232 American Academy of Pediatric DentistryPediatric Dentistry – 23:3, 2001
cl inical sect ion
cl inical sect ion
Dentinogenesis imperfecta: an early treatment strategy
Shabtai Sapir DMD Joseph Shapira DMD
Dr. Sapir is a pediatric dental resident and Dr. Shapira is an associate professor, Department of Pediatric Dentistry, the
Hebrew University- Hadassah Faculty of Dental Medicine, Jerusalem, Israel. Correspond with Dr. Shapira at
Dentinogenesis imperfecta (DI) type 2 is a disease inherited in
a simple autosomal dominant mode. As soon as the teeth erupt the
parents may notice the problem and look for a pediatric dentist’s
advice and treatment. Early diagnosis and treatment of DI is rec-
ommended, as it may prevent or intercept deterioration of the teeth
and occlusion and improve esthetics.
The purpose of this article is to present the objectives, treatment
options, and problems encountered in the treatment of DI in the
early primary dentition. A two-stage treatment of a toddler under
general anesthesia is described and discussed.
This paper recommends for severe cases of DI two treatment
stages performed under general anesthesia. Stage 1 is early (around
age 18-20 months) and is directed to covering the incisors with
composite restorations and the first primary molars with preformed
crowns. Stage 2 (around age 28-30 months) seeks to protect the
second primary molars with preformed crowns and cover the ca-
nines with composite restorations. (Pediatr Dent 23:232-237,
the primary and permanent dentition. The disease is inherited
in a simple autosomal dominant mode with high penetrance
and a low mutation rate.3 The reported incidence in the USA
is 1:8000 births.4 Shields et al proposed three types of denti-
nogenesis imperfecta: DI type 1 is associated with osteogenesis
imperfecta. DI type 2 has essentially the same clinical radio-
graphic and histological features as DI type 1 but without
osteogenesis imperfecta; DI type 3 is rare and is only found in
the triracial Brandywine population of Maryland.5 It has been
suggested that DI type 2 and DI type 3 are different expres-
sions of the same gene.6,7
Clinically, with DI both dentitions are affected. The color
of the teeth varies from brown to blue, sometimes described as
amber or gray, with an opalescent sheen.3 The enamel may
show hypoplastic or hypocalcified defects in about one-third
of the patients and, in an affected patient, tends to crack away
from the defective dentin. The exposed dentin may undergo
severe and rapid attrition.3
Radiographically, the teeth have bulbous crowns with con-
stricted short roots. Initially, pulp chambers may be abnormally
wide and resemble “shell teeth,” but they will progressively
obliterate.5 Histologically, the enamel, although normal in
structure, tends to crack. The dentin-enamel junction is not
entinogenesis imperfecta (DI) or hereditary opalescent
dentin, was first described in the late 19th century.1,2
It is a localized mesodermal dysplasia affecting both
scalloped. In most cases the structure of the mantle dentin is
normal,8 whereas the dentinal tubules of the circumferential
dentin are coarse and branched and the total number of tu-
bules is reduced.8 The presence of an atubular area in the dentin
with reduced mineralization and a reduced number of odon-
toblasts are consistent findings.8 Pulpal inclusions and much
interglobular dentin are also frequent.8
The biochemical characteristics of the dentin include a col-
lagen defect and a primary defect in the calcifying matrix.9
Takagi and Sasaki suggested that the dentin in DI type 2 is
deficient in the phosphorous ion, which is important in the
early stage of odontoblastic differentiation and its mineraliza-
tion.10 Susuki et al published a case describing DI type 2 with
absent enamel prisms and abnormal mantle dentin.11
The purpose of this article is to present the objectives, treat-
ment options and problems encountered in the treatment of
DI in the early primary dentition. A two-stage treatment of a
toddler under general anesthesia is described and discussed.
In DI, the primary dentition appears more severely affected
than the permanent dentition, evidenced by rapid wear of the
teeth.3 This attrition may cause pulpal involvement with den-
tal abscesses,9 and the short, constricted roots might break
under load, thus necessitating extraction. The severe attrition
may result in a rapid decrease in the occlusal height. In the early
primary dentition, these appear to be the most immediate prob-
lems, and soon after eruption it is generally necessary to protect
the primary molars with stainless steel crowns.12
In the restorative treatment of pediatric patients, glass
ionomer with fluoride-releasing and chemically attaching ma-
terials are recommended for occlussally non-stressed areas.13 An
acid etch technique followed by composite restoration is pro-
posed as an alternative for restoration of the anterior teeth.13,14
Polycarbonate crowns may offer an alternative for the restora-
tion of the anterior primary teeth.15 An acrylic overlay denture,
resting over the remnants of crowns and roots of the primary
dentition, also has been used successfully.16
Wright has stated that the dental approach for managing
dentinogenesis imperfecta will vary with the severity of the
clinical expression, while intracoronal restoration and bonded
veneers for anterior teeth may be acceptable in mild cases, they
might not last in severe cases exhibiting enamel fracturing and
Received December 6, 2000 Revision Accepted March 11, 2001
Pediatric Dentistry – 23:3, 2001 American Academy of Pediatric Dentistry 233
cl inical sect ion
The objectives of early treatment of DI in the primary den-
tition are as follows:
1. Maintain dental health and preserve vitality, form, and size
of the dentition.
2. Provide the patient with an esthetic appearance at an early
age, in order to prevent psychological problems.
3. Provide the patient with a functional dentition.
4. Prevent loss of vertical dimension.
5. Maintain arch length.
6. Avoid interfering with the eruption of the remaining per-
7. Allow normal growth of the facial bones and temporoman-
dibular joint (TMJ).
8. Establish a rapport with the patient and the patient’s family
early in the treatment.
Treatment of the mixed and permanent dentition is chal-
lenging and frequently demands a multidisciplinary approach.
Collaboration of the pediatric dentist with a prosthodontist and
an orthodontist is often imperative. Although caries is not a
major concern in most cases, strict oral hygiene instructions
and preventive treatment is important in order to prevent car-
ies from adding to existing problems. Frequently, there is a need
to reestablish the vertical dimension of occlusion in order to
restore the occlusion in the mixed and permanent dentition.
Prosthetic restoration combined with orthodontic treatment
may be advantageous, and evaluation of the occlusion prior to
initiation of treatment is advised.18
Fig 1. Periapical radiographs of upper and lower incisors of ML at 8 months
of age, showing poorly calcified teeth and thin dentin with wide pulp
Fig 2. Preoperative clinical view at age 20 months, showing the brown-
colored teeth, severe attrition, thin bucolingual dimensions of the incisors.
Fig 3. Preoperative clinical view at age 20 months, showing the brown-
colored teeth, severe attrition, thin bucolingual dimensions of the incisors.
Fig 4. Periapical radiographs of posterior quandrants, demonstrating thin
irregular enamel and dentin, and wide pulp chambers resembling “shell
234 American Academy of Pediatric DentistryPediatric Dentistry – 23:3, 2001
cl inical sect ion
Many treatment modalities have been suggested:
overdentures, stainless steel crowns, jacket crowns, pin-retained
cast gold “thimbles” under acrylic resin crowns, stainless steel
crowns with acrylic facing, and simple, removable appli-
ances.16,19-22 A combination of partial dentures and prosthetic
crowns on the anterior teeth has also been described.23 Indi-
rect resin crown (IRC) technique to restore the maxillary
incisors has been used to treat a patient with severe enamel hy-
poplasia and attrition. This technique may offer esthetic,
affordable, and long-lasting intermediate restoration.24 Orth-
odontic treatment has been successfully performed in patients
with different degrees of DI.25-27 In less severe cases of DI, car-
bamide peroxide bleaching has been successfully used to treat
ML, an 8-month-old Caucasian girl, was brought by her par-
ents to Department of Pediatric Dentistry at the Hadassah
School of Dental Medicine in Jerusalem, Israel. The parents
had noticed that the newly erupted lower incisors were gray-
ish. The child was healthy, but the family history revealed that
the father, his cousin, and their parents had DI. The father was
treated in the Hadassah clinic 32 years ago when he was 7 years
old. His cousin was 14 years old when treated with removable
overlay bridges. The oral examination of the child revealed
partially erupted incisors with a brown blue color. The teeth
were very small. The radiographic examination revealed poorly
calcified incisors, exceptionally thin dentin, with wide pulp
chambers resembling “shell teeth” (Fig 1).
At the follow-up examination at age 14 months, severe at-
trition of the erupted teeth was noticed. That necessitated early
intervention to intercept the developing malocclusion and
tooth attrition and in order to preserve the vitality and form
of the teeth. The anxious parents were interested in beginning
treatment as early as possible. The parents expressed concern
that the child and her peers would notice the discolored teeth.
Since the father was edentulous and had undergone much suf-
fering, he was very concerned that his daughter would not
encounter the same problems. It was decided to cover the teeth
with crowns in two stages:
Stage 1 – as soon as possible, (at age 18-20 months), to cover
the incisors with composite restorations and the first pri-
mary molars with preformed crowns.
Stage 2 – (at age 28-30 months) to protect the second primary
molars with preformed crowns and to cover the canines
with composite restorations.
At 20 months of age the child was admitted to the operat-
ing room and treatment was performed under general
anesthesia. Close observation of the teeth revealed severe attri-
tion of the primary first molars. Enamel existed only in the
unerupted subgingival part of the crowns of the primary first
molars. The incisors were spaced with extremely small crowns
and with an exceptionally thin buco-lingual dimension (Figs
2-3). Radiographically, the pulp chambers were very wide, with
thin dentin, resembling “shell teeth” (Fig 4).
An attempt was made to fit polycarbonate crowns and pre-
formed polymer glass crowns (Glasstech, Austin, Tx.) for the
anterior teeth, but both were too large labiolingually. In order
not to weaken the teeth and to prevent pulp exposure, no re-
duction of tooth substance was performed. After total etching
of the incisors with 37% phosphoric acid for 30 seconds, the
teeth were coated with two layers of Single-Bond (3M).
Pedoform strip crowns (3M) were filled with composite Z-100
(3M) and cured for 80 seconds.
The preparation of the primary molars presented us with
other problems: Short tooth crowns due to insufficient erup-
tion and attrition, and thin, short, fragile roots. The cervical
enamel was reduced slightly using a tapered diamond, and care
was taken not to reduce the rest of the crown. Unitek stainless
steel crowns (3M) were shortened and individually adapted.
The crowns were glued with Glass Ionomer Cement, using only
moderate pressure (Fig 5). During the follow-up over the next
6 months, some of the composite restorations on the anterior
teeth chipped away and needed replacement. The child was re-
admitted and the restorations were replaced under sedation.
At the age of 30 months, the second stage of treatment was
conducted under general anesthesia. Preoperative clinical view
(Fig 6), shows some of the composite restorations that were
missing or chipped away and the extremely small clinical
crowns of the primary canines that underwent rapid attrition.
Fig 5. Postoperative clinical view at age 20 months, just after completing
treatment under general anesthesia.
Fig 6. Preoperative clinical view at age 30 months, showing some of the
composite restorations to be missing or chipped away. Note the right
anterolateral open bite due to pacifier use.
Pediatric Dentistry – 23:3, 2001 American Academy of Pediatric Dentistry 235
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Preoperative radiographs done at that time (Fig 7) demon-
strated the pulp obliteration of the primary incisors and the
attrition of the canines. Only minimal reduction of tooth sub-
stance was performed on the treated teeth. The second primary
molars were covered with stainless steel crowns, and the canines
were covered with pedoform strip crowns (Fig 8). The six-
month followup examination revealed the restorations to be
esthetically acceptable and functional.
The importance of restoring dental defects associated with DI
is obvious. There is no clear answer as to the best time for ini-
tiating treatment. The literature abounds with case reports and
treatment modalities for DI; in general, the recommendation
is to start treatment as early as possible.13,17 The rational use of
general anesthesia or pharmacotherapeutic regimens at an early
age enables us to safely utilize state of the art restorative tech-
niques in the treatment of young patients suffering from
hereditary dental anomalies.29 However, there are only two case
reports describing treatments of the very young child.30,31;
Daugaard-Jensen and Nielsen described 3 cases, the youngest
being a 19-month-old with DI type 1.
The initial treatment was with stainless steel crowns on the
molars which were replaced later with cast gold crowns due to
severe attrition. The cast gold crowns were not retentive and
parapulpar pins were prepared to improve retention. The an-
terior teeth were restored in one case with polycarbonate crowns
and with stainless steel crowns in another case.30 Torija and
Quijada described the dental treatment of a 3-year-old child
with DI type 1. The treatment included covering the poste-
rior teeth with stainless steel crowns and the anterior teeth with
stainless steel crowns with open face composite restorations.31
Treating a child at such an early age raises certain questions
and problems. The main dilemmas involved in the early treat-
ment of DI include:
1. High cost.
2. Risk of general anesthesia.
3. Postponing treatment could result in better coopera-
tion and obviate the need for a general anesthetic.
4. There are technical problems in restoring teeth with mini-
mal clinical crowns and wide pulp chambers.
However, the advantages of early treatment outweigh the
a. Early treatment can prevent/ intercept possible problems:
loss of the vertical dimension, possible TMJ problems, and
arch length complications, thus providing the patient with
a healthy and functional dentition.
b. The importance of an esthetic look cannot be ignored. The
smile is very important for socialization and for the child’s
“self concept” from an early age and for establishing a
positive “basic reality.”32-34
c. The concern of the parents and their wishes should not be
The problem of wide pulp chambers and thin dentin in this
very young patient presented great difficulty in the prepara-
tion of the teeth. In order to receive maximal retention for the
bonded composite, some preparation of the teeth eventually
was necessary. However, in the first stage of treatment this was
not done. It is possible that this resulted in a low retention rate
of some of the composite restorations. However, the incisors
were still protected from pulp exposure and further attrition
until the next stage of treatment, when a few of the anterior
restorations were redone.
The clinical severity of DI needs to be assessed when devel-
oping a treatment plan. When the disease is more severe, the
biochemical properties of the enamel and dentin are compro-
mised,11 and this can affect adhesion strength.
In spite of the success described earlier with composite res-
torations in treatment of DI in permanent teeth,14 success
might not be the same in severe cases of DI in the primary
Fig 8. Postoperative clinical view at age 30 months after completing the
treatment under general anesthesia.
Fig 7. Preoperative periapical radiographs of upper and lower incisors at 30
months of age, demonstrating overextension of some of the upper incisor
composite restorations and periodontal space widening. Note the pulp canal
obliteration already enident in the lower incisors.
236 American Academy of Pediatric DentistryPediatric Dentistry – 23:3, 2001
cl inical sect ion
dentition. In such cases, it may be better to prepare the teeth
with a diamond bur, take off all of the enamel that might crack
off in the future, and then bond the composite restoration to
the dentin. Rada and Hasiakos have suggested using glass
ionomer cements and dentinal adhesives as the first layer, with
a composite restoration on top, in the treatment of Amelogen-
esis Imperfecta. They hypothesized that chemical binding
might improve the durability of the restoration.35
In less severe cases, where normal enamel exists, mechani-
cal preparation of the enamel may be beneficial in reducing the
aprismatic layer which exists even in normal incisors.36 This
may expose the prismatic enamel and improve mechanical re-
In the future, and with better patient cooperation, it may
be possible to suggest transitional treatment with IRC.24 This
intermediate modality of treatment, combined with chemical
adhesion of composite resin cement and the mechanical adap-
tation of a well-adjusted crown may solve retention problems.
1. Early diagnosis and treatment of DI is recommended, as
it may prevent or intercept deterioration of the teeth and
occlusion and improve esthetics.
2. In severe cases of DI, two treatment stages of the primary
dentition under general anesthesia are recommended.
3. In moderate cases, a one-stage treatment of the primary
dentition at age 30 months may be justified.
4. Composite restoration in severe cases of DI might not
5. Long term followup is imperative in order to intercept
complications and adjust the treatment to the changes of
the dentition and occlusion.
1. Burrett WC. Description of a case having roots of a full den-
ture but no crowns. Missouri Dent J 15:117-122, 1983.
2. Talbot ES. Arrests of development and decalcification of
enamel and dentin. JADA 20:30, 1983.
3. Witkop CJ, Rao S. Inherited Defects in Tooth Structure. Bal-
timore, Williams and Wilkins; 1971:153.
4. Witkop CJ. Genetics and Dentistry. Eugen Quart 5:15-21,
5. Shields ED, Bixter D, El-Kafrawy AM. Proposal classifica-
tion for heritable human dentin defects with a description
of a new entity. Arch Oral Biol 18:543-553, 1973.
6. Boughman JA, Halloran SL, Raulston D. et al. An
autosomal-dominant form of juvenile periodontitis: its local-
ization to chromosome 4 and linkage to dentinogenesis
imperfecta and Gc. J Craniofac Genet Dev Biology 6:341-350,
7. Witkop CJ. Amelogenesis imperfecta, dentinogenesis
imperfecta and dentin dysplasia. revisited: problems of clas-
sification. J Oral Pathol 17:547-553, 1988.
8. Waltimo J, Ranta H, Lukinmaa. Ultrastructure of dentin ma-
trix in heritable dentin defects. Scanning Microscopy
9. Butler WT. Dentin matrix problems. Eur J Oral Sci 106:204-
10. Takagi Y, Sasaki S. A probable common disturbance in the
early stage of odontoblast differentiation in dentinogenesis
imperfecta type I and type 2. J Oral Pathol 17:208-212, 1988.
11. Suzuki S, Nakata M, Eto K. Clinical and histological obser-
vations of opalescent dentin associated with enamel defects.
Oral Surg Oral Med Oral Path 44:767-774, 1977.
12. Gibbard PD. The management of children and adolescents
suffering from amelogenesis imperfecta and dentinogenesis
imperfecta. International J of Orthod 12:15-25, 1974.
13. Ranta H, Lukinmaa PL, Waltimo J. Heritable dentin defects:
Nosolgy, pathology and treatment. Am J of Medical Genetics
14. Posnick WR. Treatment of hereditary opalescent dentin: re-
port of case. J Dent Child 43:46-48, 1976.
15. Stewart RE, Luke LS, Pike AR. Preformed polycarbonate
crowns for the restoration of anterior teeth. JADA 88:103-
16. Darendeliler-Kaba A, Marechaux SC.Hereditary dentinogen-
esis imperfecta: a treatment program using an overdenture. J
Dent Child 59:273-276, 1992.
17. Wright JT. The diagnosis and treatment of dentinogenesis
imperfecta and amelogenesis imperfecta. Hellenic Dental Jour-
nal 2:17-24, 1992.
18. Rivera-Morales WC, Mohl ND. Restoration of the vertical
dimention of occlusion in the severely worn dentition. Dent
Clin North Am 36:651-664, 1992.
19. Ward ML. Rehabilitation of a dentition affected by heredi-
tary dentinogenesis imperfecta. Dent Prac D Rec 10:16-18,
20. Mars M, Smith BGN. Dentinogenesis imperfecta. An inte-
grated conservative approach to treatment. Br Dent J
21. Held HW. Hereditary dentinogenesis imperfecta. Dent
Radiogr Photogr 35:3-6, 1962.
22. Bow P. Dentinogenesis imperfecta: a method of semiperma-
nent restoration. J Dent Assoc South Afr 33:293-300, 1978.
23. Mayordomo R.G, Estrela F, Anitua de Aldecoa E: Dentino-
genesis imperfecta: a case report. Quintessence International.
23:795 -802, 1992.
24. Quionez F, Hoover R, Wright JT. Transitional anterior es-
thetic restorations for patients with enamel defects. Pediatr
Dent 22:65-67, 2000.
25. Malmegren B, Lundberg M, Lmidskog S. Dentinogenesis
imperfecta in a six generation family. Swed Dent J 12:73-84,
26. Crowell MD. Dentinogenesis imperfecta: a case report. Am
J Orthod Dentofacial Orthop 113:367-371, 1998.
27. Larsson E, Nordblom A. Thirteen-year-old boy with
dentiogenesis imperfecta-pedodontic and orthodontic treat-
ment. Swed Dent J 5:213-217, 1981.
28. Croll TP, Sasa IS. Carbamide peroxide bleaching of teeth
with dentinogenesis imperfecta discoloration: report of a case.
Quintessence International. 26:683-686, 1995.