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MSK > Childrens Orthopaedics- Congenital and Neuromuscular disorders > Flashcards

Childrens Orthopaedics- Congenital and Neuromuscular disorders Flashcards

1
Q

name 4 neuromusclar disorders

A

cerebral palsy
tip toe walking
duchenne musclar dystrophy
high arch (cavus) foot

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2
Q

name 4 congenital disorders

A

club foot (CTEV)
rocker bottom foot (CVT)
neurofibromatosis
skeletal dysplasia

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3
Q

what is congenital talipes equinovarus

A

club foot

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4
Q

what is club foot

A

congenital disorder, birth defect causing abnormal foot posture

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5
Q

how can you remember the features of clubfoot

A 
CAVE 
C- cavus (high arch)
A- adductus (foot turned inwards)
V- varus 
E- equinus (inability to dorsiflex ankle due to fixed achilles)
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6
Q

who gets club feet and in which feet

A

2:1 boys

50% bilateral

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7
Q

what causes clubfoot

A 
majority idiopathic 
can be associated with:
spina bifida (myeloleningocoele)
diastrophic dwarfism 
tibial hemimelia
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8
Q

what is postural talipes

A

is normal- when a normal foot that has been held in a deformed position in the uterus. Postural talipes is correctable with gentle passive dorsiflexion of the foot

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9
Q

how do you distinguish club foot from postural talipes

A

postural talipes can dorsiflex ankle

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10
Q

how do you treat clubfoot

A

serial casting (posenti method) +/- achilles tenotomy

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11
Q

what is congenital vertical talus

A

rocker bottom feet

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12
Q

what is rocker bottom feet

A

birth defect- irreducible dislocation of talus on navicular

  • round plantar surface
  • equinus hindfoot
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13
Q

what can rocket bottom feet be associated with

A

more serious conditions

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14
Q

do both club foot and rocker bottom foot have fixed ankle equinus

A

yes

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15
Q

what is neurofibromatosis

A

congential disorder affecting the extremities, spin (scliosis), skin (neurofibromas)

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16
Q

what is the mot common type of neurofibromatosis

A

NF1

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17
Q

describe the genetics of neurofibromatosis

A

autosomal dominant, NF1 gene (neurofibromin) Ch17

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18
Q

what are the features of neurofibromatosis

A 
cafe au lait spots 
neurofibromas
freckling axilla/ inguinal region 
optic glioma 
lisch nodules 
cortical thinning/ pseudoarthritis
19
Q

what are skeletal dysplasias

A

congenital disorders involving bone and cartilage

20
Q

what are the features of skeletal dysplasias

A 
shortening of the involved bone (can affect any bone at any part)
short stature (<2SD)
can be proportionate/ disproportionate
21
Q

what is achondroplasia

A

form of skeletal dysplasia cause by a mutation in the fibroblast growth factor receptor 3 FGFR3

22
Q

how is the inheritance pattern of achondroplasia

A

autosomal dominant

23
Q

what are the features of achondroplasia

A 
normal trunk/ short limbs 
frontal bossing (prominent forehead)
genu varum 
normal intelligence 
motor delay
24
Q

how do you get neuromuscular disorders

A

are acquired throughout life- not genetic

25
Q

what is cerebral palsy

A

non progressive neuromuscular disorder caused by injury to the immature brain (<2 years)

26
Q

what can cause cerebral palsy

A

prematurity, perinatal (infection/ anoxic injuries/ meningitis)

27
Q

what are the features of cerebral palsy

A

upper muscular neuropathy, muscle weakness/ spasticity

early- abnormal muscle forces, dynamic deformity

late- contractures, fixed deformity, dislocation

28
Q

what is clasp knife spascity

A

when muscles have a high tone, will resist a certain movement and then give

29
Q

what is the functional classification of cerebral palsy

A

walking and non walking

30
Q

what are the types of cerebral palsy

A

spastic, dyskinetic, ataxia

31
Q

what are the possible treatments for cerebral palsy

A

treat centrally- benzodiazepines, baclofen

surgery- dorsal rhizotomy

botulinum toxin to paralyse muscles

32
Q

what is duchenne muscular dystrophy

A

commonest MD, inherited disorder causing progressive muscle weakness

33
Q

how is duchenne muscular dystrophy inherited

A

x linked recessive (1/3rd spontaneous)

34
Q

what is the pathophysiology of duchennes muscular dystrophy

A

abscence of dystrophin protein causes replacement of muscle with fibrofatty tissue

35
Q

when does duchenne muscular dystrophy present

A

2-5 years

36
Q

what is the treatment for duchennes muscular dystrophy

A

keep ambulatory, decrease contractures

37
Q

what are the features of duchennes muscular dystrophy

A

muscles weakness (proximal> distal)
clumsy walking
positive gowers sign (walking hands up thighs to stand)
scoliosis

38
Q

how do you diagnose duchennes musclar dystrophy

A

creatine phospholinase CPK/ muscle biopsy (absence dystropin)

39
Q

what is the prognosis for duchennes musclar dystrophy

A

10 y/o assisted walking
15 wheelchair
LE around 30 y/o

40
Q

what is pes cavus

A

high arched foot

41
Q

what is the morphology of pes cavus

A

elevated longitundinal arch and varus hindfoot

42
Q

what can cause cavus feet

A

idiopathic/ familial

2/3rds due to neurological disorder: polio, cerebral palsy, myelomeningocoele, spinal cord injury

43
Q

how do you asses cavus feet

A

x rays/ coleman block test

44
Q

what is the management for cavus feet

A

soft tissue deformity (flexible)- conservative or soft tissue surgery

fixed deformity- conservative management and bony (osteotomies) surgery

MSK (71 decks)

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