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Flashcards in Dysplasia 1 Deck (19)
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1

3 forms of Fibrous dysplasia

1. Monostotic (Mc- 79-80%)

2. Polyostotic

3. Mccune- albright syndrome (2-3%)

2

3 characteristics of mccune albright syndrome

1. early onset of puberty

2. Polyostotic fibrous dysplasia

3. Cafe au lat spons (w irregular borders)

3

Characteristics of polyostotic fibrous dysplasia

-long lesion in long bones
-expansile lucent lesions
-sclerotic rim
-mixed lesion
-shepperds crook

4

Neurofibromatosis Triad of findings

1. Cafe au lait spots (smooth borders)
2 cutaneous tumours
3. Various osseous alterations of axial and appendicular skeleton

5

What are the criteria to dx neurofibromatosis

6+ spots
1.5cm diameter
at least 2 neurofibromas

6

What are potential musculoskeletal manifestations of neurofibromatosis

Kyphoscoliosis (mc)
Vertebral scaloping posteriorly
enlarged neural foramina
Hypoplastic post elements
multiple NOF

7

What is dural ectasia + what is it associated w

widenening of the dura due to post scaloping in neurofibromatosis

8

What are pressure erosions and what are they associated w

schwannoma/neurilemmoma causing focal enlargement of IVF due to neurofibromatois

9

What is 'empty orbit' and what does it lead to

Bony defect in post orbit causing herniation of the temporal lobe into the defect (pulsating exophthalmos)

10

Wherre are multiple NOFs mc found

mc in lower extremities

11

Clinical features of Marfans Syndrome

-elongated extremities/ jt laxity/scoliosis
-sparse soft tissues
-arachnodactyly (spider fingys)
-lens dislocation
-1/3 congentital heart disease

12

Marfans syndrome x ray features

-tall vert
-post scaloping
-widened spinal canal
-anyurisms
-scoliosis

13

What addtional test should you get w someone w marfans

Doppler ultrasound

14

achondroplasia clinical features

-shortening of long bones (all bones by enchondral ossification)
-hyperlordodic L/S
-Trident Hand (gapping bw 3/4th digit)

15

Achondrolasia imaging findings (4)

-short broad pedicles
-post scalloping
-increased lordosis
-horizontal sacrum

16

What is the hallmark imaging sign of achondroplasia

Narrow spinal canal (interpediculate distance decreases by 50% as you go down to L5)

17

ddx of post scaloping

-Neurofibromatosis
-Marfans
-Achondroplasia

18

Achondroplasia pelvis signs

-short ilia
-champange glass pelvic inlet
-horizontal sacrum
-L5 will be low bw ilia

19

what is features of rhizomelic shortening

more proximal bones affected more than distal
-flared metaphysis