Dysplasia II Flashcards

1
Q

Characteristics of Cleidocranial dysosttosis

A
  • Autosomal dominent
  • affects ossification mainly at intramembranous bones
  • midline defecrs
  • absent or partly absent clavicles
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2
Q

Cleidocranial dysistosis imaging findings

A
  • absent or partly absent clavicles*
  • Spina bifida oculta
  • Cone shaped thorax*
  • Persistent frontanels/ delayed ossification of cranium
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3
Q

What should you do with people with down syndrome before manipulation

A

screen adi with flex/ext study

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4
Q

What are the 4 major clinical criteeria of osteogenesis imperfecta and how many do you need for dx

A
  1. early osteoperosis
  2. Blue sclerae
  3. Dentinogenesis imperfecta (abnormal dentition)
  4. Premature otosclerosis (conductive hearing loss)

2 for dx

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5
Q

What are the 2 forms of osteogenesis imperfecta and characteristics

A
  1. Congenita- severe (most die)

2. Tarda- normal life, fxs, bowed bones

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6
Q

What are osetogenesis imperfecta tarda imaging findings (3)

A
  1. osteopenia (pencil thin cortexes)
  2. Multiple fxs
  3. Bowing (mid shaft usually)
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7
Q

What medication tx osteogenesis imperfecta

A

-bisphosphates

–creates stripes on the bone

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8
Q

ddx of gracile bones (long + thin)

A
  • osteogeneisis imperfecta
  • neruofibromatoiss
  • marfans syndrome
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9
Q

What is osteopetrosis

A
  • failure of bone removal during turnover

- Usually density/radiopacity of bones*

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10
Q

What are the 2 forms of osteopetrosis

A

child (congenita)- usually results in death

Adult (Tarda)- hx of repeated fx, unexplained anemia

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11
Q

Clinical presentation of osteopetrosis

A
  • anemia (due to decreased bone marrow)
  • hx of recurrent fxs
  • splenomegaly
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12
Q

Imaging findings of osteopetrosis

A
  • medullary cavity is obliberated (NO distention)

- Erlenmyer flask deformity

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13
Q

What will you see on the end of the shafts of bones in osteopetrosis

A

alternating bands of sclerotic and normal bone at the ends of shafts

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14
Q

What is characteristic of osteopetrosis in the vert

A

Bone within bone type look

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15
Q

What is osteopoikilosis and where is it mc found

A

radiopaque oval densities (BILAT + SYMETRICAL)

  • ends of long bones
  • small bones
  • acatabulum
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