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

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