Boulder=a very large rock , lava =hot liquid rock which comes out of the Earth through a volcano, or the solid rock formed when it cools molten lava ; amorphous-having no fixed form or shape an amorphous mass of jelly
Logan et al. _suggested that the dentinal papilla(dentine and pulp) is responsible for the abnormalities in root development.They suggested that multiple degenerative foci within the papilla become calcified, leading to reduced growth and final obliteration of the pulp space.
A rare condition affecting the primary and permanent by dr ibrahim
Sekerci A.E., Etoz.M, Sahman.H, Sisman.Y, Nazlim.S
Dentin dysplasia (DD) is a rare hereditary
disturbance is inherited as an autosomal
unknown etiology that affects approximately
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: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
coronal dentin dysplasia
Characterized by:1.partial pulpal obliteration.
coronal pulp chambers
3. Thread-like root canals
4. Usually the absence of
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
Normal dentinal tubule
formationn appears to have been
blocked so that new dentine forms
around obstacles and takes on the
described as “lava flowing around
A 7-year-old girl was referred to the
Department complaining mobile teeth and a
swelling in the maxillary deciduous right
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
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.
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
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
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,
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
Logan et al. _suggested that the dentinal papilla is
responsible for the abnormalities in root
Sauk et al. -postulated an invagination of the
epithelial root sheath resulting in abnormal dentin
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
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
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.
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
The management of patients with DD is
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
Another approach for the treatment of teeth with
DD has included periapical surgery and
retrograde filling, which is recommended in
teeth with long roots.
•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
•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.
•Though many cases of DD type I have been described
some clinical aspects and theoretic issues remain
•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.
Root length normal in both dentitions
Clinically resemble dentinogenesis imperfecta
Radiographically have similar appearance to Type I
Pulp chambers enlarged with apical extensionthistletubeshaped or flame-shaped
Type I: Radicular
The teeth have normal crowns and
The teeth are generally exfoliated
Type II: Coronal
Primary teeth are translucent
with an amber color
Adult teeth appear normal
-autosomal dominant disturbance
Type I (radicular)
normal color & shaped in both dentition
malaligned arch, drifting and exfoliate with little or no
short or abnormal root shaped, pulp chamber & root canals
completely fill in before eruption
20 % of teeth with type I disease have apical radiolucencies
primary dentition appears as D.I., but permanent dentition is
obliterated of the pulp chamber & reduced root canals after
roots are normal in shape & proportion