Dentinogenesis Imperfecta

Developmental disturbances affecting structure of teeth- Dentinogenesis Imperfecta

1. disturbances affecting Dentin

2. Dentinogenesis Imperfecta Dentin dysplasia Regional Odontodysplasia

3. Inherited disorder of dentin formation Autosomal dominant condition affects deciduous and permanent teeth Genetic alteration : DSPP - chromosome 4; encoding dentin phosphoprotein & dentin sialoprotein Dentinogenesis Imperfecta

4. SHIELDS CLASSIFICATION TYPE –I TYPE –II TYPE-III WITKOP CLASSIFICATION DENTINOGENESIS IMPERFECTA HEREDITARY OPALASCENT DENTIN BRANDYWINE ISOLATE REVISED CLASSIFICATION DENTINOGENESIS IMPERFECTA 1 DENTINOGENESIS IMPERFECTA 2 CLASSIFICATIONS:

5. SYNONYMS OPALESCENT DENTIN DI WITHOUT OSTEOGENESIS IMPERFECTA OPALASCENT TEETH WITHOUT OSTEOGENESIS IMPERFECTA SHIELDS TYPE II CAPDEPONT TEETH DENTINOGENESIS IMPERFCTA 1 REVISED CLASSIFICATION

6. Teeth are blue gray or amber brown and opalescent Affected teeth have bulbous crowns- Tulip shape Enamel may split readily from dentin when subjected to occlusal stress. Severe attrition of teeth C/F:

7. Synonyms SHIELDS TYPE III BRANDYWINE TYPE DENTINOGENESIS IMPERFECTA C/F : This disorder was found in the Brandywine triracial isolate in Maryland. Affects both dentitions characterized by too little dentin formation. Dentinogenesis imperfecta 2

8. Type I - bulb shaped or bell shaped crowns with constricted cervical areas Roots  thin and spiked obliteration of coronal and radicular pulp chamber depending on age Type II  large pulp chambers with thin shell of dentin and enamel “ SHELL TEETH” R/F:

9. H/P: ENAMEL  normal DENTINAL TUBULES -reduced number TUBULES  distorted, irregular in shape, widely spaced, larger in size. Areas of uncalcified matrix. Pulpal spaces either smaller than normal or completely obliterated. Ground section of tooth

10. “ Rootless Teeth ” hereditary disease rare disturbance of dentin formation Enamel- normal abnormal pulpal morphology Classification: Type I (Radicular Type) Type II (Coronal Type) Dentin dysplasia

11. Type I (Radicular Type) both dentitions are of normal colour premature tooth loss may occur because of short roots or periapical inflammatory lesions. Radiographically: roots are extremely short pulps almost /completely obliterated periapical radiolucencies: • granulomas • cysts • chronic abscesses

12. H/P: Pulp is obliterated by calcified tubular dentin, osteodentin and fused denticles . Normal dentin formation is blocked so that new dentin forms around obstacles. Producing the characteristic lava flowing around boulders appearance and cascades of dentin .

13. Type II (Coronal Type) color of primary dentition is opalescent. permanent dentition is normal. Radiographically: Deciduous roots are extremely short pulps almost completely obliterated Permanent abnormally large pulp chambers in coronal portion of tooth ( thistle tube appearance )

14. H/P: Amorphous and atubular dentin in the radicular portion. Permanent teeth also show multiple pulp stones/ denticles .

15. Odontogenic Dysplasia/ Odontogenesis Imperfecta/ Ghost Teeth one or more teeth in a localized area are affected maxillary teeth are involved more frequently than mandibular. Eg, incisors, cuspids etiology is unknown Regional Odontodysplasia

16. teeth affected may exhibit a delay or total failure in eruption shape is altered, irregular in appearance

17. H/P: Enamel thickness varies. Marked reduction in the amount of dentin. Widening of the pre-dentin layer. Large areas of interglobular dentin and irregular tubular pattern. Pulp tissue contains free or attached pulp stones. dental follicular tissue may be enlarged & exhibits focal enamel-like calcifications called enameloid conglomerates .

18. Radiographically: reduction in radiodensity teeth assume a “ghost” appearance both enamel & dentin appear very thin pulp chamber is exceedingly large

19. Treatment: poor cosmetic appearance of teeth Extraction And prosthetic rehabilitation

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