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
