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Paediatrics Flashcards

1
Q

What is osteogensis imperfecta? What are bones like with this condition?

A

defect in maturation and organization of T1 collagen

Bones are thin with thin cortices and osteopenic

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2
Q

osteogensis imperfecta is an autosomal - condtion.

A

dominant

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3
Q

osteogensis imperfecta is characterised by which 3 signs?

A

Children with osteogensis imperfecta shop at BHS:
Blue sclera
Hearing loss
Short stature

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4
Q

Management of OI?

A

No cure
COnservative: splintage, traction or surgical stabilisation
Maintain health
Vitamin D supplements
PAMIDRONATE - type of biophosphate to prevent loss bone mass

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5
Q

What is skeletal dysplasia?

A

Short stature

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6
Q

What is achondroplasia?

A

disproportionately short limbs
prominent forehead
widened nose

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7
Q

What are the features associated with Marfan’s syndrome?

A 
High arched palate
Scoliosis
Pectus excavatum
eye Problems 
aortic aneurysm
cardiac valve incompetance
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8
Q

What is marfan’s syndrome?

A

mutation fibrillin gene

tall with disproportionately long limbs and ligamentous laxity

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9
Q

What is Ehlers-Danlos syndrome?

A

Abnormal elastin and collagen

Hypermobility, vascular fragility, easy bruised, joint instability, scoliosis

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10
Q

MSK manifestation down syndrome?

A

short
ligamentous laxity
recurrent dislocation

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11
Q

Muscular dystrophies are rare and only affect boys

A

T

usually x linked recessive

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12
Q

What is Duchenne muscular dystrophy?

A

Defect in dystrophin gene involving calcium transport

muscle weakness

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13
Q

How is Duchenne muscular dystophy diagnosis confirmed?

A

raised serum phosphokinase

abnormalities muscle biopsy

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14
Q

Duchenne muscular dystophy is obvious at birth

A

F

muscle weakness may only be noticied when child walks with difficulty

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15
Q

Duchenne muscular dystophy can cause cardiac and resp failure

A

T

death typical early 20s

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16
Q

What is the cause of cerebral palsy?

A

Insult to brain before or after birth

genes, brain malformation, early pregnancy, intrauterine infection

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17
Q

Incidence of brachial plexus injury is around 2/100

A

F

2/1000

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18
Q

brachial plexus injury commonly arises in large babies

A

T

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19
Q

What is Klumpke’s palsy?

A

lower brachial plexus injry caused by forceful adduction

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20
Q

Consequences of klumpke’s palsy?

A

paralysis of intrinsic hand muscles - fingers typically flexed WAITER’S TIP POSTURE
Horner’s syndrome (drooping eyelid and bloodshot eyes)

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21
Q

At birth children have - knees

A

varus

bow legs

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22
Q

At 14 months the legs become..

A

straight

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23
Q

Children develop - knees at age 3 which gradually become less extreme at age 7/9

A

valgus (knock knees)

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24
Q

Lower limb variations are abnormal in children if:

A

greater than 2 yrs old
pain
unilateral

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25
What is femoral neck anteversion? What does it cause?
femoral neck slightly pointing forwards | in toeing
26
femoral neck anteversion is a surgical emergency
F | part of normal anatomy
27
At birth all feet are flat
T
28
What is mobile flat footedness?
Only present on weight bearing | flattened medial arch forms with dorsiflexion of great toe
29
rigid type flat foot is normal and requires no intervention
F | suggests underlying bony abnormality
30
Curly toes will correct without intervention
T
31
What is the ortolani manouvere?
For dislocted hip: | abduction and anteromedially directed force causes reduction
32
What is the Barlow manouvere?
To dislocate the hip: adduction and posterior force
33
What is DDH?
developmental dysplasia of hip | dislocation or subluxation of femoral head during perinatal period
34
RIsk factors for DDH?
Born in BREACH position (bottom first) FH THESE BABIES HAVE ROUTINE ULTRASOUND
35
X rays can be used to diagnose DDH
F | femoral head unossified until 4-6 months
36
How is DDH treated in patients <2yrs old
``` PAVLIK HARNESS (high success rate!) maintaining reduction via abduction and comfortable flexion used for 6 wks full time + 6 wks part time ```
37
Why is early diagnosis key in DDH?
over 2 yrs old NEVER NORMAL | open reduction needed
38
What is the commonest cause of hip pain in childhood?
Transient synovitis of hip
39
Transient synovitis commonly occurs shortly after an upper resp tract infection
T
40
How does transient synovitis commonly present?
Limp/ relucatance to weight bear Restricted range motion low grade fever
41
Treatment transient synovitis?
NSAIDs and rest self limiting if pain not resolved within few weeks check another cause pain
42
What is Perthes disease?
Idiopathic osteochondritis of femoral head
43
Perthes disease pathology?
Femoral head transiently loses its blood supply | Resulting in necrosis, sclerosis, fragmentation, reossification and remodelling
44
Perthes: Affected children present with pain and a limp, most cases are unilateral
T
45
What is the FIRST CLINICAL SIGN of perthes disease?
Loss of internal rotation
46
Phenotype of perthes disease patient?
Small hyperactive boy
47
Treatment perthes disease?
Attempt influence shape femoral head activity modification bracing
48
What is SUFE?
slipped upper femoral epiphyses | femoral head slips inferiorly in relation to femoral neck
49
What is the pathology in SUFE?
``` growth plate (physis) not strong enough to support body weight femoral epiphyses slips due to strain ```
50
Typical phenotype SUFE?
black overweight adolescent boy | Hani'el is a sufi
51
How does SUFE present?
Limp Pain midgroin CAN PRESENT PURELY WITH KNEE PAIN
52
Why can sufe present with only knee pain?
Obturator nerve supplies femur and knee
53
What is the PREDOMINANT clinical sign in sUFE?
Loss of internal rotation of the hip
54
Adolescent body weight increase and sporting activities can commonly lead to what?
knee extensor mechanism pain
55
Patellar tendonitis is self-limiting
T
56
What is the aetiology of anterior knee pain (patellofemoraldysfunction)
muscle imbalance ligamentous laitiy genu valgum, wide hips, femoral neck anteversion ADOLESCENT GIRLS
57
anterior knee pain (patellofemoraldysfunction) is self limiting
T
58
younger patients have a lower chance of healing a meniscal tear
F | higher chance
59
What is the deformity in talipes equinovarus?
aka clubfoot Ankle plantar flexion (equinous) Supination of forefoot Varus alignment of forefoot
60
girls are twice as likely to get clubfoot
F | boys are twice as likely to get clubfoot
61
What is the ponseti technique?
Plaster cast to correct talipes equinovarus deformity
62
talipes equinovarus can result in fixed deformity if presentation is delayed
T
63
80% of children with a ponseti plaster require tenotomy of achilles tendon
T
64
Back pain in children and adolescents is not a red flag sign
F | IT TOTALLY IS
65
In patients presenting with SUFE approximately 1/3 of cases are bilateral
T
66
If a flat foot is described as rigid,what is a possible cause of the flat foot?
tarsal coalition -where the bones of the midfoot have an abnormal bony or cartilaginous connection
67
If a flat foot is described as dynamic, present when the foot is weightbearing only, a type of mobile/flexible flat foot) what is a possible cause of the flat foot?
ligamentous laxity
68
Patellar dislocations, are fairly common and the patient will usually be able to tell you that their knee cap moved round to the outside of their knee. They spontaneously relocate when the knee is straightened.
T
69
Developmental dysplasia of the hip affects more girls than boys
T
70
The patellar 'always' dislocates in which direction?
laterally
71
20% of patients who suffer a patella dislocation will sustain a second one
T

MSK (8 decks)

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