Week 3 - MSK Paediatric Orthopaedics Formative

Question 1

True or false: Osteogenesis imperfecta is a genetic disorder with both autosomal dominent and recessive models of inheritance. Select one: True False

Answer 1

The majority of cases of osteogenesis imperfecta are autosomal dominant. Rarer cases are autosomal recessive and are either fatal in the perinatal period or associated with spinal deformity. The correct answer is ‘True’.

Question 2

True or false: The age at time of diagnosis of Perthes disease of the hip is inversely proportional to the prognosis. Select one: True False

Answer 2

The younger the patient is at the time of diagnosis, the greater the proportion of duration of hip development during which the hip can be protected with limited weight bearing etc. Therefore, better outcomes are associated with diagnosis at the younger end of the presenting group. The correct answer is ‘True’.

Question 3

Slipped Upper Femoral Epiphysis (SUFE) is a condition mainly affectin goverweight pre‐pubertal adolescent boys where the femoral head epiphysis slips inferiorly in relation to the femoral neck. Which of the following features is NOT common in a patient presenting with SUFE? Select one: * a. Gross shortening of the affected limb * b. Limp * c. Loss of internal rotation * d. Pain in the hip (groin) * e. Pain in the knee

Answer 3

Gross shortening of the affected limb * This is not commonly a feature of SUFE. * This would make one think of a more chronic condition, such as a missed DDH.

Question 4

Cerebral Palsy (CP) is a neuromuscular disorder with onset before 2‐3 years of age due to an insult to the immature brain before, during or after birth. The expression of the disease and its severity are variable depending on the area of the brain affected. In Ataxic cerebral palsy, co‐ordination and balance are impaires. Which area of the brain is affected in Ataxic cerebral palsy?

Answer 4

Cerebellum

Question 5

In relation to flat feet, the key to diagnosis and management (when required) is first determining if there is mobile/flexible or fixed/rigid deformity. Mobile/flexible flat feet are those where the medial arch reforms with dorsiflexion of the great toe. In the rigid type of flat footedness, the arch remains flat regardless of load or great toe dorsiflexion. If a flat foot is described as rigid, which of the following is a possible cause of the flat foot? Select one: * a. Familial flat footedness * b. Tarsal coalition * c. Underlying ligamentous laxity

Answer 5

Tarsal coalition

Question 6

In‐toeing refers to a child who, when walking and standing will have feet that point toward the midline. The abnormality is often exaggerated when running and children are felt by their parents to be clumsy and wear through shoes at an alarming rate. There are a number of causes of in‐toeing. State the likely cause of intoeing in relation to the statement * On standing you observe the child has mild intoeing and apparent knock knees. However, xrays taken of the knees when lying down doesn’t show any evidence of an actual genu valgum.

Answer 6

Femoral neck anteversion – as part of normal anatomy the femoral neck is slightly anteverted (pointing forwards). Excess femoral neck anteversion can give the appearance of in‐toeing (as well as knock knees).

Question 7

State the likely cause of intoeing in relation to the statement * On standing you observe the child has mild intoeing. The rotational and coronal alignment of the hips, knees and ankles appear normal and you suspect that the intoeing is due to a forefoot deformity.

Answer 7

Forefoot adduction – can cause the apparent in‐toeing. There remains debate about whether surgery is useful in cases which persist after 7 or 8 years of age. Certainly surgery should not be considered before this age as the majority resolve in time.

Question 8

State the likely cause of intoeing in relation to the statement * On standing you observe the child has mild intoeing. The alignment of the knees appears neutral. You notice that the tibial tuburcle appears internally roated and, in fact, the foot is normally alignment when you take this into account.

Answer 8

Internal tibial torsion – the bone can be rotated inward about its vertical axis but as this is a normal variation, it should ignored.

Question 9

Normal developmental milestones: Knowledge of the normal neurological devolopment timeline is important as it allows one to recognise developmental delay, a possible sign of neuromuscular disease. By roughly what age should a child be able to control their own head? Select one: * a. 6 months * b. 6-8 weeks * c. 3 months * d. 3 weeks

Answer 9

Usually by 6 weeks (option B 6-8 weeks)

Question 10

True or false: Talipes Equinovarus (Clubfoot) is more common in boys than girls? Select one: True False

Answer 10

True - boys are twice as commonly affected than girls

Question 11

True or false: Osteogenesis imperfecta is a defect of the maturation and organization of type II collagen (which accounts for most of the organic composition of bone). Select one: True False

Answer 11

Osteogenesis imperfecta is a defect of the maturation and organization of type I collagen (which accounts for most of the organic composition of bone).

Question 12

The terminology varus and valgus is used to describe the aligment of a limb/joint in the coronal plane (when viewed from the front or back). Match the terms to their correct definition: * Angluation of the distal segment of a bone or joint towards the midline * Angluation of the distal segment of a bone or joint away from the midline * Angluation of the proximal segment of a bone or joint away from the midline

Answer 12

* Varus → Angulation of the distal segment of a bone or joint towards the midline * Valgus → Angulation of the distal segment of a bone or joint away from the midline

Question 13

True or false: Progressive muscular weakness is the primary feature of Duchenne’s muscular dystrophy. Select one: True False

Answer 13

A defect in the dystrophin gene involved in calcium transport results in muscle weakness which may only be noticed when the boy starts to walk with difficulty standing (see Gower’s sign below) and going up stairs. Progressive muscle weakness follows and by the age of 10 or so, they can no longer walk and by age 20, progressive cardiac and respiratory failure develop with death typically in the early 20s. The correct answer is ‘True’.

Question 14

Spina bifida is a congenital disorder where the two halves of the posterior vertebral arch fail to fuse, probably in the first six weeks of gestation. In its more severe form, spina bifida cystica, the contents of the vertebral canal herniate through the defect. In a myelomeningocle, what does the herniation contain? Select one: * Cerebrospinal fluid, meninges and spinal cord/cauda equina * Cerebrospinal fluid and meninges * Cerebrospinal fluid and spinal cord/cauda equina * Cerebrospinal fluid

Answer 14

Cerebrospinal fluid, meninges and spinal cord/cauda equina

Question 15

In relation to obstetric brachial plexus injury, Erb’s palsy results in loss of motor innervation of the deltoid, supraspinatus, infraspinatus, biceps and brachilais muscles. At what level are nerve roots injured in Erb’s palsy? Select one: * C6 & C7 * C5 & C6 * C8 & T1

Answer 15

Erb’s palsy is caused by damage to the nerve roots C5 & C6. The loss of motor function to the deltoid, supraspinatus, infraspinatus, biceps and brachilais muscles resluts in the classic waiter’s tip posture (internal rotation of the humerus secondary to unopposed subscapularis muscle action).

Question 16

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Answer 16

* The patellar ‘always’ dislocates laterally. This is due to the pull of the quads tendon being more lateral than medial and that the lateral femoral condyle is more likely to be deficienct (shallower) compared to the medial side. * The often tense effusion in the joint consists of blood from the bleeding soft tissue structures torn as the patellar dislocates (medial patello-femoral ligamanet; medial retinaculum). * Approxiamtely 20% of patients who suffer a patella dislocation will sustain a second one.

Question 17

* You are an FY working in A&E when you are asked to review a 12 year old girl with a very sore and swollen knee following a twisting injury she sustained when she jumped off a wall. She tells you that she landed awkwardly and ‘saw her knee go out of place’. It went back in when she straightened it out. It’s now very sore and swelled up immediately. You suspect the patient has dislocated her patella, although it spontaneously relocated when the knee was straightened. * Where is the clue in this question that the swelling is likely to be from bleeding?

Answer 17

The clue here is that the knee very quickly became swollen. This is more likely to be down to bleeding, which happens quickly, as compared to a synovial fluid collection which tend to develop over several hours/days.

Question 18

The problem of intoeing is usually manageed surgically, with operations when the child is as young as possible, to correct the deformity and allow normal development of the lower limbs. Select one: True False

Answer 18

In‐toeing refers to a child who, when walking and standing will have feet that point toward the midline. The abnormality is often exaggerated when running and children are felt by their parents to be clumsy and wear through shoes at an alarming rate. * The problem is rarely managed surgically.

Question 19

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Answer 19

The correct answer is: Examine the right hip joint Hip pathology can present with knee symptoms and this can occur with adults and particularly in chidren who find it difficult to localise symptoms. This is because of shared innervation between the two joints. Therefore, if a patient presents with knee pain but their knee examination is normal, ALWAYS examine the hip above and consider a hip pathology/diagnosis.

Question 20

In relation to Slipped Upper Femoral Epiphysis (SUFE), which group is particularly susceptible to developing this paediatric hip condition? Select one: * a. Underweight pre-pubertal adolescent boys. * b. Underweight boys aged 2 - 7. * c. Overweight boys aged 4 - 9. * d. Overweight pre-pubertal adolescent boys.

Answer 20

Slipped Upper Femoral Epiphysis is a condition mainly affecting overweight pre‐pubertal adolescent boys where the femoral head epiphysis slips inferiorly in relation to the femoral neck. Girls are less commonly affected and hypothyroidism or renal disease may predispose to SUFE. Perthes disease of the hip is more common in active boys with a short stature aged between 4 - 9.

Question 21

Anterior knee pain (patello-femoral dysfunction) is common in adolescence, especially in girls. The aetiology is unclear. From the list below, select the factors which we think may play a part in the development of patello-femoral dysfunction: Select one or more: * a. Genu varus (bow legged) * b. Diabetes mellitus * c. Ligamentous laxity * d. Genu valgus (knock knees) * e. Muscular imbalance

Answer 21

The correct answers are: Ligamentous laxity, Genu valgus (knock knees), Muscular imbalance * Anterior knee pain (patellofemoral dysfunction) is common in adolescence, especially in girls. The aetiology is unclear and may be due to muscle imbalance, ligamentous laxity and subtle skeletal predisposition (genu valgum, wide hips, femoral neck anteversion

Question 22

The most common cause of paediatric hip pain is: Select one: a. Developmental dysplasia of the hip (DDH) b. Septic arthritis c. Perthes disease d. Transient synovitis

Answer 22

Transient synovitis of the hip

Question 23

Normal developmental milestones: Knowledge of the normal neurological devolopment timeline is important as it allows one to recognise developmental delay, a possible sign of neuromuscular disease. By roughly what age should a child be able to speak a few words? Select one: * a. 9-12 months * b. 3-6 months * c. 6-9 months * d. 12-18 months

Answer 23

Usually by 12 months (option A 9-12 months)