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Flashcards in Dysplasia II Deck (15)
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1

Characteristics of Cleidocranial dysosttosis

- Autosomal dominent
-affects ossification mainly at intramembranous bones
- midline defecrs
-absent or partly absent clavicles

2

Cleidocranial dysistosis imaging findings

-absent or partly absent clavicles*
-Spina bifida oculta
-Cone shaped thorax*
-Persistent frontanels/ delayed ossification of cranium

3

What should you do with people with down syndrome before manipulation

screen adi with flex/ext study

4

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

1. early osteoperosis

2. Blue sclerae

3. Dentinogenesis imperfecta (abnormal dentition)

4. Premature otosclerosis (conductive hearing loss)

2 for dx

5

What are the 2 forms of osteogenesis imperfecta and characteristics

1. Congenita- severe (most die)

2. Tarda- normal life, fxs, bowed bones

6

What are osetogenesis imperfecta tarda imaging findings (3)

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

7

What medication tx osteogenesis imperfecta

-bisphosphates

--creates stripes on the bone

8

ddx of gracile bones (long + thin)

-osteogeneisis imperfecta
-neruofibromatoiss
-marfans syndrome

9

What is osteopetrosis

-failure of bone removal during turnover
-Usually density/radiopacity of bones*

10

What are the 2 forms of osteopetrosis

child (congenita)- usually results in death

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

11

Clinical presentation of osteopetrosis

- anemia (due to decreased bone marrow)
-hx of recurrent fxs
-splenomegaly

12

Imaging findings of osteopetrosis

-medullary cavity is obliberated (NO distention)
-Erlenmyer flask deformity

13

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

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

14

What is characteristic of osteopetrosis in the vert

Bone within bone type look

15

What is osteopoikilosis and where is it mc found

radiopaque oval densities (BILAT + SYMETRICAL)

-ends of long bones
-small bones
-acatabulum