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MSK > Paediatric Orthopaedics > Flashcards

Paediatric Orthopaedics Flashcards

1
Q

What is Osteogenisis Imperfecta

A

a.k.a brittle bone disease

Defect in maturation and organisation of type 1 collagen

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2
Q

Can be autosomal dominant or recessive

Recessive is more fatal

A

Osteogenisis imperfecta

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3
Q

Clinical signs of osteogenesis imperfect

A

Blue sclera
Thin bones - prone to fractures
Short stature
Hearing loss

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4
Q

Genetic Disorder leading to short stature which has >300 different types

A

Skeletal Dysplasias

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5
Q

Most common type of skeletal dysplasia

A

Achondroplasia

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6
Q

Most common symptoms of achondroplasia

A 
Prominent forehead
Lax joints
Widened nose
Disproportionate or proportionate limb growth
Normal mental development
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7
Q

Treatment/Management of skeletal dysplasias

A

Supportive management
Prevent spinal compression
Growth hormones rarely effective

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8
Q

Patient displays double jointed party tricks
Prone to soft tissue injuries and dislocations
5% of population

A

Generalised Joint Laxity

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9
Q

Autosomal dominant mutation in the fibrillin gene

A

Marfan’s Syndrome

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10
Q

Features associated with Marfan’s

A 
Disproportionately long limbs
Ligament laxity
Scoliosis
High arched palate
Retinal detachment 
AAA
Cardiac Valve abnormalities
Pneomothorax's
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11
Q

Heterogeneous Autosomal Dominant Disease causing abnormal elastin and collagen formation

A

Ehler’s Danlos Syndrome

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12
Q

Clinical Features of Ehler’s Danlos Syndrome

A

Joint instability
Scoliosis
Hyper mobility
Vascular Fragility

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13
Q

What is caused by trisonomy 21?

A

Down’s Syndrome

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14
Q

What is a big risk factor of Down’s syndrome?

A

Instability of the atlanto-axial joint in cervical spine

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15
Q

Rare and mainly x-linked conditions

A

Muscular Dystrophies

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16
Q

Defect in the dystrophin gene involved in calcium transport, resulting in muscle weakness, which is noticed in males when they first start walking

A

Duchene Muscle Dystrophy

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17
Q

How do you confirm the diagnosis of Duchene Muscular Dystrophy?

A

Raised Serum Creatinine Phosphokinase

Abnormal muscle Biopsy

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18
Q

Similar to Duchene’s but with a longer life expectancy (about 30-40 years)

A

Becker’s Muscle Dystrophy

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19
Q

Occur due to abnormal or deficient motor neuron signals to skeletal muscle.

A

Neuromuscular disorders

Can be due to brain or spinal cord defect OR nerve problems

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20
Q

Caused by insult to immature brain before the age of three. Can be spastic, ataxic, athetoid or dystonic and results in paralysis

A

Cerebral Palsy

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21
Q

Define Spastic CP

A

Most common form

Due to damage of motor cortex

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22
Q

Damage in the cerebellum resulting in affected co-ordination

A

Ataxic Cerebral Palsy (CP)

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23
Q

Uncontrolled writhing caused by basal ganglia and pyramidal tract damage

A

Athetoid Cerebral Palsy

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24
Q

Dystonic CP

A

Damage to Basal Ganglia resulting in repetitive movements

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25
Q

Treatments for cerebral palsy

A

Botox injections
Neurosurgical procedures
Intrathecal Baclofen

26
Q

Congenital Disorder of the Spinal Cord, resulting in two halves of the posterior vertebral arch failing to fuse

A

Spina Bifida

27
Q

Mild Spinal Bifida - Name and Symptoms

A

Referred to as Spinal Bifida Occulta
Appears as a skin tag, lipoma or hairy patch
Causes tethering of spinal cord and roots creating high arches and toe clawing

28
Q

Name of Severe Spina Bifida

A

Spinal Bifida Cystica

29
Q

Symptoms of Spina Bifida Cystica

A

Herniation of the meninges with no skin covering which may or may not come with motorneuron deficits

30
Q

Herniation of meninges only with no neurological defect

A

Meningiocele

31
Q

Herniation of meninges and cauda-equina roots with neurological effect

A

Myelomeningiocele

32
Q

Treatment of spina bifida

A

Closure within 48 hours

33
Q

A viral infection which affects motor anterior horn cells in the spinal cord and brainstem

A

Polio

34
Q

How does the polio virus enter the body?

A

Via the GI tract

35
Q

Most common limb malformation where 2 digits fused - usually digits 3 and 4

A

Syndactyly

36
Q

When an extra digit is formed

A

Polydactyly

37
Q

One of the most common limb deficiencies which causes part or all of the fibula to be missing.

A

Fibular Hemimelia

38
Q

Erb’s Palsy

A

Caused by damage to brachial plexus at C5 and C6 level causing inner rotation of humerus
Damage of biceps, deltoid and brachialis

39
Q

Klumpke’s Palsy

A

Rare
Low brachial plexus injury in C8 and T1
Paralysis of intrinsic hand muscles finger flexion and potential horner’s syndrome

40
Q

A cause of intoeing and knock knees due to excessive femoral neck anteverions

A

Femoral Neck Anteversion

41
Q

Internal tibial torsion

A

Bone rotated medially, resulting in in-toeing. Normal variation so is usually ignored.

42
Q

Genu Varum

A

Growth disorder of the medial proximal tibial physis

a.k.a Blount’s Disease

43
Q

Genu Valgum

A

Can be idiopathic or caused by rickets, trauma, neurofibromatosis and echondromatosis.

44
Q

Higher risk of medial osteoarthritis

A

Genu Varum

45
Q

What does rigid flat foot imply?

A

Tarsal Coalition OR some other cause of bone abnormalities.

46
Q

Self limiting inflammation of the synovial hip joint caused by a viral upper respiratory tract infection which is the commonest cause of hip pain in children

A

Transient Synovitis of the hip

47
Q

Dislocation of Subluxation of the femoral head during perinatal period resulting in affected hip development

A

Developmental Dysplasia of the Hip

48
Q

Treatment for Developmental Dysplasia of the Hip

A

Paulik Harness full time for 6 weeks and then part time for a further 6 weeks

49
Q

Test for diagnosing Developmental Dysplasia of the Hip

A

Barlow’s Test: Flex and move hip posteriorly, and it should dislocate.
Ultrasound: Will detect shallow acetabulum

50
Q

Treatment of Transient Synovitis of the hip

A

NSAIDs and rest

May require surgical drainage if severe or an abscess forms

51
Q

Idiopathic Osteochondritis of the femoral head which is common in boys aged 4-9

A

Perthe’s Disease

52
Q

Condition common in overweight, pre-pubescent boys, which can present as KNEE pain!

A

Slipped Upper Femoral Epiphyses

53
Q

What is Osgood-Schlatters?

A

Inflammation of a growing tubercle where the tendon attaches, caused by repetitive strain
a.k.a apophysitis

54
Q

Sinding-Larson-Johanssen Disease

A

Inflammation of the patellar inferior pole

55
Q

Anterior knee pain is more common in males or females?

A

Females

Not sure why, theory is due to hip development and widening

56
Q

What can a patellar dislocation result in>

A

Osteochondral fractures - this is where fragments of the bone may break off and require arthroscopic

57
Q

Osteochondritis which causes bone fragments to break off; most commonly in the knee.
Presents with poorly localised knee pain and effusions

A

Osteochondirits Dissecans

58
Q

Talipes Equinovarus

A

Club Foot

Congenital deformation of the navicular, talus and clacaneous joints resulting in abnormal alignment

59
Q

Tarsal Coalition

A

Abnormal bridge between talus-calcaneous or navicular-calcaneous resulting in painful flat foot.
May require surgery

60
Q

An idiopathic lateral curvature of the spine which ranges from mild to severe and causes lung problems and pain

A

Scoliosis

61
Q

Surgical Treatment of Scoliosis

A

Requires vertebral fusions and long rods to connect posterior elements
High risk of spinal cord injury in greater curves

62
Q

Slippage of a vertebral disc over another, due to either a stress fracture or congenital disorder, resulting in low back pain, radiculopathy and paradoxical ‘flat back’, resulting in waddling gait

A

Spondylolithesis

MSK (5 decks)

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