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Topics to be discussed:

-Cleidocranial dysplasia-Hemifacial hyperplasia-Segmental odontomaxillary dysplasia-Lingual salivary gland depression-Focal osteoporotic bone marrow

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Cleidocranial dysplasia

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•Autosomal dominant syndrome affecting bones and teeth•Affect both sexes equally•Can be inherited or as a result of sporadic mutation-Runx2 gene

CCD affect mainly skull, clavicle and dentition

-Face appear small in contrast to cranium-Bridge of nose may be broad and

depressed Hypertelorism

-Aplasia/hypoplasia of clavicle

Clinical features:

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parietal +Frontal bossingUnderdeveloped maxillaExcessive mobility of shoulderAppear shorter than unaffected relative

-Prolonged retention of 1˚-Delayed eruption of 2 ˚-Often have unerupted supernumerary teeth

-extraction of 1 ˚ doesn’t adequately stimulate eruption

of 2 ˚

Lack of development of parietal bone

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-a light-bulb shape due to brachycephaly-delayed or failure of fontanelles-open skull suture

Lateral skull film

Posteroanterior skull film

Radiographic features

Maxillary micrognathia-underdeveloped maxilla and

paranasal sinus

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Absence of clavicle Open fontanel

Wormian (sutural) bones in the occipital region

Chest radiograph

Lateral radiograph

• 3D CT reconstruction with oblique orientation

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Parietal bossing

Open metopic suture

Frontal bossing

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Lack of normal coronoid process

Multiple unerupted supernumerary teethMostly anterior max. n PM region

Prolonged retention of 1˚ dentition

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Multiple of unerupted teeth

Axial CT view

• done by :--Family history-excessive mobility of movement-Examination of skull-Radiographic finding

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Diagnosis

other disease associated with supernumerary and multiple unerupted teeth

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2)Pycnodysotosis1)Gardner’s syndrome

Presence of clavicleAbnormally dense brittle bone

Short statue

Bone osteoma

Multiple intestinal polyps

Differential Diagnosis

-removal of 1˚ + supernumerary teeth-removal of bone overlying 2˚ to expose crown

when half of the root is formed-monitor for any distal molar or cyst-surgical treatment for esthetic reason-CT scan

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Management

Hemifacial dysplasia

• Hemifacial hypertrophy, hemi hyperplasia

• A condition that half of face including max. (alone @ with mand) @ other part of body to grow to unusual proportion

• Cause:-unknown,may associate with genetic disease (Beckwith-Weidemann syndrome)

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-Usually begin at birth-Often occur with other abnormalities: mental

deficiency,skin abnormalities,compensatory scoliosis,genitourinary tract

anomalies and neoplasm e.g. Wilms’ tumor of

kidney,adrenocorticol tumor,hepatoblastoma (Beckwith-

Weidemann syndrome)-F=M affected Dentition

-Unilateral enlargement-accelerated development

-premature loss of 1˚-enlarged tongue and

alveolar one in affected side

clinical feature

If not detected during birth, it may become apparent

during growth

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-dysmorphic faceleft hemi facial hypertrophy

ear lobe crease

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Radiographic feature :

Rapid enlargement on right side of maxilla only-accelerated dental development for this 5-year-old patient

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Enlargement of bones include

mand.maxilla,zygoma,frontal and temporal bone

Enlargement of maxillary

canine,1st PM

CT axial image

3D CT scan

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1)Hemifacial hypoplasia of the opposite side

2)Arteriovernous aneurysm

3) Hemangioma

4) Congenital lymphadema

5)Severe condylar hyperplasia that may involve half of mandible

Differential Diagnosis

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D/D for case that limited to one side of maxillary

1)Monostatic fibrous dysplasia--A rare bone disorder characterized by benign bone growths which can

cause very painful swellings and bone deformities and makes bone

prone to fractures.

2)Segmental odontomaxillary dysplasia

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-no significant case reported with long term follow-up , hence no definitive recommended treatment -generally those with suspected HH should be referred to a medical geneticist for diagnosis and early detection of genetic syndrome associated

Management

Segmental odontomaxillary dysplasia

-Hemimaxillofacial dysplasia• A developmental abnormality of unknown

etiology that affect posterior alveolar process of one side of maxilla including teeth and attached gingiva

• Detected most in Childhood

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--fullness of the right upper lip

due to enlargement of

the alveolar process.

delayed eruption of

teeth on the affected side

Clinical features:-Always unilateral enlargement of alveolar

process, gingiva and teeth-Frequently missing teeth (mostly PM)

-Some teeth may unerupted at the affected side-unilateral hypertrichosis + mild facial

enlargement some cases

hypertrichosisfacial enlargementIntra-oral mirror image

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-a radiodensity that reduced the size of the

right maxillary sinus- Both PM in the affected hemimaxilla were present

-Maxilary sinus does not pneumatize the alveolar process-large left max.deciduos molars-lack of formation of bicuspids-delayed eruption of first molar -dense bone pattern of left maxillary alveolar process

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-Coarse trabecular pattern of right maxillary alveolar process

-delayed eruption of maxillary right 1st PM and molars

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D/D :

1) Segmental hemifacial hyperplasia – not associated with coarse vertically orieted trabeculae

2)Monostatic fibrous dysplasia-not associated with missing teeth

3) Regional odontodysplasia -the teeth appear more radiolucent than normal, so described as "ghost teeth"

Lingual salivary depression

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•Asymptomatic•Incidently finding

-lingual mandibular bone depression,developmental salivary gland defect, stafne’s defect,stafne’s bone cyst,static bone activity,latent bone cyst

-A group of concavities in lingual surface of mandible where depression is lined with an intact outer complex

Common location-within submandibular gland fossa-often close to inferior border of mandibular

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Sharpely defined radiolucencies beneath the mandibular canal in region of submandibular gland

fossa

The defect can erode the

inferior border of mandible

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Anterior variant within

sublingual gland fossa

Unusual variant with superior

position above ID canal

When defect is related to sublingual gland and appear above the

canal,D/D could be odontogenic lesion

CT scan

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-well defined defect

-Defect extending from mesial surface of the

mandible

-radiolucent tissue within the defectAxial bone window

Axial soft tissue window

3D reformatted CT image

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Differential Diagnosis :

•Appearance + location of radiographic image of the dfect are characteristic and easily identified

•Epicenter of Odontogenic lesion is located above inf.alveolar canal

•When defect is related to sublingual gland and appear above the canal,D/D could be odontogenic lesion

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Management

• recognition of lesion should preclude any treatment @ surgical exploration @ need for advancing image e.g CT

•Defect may increase with time

•Destruction of well defined cortex of defect may indicate neoplasm

Focal osteoporatic bone marrow• Marrow space• A radiologic term to indicate presence of radiolucent

defects within the cancellous portion of jaw• Histologic exam-> normal area of hematopoitec or

fatty marrow• Etiology-unknown but is belief to be due to a) bone marrow hyperplasia b) persistent embryologic marrow remnant c) site of abnormal healing after extraction , trauma or

local inflammation

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• Asymptomatic• Incidental radiograph finding• More common in middle aged-women• It is consider as variation of normal anatomy

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Clinical features:

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1)Internal aspect is Seen as a

radiolucency

2)Radiolucent due to few

internal trabeculae

present

3)Periphery vary from

well defined to ill defined

Radiograph features

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Lesion located in furcation area of

mandibulan 1st molar 4)Yet,PDL and lamina

dura are intact

• Could have same appearance-Simple bone cyst –no bone reaction at

periphery of it-Early inflammatory lesion with not yet

stimulated a visible osteoblastic process• If occur in furcation region @ apex of tooth

suspect inflammatory lesion

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DIFFERENTIAL DIAGNOSIS

• No treatment required• If in doubt , prescribe longitudinal study with

films at 3-months interval -the bone marrow should not increase in size

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Management

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