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1. Topics to be discussed: -Cleidocranial dysplasia -Hemifacial hyperplasia -Segmental odontomaxillary dysplasia -Lingual salivary gland depression -Focal.

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Presentation on theme: "1. Topics to be discussed: -Cleidocranial dysplasia -Hemifacial hyperplasia -Segmental odontomaxillary dysplasia -Lingual salivary gland depression -Focal."— Presentation transcript:

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

3 Cleidocranial dysplasia 3 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:

4 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 ˚ doesnt adequately stimulate eruption of 2 ˚

5 Lack of development of parietal bone 5 -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

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

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

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

9 9 Multiple of unerupted teeth Axial CT view

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

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

12 -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 12 Management

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) 13

14 14 -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

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

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

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

18 18 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

19 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 20 - 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

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 21

22 22 --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 hypertrichosis facial enlargement Intra-oral mirror image

23 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 24 -Coarse trabecular pattern of right maxillary alveolar process -delayed eruption of maxillary right 1 st PM and molars

25 25 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"

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

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

28 28 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

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

30 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 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 32

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

34 34 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

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

36 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 36 DIFFERENTIAL DIAGNOSIS

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

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