INTRODUCTION

On entering the station you are presented with a girl approximately 9 years old. You are told, ‘A GP has referred this child because he thinks she has a heart murmur. What do you think?’.

CLINICAL SCENARIO

The girl is comfortable at rest. You commence your routine cardiovascular examination. There are no abnormalities until you commence auscultation of the precordium. You hear a systolic murmur at the lower left sternal edge but also, and if not louder, in the aortic and pulmonary areas. You are uncertain as to whether this is an ejection systolic or pansystolic murmur. You feel a thrill in the suprasternal notch. You find no evidence of heart failure and can find no scars.

Where must you examine in order to clinch your diagnosis?

If this is positive what else will you look at?

Which syndromes are linked to this cardiac abnormality?

INTRODUCTION

On entering the station you are given the instruction, ‘Please examine this 16-year-old boy’s abdominal system, paying close attention to his nutritional status. Why do you think he has a scar?’.

CLINICAL SCENARIO

The boy looks smaller than you would expect for his age and has a gaunt appearance. Peripheral examination potentially shows some wasting of the hands, his conjunctivae are pale but his mouth is free from ulcers. You note that his muscle bulk looks reduced and offer to test this. There is a large laparotomy scar across his stomach but apart from some mild tenderness in the left iliac fossa abdominal examination is unremarkable.

How do you test his muscle bulk?

What else will you ask for and examine?

How do you present this patient?

INTRODUCTION

On entering the station you are presented with an African girl approximately 10 years old with an obvious movement disorder. You are asked to comment on her appearance and the abnormal movements.

CLINICAL SCENARIO

The child appears agitated and has persistent jerking movements of her arms. They move in an uncoordinated fashion, obviously to the girl’s distress. She is unable to keep her hands held together. Occasionally she demonstrates some facial grimacing.

How will you approach your examination?

What important differentials should you be aware of?

You are told she was a normal child 3 months ago and there is no family history of metabolic or neurological disorder. What investigations would you consider?

INTRODUCTION

On entering the room you are presented with a child approximately 5 years old. You are asked to examine his respiratory system to discover why he is coughing so much.

CLINICAL SCENARIO

The child is sitting on his mother’s knee. You ask to move him to the examining couch but his mother is uncertain as to whether he will cooperate. You explain that you would like at least to try. As soon as he is lifted towards the bed he starts crying. You quickly ask the mother to sit him on her lap and explain to the examiner that it may be easier to examine the child while he is settled.

On inspection he has a respiratory rate of 35 but no obvious recession. He has no scars, does not look cyanosed or anaemic but does cough during the examination. At one point he produces some mucky sputum.

While sitting on his mother’s lap he lets you examine him. His chest is not overtly hyperexpanded and there is good air entry, apart from the right base which has reduced air entry with multiple crackles and creps. The percussion note at this point is equivocal. The left side of his chest sounds clear.

Of interest, he has a cannula in his left hand. There is no oxygen in the room. What else do you need to know to make your diagnosis?

INTRODUCTION

On entering the room you are presented with a child approximately 2 years old. You are asked to comment on any striking features and discuss how this might relate to the child’s anaemia.

CLINICAL SCENARIO

On general inspection the child is pale and looks small for his age. He is playing with a toy in his mother’s arms and you notice an abnormality to his right hand. On closer inspection he appears to have two thumbs but both are hypoplastic. The more proximal lacks a distal phalangeal segment and the distal is attached by only a small piece of skin (see Fig. 3).

What more do you want to examine in his upper limbs?

After presenting your findings, which other parts of the body will you examine?

Just before presenting your overall findings you notice some café-au-lait spots on the child’s chest. What could the diagnosis be?

INTRODUCTION

You are informed that you are to assess this child’s speech and language.

CLINICAL SCENARIO

A child about 5 years old is playing with a ball in the centre of the room. You notice immediately that he has hemifacial hypoplasia. His left eye, cheek and ear are deformed. His external auditory meatus is nearly entirely absent on the affected side, with his eye present but obstructed on the lateral aspect by overlying skin. His mandible is not well formed and causes his mouth to appear lopsided.

How do you assess his hearing?

Will the dysmorphism affect how you approach this case?

What can a 5-year-old child do?

SETTING

You are a specialist registrar working at a district general hospital which has a large number of medical students.

SCENARIO

It is the start of the new academic year and the next intake of paediatric medical students have just started their attachment. All the students are to undergo training in basic life support (airway, breathing and circulation) of an infant. You are to instruct Robert in how to manage a 2-year-old child who is not breathing and does not have a pulse. You may not complete the task in 9 minutes but you should teach in a systematic manner, ensuring Robert understands the tasks he must perform and that he performs them correctly. A mannequin with appropriate aids has been provided.

The scenario is that Robert finds this child in the hospital car park. He is not in danger and is able to send for help. He must provide basic life support until the paediatric crash team arrive.

STATION 8

This station assesses your ability to communicate appropriate, factually correct information in an effective way within the emotional context of the clinical setting:

SETTING

You are a specialist registrar in a busy paediatric intensive care unit.

SCENARIO

Stephanie, a 5-year-old girl, was involved in a high-impact road traffic

accident and has been on your unit for 8 days. She has suffered severe brain injury and is making no respiratory or neurological effort. A brain stem death test is to be performed by the consultant shortly after a long discussion with the parents. Stephanie’s brother, Mark, who is 18, has been asking questions about the test and what it means. Her parents have asked for a doctor to speak to him as they feel too traumatised. The consultant asks you to speak to Mark.

BACKGROUND

Stephanie was a previously healthy girl who was hit by a car while crossing the road. Mark has only just arrived on the unit, having had to return from holiday. He is about to go to university to study law. He is unsure what brain stem death is and why the doctors can’t leave her on the ventilator until her head is better and she wakes up. He is obviously extremely upset at not having been able to come home earlier.

Please sensitively discuss with Mark why you are performing a brain stem death test and what this might involve. You do not need to talk about Stephanie’s management previously. The brain stem will show brain stem death. There will be no chance of recovery.

INFORMATION

You are a specialist registrar working in an outpatient clinic in a general district hospital. You receive the following letter from a GP:

BACKGROUND

Steven was diagnosed 6 years ago and has been on regular subcutaneous insulin since. His control has generally been very good and his last HbA1c measured at the diabetic review clinic last year was 6.5%.

Take a history from Steven and his mother and present your management plan to the examiner.

DIAGNOSIS: AORTIC STENOSIS

It would be nice if every child seen in the cardiovascular station has obvious signs and symptoms – but they don’t. You may have a child with an unusual congenital heart defect whose abnormality is impossible to determine on clinical examination. So it is important to know what to rule out if your child has a murmur of indeterminate origin.

In this case you know the child has a systolic murmur without the presence of previous surgery or heart failure. What could this be?

As the child is not cyanosed, and there are no scars, this child does not have an uncorrected cyanotic heart condition (tetralogy of Fallot, pulmonary atresia without a ventricular septal defect, etc.). Ventricular septal defect is a fair guess in a well child, although it is unusual to have a defect which isn’t loudest at the lower left sternal edge. How do you rule out potentially more sinister defects? Pulmonary stenosis classically presents with an ejection systolic murmur maximal at the left sternal edge with an ejection click and split second heart sound. If all patients presented classically I think the exam would be a lot more fun! Fortunately aortic stenosis has a sign which cannot be present with other anomalies: a carotid thrill. The textbooks tell you a suprasternal thrill but pulmonary stenosis may also produce this. If a thrill is felt in the carotid region (and it is obvious, I promise!) then the murmur must be due to aortic stenosis. If you don’t feel a thrill you are not much better off, as it could still be aortic stenosis, and all the other differentials! But it is an important negative.

If you ascertain the presence of a thrill then you should start feeling comfortable as there are a number of other features to start looking for, all relatively easy to learn.

Did you remember to ask the examiner to check peripheral pulses and perform a blood pressure?

REMINDERS

Turner’s syndrome (full details can be found on p. 184) 5X (although mosaicism possible)

Proportional short stature

Cardiac disorders are the cause of increased mortality in this syndrome

Aortic coarctation most common cardiac defect

Dental malocclusion increased, so prophylaxis vital

Predisposition to keloid scar formation (child may have cardiac surgical scars)

Indication for using growth hormone (and consider using steroid to increase final height).

Aortic stenosis	Tips
Exam	Apex beat (do you always examine this?) may be displaced
	Suprasternal and carotid thrill
	Systolic murmur in aortic region and left sternal edge
	Have you felt for peripheral pulses? (Associated with coarctation)
	The slow rising pulse is a very difficult sign to elicit
	Have you listened to the back? (Not always helpful but there shouldn’t be radiation, unlike in coarctation)
	A budding cardiology professor may pick up the diastolic murmur of aortic incompetence, which is more likely to occur post-surgery
Investigation	ECG normal in the well child but may show signs of left ventricular hypertrophy: • Tall R waves and inverted T waves in V5-V6 • CXR may show a prominent left ventricle • ECHO for diagnosis and management (assessing gradient)

Management Generally a conservative approach with the guideline that a gradient of < 60 mmHg across the valve requires no treatment.   Surgical treatment is via balloon valvuloplasty and will have no obvious scar!   Remember the murmur may remain post-surgery Associated conditions You should know and be able to quickly recite associations with:

DIAGNOSIS: INFLAMMATORY BOWEL DISEASE

It is potentially possible to complete a large proportion of your SHO training with little experience of surgery and scars. Severe inflammatory bowel disease is generally treated by specialist gastroenterologists and surgeons and unless you have done a specific attachment your experience may be limited to the textbook. Knowledge of common surgical scars will either impress examiners or save embarrassment, depending on which way you look at it!

Nutritional assessment is commonly overlooked in revision. An entire short case may be devoted to it, so make sure you have an understanding of the principles involved.

Nutritional assessment	Notes
General	Weight /height /BMI
	Nasogastric placement
	Long-term venous access
	Does the patient look well?
Storage	Subcutaneous fat (mid-arm, subscapular and axillary)
	Muscle bulk (biceps, triceps and quadriceps) Prominence of iliac bones
Face	Pale conjunctiva
	Oral ulceration Dental caries
Skin	Liver disease and vitamin deficiency demonstrated by jaundice and bruising
	Rashes
	Excoriations
	Erythema nodosum
Hands	Pallor (of palmar creases)
	Clubbing of fingers
	Palmar erythema
	Koilonychia (dystrophy of the fingernails in which they are thinned and concave with raised edges; ‘spoon-shaped nails’)
	Leuconychia (white spots on the nails)
Abdomen	Distension
	Hepatosplenomegaly
	Surgical scars
Other	Gait examination: tests strength and any neurological impairment
	Blood pressure and pulse (standing and lying to measure postural change)
	Urinalysis and stool analysis as appropriate Pubertal assessment

Just because in books patients with Crohn’s disease have aphthous ulcers (do you actually know what these are?), gum hyperplasia, little subcutaneous fat, anaemia, growth delay, peri-anal skin tags and signs of steroid toxicity doesn’t mean your patient will.

[Aphthous ulcer: a shallow individual ulcer that is round or oval in shape. The ulcer will usually be no more than ¼ inch in diameter. The centre of the ulcer will be covered with a loosely attached white or greyish membrane. The edges of the ulcer will be regular (non-jagged) and surrounded by a reddish halo. The tissue adjacent to the ulcer will be healthy in appearance.]

Your growth-delayed, slightly pale teenager with a scar without signs of lymphoreticular disease (i.e. oncological) is likely to have an inflammatory bowel disease. If they have GI involvement outside of the colon, as evidenced by oral ulceration, then they are likely to have Crohn’s but this is not absolute. What you must do is convince the examiner you are able to acknowledge growth delay or nutritional deficiency in a sensitive manner. You must then assess the most likely cause of this. Finally, be able to suggest some investigations and management both for the acute and non-acute situation.

Inflammatory bowel disease	Tips
Exam	Weight/height/BMI/head circumference
	Clubbing (rare)
	Cushingoid appearance or striae
	Scars from acute surgery (obstruction) or non-acute surgery (growth failure, debilitating symptoms, colectomy/pouch or prevention of malignancy), perineum, looking for anal fissures and skin tags
Investigations	Blood tests:
	(Leucocytosis, raised ESR, low protein, renal impairment, consider vitamins and minerals in long-term follow-up)
	Stool:
	Culture to exclude other diagnoses
	α1-Antitrypsin (reflects bowel protein loss)
	Imaging/endoscopy:
	Left wrist X-ray for bone age
	AXR acutely
	Barium meal/follow-through dependent on position of lesion
Management: Crohn’s	Multidisciplinary approach
	Use of steroids (orally or intravenous)
	Aware of sulfasalazine for small bowel disease
	May need steroid sparing agents: azathioprine, ciclosporin or methotrexate
	Nutritional therapies including use of overnight feeds
Management: ulcerative colitis	Multidisciplinary approach
	Steroid enemas or oral steroids
	Sulfasalazine for acute situation
	Yearly colonoscopy when disease > 10-15 years

An example answer would be:

REMINDER

A laparotomy scar will run transverse through the centre of the abdomen (see Fig. 4).

CAN YOU …

Talk through your examination of a child’s nutritional status with a colleague?

DIAGNOSIS: SYDENHAM’S CHOREA

The art of observation before examination is persistently stressed throughout your medical training. Although you may not have done this as a medical student it is essential you have the ability to ‘hang back’. Engaging your brain before putting your mouth in gear will stop you blurting out an answer you will be unable to defend.

It is vital before the exam that you are able to talk about observed features in a systematic fashion. It is actually a very difficult thing to do; try talking about colleagues’ gaits. Can you make an efficient summary without stopping, mumbling and being imprecise? The longer you are able to discuss things, the less time you have to be observed examining them – much the harder task!

Learning long lists of differentials is only helpful if you are able to pick up the signs that apply to them. In this situation you are expected to recognise chorea and possibly athetosis (with the appreciation that a written description is no substitute for actually having seen the clinical sign). The most obvious differential is a tic or habit spasm. These are repetitive and have a classical stereotypical pattern, but most importantly can be briefly controlled by the subject. The performance of the movement provides some brief relief.

It is useful to have a good understanding of the potential causes and apply those to the child in question. The examiner will expect you to be able to describe the irregular jerking movements and how the patient seems to have little control over them. They would then expect you to be able to mention additional signs (seen or unseen) that would aid a diagnosis, not just a list of differentials.

In the former you instantly lose the trust of the mother and the examiner wonders where you learned your bedside manner. The second demonstrates you are not just a walking textbook but able to apply your knowledge systematically and relevantly. This child had Sydenham’s chorea.

Chorea	Notes PASS
Anticonvulsant side effect
Cerebral palsy – dyskinetic	Look specifically for athetosis (slow writhing movements) as cerebral palsy is the commonest cause of this
	Assess intellectual impairment, spastic posturing and gait
Sydenham’s chorea	Acute-onset choreiform movements
	May have ‘milkmaid’s grip’ – inconsistent hard and weak grasp of examining finger
	Extended hands tend to pronate
	Anaemia, leucocytosis and potential increased ESR
	Check ASO titre
	Throat swab for Group A haemolytic streptococci There may be a psychological element (emotional lability and depression)
	Treat with dopaminergic blockers (haloperidol)
	Remember this is a major manifestation of rheumatic fever and so is likely to need antistreptococcal rheumatic fever prophylaxis
Wilson’s disease	Family history (autosomal recessive)
	Difficulty excreting copper in bile
	Low ceruloplasmin and increased urinary excretion of copper
	Evidence of liver failure (must examine abdomen of children with chorea)
	Copper chelate with D-penicillamine
Degenerative conditions (?appropriate for exam)	Huntington’s disease, Lesch-Nyhan, Moyamoya

The following websites have video clips of choreiform movements and tics. The former is a superb revision aid, especially for developmental progression in the under-2-year-old (go to pediNeurologic exam):

The movements may be controlled by haloperidol, although it is important to discuss some of the important side effects and caveats with parents before commencing treatment. This would easily be the basis for a communication station.

Criteria for diagnosis of rheumatic fever (need two major or one major and two minor plus evidence of streptococcal disease)

Major	Plus
Carditis	Increased titres of antistreptolysin O
Polyarthritis	Positive throat culture for Group A streptococci
Sydenham’s chorea
Erythema marginatum
Subcutaneous nodules (not erythema nodosum)
Minor	After acute episode
Previous rheumatic fever	Need regular penicillin
Polyarthralgia	Follow-up of possible valvular damage
Fever
Elevated CRP, ESR, WCC
Prolonged P-R interval

CAN YOU …

List the causes of chorea?

Describe which investigations you would perform on a child you suspect has Wilson’s disease?

DIAGNOSIS: RIGHT BASAL PNEUMONIA

The new exam mark sheets have a specific box to assess whether the child is cooperative or not for the exam. As long as you have not provoked the child by doing something inappropriate or rough you cannot be penalised. In the stress of the exam it is easy to forget the ‘child’ and only remember the ‘system’. Part of your introductory patter should involve some play with the child. Most importantly, get mother on your side! To some candidates this comes naturally but to others it may not be normal (or sometimes expected) for them to be involved in child’s play. It is a skill as hard as picking up an atrial septal defect and probably should be practised as much! Chronic respiratory disease is an easy provider of signs and symptoms so is well utilised in the exam. However, there is no reason that a previous acutely unwell child may be used. In this case there are specific features to elucidate, so have you been listening? Which side were the abnormal breath sounds on? Is there an inhaler/monitor/sputum pot by the bed? Do not stop listening until you are able to tell the examiner:

This child is recovering from a right basal pneumonia but in order to make this diagnosis you must also know:

CAN YOU …

List techniques you would use to entertain/distract a 4-year-old child?

DIAGNOSIS: FANCONI’S ANAEMIA

Having remembered long lists of odd syndromes and genetic variants for the previous two exams it would be nice to think there was something you could forget! Unfortunately, the ‘other’ station may bring up the odd curiosity despite the College’s statement that this should be an exam of normal practice. Much like dermatological problems being linked to underlying neurological deficits, abnormalities of the arms, especially the radius and thumb, are linked to haematological disease. The two most common textbook examples are TAR (thrombocytopenia absent radius syndrome) and in this case Fanconi’s anaemia. Sometimes you just have to know your stuff!

Fanconi’s anaemia	Notes
Signs and symptoms	Anaemia causing weakness
	Neutropenia causing infections
	Thrombocytopenia causing bleeding and bruising
	Commonly have abnormal pigmentation of the skin
	May have short stature with skeletal malformations (absent thumb and radius – check it’s there!)
	Renal anomalies
Laboratory findings	Macrocytic anaemia
	Thrombocytopenia precedes severe aplastic anaemia
	Rule out idiopathic thrombocytopenic purpura
Complications	Generally related to haematological dysfunction but there is also a significant risk of developing a malignancy, especially leukaemias and solid tumours

DIAGNOSIS: TREACHER COLLINS SYNDROME

Do you know your syndromes? For this case it actually doesn’t matter, as you were asked to ‘assess this child’s speech and language’. In fact, it is very important not to get distracted by the obvious abnormalities. Even knowing the syndrome, if you cannot do a quick assessment of speech and hearing you will not be gaining the marks. Remember the College specifically say they are not testing you against the trained developmental paediatrician but the first-year registrar. Accepting this child is unlikely to present to an outpatient clinic at this age with concerns about speech and language, this must still be the approach to take.

Unless definitely told otherwise, it is appropriate to ask the mother about any concerns she has with regard to hearing and speech. The examiner may use this to ask further questions about how you would test specific deficits. Make sure you have seen audiological tests performed. You may well be asked to explain them in the communication skills station!

In the exam, as you do not have access to audiometry you must utilise simpler tests such as distraction testing. In fact, this simple test may be utilised for any age. While playing with the child (without noise), if you ask the examiner to call his name from behind him and he looks round every time then some element of hearing must be present (the converse is not true!). It can be difficult to show differences in each ear but the McCormick toy test may be used. Although used for a slightly younger age group (2–4 years) you do not have formal audiological testing to hand and neurological exam on the eighth cranial nerve is not an appropriate first step for this station. Pairs of similar sounding toys are placed in front of the child and the name of one whispered in each ear. It is important that the child cannot see your mouth as some children may have learnt basic lip-reading.

Listening to the child talk is vital as it is a functional sign of audiological ability. Poor quality or limited language for a 5-year-old in the absence of motor problems is likely to represent a degree of hearing impairment. Given the malformations, this may seem like an obvious diagnosis. The examiner is looking for how you approach the history and child.

Have you commented that the fine and gross motor skills appear normal? Is the child playing with toys that produce noise? How does he interact with his mother?

Note in the guidelines to the exam that the College specifically state that the child will have a developmental age of less than 5 years. This does not mean the child will be less than 5 years. It does mean that as long as you know development milestones up to 5 years of age you should at least be able to comment on whether the child is underachieving.

Causes of speech delay are listed below. Again, you are being assessed on your ability to recognise developmental abnormality, not to provide a list of differential diagnosis. This list should help you look for things to confirm your initial suspicions and maybe make comments on other features that support this – proving to the examiner you really are a well-rounded paediatrician!

	At 5 years
Gross motor	Stands on one foot for up to 10 seconds (and can therefore hop)
	Interest in climbing
	May be able to skip
Fine motor and vision	Copies triangle
	Draws person with body details
	Uses fork and spoon but not knife
Speech and hearing Can count 10 objects
Knows if morning or afternoon Can read words
Social	Knows name and address
	Can use large buttons in dressing and undressing
	Knows right and left hand
	Can do simple household tasks (helps with dishes)

CAUSES OF SPEECH DELAY

This child had Treacher Collins syndrome with left-sided hearing loss but no other developmental delay.

TYPE OF HEARING TEST

•Distraction tests	6–12 months
•Speech discrimination tests	2–4 years
•Performance tests	24–30 months
• Pure tone audiometry	4–5 years
• Electrical response audiometry	Neonate
• Brain stem auditory evoked responses	Neonate
•Evoked otoacoustic emissions	Any age

REMINDER

REMINDER

Examination must include:

Rapid, short- and long-acting insulins are all available to enable:

General requirement 0.7-1.0 Units/day, increasing to 1.0-1.5 Units/day with the pubertal growth spurt.