Diseases of bone &

osteodystrophies
 Learning objectives

By the end of this class you should be able to

* Understand the meaning of bone dystrophies
* Recall the different types of jaw osteodystrophies
* Describe the various types of jaw osteodystrophies-
clinical and radiographic manifestations
 Osteodystrophies definition

* Osteodystrophies are disorders of bone other than

neoplastic and inflammatory conditions.

What are its implications in dentistry?

Classification of osteodystrophies in
OMF region

* Fibro cement osseous lesions

* Giant cell lesions
* Inherited and developmental disorders of bone
* Metabolic disorders of bone
* Miscellaneous
 Working Classification Of Fibro-Osseous
Lesions By Mico M. Malek (1987)

* 1. Developmental Disorders
* A. Fibrous Cortical Defects (Non Ossifying Fibroma)
* B. Fibrous Dysplasia
* 2. Reactive Reparative Lesions
* A. Traumatic Periosteitis
* B. Periosteitis Ossificans
* C. Osseous Keloid
* D. Periapical Cemental Dysplasia & Florid Cemento-Osseous
Dysplasia
* E. Sclerosing Osteomyelitis (Focal & Diffuse Type)
* F. Osteitis Deformans
* 3. Fibromatosis
* A. Desmoplastic Fibroma (Intraosseous Fibromatosis)
* 4. Neoplasms
* A. Tooth Bearing Areas Only i. Cementoblastoma
ii. Periodontoma 1. Central 2.
Peripheral
* B. All Cranio-Facial Bones (Including Tooth Bearing Areas)
* i. Osteoma 1. Trabecular 2. Compact
* ii. Osteoid Osteoma
* iii. Psammous Desmo-Osteoblastoma
* iv. Trabecular Desmo-Osteoblastoma
WHO Classification Of Fibro-Osseous
Lesions(2005)
1) Ossifying Fibroma (OF)
2) Fibrous Dysplasia
3) Osseous Dysplasia
a. Periapical Osseous Dysplasia
b. Focal Osseous Dysplasia
c. Florid Osseous Dysplasia
d. Familial Gigantiform Cementoma
4) Central Giant Cell Granuloma
5) Cherubism
6) Aneurysmal Bone Cyst
7) Solitary Bone Cyst P
 Fibro osseous lesions

* Bone is replaced by cellular fibrous tissue which

gradually matures with the formation of woven bone,
lamellar bone, or very dense amorphous mineralization.

* Radiographic correlation- ???

Diagnosis of fibro/cemento osseous
lesions

Clinical
Radiological
and
Histopathological correlation

Not by features in isolation

 Fibrous Dysplasia

* Benign
* Self limiting
* Unencapsulated pathology
* normally occurring in young subjects.
* Painless swelling of the bone involved.
* Usually, a self-limiting disease
 Osseous Dysplasia

* Periapical osseous dysplasia

* Florid osseous dysplasia
* Familial gigantiform cementoma
Periapical osseous dysplasia
Florid osseous dysplasia
 Cemento ossifying fibroma

* Benign neoplasm of the bone- debatable

* Arising exclusively in the jaws, facial bones and skull
* Typically causes a painless swelling in the mandibular
premolar and molar region
* Females>males
* Fibro osseous lesion
 Cemento ossifying fibroma

* Radiographically-starts as small radiolucency and

expands slowly
* Calcification develops centrally as the lesion enlarges
* Most become densely calcified
* The lesion has a sharply defined margin often within
radiolucent rim surrounded by a narrow zone of
cortication
Cemento ossifying fibroma
Juvenile aggressive cemento ossifying
fibroma

* Commoner in children
 Cementoblastoma

* Patients mostly under 25 years

* Lesion usually attached to apex of mandibular molar or
premolar.
 Giant cell lesions

True giant cell lesions

* Central giant cell granuloma
* Peripheral giant cell granuloma
* Brown tumor of hyperparathyroidism
* Giant cell tumour
* Cherubism

Lesions that may contain giant cells

* Paget’s disease
* Fibrous dysplasia
* Aneurysmal bone cyst
 Central giant cell granuloma

* Benign reactive and hyperplastic lesion of unknown etiology

* More common in young females,
* Very expansile and may be destructive may penetrate cortical bone
and periosteum
* Solid but appear as unilocular or multilocular cyst like
radiolucency
* Forms in alveolar ridge, anterior to 1st molar in the mandible and
anterior to cuspid in the maxilla
* No changes in the blood chemistry
* Treated by curettage
* Lobulated mass of proliferating vascular connective tissue packed
with giant cells
 Cherubism

* Rare Inherited as an autosomal dominant trait

* Early childhood 2-6 years
* Signs- painless, firm, bilateral enlargement of the lower face
* Angle regions of mandible affected symmetrically giving
typical chubby face
* Symmetrical involvement of maxillae also in severe cases-
“eyes towards the heaven”
* Lesions regress with skeletal maturation and normal facial
contour restored
* Radiographically lesions appear as multilocular cyst like
areas
* Location- bilateral, epicentre is posterior mandible,
lesion grows in anterior direction, extends to midline
* Periphery- well defined
* Internal structure- fine granular bone, wispy trabeculae,
multilocular
* Effects on surrounding structure-expansion,
displacement of teeth
Paget’s Disease(osteitis deformans)

* Skeletal disorder-abnormal resorption and deposition

* Old age, male to female ratio- 2:1
* Affected bone –enlarged and deformed- Bowing of long
bones
* Jaws enlargement- malocclusion, dentures- tight &
poorly fitting
* Enlarged skull size
* elevated serum alkaline phosphatase and urine
hydroxyproline.
* Radiographic features-
* 3 stages- "hot" or "active" phase (osteolytic dominant, with
fibrous replacement of marrow and vascular hypertrophy)
* "mixed" phase (disorganized lamellar bone deposition plus
osteolysis; rapid bone turnover)
* "cold" phase (osteoblastic dominant; pagetoid woven bone
replaced by islands of lamellar bone, but haversian systems
and remodeling along lines of stress do not occur).
Radiographs-conventional, Bone scan
 Treatment

Goal is to arrest the osteolytic process

* Calcitonin (inhibits bone resorption)
* Disphosphonates (inhibit demineralization)
* Mithromicin (cytotoxic antibiotic)
 Osteogenesis imperfecta

* General bone disease with a

* triad of clinical features:

multiple fractures
blue sclera
hearing loss
* Short stature
* Triangular-shaped face
* Breathing problems
* Hearing loss
* Brittle teeth
* Bone deformities, such as bowed legs or scoliosis
 Osteopetrosis

* Failure to absorb calcified cartilage and primitive bone

due to deficient activity of osteoclasts
* Synonyms – marble bone disease, albers-schonberg
disease
dysostosis [dis″os-to´sis]defective ossification; a defect in
the normal ossification of fetal cartilages.

Dysostosis
 Cleidocranial Dysplasia

* Aplasia or hypoplasia of clavicles

Skull shows
* Delayed closure of fontanels
* Open skull sutures
* Sunken sagittal suture
* Frontal and occipital bossing
* Widened cranium
 Jaws show

* Underdeveloped maxilla-maxillary micrognathia

* Multiple supernumerary teeth-anterior to permanent
molars
* Unerupted or delayed eruption of permanent teeth
* Prolonged retention of primary teeth
* Mandibular prognathism-normal in size
* Sometimes multiple dentigerous cysts.
 Craniofacial dysostosis

* An autosomal dominant condition marked by a pointed

or conical skull, protruding wide-set eyes, strabismus,
parrot-beaked nose, and hypoplastic maxilla with
relative mandibular prognathism.
* AKA Crouzon's disease
 Common features

* Narrow/High-arched palate
* Posterior bilateral cross-bite
* Hypodontia (missing some teeth)
* Crowding of teeth
* maxillary hypoplasia- resultant masticatory
inefficiency
 Mandibulofacial dysostosis

* A hereditary disorder occurring in two different forms:

* The complete form is FRANCESCHETTI SYNDROME and the

incomplete form is TREACHER COLLINS SYNDROME.
* Downslanting eyes
(ANTIMONGOLOID palpebral fissures);
* Absence of all or part of the lower lid;
* Absence of zygomatic bones that appear
depressed;
* a prominent nose,
* wide mouth, and small receding chin;
* underdeveloped, malformed ears
* and small tufts of hair in front of the ears.
* There is often, but not always, some degree
of HEARING LOSS, usually conductive.
 Metaphyseal dysostosis

* Skeletal abnormality - epiphyses are normal or nearly

normal
metaphyseal tissues replaced by masses of cartilage

interference with endochondral bone formation and

expansion and thinning of the metaphyseal cortices.
 Orodigitofacial
dysostosis (orofaciodigital syndrome)

* Hyperplasia of the frenula,

* Cleft tongue
* Cleft palate.
* Less commonly there is absence of the lower lateral
incisors, alopecia or dryness of the hair, anomalies of the
facial or cranial bones, anomalies of the hands, or
mental retardation.
 Achondroplasia

* Most common type of genetic skeletal disorders

* Manifest as short limb dwarfism
* Failure of normal cartilage proliferation in the epiphysis
and base of the skull
* Normal intelligence.
* CNS not affected.
 Hyperparathyroidism

Two types - Primary and secondary hyperparathyroidism

* Predominantly in middle aged females
* Excessive parathormone secreting adenomas
* Present with generalized osteoclastic activity with fibrosis of
marrow
* In addition, focal areas of bone resorption result in brown
tumour formation.
* Usually present with giant cell epulis and cystic lesions.
 Treatment

* Surgical removal of adenoma and subtototal excision of

hyperplasia
 Rickets and osteomalacia

* Due to deficiency or resistance to Vitamin D

* Present with reduced bone density and failure of bone
mineralization.
Thank you