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Oral candidiasis > 14. Diseases of the facial bones and joints > Flashcards

14. Diseases of the facial bones and joints Flashcards

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1
Q

What are the 4 layers in the structure of bone?

A

Outer, dense compact bone
Trabecular or cancellous bone
Central medullary cavity
Periosteum

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2
Q

Where is marrow found and what types can it be?

A

In the medullary cavity
Red or yellow marrow

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3
Q

What are the 2 layers of the periosteum?

A

Fibrous layer
Cellular layer

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4
Q

What does fibrous periosteum contain?

A

Sharpey’s fibres that anchor down into the bone and attach the periosteum to the bone

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5
Q

What is bone composed of?

A

Osteoid- Type 1 collagen that becomes mineralised.
Cells- osteoblasts, osteocytes, osteoclasts

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6
Q

What are the 2 types of bones?

A

Mature lamellar
Woven bone

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7
Q

What gene does osteogenesis imperfecta affect?

A

Type 1 collagen genes
COL1A1 COL1A2

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8
Q

What genes do the 4 rare types of osteogenesis imperfecta affect?

A

CRTAP
LEPRE1

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9
Q

What is the clinical presentation of osteogenesis imperfecta?

A

Brittle bones
Deafness- ear ossicles deformation
Ligament laxity
Thin aortic and heart valves
Blood vessel fragility
Short stature

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10
Q

What is the pathogenesis of osteogenesis imperfecta?

A

Amino acid substitution in the triple helix (glycine).

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11
Q

What are the types of osteogenesis imperfecta?

A

Type 1- common, mildest, AD, deafness, bone fragility, blue-grey sclera +-DI
Type 2- in utero fractures, respiratory dys, fatal, AD and AR
Type 3- rarers, progressive bone deformities, DI
Type 4- White sclera, same as type 1

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12
Q

What gene causes opalescent teeth in osteogenesis imperfecta?

A

COL1A

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13
Q

What gene causes dentinogenesis imperfecta?

A

DSPP

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14
Q

What are the features of dentinogenesis imperfecta?

A

Opalescent Amber teeth
Affects primary more than secondary
Normal colour
Short roots/obliterated canals
Decreased caries
Increased attrition
Decreased dentinal tubules or atubular

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15
Q

What is cleidocrania dysplasia due to?

A

Mutation in RUNX2 gene- controls osteoblast differentiation
Autosomal dominant

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16
Q

What are the symptoms of cleidocranial dysplasia?

A

Aplasia of clavicles
Frontal bossing- delayed ossification of fontanelles
Short stature
Hypertelorism
Delayed eruption of secondary teeth
Supernumerary teeth
Prognathism

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17
Q

What occurs in osteopetrosis?

A

Reduced bone resorption and diffuse symmetric skeletal sclerosis

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18
Q

What is the inheritance of osteopetrosis?

A

2 modes
Autosomal dominant more rare than autosomal recessive
Autosomal recessive is fatal if untreated

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19
Q

What is the pathogenesis of osteopetrosis?

A

Defect in carbonic anhydrase 2
Mutation in CLCN7- proton pump/gene defect
Causes defect in osteoclastic function and so failed remodelling

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20
Q

What are the general symptoms of osteopetrosis?

A

CN palsy- sclerosis of foramina at base of skull compresses nerves
Enlarged spleen
Anaemia
Increased fracture
Recurrent infection

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21
Q

What are the dental symptoms of osteopetrosis?

A

Malformed short roots and unerupted teeth
Osteomyeleitis after extractions
Delayed eruption
Root and bone fractures
Unusual dentition

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22
Q

What are the radiographic findings of osteopetrosis?

A

Abnormally dense bones
Chalky white appearance
Reduced marrow spaces

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23
Q

What is the treatment for osteopetrosis?

A

Bone marrow transplant

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24
Q

What is the genetic reason for achondroplasia?

A

Mutation in fibroblast growth factor receptor 3 gene
Abnormal cartilage formation which affects endochondral ossification

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25
What disease is achondroplasia associated with?
Mutations of the FGRF3 also associated with Crouzon syndrome
26
Which type of achondroplasia is fatal?
Homozygous form
27
What are the features of achondroplasia?
Normal sized head, trunk Protuberant abdomen Short limbs Retrusive middle third of face- due to lack of development of the base of skull Severe Malocclusions
28
What are the treatment for achondroplasia?
Surgery for cleft palate Orthodontics
29
What are the symptoms of cherbusim?
Individuals appear normal at birth AD 2-4- painless symmetrical swelling of mandible, and sometimes maxilla 7- lesions become static or regress Chubby face Rim of sclera visible underneath iris Puberty onwards see improvement but cosmetic surgery may be necessary
30
What gene does cherubism involve?
Mutation in SH3BP2- function unclear thought to be involved in cell signalling in bone remodelling
31
What do you see in radiographs of cherbusim?
Multiocular radiolucencies Thinning or perforation of cortical bone
32
What are the dental anomalies of cherubism?
Early loss of deciduous teeth Displacement Abnormal unerupted permanent teeth
33
What is the histology of cherubism?
Cellular and vascular fibrous tissue Focal collections of multinucleated giant cells Haemosiderin Metaplastic bone
34
How does Gardner's syndrome occur?
Autosomal dominant- APC gene Recessive form- MUTYH gene
35
What are the symptoms of Gardners syndrome?
1. Multiple polyposis of the large intestine- premalignant 2. Osseous anomalies 3. Dental anomalies- supernumerary, odontome, impacted 4. Epidermoid cysts of skin- scalp and back 5. Desmoid tumours
36
What osseous anomalies are seen in Gardner's syndrome?
Hazy sclerosis of jaw bones Dense bone islands of jaw bones Osteomas of the bones. Jaw and skull/long bones
37
How does fibrous dysplasia occur?
Somatic mutation in GNAS1 gene- G protein that regulates cAMP formation Overproduction of cAMP leads to overexpression of c-fos, a protooncogene responsible for regulating proliferation and differentiation of osteoblasts
38
What are the clinical forms of fibrous dysplasia?
Monostotic- most common- frequent in jaws Polyostotic McCune-Albright Syndrome
39
What is McCune-Albright syndrome?
Polyostotic fibrous dysplasia Cafe au lait spots in skin Endocrine abnormalities such as precocious puberty, thyroidal cysts, pituitary adenoma, large hands and feet, cushing syndrome
40
What is the radiographic appearance of fibrous dysplasia?
Orange peel ground glass appearance Blends Goes from radiolucent to radiopaque Expansile mass
41
What is the histology of fibrous dysplasia?
Bone replaced by cellular or collagenous fibrous tissue Metaplastic bone (woven or osteoid) arranged in irregular trabecular pattern forming chinese characters Fuses with the surrounding bone unlike cemento-ossifying fibroma
42
How does fibrous dysplasia present as?
Common in jaws Swelling painless Not well circumscribed Lesions expand during skeletal growth then stop Not radiosensitive- if irradiated can transform into sarcomas Surgical treatment preferable after growth phase
43
What is the definition of osteitis?
Localised inflammation that does not spread through marrow spaces
44
What is the definition of osteomyelitis?
Extensive inflammation that does spread through marrow spaces.
45
What is periostitis?
Inflammation of periosteum
46
What is alveolar osteitis?
Localised inflammation that occurs in some extractions
47
What can alveolar osteitis be due to?
Failure of blood clot formation Disintegration of blood clot
48
How can a blood clot fail to form in alveolar osteititis?
Osteopetrosis Paget's disease Poor blood supply Vasoconstrictor Radiotherapy
49
How can a clot become disintegrated in alveolar osteititis?
Mouthwashes Protelytic bacteria Empty socket retains food and debris and becomes infected Osteoclasts separate dead bone forming sequestra Localised inflammation, slow healing, granulation tissue
50
What is the tx for alveolar osteititis?
Remove debris Alveogyl Irrigate with CHX Sequestrum removal
51
What are the symptoms of alveolar osteitis?
Severe pain Foul taste Foul smell Denuded bone may be visible
52
How does the socket heal after extraction?
Socket fills with blood Formation of granulation tissue Osteoclasts cause resorption of crestal alveolar bone Gingival epithelium migrates across defect 10-14 days- formation of granulation tissue, and osteoblasts form woven bone 4-6 weeks- socket healed by repair, contains woven bone, outline of socket visible on rads 20-30 weeks- woven bone remodelled, formation of cortical cancellous bone, socket obliterated, alveolar bone reduces in height
53
What is focal sclerosing osteititis?
Sequelae of periapical inflammation Mild irritation Infection High tissue resistance
54
What does focal sclerosing osteitits look like on radiograph?
Asymptomatic Dense area at apex, but not attached to the tooth
55
What is the differential for focal sclerosing osteitis?
Sclerotic bone islands
56
What is the histology of focal sclerosing osteitits?
Plasma cells Lymphocytes Fibrous marrow tissue Increase in thickness and number of bony trabaculae
57
What types of suppurative osteomyelitis are there?
Acute or chronic Chronic lasts more than 1 month
58
Where does suppurative osteomyelitis occur most?
Mandible more common- occurs in areas with high blood supply
59
What are the causes for suppurative osteomyelitis?
Abscess Fracture Local trauma Penetrating wounds Extraction
60
What is the pathology of suppurative ostemyelitiis?
Microorganisms proliferative in bone marrow spaces Acute inflammation Tissue necrosis Accumulation of pus
61
What happens after suppurative osteoymyeleitis?
Sequesterum- formation of necrotic bone surrounded by pus Involucrum- formation of new bone outside the original
62
What are the clinical features of suppurative ostemyelitis?
Pain Pyrexia Paraesthesia of lip Swelling Trismus Intra-oral and extra-oral sinus Tooth mobility
63
What is the histology of suppurative ostemyelitis?
Necrotic bone surrounded by inflammatory cells and pus Involucrum
64
What is the radiology finding of suppurative ostemyelitis?
Early stages- normal 10-14 days- irregular, moth-eaten radiolucency
65
What is diffuse sclerosing osteomyelitis?
Similar to focal sclerosing, but more generalised Proliferative reaction in response to low grade infection Occurs in older age group
66
What is chronic osteomyelitis with proliferative periostitis?
Occurs in mandible of children and young adults Low grade apical inflammation reaching cortical bone Periosteal reaction seen- thickening and swelling of bone- onion skin
67
What are the histological features of chronic osteomyelitis with proliferative periostitis?
Fibrous marrow Chronic inflammatory cells Parallel layers of woven bone
68
What is chronic periostitis associated with?
Hyaline bodies- pulse granulomas Hyaline ring shaped material Fibrous thickening of periosteum Pus May be due to vegetable material implanted through tooth socket, raised flap, traumatic ulceration, root canal
69
What is phosphorus necrosis?
White phosphorus P4O10 converts to bisphosphonates Causes MRONJ
70
What is osteoradionecrosis?
Radiation causes proliferation of blood vessels intima- endarteritis obliterans Reduction in blood supply, especially mandible, thrombosis
71
When does osteoradionecrosis develop?
Months or years after radiotherapy
72
What is osteoradionecrosis clinically similar to?
Osteomyelitis- pain swelling slow recovery, may be asymptomatic
73
What should you do to head and neck radiotherapy patients to avoid osteoradionecrosis?
Leave only sound teeth and remove others at least 2 weeks before treatment

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