A rare condition affecting the primary and permanent by dr ibrahim
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Transcript of A rare condition affecting the primary and permanent by dr ibrahim
JOMR20131(1) 13-16Sekerci AE EtozM SahmanH SismanY NazlimS
DR IBRAHIM
Dentin dysplasia (DD) is a rare hereditary disturbance is inherited as an autosomal dominant trait
unknown etiology that affects approximately 1 100000
In 1972 Witkop classified it into type I and type II which affect both dentitions
Shafer WG Hine MK Levy BM Developmental disturbances of oral and paraoral structures In A text book of oral pathology 4 thed Philadelphia Elsevier Science Saunders Co 2003 p 2-85
Radicular dentin dysplasia
Characterized by-
1Both dentitions are affected
2Normal appearing crowns
3No or only rudimentary root development (rootless teeth)
4Incomplete or total obliteration of the pulp chamber
5Teeth may exhibit extreme mobility and exfoliate prematurely
coronal dentin dysplasia
Characterized by-
1partial pulpal obliteration
2Thistle-tube-or flame-shapedcoronal pulp chambers
3 Thread-like root canals
4 Usually the absence of
periapical radiolucencies
5 In this type of anomaly
teeth roots are of normal
shape and contour
The enamel and the immediately subjacent dentin appear normal
Deeper layers of dentin show an atypical tubular pattern with an amorphous atubular area and irregular organization
Normal dentinal tubule formationn appears to have been blocked so that new dentine forms around obstacles and takes on the characteristic appearances described as ldquolava flowing around bouldersrdquo
A 7-year-old girl was referred to the Department complaining mobile teeth and a swelling in the maxillary deciduous right incisor area
The patient had lost her mandibular central incisors teeth by the age of 3 and mandibularleft primary second molar by the age of 5 Her older brother had a similar condition
The patients mother became edentulous at an early age and had required full maxillary and mandibular dentures
The grandfather of the girl stated that he too had a history of delayed eruption He further stated that he had lost most of his teeth at an early age because of spontaneous exfoliation which had necessitated a full denture in the maxilla and a partial denture in the mandible by the end of his adolescence
Dental examination of the patients father revealed no evidence similar condition
The patients medical history revealed no evidence of disturbance in general health
The patient was having class III malocclusion with spacing between maxillary and mandibular anteriors
Anterior open bite and mandibular prognatie
All the teeth were normal in shape and size There was a painful fistule on the buccal region of the maxillary right primary central incisor Periapical radiolucencies were present at the permanent upper central incisors
a The intraoral examination revealed normal size morphology and color of teeth
The radiograph revealed features of dentine dysplasia type I with normal appearance of crown but no root development
The maxillary primary central incisors right lateral incisor mandibular primary right canine first and second molars mandibular left second premolar was extracted owing to extensive mobility
The ground section was - superficial dentin of the crown appeared normal and the deeper layers of dentin had an atypical tubular pattern but the pulp chamber was obliterated by an unusual type of calcified material consisting of dentin
In the present case-
The calcified pulp chambers
Rootless teeth
Periapical radiolucent areas and the nature of the periapical lesion were characteristic findings for the diagnosis of DD type I
The etiology of DD is still unknown however several theories have been proposed in the dental literature
Logan et al _suggested that the dentinal papilla is responsible for the abnormalities in root development
Sauk et al -postulated an invagination of the epithelial root sheath resulting in abnormal dentin formation
Wesley et al disagreed with this suggestion and proposed that the condition is caused by an abnormal interaction of odontoblasts with ameloblasts leading to abnormal differentiation andor function of these odontoblasts
The cause of periapical radiolucencies in DD type I is not understood
Steidler et al-suggest that they are the result of pulpal necrosis occurring either secondary to caries or spontaneously
DD is recognized as a genetic disorder and is thought possibly to be a single gene mutation
When multiple family members have a similar pattern of pathosis this supports a diagnosis of a hereditary condition
An attempt to identify a familial history in our case the patients brother mother and grandfather had similar condition Although from the patients description
Histologically-the immediately subjacent dentin and the enamel appear normal
Deeper layers of dentin with demonstrate an atypical tubular pattern with amorphous atubular globular or nodular masses of abnormal dentin are seen
The periapical radiolucent areas seen in most cases of dentinal dysplasia have been interpreted as radicular cysts however in some cases a diagnosis of periapical granuloma has been reported
The management of patients with DD is difficult
Extraction
Follow-up and routine conservative treatment is another choice of treatment plan in DD
Maintenance of periodontal health
Endodontic treatment is contraindicated in teeth with total obliteration of root canals and pulp chambers
Another approach for the treatment of teeth with DD has included periapical surgery and retrograde filling which is recommended in teeth with long roots
bullSince these patients usually have early exfoliation of the teeth and consequently maxilla-mandibular bony atrophy treatment with a combination of onlay bone grafting and a sinus lift operation to accomplish implant placement can be used successfullybullThough a malalignment of the arch is one of the most common characteristics of this disorder orthodontic correction should not be applied in a routine fashion bullIn the majority of cases despite early diagnosis and the provision of regular dental care teeth are lost because of spontaneous abscess formation bullThere is no treatment for severe cases of DD type I other than extraction of symptomatic teeth Even for special cases onlay autogenous bone grafting and sinus lift technique are well-tested methods of augmentation
bullTherapy including extraction of all teeth curettage of cystic alteration and functional rehabilitation by the insertion of a conventional complete denture has been presented previously by Neumann et al
bullMunoz-Guerra et al _ reported successful treatment of a 24-year old female after onlaybone grafting and sinus augmentation
bullThough many cases of DD type I have been described some clinical aspects and theoretic issues remain uncertain bullEarly diagnosis of the condition is important for the initiation of effective preventive treatment Follow-up studies on patients with DD type I might provide insights into treatment strategies bullFuture molecular genetic research may determine precise information about the gene locus responsible for DD type I
Dentin dysplasia
1048708 Type II
1048708 Coronal type
1048708 Root length normal in both dentitions
1048708 Primary teeth
1048708 Clinically resemble dentinogenesis imperfecta
1048708 Radiographically have similar appearance to Type I
1048708 Permanent teeth
1048708 Normal coloration
1048708 Pulp chambers enlarged with apical extension-thistletube-
shaped or flame-shaped
Dd type 11
Autosomal dominant
Type I RadicularThe teeth have normal crowns and
abnormal rootsThe teeth are generally exfoliated
prematurely
Type II CoronalPrimary teeth are translucent with an amber colorAdult teeth appear normal
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
Dentin dysplasia (DD) is a rare hereditary disturbance is inherited as an autosomal dominant trait
unknown etiology that affects approximately 1 100000
In 1972 Witkop classified it into type I and type II which affect both dentitions
Shafer WG Hine MK Levy BM Developmental disturbances of oral and paraoral structures In A text book of oral pathology 4 thed Philadelphia Elsevier Science Saunders Co 2003 p 2-85
Radicular dentin dysplasia
Characterized by-
1Both dentitions are affected
2Normal appearing crowns
3No or only rudimentary root development (rootless teeth)
4Incomplete or total obliteration of the pulp chamber
5Teeth may exhibit extreme mobility and exfoliate prematurely
coronal dentin dysplasia
Characterized by-
1partial pulpal obliteration
2Thistle-tube-or flame-shapedcoronal pulp chambers
3 Thread-like root canals
4 Usually the absence of
periapical radiolucencies
5 In this type of anomaly
teeth roots are of normal
shape and contour
The enamel and the immediately subjacent dentin appear normal
Deeper layers of dentin show an atypical tubular pattern with an amorphous atubular area and irregular organization
Normal dentinal tubule formationn appears to have been blocked so that new dentine forms around obstacles and takes on the characteristic appearances described as ldquolava flowing around bouldersrdquo
A 7-year-old girl was referred to the Department complaining mobile teeth and a swelling in the maxillary deciduous right incisor area
The patient had lost her mandibular central incisors teeth by the age of 3 and mandibularleft primary second molar by the age of 5 Her older brother had a similar condition
The patients mother became edentulous at an early age and had required full maxillary and mandibular dentures
The grandfather of the girl stated that he too had a history of delayed eruption He further stated that he had lost most of his teeth at an early age because of spontaneous exfoliation which had necessitated a full denture in the maxilla and a partial denture in the mandible by the end of his adolescence
Dental examination of the patients father revealed no evidence similar condition
The patients medical history revealed no evidence of disturbance in general health
The patient was having class III malocclusion with spacing between maxillary and mandibular anteriors
Anterior open bite and mandibular prognatie
All the teeth were normal in shape and size There was a painful fistule on the buccal region of the maxillary right primary central incisor Periapical radiolucencies were present at the permanent upper central incisors
a The intraoral examination revealed normal size morphology and color of teeth
The radiograph revealed features of dentine dysplasia type I with normal appearance of crown but no root development
The maxillary primary central incisors right lateral incisor mandibular primary right canine first and second molars mandibular left second premolar was extracted owing to extensive mobility
The ground section was - superficial dentin of the crown appeared normal and the deeper layers of dentin had an atypical tubular pattern but the pulp chamber was obliterated by an unusual type of calcified material consisting of dentin
In the present case-
The calcified pulp chambers
Rootless teeth
Periapical radiolucent areas and the nature of the periapical lesion were characteristic findings for the diagnosis of DD type I
The etiology of DD is still unknown however several theories have been proposed in the dental literature
Logan et al _suggested that the dentinal papilla is responsible for the abnormalities in root development
Sauk et al -postulated an invagination of the epithelial root sheath resulting in abnormal dentin formation
Wesley et al disagreed with this suggestion and proposed that the condition is caused by an abnormal interaction of odontoblasts with ameloblasts leading to abnormal differentiation andor function of these odontoblasts
The cause of periapical radiolucencies in DD type I is not understood
Steidler et al-suggest that they are the result of pulpal necrosis occurring either secondary to caries or spontaneously
DD is recognized as a genetic disorder and is thought possibly to be a single gene mutation
When multiple family members have a similar pattern of pathosis this supports a diagnosis of a hereditary condition
An attempt to identify a familial history in our case the patients brother mother and grandfather had similar condition Although from the patients description
Histologically-the immediately subjacent dentin and the enamel appear normal
Deeper layers of dentin with demonstrate an atypical tubular pattern with amorphous atubular globular or nodular masses of abnormal dentin are seen
The periapical radiolucent areas seen in most cases of dentinal dysplasia have been interpreted as radicular cysts however in some cases a diagnosis of periapical granuloma has been reported
The management of patients with DD is difficult
Extraction
Follow-up and routine conservative treatment is another choice of treatment plan in DD
Maintenance of periodontal health
Endodontic treatment is contraindicated in teeth with total obliteration of root canals and pulp chambers
Another approach for the treatment of teeth with DD has included periapical surgery and retrograde filling which is recommended in teeth with long roots
bullSince these patients usually have early exfoliation of the teeth and consequently maxilla-mandibular bony atrophy treatment with a combination of onlay bone grafting and a sinus lift operation to accomplish implant placement can be used successfullybullThough a malalignment of the arch is one of the most common characteristics of this disorder orthodontic correction should not be applied in a routine fashion bullIn the majority of cases despite early diagnosis and the provision of regular dental care teeth are lost because of spontaneous abscess formation bullThere is no treatment for severe cases of DD type I other than extraction of symptomatic teeth Even for special cases onlay autogenous bone grafting and sinus lift technique are well-tested methods of augmentation
bullTherapy including extraction of all teeth curettage of cystic alteration and functional rehabilitation by the insertion of a conventional complete denture has been presented previously by Neumann et al
bullMunoz-Guerra et al _ reported successful treatment of a 24-year old female after onlaybone grafting and sinus augmentation
bullThough many cases of DD type I have been described some clinical aspects and theoretic issues remain uncertain bullEarly diagnosis of the condition is important for the initiation of effective preventive treatment Follow-up studies on patients with DD type I might provide insights into treatment strategies bullFuture molecular genetic research may determine precise information about the gene locus responsible for DD type I
Dentin dysplasia
1048708 Type II
1048708 Coronal type
1048708 Root length normal in both dentitions
1048708 Primary teeth
1048708 Clinically resemble dentinogenesis imperfecta
1048708 Radiographically have similar appearance to Type I
1048708 Permanent teeth
1048708 Normal coloration
1048708 Pulp chambers enlarged with apical extension-thistletube-
shaped or flame-shaped
Dd type 11
Autosomal dominant
Type I RadicularThe teeth have normal crowns and
abnormal rootsThe teeth are generally exfoliated
prematurely
Type II CoronalPrimary teeth are translucent with an amber colorAdult teeth appear normal
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
Radicular dentin dysplasia
Characterized by-
1Both dentitions are affected
2Normal appearing crowns
3No or only rudimentary root development (rootless teeth)
4Incomplete or total obliteration of the pulp chamber
5Teeth may exhibit extreme mobility and exfoliate prematurely
coronal dentin dysplasia
Characterized by-
1partial pulpal obliteration
2Thistle-tube-or flame-shapedcoronal pulp chambers
3 Thread-like root canals
4 Usually the absence of
periapical radiolucencies
5 In this type of anomaly
teeth roots are of normal
shape and contour
The enamel and the immediately subjacent dentin appear normal
Deeper layers of dentin show an atypical tubular pattern with an amorphous atubular area and irregular organization
Normal dentinal tubule formationn appears to have been blocked so that new dentine forms around obstacles and takes on the characteristic appearances described as ldquolava flowing around bouldersrdquo
A 7-year-old girl was referred to the Department complaining mobile teeth and a swelling in the maxillary deciduous right incisor area
The patient had lost her mandibular central incisors teeth by the age of 3 and mandibularleft primary second molar by the age of 5 Her older brother had a similar condition
The patients mother became edentulous at an early age and had required full maxillary and mandibular dentures
The grandfather of the girl stated that he too had a history of delayed eruption He further stated that he had lost most of his teeth at an early age because of spontaneous exfoliation which had necessitated a full denture in the maxilla and a partial denture in the mandible by the end of his adolescence
Dental examination of the patients father revealed no evidence similar condition
The patients medical history revealed no evidence of disturbance in general health
The patient was having class III malocclusion with spacing between maxillary and mandibular anteriors
Anterior open bite and mandibular prognatie
All the teeth were normal in shape and size There was a painful fistule on the buccal region of the maxillary right primary central incisor Periapical radiolucencies were present at the permanent upper central incisors
a The intraoral examination revealed normal size morphology and color of teeth
The radiograph revealed features of dentine dysplasia type I with normal appearance of crown but no root development
The maxillary primary central incisors right lateral incisor mandibular primary right canine first and second molars mandibular left second premolar was extracted owing to extensive mobility
The ground section was - superficial dentin of the crown appeared normal and the deeper layers of dentin had an atypical tubular pattern but the pulp chamber was obliterated by an unusual type of calcified material consisting of dentin
In the present case-
The calcified pulp chambers
Rootless teeth
Periapical radiolucent areas and the nature of the periapical lesion were characteristic findings for the diagnosis of DD type I
The etiology of DD is still unknown however several theories have been proposed in the dental literature
Logan et al _suggested that the dentinal papilla is responsible for the abnormalities in root development
Sauk et al -postulated an invagination of the epithelial root sheath resulting in abnormal dentin formation
Wesley et al disagreed with this suggestion and proposed that the condition is caused by an abnormal interaction of odontoblasts with ameloblasts leading to abnormal differentiation andor function of these odontoblasts
The cause of periapical radiolucencies in DD type I is not understood
Steidler et al-suggest that they are the result of pulpal necrosis occurring either secondary to caries or spontaneously
DD is recognized as a genetic disorder and is thought possibly to be a single gene mutation
When multiple family members have a similar pattern of pathosis this supports a diagnosis of a hereditary condition
An attempt to identify a familial history in our case the patients brother mother and grandfather had similar condition Although from the patients description
Histologically-the immediately subjacent dentin and the enamel appear normal
Deeper layers of dentin with demonstrate an atypical tubular pattern with amorphous atubular globular or nodular masses of abnormal dentin are seen
The periapical radiolucent areas seen in most cases of dentinal dysplasia have been interpreted as radicular cysts however in some cases a diagnosis of periapical granuloma has been reported
The management of patients with DD is difficult
Extraction
Follow-up and routine conservative treatment is another choice of treatment plan in DD
Maintenance of periodontal health
Endodontic treatment is contraindicated in teeth with total obliteration of root canals and pulp chambers
Another approach for the treatment of teeth with DD has included periapical surgery and retrograde filling which is recommended in teeth with long roots
bullSince these patients usually have early exfoliation of the teeth and consequently maxilla-mandibular bony atrophy treatment with a combination of onlay bone grafting and a sinus lift operation to accomplish implant placement can be used successfullybullThough a malalignment of the arch is one of the most common characteristics of this disorder orthodontic correction should not be applied in a routine fashion bullIn the majority of cases despite early diagnosis and the provision of regular dental care teeth are lost because of spontaneous abscess formation bullThere is no treatment for severe cases of DD type I other than extraction of symptomatic teeth Even for special cases onlay autogenous bone grafting and sinus lift technique are well-tested methods of augmentation
bullTherapy including extraction of all teeth curettage of cystic alteration and functional rehabilitation by the insertion of a conventional complete denture has been presented previously by Neumann et al
bullMunoz-Guerra et al _ reported successful treatment of a 24-year old female after onlaybone grafting and sinus augmentation
bullThough many cases of DD type I have been described some clinical aspects and theoretic issues remain uncertain bullEarly diagnosis of the condition is important for the initiation of effective preventive treatment Follow-up studies on patients with DD type I might provide insights into treatment strategies bullFuture molecular genetic research may determine precise information about the gene locus responsible for DD type I
Dentin dysplasia
1048708 Type II
1048708 Coronal type
1048708 Root length normal in both dentitions
1048708 Primary teeth
1048708 Clinically resemble dentinogenesis imperfecta
1048708 Radiographically have similar appearance to Type I
1048708 Permanent teeth
1048708 Normal coloration
1048708 Pulp chambers enlarged with apical extension-thistletube-
shaped or flame-shaped
Dd type 11
Autosomal dominant
Type I RadicularThe teeth have normal crowns and
abnormal rootsThe teeth are generally exfoliated
prematurely
Type II CoronalPrimary teeth are translucent with an amber colorAdult teeth appear normal
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
coronal dentin dysplasia
Characterized by-
1partial pulpal obliteration
2Thistle-tube-or flame-shapedcoronal pulp chambers
3 Thread-like root canals
4 Usually the absence of
periapical radiolucencies
5 In this type of anomaly
teeth roots are of normal
shape and contour
The enamel and the immediately subjacent dentin appear normal
Deeper layers of dentin show an atypical tubular pattern with an amorphous atubular area and irregular organization
Normal dentinal tubule formationn appears to have been blocked so that new dentine forms around obstacles and takes on the characteristic appearances described as ldquolava flowing around bouldersrdquo
A 7-year-old girl was referred to the Department complaining mobile teeth and a swelling in the maxillary deciduous right incisor area
The patient had lost her mandibular central incisors teeth by the age of 3 and mandibularleft primary second molar by the age of 5 Her older brother had a similar condition
The patients mother became edentulous at an early age and had required full maxillary and mandibular dentures
The grandfather of the girl stated that he too had a history of delayed eruption He further stated that he had lost most of his teeth at an early age because of spontaneous exfoliation which had necessitated a full denture in the maxilla and a partial denture in the mandible by the end of his adolescence
Dental examination of the patients father revealed no evidence similar condition
The patients medical history revealed no evidence of disturbance in general health
The patient was having class III malocclusion with spacing between maxillary and mandibular anteriors
Anterior open bite and mandibular prognatie
All the teeth were normal in shape and size There was a painful fistule on the buccal region of the maxillary right primary central incisor Periapical radiolucencies were present at the permanent upper central incisors
a The intraoral examination revealed normal size morphology and color of teeth
The radiograph revealed features of dentine dysplasia type I with normal appearance of crown but no root development
The maxillary primary central incisors right lateral incisor mandibular primary right canine first and second molars mandibular left second premolar was extracted owing to extensive mobility
The ground section was - superficial dentin of the crown appeared normal and the deeper layers of dentin had an atypical tubular pattern but the pulp chamber was obliterated by an unusual type of calcified material consisting of dentin
In the present case-
The calcified pulp chambers
Rootless teeth
Periapical radiolucent areas and the nature of the periapical lesion were characteristic findings for the diagnosis of DD type I
The etiology of DD is still unknown however several theories have been proposed in the dental literature
Logan et al _suggested that the dentinal papilla is responsible for the abnormalities in root development
Sauk et al -postulated an invagination of the epithelial root sheath resulting in abnormal dentin formation
Wesley et al disagreed with this suggestion and proposed that the condition is caused by an abnormal interaction of odontoblasts with ameloblasts leading to abnormal differentiation andor function of these odontoblasts
The cause of periapical radiolucencies in DD type I is not understood
Steidler et al-suggest that they are the result of pulpal necrosis occurring either secondary to caries or spontaneously
DD is recognized as a genetic disorder and is thought possibly to be a single gene mutation
When multiple family members have a similar pattern of pathosis this supports a diagnosis of a hereditary condition
An attempt to identify a familial history in our case the patients brother mother and grandfather had similar condition Although from the patients description
Histologically-the immediately subjacent dentin and the enamel appear normal
Deeper layers of dentin with demonstrate an atypical tubular pattern with amorphous atubular globular or nodular masses of abnormal dentin are seen
The periapical radiolucent areas seen in most cases of dentinal dysplasia have been interpreted as radicular cysts however in some cases a diagnosis of periapical granuloma has been reported
The management of patients with DD is difficult
Extraction
Follow-up and routine conservative treatment is another choice of treatment plan in DD
Maintenance of periodontal health
Endodontic treatment is contraindicated in teeth with total obliteration of root canals and pulp chambers
Another approach for the treatment of teeth with DD has included periapical surgery and retrograde filling which is recommended in teeth with long roots
bullSince these patients usually have early exfoliation of the teeth and consequently maxilla-mandibular bony atrophy treatment with a combination of onlay bone grafting and a sinus lift operation to accomplish implant placement can be used successfullybullThough a malalignment of the arch is one of the most common characteristics of this disorder orthodontic correction should not be applied in a routine fashion bullIn the majority of cases despite early diagnosis and the provision of regular dental care teeth are lost because of spontaneous abscess formation bullThere is no treatment for severe cases of DD type I other than extraction of symptomatic teeth Even for special cases onlay autogenous bone grafting and sinus lift technique are well-tested methods of augmentation
bullTherapy including extraction of all teeth curettage of cystic alteration and functional rehabilitation by the insertion of a conventional complete denture has been presented previously by Neumann et al
bullMunoz-Guerra et al _ reported successful treatment of a 24-year old female after onlaybone grafting and sinus augmentation
bullThough many cases of DD type I have been described some clinical aspects and theoretic issues remain uncertain bullEarly diagnosis of the condition is important for the initiation of effective preventive treatment Follow-up studies on patients with DD type I might provide insights into treatment strategies bullFuture molecular genetic research may determine precise information about the gene locus responsible for DD type I
Dentin dysplasia
1048708 Type II
1048708 Coronal type
1048708 Root length normal in both dentitions
1048708 Primary teeth
1048708 Clinically resemble dentinogenesis imperfecta
1048708 Radiographically have similar appearance to Type I
1048708 Permanent teeth
1048708 Normal coloration
1048708 Pulp chambers enlarged with apical extension-thistletube-
shaped or flame-shaped
Dd type 11
Autosomal dominant
Type I RadicularThe teeth have normal crowns and
abnormal rootsThe teeth are generally exfoliated
prematurely
Type II CoronalPrimary teeth are translucent with an amber colorAdult teeth appear normal
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
The enamel and the immediately subjacent dentin appear normal
Deeper layers of dentin show an atypical tubular pattern with an amorphous atubular area and irregular organization
Normal dentinal tubule formationn appears to have been blocked so that new dentine forms around obstacles and takes on the characteristic appearances described as ldquolava flowing around bouldersrdquo
A 7-year-old girl was referred to the Department complaining mobile teeth and a swelling in the maxillary deciduous right incisor area
The patient had lost her mandibular central incisors teeth by the age of 3 and mandibularleft primary second molar by the age of 5 Her older brother had a similar condition
The patients mother became edentulous at an early age and had required full maxillary and mandibular dentures
The grandfather of the girl stated that he too had a history of delayed eruption He further stated that he had lost most of his teeth at an early age because of spontaneous exfoliation which had necessitated a full denture in the maxilla and a partial denture in the mandible by the end of his adolescence
Dental examination of the patients father revealed no evidence similar condition
The patients medical history revealed no evidence of disturbance in general health
The patient was having class III malocclusion with spacing between maxillary and mandibular anteriors
Anterior open bite and mandibular prognatie
All the teeth were normal in shape and size There was a painful fistule on the buccal region of the maxillary right primary central incisor Periapical radiolucencies were present at the permanent upper central incisors
a The intraoral examination revealed normal size morphology and color of teeth
The radiograph revealed features of dentine dysplasia type I with normal appearance of crown but no root development
The maxillary primary central incisors right lateral incisor mandibular primary right canine first and second molars mandibular left second premolar was extracted owing to extensive mobility
The ground section was - superficial dentin of the crown appeared normal and the deeper layers of dentin had an atypical tubular pattern but the pulp chamber was obliterated by an unusual type of calcified material consisting of dentin
In the present case-
The calcified pulp chambers
Rootless teeth
Periapical radiolucent areas and the nature of the periapical lesion were characteristic findings for the diagnosis of DD type I
The etiology of DD is still unknown however several theories have been proposed in the dental literature
Logan et al _suggested that the dentinal papilla is responsible for the abnormalities in root development
Sauk et al -postulated an invagination of the epithelial root sheath resulting in abnormal dentin formation
Wesley et al disagreed with this suggestion and proposed that the condition is caused by an abnormal interaction of odontoblasts with ameloblasts leading to abnormal differentiation andor function of these odontoblasts
The cause of periapical radiolucencies in DD type I is not understood
Steidler et al-suggest that they are the result of pulpal necrosis occurring either secondary to caries or spontaneously
DD is recognized as a genetic disorder and is thought possibly to be a single gene mutation
When multiple family members have a similar pattern of pathosis this supports a diagnosis of a hereditary condition
An attempt to identify a familial history in our case the patients brother mother and grandfather had similar condition Although from the patients description
Histologically-the immediately subjacent dentin and the enamel appear normal
Deeper layers of dentin with demonstrate an atypical tubular pattern with amorphous atubular globular or nodular masses of abnormal dentin are seen
The periapical radiolucent areas seen in most cases of dentinal dysplasia have been interpreted as radicular cysts however in some cases a diagnosis of periapical granuloma has been reported
The management of patients with DD is difficult
Extraction
Follow-up and routine conservative treatment is another choice of treatment plan in DD
Maintenance of periodontal health
Endodontic treatment is contraindicated in teeth with total obliteration of root canals and pulp chambers
Another approach for the treatment of teeth with DD has included periapical surgery and retrograde filling which is recommended in teeth with long roots
bullSince these patients usually have early exfoliation of the teeth and consequently maxilla-mandibular bony atrophy treatment with a combination of onlay bone grafting and a sinus lift operation to accomplish implant placement can be used successfullybullThough a malalignment of the arch is one of the most common characteristics of this disorder orthodontic correction should not be applied in a routine fashion bullIn the majority of cases despite early diagnosis and the provision of regular dental care teeth are lost because of spontaneous abscess formation bullThere is no treatment for severe cases of DD type I other than extraction of symptomatic teeth Even for special cases onlay autogenous bone grafting and sinus lift technique are well-tested methods of augmentation
bullTherapy including extraction of all teeth curettage of cystic alteration and functional rehabilitation by the insertion of a conventional complete denture has been presented previously by Neumann et al
bullMunoz-Guerra et al _ reported successful treatment of a 24-year old female after onlaybone grafting and sinus augmentation
bullThough many cases of DD type I have been described some clinical aspects and theoretic issues remain uncertain bullEarly diagnosis of the condition is important for the initiation of effective preventive treatment Follow-up studies on patients with DD type I might provide insights into treatment strategies bullFuture molecular genetic research may determine precise information about the gene locus responsible for DD type I
Dentin dysplasia
1048708 Type II
1048708 Coronal type
1048708 Root length normal in both dentitions
1048708 Primary teeth
1048708 Clinically resemble dentinogenesis imperfecta
1048708 Radiographically have similar appearance to Type I
1048708 Permanent teeth
1048708 Normal coloration
1048708 Pulp chambers enlarged with apical extension-thistletube-
shaped or flame-shaped
Dd type 11
Autosomal dominant
Type I RadicularThe teeth have normal crowns and
abnormal rootsThe teeth are generally exfoliated
prematurely
Type II CoronalPrimary teeth are translucent with an amber colorAdult teeth appear normal
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
A 7-year-old girl was referred to the Department complaining mobile teeth and a swelling in the maxillary deciduous right incisor area
The patient had lost her mandibular central incisors teeth by the age of 3 and mandibularleft primary second molar by the age of 5 Her older brother had a similar condition
The patients mother became edentulous at an early age and had required full maxillary and mandibular dentures
The grandfather of the girl stated that he too had a history of delayed eruption He further stated that he had lost most of his teeth at an early age because of spontaneous exfoliation which had necessitated a full denture in the maxilla and a partial denture in the mandible by the end of his adolescence
Dental examination of the patients father revealed no evidence similar condition
The patients medical history revealed no evidence of disturbance in general health
The patient was having class III malocclusion with spacing between maxillary and mandibular anteriors
Anterior open bite and mandibular prognatie
All the teeth were normal in shape and size There was a painful fistule on the buccal region of the maxillary right primary central incisor Periapical radiolucencies were present at the permanent upper central incisors
a The intraoral examination revealed normal size morphology and color of teeth
The radiograph revealed features of dentine dysplasia type I with normal appearance of crown but no root development
The maxillary primary central incisors right lateral incisor mandibular primary right canine first and second molars mandibular left second premolar was extracted owing to extensive mobility
The ground section was - superficial dentin of the crown appeared normal and the deeper layers of dentin had an atypical tubular pattern but the pulp chamber was obliterated by an unusual type of calcified material consisting of dentin
In the present case-
The calcified pulp chambers
Rootless teeth
Periapical radiolucent areas and the nature of the periapical lesion were characteristic findings for the diagnosis of DD type I
The etiology of DD is still unknown however several theories have been proposed in the dental literature
Logan et al _suggested that the dentinal papilla is responsible for the abnormalities in root development
Sauk et al -postulated an invagination of the epithelial root sheath resulting in abnormal dentin formation
Wesley et al disagreed with this suggestion and proposed that the condition is caused by an abnormal interaction of odontoblasts with ameloblasts leading to abnormal differentiation andor function of these odontoblasts
The cause of periapical radiolucencies in DD type I is not understood
Steidler et al-suggest that they are the result of pulpal necrosis occurring either secondary to caries or spontaneously
DD is recognized as a genetic disorder and is thought possibly to be a single gene mutation
When multiple family members have a similar pattern of pathosis this supports a diagnosis of a hereditary condition
An attempt to identify a familial history in our case the patients brother mother and grandfather had similar condition Although from the patients description
Histologically-the immediately subjacent dentin and the enamel appear normal
Deeper layers of dentin with demonstrate an atypical tubular pattern with amorphous atubular globular or nodular masses of abnormal dentin are seen
The periapical radiolucent areas seen in most cases of dentinal dysplasia have been interpreted as radicular cysts however in some cases a diagnosis of periapical granuloma has been reported
The management of patients with DD is difficult
Extraction
Follow-up and routine conservative treatment is another choice of treatment plan in DD
Maintenance of periodontal health
Endodontic treatment is contraindicated in teeth with total obliteration of root canals and pulp chambers
Another approach for the treatment of teeth with DD has included periapical surgery and retrograde filling which is recommended in teeth with long roots
bullSince these patients usually have early exfoliation of the teeth and consequently maxilla-mandibular bony atrophy treatment with a combination of onlay bone grafting and a sinus lift operation to accomplish implant placement can be used successfullybullThough a malalignment of the arch is one of the most common characteristics of this disorder orthodontic correction should not be applied in a routine fashion bullIn the majority of cases despite early diagnosis and the provision of regular dental care teeth are lost because of spontaneous abscess formation bullThere is no treatment for severe cases of DD type I other than extraction of symptomatic teeth Even for special cases onlay autogenous bone grafting and sinus lift technique are well-tested methods of augmentation
bullTherapy including extraction of all teeth curettage of cystic alteration and functional rehabilitation by the insertion of a conventional complete denture has been presented previously by Neumann et al
bullMunoz-Guerra et al _ reported successful treatment of a 24-year old female after onlaybone grafting and sinus augmentation
bullThough many cases of DD type I have been described some clinical aspects and theoretic issues remain uncertain bullEarly diagnosis of the condition is important for the initiation of effective preventive treatment Follow-up studies on patients with DD type I might provide insights into treatment strategies bullFuture molecular genetic research may determine precise information about the gene locus responsible for DD type I
Dentin dysplasia
1048708 Type II
1048708 Coronal type
1048708 Root length normal in both dentitions
1048708 Primary teeth
1048708 Clinically resemble dentinogenesis imperfecta
1048708 Radiographically have similar appearance to Type I
1048708 Permanent teeth
1048708 Normal coloration
1048708 Pulp chambers enlarged with apical extension-thistletube-
shaped or flame-shaped
Dd type 11
Autosomal dominant
Type I RadicularThe teeth have normal crowns and
abnormal rootsThe teeth are generally exfoliated
prematurely
Type II CoronalPrimary teeth are translucent with an amber colorAdult teeth appear normal
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
The grandfather of the girl stated that he too had a history of delayed eruption He further stated that he had lost most of his teeth at an early age because of spontaneous exfoliation which had necessitated a full denture in the maxilla and a partial denture in the mandible by the end of his adolescence
Dental examination of the patients father revealed no evidence similar condition
The patients medical history revealed no evidence of disturbance in general health
The patient was having class III malocclusion with spacing between maxillary and mandibular anteriors
Anterior open bite and mandibular prognatie
All the teeth were normal in shape and size There was a painful fistule on the buccal region of the maxillary right primary central incisor Periapical radiolucencies were present at the permanent upper central incisors
a The intraoral examination revealed normal size morphology and color of teeth
The radiograph revealed features of dentine dysplasia type I with normal appearance of crown but no root development
The maxillary primary central incisors right lateral incisor mandibular primary right canine first and second molars mandibular left second premolar was extracted owing to extensive mobility
The ground section was - superficial dentin of the crown appeared normal and the deeper layers of dentin had an atypical tubular pattern but the pulp chamber was obliterated by an unusual type of calcified material consisting of dentin
In the present case-
The calcified pulp chambers
Rootless teeth
Periapical radiolucent areas and the nature of the periapical lesion were characteristic findings for the diagnosis of DD type I
The etiology of DD is still unknown however several theories have been proposed in the dental literature
Logan et al _suggested that the dentinal papilla is responsible for the abnormalities in root development
Sauk et al -postulated an invagination of the epithelial root sheath resulting in abnormal dentin formation
Wesley et al disagreed with this suggestion and proposed that the condition is caused by an abnormal interaction of odontoblasts with ameloblasts leading to abnormal differentiation andor function of these odontoblasts
The cause of periapical radiolucencies in DD type I is not understood
Steidler et al-suggest that they are the result of pulpal necrosis occurring either secondary to caries or spontaneously
DD is recognized as a genetic disorder and is thought possibly to be a single gene mutation
When multiple family members have a similar pattern of pathosis this supports a diagnosis of a hereditary condition
An attempt to identify a familial history in our case the patients brother mother and grandfather had similar condition Although from the patients description
Histologically-the immediately subjacent dentin and the enamel appear normal
Deeper layers of dentin with demonstrate an atypical tubular pattern with amorphous atubular globular or nodular masses of abnormal dentin are seen
The periapical radiolucent areas seen in most cases of dentinal dysplasia have been interpreted as radicular cysts however in some cases a diagnosis of periapical granuloma has been reported
The management of patients with DD is difficult
Extraction
Follow-up and routine conservative treatment is another choice of treatment plan in DD
Maintenance of periodontal health
Endodontic treatment is contraindicated in teeth with total obliteration of root canals and pulp chambers
Another approach for the treatment of teeth with DD has included periapical surgery and retrograde filling which is recommended in teeth with long roots
bullSince these patients usually have early exfoliation of the teeth and consequently maxilla-mandibular bony atrophy treatment with a combination of onlay bone grafting and a sinus lift operation to accomplish implant placement can be used successfullybullThough a malalignment of the arch is one of the most common characteristics of this disorder orthodontic correction should not be applied in a routine fashion bullIn the majority of cases despite early diagnosis and the provision of regular dental care teeth are lost because of spontaneous abscess formation bullThere is no treatment for severe cases of DD type I other than extraction of symptomatic teeth Even for special cases onlay autogenous bone grafting and sinus lift technique are well-tested methods of augmentation
bullTherapy including extraction of all teeth curettage of cystic alteration and functional rehabilitation by the insertion of a conventional complete denture has been presented previously by Neumann et al
bullMunoz-Guerra et al _ reported successful treatment of a 24-year old female after onlaybone grafting and sinus augmentation
bullThough many cases of DD type I have been described some clinical aspects and theoretic issues remain uncertain bullEarly diagnosis of the condition is important for the initiation of effective preventive treatment Follow-up studies on patients with DD type I might provide insights into treatment strategies bullFuture molecular genetic research may determine precise information about the gene locus responsible for DD type I
Dentin dysplasia
1048708 Type II
1048708 Coronal type
1048708 Root length normal in both dentitions
1048708 Primary teeth
1048708 Clinically resemble dentinogenesis imperfecta
1048708 Radiographically have similar appearance to Type I
1048708 Permanent teeth
1048708 Normal coloration
1048708 Pulp chambers enlarged with apical extension-thistletube-
shaped or flame-shaped
Dd type 11
Autosomal dominant
Type I RadicularThe teeth have normal crowns and
abnormal rootsThe teeth are generally exfoliated
prematurely
Type II CoronalPrimary teeth are translucent with an amber colorAdult teeth appear normal
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
The patients medical history revealed no evidence of disturbance in general health
The patient was having class III malocclusion with spacing between maxillary and mandibular anteriors
Anterior open bite and mandibular prognatie
All the teeth were normal in shape and size There was a painful fistule on the buccal region of the maxillary right primary central incisor Periapical radiolucencies were present at the permanent upper central incisors
a The intraoral examination revealed normal size morphology and color of teeth
The radiograph revealed features of dentine dysplasia type I with normal appearance of crown but no root development
The maxillary primary central incisors right lateral incisor mandibular primary right canine first and second molars mandibular left second premolar was extracted owing to extensive mobility
The ground section was - superficial dentin of the crown appeared normal and the deeper layers of dentin had an atypical tubular pattern but the pulp chamber was obliterated by an unusual type of calcified material consisting of dentin
In the present case-
The calcified pulp chambers
Rootless teeth
Periapical radiolucent areas and the nature of the periapical lesion were characteristic findings for the diagnosis of DD type I
The etiology of DD is still unknown however several theories have been proposed in the dental literature
Logan et al _suggested that the dentinal papilla is responsible for the abnormalities in root development
Sauk et al -postulated an invagination of the epithelial root sheath resulting in abnormal dentin formation
Wesley et al disagreed with this suggestion and proposed that the condition is caused by an abnormal interaction of odontoblasts with ameloblasts leading to abnormal differentiation andor function of these odontoblasts
The cause of periapical radiolucencies in DD type I is not understood
Steidler et al-suggest that they are the result of pulpal necrosis occurring either secondary to caries or spontaneously
DD is recognized as a genetic disorder and is thought possibly to be a single gene mutation
When multiple family members have a similar pattern of pathosis this supports a diagnosis of a hereditary condition
An attempt to identify a familial history in our case the patients brother mother and grandfather had similar condition Although from the patients description
Histologically-the immediately subjacent dentin and the enamel appear normal
Deeper layers of dentin with demonstrate an atypical tubular pattern with amorphous atubular globular or nodular masses of abnormal dentin are seen
The periapical radiolucent areas seen in most cases of dentinal dysplasia have been interpreted as radicular cysts however in some cases a diagnosis of periapical granuloma has been reported
The management of patients with DD is difficult
Extraction
Follow-up and routine conservative treatment is another choice of treatment plan in DD
Maintenance of periodontal health
Endodontic treatment is contraindicated in teeth with total obliteration of root canals and pulp chambers
Another approach for the treatment of teeth with DD has included periapical surgery and retrograde filling which is recommended in teeth with long roots
bullSince these patients usually have early exfoliation of the teeth and consequently maxilla-mandibular bony atrophy treatment with a combination of onlay bone grafting and a sinus lift operation to accomplish implant placement can be used successfullybullThough a malalignment of the arch is one of the most common characteristics of this disorder orthodontic correction should not be applied in a routine fashion bullIn the majority of cases despite early diagnosis and the provision of regular dental care teeth are lost because of spontaneous abscess formation bullThere is no treatment for severe cases of DD type I other than extraction of symptomatic teeth Even for special cases onlay autogenous bone grafting and sinus lift technique are well-tested methods of augmentation
bullTherapy including extraction of all teeth curettage of cystic alteration and functional rehabilitation by the insertion of a conventional complete denture has been presented previously by Neumann et al
bullMunoz-Guerra et al _ reported successful treatment of a 24-year old female after onlaybone grafting and sinus augmentation
bullThough many cases of DD type I have been described some clinical aspects and theoretic issues remain uncertain bullEarly diagnosis of the condition is important for the initiation of effective preventive treatment Follow-up studies on patients with DD type I might provide insights into treatment strategies bullFuture molecular genetic research may determine precise information about the gene locus responsible for DD type I
Dentin dysplasia
1048708 Type II
1048708 Coronal type
1048708 Root length normal in both dentitions
1048708 Primary teeth
1048708 Clinically resemble dentinogenesis imperfecta
1048708 Radiographically have similar appearance to Type I
1048708 Permanent teeth
1048708 Normal coloration
1048708 Pulp chambers enlarged with apical extension-thistletube-
shaped or flame-shaped
Dd type 11
Autosomal dominant
Type I RadicularThe teeth have normal crowns and
abnormal rootsThe teeth are generally exfoliated
prematurely
Type II CoronalPrimary teeth are translucent with an amber colorAdult teeth appear normal
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
a The intraoral examination revealed normal size morphology and color of teeth
The radiograph revealed features of dentine dysplasia type I with normal appearance of crown but no root development
The maxillary primary central incisors right lateral incisor mandibular primary right canine first and second molars mandibular left second premolar was extracted owing to extensive mobility
The ground section was - superficial dentin of the crown appeared normal and the deeper layers of dentin had an atypical tubular pattern but the pulp chamber was obliterated by an unusual type of calcified material consisting of dentin
In the present case-
The calcified pulp chambers
Rootless teeth
Periapical radiolucent areas and the nature of the periapical lesion were characteristic findings for the diagnosis of DD type I
The etiology of DD is still unknown however several theories have been proposed in the dental literature
Logan et al _suggested that the dentinal papilla is responsible for the abnormalities in root development
Sauk et al -postulated an invagination of the epithelial root sheath resulting in abnormal dentin formation
Wesley et al disagreed with this suggestion and proposed that the condition is caused by an abnormal interaction of odontoblasts with ameloblasts leading to abnormal differentiation andor function of these odontoblasts
The cause of periapical radiolucencies in DD type I is not understood
Steidler et al-suggest that they are the result of pulpal necrosis occurring either secondary to caries or spontaneously
DD is recognized as a genetic disorder and is thought possibly to be a single gene mutation
When multiple family members have a similar pattern of pathosis this supports a diagnosis of a hereditary condition
An attempt to identify a familial history in our case the patients brother mother and grandfather had similar condition Although from the patients description
Histologically-the immediately subjacent dentin and the enamel appear normal
Deeper layers of dentin with demonstrate an atypical tubular pattern with amorphous atubular globular or nodular masses of abnormal dentin are seen
The periapical radiolucent areas seen in most cases of dentinal dysplasia have been interpreted as radicular cysts however in some cases a diagnosis of periapical granuloma has been reported
The management of patients with DD is difficult
Extraction
Follow-up and routine conservative treatment is another choice of treatment plan in DD
Maintenance of periodontal health
Endodontic treatment is contraindicated in teeth with total obliteration of root canals and pulp chambers
Another approach for the treatment of teeth with DD has included periapical surgery and retrograde filling which is recommended in teeth with long roots
bullSince these patients usually have early exfoliation of the teeth and consequently maxilla-mandibular bony atrophy treatment with a combination of onlay bone grafting and a sinus lift operation to accomplish implant placement can be used successfullybullThough a malalignment of the arch is one of the most common characteristics of this disorder orthodontic correction should not be applied in a routine fashion bullIn the majority of cases despite early diagnosis and the provision of regular dental care teeth are lost because of spontaneous abscess formation bullThere is no treatment for severe cases of DD type I other than extraction of symptomatic teeth Even for special cases onlay autogenous bone grafting and sinus lift technique are well-tested methods of augmentation
bullTherapy including extraction of all teeth curettage of cystic alteration and functional rehabilitation by the insertion of a conventional complete denture has been presented previously by Neumann et al
bullMunoz-Guerra et al _ reported successful treatment of a 24-year old female after onlaybone grafting and sinus augmentation
bullThough many cases of DD type I have been described some clinical aspects and theoretic issues remain uncertain bullEarly diagnosis of the condition is important for the initiation of effective preventive treatment Follow-up studies on patients with DD type I might provide insights into treatment strategies bullFuture molecular genetic research may determine precise information about the gene locus responsible for DD type I
Dentin dysplasia
1048708 Type II
1048708 Coronal type
1048708 Root length normal in both dentitions
1048708 Primary teeth
1048708 Clinically resemble dentinogenesis imperfecta
1048708 Radiographically have similar appearance to Type I
1048708 Permanent teeth
1048708 Normal coloration
1048708 Pulp chambers enlarged with apical extension-thistletube-
shaped or flame-shaped
Dd type 11
Autosomal dominant
Type I RadicularThe teeth have normal crowns and
abnormal rootsThe teeth are generally exfoliated
prematurely
Type II CoronalPrimary teeth are translucent with an amber colorAdult teeth appear normal
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
The radiograph revealed features of dentine dysplasia type I with normal appearance of crown but no root development
The maxillary primary central incisors right lateral incisor mandibular primary right canine first and second molars mandibular left second premolar was extracted owing to extensive mobility
The ground section was - superficial dentin of the crown appeared normal and the deeper layers of dentin had an atypical tubular pattern but the pulp chamber was obliterated by an unusual type of calcified material consisting of dentin
In the present case-
The calcified pulp chambers
Rootless teeth
Periapical radiolucent areas and the nature of the periapical lesion were characteristic findings for the diagnosis of DD type I
The etiology of DD is still unknown however several theories have been proposed in the dental literature
Logan et al _suggested that the dentinal papilla is responsible for the abnormalities in root development
Sauk et al -postulated an invagination of the epithelial root sheath resulting in abnormal dentin formation
Wesley et al disagreed with this suggestion and proposed that the condition is caused by an abnormal interaction of odontoblasts with ameloblasts leading to abnormal differentiation andor function of these odontoblasts
The cause of periapical radiolucencies in DD type I is not understood
Steidler et al-suggest that they are the result of pulpal necrosis occurring either secondary to caries or spontaneously
DD is recognized as a genetic disorder and is thought possibly to be a single gene mutation
When multiple family members have a similar pattern of pathosis this supports a diagnosis of a hereditary condition
An attempt to identify a familial history in our case the patients brother mother and grandfather had similar condition Although from the patients description
Histologically-the immediately subjacent dentin and the enamel appear normal
Deeper layers of dentin with demonstrate an atypical tubular pattern with amorphous atubular globular or nodular masses of abnormal dentin are seen
The periapical radiolucent areas seen in most cases of dentinal dysplasia have been interpreted as radicular cysts however in some cases a diagnosis of periapical granuloma has been reported
The management of patients with DD is difficult
Extraction
Follow-up and routine conservative treatment is another choice of treatment plan in DD
Maintenance of periodontal health
Endodontic treatment is contraindicated in teeth with total obliteration of root canals and pulp chambers
Another approach for the treatment of teeth with DD has included periapical surgery and retrograde filling which is recommended in teeth with long roots
bullSince these patients usually have early exfoliation of the teeth and consequently maxilla-mandibular bony atrophy treatment with a combination of onlay bone grafting and a sinus lift operation to accomplish implant placement can be used successfullybullThough a malalignment of the arch is one of the most common characteristics of this disorder orthodontic correction should not be applied in a routine fashion bullIn the majority of cases despite early diagnosis and the provision of regular dental care teeth are lost because of spontaneous abscess formation bullThere is no treatment for severe cases of DD type I other than extraction of symptomatic teeth Even for special cases onlay autogenous bone grafting and sinus lift technique are well-tested methods of augmentation
bullTherapy including extraction of all teeth curettage of cystic alteration and functional rehabilitation by the insertion of a conventional complete denture has been presented previously by Neumann et al
bullMunoz-Guerra et al _ reported successful treatment of a 24-year old female after onlaybone grafting and sinus augmentation
bullThough many cases of DD type I have been described some clinical aspects and theoretic issues remain uncertain bullEarly diagnosis of the condition is important for the initiation of effective preventive treatment Follow-up studies on patients with DD type I might provide insights into treatment strategies bullFuture molecular genetic research may determine precise information about the gene locus responsible for DD type I
Dentin dysplasia
1048708 Type II
1048708 Coronal type
1048708 Root length normal in both dentitions
1048708 Primary teeth
1048708 Clinically resemble dentinogenesis imperfecta
1048708 Radiographically have similar appearance to Type I
1048708 Permanent teeth
1048708 Normal coloration
1048708 Pulp chambers enlarged with apical extension-thistletube-
shaped or flame-shaped
Dd type 11
Autosomal dominant
Type I RadicularThe teeth have normal crowns and
abnormal rootsThe teeth are generally exfoliated
prematurely
Type II CoronalPrimary teeth are translucent with an amber colorAdult teeth appear normal
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
The maxillary primary central incisors right lateral incisor mandibular primary right canine first and second molars mandibular left second premolar was extracted owing to extensive mobility
The ground section was - superficial dentin of the crown appeared normal and the deeper layers of dentin had an atypical tubular pattern but the pulp chamber was obliterated by an unusual type of calcified material consisting of dentin
In the present case-
The calcified pulp chambers
Rootless teeth
Periapical radiolucent areas and the nature of the periapical lesion were characteristic findings for the diagnosis of DD type I
The etiology of DD is still unknown however several theories have been proposed in the dental literature
Logan et al _suggested that the dentinal papilla is responsible for the abnormalities in root development
Sauk et al -postulated an invagination of the epithelial root sheath resulting in abnormal dentin formation
Wesley et al disagreed with this suggestion and proposed that the condition is caused by an abnormal interaction of odontoblasts with ameloblasts leading to abnormal differentiation andor function of these odontoblasts
The cause of periapical radiolucencies in DD type I is not understood
Steidler et al-suggest that they are the result of pulpal necrosis occurring either secondary to caries or spontaneously
DD is recognized as a genetic disorder and is thought possibly to be a single gene mutation
When multiple family members have a similar pattern of pathosis this supports a diagnosis of a hereditary condition
An attempt to identify a familial history in our case the patients brother mother and grandfather had similar condition Although from the patients description
Histologically-the immediately subjacent dentin and the enamel appear normal
Deeper layers of dentin with demonstrate an atypical tubular pattern with amorphous atubular globular or nodular masses of abnormal dentin are seen
The periapical radiolucent areas seen in most cases of dentinal dysplasia have been interpreted as radicular cysts however in some cases a diagnosis of periapical granuloma has been reported
The management of patients with DD is difficult
Extraction
Follow-up and routine conservative treatment is another choice of treatment plan in DD
Maintenance of periodontal health
Endodontic treatment is contraindicated in teeth with total obliteration of root canals and pulp chambers
Another approach for the treatment of teeth with DD has included periapical surgery and retrograde filling which is recommended in teeth with long roots
bullSince these patients usually have early exfoliation of the teeth and consequently maxilla-mandibular bony atrophy treatment with a combination of onlay bone grafting and a sinus lift operation to accomplish implant placement can be used successfullybullThough a malalignment of the arch is one of the most common characteristics of this disorder orthodontic correction should not be applied in a routine fashion bullIn the majority of cases despite early diagnosis and the provision of regular dental care teeth are lost because of spontaneous abscess formation bullThere is no treatment for severe cases of DD type I other than extraction of symptomatic teeth Even for special cases onlay autogenous bone grafting and sinus lift technique are well-tested methods of augmentation
bullTherapy including extraction of all teeth curettage of cystic alteration and functional rehabilitation by the insertion of a conventional complete denture has been presented previously by Neumann et al
bullMunoz-Guerra et al _ reported successful treatment of a 24-year old female after onlaybone grafting and sinus augmentation
bullThough many cases of DD type I have been described some clinical aspects and theoretic issues remain uncertain bullEarly diagnosis of the condition is important for the initiation of effective preventive treatment Follow-up studies on patients with DD type I might provide insights into treatment strategies bullFuture molecular genetic research may determine precise information about the gene locus responsible for DD type I
Dentin dysplasia
1048708 Type II
1048708 Coronal type
1048708 Root length normal in both dentitions
1048708 Primary teeth
1048708 Clinically resemble dentinogenesis imperfecta
1048708 Radiographically have similar appearance to Type I
1048708 Permanent teeth
1048708 Normal coloration
1048708 Pulp chambers enlarged with apical extension-thistletube-
shaped or flame-shaped
Dd type 11
Autosomal dominant
Type I RadicularThe teeth have normal crowns and
abnormal rootsThe teeth are generally exfoliated
prematurely
Type II CoronalPrimary teeth are translucent with an amber colorAdult teeth appear normal
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
The ground section was - superficial dentin of the crown appeared normal and the deeper layers of dentin had an atypical tubular pattern but the pulp chamber was obliterated by an unusual type of calcified material consisting of dentin
In the present case-
The calcified pulp chambers
Rootless teeth
Periapical radiolucent areas and the nature of the periapical lesion were characteristic findings for the diagnosis of DD type I
The etiology of DD is still unknown however several theories have been proposed in the dental literature
Logan et al _suggested that the dentinal papilla is responsible for the abnormalities in root development
Sauk et al -postulated an invagination of the epithelial root sheath resulting in abnormal dentin formation
Wesley et al disagreed with this suggestion and proposed that the condition is caused by an abnormal interaction of odontoblasts with ameloblasts leading to abnormal differentiation andor function of these odontoblasts
The cause of periapical radiolucencies in DD type I is not understood
Steidler et al-suggest that they are the result of pulpal necrosis occurring either secondary to caries or spontaneously
DD is recognized as a genetic disorder and is thought possibly to be a single gene mutation
When multiple family members have a similar pattern of pathosis this supports a diagnosis of a hereditary condition
An attempt to identify a familial history in our case the patients brother mother and grandfather had similar condition Although from the patients description
Histologically-the immediately subjacent dentin and the enamel appear normal
Deeper layers of dentin with demonstrate an atypical tubular pattern with amorphous atubular globular or nodular masses of abnormal dentin are seen
The periapical radiolucent areas seen in most cases of dentinal dysplasia have been interpreted as radicular cysts however in some cases a diagnosis of periapical granuloma has been reported
The management of patients with DD is difficult
Extraction
Follow-up and routine conservative treatment is another choice of treatment plan in DD
Maintenance of periodontal health
Endodontic treatment is contraindicated in teeth with total obliteration of root canals and pulp chambers
Another approach for the treatment of teeth with DD has included periapical surgery and retrograde filling which is recommended in teeth with long roots
bullSince these patients usually have early exfoliation of the teeth and consequently maxilla-mandibular bony atrophy treatment with a combination of onlay bone grafting and a sinus lift operation to accomplish implant placement can be used successfullybullThough a malalignment of the arch is one of the most common characteristics of this disorder orthodontic correction should not be applied in a routine fashion bullIn the majority of cases despite early diagnosis and the provision of regular dental care teeth are lost because of spontaneous abscess formation bullThere is no treatment for severe cases of DD type I other than extraction of symptomatic teeth Even for special cases onlay autogenous bone grafting and sinus lift technique are well-tested methods of augmentation
bullTherapy including extraction of all teeth curettage of cystic alteration and functional rehabilitation by the insertion of a conventional complete denture has been presented previously by Neumann et al
bullMunoz-Guerra et al _ reported successful treatment of a 24-year old female after onlaybone grafting and sinus augmentation
bullThough many cases of DD type I have been described some clinical aspects and theoretic issues remain uncertain bullEarly diagnosis of the condition is important for the initiation of effective preventive treatment Follow-up studies on patients with DD type I might provide insights into treatment strategies bullFuture molecular genetic research may determine precise information about the gene locus responsible for DD type I
Dentin dysplasia
1048708 Type II
1048708 Coronal type
1048708 Root length normal in both dentitions
1048708 Primary teeth
1048708 Clinically resemble dentinogenesis imperfecta
1048708 Radiographically have similar appearance to Type I
1048708 Permanent teeth
1048708 Normal coloration
1048708 Pulp chambers enlarged with apical extension-thistletube-
shaped or flame-shaped
Dd type 11
Autosomal dominant
Type I RadicularThe teeth have normal crowns and
abnormal rootsThe teeth are generally exfoliated
prematurely
Type II CoronalPrimary teeth are translucent with an amber colorAdult teeth appear normal
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
In the present case-
The calcified pulp chambers
Rootless teeth
Periapical radiolucent areas and the nature of the periapical lesion were characteristic findings for the diagnosis of DD type I
The etiology of DD is still unknown however several theories have been proposed in the dental literature
Logan et al _suggested that the dentinal papilla is responsible for the abnormalities in root development
Sauk et al -postulated an invagination of the epithelial root sheath resulting in abnormal dentin formation
Wesley et al disagreed with this suggestion and proposed that the condition is caused by an abnormal interaction of odontoblasts with ameloblasts leading to abnormal differentiation andor function of these odontoblasts
The cause of periapical radiolucencies in DD type I is not understood
Steidler et al-suggest that they are the result of pulpal necrosis occurring either secondary to caries or spontaneously
DD is recognized as a genetic disorder and is thought possibly to be a single gene mutation
When multiple family members have a similar pattern of pathosis this supports a diagnosis of a hereditary condition
An attempt to identify a familial history in our case the patients brother mother and grandfather had similar condition Although from the patients description
Histologically-the immediately subjacent dentin and the enamel appear normal
Deeper layers of dentin with demonstrate an atypical tubular pattern with amorphous atubular globular or nodular masses of abnormal dentin are seen
The periapical radiolucent areas seen in most cases of dentinal dysplasia have been interpreted as radicular cysts however in some cases a diagnosis of periapical granuloma has been reported
The management of patients with DD is difficult
Extraction
Follow-up and routine conservative treatment is another choice of treatment plan in DD
Maintenance of periodontal health
Endodontic treatment is contraindicated in teeth with total obliteration of root canals and pulp chambers
Another approach for the treatment of teeth with DD has included periapical surgery and retrograde filling which is recommended in teeth with long roots
bullSince these patients usually have early exfoliation of the teeth and consequently maxilla-mandibular bony atrophy treatment with a combination of onlay bone grafting and a sinus lift operation to accomplish implant placement can be used successfullybullThough a malalignment of the arch is one of the most common characteristics of this disorder orthodontic correction should not be applied in a routine fashion bullIn the majority of cases despite early diagnosis and the provision of regular dental care teeth are lost because of spontaneous abscess formation bullThere is no treatment for severe cases of DD type I other than extraction of symptomatic teeth Even for special cases onlay autogenous bone grafting and sinus lift technique are well-tested methods of augmentation
bullTherapy including extraction of all teeth curettage of cystic alteration and functional rehabilitation by the insertion of a conventional complete denture has been presented previously by Neumann et al
bullMunoz-Guerra et al _ reported successful treatment of a 24-year old female after onlaybone grafting and sinus augmentation
bullThough many cases of DD type I have been described some clinical aspects and theoretic issues remain uncertain bullEarly diagnosis of the condition is important for the initiation of effective preventive treatment Follow-up studies on patients with DD type I might provide insights into treatment strategies bullFuture molecular genetic research may determine precise information about the gene locus responsible for DD type I
Dentin dysplasia
1048708 Type II
1048708 Coronal type
1048708 Root length normal in both dentitions
1048708 Primary teeth
1048708 Clinically resemble dentinogenesis imperfecta
1048708 Radiographically have similar appearance to Type I
1048708 Permanent teeth
1048708 Normal coloration
1048708 Pulp chambers enlarged with apical extension-thistletube-
shaped or flame-shaped
Dd type 11
Autosomal dominant
Type I RadicularThe teeth have normal crowns and
abnormal rootsThe teeth are generally exfoliated
prematurely
Type II CoronalPrimary teeth are translucent with an amber colorAdult teeth appear normal
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
The etiology of DD is still unknown however several theories have been proposed in the dental literature
Logan et al _suggested that the dentinal papilla is responsible for the abnormalities in root development
Sauk et al -postulated an invagination of the epithelial root sheath resulting in abnormal dentin formation
Wesley et al disagreed with this suggestion and proposed that the condition is caused by an abnormal interaction of odontoblasts with ameloblasts leading to abnormal differentiation andor function of these odontoblasts
The cause of periapical radiolucencies in DD type I is not understood
Steidler et al-suggest that they are the result of pulpal necrosis occurring either secondary to caries or spontaneously
DD is recognized as a genetic disorder and is thought possibly to be a single gene mutation
When multiple family members have a similar pattern of pathosis this supports a diagnosis of a hereditary condition
An attempt to identify a familial history in our case the patients brother mother and grandfather had similar condition Although from the patients description
Histologically-the immediately subjacent dentin and the enamel appear normal
Deeper layers of dentin with demonstrate an atypical tubular pattern with amorphous atubular globular or nodular masses of abnormal dentin are seen
The periapical radiolucent areas seen in most cases of dentinal dysplasia have been interpreted as radicular cysts however in some cases a diagnosis of periapical granuloma has been reported
The management of patients with DD is difficult
Extraction
Follow-up and routine conservative treatment is another choice of treatment plan in DD
Maintenance of periodontal health
Endodontic treatment is contraindicated in teeth with total obliteration of root canals and pulp chambers
Another approach for the treatment of teeth with DD has included periapical surgery and retrograde filling which is recommended in teeth with long roots
bullSince these patients usually have early exfoliation of the teeth and consequently maxilla-mandibular bony atrophy treatment with a combination of onlay bone grafting and a sinus lift operation to accomplish implant placement can be used successfullybullThough a malalignment of the arch is one of the most common characteristics of this disorder orthodontic correction should not be applied in a routine fashion bullIn the majority of cases despite early diagnosis and the provision of regular dental care teeth are lost because of spontaneous abscess formation bullThere is no treatment for severe cases of DD type I other than extraction of symptomatic teeth Even for special cases onlay autogenous bone grafting and sinus lift technique are well-tested methods of augmentation
bullTherapy including extraction of all teeth curettage of cystic alteration and functional rehabilitation by the insertion of a conventional complete denture has been presented previously by Neumann et al
bullMunoz-Guerra et al _ reported successful treatment of a 24-year old female after onlaybone grafting and sinus augmentation
bullThough many cases of DD type I have been described some clinical aspects and theoretic issues remain uncertain bullEarly diagnosis of the condition is important for the initiation of effective preventive treatment Follow-up studies on patients with DD type I might provide insights into treatment strategies bullFuture molecular genetic research may determine precise information about the gene locus responsible for DD type I
Dentin dysplasia
1048708 Type II
1048708 Coronal type
1048708 Root length normal in both dentitions
1048708 Primary teeth
1048708 Clinically resemble dentinogenesis imperfecta
1048708 Radiographically have similar appearance to Type I
1048708 Permanent teeth
1048708 Normal coloration
1048708 Pulp chambers enlarged with apical extension-thistletube-
shaped or flame-shaped
Dd type 11
Autosomal dominant
Type I RadicularThe teeth have normal crowns and
abnormal rootsThe teeth are generally exfoliated
prematurely
Type II CoronalPrimary teeth are translucent with an amber colorAdult teeth appear normal
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
Steidler et al-suggest that they are the result of pulpal necrosis occurring either secondary to caries or spontaneously
DD is recognized as a genetic disorder and is thought possibly to be a single gene mutation
When multiple family members have a similar pattern of pathosis this supports a diagnosis of a hereditary condition
An attempt to identify a familial history in our case the patients brother mother and grandfather had similar condition Although from the patients description
Histologically-the immediately subjacent dentin and the enamel appear normal
Deeper layers of dentin with demonstrate an atypical tubular pattern with amorphous atubular globular or nodular masses of abnormal dentin are seen
The periapical radiolucent areas seen in most cases of dentinal dysplasia have been interpreted as radicular cysts however in some cases a diagnosis of periapical granuloma has been reported
The management of patients with DD is difficult
Extraction
Follow-up and routine conservative treatment is another choice of treatment plan in DD
Maintenance of periodontal health
Endodontic treatment is contraindicated in teeth with total obliteration of root canals and pulp chambers
Another approach for the treatment of teeth with DD has included periapical surgery and retrograde filling which is recommended in teeth with long roots
bullSince these patients usually have early exfoliation of the teeth and consequently maxilla-mandibular bony atrophy treatment with a combination of onlay bone grafting and a sinus lift operation to accomplish implant placement can be used successfullybullThough a malalignment of the arch is one of the most common characteristics of this disorder orthodontic correction should not be applied in a routine fashion bullIn the majority of cases despite early diagnosis and the provision of regular dental care teeth are lost because of spontaneous abscess formation bullThere is no treatment for severe cases of DD type I other than extraction of symptomatic teeth Even for special cases onlay autogenous bone grafting and sinus lift technique are well-tested methods of augmentation
bullTherapy including extraction of all teeth curettage of cystic alteration and functional rehabilitation by the insertion of a conventional complete denture has been presented previously by Neumann et al
bullMunoz-Guerra et al _ reported successful treatment of a 24-year old female after onlaybone grafting and sinus augmentation
bullThough many cases of DD type I have been described some clinical aspects and theoretic issues remain uncertain bullEarly diagnosis of the condition is important for the initiation of effective preventive treatment Follow-up studies on patients with DD type I might provide insights into treatment strategies bullFuture molecular genetic research may determine precise information about the gene locus responsible for DD type I
Dentin dysplasia
1048708 Type II
1048708 Coronal type
1048708 Root length normal in both dentitions
1048708 Primary teeth
1048708 Clinically resemble dentinogenesis imperfecta
1048708 Radiographically have similar appearance to Type I
1048708 Permanent teeth
1048708 Normal coloration
1048708 Pulp chambers enlarged with apical extension-thistletube-
shaped or flame-shaped
Dd type 11
Autosomal dominant
Type I RadicularThe teeth have normal crowns and
abnormal rootsThe teeth are generally exfoliated
prematurely
Type II CoronalPrimary teeth are translucent with an amber colorAdult teeth appear normal
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
Histologically-the immediately subjacent dentin and the enamel appear normal
Deeper layers of dentin with demonstrate an atypical tubular pattern with amorphous atubular globular or nodular masses of abnormal dentin are seen
The periapical radiolucent areas seen in most cases of dentinal dysplasia have been interpreted as radicular cysts however in some cases a diagnosis of periapical granuloma has been reported
The management of patients with DD is difficult
Extraction
Follow-up and routine conservative treatment is another choice of treatment plan in DD
Maintenance of periodontal health
Endodontic treatment is contraindicated in teeth with total obliteration of root canals and pulp chambers
Another approach for the treatment of teeth with DD has included periapical surgery and retrograde filling which is recommended in teeth with long roots
bullSince these patients usually have early exfoliation of the teeth and consequently maxilla-mandibular bony atrophy treatment with a combination of onlay bone grafting and a sinus lift operation to accomplish implant placement can be used successfullybullThough a malalignment of the arch is one of the most common characteristics of this disorder orthodontic correction should not be applied in a routine fashion bullIn the majority of cases despite early diagnosis and the provision of regular dental care teeth are lost because of spontaneous abscess formation bullThere is no treatment for severe cases of DD type I other than extraction of symptomatic teeth Even for special cases onlay autogenous bone grafting and sinus lift technique are well-tested methods of augmentation
bullTherapy including extraction of all teeth curettage of cystic alteration and functional rehabilitation by the insertion of a conventional complete denture has been presented previously by Neumann et al
bullMunoz-Guerra et al _ reported successful treatment of a 24-year old female after onlaybone grafting and sinus augmentation
bullThough many cases of DD type I have been described some clinical aspects and theoretic issues remain uncertain bullEarly diagnosis of the condition is important for the initiation of effective preventive treatment Follow-up studies on patients with DD type I might provide insights into treatment strategies bullFuture molecular genetic research may determine precise information about the gene locus responsible for DD type I
Dentin dysplasia
1048708 Type II
1048708 Coronal type
1048708 Root length normal in both dentitions
1048708 Primary teeth
1048708 Clinically resemble dentinogenesis imperfecta
1048708 Radiographically have similar appearance to Type I
1048708 Permanent teeth
1048708 Normal coloration
1048708 Pulp chambers enlarged with apical extension-thistletube-
shaped or flame-shaped
Dd type 11
Autosomal dominant
Type I RadicularThe teeth have normal crowns and
abnormal rootsThe teeth are generally exfoliated
prematurely
Type II CoronalPrimary teeth are translucent with an amber colorAdult teeth appear normal
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
The management of patients with DD is difficult
Extraction
Follow-up and routine conservative treatment is another choice of treatment plan in DD
Maintenance of periodontal health
Endodontic treatment is contraindicated in teeth with total obliteration of root canals and pulp chambers
Another approach for the treatment of teeth with DD has included periapical surgery and retrograde filling which is recommended in teeth with long roots
bullSince these patients usually have early exfoliation of the teeth and consequently maxilla-mandibular bony atrophy treatment with a combination of onlay bone grafting and a sinus lift operation to accomplish implant placement can be used successfullybullThough a malalignment of the arch is one of the most common characteristics of this disorder orthodontic correction should not be applied in a routine fashion bullIn the majority of cases despite early diagnosis and the provision of regular dental care teeth are lost because of spontaneous abscess formation bullThere is no treatment for severe cases of DD type I other than extraction of symptomatic teeth Even for special cases onlay autogenous bone grafting and sinus lift technique are well-tested methods of augmentation
bullTherapy including extraction of all teeth curettage of cystic alteration and functional rehabilitation by the insertion of a conventional complete denture has been presented previously by Neumann et al
bullMunoz-Guerra et al _ reported successful treatment of a 24-year old female after onlaybone grafting and sinus augmentation
bullThough many cases of DD type I have been described some clinical aspects and theoretic issues remain uncertain bullEarly diagnosis of the condition is important for the initiation of effective preventive treatment Follow-up studies on patients with DD type I might provide insights into treatment strategies bullFuture molecular genetic research may determine precise information about the gene locus responsible for DD type I
Dentin dysplasia
1048708 Type II
1048708 Coronal type
1048708 Root length normal in both dentitions
1048708 Primary teeth
1048708 Clinically resemble dentinogenesis imperfecta
1048708 Radiographically have similar appearance to Type I
1048708 Permanent teeth
1048708 Normal coloration
1048708 Pulp chambers enlarged with apical extension-thistletube-
shaped or flame-shaped
Dd type 11
Autosomal dominant
Type I RadicularThe teeth have normal crowns and
abnormal rootsThe teeth are generally exfoliated
prematurely
Type II CoronalPrimary teeth are translucent with an amber colorAdult teeth appear normal
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
bullSince these patients usually have early exfoliation of the teeth and consequently maxilla-mandibular bony atrophy treatment with a combination of onlay bone grafting and a sinus lift operation to accomplish implant placement can be used successfullybullThough a malalignment of the arch is one of the most common characteristics of this disorder orthodontic correction should not be applied in a routine fashion bullIn the majority of cases despite early diagnosis and the provision of regular dental care teeth are lost because of spontaneous abscess formation bullThere is no treatment for severe cases of DD type I other than extraction of symptomatic teeth Even for special cases onlay autogenous bone grafting and sinus lift technique are well-tested methods of augmentation
bullTherapy including extraction of all teeth curettage of cystic alteration and functional rehabilitation by the insertion of a conventional complete denture has been presented previously by Neumann et al
bullMunoz-Guerra et al _ reported successful treatment of a 24-year old female after onlaybone grafting and sinus augmentation
bullThough many cases of DD type I have been described some clinical aspects and theoretic issues remain uncertain bullEarly diagnosis of the condition is important for the initiation of effective preventive treatment Follow-up studies on patients with DD type I might provide insights into treatment strategies bullFuture molecular genetic research may determine precise information about the gene locus responsible for DD type I
Dentin dysplasia
1048708 Type II
1048708 Coronal type
1048708 Root length normal in both dentitions
1048708 Primary teeth
1048708 Clinically resemble dentinogenesis imperfecta
1048708 Radiographically have similar appearance to Type I
1048708 Permanent teeth
1048708 Normal coloration
1048708 Pulp chambers enlarged with apical extension-thistletube-
shaped or flame-shaped
Dd type 11
Autosomal dominant
Type I RadicularThe teeth have normal crowns and
abnormal rootsThe teeth are generally exfoliated
prematurely
Type II CoronalPrimary teeth are translucent with an amber colorAdult teeth appear normal
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
bullTherapy including extraction of all teeth curettage of cystic alteration and functional rehabilitation by the insertion of a conventional complete denture has been presented previously by Neumann et al
bullMunoz-Guerra et al _ reported successful treatment of a 24-year old female after onlaybone grafting and sinus augmentation
bullThough many cases of DD type I have been described some clinical aspects and theoretic issues remain uncertain bullEarly diagnosis of the condition is important for the initiation of effective preventive treatment Follow-up studies on patients with DD type I might provide insights into treatment strategies bullFuture molecular genetic research may determine precise information about the gene locus responsible for DD type I
Dentin dysplasia
1048708 Type II
1048708 Coronal type
1048708 Root length normal in both dentitions
1048708 Primary teeth
1048708 Clinically resemble dentinogenesis imperfecta
1048708 Radiographically have similar appearance to Type I
1048708 Permanent teeth
1048708 Normal coloration
1048708 Pulp chambers enlarged with apical extension-thistletube-
shaped or flame-shaped
Dd type 11
Autosomal dominant
Type I RadicularThe teeth have normal crowns and
abnormal rootsThe teeth are generally exfoliated
prematurely
Type II CoronalPrimary teeth are translucent with an amber colorAdult teeth appear normal
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
bullThough many cases of DD type I have been described some clinical aspects and theoretic issues remain uncertain bullEarly diagnosis of the condition is important for the initiation of effective preventive treatment Follow-up studies on patients with DD type I might provide insights into treatment strategies bullFuture molecular genetic research may determine precise information about the gene locus responsible for DD type I
Dentin dysplasia
1048708 Type II
1048708 Coronal type
1048708 Root length normal in both dentitions
1048708 Primary teeth
1048708 Clinically resemble dentinogenesis imperfecta
1048708 Radiographically have similar appearance to Type I
1048708 Permanent teeth
1048708 Normal coloration
1048708 Pulp chambers enlarged with apical extension-thistletube-
shaped or flame-shaped
Dd type 11
Autosomal dominant
Type I RadicularThe teeth have normal crowns and
abnormal rootsThe teeth are generally exfoliated
prematurely
Type II CoronalPrimary teeth are translucent with an amber colorAdult teeth appear normal
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
Dentin dysplasia
1048708 Type II
1048708 Coronal type
1048708 Root length normal in both dentitions
1048708 Primary teeth
1048708 Clinically resemble dentinogenesis imperfecta
1048708 Radiographically have similar appearance to Type I
1048708 Permanent teeth
1048708 Normal coloration
1048708 Pulp chambers enlarged with apical extension-thistletube-
shaped or flame-shaped
Dd type 11
Autosomal dominant
Type I RadicularThe teeth have normal crowns and
abnormal rootsThe teeth are generally exfoliated
prematurely
Type II CoronalPrimary teeth are translucent with an amber colorAdult teeth appear normal
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
Dd type 11
Autosomal dominant
Type I RadicularThe teeth have normal crowns and
abnormal rootsThe teeth are generally exfoliated
prematurely
Type II CoronalPrimary teeth are translucent with an amber colorAdult teeth appear normal
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
Autosomal dominant
Type I RadicularThe teeth have normal crowns and
abnormal rootsThe teeth are generally exfoliated
prematurely
Type II CoronalPrimary teeth are translucent with an amber colorAdult teeth appear normal
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
-autosomal dominant disturbance
rare (1100000)
Type I (radicular) normal color amp shaped in both dentition
malaligned arch drifting and exfoliate with little or no trauma
short or abnormal root shaped pulp chamber amp root canals completely fill in before eruption
20 of teeth with type I disease have apical radiolucencies
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion
TypeII (coronal)
primary dentition appears as DI but permanent dentition is normal
obliterated of the pulp chamber amp reduced root canals after eruption
roots are normal in shape amp proportion