Early Rehabilitation of Incisors with Dentinogenesis Imperfecta Type II – Case Report

Early Rehabilitation of Incisors with Dentinogenesis Imperfecta Type

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Ana Paula CA Beltrame*/ Maíra Mery Rosa**/ Ricardo AT Noschang***/ Izabel CS Almeida****

Dentinogenesis imperfecta is an phenotypic alteration in the formation of the organic matrix. It causes the
rapid and progressive wear of tooth structure, which may compromise tooth function and aesthetics. This is a
case of a 1y, 8m-old child with dentinogenesis imperfecta. All teeth presented with an opalescent appearance
and grayish color hue. Compromised structural integrity was noted as excessive wear and fracturing of the
enamel from the dentin layer. With low doses of midazolam (oral) and chloral hydrate (rectal) administration,
in a hospital environment, sedation was used to aid full mouth rehabilitation. Direct bonded restorations
were performed on primary maxillary incisors and indirect restorations, pre-made on a plaster model
using composite resin, were performed on primary mandibular incisors. After 32 months, we observed that
diagnosis and early treatment allowed preventive maintenance of the patient’s primary dentition, maintaining
tooth function and esthetics.

Key words: dentinogenesis imperfecta, composite resin, resin veneers.

INTRODUCTION

D
III, in addition to changes related to the color of the teeth, there are
entinogenesis imperfecta (D.I.) is a tooth formation large pulp chambers with little calcification, pulp exposure, and
disorder that affects 1/8000 individuals.1 It is associated generally shell teeth.
with an inherited genetic alteration of autosomal dominant In dentinogenesis imperfecta, tooth enamel usually develops
origin with the mutation of chromosome 4q21.2 This mutation alters properly, however, the enamel-dentine junction is smooth, wrin-
the protein dentin sialophosphoprotein (DSPP) and its development, kle-free, unlike what is considered the norm.4,5 This characteristic
which generates a malformation in the organic matrix of the dentin.2 favors enamel detachment and fractures, exposing the underlying
It affects both dentitions, being usually more intense in the malformed dentin.5
primary dentition and can be classified into three types:3 Type I, Below the amelo-dentin junction, due to structural malfor-
in addition to changes in the teeth, is associated with osteogenesis mation, the dentin appears altered, with low mineralization,
imperfecta; Type II is the most common, presenting the similar irregularities, few and often obliterated dentinal tubules, causing
dental conditions but not associated with osteogenesis imper- higher fragility.6 The exposed dentin wears quickly, leading to
fecta; Type III is very rare and has been observed in a group with a reduction in the vertical dimension, pulp exposure and even
tri-racial miscegenation (Native American, African American and root fracture under load, since the roots are narrow and slightly
European Caucasian) in the Maryland region, in the US. In Type mineralized.4,5,7 This change in dentin mineralization also affects
the color of the tooth.6
Individuals with this phenotype clinically present teeth color
From the Department of Dentistry, Graduate Program in Dentistry, Concen- change ranging from blue-gray to yellowish brown and increased
tration Area–Pediatric Dentistry, Federal University of Santa Catarina, opalescence.1,4 Radiography shows bulbar crowns, constriction in
Florianópolis, Santa Catarina, Brazil. the cervical region of the teeth and narrow roots.2,5 In initial assess-
*Ana Paula C A Beltrame, DDS, MS, PhD student.
**Maíra Mery Rosa, DDS.
ments, individuals may present increased pulp chamber, which
***Ricardo A T Noschang, DDS, MS, PhD student. later suffers a process of obliteration due to dentin deposition.7
****Izabel C S Almeida, DDS, MS, PhD, Full Professor. Through discoloration and abnormal wear patterns, this pheno-
typic alteration is a chronic, progressive degradation of the child’s
Send all correspondence to: dentition that, typically, negatively influences the child’s peer/
Ricardo Augusto Tomaz Noschang social relationships.4
Elizeu di Bernardi 200, loja. 06
Zip Code: 88101-050
In addition to the functional and aesthetic results, the treatment
Phone: 55 48 9106 9042 prevents significant wear of the tooth and the possibility of tooth
E-mail: ricardo.noschang@posgrad.ufsc.br loss.5 Thus, the diagnosis should be made as early as possible, as
well as the implementation of treatment.4

112 The Journal of Clinical Pediatric Dentistry Volume 41, Number 2/2017
 Early Rehabilitation of Incisors with Dentinogenesis Imperfecta Type II – Case Report

In the case of children, in addition to the actual clinical proce- of the Federal University of Santa Catarina, under sedation with an
dures, there is a need to manage behavioral cooperation because this initial oral dose of midazolam (0.3 ml/Kg) and followed by a rectally
is generally a complicating, limiting factor. administered chloral hydrate (1 ml/Kg, enema 10%), performed and
Several treatments for improving oral conditions have been managed by the medical anesthesiologist, and the direct restorations
proposed, including direct bonded composite restorations, veneers of the primary maxillary incisors (51,52,61,62) with composite resin,
and indirect fabricated composite resin restorations, as well as, dental were carried out by two pediatric dental residents. Since there was
implants and full mouth rehabilitation with partial or full dentures.2,4 no need for tooth preparation or additional retention, local anesthesia
The objective of this paper is to present the diagnosis of a 1 year, was not required or used. The teeth received prophylaxis, etching
8 month old pediatric patient of D.I. and the subsequent treatment and application of adhesive (Single Bond, 3M of Brasil, Brazil).

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using direct and indirect composite resin restorations and the asso- The restorations were made using acetate crowns (TDV, Pomerode,
ciated follow-up care. Brazil) and relative isolation. Acetate crowns were tested, adjusted,
drilled for draining excesses and filled with composite resin
Case Report (Charisma, Heraeus Kulzer, Hanau, Germany) in color A1 (Figure
The patient LML, 1-year, 8-month-old, attended the Pediatric 3). They were adapted to the teeth and light-cured on the labial and
Dental Clinic of the Federal University of Santa Catarina, in Flori- lingual surfaces for 40 seconds each. For the reconstruction of teeth
anópolis, Brazil. The patient’s mother, accompanied her daughter 71, 72, 81 and 82, an addition silicone (Express XT, 3M ESPE,
at the initial exam and reported that her daughter’s teeth began to Germany) model was made in a dental office, with a double dose of
break as soon as they erupted, subsequently presenting with sensi- the catalyst, along with a special stone plaster working model. With
tivity while ingesting cold foods. In her history, it was found that the the aid of the plaster-working model, restorations were pre-made
child had good health with no significant history of disease. Upon with composite resin (Charisma, Heraeus Kulzer, Hanau, Germany),
clinical examination, an incomplete primary dentition with absence also in color A1 (Figure 4), incrementally fabricated to ensure proper
of the primary second molars was observed. The remaining teeth UV light polymerization. In the following appointment, with the
had an opalescent sheen with a grayish hue. All remaining teeth patient under sedation as previously described, the lower incisors
showed incisal wear and fractures into the dentinal layer; however, were prepared with prophylaxis, etching, application of adhesive
the primary mandibular incisors had significantly more coronal agent (Single Bond, 3M do Brasil, São Paulo, Brazil), and ultimately
tooth wear (Figure 1). Clinically, these signs were consistent with cemented to lower anterior incisors with resin cement (RelyX, 3M
the diagnosis of dentinogenesis imperfecta. No carious lesions were ESPE, Hanau, Germany). The final restorative appointment was held
observed and parent reaffirmed the child’s healthy food and hygiene in the dental office, where the indirect restoration’s occlusion was
habits. Upon conducting an oral clinical examination of the patient’s adjusted and provided a final polish (Figure 5).
mother, highly similar opalescent-grayish sheen was observed on her The patient was seen after three months for evaluation of the
remaining natural teeth with the majority of her other teeth having restorations and observation of the erupting natural teeth’s condi-
partial-full coverage restorations. The treatment plan was organized tion. In the second evaluation, after six months, the restorations
in three subsequent phases (prophylaxis, restorative, and follow-up were intact and the deciduous dentition had completely erupted,
care). In order to further reduce the sensitivity until the restorative with no clinically observable loss of molars and/or canines. After
procedures were defined and implemented, the first treatment phase 32 months of observation the restorations continue to play it’s role
included prophylaxis with an application of fluoride varnish. A with success (Figure 6).
total of four visits to apply fluoride varnish and proper prophylaxis
was required. The second treatment phase required four separate DISCUSSION
appointments to clinically apply the planned restorations of choice. In this case of dentinogenesis imperfecta where an pediatric
The anterior primary maxillary incisors lacked significant wear and patient age <2 years old, the current early diagnosis and treatment
direct restorations with composite resin were planned. Whereas, was to manage the process of tooth wear that will likely progress
due to the significant loss of viable dental structure and possibly to teeth sensitivity, pulp damage/exposure, and problems related to
enabling greater longevity of the treatment for the primary lower the change of the vertical dimension. Additionally, as the degrada-
incisors, the treatment option selected was for indirect composite tion, wear, and discoloration of teeth rapidly progress cases there
resin restorations (prefabricated on a plaster stone model). After is an even greater opportunity/need to treat and restore aesthetics
completion of the restoring procedures, two quarterly follow-up and function.5 This aesthetic concern observed in most cases of D.I.
visits were also scheduled. have a high correlation to negatively patient’s self-esteem, addi-
During treatment, due to the young age of the patient and the tionally damaging their interpersonal relationships, and is directly
need to control her behavior, the dental team opted for a medical linked to their quality of life, regardless of the age range in which
anesthesiologist to administer sedation for all restorative treatment the patient is included.4
appointments. Informed consent was obtained from parents of the Previously, the indicated treatment of dentinogenesis imperfecta
child for the treatment described. was to postpone the intervention until the beginning of the perma-
The first phase of the clinical treatment was performed in a nent dentition.4 This orientation, however, caused the need for major
dental office, with immobilization of the child by her mother, so rehabilitation. Delgado et al. cited that there is no formal protocol as
that the prophylaxis consultations and topical application of 5% for the ideal age for treatment, suggesting that a thorough analysis
fluoride varnish (Duraphat®, Colgate-Palmolive Ltda, São Paulo, of each case should be made, taking into account the stage of teeth
Brazil) could be performed (Figure 2). Subsequently, the patient wear.5 However, Bouvier et al, emphasized the need for treatment
was admitted in a hospital environment at the University Hospital

The Journal of Clinical Pediatric Dentistry Volume 41, Number 2/2017 113
Early Rehabilitation of Incisors with Dentinogenesis Imperfecta Type II – Case Report

Figure 1. Initial aspect of the patient, with wear in the anterior Figure 2. Application of fluoride varnish.
incisors.

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Figure 3. Reconstruction of tooth 51.

Figure 4. Restorations completed on the working model.

Figure 5. Appearance of completed composite resin

Figure 6. 32 months follow up

114 The Journal of Clinical Pediatric Dentistry Volume 41, Number 2/2017
 Early Rehabilitation of Incisors with Dentinogenesis Imperfecta Type II – Case Report

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teeth offered sufficient support, the primary maxillary incisors were 6. Leal CT, Martins LD, Verli FD, Souza MAL, Ramos-Jorge ML. Case report:
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to enamel and dentin in cases of D.I is questionable.8 There is the
9. Bektas O, Arica B, Teber S, Ylmaz A, Zeybek H,Kaymak S, Deda G. Chloral
assumption that there is no adequate formation of the hybrid layer hydrate and/or hydroxyzine for sedation in pediatric EEG recording. Brain
and this would compromise the quality and life span of restorations. & Development 36:130–136, 2014.
According to Leal et al., the decision for the composite resin tech- 10. Jain M, Singla , Bhushan BAK, Kumar S,Bhushan A. Esthetic rehabilita-
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In order for restorative procedures to be properly performed, it
was necessary to control the patient’s behavior using sedative agents.
A licensed medical anesthesiologist administered an initial oral dose
of midazolam, followed by a rectally administered dose of chloral
hydrate, substances that synergistically promote a longer working
time and more predictable sedative control of the patient. Both were
proven to be clinically safe for use on children and efficacious with
few side effects in case of short-term sedation.9 In the upper teeth,
the use of acetate crowns made the implementation of restorations
quicker and easier, considerably reducing clinical time, favoring the
sculpture of teeth and a good cosmetic result.10 In the mandibular
arch, due to the significant loss of structure, small size in the mesi-
al-distal direction and shape of the crowns, the restorations were
performed with composite resin, but through the indirect technique.
The patient’s mother was examined and also had dental charac-
teristics of dentinogenesis imperfecta. Since D.I. is a genetic muta-
tion, family members such as grandparents, uncles and cousins can
present phenotypic expression of this disease as well.2 Patients diag-
nosed with dentinogenesis imperfecta and their families should be
informed of the possibility of transferring this genetic abnormality
to future generations.5
After the fifth follow-up visit (thirty two months later), all
restorations remained intact, promoting function and aesthetic
maintenance, as well as, preventing further wear of tooth structure,
pulp damage and the other various complications. We emphasize
the importance of early treatment, which promotes a favorable
prognosis and the need for regular monitoring of the patient for the
maintenance of the results.

CONCLUSION
This case report is an example that correct diagnosis and early
treatment can provide dental function and esthetics in a very young
patient affected with dentinogenesis imperfecta, maintaining and
protecting the patient’s primary dentition with long term results.

The Journal of Clinical Pediatric Dentistry Volume 41, Number 2/2017 115