BONE DISORDERS (ppt) Flashcards by Love Dog

T/F

OSTEOGENESIS IMPERFECTA is autosomal dominant

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“Brittle Bones”

OSTEOGENESIS IMPERFECTA

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Fragilatis Ossium

OSTEOGENESIS IMPERFECTA

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Osteopsathyrosis

OSTEOGENESIS IMPERFECTA

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Lobstein’s Disease

OSTEOGENESIS IMPERFECTA

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abnormality in the type I collagen (bone, ligament, dentin, and sclera)

OSTEOGENESIS IMPERFECTA

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fragility of bones

OSTEOGENESIS IMPERFECTA

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OSTEOGENESIS IMPERFECTA has 2 Forms:

Vrolik’s type
Lobstein type

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T/F

OSTEOGENESIS IMPERFECTA increase bone mass

False

(INCREASE bone mass)

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Extreme fragility /porosity of bones

OSTEOGENESIS IMPERFECTA

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abnormal electrical response of muscles
Triangular-shaped head
Pale, blue sclera

A. OSTEOGENESIS IMPERFECTA
B. INFANTILE CORTICAL HYPEROSTOSIS
C. CLEIDOCRANIAL DYSPLASIA
D. CRANIOFACIAL DYSOSTOSIS
E. MANDIBULOFACIAL DYSOSTOSIS
F. PIERRE ROBIN SYNDROME
G. MARFAN SYNDROME
H. DOWN SYNDROME
I. OSTEOPETROSIS
J. ACHONDROPLASIA
K. OSTEITIS DEFORMANS
L. GENERALIZED CORTICAL HYPEROSTOSIS
M. MASSIVE OSTEOLYSIS
N. FIBROUS DYSPLASIA OF BONE
O. POLYOSTOTIC FIBROUS DYSPLASIA
P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS
Q. CHERUBISM

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Deafness
Skin: thin and translucent
Subcutaneous capillary bleeding

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Oral Manifestations / Radiographic Findings

teeth: bulbous crowns, with narrow, constricted roots and obliterated canals

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Oral Manifestations / Radiographic Findings

blue-gray to yellowish-brown teeth
Anterior and posterior cross bites and open bites
maxillary hypoplasia rather than mandibular hyperplasia

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Histologic Features:
1. bone: thin cortices, immature spongy bone

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*cortical bone replaced with fibrous tissue
* retarded osteoblastic activity

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Treatment and prognosis of OSTEOGENESIS IMPERFECTA

Treatment and Prognosis:
> no definite treatment (bisphonates)
> immobilization of fractures
> may improve after puberty

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Caffey’s Disease,

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Clinical Features:
1. tender, deeply-placed soft tissue swellings
2. systemic manifestations
3. cortical thickening

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Caffey-Silverman Syndrome

Infantile cortical hyperostosis

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DOral Manifestations:
1. assymetric deformity of mandible
2. facial swelling

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©Radiographic Manifestations:
1. unilateral / bilateral
thickening of cortex

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@Prognosis:
1. may regress without treatment
2. may recurr
3. conservative treatment

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Marie and Sainton’s Disease

. CLEIDOCRANIAL DYSPLASIA

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Scheuthauer-Marie-Sainton Syndrome, Mutational Dysostosis

. CLEIDOCRANIAL DYSPLASIA

Vertical midline furrow with frontal bossing A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

> Depressed midface > Prominent chin A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

Oral Manifestations: prolonged retention of deciduous teeth and delayed eruption of succedaneous teeth A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

Oral Manifestations: *high, narrow arched palate *underdeveloped maxilla and maxillary sinus A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

CLEIDOCRANIAL DYSPLASIA treatment

Treatment: 1. care of deciduous teeth 2. multidisciplinary approach

•Clinical Features: 1. affects head, face 2. other areas: Spina bifida occulta A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

T/F Craniofacial Dysostosis: •Cranial synostosis with syndactyly

False •Cranial synostosis WITHOUT syndactyly

T/F Craniofacial Dysostosis is hereditary

Triangular frontal defect Steep forehead Hypertelorism Exophthalmus Antimongoloid slope of eyes A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

Parrot-beak nose A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

Frog-like features A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

Oral Features: 1. hypoplasia of maxilla 2. mandibular prognathism 3. high-arched palate A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

Craniofacial Dysostosis treatment

Treatment and Prognosis: 1. craniectomy; cosmetic surgery 2. may lead normal lives 3. may or may not be retarted

coloboma of the outer portion of the lower eyelids A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

*deficiency of eyelashes *malformation of middle and inner ear *ear: underdeveloped; malformed; or prominent A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

(Treacher-Collin Syndrome)

MANDIBULOFACIAL DYSOSTOSIS

*blind fistulas between angles of the ear and angle of mandible * atypical hair growth *hypoplasia of malar bones and mandible A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

*increased anterior facial height *. anterior open bite * small chin A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

bird-like or fish like features A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

E. MANDIBULOFACIAL DYSOSTOSIS

Other Features: 1. grossly underdeveloped paranasal sinuses 2. absent / deficient auditory ossicles, cochlea, and vestibular apparatus A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

E. MANDIBULOFACIAL DYSOSTOSIS

normal cranial vault A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

E. MANDIBULOFACIAL DYSOSTOSIS

MANDIBULOFACIAL DYSOSTOSIS treatment

©Can live a normal life span; reconstruction of deformities

Anomalad

Pierre Robin Syndrome

Primary defect of Pierre Robin Syndrome

Micrognathia

Secondary defect of Pierre Robin Syndrome

Cleft palate

Tertiary defect of Pierre Robin Syndrome

*respiratory difficulty *glossoptosis

is a malformation together with its subsequently derived structural changes, the primary defect setting off a series of secondary or even tertiary events resulting in multiple anomalies.

anomalad

Arachnodactyly A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

G. MARFAN SYNDROME

autosomal dominant; defect on chromosome 15 codes for the connective tissue protein, fibrillin A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

G. MARFAN SYNDROME

musculoskeletal, cardiac, and ocular problems predominate. A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

G. MARFAN SYNDROME

1. disproportionately long thin extremities A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

G. MARFAN SYNDROME

funnel chest pigeon breast A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

G. MARFAN SYNDROME

kyphosis / scoliosis A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

G. MARFAN SYNDROME

*dislocation of lens of eyes; nearsightedness *pale blue sclera *cardiovascular complications A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

G. MARFAN SYNDROME

Oral Manifestations: 1. high-arched palate 2. bifid uvula 3. malocclusions 4. odontogenic cysts A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

G. MARFAN SYNDROME

TMJ dysarthrosis A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

G. MARFAN SYNDROME

cardiovascular compromise: most common cause of patient death. A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

G. MARFAN SYNDROME

Txt of Marfan Syndrome

* no specific treatment *management of the cardiovascular manifestations *Patient longevity now approaches that of persons without Marfan syndrome

T/F Etiology of Down Syndrome is advanced maternal age

*uterine and placental abnormalities * chromosomal aberrations A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

H. DOWN SYNDROME

subnormal mentality with cranial and facial deformities A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

H. DOWN SYNDROME

Features: 1. sexual underdevelopment 2. cardiac abnormalities 3. hypermobility of joints A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

H. DOWN SYNDROME

*short stature with short hands and fingers *may have acute leukemia A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

H. DOWN SYNDROME

Oral Features: 1. high-arched palate 2. microdontia 3. enamel hypoplasia 4. teeth with abnormal shape and form 5. abnormal eruption sequence 6. periodontal disease A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

H. DOWN SYNDROME

T/ F Oral features of DOWN SYNDROME, is high-arched palate and periodontal disease

• Macroglossia • open-mouthed appearance • Bifid uvula A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

H. DOWN SYNDROME

2 Main Types: 1. Benign Dominant 2. Malignant Recessive A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

I. OSTEOPETROSIS

T/f DOWN SYNDROME has Teeth with short roots

Marble Bone Disease

OSTEOPETROSIS

Albers-Schonberg Disease

OSTEOPETROSIS

Osteosclerosis Fragilis Generalista

OSTEOPETROSIS

Radiographic Features: * dense bone * obliterated sinuses *. hypoplastic maxilla A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

I. OSTEOPETROSIS

OSTEOPETROSIS: multiple pathologic fractures A. Benign Dominant Form B. Malignant Recessive Form

OSTEOPETROSIS: bone pain A. Benign Dominant Form B. Malignant Recessive Form

OSTEOPETROSIS: cranial nerve palsy A. Benign Dominant Form B. Malignant Recessive Form

OSTEOPETROSIS: osteomyelitis A. Benign Dominant Form B. Malignant Recessive Form

OSTEOPETROSIS: 1. optic atrophy 2. hepatosplenomegaly A. Benign Dominant Form B. Malignant Recessive Form

OSTEOPETROSIS: *poor growth *frontal bossing A. Benign Dominant Form B. Malignant Recessive Form

OSTEOPETROSIS: loss of hearing A. Benign Dominant Form B. Malignant Recessive Form

OSTEOPETROSIS: pathologic fractures A. Benign Dominant Form B. Malignant Recessive Form

OSTEOPETROSIS: *facial palsy *genu valgum A. Benign Dominant Form B. Malignant Recessive Form

T/ F OSTEOPETROSIS’ Oral Manifestation are 1. enamel hypoplasia 2. slow root development 3. caries - prone 4. delayed tooth eruption

Radiographic Features: roots of teeth "invisible" A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

I. OSTEOPETROSIS

T/ F Osteopetrosis develop osteomyelitis

T/ F Osteopetrosis develop genu valgum

Treatment for osteopetrosis: Adult and infantile

Infantile osteopetrosis: Calcitriol, Erythropoietin' Corticosteroids, Gamma interferon Adult osteopetrosis: requires no treatment by itself complications might require intervention.

Features: 1. short stature with thickened musculature 2. brachycephalic skull 3. small hands and stubby fingers A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

J. ACHONDROPLASIA

T/ F osteopetrosis: Prognosis: remarkable change after bone marrow transplantation. Adult osteopetrosis: good long-term survival rates

T/ F ACHONDROPLASIA is an Autosomal Dominant

*lumbar lordosis * limited joint movement *. unusual physical strength and ability *normal intelligence A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

J. ACHONDROPLASIA

Oral Features: 1. mandibular prognathism / maxillary retrusion A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

J. ACHONDROPLASIA

Histologic Features: 1. aplasia of the zone of calcification of endochondral growth 2. disruption of the longitudinal growth of bone A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

J. ACHONDROPLASIA

T/F Achondroplasia’s treatment and prognosis is none and they lives a normal lives

T/ F OSTEITIS DEFORMANS has specific etiology

False (Non-specific etiology)

T/ F OSTEITIS DEFORMANS Clinical features: 1. pathologic fractures 2. bone pain 3. severe headache 4. dizziness 5. deafness and blindness 5. facial paralysis 6. weakness

T/F OSTEITIS DEFORMANS progressive skull enlargement, deformed spine, femur, tibia

T/F OSTEITIS DEFORMANS broadening and flattening of chest, areas affected feels warm

Affected bones (sacral and lumbar vertebrae, pelvis, tibia, femur) soften and bend A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

K. OSTEITIS DEFORMANS

Oral Manifestations: 1. progressive enlargement of maxilla with widening and flattening of the palate 2. teeth: loose, migrating 3. open-mouth appearance A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

K. OSTEITIS DEFORMANS

Hypercementosis Loss of lamina dura mosaic-bone appearance A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

K. OSTEITIS DEFORMANS

Histologic Feature: 1. mosaic-bone appearance or jigsaw puzzle appearance of bone resorption and deposition A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

K. OSTEITIS DEFORMANS

Osteosarcoma due to Paget's disease A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

K. OSTEITIS DEFORMANS

Paget’s disease of bone A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

K. OSTEITIS DEFORMANS

O Features: 1. swelling at the angles of the mandible and bridge of nose 2. loss of vision and facial sensation 3. facial paralysis 4. deafness 5. overgrowth of alveolar process A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

L. GENERALIZED CORTICAL HYPEROSTOSIS

T/F GENERALIZED CORTICAL HYPEROSTOSIS has no treatment and patient can live normal lives

Features: 1. pathologic fractures after minor trauma 2. destruction of mandible 3. facial asymmetry A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

M. MASSIVE OSTEOLYSIS

polyostotic: clavicle, ilium, ishium, humerus, ribs, sacrum A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

M. MASSIVE OSTEOLYSIS

Cortical and cancellous bone showing osteoporosis with an increased number of capillaries and small arterioles A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

M. MASSIVE OSTEOLYSIS

T/F . MASSIVE OSTEOLYSIS has No specific treatment

osteolytic process involving the whole of the femur and vital teeth with ill-defined radiolucency A. OSTEOGENESIS IMPERFECTA B. INFANTILE CORTICAL HYPEROSTOSIS C. CLEIDOCRANIAL DYSPLASIA D. CRANIOFACIAL DYSOSTOSIS E. MANDIBULOFACIAL DYSOSTOSIS F. PIERRE ROBIN SYNDROME G. MARFAN SYNDROME H. DOWN SYNDROME I. OSTEOPETROSIS J. ACHONDROPLASIA K. OSTEITIS DEFORMANS L. GENERALIZED CORTICAL HYPEROSTOSIS M. MASSIVE OSTEOLYSIS N. FIBROUS DYSPLASIA OF BONE O. POLYOSTOTIC FIBROUS DYSPLASIA P. MONOSTOTIC FIBROUS DYSPLASIA OF THE JAWS

M. MASSIVE OSTEOLYSIS

Lesions affecting multiple bones A. McCune-Albright Syndrome B. Jaffe type

(Polyostotic Fibrous Dyplasia) A. McCune-Albright Syndrome B. Jaffe type

Café-au-lait pigmentation A. McCune-Albright Syndrome B. Jaffe type

Endocrine disturbances A. McCune-Albright Syndrome B. Jaffe type

> Pathologic fractures with pain and deformity > Teeth: disturbed eruption pattern; jaw expansion A. McCune-Albright Syndrome B. Jaffe type

Histologic feature: Chinese script-writing appearance A. McCune-Albright Syndrome B. Jaffe type

Leontiasis ossea A. Monostotic Fibrous Dysplasia B. Polyostotic Fibrous Dyplasia

massive jaw expansion A. Monostotic Fibrous Dysplasia B. Polyostotic Fibrous Dyplasia

ground glass or orange peel appearance A. Monostotic Fibrous Dysplasia B. Polyostotic Fibrous Dyplasia

Localized intramedullary fibrous lesion with curved woven bone Chinese letters (thick material) A. Monostotic Fibrous Dysplasia B. Polyostotic Fibrous Dyplasia

-osteoid material surround by fibrous stroma A. Monostotic Fibrous Dysplasia B. Polyostotic Fibrous Dyplasia

Fibroblasts in woven bone, Oral hyperpigmentation A. Monostotic Fibrous Dysplasia B. Polyostotic Fibrous Dyplasia

cafe-au-lait A. Monostotic Fibrous Dysplasia B. Polyostotic Fibrous Dyplasia

Treatment of Fibrous Dysplasia:

> Surgery: mainly to alleviate functional disturbances > radiation therapy is contraindicated

T/ f CHERUBISm is an autosomal dominant

Slow-growing expansion of posterior portion of maxilla and mandible

CHERUBISM

Radiographic appearance: soap-bubble appearance

CHERUBISM

Tx: self-limiting ; conservative curettage of lesion with bone recontouring

CHERUBISM

Oral Manifestations: > Premature loss of primary teeth > Displacement of follicles of permanent teeth

CHERUBISM

> Ectopic eruption > Permanent teeth may be missing or malformed

CHERUBISM

bilateral expansion of rami

CHERUBISM