Bone pathology I - cherubism and noonan's

Question 1

cherubism?

Answer 1

mutation in a gene that encodes a cell signalling molecule involved in the regulation of inflammation and bone

‘cherubism’ relates to the clinical appearance

Question 2

cherubism rare or common?

Answer 2

rare

Question 3

males or females more affcted by cherubism?

Answer 3

men

Question 4

clinical presentation of cherubism?

Answer 4

painless swellings of the jaws

ages 2-4

symmetrical swellings

mandible or mandible and maxilla

rapid growth until age of 7

growth stops and the lesions start to regress as the pt reaches adult life

fullness of cheeks (chubby face)

inflammation of submandibular gland/lymph nodes

stretching of kin under the eyes

Question 5

where does cherubism usually start?

Answer 5

angle of the mandible

Question 6

is cherubism self-limiting?

Answer 6

yes

Question 7

effect of cherubism dentistry?

Answer 7

premature loss of deciduous teeth

displacement, lack of eruption and failure of development or permanent teeth

Question 8

radiographic appearance of cherubism?

Answer 8

sharply defined multilocular radiolucency’s

expansion and thinning of the cortical plates

mandibular lesions begin at the angle of the mandible and spread to the body and ramus

condyle is saved

maxillary lesions are confined to the tuberosities

sinus might be obliterated

teeth develop might not erupt

Question 9

bone pathological disease shown?

Answer 9

cherubism

Question 10

bone pathological disease shown?

Answer 10

cherubism

Question 11

histopathology of cherubism?

Answer 11

GIANT CELL LESION - with many many nuclei

cellular and vascular fibrous tissue with a various number of multinucleated osteoclast like giant cells

concentrated in focal areas - focal collections of giant cells

round thin-walled vascular channels

giant cells e.g. osteoclasts and multinucleated

Question 12

what are maxillary lesions in cherubism confined to?

Answer 12

tuberosities

Question 13

what is a giant cell lesion?

Answer 13

wall is replaced with a fibrous tissue with lost of multinucleated osteoclast-like giant cells

Question 14

change in cherubism over time?

Answer 14

as the activity of the lesion decreases, the lesions become more fibrous with a reduction in the number of giant cells and deposition of metaplastic bone

starts being radiolucent and eventually radiopaque and appear radiopaque

Question 15

behaviour of cherubism?

Answer 15

self-limiting condition

improvement of the facial appearance from puberty onwards

conservative cosmetic surgery is often required

radiographically, bony infillings of the lesion

radiolucency’s (outline of the lesions) might remain well into old age

Question 16

noonan’s syndrome?

Answer 16

a complex genetic disorder

giant cell lesions replace bone and joints

most frequent in jaws

features include: congenital heart disease, feeding problems, speech and hearing difficulties

heavy eyelids, tight neck skin and low-set ears

Question 17

noonans syndrome can resemble what diseases?

Answer 17

giant cell granuloma or cherubism clinically