10. Fibrous Dysplasia and Pagets Flashcards Preview

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Flashcards in 10. Fibrous Dysplasia and Pagets Deck (67)
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1

Three types of Fibrous Dysplasia

1. Monostotic fibrous dysplasia 70% ~age 14
2. Polyostotic fibrous dysplasia 27% ~ age 11
3. Polyostotic fibrous dysplasia with endocrine abnormalities
(McCune-Albright Syndrome) ~3% ~ age 8

2

Slowly progressing benign disorder of fibro-osseous tissue throughout life replacing normal bone tissue

Fibrous Dysplasia

3

“Great Imitators of Bone Disease”

Fibrous Dysplasia and Paget’s disease

4

Polyostotic FD may have

bowing deformities and pathologic fx

5

Shepherd’s Crook deformity and Leg length discrepancy

Polyostotic FD

6

Enlargement and deformity of cranial
and facial bones

Leontiasis Ossea

7

Dark brown pigmented skin macules due to increased melanin in epidermis

Café au Lait Spots (Coast of Maine appearance)

8

ground-glass matrix

Fibrous Dysplasia

9

ground-glass matrix is
due to lack of
normal

cortical and
trabecular organization

10

most common etiology of benign rib lesions

Fibrous dysplasia

11

Main Pathologic Process of Café au lait spots is proliferation of

Melanocytes

12

Higher incidence of pathologic fx and Shepherd’s Crook deformity

Polyostotic FD

13

polyostotic FD with skin
pigmentation and precocious sexual development in Females

McCune-Albright Syndrome

14

occasional increase in alkaline
phosphatase and osteocalcin

Fibrous dysplasia

15

Multilocular cystic lesions with asymmetric distribution in mandible and maxilla and lower face swelling

cherubism

16

“Eyes raised to heaven attitude”

cherubism

17

Familial multilocular cystic disease of the jaws

cherubism

18

Bone is replaced by abnormal proliferation of fibrous tissue and poorly formed bony trabeculae of woven bone

Fibrous Dysplasia

19

Monostotic FD is mc in

ribs and proximal femur

20

Radiolucent Loculated, trabeculated, Ground glass or smokey appearance and Spares subarticular surface of bone

Monostotic FD

21

Thick sclerotic margin in Monostotic FD, aka a

rind of sclerosis

22

Symptoms of Polyostotic FD are usually from

bowing deformities and pathologic fx

23

Incomplete septa or ridges in Polyostotic FD result in

loculation of lesions

24

along convex surface of deformed cortex

Pseudofractures

25

may occur in areas of pathologic fx nonunion

Pseudoarthrosis

26

may look like FD in skull

Stage 2 of Paget’s

27

Paget’s thickens outer table of skull, whereas FD ____ outer table

thins

28

not commonly involved by FD

Spine

29

Paget’s Disease, Aka

osteitis deformans

30

Osteolysis followed by extensive attempts at repair

Paget’s Disease