1. DEVELOPMENTAL DISTURBANCES OF THE FACE AND JAWS (PART TWO)
By Rubiah Mohd Nor

2. Topics to be discussed:
-Cleidocranial dysplasia
-Hemifacial hyperplasia
-Segmental odontomaxillary dysplasia
-Lingual salivary gland depression
-Focal osteoporotic bone marrow

3. Cleidocranial dysplasia
Autosomal dominant syndrome affecting bones and teeth
Affect both sexes equally
Can be inherited
or as a result of sporadic mutation-Runx2 gene
Clinical features:
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

4. 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 ˚ doesn’t adequately stimulate eruption of 2 ˚

5. -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
Maxillary micrognathia
-underdeveloped maxilla and paranasal sinus
Posteroanterior skull film

6. Wormian (sutural) bones in the occipital region
Absence of clavicle
Open fontanel
Chest radiograph
Wormian (sutural) bones in the occipital region
Lateral radiograph

7. 3D CT reconstruction with oblique orientation
Parietal bossing
Frontal bossing
Open metopic suture

8. Multiple unerupted supernumerary teeth
Mostly anterior max. n PM region
Lack of normal coronoid process
Prolonged retention of 1˚ dentition

9. Multiple of unerupted teeth
Axial CT view

10. -excessive mobility of movement -Examination of skull
Diagnosis
done by :-
-Family history
-excessive mobility of movement
-Examination of skull
-Radiographic finding

11. Differential Diagnosis
other disease associated with supernumerary and multiple unerupted teeth
2)Pycnodysotosis
1)Gardner’s syndrome
Short statue
Bone osteoma
Presence of clavicle
Abnormally dense brittle bone
Multiple intestinal polyps

12. 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 reason-CT scan

13. Hemifacial dysplasia Hemifacial hypertrophy, hemi hyperplasia
A condition that half of face including max. with other part of body to grow to unusual proportion
Cause:-unknown,may associate with genetic disease (Beckwith-Weidemann syndrome)

14. -Usually begin at birth
clinical feature
-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

15. If not detected during birth, it may become apparent during growth
ear lobe crease
-dysmorphic face
left hemi facial hypertrophy

16. Radiographic feature :
Rapid enlargement on right side of maxilla only
-accelerated dental development for this 5-year-old patient

17. Enlargement of bones include mand.maxilla,zygoma,
frontal and temporal bone
Enlargement of maxillary canine,1st PM
3D CT scan
CT axial image

18. Differential Diagnosis
2)Arteriovernous aneurysm
1)Hemifacial hypoplasia of the opposite side
3) Hemangioma
5)Severe condylar hyperplasia that may involve half of mandible
4) Congenital lymphadema

19. 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

20. Management
-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

21. 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

22. -Always unilateral enlargement of alveolar process, gingiva and teeth
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
--fullness of the right upper lip due to enlargement of the alveolar process.
delayed eruption of teeth on the affected side
hypertrichosis
facial enlargement
Intra-oral mirror image

23. -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

24. -Coarse trabecular pattern of right maxillary alveolar process
-delayed eruption of maxillary right 1st PM and molars

25. 2)Monostatic fibrous dysplasia -not associated with missing teeth
D/D :
1) Segmental hemifacial hyperplasia – not associated with coarse vertically orieted trabeculae
2)Monostatic fibrous dysplasia
-not associated with missing teeth
Monostatic fibrous dysplasia-Fibrous dysplasia: poorly defined radiopaque lesion of the right maxilla.
3) Regional odontodysplasia
-the teeth appear more radiolucent than normal, so described as "ghost teeth"

26. Lingual salivary depression
-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
Asymptomatic
Incidently finding
Common location
-within submandibular gland fossa
-often close to inferior border of mandibular

27. The defect can erode the inferior border of mandible
Sharpely defined radiolucencies beneath the mandibular canal in region of submandibular gland fossa
The defect can erode the inferior border of mandible

28. Unusual variant with superior position above ID canal
Anterior variant within sublingual gland fossa
When defect is related to sublingual gland and appear above the canal,D/D could be odontogenic lesion

29. CT scan -well defined defect -radiolucent tissue within the defect
Axial soft tissue window
Axial bone window
-Defect extending from mesial surface of the mandible
3D reformatted CT image

30. 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

31. Management
recognition of lesion should preclude any surgical need for advancing image e.g CT
Defect may increase with time
Destruction of well defined cortex of defect  may indicate neoplasm

32. 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

33. Incidental radiograph finding More common in middle aged-women
Clinical features:
Asymptomatic
Incidental radiograph finding
More common in middle aged-women
It is consider as variation of normal anatomy

34. 2)Radiolucent due to few internal trabeculae present
Radiograph features
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

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

36. 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 apex of tooth suspect inflammatory lesion

37. -the bone marrow should not increase in size
Management
No treatment required
If in doubt , prescribe longitudinal study with films at 3-months interval
-the bone marrow should not increase in size

38. Thank you