Orthopaedics unit 3 orthopaedic conditions in childhood - deck 2

Question 1

Abnormal developments of the spine in what months ? may result in the abnormal formation of the spinal cord and vertebrae - resulting in spina bifida

Answer 1

The first 3 months of a newly forming baby

Question 2

What is Spina bifida ?

Answer 2

Spina bifida is a congenital disorder where the two halves of the posterior vertebral arch fail to fuse

Question 3

What is the 2 main forms of spina bifida ?

Answer 3

1. Spina bifida occulta - this is the mildest form of spina bifida
2. Spina bifida cystica - this is the more severe form

Question 4

Describe what diastamatomyelia is

Answer 4

• This is tethering of the spinal cord to the higher lumbar vertebrae during growth.

Question 5

What is spina bifida occulta and how does it affect someone with it ?

Answer 5

This may be a minor bony abnormality which affects 2% of the population.

It is usually of no significance but some people with it may develop mechanical backache and rarely some may get diastamatomyelia.

Question 6

What is spina bifida cystica and describe the difference in the 2 herniations that can be present

Answer 6

Spina bifida = when the neural plate tissues open with little or no skin or bony cover 2 herniations may occur:

1. The nerve tissue may be covered by a cyst (a meningocele) this is when the meninges alone herniate
2. or the nerve tissue may be incorporated in the cyst wall (a meningomyelocele) when the spinal cord or cauda equina herniate with the meninges

Question 7

What condition do many children with spina bifida cystica also have and what can this condition lead to?

Answer 7

• Hydrocephalus - an abnormal increase in the amount of fluid in the brain
• This condition can lead to mental retardation and increase in size of the head

Question 8

All children with spina bifida cystica die at birth - T or F?

Answer 8

False - many die at birth but some survive and undergo surgery to close the lesion on their back

Question 9

The children with spina bifida cystica which survive at brith and successfully have the lesion closed, what sort of problems are they expected to have ?

Answer 9

• Paralysis
• Growth deformities through muscle imbalance
• Incontinence.
• Many are mentally retarded

Question 10

What body part do many children with spina bifida cystica need surgery on ?

Answer 10

Many of the children need early surgery to their feet to maintain a functional shape.

Question 11

Why should every effort be made to keep children with spina bifida cystica mobilsed until adolescence and what do they often use to help walk?

Answer 11

• So that they grow to a reasonable size
• Splints and hand-held aids often used to help mobilise

Question 12

As someone reaches adolescence what do many of them move to using to mobilise and why ?

Answer 12

A wheelchair - because it is easier for them socially

Question 13

What is cerebral palsy ?

Answer 13

This is a condition caused by an abnormality of the brain. This is often caused by damage to the brain at birth and results in delayed, or arrested development of the nervous and musculoskeletal systems

Question 14

What is the typical presentation of someone with CP

Answer 14

• Uninhibited spinal reflexes but lack the co-ordination and purpose of movement normally controlled by the brain.
• This results in a spastic type of paralysis.

Question 15

Describe how the muscles are affected in people with cerebral palsy:

Answer 15

Some muscles contract strongly in an uncoordinated way (spastic) whilst others are very weak and flaccid.

Question 16

The imbalances of the muscles (spasticity in some muscles and weakness in others) in people with CP can result in the development of what abnormalities ?

Answer 16

Abnormal muscle and bone growth, with secondary deformities of joints.

Question 17

Describe the following common patterns of CP presentation:

• Hemiparesis
• Paraparesis
• Quadraparesis

Answer 17

• Hemiparesis = When one arm and the leg on the same side are affected
• Paraparesis = both legs are affected
• Quadraparesis = all for limbs are affected

Question 18

What associated conditions do children with CP often have ?

Answer 18

Mental retardation

Blind &/ or deaf

Question 19

Appreciate this about CP patients:

It is important to recognise that some people have very minor degrees of spasticity which may only affect one muscle group. A common sign is toe-walking in adolescence. Examination reveals a calf muscle spasticity and some sufferers may need tendo-achilles lengthening before growth ceases.

Question 20

What are some of the treatment options for CP?

Answer 20

• Deformities can be minimised by careful physiotherapy
• Spintage may be used
• Careful use of surgery, to either lengthen tight muscles or to denervate them or (occasionally) to move them

Question 21

Why should splintage be used with caution in the treatment of CP ?

Answer 21

Overzealous splintage can lead to increased muscle spasm and, ultimately, deformity

Question 22

Define what scoliosis is

Answer 22

This is a abnormal curvature of the spine with a rotatory abnormality of the vertebrae

It is a 3-D deformity based on an abnormal lordosis of the spine, which leads to buckling and twisting of the vertebral column as a result of the action of muscles and gravity.

Question 23

What are the main causes of scoliosis ?

Answer 23

It may be caused by congenital abnormalities of the vertebrae or by neuromuscular imbalance but most cases have no known causes (i.e. they are idiopathic).

Question 24

Scoliosis is more common in m or f ?

Answer 24

Females - think julia

Question 25

What is the principle disturbances caused by scoliosis ?

Answer 25

• Cosmetic - poor appearance due to spinal contracture
• But it does not usually cause physiological disturbances

Question 26

Describe the typical presentation of a child with scoliosis

Answer 26

• The child usually complains of the twisting of the ribs which causes a hump on one side of the shoulder.
• Girls may also complain that their skirts hang crookedly.
• It may be painful although this is usually secondary to the anxiety and distress caused by what is commonly known as a sinister condition.

Question 27

Describe the management of scoliosis

Answer 27

Not all curves progress (get worse), if the curve is progressive, or is causing distress, treatment should be offered by surgical correction

Note - This is very complex surgery

Question 28

Who should all children with progressive scoliosis or those demanding treatment be referred to ?

Answer 28

All should be referred for a specialist opinion

Question 29

A limp at any age must be taken seriously, state the main causes of limp in childhood which must be excluded in each of the following age ranges:

• From birth (i.e. always should be ecluded)
• 4-10 years old
• 10-15 years old

Answer 29

• From birth (i.e. always should be ecluded) - CDH and infection of the hip
• 4-10 years old - Perthes disease
• 10-15 years old - Slipped upper femoral epiphysis (SUFE)

Question 30

What is perthes disease?

Answer 30

It is an idiopathic (unknown cause) osteochondirits (fragmentation of the bone and underlying cartilage) of the femoral head epiphysis.

The condition is thought to be an avascular necrosis of the growing femoral head. The cause, nevertheless, is obscure. Eventually the head will re-vascularise and re-ossify but it may be enlarged and deformed

Question 31

Define osteochondirits

Answer 31

fragmentation of the bone and overlying cartilage

Question 32

Between what ages does perthes disease occur ?

Answer 32

4-9 hence suspect it in those between 4-10 (as they are 9 until turn 10 and this says between 4&10)

Question 33

Is perthes disease more common in males or females ?

Answer 33

Males

Question 34

Does perthes disease usually occur unilaterally or bilaterally ?

Answer 34

• Usually unilaterally
• but 20% of cases may occur bilaterally

Question 35

What is the incidence rate of perthes disease?

Answer 35

up to 5 per thousand children

Question 36

Describe the typical presenting features of a child with perthes disease?

Answer 36

A painful limp followed by a slow recovery

Question 37

Describe the changes seen on x-ray and US in children with perthes disease

Answer 37

• Radiologically the femoral head may be normal on first presentation but it fragments to a greater or lesser degree. Radiographs repeated after a month may show previously unrecorded changes.
• US reveals excess fluid in the hip joint.

Question 38

Describe the management of a child with perthes disease

Answer 38

The strategy of treatment is to maintain the head concentrically within the acetabulum until the natural process of the disease runs its course.

Minor degrees of the condition, involving up to half of the femoral head, need no treatment

In older children, when the condition affects the femoral head, the prognosis is less good. The child will return to normal in the short term, but will be prone to secondary osteoarthritis in early middle age.

In severe cases, careful follow-up with periods of traction to alleviate symptoms of pain and limp is probably all that can be done to help these children.

Question 39

What is a slipped upper femoral epiphysis (SUFE)?

Answer 39

It is a condition in which there is a slippage of the epiphysis of the femoral head on the femoral neck so that the head is abnormally tilted.

Question 40

Who is most commonly affected by SUFE’s ?

Answer 40

Boys

Question 41

What boys and girls are most likely to develop a SUFE?

Answer 41

Boys of around 12 years old who are sexually immature (pre-pubertal) for their age and in girls who are a little older and have recently undergone an adolescent growth spurt.

Question 42

What are the typical presenting features of a child with a SUFE?

Answer 42

• Child has a painful limp, however this limp may not be particularly painful in some cases
• Pain radiating to the knee
• The slip may occur acutely or it may be preceded by many months of discomfort without clinical or radiological signs

Question 43

Why can hip pathology present as knee pain?

Answer 43

Due to the sensory distribution of the obturator nerve

Question 44

What must any child presenting with knee pain also have examined (along with the knee obv)?

Answer 44

Their hip

Question 45

What condition should all young adolescents with a painful hip must be regarded as having until clinically and radiologically excluded ?

Answer 45

SUFE

Question 46

What additional xray view must be obtained to reliably exclude a SUFE in a child?

Answer 46

A lateral view as minor degrees of slippage may be missed otherwise

Question 47

What is the treatment of SUFE’s?

Answer 47

Treatment is surgical.

If the slippage is minor, the hip should be pinned in its new deformed position.

If the slippage is major, a gentle attempt to replace the head on the neck by manipulation may be attempted although the risk of avascular necrosis is high.

The child’s other hip should be observed, using radiography at regular intervals, and pinned if any suspicion of slippage arises. The pins are best removed after fusion of the epiphysis at around eighteen years of age.