Circuit E
STATION 1
This station assesses your ability to elicit clinical signs:
This is a 9-minute station of clinical interaction. You will have up to 4 minutes beforehand to prepare yourself. No additional information will be given or is necessary before commencing the station. When the bell sounds you will be invited into the examination room.
CLINICAL SCENARIO
The baby is well grown and does not look dysmorphic. There is no peripheral cyanosis or stigmata of cardiovascular disease. You cannot see any scars or feel any thrills. On auscultation you note an ejection systolic murmur in the pulmonary area and an ejection click.
What have you examined to conclude there are no stigmata of cardiovascular disease?
STATION 2
This station assesses your ability to elicit clinical signs:
This is a 9-minute station of clinical interaction. You will have up to 4 minutes beforehand to prepare yourself. No additional information will be given or is necessary before commencing the station. When the bell sounds you will be invited into the examination room.
CLINICAL SCENARIO
On inspection you note he is short for his age. He is sitting bare-chested on an inclined bench. He lacks muscle bulk and has no chest or axillary hair. You note a thoracolumbar kyphosis and bilateral hearing aids. He has a prominent forehead, large tongue and pronounced lips.
On assessing the abdominal system you note the positive findings of hepatosplenomegaly and a scar in the left inguinal region.
Can you bring these findings together in one diagnosis?
His eye examination is normal.
STATION 3
This station assesses your ability to elicit clinical signs:
This is a 9-minute station of clinical interaction. You will have up to 4 minutes beforehand to prepare yourself. No additional information will be given or is necessary before commencing the station. When the bell sounds you will be invited into the examination room.
INTRODUCTION
You are asked to comment on and examine as appropriate a 9-month-old baby boy who is with his mother.
CLINICAL SCENARIO
You immediately notice a protuberance over the left side of his skull and a pipe-like structure crossing the triangles of his neck, with a small scar in the left upper quadrant of his abdomen. His head appears slightly larger than normal for his body size. You note that he has horizontal nystagmus with a convergent left squint.
As he is happily playing with a toy you are using to entertain him, you note he does not move his left arm or leg. He has generally increased tone on his left side with decreased power and brisk reflexes.
STATION 4
This station assesses your ability to elicit clinical signs:
This is a 9-minute station of clinical interaction. You will have up to 4 minutes beforehand to prepare yourself. No additional information will be given or is necessary before commencing the station. When the bell sounds you will be invited into the examination room.
INTRODUCTION
On entering the station you are asked to examine a 12-year-old girl who has recently moved to the UK from India. She has been referred to you because she has suffered from a chronic cough for several years. The GP has found that a series of antibiotics and a course of oral steroids have been unhelpful.
CLINICAL SCENARIO
The girl of Asian origin is very shy and quiet. On examination you notice early clubbing, a hyperexpanded chest, Harrison sulci and coarse crackles on auscultation. While listening to her chest you notice her heart sounds are quiet and discover her apex to be on the right-hand side.
What is the likely diagnosis and what else would you like to examine?
STATION 5
This station assesses your ability to elicit clinical signs:
This is a 9-minute station of clinical interaction. You will have up to 4 minutes beforehand to prepare yourself. No additional information will be given or is necessary before commencing the station. When the bell sounds you will be invited into the examination room.
INTRODUCTION
On entering the room you notice a young girl of 2 years. You are asked to inspect her arms and then go on to examine the relevant system.
CLINICAL SCENARIO
She appears well, although you suspect that she may have learning difficulties from her behaviour and apparent microcephaly. You notice in particular hypopigmented streaks and patches and cutaneous atrophy over her trunk and arms, and poor dentition.
Which system do you go on to examine?
STATION 6
This station assesses your ability to assess specifically requested areas in a child with a developmental problem:
This is a 9-minute station of clinical interaction. You will have up to 4 minutes beforehand to prepare yourself. No additional information will be given or is necessary before commencing the station. When the bell sounds you will be invited into the examination room.
INTRODUCTION
You are asked to assess the vision of a 6-month-old infant. You are provided with a range of development assessment tools to aid you in your efforts.
CLINICAL SCENARIO
On initial inspection you do not notice any abnormality. The baby appears well grown and is sitting on his mother’s lap with support. You take an object and offer it to the child but get no response. The child does not attempt to grab the object but when placed in his hands he does demonstrate a palmar grasp.
You then use a red ball to see if the child will fix and follow through 180° but get no response. The child fails to fix either on your face or that of the mother and there is only a brief interest on fixing to a light.
STATION 7
This station assesses your ability to communicate appropriate, factually correct information in an effective way within the emotional context of the clinical setting:
This is a 9-minute station consisting of spoken interaction. You will have up to 2 minutes before the start of the station to read this sheet and prepare yourself. You may make notes on the paper provided.
When the bell sounds you will be invited into the examination room. Please take this instruction sheet with you. The examiner will not ask questions during the 9 minutes but will warn you when you have approximately 2 minutes left.
You are not required to examine a patient.
The encounter should be focused on the task; you will be penalised for asking irrelevant questions or providing superfluous information. You will be marked on your ability to communicate, not the speed with which you convey information. You may not have time to complete the communication.
SCENARIO
You have been asked to speak to the parents of Oliver, a 2-year-old child with cerebral palsy. Oliver needs a cannula but is extremely difficult to gain access to. An SHO has had repeated attempts and despite parents’ suggestions to ask for help the SHO continued regardless. Parents eventually became extremely angry and are demanding to speak to the consultant. They would like to take their child to another hospital.
BACKGROUND
Oliver was born at 25 weeks and had a very rocky time on the neonatal unit. He has suffered from sepsis, necrotising enterocolitis and required oxygen for his first year of life. He has severe developmental delay and is awaiting a gastrostomy/fundoplication to help him feed as he suffers from severe reflux. He has been admitted with gastroenteritis and is mildly-moderately dehydrated. He is not shocked but will require fluid therapy, which he has not tolerated by bolus nasogastric feed.
It is a weekend and the consultant is currently in consultation with the parents of a child with suspected non-accidental injury. He will not be available for at least 20 minutes.
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:
This is a 9-minute station consisting of spoken interaction. You will have up to 2 minutes before the start of the station to read this sheet and prepare yourself. You may make notes on the paper provided.
When the bell sounds you will be invited into the examination room. Please take this instruction sheet with you. The examiner will not ask questions during the 9 minutes but will warn you when you have approximately 2 minutes left.
You are not required to examine a patient.
The encounter should be focused on the task; you will be penalised for asking irrelevant questions or providing superfluous information. You will be marked on your ability to communicate, not the speed with which you convey information. You may not have time to complete the communication.
SCENARIO
An 18-month-old child (Kate) has been admitted with a febrile convulsion. You must explain the diagnosis to the father. No further medical tests are necessary.
BACKGROUND
Kate had a viral upper respiratory tract infection and had a typical febrile convulsion (generalised, less than 5 minutes, complete neurological recovery). Examination of Kate was entirely normal and there are no features suggestive of meningitis or raised intracranial pressure. Kate does not require further tests and will be observed overnight for parental reassurance.
STATION 9
This station assesses your ability to take a focused history and explain to the parent your diagnosis or differential management plan:
This is a 22-minute station of spoken interaction. You will have up to 4 minutes beforehand to prepare yourself. The scenario is below. Be aware that you should focus on the task given. You will be penalised for asking irrelevant questions or providing superfluous information. When the bell sounds you will be invited into the examination room. You will have 13 minutes with the patient (with a warning when you have 4 minutes left). You will then have a short period to reflect on the case while the patient leaves the room. You will then have 9 minutes with the examiner.
INFORMATION
You are a specialist registrar in a general paediatric clinic and receive the following letter from a GP regarding your next patient:
I would be grateful if you would see this young boy. His family have just moved into the area from Pakistan and from a brief letter I received from the family I believe he has a VSD. He is very small for his age and I am concerned about his growth. Thank you for your help in this matter.
Take a relevant history from Hisham’s parents with regard to his potential problems. You do not need to examine Hisham or explain to the parents your management plan. You should be prepared to discuss this with the examiner.
COMMENTS ON STATION 1
DIAGNOSIS: PULMONARY STENOSIS (PS)
These findings suggest a diagnosis of pulmonary stenosis and in particular with the stenosis being at the level of the valve (in view of the click). In the exam diagnosis of this murmur would be entirely dependent on your being able to localise a systolic murmur to the pulmonary area. The click is an added bonus which will clinch the diagnosis but may not be picked up (apparently best heard at the third left intercostal space in expiration.) Textbooks also suggest the presence of a right ventricular heave (this will be felt at the left sternal border).
Pulmonary stenosis is an example of an acyanotic heart condition (critical pulmonary stenosis as a neonate has a different pathophysiology due to shunting and is a cyanotic heart condition). Do not forget to listen for a possible ventricular septal defect, which would indicate tetralogy of Fallot. Other valve or hole defects may also be present, e.g. atrial septal defect or patent ductus arteriosus in a more complicated cardiac lesion. Do not forget to look for a scar in the mid-axillary line; this may represent a scar after a Blalock-Taussig shunt (palliative procedure).
Important differentials to exclude are an atrial septal defect (also an ejection systolic murmur in the pulmonary area but you should hear a wide, fixed, split second heart sound – heard only by experts!) and aortic stenosis (louder in the aortic region and generally associated with a carotid or suprasternal thrill). There may be a suprasternal thrill with PS; a carotid thrill is diagnostic of aortic stenosis. If the murmur is soft, with no radiation, consider the possibility that this is an innocent pulmonary flow murmur.
Please see table below for investigations and management of PS.
NOONAN’S SYNDROME
This syndrome shares many phenotypic similarities with Turner’s syndrome but can occur in both sexes. It can be inherited in an autosomal dominant pattern – chromosome 12q.
• Facial dysmorphism, e.g. hypertelorism, down-slanting palpebral fissures, webbed neck, triangular facies, ptosis
| Investigations: | |
| CXR | Often normal but may see a prominent pulmonary artery or decreased pulmonary vascular markings in more severe disease |
| ECG | Normal if mild. If moderate to severe – right axis deviation and right ventricular hypertrophy In Noonan’s you get a superior axis |
| ECHO | A gradient of > 40 mmHg would indicate a need for surgery or the right ventricular pressure is > 60 mmHg |
| Management: | |
| Multidisciplinary | Cardiologist, local paediatrician – local and tertiary referral centre |
| Conservative/medical | Adequate nutrition and growthMay only need clinical review and no need for surgery if mildMay need diuretics if associated significant shunts Need alprostadil (PGE1) in the presence of cyanotic congenital heart disease during the neonatal period Prophylaxis during surgical procedures |
| Surgical | Cardiac catheterisationBalloon valvuloplasty is the corrective treatment of choice |
| Associated conditions include: | Noonan’s syndrome Tetralogy of Fallot |
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