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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
Autosomal dominant syndrome
affecting bones and teeth
Affect both sexes equally
Can be inherited
or as a result of sporadic mutationRunx2 gene
Clinical
CCD affectfeatures:
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
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parietal +Frontal bossing

Underdeveloped maxilla
Excessive mobility of shoulder
Appear shorter than
unaffected relative

-Prolonged retention of
1
-Delayed eruption of 2
-Often have unerupted
supernumerary teeth
-extraction of 1
doesnt adequately
stimulate eruption of 2
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-a light-bulb shape due to

brachycephaly
-delayed or failure of
fontanelles
-open skull suture

Radiographic
features

Lack of
development
of parietal
bone

Lateral skull film

Posteroanterior skull film

Maxillary micrognathia
-underdeveloped maxilla
and paranasal sinus

Absence of
clavicle

Open fontanel

Chest radiograph

Wormian
(sutural)
bones in
the
occipital
region

Lateral radiograph
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 3D CT reconstruction with oblique orientation

Parietal bossing

Frontal bossing
Open metopic suture
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Multiple unerupted
supernumerary
teeth
Mostly anterior
max. n PM region

Lack of
normal
coronoid
process

Prolonged
retention of 1
dentition

Multiple of
unerupted
teeth

Axial CT view

Diagnosis

done by :-Family history

-excessive mobility of movement
-Examination of skull
-Radiographic finding

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Differential
Diagnosis
other disease associated with supernumerary and multiple
unerupted teeth

1)Gardners syndrome

2)Pycnodysotosis
Short statue

Bone osteoma

Presence of clavicle
Abnormally dense brittle b
Multiple intestinal polyps

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Management

-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 reasonCT scan

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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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clinical feature
-Usually begin at birth
-Often occur with other
abnormalities: mental
deficiency,skin
abnormalities,compensator
y 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

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If not detected during

birth, it may become
apparent during
growth

ear lobe
crease

-dysmorphic face
left hemi facial
hypertrophy

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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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Enlargeme
nt of
maxillary
canine,1st
PM

Enlargement of bones
include
mand.maxilla,zygoma
,
frontal and temporal
bone

3D CT scan
CT axial image

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Differential
Diagnosis

2)Arteriovernous
aneurysm

1)Hemifacial hypoplasia
of the opposite side
3) Hemangioma

4) Congenital lymphadema

5)Severe condylar hyperplasia that

may involve half of mandible

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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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Manageme
nt
-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

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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.

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

delayed
eruption of
teeth on
the
affected
side

Intra-oral mirror image

hypertrichosis
facial
enlargement
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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
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-dense bone pattern of left

-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

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Lingual salivary depression

-lingual mandibular bone depression,developmental salivary
gland defect, stafnes defect,stafnes 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
Asymptomatic
Incidently finding

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
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CT scan

-well
defined
defect

Axial bone window

-radiolucent tissue
within the defect

Axial soft tissue window

-Defect extending
from mesial surface
of the mandible

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

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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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Clinical features:

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

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Radiograph
features
1)Internal
aspect is
Seen as a
radiolucen
cy

3)Periphe
ry vary
from well
defined
to ill
defined

2)Radiolucent
due to few
internal
trabeculae
present

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Lesion located in
furcation area of
mandibulan 1st
molar 4)Yet,PDL
and lamina dura
are intact
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DIFFERENTIAL
DIAGNOSIS

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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Management

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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