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Pediatric PT > Arthrogryposis and Osteogenesis Imperfecta > Flashcards

Arthrogryposis and Osteogenesis Imperfecta Flashcards

1
Q

What is Arthrogryposis Multiplex Congenita?

A

A congenital, non-progressive neuromuscular syndrome that has degeneration of the anterior horn cell

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2
Q

When is Arthrogryposis Multiplex Congenita usually diagnosed?

A

insult during the first trimester while in utero (presence of club feet)

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3
Q

What is the clinical presentation fo Arthrogryposis Multiplex Congenita?

A
  • 2+ contractures
  • weakness
  • fibrosis
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4
Q

What is the etiology of Arthrogryposis Multiplex Congenita?

A

unknown

Many possible contributors:
- Hyperthermia
- Prenatal viral infection
- Mother/fetus vascular compromise
- Uterine vascular compromise
- Septum in the uterus

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5
Q

What disorders is Arthrogryposis Multiplex Congenita commonly associated with?

A

neurogenic and myopathic disorders

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6
Q

Distal Arthtogryposis is associated with what error?

A

chromosomal error

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7
Q

What is the most common form of arthrogryposis?

A

Amyloplasia

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8
Q

What is the presentation of amyloplasia?

A
  • decrease in fetal movement
  • lack of growth and muscular development
  • contractures/deformities
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9
Q

Distal arthrogryposis is inherited as a autosomal (recessive/dominant) trait.

A

autosomal dominant

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10
Q

Some forms of Arthrogryposis Multiplex Congenita have been mapped to what chromosomes?

A

5, 9, 11

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11
Q

(true/false) The majority of Arthrogryposis Multiplex Congenita cases are genetic

A

FALSE

–> hence, why they are difficult to detect early

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12
Q

What type of ultrasound will help detect anomalies and decreased fetal movement?

A

Level II ultrasound

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13
Q

What lab value helps detect maternal infection during the first 6 months in utero?

A

immunoglobulin

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14
Q

(true/false) Muscles are formed NORMALLY prenatally in those with arthrogryposis but are replaced by fibrous and fatty tissue during fetal development

A

true

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15
Q

If an infant is diagnosed in utero with arthrogryposis, how can a mother help exercise the baby?

A
  • deep breathing
  • light exercise
  • daily caffeine intake
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16
Q

(true/false) The chance of arthrogryposis at joints is almost even.

A

true

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17
Q

Describe Presentation I of arthrogryposis.

A

Hips: flexion; dislocated

Knees: extension

Clubfeet (equinovarus)

Shoulders: internally rotated

Elbows: flexed

Wrists: flexed; ulnarly deviated

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18
Q

Describe presentation II of arthrogryposis?

A

Hips: abducted; externally rotated

Knees: flexion

Clubfeet

Shoulders: internally rotated

Elbows: extended

Wrists: flexed and ulnarly deviated

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19
Q

What surgical method is most commonly used to treat clubfeet?

A

Ponseti Method
–> involves constant serial casting

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20
Q

What is the standing goal in infants with arthrogryposis during the first year?

A

Standing for 2 hours/day

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21
Q

How often should AFOs be worn during the day in an infant with arthrogryposis?

A

22 hours/day

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22
Q

What are the stretching parameters for infants with arthrogryposis?

A

3-5 sets x 3-5 reps
20-30 second hold

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23
Q

What is the primary goal for preschoolers with arthrogryposis?

A

improve independence

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24
Q

What are the primary interventions for preschoolers with arthrogryposis?

A

stretching and bracing

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25
Q

When does efficient ambulation become an issue in children with arthrogryposis?

A

school age –> alternate means of mobility may be necessary

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26
Q

What are the primary goal for school aged children with arthrogryposis?

A
  • education
  • independent mobility
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27
Q

(true/false) Splinting and stretching are not important for school age children with arthrogryposis

A

FALSE (it is)

28
Q

(true/false) Individuals with arthrogryposis who needed assistance with ADL’s continued to require assistance into adulthood

A

true

29
Q

What can be an issue during transitioning to adulthood in those with arthrogryposis?

A
  • Pain
  • activity limitations due to decreased ROM and strength
30
Q

(true/false) With arthrogryposis, the number of painful regions was associated with decreased mobility

A

true

31
Q

What is osteogenesis imperfecta?

A

Abnormality in type I collagen that affects bone formation

32
Q

What is the most severe classification of osteogenesis imperfecta?

A

osteogenisis imperfecta congenita

high mortality rate and poor prognosis

33
Q

What are characteristics of osteogenisis imperfecta congenita?

A
  • Numerous fractures at birth
  • Dwarfism
  • Bowing of long bones
  • Blue Sclerae
  • Dentinogenesis imperfecta
34
Q

osteogenisis imperfecta congenita has a high mortality rate secondary to what?

A

Intracranial hemorrhage at birth or recurring respiratory tract infections during infancy

35
Q

What is the mild form of osteogenesis imperfecta?

A

Osteogenesis Imperfecta Tarda

36
Q

What are the characteristics of osteogenesis imperfecta tarda type I (OI tarda gravis)?

A
  • dentinogenesis imperfecta
  • short stature
  • LE bowing
37
Q

What are the characteristics of osteogenesis imperfecta tarda type II (OI tarda levis)?

A
  • average height
  • good prognosis for ambulation
  • least disabling form of OI
38
Q

What is the least disabling form of OI?

A

OI Tarda Type II (OI tarda levis)

39
Q

definition: blue-yellow, small, misshapen teeth result from a defect in synthesis of type I collagen.
It is found in patients with osteogenesis imperfecta.

A

Dentinogenesis

40
Q

What tests are used to detect OI prenatally?

A
  • US
  • chorionic villus sampling
  • amniocentesis
41
Q

What tests are used to detect OI postnatally?

A
  • collagen analysis
  • DNA test
  • CT
  • MRI
  • urine
  • bone density
42
Q

What medications are used for medical management of OI?

A
  • biophosphonates (reduce Fx and improve density)
  • vitamin D
43
Q

What FX management is used for treatment of OI?

A

internal fixation with intermedullary rods

44
Q

What are indications for internal fixation in those with OI?

A
  • multiple Fx
  • long bone deformity
  • impaired function
45
Q

What do internal fixating rods help prevent in those with OI?

A

bowing after Fx

46
Q

What are s/s of OI?

A
  • dislocation
  • Fx
  • bone pain
  • thin/smooth skin
  • nose bleeds
  • diaphoresis
  • hyperpyrexia
47
Q

What is hyperpyrexia?

A

temperature > 106

48
Q

What is epistaxis?

A

nosebleeds

49
Q

What is diaphoresis?

A

profuse sweating

50
Q

What are secondary impairments associated with OI?

A
  • scoliosis
  • kyphosis
  • respiratory complications
51
Q

How is prognosis of OI determined?

A

Dependent upon type of OI

52
Q

What factors of OI are associated with a high mortality rate?

A
  • multiple Fx prenatally
  • Fx during birth
53
Q

What should you avoid in those with OI during infancy?

A
  • PROM
  • pull –> sit with traction on UEs
  • force through long bones
54
Q

When does fracture frequency decrease in those with OI?

A

school age

55
Q

What are concerns during adulthood in those with OI?

A
  • deafness
  • scoliosis and/or kyphosis
  • pain
56
Q

What is the priority for adults with OI?

A

living independently

57
Q

What AD do those with OI commonly use while ambulating community distances?

A

W/C

58
Q

(true/false) The majority of those with OI do not meet exercise guidelines of 30 mins/day.

A

FALSE (they do)

59
Q

(true/false) those with OI have a low satisfaction with life

A

FALSE (high satisfaction)

60
Q

(true/false) Difficulties in those with OI are found in only 1 aspect of the ICF model.

A

FALSE (found in all domains)

61
Q

What was commonly found in OI patients during adulthood?

A

pain and scolisosis

62
Q

___% of children abused have a disability.

A

14%

63
Q

The rate of abuse in children with a disability is ___x.

A

3x

64
Q

(true/false) Maltreatment can lead to further disability

A

true

65
Q

(true/false) Increased risk of maltreatment is not consistent across disability type

A

true

66
Q

What are the highest risks of experiencing malpractice?

A
  • mental/behavioral disorders
  • conduct disorder
  • intellectual disability
67
Q

(true/false) There is an increased risk of malpractice in those with ASD, Down syndrome, spina bifida, cleft lip/palate

A

FALSE (no increased risk)

–> Reasons for no increased risk in some populations include: dx are well supported, DS is screened for during pregnancy and parents make choice to continue pregnancy, parents tended to be older and better off socioeconomically

Pediatric PT (14 decks)

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