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DEVELOPMENTAL ORO-FACIAL
DISTURBANCES PART I
Dr. Ali Tahir
B.D.S, R.D.S,
M.Phil Oral Pathology
ORAL PATHOLOGY

 It is the speciality of dentistry and pathology that deals
  with the nature, identification and management of




                                                           Dr. Ali Tahir
  diseases affecting the oral & maxillofacial regions
 It is a science that investigates the causes, processes &
  effects of these diseases
 The practice of oral pathology includes research,
  diagnosis of disease using clinical, radiographic,
  microscopic      biochemical      or   other     necessary
  examinations & investigations
DISTURBANCES IN SIZE
              Microdontia
 When one or more teeth are smaller than
 normal




                                                            Dr. Ali Tahir
 Generalized
  True generalized: When all teeth are uniformly smaller
   than normal
   Cause: Pituitary Dwarfism, Down’s syndrome
  Relative generalized: When mandible or maxilla are
   somewhat larger (Macrognathia) but teeth are of normal
   size
 Localized
  When one tooth is involved
  Maxillary lateral incisors are the most common
Dr. Ali Tahir
MICRODONTIA
DISTURBANCES IN SIZE
                    Macrodontia
  When one or more teeth are larger than normal




                                                              Dr. Ali Tahir
Generalized
 True Generalized: When all teeth are uniformly larger
 Relative Generalized: When maxilla/mandible is smaller
  in size (Micrognathia) but teeth are of normal size
Regional
 It is localized e.g; Hemifacial hypertrophy (unilateral),
  segmental odontomaxillary dysplasia
 Rhizomegaly :
      When only roots are larger than normal
Dr. Ali Tahir
MACRODONTIA
DISTURBANCES IN NUMBER
                      Anodontia
 Congenital absence of all teeth




                                                      Dr. Ali Tahir
 Associated with Hereditary Ectodermal Dysplasia
                     Hypodontia
 Congenital absence of one or more teeth
 Third molars, maxillary lateral incisors are most
  commonly absent teeth consecutively
Dr. Ali Tahir
DISTURBANCES IN NUMBER
Supernumerary Teeth
Teeth in excess of normal number




                                                                        Dr. Ali Tahir
 More common in maxilla (90%)

 Examples

  Maxilla
    Mesiodens
    Paramolars
    Lateral incisors
   Mandible
    Premolars
    paramolars
     Multiple supernumerary teeth are seen in Cliedocranial dysplasia
     and gardner sydrome
SUPERNUMERARY TEETH




                      Dr. Ali Tahir
DISTURBANCES IN ERUPTION
Premature Eruption
 Natal Teeth:




                                                              Dr. Ali Tahir
       Erupted decidous teeth present at time of birth
   Neonatal:
       Deciduous teeth that erupt in first 30 days of life

    Premature eruption of entire permanent dentition should
      suspect the possibility of hyperthyroidism
Dr. Ali Tahir
NATAL TEETH
DISTURBANCES IN ERUPTION
Delayed Eruption
 Eruption later than the normal age of eruption




                                                   Dr. Ali Tahir
 Usually idiopathic

 Or associated with systemic conditions such as
  rickets, cliedocranial dysplasia, cretinism
 Gingival fibromatosis
DISTURBANCES IN ERUPTION
Impacted teeth
Teeth with eruption that is impeded by a




                                              Dr. Ali Tahir
  physical barrier
 Causes:
   Dental crowding
   Supernumerary teeth
   Odontogenic cysts
   Odontogenic tumors (odontomas)

 Most common are mandibular and maxillary
 third molars followed by maxillary cuspids
IMPACTED TEETH
   Classified according to their orientation as
     Mesioangular




                                                   Dr. Ali Tahir
     Distoangular
     Horizontal
     Vertical

   According to their stage of eruption
     Completely impacted (within bone)
     Partially impacted (partly in soft tissue)
IMPACTED TEETH


Partially impacted teeth that communicate with the oral




                                                          Dr. Ali Tahir
  cavity are more prone to pericoronitis
 Complications
   Root resorption of adjacent normal tooth
   Infection & pain
   Dentigerous cyst
   External resorption of impacted tooth
IMPACTED TEETH




                 Dr. Ali Tahir
DISTURBANCES IN ERUPTION
Eruption Sequestrum
  A small spicule of calcified tissue that is extruded




                                                         Dr. Ali Tahir
  through the alveolar mucosa that overlies an
  erupting molar
DISTURBANCES IN SHAPE
Dilaceration
  A sharp bend or




                             Dr. Ali Tahir
  angulation involving the
  root of the tooth
Causes:
 Trauma during tooth
  formation
 Continued root
  formation during a
  curved or tortuous path
  of eruption
DISTURBANCES IN SHAPE
Taurodontism
  A molar with an




                             Dr. Ali Tahir
  elongated crown and
  apically placed
  furcation of roots
  resulting in an enlarged
  rectangular pulp
  chamber.
  Occurs because of late
  invagination of
  Hertwig’s Epithelial
  Root Sheath
DISTURBANCES IN SHAPE
Dens Invaginatus
 Developmental




                           Dr. Ali Tahir
 anomaly characterized
 by a deep enamel lined
 pit that extends for
 varying depths into the
 underlying       dentin
 displacing the pulp
 chamber
DENS IN DENT AND SUPERNUMERARY
CUSP
Supernumerary
 cusps




                                 Dr. Ali Tahir
 Teeth           containing
 additional cusp
 Example:       Cusp      of
 caribili, Talon cusp
DISTURBANCES IN SHAPE
Dens Evaginatus
 Characterized by




                        Dr. Ali Tahir
 cusp like
 supernumerary
 enamel protrusion
 on occlusal or
 lingual surface of
 crown
Gemination
  Single rooted tooth with




                             Dr. Ali Tahir
  unusual wide, partly
  divided crown or two
  separate crowns.
  Cause: Because of
  partial division of a
  single tooth gem
 Teeth count is normal
DISTURBANCES IN SHAPE
Fusion
 Abnormally shaped




                          Dr. Ali Tahir
 tooth with wide crown
 or a normal crown with
 additional root(s)
 Cause: Results from
 the union of two tooth
 germs
DISTURBANCES IN SHAPE
Concrescence
 Union of the roots of




                          Dr. Ali Tahir
 two or more normal
 teeth by confluence of
 their cementum.
 Cause: Trauma, Inter-
 septal bone loss
DISTURBANCES IN SHAPE
Hypercementosis
 One or more teeth with excessive deposition of




                                                  Dr. Ali Tahir
 cementum of roots
 Causes: Increase/Decreased occlusal forces,
 Paget’s disease, hyperpituitarism, chronic
 inflammation
Cervical Enamel
 Projections




                         Dr. Ali Tahir
 Apical extension of
 coronal enamel beyond
 the smooth cervical
 margin
 Hemispheric structures
  that may consist




                            Dr. Ali Tahir
  entirely of enamel or
  may contain underlying
  dentin & pulp
 Mostly present in roots
  of maxillary or
  mandibular molars
DISTURBANCES IN STRUCTURE OF ENAMEL
   Acquired
    Environmental factors




                                               Dr. Ali Tahir
     Bacterial (syphilis), viral infections
     Inflammation
     Nutritional deficiencies
     Chemical injuries
     Trauma

   Genetic
       Amelogenesis Imperfecta
ACQUIRED DISTURBANCES
Focal enamel hypoplasia
 Localized enamel hypoplasia involving one or two




                                                          Dr. Ali Tahir
  teeth
       Example: Turner tooth, results from localized
        inflammation or trauma during tooth development
   Enamel has pitting areas or deformed with
    yellowish or brownish discoloration
ACQUIRED DISTURBANCES
Generalized Enamel Hypoplasia
 Systemic or Environmental factors inhibit
  functioning ameloblasts.




                                                            Dr. Ali Tahir
 Enamel has horizontal lines of small pits or
  grooves

 Example:
     Hutchinson’s incisors and mulberry molars due to
      congenital syphilis
     Neonatal line
     Flourosis
   Can also be seen in hypocalcemia (Vit. D
    deficiency), measles, chicken pox, scarlet fever, Vit
    A & C deficiency
GENERALIZED ENAMEL HYPOPLASIA




                                Dr. Ali Tahir
ACQUIRED DISTURBANCES
Flourosis (Flouride mottling)
 Minimal Flourosis:




                                                       Dr. Ali Tahir
  Smooth enamel surface with white flecks
 Mild Flourosis:
  Smooth enamel surface with white opaque areas
 Moderate to severe:
  Pitting and brownish discoloration
 Severe:
  Enamel is softer and weaker than normal, resulting
  in excessive wear
Teeth are largely resistant to caries
Dr. Ali Tahir
HEREDITARY DISTURBANCES
Amelogenesis Imperfecta
  A heterogeneous group of genetic disorders




                                                     Dr. Ali Tahir
  exhibiting faulty enamel formation (affects both
  primary & permanent dentition)
Normal enamel formation:
1. Enamel matrix formation
2. Mineralization of enamel
3. Enamel maturation (secondary mineralization)


Accordingly three types of AI is identified
TYPES
Clinical Features:
 Hypoplastic (focal or generalized)
       Decreased enamel formation by disturbance in function of
        ameloblasts




                                                                   Dr. Ali Tahir
       Enamel is thinner than normal
       Radiodensity is greater than that of dentin
   Hypocalcified
     Defect in mineralization of enamel
     Enamel is off normal thickness but softer than normal
     Can be easily removed with a blunt instrument
     Radiodensity is lesser than that of dentin
   Haypomaturation
       Focal or generalized areas of immature enamel
        crystallites
       Enamel of normal thickness, but less harder and is
        radiolucent e.g. snow capped teeth
WITKOP/SAUK CLASSIFICATION
   Hypoplastic
       Pitted, autosomal dominant
       Local, autosomal dominant




                                               Dr. Ali Tahir
       Smooth, autosomal dominant
       Rough, autosomal dominant
       Rough, autosomal recessive
       Smooth, X-linked
   Hypocalcified
     Autosomal dominant
     Autosomal recessive
   Hypomaturation
     Autosomal dominant (with taurodontism)
     X-linked recessive
     Pigmented, autosomal recessive
     Snow-capped teeth
Dr. Ali Tahir
DISTURBANCES IN STRUCTURE OF DENTIN
Acquired
 Turner tooth
 Regional odontodysplasia




                                                           Dr. Ali Tahir
Genetic
 Dentinogenesis Imperfecta
     Type I
     Type II
     Type III
   Dentin Dysplasia
     Type I
     Type II
   Familial hypophosphatemia (Vit. D resistant rickets)
DENTINOGENESIS IMPERFECTA
A hereditary (autosomal dominant) defect
  consisting of opalescent teeth composed of
  irregularly formed & undermineralized dentin
  that obliterates the pulp chambers & canals




                                                          Dr. Ali Tahir
Types:
   Type 1
       Associated with osteogenesis imperfecta.
       Characterized by bluish tint to sclera
   Type 2
       Hereditary opalescent dentin
       Most common type
   Type 3
       Also called brandywine type
       Clinically the same as type 1 & II
       Multiple pulpal exposures of decidous dentition
DENTINOGENESIS IMPERFECTA
Clinical Features
 Both dentitions are affected
 Oplaescent teeth with bluish grey to brownish




                                                    Dr. Ali Tahir
  or yellowish discoloration
 Abnormally soft dentin, enamel easily chipped
  off
 Despite the exposure of dentin, teeth are not
  prone to caries
Histopathology
 The mantle dentin is normal whereas remaining
  dentin is severely dysplastic
 Amorphous matrix with globular & inter-globular
  areas of mineralization
 Irregularly widely spaced disoriented dentinal
  tubules
Dr. Ali Tahir
RADIOGRAPHICALLY
   Type I & II
     Bulb shaped crowns




                                                          Dr. Ali Tahir
     Constricted cemento-enamel junction
     Thin roots
     Varying degrees of obliteration of pulp chamber &
      canals
   Type III
       Same features or may show extremely large pulp
        chambers surrounded by thin shell of dentin
DENTIN DYSPLASIA
A hereditary defect in dentin formation where root
  dentin is abnormal & gnarled, roots are




                                                     Dr. Ali Tahir
  shortened & tapered

 Also called Rootless teeth
 Autosomal dominant

 Two types
     Type I (radicular)
     Type II (coronal)
DENTIN DYSPLASIA
Types:
1) Radicular Dentin Dysplaisa:




                                                                  Dr. Ali Tahir
   Brownish or bluish translucency in cervical region
   More common than type II
   All teeth in both dentitions are affected
   Teeth often show increased mobility & exfoliate prematurely

Histopathology:
   Enamel and mantle dentin is normal
   Remaining coronal and root dentin consists of nodular
    masses composed of tubular dentin and osteo-dentin
   Slit-like pulp remnants may be seen between nodular masses
   Gives an appearance of ‘lava flowing around boulders’
Dr. Ali Tahir
RADIOGRAPHICAL FEATURES
 Short blunt roots, may
  be absent entirely




                           Dr. Ali Tahir
 Mandibular molars
  have W-shaped roots
 Dentition may show
  obliteration of pulp
  chambers & canals
 Crescent shaped
  remnants of pulp may
  be seen
Dr. Ali Tahir
DENTIN DYSPLASIA TYPE II (CORONAL)
 Both dentitions affected
 Deciduous teeth with bluish-grey, brownish or
  yellowish discoloration




                                                                    Dr. Ali Tahir
 Same translucent, opalescent appearance as in DI
 Permanent teeth have normal clinical appearance
Histopathology:
 Deciduous teeth
       Normal zone of mantle dentin that changes abruptly into
        dense amorphous dentin with irregular arrangement of
        tubules.
   Permanent teeth
     Globular and inter-globular areas of dentin in pulpal third
      of dentin with atubular root dentin
     Has pulp stones in pulp chamber
     Narrow pulp canals
RADIOGRAPHIC FEATURES
   Deciduous teeth show
    obliteration of pulp
    chambers & canals as




                               Dr. Ali Tahir
    seen in DD type I & DI
   Roots are normal
   Pulp chambers of
    permanent teeth are
    enlarged
   Thistle-tube or flame
    shaped pulp chamber
   Pulpal calcifications in
    coronal pulp chamber
REGIONAL ODONTODYSPLASIA (GHOST
TEETH)

Defective formation of enamel and dentin with
 abnormal pulp and follicle calcifications with




                                                  Dr. Ali Tahir
 surrounding soft tissue hyperplaisa along-
 with accumulations of spherical
 calcifications and odontogenic rests
CLINICAL FEATURES
 More common in maxilla
 Affects several adjacent teeth in the same quadrant




                                                        Dr. Ali Tahir
 Mostly in permanent dentition

 Deformed teeth with soft, leathery surface

 Discolored, yellowish brown

Histopathology
 Dysplastic, globular and interglobular dentin

 Widened predentin layer

 Enlarged pulp chamber with pulp stones
RADIOGRAPHIC FEATURES
 Ghost teeth
 Decreased




                        Dr. Ali Tahir
  radiodensity
 Enamel & dentin are
  very thin
 Large pulp chambers

 Pulp stones
Dr. Ali Tahir
DISTURBANCES IN STRUCTURE OF
CEMENTUM
Hypophosphatasia
 Disorder of bone mineralization caused by




                                                    Dr. Ali Tahir
  deficiency in alkaline phosphatase in serum and
  tissues
 Autosomal recessive/dominant
 Delayed formation and eruption of dentition
 Premature loss of primary teeth
 Spontaneous loss of permanent teeth
Radiographically
 Enlarged pulp chambers & pulp canals
Histopathology
 Absence or marked reduction of cementum
HYPOPHOSPHATASIA




                   Dr. Ali Tahir

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Developmental oro facial disturbances part 1

  • 1. DEVELOPMENTAL ORO-FACIAL DISTURBANCES PART I Dr. Ali Tahir B.D.S, R.D.S, M.Phil Oral Pathology
  • 2. ORAL PATHOLOGY  It is the speciality of dentistry and pathology that deals with the nature, identification and management of Dr. Ali Tahir diseases affecting the oral & maxillofacial regions  It is a science that investigates the causes, processes & effects of these diseases  The practice of oral pathology includes research, diagnosis of disease using clinical, radiographic, microscopic biochemical or other necessary examinations & investigations
  • 3. DISTURBANCES IN SIZE Microdontia When one or more teeth are smaller than normal Dr. Ali Tahir Generalized  True generalized: When all teeth are uniformly smaller than normal Cause: Pituitary Dwarfism, Down’s syndrome  Relative generalized: When mandible or maxilla are somewhat larger (Macrognathia) but teeth are of normal size Localized  When one tooth is involved  Maxillary lateral incisors are the most common
  • 5. DISTURBANCES IN SIZE Macrodontia When one or more teeth are larger than normal Dr. Ali Tahir Generalized  True Generalized: When all teeth are uniformly larger  Relative Generalized: When maxilla/mandible is smaller in size (Micrognathia) but teeth are of normal size Regional  It is localized e.g; Hemifacial hypertrophy (unilateral), segmental odontomaxillary dysplasia  Rhizomegaly :  When only roots are larger than normal
  • 7. DISTURBANCES IN NUMBER Anodontia  Congenital absence of all teeth Dr. Ali Tahir  Associated with Hereditary Ectodermal Dysplasia Hypodontia  Congenital absence of one or more teeth  Third molars, maxillary lateral incisors are most commonly absent teeth consecutively
  • 9. DISTURBANCES IN NUMBER Supernumerary Teeth Teeth in excess of normal number Dr. Ali Tahir  More common in maxilla (90%)  Examples Maxilla  Mesiodens  Paramolars  Lateral incisors Mandible  Premolars  paramolars Multiple supernumerary teeth are seen in Cliedocranial dysplasia and gardner sydrome
  • 10. SUPERNUMERARY TEETH Dr. Ali Tahir
  • 11. DISTURBANCES IN ERUPTION Premature Eruption  Natal Teeth: Dr. Ali Tahir  Erupted decidous teeth present at time of birth  Neonatal:  Deciduous teeth that erupt in first 30 days of life Premature eruption of entire permanent dentition should suspect the possibility of hyperthyroidism
  • 13. DISTURBANCES IN ERUPTION Delayed Eruption  Eruption later than the normal age of eruption Dr. Ali Tahir  Usually idiopathic  Or associated with systemic conditions such as rickets, cliedocranial dysplasia, cretinism  Gingival fibromatosis
  • 14. DISTURBANCES IN ERUPTION Impacted teeth Teeth with eruption that is impeded by a Dr. Ali Tahir physical barrier  Causes:  Dental crowding  Supernumerary teeth  Odontogenic cysts  Odontogenic tumors (odontomas)  Most common are mandibular and maxillary third molars followed by maxillary cuspids
  • 15. IMPACTED TEETH  Classified according to their orientation as  Mesioangular Dr. Ali Tahir  Distoangular  Horizontal  Vertical  According to their stage of eruption  Completely impacted (within bone)  Partially impacted (partly in soft tissue)
  • 16. IMPACTED TEETH Partially impacted teeth that communicate with the oral Dr. Ali Tahir cavity are more prone to pericoronitis  Complications  Root resorption of adjacent normal tooth  Infection & pain  Dentigerous cyst  External resorption of impacted tooth
  • 17. IMPACTED TEETH Dr. Ali Tahir
  • 18. DISTURBANCES IN ERUPTION Eruption Sequestrum A small spicule of calcified tissue that is extruded Dr. Ali Tahir through the alveolar mucosa that overlies an erupting molar
  • 19. DISTURBANCES IN SHAPE Dilaceration A sharp bend or Dr. Ali Tahir angulation involving the root of the tooth Causes:  Trauma during tooth formation  Continued root formation during a curved or tortuous path of eruption
  • 20. DISTURBANCES IN SHAPE Taurodontism A molar with an Dr. Ali Tahir elongated crown and apically placed furcation of roots resulting in an enlarged rectangular pulp chamber. Occurs because of late invagination of Hertwig’s Epithelial Root Sheath
  • 21. DISTURBANCES IN SHAPE Dens Invaginatus Developmental Dr. Ali Tahir anomaly characterized by a deep enamel lined pit that extends for varying depths into the underlying dentin displacing the pulp chamber
  • 22. DENS IN DENT AND SUPERNUMERARY CUSP Supernumerary cusps Dr. Ali Tahir Teeth containing additional cusp Example: Cusp of caribili, Talon cusp
  • 23. DISTURBANCES IN SHAPE Dens Evaginatus Characterized by Dr. Ali Tahir cusp like supernumerary enamel protrusion on occlusal or lingual surface of crown
  • 24. Gemination Single rooted tooth with Dr. Ali Tahir unusual wide, partly divided crown or two separate crowns. Cause: Because of partial division of a single tooth gem  Teeth count is normal
  • 25. DISTURBANCES IN SHAPE Fusion Abnormally shaped Dr. Ali Tahir tooth with wide crown or a normal crown with additional root(s) Cause: Results from the union of two tooth germs
  • 26. DISTURBANCES IN SHAPE Concrescence Union of the roots of Dr. Ali Tahir two or more normal teeth by confluence of their cementum. Cause: Trauma, Inter- septal bone loss
  • 27. DISTURBANCES IN SHAPE Hypercementosis One or more teeth with excessive deposition of Dr. Ali Tahir cementum of roots Causes: Increase/Decreased occlusal forces, Paget’s disease, hyperpituitarism, chronic inflammation
  • 28. Cervical Enamel Projections Dr. Ali Tahir Apical extension of coronal enamel beyond the smooth cervical margin
  • 29.  Hemispheric structures that may consist Dr. Ali Tahir entirely of enamel or may contain underlying dentin & pulp  Mostly present in roots of maxillary or mandibular molars
  • 30. DISTURBANCES IN STRUCTURE OF ENAMEL  Acquired Environmental factors Dr. Ali Tahir  Bacterial (syphilis), viral infections  Inflammation  Nutritional deficiencies  Chemical injuries  Trauma  Genetic  Amelogenesis Imperfecta
  • 31. ACQUIRED DISTURBANCES Focal enamel hypoplasia  Localized enamel hypoplasia involving one or two Dr. Ali Tahir teeth  Example: Turner tooth, results from localized inflammation or trauma during tooth development  Enamel has pitting areas or deformed with yellowish or brownish discoloration
  • 32. ACQUIRED DISTURBANCES Generalized Enamel Hypoplasia  Systemic or Environmental factors inhibit functioning ameloblasts. Dr. Ali Tahir  Enamel has horizontal lines of small pits or grooves  Example:  Hutchinson’s incisors and mulberry molars due to congenital syphilis  Neonatal line  Flourosis  Can also be seen in hypocalcemia (Vit. D deficiency), measles, chicken pox, scarlet fever, Vit A & C deficiency
  • 34. ACQUIRED DISTURBANCES Flourosis (Flouride mottling)  Minimal Flourosis: Dr. Ali Tahir Smooth enamel surface with white flecks  Mild Flourosis: Smooth enamel surface with white opaque areas  Moderate to severe: Pitting and brownish discoloration  Severe: Enamel is softer and weaker than normal, resulting in excessive wear Teeth are largely resistant to caries
  • 36. HEREDITARY DISTURBANCES Amelogenesis Imperfecta A heterogeneous group of genetic disorders Dr. Ali Tahir exhibiting faulty enamel formation (affects both primary & permanent dentition) Normal enamel formation: 1. Enamel matrix formation 2. Mineralization of enamel 3. Enamel maturation (secondary mineralization) Accordingly three types of AI is identified
  • 37. TYPES Clinical Features:  Hypoplastic (focal or generalized)  Decreased enamel formation by disturbance in function of ameloblasts Dr. Ali Tahir  Enamel is thinner than normal  Radiodensity is greater than that of dentin  Hypocalcified  Defect in mineralization of enamel  Enamel is off normal thickness but softer than normal  Can be easily removed with a blunt instrument  Radiodensity is lesser than that of dentin  Haypomaturation  Focal or generalized areas of immature enamel crystallites  Enamel of normal thickness, but less harder and is radiolucent e.g. snow capped teeth
  • 38. WITKOP/SAUK CLASSIFICATION  Hypoplastic  Pitted, autosomal dominant  Local, autosomal dominant Dr. Ali Tahir  Smooth, autosomal dominant  Rough, autosomal dominant  Rough, autosomal recessive  Smooth, X-linked  Hypocalcified  Autosomal dominant  Autosomal recessive  Hypomaturation  Autosomal dominant (with taurodontism)  X-linked recessive  Pigmented, autosomal recessive  Snow-capped teeth
  • 40. DISTURBANCES IN STRUCTURE OF DENTIN Acquired  Turner tooth  Regional odontodysplasia Dr. Ali Tahir Genetic  Dentinogenesis Imperfecta  Type I  Type II  Type III  Dentin Dysplasia  Type I  Type II  Familial hypophosphatemia (Vit. D resistant rickets)
  • 41. DENTINOGENESIS IMPERFECTA A hereditary (autosomal dominant) defect consisting of opalescent teeth composed of irregularly formed & undermineralized dentin that obliterates the pulp chambers & canals Dr. Ali Tahir Types:  Type 1  Associated with osteogenesis imperfecta.  Characterized by bluish tint to sclera  Type 2  Hereditary opalescent dentin  Most common type  Type 3  Also called brandywine type  Clinically the same as type 1 & II  Multiple pulpal exposures of decidous dentition
  • 42. DENTINOGENESIS IMPERFECTA Clinical Features  Both dentitions are affected  Oplaescent teeth with bluish grey to brownish Dr. Ali Tahir or yellowish discoloration  Abnormally soft dentin, enamel easily chipped off  Despite the exposure of dentin, teeth are not prone to caries Histopathology  The mantle dentin is normal whereas remaining dentin is severely dysplastic  Amorphous matrix with globular & inter-globular areas of mineralization  Irregularly widely spaced disoriented dentinal tubules
  • 44. RADIOGRAPHICALLY  Type I & II  Bulb shaped crowns Dr. Ali Tahir  Constricted cemento-enamel junction  Thin roots  Varying degrees of obliteration of pulp chamber & canals  Type III  Same features or may show extremely large pulp chambers surrounded by thin shell of dentin
  • 45. DENTIN DYSPLASIA A hereditary defect in dentin formation where root dentin is abnormal & gnarled, roots are Dr. Ali Tahir shortened & tapered  Also called Rootless teeth  Autosomal dominant  Two types  Type I (radicular)  Type II (coronal)
  • 46. DENTIN DYSPLASIA Types: 1) Radicular Dentin Dysplaisa: Dr. Ali Tahir  Brownish or bluish translucency in cervical region  More common than type II  All teeth in both dentitions are affected  Teeth often show increased mobility & exfoliate prematurely Histopathology:  Enamel and mantle dentin is normal  Remaining coronal and root dentin consists of nodular masses composed of tubular dentin and osteo-dentin  Slit-like pulp remnants may be seen between nodular masses  Gives an appearance of ‘lava flowing around boulders’
  • 48. RADIOGRAPHICAL FEATURES  Short blunt roots, may be absent entirely Dr. Ali Tahir  Mandibular molars have W-shaped roots  Dentition may show obliteration of pulp chambers & canals  Crescent shaped remnants of pulp may be seen
  • 50. DENTIN DYSPLASIA TYPE II (CORONAL)  Both dentitions affected  Deciduous teeth with bluish-grey, brownish or yellowish discoloration Dr. Ali Tahir  Same translucent, opalescent appearance as in DI  Permanent teeth have normal clinical appearance Histopathology:  Deciduous teeth  Normal zone of mantle dentin that changes abruptly into dense amorphous dentin with irregular arrangement of tubules.  Permanent teeth  Globular and inter-globular areas of dentin in pulpal third of dentin with atubular root dentin  Has pulp stones in pulp chamber  Narrow pulp canals
  • 51. RADIOGRAPHIC FEATURES  Deciduous teeth show obliteration of pulp chambers & canals as Dr. Ali Tahir seen in DD type I & DI  Roots are normal  Pulp chambers of permanent teeth are enlarged  Thistle-tube or flame shaped pulp chamber  Pulpal calcifications in coronal pulp chamber
  • 52. REGIONAL ODONTODYSPLASIA (GHOST TEETH) Defective formation of enamel and dentin with abnormal pulp and follicle calcifications with Dr. Ali Tahir surrounding soft tissue hyperplaisa along- with accumulations of spherical calcifications and odontogenic rests
  • 53. CLINICAL FEATURES  More common in maxilla  Affects several adjacent teeth in the same quadrant Dr. Ali Tahir  Mostly in permanent dentition  Deformed teeth with soft, leathery surface  Discolored, yellowish brown Histopathology  Dysplastic, globular and interglobular dentin  Widened predentin layer  Enlarged pulp chamber with pulp stones
  • 54. RADIOGRAPHIC FEATURES  Ghost teeth  Decreased Dr. Ali Tahir radiodensity  Enamel & dentin are very thin  Large pulp chambers  Pulp stones
  • 56. DISTURBANCES IN STRUCTURE OF CEMENTUM Hypophosphatasia  Disorder of bone mineralization caused by Dr. Ali Tahir deficiency in alkaline phosphatase in serum and tissues  Autosomal recessive/dominant  Delayed formation and eruption of dentition  Premature loss of primary teeth  Spontaneous loss of permanent teeth Radiographically  Enlarged pulp chambers & pulp canals Histopathology  Absence or marked reduction of cementum
  • 57. HYPOPHOSPHATASIA Dr. Ali Tahir