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BY Dr shabeel pn
INTRODUCTION
IS AN INHERITED DISORDER OF DENTIN FORMATION AUTOSOMAL DOMINANT CONDITION AFFECTS DECIDOUS AND PERMANENT TEETH
CLASSIFICATIONS:
SHIELDS CLASSIFICATION 1. TYPE I 2. TYPE II 3. TYPE-III
REVISED CLASSIFICATION
1. 2.
SHIELDS CLASSIFICATION
TYPE I:OCCURS IN PATIENTS AFFECTED WITH OSTEOGENESIS IMPERFECTA TYPE II :IS NOT ASSOCIATED WITH WITH OSTEOGENESIS IMPERFECTA TYPE III:BRANDYWINE TYPE RARE CONDITION ,SEEN IN RACIAL ISOLATE OF MARYLAND,EXHIBITS MULTIPLE PULP EXPOSURES AND PERIAPICAL LESIONS IN DECIDOUS DENTITION.
REVISED CLASSIFICATION
DENTINOGENESIS IMPERFECTA 1 DI WITHOUT OSTEOGENESIS IMPERFECTA CORRESPONDS TO TYPE II OF SHIELDS CLASSIFICATION DENTINOGENESIS IMPERFECTA 2 CORRESPONDS TO TYPE III OF SHIELDS CLASSIFICATION
THERE IS NO SUBSTITUTE IN THE PRESENT CLASSIFICATION FOR THE CATEGORY DESIGNATED AS TYPE I IN THE SHIELDS CLASSIFICATION
DENTINOGENESIS IMPERFCTA 1
SYNONYMS OPALESCENT DENTIN
DI WITHOUT OSTEOGENESIS IMPERFECTA OPALASCENT TEETH WITHOUT OSTEOGENESIS IMPERFECTA DENTINOGENESIS IMPERFECTA SHIELDS TYPE II CAPDEPONT TEETH
ETIOLOGY DGI 1
CAUSED BY MUTATION IN THE DENTIN SIALO PHOSPHO PROTEIN( DSPP) gene ENCODING DENTIN PHOSPHOPROTEIN AND DENTIN SIALOPROTIEN
CLEARLY DISTINCT FROM OI WITH OPALASCENT TEETH, & AFFECTS ONLY THE TEETH NO INCREASED FREQUENCY OF BONE FRACTURE IS SEEN
FREQUENCY: 1 IN 6000-8000
Dentinogenesis imperfecta 2
Synonyms
ETIOLOGY DGI 2
SOME RESEARCHERS SAY IT IS A SEPARATE MUTATION FROM DGI 1 SHIELD et al 1973 STATED THAT MARKEDLY ENLARGED PULP CHAMBERS AND PULP EXPOSURES OCCURS IN DECIDOUS TEETH DO NOT OCCUR IN DGI 1 WITKOP 1975 SUGGESTED BOTH ARE SAME RECENT STUDIES SUGESTS BOTH ARE RESULT OF MUTATION IN TWO TIGHTLY LINKED GENES 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
CLINICAL FEATURES
Affects males and females equally Teeth are blue gray or amber brown and opalescent Few days after eruption teeth may achieve a normal color ,following which they become translucent
Finally become gray or brown with bluish reflection from enamel Enamel may split readily from dentin when subjected to occlusal stress Severe attrition of teeth Obliterated pulp chambesr
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
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
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
IN SOME CASE THERE MAY BE HYPOMINERALISED AREA ON THE ENAMEL
Case
A child patient patient affected with dentinogenesis imperfecta .Exposed teeth have a grayish opalescent appearance.
RADIOGRAPHIC FEATURES
BULB SHAPED OR BELL SHAPED CROWNS OF TEETH WITH CONSTRICTED CERVICAL AREAS ROOTSTHIN AND SPIKED OBLITRATION OF CORONAL AND RADICULAR PULP CHAMBER DEPENDING ON AGE CEMENTUM,ALVEOLAR BONE,AND PDL APPEAR NORMAL TYPE 2LARGE PULP CHAMBERS WITH THIN SHELL OF DENTIN AND ENAMEL SHELL
TEETH
HISTOPATHOLOGY
ENAMELNORMAL MANTLE DENTIN (NARROW ZONE OF DENTIN BELOW ENAMEL)NORMAL REMAINING DENTIN SEVERLY DYSPLASTIC WITH VAST AREAS OF AMORPHOUS MATRIX WITH GLOBULAR OR INTERGLOBULAR FOCI OF MINERALISATION REDUCED NUMBER OF DENTINAL TUBULES
TUBULES DISTORED,IRREGULAR IN SHAPE,WIDELY SPACED ,LARGER IN SIZE ABSENCE OF ODONTOBLASTIC PROCESSES AND PRESENCE OF DEGENERATING CELLULAR DEBRIS INSTEAD LARGE AREA OF ATUBULAR DENTIN PULP CHAMBER AND ROOT CANAL OBLITERATED BY ABNORMAL DENTIN DEPOSITION DEJSMOOTH OR FLATTNED INSTEAD OF SCALLOPED (RESPONSIBLE FOR EARLY CHIPPING OF ENAMEL)
TREATMENT
1.
2. 3.
AIMED AT PREVENTING LOSS OF ENAMEL AND DENTIN THROUGH ATTRITION Mild moderate cases( no enamel # or rapid wear of teeth) Routine restorative techniques eg:amalgam,composite Bonding of veneers for estheticsmask opalescence of anterior teeth Bleeching to an extend lightens the color
Severe cases: (significant enamel # and rapid wear) 1. Full coverage crown restoration 2. Primary teethstainless steel in posteriors stainless steel with open face composite for anterior teeth
PARTIAL APPLIANCES EXERTING PRESSURE ON TEETH SHOULD BE USED WITH CAUTION AS ROOTS CAN GET FRACTURED EASILY FILLINGS ARE NOT PERMANENT DUE TO SOFT DENTIN NEWER COMPOSITES WITH DENTIN BONDING AGENTS USED IN AREAS SUBJECTED TO OCCLUSAL WEAR
OVERLAY DENTURES THAT ARE PLACED ON TEETH THAT ARE COVERED WITH FLOURIDE RELEASING GIC USED
IN EXTENSIVE ATTRITION VERTICAL DIMENTION REBULIT BY PLACING NONPRECIOUS METAL CASTINGS WITH ADHESIVE LUTING AGENT ON TEETH WHICH HAVE NOT BEEN SUBJECT TO ANY PREPARATION OR EXCESSIVE OCCLUSAL LOAD Periapical abscess occur due to pulp exposure or pulp obliterationthorough periodic radiographsapical surgery
Before treatment
Before treatment
After treatment
Bibliography
Shafers text book of oral pathology (5th edition) Oral and maxillofacial pathology-Neville (2nd edition) Essentials of oral pathology-Swapan Kumar Purkait (2nd edition)
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