1
Q
Osteogenesis Imperfecta
A
- Mutation
- COL1A1 (Chromosome 12)
- COL1A2 (Chromosome 7)
- Types:
- Mild (type 1)
- increased fracture of limbs
- hearing loss or premature osteoporosis
- Moderate (Type 3-9)
- more bone fractures and deformities
- Cardiovascular defects
- short stature
- hyperflexible
- Lethal (type 2)
- prenatal lesions
- pulmonary failure
- prenatal lesions
- Mild (type 1)
2
Q
Osteogenesis Imperfecta: Bone Manifestations
A
- Brittle bones
- Short Stature
- Basilar Skull deformities
- pressure on cranial nerves
3
Q
Osteogenesis imperfecta: Clinical Manifestations
A
- Blue sclera
- Wormian bones
- increased Class III Malocclusion
4
Q
Osteogenesis Imperfecta: Oral Manifestations
A
- Opalescent teeth
- wear quickly
- Yellow/gray color
- Radiograph:
- CEJ constriction→Tulip shaped teeth
- short and blunted roots
5
Q
Osteopetrosis
A
- aka: Marble Bone Disease
- Erlenmeyer Flask Appearance
- Types:
- Malignant Type
- Intermediate Type
- Benign Type
6
Q
Osteopetrosis: Malignant Type
A
- aka autosomal recessive infantile type
- Severe form
- at birth/early age
- Anemia
- Hypertelorism (Broad Face)
- widespread skeletal density (X-ray)
- increased density in all bones
- Poor prognosis (<1 year after birth)
7
Q
Osteopetrosis: Intermediate Type
A
- aka Autosomal Recessive Intermediate Type
- Asymptomatic at birth
- fractures appear at end of 1st decade
- Less severe form of infantile
- RANKL
- RANK
8
Q
Osteopetrosis: Benign Type
A
- Aka Autosomal Dominant Adult Type
- Most common type
- Adolescents (10-19)
- less severe manifestations
- 40% asymptomatic
- LRP5
- bone formation by osteoblasts
- CLCN7
- chloride channel
9
Q
Osteopetrosis: Management
A
- No Tx=poor prognosis
- die during 1st decade
- Bone marrow transplant
- Corticosteroids
- limit calcium intake
10
Q
Massive Osteolysis
A
- Aka
- Vanishing Bone Disease
- Phantom Bone Disease
- Mostly children and young adults
- Spontaneous progressive destruction of 1+ bones
- replaced w/vascular proliferation
- fills w/fibrous tissue (does not regenerate/repair)
11
Q
Massive Osteolysis: Clinical Features
A
- mobile teeth
- pain
- malocclusion
- deviation of mandible
- obvious deformities
12
Q
Massive Osteolysis: Radiographic Findings
A
- Large portions of bone disappear
- New area→before obvious radiolucency
- loss of lamina dura
- thinning of cortical plates
13
Q
Massive Osteolysis: Management
A
- Radiation Therapy
- most successful
- risk-post radiation sarcoma
- Bisphosphonate Therapy
14
Q
Cleidocranial Dysplasia
A
- Autosomal Dominant mutation
- RUNX2 gene on chromosome 6q21
- Bone defects of:
- clavicles
- skull
15
Q
Cleidocranial Dysplasia: Clinical Features
A
- Short Stature
- Long “Swan” Neck
- Hypertelorism
- Depressed Nasal Bridge
- Delayed Closing of Skull sutures
16
Q
Cleidocranial Dysplasia: Oral Manifestations
A
- Maxilla:
- thin zygomatic arch
- Small/absent maxillary sinus
- Numerous impacted teeth
- permanent teeth delayed or failure to erupt
- retention of deciduous teeth
- Palate-Narrow, High arch
- increased prevalence of cleft palate
17
Q
Cleidocranial Dysplasia: Management
A
- Full mouth extraction and give denture
- Before adult
- prevent lower face height and mandibular prognathism
18
Q
Focal Osteoporotic Bone Marrow Defect
A
- area of hematopoietic marrow→ produce radiolucency
- Asymptomatic
- identified by accident
- Women
- Posterior Mandible
- Incisional Biopsy
- find bone marrow
- necessary to establish dx
19
Q
Idiopathic Osteosclerosis
A
- focally/focused increased bone density
- unknown cause
- must rule out
- inflammation
- dysplastic
- neoplastic
- systemic disorders
- If multiple lesions-rule out Gardner Syndrome
20
Q
Paget’s Disease of Bone
A
- aka osteitis deformans
- 2nd most common metabolic bone disorder
- osteoporosis=1st
- Anarchic resorption and deposition of bone
- result=distortion and weakened
- Angio-Saxon ancestor
- rare before 40 y.o.
- most are asymptomatic
- some pain due to turnover or secondary complications
21
Q
Paget’s disease of Bone: Clinical Manifestation
A
- Monkey Leg Stance
- Bone gets thicker and thinner over time
22
Q
Paget’s disease of Bone: Head and Neck Manifestations
A
- Enlarged middle ⅓ of Face
- generalized hypercementosis
- Osteoporosis circumscripta
- large radiolucency in skull
- early stage
- cotton wool appearance
- Lincoln’s Sign (Black Beard)
- Mandible
23
Q
Paget’s disease of Bone: Histology
A
- Jigsaw Puzzle w/Basophilic reverse lines
- “mosaic appearance”
- looks like enamel formation
- “mosaic appearance”
24
Q
Paget’s Disease of Bone: Complications
A
- Hypercementosis
- Osteomyelitis (late disease)
- Osteosarcoma
- Giant Cell tumors
25
Central Giant Cell Granuloma
* Not a true granuloma
* Unknown Etiology
* Type:
* Non-aggressive: Mainly
* Aggressive
* Multifocal presentaiton
* Cherubism
* Hyperparathyroidism
26
Central Giant Cell Granuloma: Non-aggressive granuloma
* most common
* small, asymptomatic
* slow, painless jaw expansion
27
Central Giant Cell Granuloma: Aggressive Type
* rapid growth
* painful
* Cortical Proliferation
* root resorption
* tooth displacement
28
Cherubism
* Manifestation of Central Giant Cell Granuloma
* Eyes turned up to heaven
* 2 balls at chin
* displaced teeth
29
Hyperparathyroidism
* manifestation of Central Giant Cell Granuloma
* _Brown tumor_
* involves:
* mandible
* clavicle
* Secondary Hyperparathyroidism of chronic renal disease
* _Renal Osteodystrophy_
* striking enlargement of jaw
* Secondary HPT
30
Simple Bone Cyst
* aka traumatic bone cyst
* empty or fluid filled cavity in bone
* no epithelium
* Unknown Etiology:
* Trauma-hemorrhage Theory
* internal hematoma due to trauma
* Mostly In long bone
* Humorous
* proximal femur
* Painless swelling
* posterior mandible
* apex of teeth don't have support
31
Aneurysmal Bone Cyst
* Intraosseous rdiolucency
* blood filled
* surrounded by fibrotic tissue and reactive bone
* Classified as::
* Primary-arising de novo
* Secondary: associated with another bone disease
* Oral Manifestations:
* Rapidly enlarging swelling
* Mandible\>maxilla
* cortical expansion and thinning
* **Blood-soaked sponge**
* Histology:
* Contains Multinucleated Giant cells
32
Fibrous Dysplasia
* Benign
* bone replace with cellular fibrous tissue and irregular trabeculations
* Types:
* _Monostotic Fibrous Dysplasia_
* _Polystotic Fibrous Dysplasia_
33
Monostotic Fibrous Dysplasia
* Type of Benign Fibrous Dysplasia
* mostly limited to 1 bone
* "Ground Glass Appearance
* Superior displacement of Inferior alveolar nerve is possible
* **Craniofacial Fibrous Dysplasia**
34
Polyostotic Fibrous Dysplasia:
* Type of Benign Fibrous Dysplasia
* 2+ bones
* Dx before 10 y.o.
* Painful
* associated with:
* Jaffe-Lichtenstein Syndrome
* McCune-Albright Syndrome
* Mazabraud syndrome
* “Coast of maine”
35
Cemento-Osserous Dysplasia
* **Most common Benign Fibrooseous lesion (BFOL) in clinic**
* tooth bearing areas
* derived from:
* PDL or…
* bone=_reactive lesion_
* Types:
* Focal COD
* Periapical COD
* Florid COD
36
Focal Cemento-Osseous Dysplasia
* Type of COD
* single site
* **mainly Posterior Mandible**
* Caucasian Females
* mixed radiolucent and radiopaque pattern
* well defined w/slightly irregular borders
* Vitality test
37
Periapical Memento-Osseous Dysplasia
* Type of COD
* Mainly Periapical region of anterior mandible
* African American Females
* Early Stage:
* circumscribed radiolucency at apex
* End Stage
* circumscribed dense calcification surrounded by narrow radiolucent rim
38
Florid Cements-Osseous Dysplasia
* multifocal involvement (more than one focus)
* African American female
* middle aged
* Dx:
* multiple lesions w/vital anterior teeth
* no need for biopsy?
39
Familial Gigantiform Cementoma
* Autosomal Dominant Trait
* Significant facial deformity
* limited to jaws-Maxilla and mandible
* Radiograph:
* look similar to COD
* multiple radiolucencies in periodical regions
* Tx:
* reseection
* if treated to early, may grow back
40
Ossifying Fibroma
* True neoplasm w/growth potential
* mostly women
* 3rd-4th decade
* Mandible
* premolar/molar area
* commonly identified in pt w/**Hyperthyroidism-Jaw Tumor**
*
Week 77
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