Exam 1--Sec. 1 Musculoskeletal Pathologies Flashcards

1
Q

What is the term for a disorder of bony development, which commonly results in defective ossification of the skeletal system?

A

Dysostosis

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2
Q

What are some examples of Dysostosis?

A
  • polydactyly
  • syndactyly
  • aplasia (absense of bodily structure)
  • cleidocranial dysostosis
  • Klippel-Feil Syndrome
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3
Q

Following what irregularity does dysostoses develop after?

A

following irregular mesenchymal cell migration, during embryogenesis

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4
Q

What is Dysplasia?

A

a musculoskeletal pathology resulting from a mutation that interfers with the body’s ability to perform normal growth or regulate normal musculoskeletal homeostasis

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5
Q

What are examples of dysplasia?

A
  • type 1 spondylolisthesis (the dysplastic type)
  • osteogenesis imperfecta
  • osteopetrosis
  • achondroplasia

~could be isolated or widespread

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6
Q

What is dwarfism?

A

a description of stunted growth; does not achieve mature height of at least 4’11’’

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7
Q

What are some pathologies that may result in dwarfism?

A
  • Turner syndrome
  • osteogenesis imperfecta
  • achondroplasia
  • hypopituitarism
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8
Q

What is a form of skeletal dysostosis that involves congenital abnormalities of bones and teeth with clavicles that are underdeveloped or absent?

A

cleidocranial dysostosis

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9
Q

What is the frequency that cleidocranial dysostosis occurs?

A

1 in a million inds.

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10
Q

What occurs with the skull bones in someone with cleidocranial dysostosis?

A

have delayed fusion, result in delayed closure of cranial sutures, causing enlarged frontanelles (“soft spots”), typically have normal intelligence though

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11
Q

About 70% of inds. with cleidocranial dysostosis have a mutation in what gene? What does that gen involve? How is it inherited?

A

mutation in RUNX2 gene; involves cartilaginous and osseous tissue development; is “master switch” and regulates other genes

inheritence: autosomal dominant

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12
Q

What syndrome is a skeletal dysostosis and is defined by presence of two or more cervical vertebrae having abnormal fusion?

A

Klippel-Feil Syndrome

Occurs ~ 1 in 40,000

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13
Q

What abnormalities in the neck and other places in the body does Klippel-Feil syndrome cause?

A
  • shortened neck height and low-appearing hairline and limited motion in cervical spine
  • cause cervical neck pain and headaches
    Other:
  • renal abnormalities, cardiac abnormalities, and hearing problems
    ~Sprengel’s deformity
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14
Q

What is Sprengel’s Deformity and what other disorder is it characteristically ass. with?

A

is a skeletal abnormality of the scapula due to failure of scapula to descend during embryogenesis –> may involve congenital bony fusion (ankylosis) b/w scapula and lower C or upper T spine

ass. with Klippel-Feil syndrome

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15
Q

What is the most common form of skeletal dysplasia and what gene is involved?

A

Achondroplasia; a gain-of-function mutation in FGFR3 gene; usually the gene suppresses endochondral growth; these affects long bone elongation of extremity bones

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16
Q

How does achondroplasia occur?

A
  • inherited ~10% cases, autosomal dominant

- most are a sporadic mutation in FGFR3 gene w/in father’s sperm

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17
Q

What increases the risk of one having achondroplasia due to a spontaneous FGFR3 gene?

A

advanced paternal age increases the risk of father’s sperm developing the spontaneous mutation

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18
Q

What are characteristics of achondroplasia?

A
  • short extremity bones
  • trident hand appearance
  • stenosis of foramen magnum
  • frontal bossing
  • midface hypoplasia
  • lumbar spinal stenosis
  • bullet vertebrae (scoliosis and extreme kyphosis or lordosis)
  • lower extremity radiculopathy (from lumbar stenosis)
  • normal intelligence
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19
Q

What is the most common form of lethal dwarfism that is incompatible with postnatal life and how common is it? How is the thorax affected?

A

Thanatophoric dwarfism (~ 1 in 20,000)

Severely underdeveloped thorax does not provide enough space for normal heart and lung development

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20
Q

What is severely diminished in Thanatophoric dwarfism? What gene is mutated?

A

chondrocyte activity–resulting in inhibited endochondral ossification

same FGFR3 gene mutation as in traditional achondroplasia

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21
Q

What is the most common cause of death of thanatophoric dwarfism?

A

respiratory failure (due to underdeveloped thorax)

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22
Q

What dysplasia disorder is due to mutation in formation of type 1 collagen, causing it to take on irregular shape resulting in premature breakdown?

A

Osteogenesis imperfecta (AKA brittle bone disease, type 1 collagen disease, or OI)

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23
Q

What tissues are affected in osteogenesis imperfecta, which tissues are prominently affected??

A

all tissues containing type 1 collagen; esp. bones, teeth, eyes, skin, and joints

~hearing issues, small teeth

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24
Q

What are two unique features of osteogenesis imperfecta?

A
  • severely under-mineralized bony tissues = very prone to fracture
  • “blue sclerae”–> due to more translucent sclerae of eyes and vascular choroid layer showing bluish color
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25
Q

T/F. Type I Osteogenesis imperfecta is incompatible with life.

A

False. it IS compatible with life

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26
Q

How is Type I osteogenesis imperfecta most commonlly recognized?

A

in childhood due to multiple fractures, bowing of weight-bearing lower extremity bones, dwarfism, or scoliosis

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27
Q

What do we need to keep in mind with patients with Type I osteogenesis imperfecta?

A

severe skeletal fragility and DO NOT use HVLA adjusting

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28
Q

Is Type II osteogenesis imperfecta compatible with post-natal life? why or why not?

A

NO! Due to extreme skeletal fragility and ind. suffers multiple fracture in utero or during birth process and usually causes lethal intracranial hemorrhage

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29
Q

Children that are treated with bisphosphonates for their osteogenesis imperfecta will have what bone appearance on x-ray?

A

Zebra Stripe Sign

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30
Q

What describes a group of conditions that are characterized by reduced osteoclast formation/activity? What does it result in?

A

Osteopetrosis (AKA marble bone disease)

Results in widespread skeletal sclerosis

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31
Q

What does sclerosis mean?

A

abnormal hardening of a body tissue

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32
Q

What are the affects to the bone in osteopetrosis?

A

increased skeletal density, and therefore more prone to fractures due to ridged and inflexible

“chalk stick” pathological fractures, straight across

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33
Q

What are some clinical features of someone with osteopetrosis?

A

bone marrow mineralizes—therefore reduced hematopoiesis

  • decreased RBCs = anemia
  • decreased WBCs = infections
  • Erlenmeyer flask deformity
  • CN defects due to foraminal stenosis
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34
Q

What is osteoporosis?

A

a common condition involving a severe reduction in bone mineral density, defined as being at least -2.5 standard deviations from normal mineralization

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35
Q

What is the primary complication from osteoporosis and where does that most commonly occur?

A

fractures in trachecular bone (VBs of spine, femur, or calcaneus)

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36
Q

How is bone mineral density evaluated?

A

DEXA scan determines how many standard deviations patient’s bone mineralization is away from normal of a 25 yr old = “T-score”

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37
Q

What is a T-score vs a Z-score?

A

T-score = compare you to age of same sex of optimal time period

Z-score = compare you to inds your age, sex, and weight and where they are at

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38
Q

What is osteopenia?

A

a term used for bone mineralization below normal, but not yet severity of osteoporosis; therefore b/w 1 and 2.5 standard deviations below normal

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39
Q

When does bone mineralization peak and what happens to it after that?

A

young adulthood, age 25-30, then decreases ~0.5% every year after

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40
Q

What factors influence inds bone mineral density?

A

genetic factors, diet, lifestyle choices (smoke/drink), level of physical activity, muscle strength, age, hormones

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41
Q

What is the term for when osteoblast activity decreases with age and there is age-related loss in bone mineralization? Why does this occur?

A

senile osteoporosis

  • older adults more likely to be inactive
  • after menopause rates increase of osteoclast activity and due to reduced estrogen levels
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42
Q

T/F. Osteopenia and osteoporosis are asymptomatic.

A

True. Until there is a fracture

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43
Q

Where are the MC locates for osteoporosis-related fractures?

A
  • femoral neck

- VBs–> cause compression fracture MC in thoracolumbar

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44
Q

What is the term for a loss of overall height observable with advanced osteoporosis?

A

“Dowager’s hump”

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45
Q

What are complications from osteoporosis-related fractures?

A
  • immobility
  • spinal deformity (lateral curve in T/L or lordosis in L)
  • pneumonia
  • pulmonary embolism
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46
Q

How can one help prevent osteoporosis?

A
  • max. deposition of bone mineralization in childhood/young adulthood
  • regular physical exercise
  • diet rich in Vit. D and get UV exposure
  • good vision and hearing
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47
Q

What medication can be used to help decrease osteoclast activity?

A

bisphosphonates

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48
Q

What are two metabolic bone disease that develop after Vitamin D deficiency?

A

Rickets and Osteomalacia

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49
Q

What is Rickets?

A

develpos in children due to deficiency in Vit. D; reduced bone deposition in epiphyseal region (growth plate); causes stunt growth, bowing of legs, and increased risk of fractures, and abnorm spinal curvatures

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50
Q

What is osteomalacia?

A
  • develops in adults due to Vit. D deficiency
  • faulty bone turnover and bone left under-mineralized
  • increases risk of fracture
  • less severe than Rickets
  • mimics osteoporosis
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51
Q

What is hyperparathyroidism?

A

involves elvated PTH, which regulates blood calcium levels by stimulating osteoclast activity which release Ca++ into blood

52
Q

T/F. Primary hyperparathyroidism involves other processes in the body that causes increase PTH production.

A

False, (this is describing secondary hyperparathyroidism)

primary– involves parathyroid glands overproducing PTH

53
Q

What is the primary cause of primary hyperparathyroidism? and who is most likely to develop it?

A

adenoma of parathyroid gland

women around age 50

54
Q

What does Secondary hyperparathyroidism involve?

A

other processes in body that cause increase production of PTH

55
Q

What is the primary cause of secondary hyperparathyroidism?

A

chronic kidney disease–> which causes hypocalcemia–> and parathyroid gland response by elevating PTH production

56
Q

What is a less common cause of secondary hyperparathyroidism?

A

vitamin D deficiency–> which interfers with calcium homeostatsis–> causes hypocalcemia–> stimulates PTH levels

57
Q

What is the MC sign of hyperparathyroidism? What is the MC symptom?

A

MC sign = hypercalcemia

MC symptom = kidney stones

58
Q

What are some other conditions patients with hyperparathyroidism may experience?

A
  • weakness
  • depression
  • abdominal pain
  • peptic ulcer
  • nausea
  • anorexia
  • short-term memory loss
  • bone pain
  • myalgia (muscle pain)
  • joint pain
  • anorexia
  • body-wide osteoporosis
59
Q

T/F. Skeletal changes ass. with hyperparathyroidism involve resorption of cortical and trabecular bone?

A

true. and is widespread

60
Q

Hyperparathyroidism does affect the skeleton in a widespread manner, but what are the most characteristically affected areas?

A
  • phalanges
  • VBs
  • calvaria
  • femurs
  • tibias
61
Q

For hyperparathyroidism, what are the radiological appearances for these locations:

  1. VBs
  2. Phalanges
  3. Calvaria
  4. Long bones of lower extremeties
A
  1. Rugger jersey spine
  2. Resorption of cortex on radial side of middle and distal phalanges
  3. salt and pepper skull
  4. osteitis fibrosa cystica
62
Q

What is the brown “tumor” characteristic of? How is it caused?

A

hyperparathyroidism
- develop following localized osteolysis –> one weakens and hemorrhage occurs–> macrophages come–> fibrotic tissue fills area

63
Q

Localized areas of osteolysis in hyperparathryroidism may cause brown “tumor”, but what else may it cause?

A

osteitis fibrosa cystica–> areas of “cystic degeneration”

64
Q

What are the three stages of Paget’s Disease?

A
  1. Osteolysis stage
  2. mixed osteoclastic-osteoblastic stage
  3. osteosclerotic (burned-out) stage
65
Q

What are the results of the the three stages of Paget’s Disease?

A

increased bone mass, but structurally weak

66
Q

Who does Paget Disease seem to effect and what geographic locations? How common?

A

(males 2x as likely) Caucasian males around age 70; in North America, AUS< New Zealand, Europe
~1%, but up to 10% if older than 80yrs

67
Q

T/F. Most patients with Paget Disease are asymptomatic and diagnosis is discovered incidentally.

A

True, ~80%

68
Q

What is elevated in serum of someone with Paget’s Disease?

A

levels of alkaline phosphatase–> represents osteoblastic activity

69
Q

If Paget’s disease is symptomatic, what are some symptoms?

A
  • back and neck pain (MC)
  • bony deformation (bowing)
  • increased fracture risk
  • headache
  • CN compression
  • “ivory vertebra sign”
70
Q

What are the three things that can cause an “ivory vertebra sign”?

A
  1. Paget Disease
  2. Metastatic cancer (prostate)
  3. lymphoma
71
Q

How is the bone of an x-ray described for Paget’s disease?

A

“shaggy” appearance. irregular mosaic or “jigsaw puzzle” pattern

72
Q

Where is Paget’s disease most likely to develop on the skeleton?

A

axial skeleton and proximal femurs

  • pelvis, sacrum, skull(increase density)
73
Q

What percentage of Paget’s disease cases transform into cancer and what is usually the prognosis?

A

~1% or less transition into osteosarcoma–and is very aggressive and has a high mortality rate

74
Q

What is the etiology of Paget’s Disease?

A

idiopathic

75
Q

What is a fracture?

A

abbreviated Fx.

- is complete or partial loss of bone integrity

76
Q

Describe a complete fracture.

A

fracture completely separates; complete separation of bones cortex

77
Q

When is a complete fracture MC?

A

at site of pathological fracture in older adults

78
Q

Describe an incomplete fracture.

A

does not separate the fragments of fractured bone

79
Q

Where and when are incomplete fractures MC?

A

in long bones in pediatric inds

80
Q

What is a fracture called when the skin overlying the fracture remains intact?

A

Closed (simple) fracture

- decreases risk of inf.

81
Q

What is a compound (open) fracture?

A

fracture is in communication with overlying skin; increases risk of inf.

82
Q

What is fracture called when it is splintered into multiple fragments? When does this characteristically occur?

A

comminuted fracture; typically due to high-velocity forces

83
Q

What is a displaced fracture?

A

when ends of bones at fracture site are not in alignment

84
Q

Describe a stress fracture.

A

slowly-developing fracture following increased physical activity and repetitive loading

85
Q

What makes a fracture painful?

A

the disruption of sensitive periosteum

86
Q

What are the optimal steps of a fracture healing?

A
Begins immediately and hematoma forms
Week 1: soft callus
Week 2: bony callus and woven bone
Weeks 6-8: endochondral oss. and bony callus 
~lifetime of remodeling
87
Q

What 6 things can delay a fracture from healing?

A
  1. nonunion
  2. splintered
  3. not immobilized
  4. infected
  5. ind. malnourished
  6. older adult
88
Q

What are fractures called that involve the epiphyseal growth plate and what individuals can they occur in?

A

Salter-Harris Fractures (SHF); ind. must have “open” or active growth plates–limited to pediatrics who have not reached skeletal maturity

89
Q

How common are Salter-Harris Fractures?

A

occur in about 15% of all pediatric longbone fractures

90
Q

Describe a Type I Salter-Harris Fracture. What is the prognosis?

A

extends through entire growth plate (physis)

- favorable prognosis

91
Q

Describe a Type II Salter-Harris fracture. What is the prognosis?

A

extends from growth plate into metaphysis

- favorable prognosis

92
Q

Describe a Type III SHF. What is the prognosis

A

extends from growth plate into epiphysis

- limited prognosis, due to joint involvement

93
Q

Describe a Type IV SHF. What is the prognosis?

A

extends through growth plate, metaphysis, and epiphysis

- poor prognosis

94
Q

Describe a Type V SHF. What is the prognosis?

A

compression fracture of growth plate

- poor prognosis

95
Q

From Type 1 - Type V, what are the prevalence of each when it comes to all SHFs?

A
Type I - 5%
Type II - 75%
Type III - 10%
Type IV - 10%
Type V - <1%
96
Q

What type is the most common Salter-Harris fracture?

A

Type II (extends from growth plate into epiphysis)

97
Q

What is Avascular Necrosis (AVN)?

A

a site of necrosis due to reduced blood supply (ischemia)

98
Q

Avascular Necrosis (AVN) may occur following a fracture or joint dislocation. What are some other causes?

A
  • autoimmune vasculitis (Type III hypersensitivity)
  • prolonged use of corticosteroids
  • ionizing radiaton
  • blood clots
  • blood disorders increasing clotting
99
Q

About _____ percent of all Avascular Necrosis (AVN) are ______.

A

25%; idopahthic

100
Q

T/F. Many Avascular necrosis (AVNs) are asymptomatic.

A

True

and heal via “creeping substitution”

101
Q

What may occur with a subchondral Avascular necrosis (AVNs) that collapse?

A

may cause surface irregularities at joint, which accelerate osteoarthrities = secondary osteoarthritis

102
Q

What is the term given to an Avascular necrosis (AVN) that develops at the subchondral area of a joint?

A

Osteochondritis dissecans

-is pain due to death of osseous and cartilaginous tissues

103
Q

What are the MC locations for osteochondritis dissecans?

A

knee and hip joints

104
Q

What age do most Avascular necrosis (AVN) cases occur?

A

ages 30-50

105
Q

What is a site of bone marrow inflammation, due to an infection?

A

osteomyelitis (bacterial inf.)

106
Q

In osteomyelitis, what is the term used for the site of infected bone that dies?

A

sequestrum

107
Q

In osteomyelitis, what do you call the irregular bone surrounding the sequestrum?

A

invelucrum

108
Q

Many cases of ______ will produce pus, which exits the site via a _____ ______.

A

pyogenic; draining sinus

109
Q

What are the two types of osteomyelitis?

A
  1. Pyogenic osteomyelitis

2. Mycobacterial osteomyelitis (TB osteomyelitis, or skeletal TB)

110
Q

What are the three way that an infection reaches the bone marrow in Pyogenic osteomyelitis?

A
  1. (MC) hemoatogenous spread
  2. extension from nearby inf.
  3. direct implantation form trauma or compound fx.
111
Q

What are the MC sites of Pyogenic Osteomyelitis?

A

long bones of extremities

112
Q

What is the MC bacterial microbial origin for Pyogenic Osteomyelitis?

A

Staph. aureus ~90% all cases

113
Q

In neonates, what are the two MC bacterial microbes to cause Pyogenic osteomyelitis?

A

E. coli and Group B Strep.

114
Q

Inds. with sickle cell disease are most likely to develop Pyogenic osteomyelitis from what bacterial microbe?

A

Salmonella spp.

115
Q

What is it called when TB causes the bone marrow infection?

A

Mycobacterial Osteomyelitis, or TB Osteomyelitis, or Skeletal TB

116
Q

Where does Mycobacterial Osteomyelitis usually develop from?

A

in patients with pulmonary TB, inf. begins in lungs and spread to lymph and blood

117
Q

Is what percent of all cases of pulmonary TB does Mycobacterial osteomyelitis develop?

A

3%; usually in ind. with suppressed immune system

118
Q

What symptoms can Mycobacterial osteomyelitis cause?

A
  • severe destruction and caseous granulomas
  • low-grade fever
  • chills
  • chachexia
119
Q

Where is the inf. in Mycobacterial osteomyelitis MC occur?

A

long bones of extremities and VBs

120
Q

When the vertebral bodies are involved are destructed over time in Mycobacterial osteomyelitis, what is it called?

A

Pott’s disease

121
Q

T/F. Mycobacterial osteomyelitis is MC results of acute infection.

A

False; MC due to a chronic inf.

122
Q

What is congenital kyphosis?

A

results from abnormalities occurring during embryogenesis, regarding formation or segmentation of spinal column

123
Q

Is Type I or Type II congenital kyphosis more common?

A

Type I (involving incomplete formation of VB)

124
Q

What type of congenital kyphosis involves failed segmentation of VBs?

A

Type II

125
Q

Where does congenital kyphosis occur? What can it cause?

A

thoracic spine; cause compression of spinal cord–> paraplegia