Dentinogenesis imperfecta dr shabeel pn BY Dr shabeel pn
INTRODUCTION <ul><li>IS  AN INHERITED DISORDER OF DENTIN FORMATION </li></ul><ul><li>AUTOSOMAL DOMINANT CONDITION </li></u...
CLASSIFICATIONS: <ul><li> SHIELDS CLASSIFICATION </li></ul><ul><li>TYPE –I </li></ul><ul><li>TYPE –II </li></ul><ul><li>T...
SHIELDS CLASSIFICATION <ul><li>TYPE  I:OCCURS IN PATIENTS AFFECTED WITH OSTEOGENESIS IMPERFECTA </li></ul><ul><li>TYPE II ...
REVISED CLASSIFICATION <ul><li>DENTINOGENESIS IMPERFECTA 1 </li></ul><ul><li>DI WITHOUT OSTEOGENESIS IMPERFECTA </li></ul>...
DENTINOGENESIS IMPERFCTA 1 <ul><li>SYNONYMS   </li></ul><ul><li>OPALESCENT DENTIN </li></ul><ul><li>DI WITHOUT OSTEOGENESI...
ETIOLOGY –DGI 1  <ul><li>CAUSED BY MUTATION IN THE DENTIN SIALO PHOSPHO PROTEIN( DSPP) gene ENCODING DENTIN PHOSPHOPROTEIN...
<ul><li>CLEARLY DISTINCT FROM OI WITH OPALASCENT TEETH, & AFFECTS ONLY THE TEETH </li></ul><ul><li>NO INCREASED FREQUENCY ...
Dentinogenesis imperfecta 2 <ul><li>Synonyms   </li></ul><ul><li>SHIELDS TYPE III </li></ul><ul><li>BRANDYWINE TYPE DENTIN...
ETIOLOGY –DGI 2 <ul><li>SOME RESEARCHERS SAY IT IS A SEPARATE MUTATION FROM DGI 1 </li></ul><ul><li>SHIELD et al 1973 STAT...
CLINICAL FEATURES <ul><li>Affects males and females equally </li></ul><ul><li>Teeth are blue gray or amber brown and opale...
<ul><li>Finally become gray or brown with bluish reflection from enamel </li></ul><ul><li>Enamel may split readily from de...
<ul><li>TEETH ARE NOT SENSITIVE EVEN IF MOST OF THE SURFACE ENAMEL IS LOST AS DENTINAL TUBULES ARE HAPHAZARDLY ARRANGED AN...
Case   <ul><li>A  child patient patient affected with dentinogenesis imperfecta .Exposed teeth have a grayish opalescent a...
RADIOGRAPHIC FEATURES   <ul><li>BULB SHAPED OR BELL SHAPED CROWNS OF TEETH WITH CONSTRICTED CERVICAL AREAS  </li></ul><ul>...
 
HISTOPATHOLOGY   <ul><li>ENAMEL  NORMAL </li></ul><ul><li>MANTLE DENTIN (NARROW ZONE OF DENTIN BELOW ENAMEL)  NORMAL </l...
<ul><li>TUBULES   DISTORED,IRREGULAR IN SHAPE,WIDELY SPACED ,LARGER IN  SIZE  </li></ul><ul><li>ABSENCE OF ODONTOBLASTIC ...
CHEMICAL AND PHYSICAL FEATURES <ul><li>INCREASED WATER CONTENT (60 % THAN NORMAL) </li></ul><ul><li>DECREASED MINERAL CONT...
TREATMENT   <ul><li>AIMED AT PREVENTING LOSS OF ENAMEL AND DENTIN THROUGH ATTRITION  </li></ul><ul><li>Mild –moderate case...
<ul><li>Severe cases:  (significant enamel # and rapid wear) </li></ul><ul><li>Full coverage crown restoration </li></ul><...
<ul><li>PARTIAL APPLIANCES EXERTING PRESSURE ON TEETH SHOULD BE USED WITH CAUTION AS ROOTS CAN GET FRACTURED EASILY </li><...
<ul><li>IN EXTENSIVE ATTRITION VERTICAL DIMENTION REBULIT BY PLACING NON-PRECIOUS METAL CASTINGS WITH ADHESIVE LUTING AGEN...
Before treatment
Before treatment
After treatment
Bibliography <ul><li>Shafer’s text book of oral pathology (5 th  edition) </li></ul><ul><li>Oral and maxillofacial patholo...
<ul><li>THANK YOU </li></ul>
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Dentinogenesis Imperfecta

  1. 1. Dentinogenesis imperfecta dr shabeel pn BY Dr shabeel pn
  2. 2. INTRODUCTION <ul><li>IS AN INHERITED DISORDER OF DENTIN FORMATION </li></ul><ul><li>AUTOSOMAL DOMINANT CONDITION </li></ul><ul><li>AFFECTS DECIDOUS AND PERMANENT TEETH </li></ul>
  3. 3. CLASSIFICATIONS: <ul><li> SHIELDS CLASSIFICATION </li></ul><ul><li>TYPE –I </li></ul><ul><li>TYPE –II </li></ul><ul><li>TYPE-III </li></ul><ul><li> REVISED CLASSIFICATION </li></ul><ul><li>DENTINOGENESIS IMPERFECTA 1 </li></ul><ul><li>DENTINOGENESIS IMPERFECTA 2 </li></ul><ul><li> WITKOP CLASSIFICATION </li></ul><ul><li>DENTINOGENESIS IMPERFECTA </li></ul><ul><li>HEREDITARY OPALASCENT DENTIN </li></ul><ul><li>BRANDYWINE ISOLATE </li></ul>
  4. 4. SHIELDS CLASSIFICATION <ul><li>TYPE I:OCCURS IN PATIENTS AFFECTED WITH OSTEOGENESIS IMPERFECTA </li></ul><ul><li>TYPE II :IS NOT ASSOCIATED WITH WITH OSTEOGENESIS IMPERFECTA </li></ul><ul><li>TYPE III:’’BRANDYWINE TYPE” RARE CONDITION ,SEEN IN RACIAL ISOLATE OF MARYLAND,EXHIBITS MULTIPLE PULP EXPOSURES AND PERIAPICAL LESIONS IN DECIDOUS DENTITION. </li></ul>
  5. 5. REVISED CLASSIFICATION <ul><li>DENTINOGENESIS IMPERFECTA 1 </li></ul><ul><li>DI WITHOUT OSTEOGENESIS IMPERFECTA </li></ul><ul><li>CORRESPONDS TO TYPE II OF SHIELDS CLASSIFICATION </li></ul><ul><li>DENTINOGENESIS IMPERFECTA 2 </li></ul><ul><li>CORRESPONDS TO TYPE III OF SHIELDS CLASSIFICATION </li></ul><ul><li>THERE IS NO SUBSTITUTE IN THE PRESENT CLASSIFICATION FOR THE CATEGORY DESIGNATED AS TYPE I IN THE SHIELDS CLASSIFICATION </li></ul>
  6. 6. DENTINOGENESIS IMPERFCTA 1 <ul><li>SYNONYMS </li></ul><ul><li>OPALESCENT DENTIN </li></ul><ul><li>DI WITHOUT OSTEOGENESIS IMPERFECTA </li></ul><ul><li>OPALASCENT TEETH WITHOUT OSTEOGENESIS IMPERFECTA </li></ul><ul><li>DENTINOGENESIS IMPERFECTA </li></ul><ul><li>SHIELDS TYPE II </li></ul><ul><li>CAPDEPONT TEETH </li></ul>
  7. 7. ETIOLOGY –DGI 1 <ul><li>CAUSED BY MUTATION IN THE DENTIN SIALO PHOSPHO PROTEIN( DSPP) gene ENCODING DENTIN PHOSPHOPROTEIN AND DENTIN SIALOPROTIEN </li></ul>
  8. 8. <ul><li>CLEARLY DISTINCT FROM OI WITH OPALASCENT TEETH, & AFFECTS ONLY THE TEETH </li></ul><ul><li>NO INCREASED FREQUENCY OF BONE FRACTURE IS SEEN </li></ul><ul><li>FREQUENCY: 1 IN 6000-8000 </li></ul>
  9. 9. Dentinogenesis imperfecta 2 <ul><li>Synonyms </li></ul><ul><li>SHIELDS TYPE III </li></ul><ul><li>BRANDYWINE TYPE DENTINOGENESIS IMPERFECTA </li></ul>
  10. 10. ETIOLOGY –DGI 2 <ul><li>SOME RESEARCHERS SAY IT IS A SEPARATE MUTATION FROM DGI 1 </li></ul><ul><li>SHIELD et al 1973 STATED THAT MARKEDLY ENLARGED PULP CHAMBERS AND PULP EXPOSURES OCCURS IN DECIDOUS TEETH DO NOT OCCUR IN DGI 1 </li></ul><ul><li>WITKOP 1975 SUGGESTED BOTH ARE SAME </li></ul><ul><li>RECENT STUDIES SUGESTS BOTH ARE RESULT OF MUTATION IN TWO TIGHTLY LINKED GENES </li></ul><ul><li>MacDOUGALL et al 1999 STATED DGI 2 DIFFER FROM DGI 1 BY THE PRESENCE OF MULTIPLE PULP EXPOSURES ,NORMAL NON MINERALISED PULP CHAMBERS ,AND GENERAL APPEARANCE OF SHELL TEETH </li></ul>
  11. 11. CLINICAL FEATURES <ul><li>Affects males and females equally </li></ul><ul><li>Teeth are blue gray or amber brown and opalescent </li></ul><ul><li>Few days after eruption teeth may achieve a normal color ,following which they become translucent </li></ul>
  12. 12. <ul><li>Finally become gray or brown with bluish reflection from enamel </li></ul><ul><li>Enamel may split readily from dentin when subjected to occlusal stress </li></ul><ul><li>Severe attrition of teeth </li></ul><ul><li>Obliterated pulp chambesr </li></ul><ul><li>Sauk et al .(1976)  increase in glycosaminoglycans in EDTA soluble dentin in teeth from patients with this disorder as compared to controls and less GAG in EDTA insoluble residue </li></ul>
  13. 13. <ul><li>TEETH ARE NOT SENSITIVE EVEN IF MOST OF THE SURFACE ENAMEL IS LOST AS DENTINAL TUBULES ARE HAPHAZARDLY ARRANGED AND MOST OF THEM ARE DEVOID OF ODONTOBLASTIC PROCESSES </li></ul><ul><li>DENTIN IS SOFT AND EASILY PENETRABLE BUT NOT CARIES PRONE BECAUSE OF STRUCTURAL CHANGE IN DENTIN  LITTLE SCOPE FOR MICRORGANISM ENTRY DUE TO OBLITERATED DENTINAL TUBULES </li></ul><ul><li>IN SOME CASE THERE MAY BE HYPOMINERALISED AREA ON THE ENAMEL </li></ul>
  14. 14. Case <ul><li>A child patient patient affected with dentinogenesis imperfecta .Exposed teeth have a grayish opalescent appearance. </li></ul>
  15. 15. RADIOGRAPHIC FEATURES <ul><li>BULB SHAPED OR BELL SHAPED CROWNS OF TEETH WITH CONSTRICTED CERVICAL AREAS </li></ul><ul><li>ROOTS  THIN AND SPIKED </li></ul><ul><li>OBLITRATION OF CORONAL AND RADICULAR PULP CHAMBER DEPENDING ON AGE </li></ul><ul><li>CEMENTUM,ALVEOLAR BONE,AND PDL APPEAR NORMAL </li></ul><ul><li>TYPE 2  LARGE PULP CHAMBERS WITH THIN SHELL OF DENTIN AND ENAMEL “ SHELL TEETH” </li></ul>
  16. 17. HISTOPATHOLOGY <ul><li>ENAMEL  NORMAL </li></ul><ul><li>MANTLE DENTIN (NARROW ZONE OF DENTIN BELOW ENAMEL)  NORMAL </li></ul><ul><li>REMAINING DENTIN  SEVERLY DYSPLASTIC WITH VAST AREAS OF AMORPHOUS MATRIX WITH GLOBULAR OR INTERGLOBULAR FOCI OF MINERALISATION </li></ul><ul><li>REDUCED NUMBER OF DENTINAL TUBULES </li></ul>
  17. 18. <ul><li>TUBULES  DISTORED,IRREGULAR IN SHAPE,WIDELY SPACED ,LARGER IN SIZE </li></ul><ul><li>ABSENCE OF ODONTOBLASTIC PROCESSES AND PRESENCE OF DEGENERATING CELLULAR DEBRIS INSTEAD </li></ul><ul><li>LARGE AREA OF ATUBULAR DENTIN </li></ul><ul><li>PULP CHAMBER AND ROOT CANAL OBLITERATED BY ABNORMAL DENTIN DEPOSITION </li></ul><ul><li>DEJ  SMOOTH OR FLATTNED INSTEAD OF SCALLOPED (RESPONSIBLE FOR EARLY CHIPPING OF ENAMEL) </li></ul>
  18. 19. CHEMICAL AND PHYSICAL FEATURES <ul><li>INCREASED WATER CONTENT (60 % THAN NORMAL) </li></ul><ul><li>DECREASED MINERAL CONTENT </li></ul><ul><li>DENSITY, X-RAY ABSORPTION AND HARDNESS ARE LOW </li></ul><ul><li>MICROHARDNESS NEAR TO CEMENTUM </li></ul>
  19. 20. TREATMENT <ul><li>AIMED AT PREVENTING LOSS OF ENAMEL AND DENTIN THROUGH ATTRITION </li></ul><ul><li>Mild –moderate cases( no enamel # or rapid wear of teeth) </li></ul><ul><li>Routine restorative techniques eg:amalgam,composite </li></ul><ul><li>Bonding of veneers  for esthetics  mask opalescence of anterior teeth </li></ul><ul><li>Bleeching to an extend lightens the color </li></ul>
  20. 21. <ul><li>Severe cases: (significant enamel # and rapid wear) </li></ul><ul><li>Full coverage crown restoration </li></ul><ul><li>Primary teeth  stainless steel in posteriors </li></ul><ul><li>stainless steel with open face composite for anterior teeth </li></ul><ul><li>permanent teeth  porcelain fused metal crowns </li></ul>
  21. 22. <ul><li>PARTIAL APPLIANCES EXERTING PRESSURE ON TEETH SHOULD BE USED WITH CAUTION AS ROOTS CAN GET FRACTURED EASILY </li></ul><ul><li>FILLINGS ARE NOT PERMANENT DUE TO SOFT DENTIN </li></ul><ul><li>NEWER COMPOSITES WITH DENTIN BONDING AGENTS USED IN AREAS SUBJECTED TO OCCLUSAL WEAR </li></ul><ul><li>OVERLAY DENTURES THAT ARE PLACED ON TEETH THAT ARE COVERED WITH FLOURIDE RELEASING GIC USED </li></ul>
  22. 23. <ul><li>IN EXTENSIVE ATTRITION VERTICAL DIMENTION REBULIT BY PLACING NON-PRECIOUS METAL CASTINGS WITH ADHESIVE LUTING AGENT ON TEETH WHICH HAVE NOT BEEN SUBJECT TO ANY PREPARATION OR EXCESSIVE OCCLUSAL LOAD </li></ul><ul><li>Periapical abscess occur due to pulp exposure or pulp obliteration  thorough periodic radiographs  apical surgery </li></ul>
  23. 24. Before treatment
  24. 25. Before treatment
  25. 26. After treatment
  26. 27. Bibliography <ul><li>Shafer’s text book of oral pathology (5 th edition) </li></ul><ul><li>Oral and maxillofacial pathology-Neville (2 nd edition) </li></ul><ul><li>Essentials of oral pathology-Swapan Kumar Purkait (2 nd edition) </li></ul>
  27. 28. <ul><li>THANK YOU </li></ul>
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