Dentin dysplasia is a rare genetic condition that affects tooth development. There are two types: Type I affects both dentitions and causes teeth to have normal crowns but abnormal, stunted roots. The pulp chambers and root canals are often completely obliterated. Teeth frequently exfoliate prematurely. Type II primarily affects the primary dentition, causing teeth to appear translucent. However, the permanent dentition appears normal. It is characterized by an enlarged pulp chamber and abnormal pulp canal morphology. The document describes a case study of a 7-year-old girl diagnosed with Type I dentin dysplasia based on clinical and radiographic features including rootless teeth and obliterated

1. JOMR;2013:1(1) 13-16
Sekerci A.E., Etoz.M, Sahman.H, Sisman.Y, Nazlim.S
DR IBRAHIM

2. 
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Dentin dysplasia (DD) is a rare hereditary
disturbance is inherited as an autosomal
dominant trait.
unknown etiology that affects approximately
1 :100,000.
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

3. Radicular dentin dysplasia
 Characterized by:1.Both dentitions are affected.
2.Normal appearing crowns
3.No or only rudimentary root
development (rootless teeth)
4.Incomplete or total obliteration
of the pulp chamber.
5.Teeth may exhibit extreme
mobility and exfoliate
prematurely.

4. coronal dentin dysplasia
 Characterized by:1.partial pulpal obliteration.
2.Thistle-tube-or flame-shaped
coronal 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.

5. 
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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 “lava flowing around
boulders”

6. 
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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 mandibular
left primary second molar by the age of 5. Her
older brother had a similar condition.
The patient's mother became edentulous at an
early age and had required full maxillary and
mandibular dentures

7. 
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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 patient's father
revealed no evidence similar condition.

8. 
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The patient's 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.

9. a. The intraoral examination revealed normal size, morphology and color of teeth

10. The radiograph revealed features of dentine dysplasia type I with normal appearance
of crown but no root development

11. 
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

12. 
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.

13. 
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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.

14. 
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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
and/or function of these odontoblasts.
The cause of periapical radiolucencies in DD type I is
not understood.

15. 
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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 patient's brother, mother, and
grandfather had similar condition. Although
from the patient's description.

16. 
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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.

17. 
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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.

18. •Since 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 successfully.
•Though 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.
•In the majority of cases, despite early diagnosis and the
provision of regular dental care, teeth are lost because of
spontaneous abscess formation.
•There 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

19. •Therapy, 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.
•Munoz-Guerra et al. _ reported successful
treatment of a 24-year old female after onlay
bone grafting and sinus augmentation.

20. •Though many cases of DD type I have been described
some clinical aspects and theoretic issues remain
uncertain.
•Early 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.
•Future molecular genetic research may determine
precise information about the gene locus responsible for
DD type I.

23. Dentin dysplasia
􀂄 Type II
􀂄 Coronal type
􀂄 Root length normal in both dentitions
􀂄 Primary teeth
􀂄 Clinically resemble dentinogenesis imperfecta
􀂄 Radiographically have similar appearance to Type I
􀂄 Permanent teeth
􀂄 Normal coloration
􀂄 Pulp chambers enlarged with apical extensionthistletubeshaped or flame-shaped

24. Dd type 11

25. Autosomal dominant
Type I: Radicular
The teeth have normal crowns and
abnormal roots
The teeth are generally exfoliated
prematurely
Type II: Coronal
Primary teeth are translucent
with an amber color
Adult teeth appear normal

26. -autosomal dominant disturbance
rare (1:100,000)
Type I (radicular)
normal color & shaped in both dentition
malaligned arch, drifting and exfoliate with little or no
trauma
short or abnormal root shaped, pulp chamber & root canals
completely fill in before eruption
20 % of teeth with type I disease have apical radiolucencies



28. TypeII (coronal)
primary dentition appears as D.I., but permanent dentition is
normal
obliterated of the pulp chamber & reduced root canals after
eruption
roots are normal in shape & proportion