Circuit D
STATION 1
This station assesses your ability to elicit clinical signs:
STATION 2
This station assesses your ability to elicit clinical signs:
STATION 3
This station assesses your ability to elicit clinical signs:
STATION 4
This station assesses your ability to elicit clinical signs:
STATION 6
This station assesses your ability to assess specifically requested areas in a child with a developmental problem:
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:
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:
STATION 9
This station assesses your ability to take a focused history and explain to the parent your diagnosis or differential management plan:
COMMENTS ON STATION 1
DIAGNOSIS: VENTRICULOSEPTAL DEFECT
Tips | |
---|---|
Exam | A loud second heart sound suggests pulmonary hypertension (from a large shunt) until proven otherwise Feel the suprasternal notch (a thrill there means AS, or rarely PS) A thrill means the murmur must be grade 4: |
Maintain normal growth with calorie supplementation
Surgery if pulmonary to systemic flow ratio > 2:1
Good dental hygiene with antibiotic prophylaxis (amoxicillin) for
procedures
REMINDER
All Down’s syndrome children should be routinely sent for an echocardiogram (VSD, AVSD). These children are at high risk of developing pulmonary hypertension and subsequently Eisenmenger’s complex with large shunts.
Neonatal | Infancy | Any |
---|---|---|
Hypoplastic left heart | VSD | SVT |
Aortic coarctation | AVSD | Myocarditis |
Aortic stenosis | PDA (large) | Cardiomyopathy |
Tricuspid atresia | TAPVD | |
Interrupted aortic arch |
COMMENTS ON STATION 2
DIAGNOSIS: HEREDITARY SPHEROCYTOSIS
Hereditary spherocytosis | Tips |
---|---|
Exam | Cholecystectomy scar (more likely the older the patient) and a splenectomy scar |
Treatment | Remember folic acid supplements Pneumococcal, meningococcal and Haemophilus influenzae B immunisation required Lifelong penicillin prophylaxis |
Acute management | Aplastic crises can occur Pigmented gallstones present in majority by second decade |
Differentials | Hereditary elliptocytosis Autoimmune haemolytic anaemia |
The mother has had her gallstones removed (also having hereditary spherocytosis!).
COMMENTS ON STATION 3
DIAGNOSIS: CEREBRAL PALSY
As your head doesn’t tend to shrink, it is likely an insult has occurred early in life.
In the neurological exam you must always be thinking:
What am I seeing? A neurologically disadvantaged child with multiple handicaps.
Why am I examining this limb? Spastic gait indicates upper motor neurone lesion.
What am I looking for? Increased tone and reflexes with clonus. Reduced power.
What am I finding? Is there wasting? Are the abnormalities symmetrical?
Prenatal | Genetic TORCH IUGR Maternal alcohol/substance misuse |
Perinatal | Hypoxic ischaemic encephalopathy |
Ventricular haemorrhage | |
Hypoglycaemia | |
Postnatal | Meningitis/encephalitis Head injury |
COMMENTS ON STATION 4
DIAGNOSIS: PREVIOUS PNEUMONIA LEADING TO BRONCHIECTASIS (UNABLE TO DETERMINE THIS FROM INFORMATION GIVEN)
A frustrating element of the exam is sometimes there not being an ‘answer’. Many children are diagnostic dilemmas and have had multiple investigations, many treatments and thousands of membership candidates prodding and poking them. It is easy to convince yourself a sign must be there. To misquote the Jedi Master Yoda: ‘Maybe not. A clinical sign or no clinical sign. There is no maybe.’
The differential diagnosis of chronic cough amongst all ages:
History and exam
Radio-imaging
Radio-imaging
History/pH study
Exam
Mantoux/CXR
Biopsy/FBC
History and radio-imaging
Immunoglobulins
Sweat test
Protease inhibitor typing
Exam | Scars on the chest may be from surgical lines pop(?for antibiotics) Hyperexpansion: Without clubbing – asthma or chronic lung disease With clubbing – cystic fibrosis or bronchiectasis Other – persistent aspiration, tracheo-oesophageal fistula Hyperexpansion may be diagnosed clinically (increased AP diameter or displaced liver) or radiologically |
Causes of clubbing | Clubbing is not seen before 6 months of age |
---|---|
Cardiac | Congenital cyanotic heart disease Subacute bacterial endocarditis |
Respiratory | Bronchiectasis/cystic fibrosis Primary ciliary dyskinesia Tuberculosis Empyema Malignancy Fibrosis |
GI | Inflammatory bowel disease Biliary cirrhosis |
COMMENTS ON STATION 5
DIAGNOSIS: NEUROFIBROMATOSIS
Unlike Station 4, when the answer might not be obtainable from the examination findings alone, it may be just you who doesn’t have a clue! In this scenario the candidate recognised the large right leg and remembered something about hemihypertrophy and renal masses (suggesting an ultrasound when asked about investigations). He was asked what else he would examine and drew a blank. The examiner then demonstrated the large ipsilateral arm and an even more obvious unilateral tongue enlargement. The candidate had no idea and left deflated and confused, only noticing the café au lait spots as he left the room. Subsequently he passed the station probably just for picking up the original sign. Until you get the mark sheet you do not know how you have performed!
Hemihypertrophy | May involve whole side of body or just one limb May be congenital Associated with Wilms’ tumours Occurs in Beckwith-Wiedemann syndrome Russell-Silver syndrome |
Regional overgrowth | Neurofibromatosis type 1 (see summary on p.190) Haemangiomas |
REMINDER
Russell-Silver dwarfism
Should have | Commonly have | May have |
---|---|---|
Growth failure | Infant hypocalcaemia | Café au lait spots |
Triangular shaped facies | Hemihypertrophy | Learning difficulty |
Poor feeding in infancy | Micrognathia | |
Clinodactyly (fifth finger) |
COMMENTS ON STATION 6
DIAGNOSIS: ISOLATED SPEECH DELAY
Candidates commonly forget that specific elements will be asked for. In this case only speech and language are being assessed, so the child copying a circle will not gain you marks. However, if told to draw a circle this shows a certain level of language appreciation and at least the ability to hear. But if the child is barely three (may not be able to draw a circle) then this is an unfair request. Therefore you need not only to know your developmental milestones inside out, but you must also be precise about the area they are testing and know how to apply them consistently.
COMMENTS ON STATION 7
‘I gather it has previously been explained to you why we need to perform a lumbar puncture, is that right? … Good. We have performed some blood tests to look for infection and make sure there will not be a risk of bleeding during the procedure. A lumbar puncture is …’
A generalised answer should include the following:
1. Introduction (name and position); explain you will not be bleeped.
2. Confirmation of mother’s relation to child and understanding of situation.
3. Does she want a relative or friend with her?
4. Explanation of the need to find a cause for the child’s illness and what a septic screen involves.
5. Specific details of a lumbar puncture.
6. Allay fears regarding neurological damage and pain.
7. Specifically state the need to hold the child securely (stating this is usually the most uncomfortable part for the child).
8. Ability to give antibiotics regardless of success of procedure.
COMMENTS ON STATION 8
1. The brain dead child: A determination of brain stem death made by accepted medical standards.
2. The permanent vegetative state: ‘A state of unawareness of self and environment in which the patient breathes spontaneously, has a stable circulation and shows cycles of eye closure and eye opening which simulate sleep and waking, for a period of 12 months following a head injury or 6 months following other causes of brain damage.’
3. The no-hope/chance situation: Treatment will delay death but will do nothing to improve the quality of life; there is no potential.
4. The ‘no-purpose’ situation: Continued treatment will not affect prognosis and may in fact make things worse. Things are likely to deteriorate with time.
5. The unbearable situation: Continued treatment and its effects are more than the family and/or child accepts, although it may be of some benefit.
How these are discussed or brought up is difficult and, I think, to the candidate’s advantage. Demonstration or awareness of principles will be the important factors. Prowess at teaching, as everyone is aware, is not proportional to the intelligence of the teacher!
A suggested strategy is as follows:
2. Assess understanding of ventricular haemorrhages in neonates.
Grade | Description | Mortality/disability |
---|---|---|
1 | Isolated germinal matrix haemorrhage | 6%/18% |
2 | Intraventricular haemorrhage with normal ventricle size | 33%/36% |
3 | Intraventricular haemorrhage of sufficient severity to dilate ventricles with blood | 60%/75% |
4 | Intraparenchymal haemorrhage | 93%/75% |
3. Does she understand the four principles of ethics (autonomy, non-maleficence, beneficence and justice)?
4. At this stage does Robert fulfil any of the criteria for withdrawing care (open to interpretation – remember you are discussing the principles; there is no right or wrong answer). Ultimately we have no way of knowing Robert’s level of functioning at this point and what his degree of neurological compromise will be.
5. Discuss that withdrawing care is a group decision that must involve the parents and multiple members of the healthcare team.
COMMENTS ON STATION 9
For the station in question the salient points are the ability to differentiate between issues which are important for the GP, i.e. regular medications, stability of condition and emergency treatment, as opposed to those which should be referred to a tertiary centre, such as treatment modalities. Tertiary centre referral is the essence of your management plan but not the answer. What the question is looking for is whether you have enough experience of chronic disease to enable you to start a pathway that provides for the complete needs of the patient:
1. A brief but thorough background of Monique’s past (how diagnosed, genetic analysis, disease progression in the first years of life).
2. Previous hospitalisations – acute or booked.
3. Respiratory status (lung function, microbiological colonisation, especially ABPA (allergic bronchopulmonary aspergillosis), current antibiotic regimens, DNase, treatment of coexisting asthma, physiotherapy).
4. Gastrointestinal disease (past and present complications, especially meconium ileus equivalent but also including rectal prolapse, growth, dietary and pancreatic supplements, dietician involvement, diabetic progression).
5. Social situation and family support.
6. Immunisations (influenza, pertussis and measles – easily forgotten!).