6. = Osteitis Fibrosa Cystica = Von Recklinghausen's disease of bone
Hyperparathyrodism
= ďďď PTH
In 2ry hyperparathyroidism, high levels of PTH do not cause hypocalcaemia
2 Types (1ry, 2ry hyperparathyroidism) are harmful to bone????!!!
9. Fibrous Dysplasia of bone
Bone is replaced by Fibrous C.T
Fibrous Dysplasia of boneFibrous Dysplasia of boneFibrous Dysplasia of bone
Bone is replaced by Fibrous C.T
proliferating gritty
Bone resorption Fibrous replacement Ossous metaplasia
Woven
Bone
10. Fibrous Dysplasia of bone
Etiology ??!!
1. Non- Neoplastic growth
2. Abnormal reaction to traumatic episode.
3. Endocrinal disturbances.
TYPES
11. Monostotic Fibrous Dysplasia
= fibrous dysplasia confined to a single bone, 6 times as polyostotic
Age 1st or 2nd decade of life (4-18 years)
Sex â = â
Site
affects only one bone particularly the jaws.
Max. > Mand.
S&S
Slowly growing painless swellingâŚ.
ď§not well circumscribed (diffuse)
ď§smooth and covered e normal mucosa.
ď§Disfiguring
stabilization and expansion stops with skeletal maturation.
12. Age 1st or 2nd decade of life (4-18 years)
Sex â = â
Site
affects only one bone particularly the jaws.
Max. > Mand.
S&S
Slowly growing painless swellingâŚ.
ď§not well circumscribed (diffuse)
ď§smooth and covered e normal mucosa.
ď§Disfiguring
stabilization and expansion stops with skeletal maturation.
13.
14. Polystotic Fibrous Dysplasia
= fibrous dysplasia involve multiple bones, up to 75% of skeleton
Age 1st or 2nd decade
Sex â>â
Site involves several bones including long bones, skull and jaws.
S&S
Slowly growing painless swellingâŚ.
ď§not well circumscribed (diffuse)
ď§smooth and covered e normal mucosa.
ď§Disfiguring
stabilization and expansion stops with skeletal maturation.
Jaffe 's syndrome Albrightâs syndrome
Polystotic fibrous dysplaisa
CafĂŠ au lait skin
pigmentation
Endocrinal disturbances
Polystotic fibrous dysplaisa
CafĂŠ au lait skin
pigmentation
16. Radiographic features
1. Mottled or Mixed
2. Radio-opaque.
ďLesions merge with normal bone at the margins (= ill defined)
ďLesion differ radiographoically according degree of osseous metaplasia
20. Pagetâs disease of bonePagetâs disease of bone
2nd most common osteodystrophic condition after osteopetrosis
1. Inflammatory disease (Paget's original believe).
2. Autoimmune disease.
3. Endocrine abnormality related to hyperthyroid disease.
4. vascular disorder.
5. Bone remodling disturbance.
6. viral infection (paramyxo v. + measles + RSV)
Etiology ??!!Phases
21. Age uncommon before age 40 (= ď 40 years)
Sex â> â (2:1)
Site
vertebral column (1st most common site)
- Sacrum > Lumbar > Thoracic > Cervical vertebrae.
- mechanical stress i.e bearing most wt. being most affected
Skull (next most frequently affected)
- mand. & max. about 17% of cases.
- Max.> Mand.
- functional stress + traction of neck muscles + stresses of mastication
S&S
severe pain.
Bones become largerâŚ.
ďĄflat bones become thicker. ďĄ round bones ď in circumference
ďĄhats and dentures may no longer fit.
Weight bearing bonesâŚâŚ
ďĄ bend under pressure ď crippling + bowing deformities. ďĄ break easily.
Deafness and Blindness , d.t. narrowing of cranial foramina.
High cardiac output failure may occur due to arteriovenous shunt.
Clinical features
34. BONE
A) Affects membranous bone
B) Affects endochondral bone
C) Affects both types of bone
OI , cherubism , osteopetrosis
achondroplasia
Cleido-cranial dysplasia
35. Cherubism
= familial fibrous dysplasia = familial multilocular cystic disease of jaws
Age early childhood bn (2-4 years)
Sex â > â
Site Mand.> Max.
S&S
Bilateral ,Firm, painless swelling
at angles of mand.
Clinical features
Pathogenesis