is a heritage osseous disease,

characterized for the bones are extremely

fragile with a defect in osseous matrix.

Etiology Heritage is transmitted as an autosomal dominant trait. A

defect of biosynthesis of type I collagen causes

osteoblasts to fail to form bone in adequate amounts

Osteogenesis

Imperfecta

Clinical feature

Osteogenesis Imperfecta

•Appears follows a family patron

•bones are very thin and lack the

usual cortex of compact bone but

development of epiphyseal

cartilages is unimpaired so that

bone can grow to their normal

length but may be so distorted by

multiple fractures.

•In severe cases, die at birth or

soon after.

•The sclera of the eyes may appear

blue because it allows see the

pigment layer through it.

•Deafness and dentinogenesis

imperfecta.

•May appear dental fracture. and

jaws fracture during extraction is

uncommon

Radiography: Dental roots are short and decrease of the radiopacity of

dentin. The bones are poor and this cortical defect

Osteogenesis Imperfecta

Management: Oral hygiene, prevention of trauma, taken care during extraction.

Osteopetrosis Is a rare genetic osseous disease in which the

bone become solidified, dense and with excessive

growth, but its brittle

Etiology: It is follows a autosomal recessive trait that is very severe and

other follows autosomal dominant trait that is less severe

Osteopetrosis

Clinical feature

•Begin at infancy, but is notable at second

decade of life .

•Great head and short height

•Spontaneous fractures for poor

elasticity.

•Optic atrophy and deafness for stenosis

of cranial channel.

•Anemia, leucopenia, and

trombocithopenia for loss marrow

•The dental eruption is retarded, and

malocclusion

•Osteomyelitis a recognized complication. Radiography:

Osseous sclerosis, with decrease of

medullar space.

Management

•Oral hygiene. Avoid extraction.Antibiotic prevention

•Bone marrow transplantation

Achondroplasia It’s a common type of genetic bone disorders,

associated to failure of normal cartilage

proliferation in the epiphyses and base of the

skull

Aetiology: Heritage, follow a autosomal dominant trait.

Clinical feature

Achondroplasia

•Is a type of genetic dwarfism.

•Very short height.

•Limbs are very short in relation to the

trunk, while the head, which is normal in

size , appears disproportionately large.

•The middle third of the face is retrusive for

a defective growth of the base of the skull

•Concave profile

•Malocclusion, with mandible is often

protrusive. For disparity of the jaws growth

•Dental dimorphisms and decrease of

dental formulae. Radiography Poor growth of base of the skull

Management: Oral hygiene

Orthodontic treatment or maybe

orthognatic treatment

Cleidocranial dysplasia It’s a rare familiar disorder with

dental and osseous disorders

Etiology: Heritage, its follow a autosomal dominant trait

Cleidocranial dysplasia

Clinical feature •Familiar patron.

•Short height

•Partial or complete absence of

clavicles, allows the patients to bring

the shoulders together in front of the

chest.

•Delayed eruption of the permanent

teeth, may remain embedded in the

jaw and may have associated

dentigerous cysts.

•Alteration of dental formulae.

Radiography:

Seen the several included teeth and

the absence of clavicles.

Treatment.

Oral hygiene. Eruption control .Orthodontic and surgical manage.

Rickets It’s a general disease associated to deficiency of

vitamin D. and may be called according to appears

time: rickets if appears in infancy and osteomalasie in

the adult

Etiology: The vitamin D is essential for calcium, and phosphorus metabolism,

its deficiency may associated to poor ingestion of vitamin, for

absorption defects, chronic kidney diseases, poor sun exposition.

This produces a deficiency of the bone calcification with inadequate

mineralization of matrix.

Rickets Clinical feature

•The main defect are broadening of the

growing ends of bone and prominent

costochondral junctions,

•Wide fontanelles, bossing of frontal

and parietal eminences. And thinning

of the back of the skull.

•Hypocalcification of the dentin in

severe case.

• Delayed eruption,

•Is not very susceptible to caries.

•In adults is seen an extreme fragile of

bone with fracture tendency.

•Radiography: the wide, thick

epiphysis and deformities. Included

teeth.

Management: 1)adequate diet. 2)Administration of 200-3000 iu daily, and

3)orthopedic treatment is needed.

Osteitis Deformans Its a chronic osseous disease that

cause bone distortion and

weakening, particularly in elderly

Etiology

Unknown

intranuclear inclusion compatible with a

paramyxo or others virus have been demonstrated in both osteoclasts and

osteoblasts

Genetic

Vascular disorders a result of gen activate

for eruptive virus in male

Osteitis Deformans Clinical feature

•Appears in male more frequently to female, after 40

years.

•Affecting mainly to sacrum, spine, skull, femora and

pelvis.

•May be widespread and is usually symmetrical. But

sometimes affected a single bone.

•Enlarged head, thickening of long bones, which bend

under stress.

•Tenderness or aching pain that may be severe

•The vault of the skull is more often affected than the

facial skeleton.

•The maxilla is more affected to mandible.

•Narrowing of foramina can produce cranial nerve

defects, like to deafness, blindness , cephalea

•The alveolar process becomes severally and grossly

enlarged, allow to periodical denture

substitution.Malocclusion.

•Hypercementosis which may attempt to extraction,

•Severe bleeding and osteomyelitis for the ischaemia of

the bone

•Histology: mosaic patterns of bone.

Radiography:

Lower density of the bone, and a characteristic aspect called cotton-

wool appearance. Hypercementosis.

Osteitis Deformans

Management:

•Oral hygiene. Calcitonins .Bisphosphonates .calcitonins

Osteomielitis is an inflammatory process of medullar

spaces and bone for several causes

Classifications:

1.Acute Suppurative Osteomielitis

2.Chronic Suppurative Osteomielitis

3. Focal Chronic Sclerosing Osteomyelitis

4.Difusse Chronic Sclerosing Osteomielitis

5.Osteomyelitis with Proliferative Periostitis (Garre Osteomyelitis)

Osteomielitis

Acute and chronic

suppurative osteomielitis

as an infection of the medullary

portion of bone that includes the

production of pus

Etiology an extension of a dentoalveolar

infection ,

a complication of a fractura,

to infections from extraction

sockets,

Rare cases are related to

bloodborne pathogens

Clinical feature •Patients will variably present with some or

all of the signs of inflammation, including

swelling, limited motion, erythema, and

warmth of the overlying tissues.

•Most will have pain.

•Most will be afebrile and have a normal

white cell count unless the infection is

severe. In such cases, a leukocytosis with

a slight left shift toward immature

neutrophils is seen.

•The chronic type ,the symptom are less

evident

•The mandible is affected much more than

is the maxilla

•will create an ischemic neuropathy,

causing lip paresthesias.

Acute and chronic

suppurative osteomielitis

X ray

Acute and chronic

suppurative osteomielitis

in acute ,the x ray findings are

poor , only the causal agent. In

chronic an irregular radiolucent

pattern with ragged borders

develops Some radiographs will

show a portion of bone

separated from the parent bone.

This has been termed a

sequestrum, and the radiolucent

band separating it from the

parent bone an involucrum

Treatment:

•Remove the infection source.

•all necrotic tissue should also be

debrided and tissue samples taken

for Gram staining, aerobic culture,

and anaerobic culture before

antibiotics are administered

•soft diet

•systematic irrigations with

antiseptics or antibiotics

•antibiotic therapy

•Hyperbaric oxygenation

Acute and chronic

suppurative osteomielitis

Chronic Sclerosing

Osteomielitis

This form is less symptomatic , with

two forms. The focal associated to

few teeth and diffuse widespread by

jaws with large evolution.

Etiology: caused by one of the Actinomyces species in a

mutualism with E corrodens. Microorganism with low virulence in

high resistant organism Clinical feature:

•more in female ,in mandible

and in blacks .

have painless period ,

associated to sharp pain

period

•no suppurate and not

drainage

•tender to palpation.

•the focal form is associated

with first or second molars

and its asymptomatic

generally

Chronic Sclerosing

Osteomielitis

x ray:

radiopaque areas with irregular

margins , can give cotton-wool

appearance. focal shows

radiopaque areas associate to

tooth affected

focal

diffuse

Treatment. •Oral hygiene

•Avoid dental extraction

•In the focal case follow up

•In the diffuse if it is symptomatic

the same care that suppurative

form

Osteomyelitis with

Proliferative Periostitis

(Garre osteomyelitis)

It is variant of a chronic osteomyelitis

found in young individuals because of

their high resistance, increased local

blood supply, and greater bone

regenerative capabilities

Clinical feature:

•It presents most often in a child or

teenager associated with an apical

infection in a mandibular posterior

tooth

•Appears in mandible, It does not

seem to occur in the maxilla

•The expansion is bone hard and not

usually painful or tender to

palpation. There is no pus, drainage,

or erythema

X ray . it is the deposition of

extracortical new bone outside an

existing intact cortex(“onion skin”

effect).widening of the cortical bone

Treatment:

To remove of the source of

infection and antibiotic therapy

Osteomyelitis with

Proliferative Periostitis

(Garre osteomyelitis)

Bibliographical References