tuberous sclerosis and neurofibromatosis do not have cardiac defects as part of their clinical and associated features.)

The other clue we have above is her very happy, chatty demeanour.

This is characteristic of Williams’ syndrome (and Down’s but the phenotypic features are not present). In the case of Williams’ the textbooks would describe it as ‘cocktail party chatter’ but do not expect this to be obviously apparent. There is no substitution for actually having seen the condition described as opposed to having simply read about it.

REMINDER

Williams’ syndrome

Facial	Systemic	Clinical
Prominent lips	Hypoplastic nails	Transient neonatal hypercalcaemia
Blue eyes	Supravalvular aorticstenosis	Mild IUGR
Microdontia	Pulmonary artery stenosis	Mild microcephaly
Snub nose	Renal artery stenosis	Mild learning difficulties
Medial eyebrow flare
Stellate pattern to iris
Short palpebral fissures

DIAGNOSIS: ISOLATED SPEECH DELAY/SPEECH DIFFICULTY

As is emphasised repeatedly in the book, it is vital to have a fluid understanding of key developmental stages. You should be able to pick out the following features:

Although the station implied delayed speech and language, the question demanded a complete developmental age assessment. Without doing any formal assessment the above has already given you a good framework in which to calculate any potential delays. In this brief segment you see a child who is probably 18–24 months with a potential speech and hearing delay.

However, it is likely the child has a good vocabulary and the problem is with articulation. You can test this by asking her to point to and then say the name of well-recognisable objects.

When listening to speech it is important to appreciate the difference between:

It is important to exclude factors such as poor input at home. This would suggest emotional deprivation or simply that parents do not or are unable to speak to their child regularly at home. Bear in mind autism, cerebral palsy or generalised developmental delay.

It is also important to remember that language problems may be receptive or expressive. Receptive language develops earlier and may begin to become appreciable from the age of 9 months. By the first year of life the child may understand 20–50 words. There is a sudden acceleration in both components from approximately 18 months. An expressive disorder may be present when the child’s vocabulary is actually quite good. A child may be able to point to multiple named objects but not able to say them.

It is difficult to know if Helen’s speech problems are part of a normal spectrum of delayed adequate phonation or represent a specific mechanical problem. In the absence of other features of developmental delay it would be most appropriate to refer this child to a speech and language therapist or health visitor. It may be that a speech and language therapist will delay active therapy until the child is older, however.

CAN YOU …

Examine the ninth, tenth, eleventh and twelfth cranial nerves?

(Look away now and talk through your examination with a colleague or in the mirror. Don’t cheat!)

A thorough working knowledge of cranial nerve exam is assumed but the above has been included as revision tends to concentrate on the earlier nerves!

REMINDER

You may remember the term ‘non-lexical utterances’ from communication skills at medical school. These are small noises or prompts, which are not true words, that act as an encouragement to the person you are speaking to. They are often associated with nodding motions of the head or arm gestures. Examples include ‘Mmmmm’, ‘Ahhhh’, ‘Uh-huh’. Some candidates will use them almost subconsciously.

DDH

REMINDER

The juvenile arthritides are an easily confusing group of conditions.