Fibro-osseous Bone Disorders Flashcards

1
Q

What is Fibrous dysplasia?

A

Skeletal anomaly in which normal bone is replaced by poorly organised immature bone and fibrous tissue

Non-neoplastic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What age group is most affected by fibrous dysplasia?

A

Children & adolescents (as affects actively growing bone & progression ceases with age)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is the cause of fibrous dysplasia?

A

Mutation in GNAS gene

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the two classifications of fibrous dysplsia?

A

1) Monostotic (affects single bone) – More common
2) Polyostotic (multiple bones)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is the presentation of fibrous dysplasia?

A

Asymmetrical, painless swelling – often in children & adolescents
May be associated with Albright syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the most common sites of fibrous dysplasia?

A

Craniofacial bones (Maxilla > Mandible) & femur

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What can be seen on this radiograph?

A

Fibrous dysplasia
Ill-defined border & loss of lamina dura
Early stage = More fibrous (radiolucent)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are some histological features to look out for in fibrous dysplasia?

A

Islands of fibrous tissue embedded within bone
Immature woven bone with lack of osteoblasts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are Ossifying/Cemento-ossifying Fibromas?

A

Benign, fibro-osseous NEOPLASM that affects jaws & craniofacial skeleton

(continue to enlarge, not self-limiting as with Fibrous Dysplasia)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are the different pathological variants of ossifying/cemento-ossifying fibromas?

A

Cemento-Ossifying Fibroma (COF)
Juvenile Trabecular Ossifying Fibroma (JTOF)
Juvenile Psammomatoid Ossifying Fibroma (JPOF)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

At what age groups do the different variants of ossifying/cemento-ossifying fibromas present?

A

Peak incidence in 3rd-4th decade (F>M 5:1) Cemento-Ossifying Fibroma (COF)

Children & Adolescents
Juvenile Trabecular Ossifying Fibroma (JTOF)
Juvenile Psammomatoid Ossifying Fibroma (JPOF)

NB: JTOF/JPOF multiple recurrence

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Describe the expansion of lesions for ossifying/cemento-ossifying fibromas

A

Painless expansion of buccal & lingual plates of affected bone

Larger lesions: Expansion of lower border of mandible or maxillary antrum floor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Radiographically what would you see for a ossifying/cemento-ossifying fibroma?

A

Well-circumscribed & clearly demarcated lesion from surrounding normal bone*

Early lesions = Radiolucent
Later = Progressively more radiopaque

*distinguishing feature from Fibrous Dysplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Radiographically how can you distinguish between a cemento-ossifying fibroma and a cemento-blastoma?

A

No connection with roots of teeth for a cemento-ossifying fibroma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the managment for Ossifying/cemento-ossifying fibromas

A

Surgical excision

NB: JTOF/JPOF multiple recurrence

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is Cemento-osseous Dysplasia?

A

NON-Neoplastic, fibro-osseous condition affecting tooth-bearing regions (alveolar bone) of mandible & maxilla

17
Q

What are the different classifications of cemento-osseouf dysplasia and what do they mean?

A

1) Periapical (typically anterior mandibular teeth)
2) Focal (single tooth)
3) Florid (multiple)
4) Familial Gigantiform Cementoma (genetic)

18
Q

Comment on the vitality of teeth affected by cemento-osseous dysplasia

A

Teeth remain vital

19
Q

What may you see radiographically for a cemento-osseous dysplasia lesion?

A

Well-circumscribed & surrounded by a thin radiolucent rim
Radiolucent, mixed or radiopaque – Increased density as lesions mature

20
Q

What is the managment for Focal Cemento-Ossifying dysplasia?

A

No tx & monitor

21
Q

What is the managment for Florid Cemento-Ossifying dysplasia?

A

Monitor closely (osteomyelitis complications)

22
Q

What is Paget’s disease?

A

Paget’s disease of bone disrupts the normal cycle of bone renewal, causing bones to become weakened and possibly deformed

23
Q

What genetic and environmental factors could potentially cause paget’s disease?

A

Genetic links = Chromosome 18q
Environmental links = Paramyxoviridae viral infection

24
Q

What age group is most affected by Pagets disease?

A

Patients over 40 years old

25
Q

What are some manifestations of Pagets disease?

A

Susceptibility to fracture and deformity of weight bearing bones
Cranial nerve disorders, deafness, vertigo and tinnitus occur as a consequence of nerve compression in foramina

26
Q

What occurs during the Osteolytic stage of Pagets disease?

What may you also see on a radiograph?

A

Excessive bone resorption with replacement by fibrous and vascular tissue

Ill-defined radiolucent areas

27
Q

What occurs during the Osteolytic and osteogenesis stage of Pagets disease?

What may you also see on a radiograph?

A

New bone formation within the fibrous tissue with simultaneous osteoclastic and osteoblastic
activity

Mixed radiolucent and opacity on radiograph

28
Q

What occurs during the Osteosclerosis stage of Pagets disease?

What may you also see on a radiograph?

A

Late phase in which the irregular haphazardly arranged bone coalesces to form dense
sclerotic bone

The bone exhibits a dense radiopaque appearance

29
Q

During the early stages of Pagets disease there is prominent resorption what are the dental implications of this?

A

Bone replaced by vascular stroma therefore there is an increased bleeding risk with XLA

30
Q

During the late stages of Pagets disease there is prominent bone apposition what are the dental implications of this?

A

Irregular bone with minimal vascularity therefore there is an increased infection risk (Osteomyelitis)

30
Q

During the late stages of Pagets disease there is prominent bone apposition what are the dental implications of this?

A

Irregular bone with minimal vascularity therefore there is an increased infection risk (Osteomyelitis)

31
Q

Why may Pagets disease make extractions more difficult?

A

Hyper-cementosis which leads to bulbous roots which in turn leads to difficulty extracting

32
Q

What medication will patients with Pagets disease be on?

A

Bisphosphontates