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J Am Dent Assoc, Vol 139, No 7, 906-914.
© 2008 American Dental Association
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CLINICAL PRACTICE

JADA Continuing Education

The Role of Dentists in Diagnosing Osteogenesis Imperfecta in Patients With Dentinogenesis Imperfecta

Cleonice Silveira Teixeira, DDS, MSc, Mara Cristina Santos Felippe, DDS, PhD, Wilson Tadeu Felippe, DDS, PhD, Yara Teresinha Corrêa Silva-Sousa, DDS, PhD and Manoel Damião Sousa-Neto, DDS, PhD


   ABSTRACT
TOP
 ABSTRACT
CASE REPORT
 DISCUSSION
 CONCLUSIONS
 REFERENCES
 
Background. Osteogenesis imperfecta (OI), also known as "brittle bone disease," can be difficult to diagnose in its mild form. The authors describe a clinical case of a diagnosis of dentinogenesis imperfecta (DI), in which a literature review combined with an analysis of dental alterations led to indications of OI involvement.

Case Description. Since DI can be associated with OI, the authors reviewed correlated studies and obtained a new medical history from the patient. They then conducted a radiographic and clinical examination of the dentition and submitted an affected third molar to scanning electron microscopy analysis. They compared their findings with descriptions of OI type I dental alterations in the literature and confirmed their diagnosis by means of a medical evaluation.

Clinical Implications. In cases in which DI is diagnosed, patients should be examined carefully and the occurrence of OI should be considered since, in its mild form, it might be misdiagnosed.

Key Words: Brittle bone disease; dentinogenesis imperfecta; diagnosis; osteogenesis imperfecta; scanning electron microscopy

Abbreviations: DEJ: Dentinoenamel junction • DI: Dentinogenesis imperfecta • EDTA: Ethylenedi-aminetetraacetic acid • OI: Osteogenesis imperfecta • SEM: Scanning electron microscopy

Dentinogenesis imperfecta (DI) is a hereditary disorder resulting in defective dentin formation in both primary and permanent teeth. It can be classified into three different forms: type I, in which the structural defects of the dentin are associated with osteogenesis imperfecta (OI); type II, which is the most common, is described as hereditary opalescent dentin and usually has with no association with any other syndrome; and type III, the first identified cases of which were in a population in Brandywine, Md.1 Although DI type I is a dental manifestation of the underlying type I collagen defect, DI types II and III are attributed to mutations affecting the dentin sialophosphoprotein gene.2,3

Teeth affected by DI have a characteristic discoloration that has been reported to be opalescent and gray, brown or yellow. The crowns have a bulbous appearance, with accentuated constriction in the cementoenamel junction. Roots are narrower than normal or corncob-shaped, and the pulp chamber and root canals can become partially or totally obliterated over time.1,4,5 The enamel of these teeth has normal characteristics; however, the enamel may shear readily from the dentin when subjected to occlusal stress due to the deficient dentinal support or abnormal scalloping of the dentinoenamel junction (DEJ).6–8 With the early loss of enamel, the exposed dentin can undergo rapid attrition and result in a loss of vertical dimension.5,9

OI, or brittle bone disease, is a genetic disorder that affects the connective tissue and is characterized by bone fragility and fractures owing to mutations in the genes that encode the chains of type I collagen, which is the major protein of bone, teeth, sclera and ligaments. It is classified on the basis of the incidence of fractures or on multiple clinical, genetic and radiographic features. Blue sclera, ligament fragility, long bone deformity, decrease in hearing capacity, growth deficiency and DI also can be related to OI.10,11 In the mild form of OI, patients generally have a normal stature, low incidence of fractures and little deformity of long bones, which are characteristics that can hamper diagnosis if the patient does not have a family history of the disorder.12–14

Dentinogenesis imperfecta is more evident in the primary teeth than in the permanent teeth of patients with osteogenesis imperfecta.

Sillence and colleagues10 described a classification based on radiographic, genetic and clinical criteria to classify types I through IV. They proposed that OI type I includes patients who have the mild form, almost normal stature and blue sclera. Type II is considered the most severe form and is lethal in the perinatal period. Type III includes patients with the classic disease manifestation, usually with moderate deformity at birth and progressively deforming bones. Type IV includes patients with extensive phenotypic variability, including mild to severe forms of OI. All of the types described can be subdivided according to the absence or presence of DI.15 Moreover, OI type IV has been subdivided into types IV, V, VI and VII according to distinct clinical and bone histology features.16–20

When DI is associated with OI, there may be several degrees of dental involvement, regardless of the type and severity of OI. DI has been reported in approximately 50 percent of OI cases,21 and it is more evident in the primary teeth than in the permanent teeth of patients with OI.11,22 DI is less severe in permanent dentition and, in some cases, an unnoticed anomaly.6,11 Therefore, if DI is confirmed, the patient should be examined carefully, and the occurrence of OI should be considered.

In this article, we describe a clinical case of DI that initially was diagnosed as hereditary opalescent dentin (DI type II). However, after we conducted a critical review of the literature and clinically, radiographically and microscopically analyzed the permanent dentition, we changed the diagnosis to DI associated with OI.


   CASE REPORT
TOP
 ABSTRACT
 CASE REPORT
DISCUSSION
 CONCLUSIONS
 REFERENCES
 
Analysis of primary dentition. An 11-month-old white girl was referred to a dentist (C.S.T.) for evaluation because her mother was concerned with the yellow-brown discoloration of her mandibular incisors. The patient appeared to be healthy and had normal neuromotor development. While taking the patient’s medical history, the dentist verified that the patient’s parents had normal dentition and no other relative had the same problem. Despite this information, the dentist diagnosed hereditary opalescent dentin (DI type II) and referred the patient to an odontopediatric clinic so that a specialist could follow her progress during her growth. The primary dentition displayed yellow-brown discoloration on all tooth surfaces, and enamel fractures, dentin exposure and attrition started when the patient was three years of age. The dentist placed resin-based composite restorations to protect the fractured areas and maintain the vertical dimension. However, when the patient was almost six years of age, she lost about one-third of the crown length owing to excessive attrition after enamel fracturing and carious lesions. The dentist observed pulp tissue exposure in the posterior teeth, even after the patient underwent several restorative procedures to prevent the progression of caries.

Clinical and radiographic analysis of permanent dentition. After primary teeth exfoliation and eruption of the permanent dentition, the patient’s esthetic situation was improved considerably. We applied a sealant to the occlusal fissures of the posterior teeth. When we performed a clinical examination when the patient was 15 years old, we found that the permanent teeth had an opalescent gray discoloration (Figure 1AGo) and seemingly were affected less than the primary dentition was. At that time, all teeth responded positively to the pulp sensitivity test.


Figure 1
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  		Figure 1. Clinical aspect of permanent teeth in a patient aged 15 years. A. Permanent teeth minimally affected with opalescent gray discoloration. Bilateral posterior crossbite is evident. B. Maxillary incisors with color close to normal; incisal margins of mandibular incisors restored because of fracture and wear (arrows). C, D. Note teeth with a gray cervical line and opalescent discoloration in areas of low enamel thickness (arrows).

 
The mandibular incisors were the most affected teeth, and the maxillary anterior teeth were the least affected (Figure 1BGo). The other teeth had only a gray cervical line and opalescent discoloration, mainly in areas of low enamel thickness (Figures 1C and DGo). We restored the incisal margins of the mandibular incisors several times owing to fracture of the enamel and dentin attrition (Figure 1BGo).

Radiographic examination (Figure 2Go) revealed crowns with a bulbous aspect and constriction in the cervical areas of the teeth. The roots, despite their normal length, were corncob-shaped, and the pulp chamber had been obliterated (Figure 2Go). We observed a supernumerary tooth in the left mandibular premolar area of the jaw (Figure 2EGo). Regarding the occlusion, we observed a bilateral posterior crossbite (Figure 1AGo) and impacted maxillary third molars (Figures 2A and 2BGo).


Figure 2
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  		Figure 2. Aspect of permanent teeth in radiographic examination of a patient aged 15 years. A–D. Note the bulbous crowns with marked cervical constriction. The roots are corncob-shaped and have pulp chamber obliteration. E. A supernumerary tooth is visible.

 
When the patient was 15 years old, the dentist (C.S.T.) took a new medical history and found that the patient had experienced 11 bone fractures that were distributed throughout her arms, hands, foot and trunk during childhood and the prepubertal period but that the fractures decreased considerably once the patient reached puberty.

Scanning electron microscopy (SEM) analysis. One of the extracted maxillary third molars was prepared and analyzed by means of SEM. We used a sound third molar extracted for orthodontic purposes from a patient who was not affected by DI and who had the same development degree.

We sectioned the teeth in the vestibular-lingual direction. We cleaned one of the sections with 17 percent ethylenediaminetetraacetic acid (EDTA) solution for one minute to remove the smear layer formed during the sectioning procedure. We took care not to apply acid inside the pulp chamber. After washing the specimens with deionized water for one minute, we immersed them in 5 percent sodium hypochlorite solution for two minutes. After we completed the fixation and dehydration processes, we sputter-coated the samples with gold and analyzed them by means of SEM.

Results of SEM analysis. Results of our SEM analysis are shown in Figures 3Go, 4Go and 5Go (pages 910, 911 and 912, respectively). SEM analysis of the DI-affected tooth showed circular spaces in the circumpulpal dentin (Figure 3AGo). At an increased magnification, we could see the globular aspect of the dentin surrounding the circular spaces (Figure 3BGo). The diameters of these spaces were between 70 and 100 micrometers (Figure 3CGo). At an increased magnification, we could see that the dentinal tubule openings had a reduced diameter (Figure 3DGo).


Figure 3
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  		Figure 3. Photomicrographs of third molar affected by dentinogenesis imperfecta (x15 magnification). A. Note circular spaces in circum-pulpal dentin. B. The globular aspect of dentin is visible (x500 magnification). C. The diameters of the circular spaces were between 70 and 100 micrometers (x100 magnification). D. Increased magnification (x500 magnification) showed that the dentinal tubule openings had a reduced diameter.

 

Figure 4
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  		Figure 4. Photomicrographs of dentinoenamel junction decalcified with 17 percent ethylenediaminetetraacetic acid (x500magnification). The dentinoenamel junction and dentinal structure below the enamel limit of the molar affected by dentinogenesis imperfecta (A) were similar to those in the control molar (B).

 

Figure 5
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  		Figure 5. Photomicrographs of dentin areas in pulp chamber after treatment with 5 percent sodium hypochlorite solution. A–D. Structural differences in the diameter and number of the dentinal tubules of control (A, B) and affected (C, D) dentin. A. Control dentin with typical calcospherite aspects (x250 magnification). B. Visible open tubules (x1,000 magnification). C. Affected dentin with wide circular spaces (arrows) surrounded by abnormally constituted matrix (x250 magnification). D. Dentinal tubules low in number and caliber (x1,000 magnification).

 
In the specimens we decalcified by using 17 percent EDTA solution (Figure 4Go), we could see the DEJ in the molar affected by DI (Figure 4AGo) and the control molar (Figure 4BGo). The DEJ and dentinal structure below the enamel limit of the affected molar (Figure 4AGo) were similar to those in the control molar (Figure 4BGo).

When we compared the circumpulpal dentin of the control (Figures 5A and 5BGo) and DI-affected (Figures 5C and 5DGo) molars, we found structural differences in the diameter and number of dentinal tubules. The control dentin had typical calcospherite aspects (Figure 5AGo) with a great number of visible and open tubules (Figure 5BGo). However, we observed wide circular spaces in the affected dentin surrounded by an abnormally constituted dentin matrix (Figure 5CGo). At higher magnification, we saw that the dentinal tubules were low in number and caliber (Figure 5DGo) when compared with those in the control molar (Figure 5BGo).

OI diagnosis. Despite the fact that the patient frequently visited orthopedic clinics and a general practitioner, the diagnosis of OI had not yet been established. Our careful analysis of the clinical, radiographic and SEM evidence of permanent dentition led to the diagnosis of DI associated with OI type I. We referred the patient to the endocrine and orthopedic departments of the University Hospital of the Federal University of Santa Catarina (Florianópolis, SC, Brazil) for a general evaluation, and a diagnosis of type I OI was confirmed.

At 18 years of age, the patient had a relatively stable clinical pattern (Figure 6Go, page 913). We continued to observe the pulp obliteration process radiographically (Figures 6B and 6CGo), and the mandibular incisors were more yellow-brown than they were when the patient was 15 years of age (Figure 6AGo). New bone fracturing due to mild trauma was reported, emphasizing the bone fragility and diagnosis of OI type I. This patient is under observation and receives instructions from her dentist and orthopedic physician about the necessity of daily care.


Figure 6
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  		Figure 6. Clinical and radiographic images of permanent teeth in a patient aged 18 years. A. Note the yellow-brown discoloration of the mandibular incisors. Note the pulp obliteration in erupted teeth (B) and root formation in the supernumerary tooth with wide pulp chamber (C).

 

   DISCUSSION
TOP
 ABSTRACT
 CASE REPORT
 DISCUSSION
CONCLUSIONS
 REFERENCES
 
Because there was no family history of DI in the clinical case we reported, we initially considered the patient’s DI to be a new genetic mutation, and we did not consider the possibility of an association with OI. OI is characterized by bone fragility and is caused by gene mutations related to the production of collagen type I. A reduction in the quantity of a structurally normal collagen results in OI type I, whereas qualitative and quantitative alterations in the collagen synthesis result in OI types II, III and IV.11,23 When associated with OI, DI type I has a recessive behavior, and new cases of genetic mutations commonly are found.1,5,23,24 Nevertheless, we initially discarded the DI type I hypothesis because the patient did not show, at that time, clinical characteristics of this disorder (for example, blue sclera, low stature, hearing loss, bone fragility and delay in neuromotor development).

Although the clinical and radiographic evidence pointed to a diagnosis of DI type II, other findings suggested the hypothesis of an association with OI. For example, some primary molars did not have intrapulpal calcification but had pulp exposure after carious lesions due to coronary fractures and abrasive processes. In cases of DI combined with OI, carious lesions seem to inhibit the obliteration process of the pulp chamber, resulting in enlargement of the canal lumen, which could cause pulp tissue exposure.25

Another dental finding was that the permanent dentition seemed less affected than the primary dentition, with a low degree of esthetic involvement. Moreover, the patient had deficient occlusion with bilateral crossbite (Figure 1AGo). These clinical observations are similar to those described by O’Connell and Marini.11 They found that, in patients with DI, tooth discoloration and attrition did not occur in permanent dentition to the same extent as in primary dentition. The yellow-brown discoloration seemed to be related to the highest gravity of DI, whereas the opalescent gray discoloration had a better prognosis. Occlusion disorders were verified in most of the patients who had a high incidence of anterior and posterior open bite and crossbite.

The low incidence of caries in the permanent dentition observed in the case we reported was important for dental structure maintenance. According to previous reports, teeth affected by DI were not more susceptible to carious lesions than were normal teeth.11,26 When the teeth affected by DI also are affected by caries, the caries progression is slow owing to the smaller amount and irregular nature of the dentinal tubules or fast abrasion of the exposed dentin. This fast attrition can be explained by hypomineralization and, consequently, reduced dentin microhardness.27

In the case we reported, the continuous dentin deposition, mainly in the first permanent molars, almost obliterated the pulp chamber; however, all of the teeth responded positively to cold stimuli. The lower density and higher water content of the dentin affected by DI may explain the response to stimuli in cases of pulp obliteration.5

Structural analysis by means of SEM of the molar affected by DI showed dentinal alterations compared with the control molar (Figures 3Go–5GoGo). The use of teeth with incomplete root formation allowed us to make a detailed evaluation of the predentin. A comparison of the calcospherites in the affected and control dentin showed dentinal tubules with reduced caliber in the case of DI. The enamel structure appeared not to be affected, and we observed no differences in the DEJs of the control and affected molars (Figures 4A and 4BGo), as also was described in other studies.8,28

The circular spaces we observed in the circumpulpal dentin of the molar affected by DI (Figure 3Go) may be caused by the fast and incorrect deposition of the dentinal tissue in this area and may be related to the inclusion of blood vessels. Lindau and colleagues8 also observed a lower number of dentinal tubules with smaller diameters in teeth with DI. Moreover, they reported circular holes filled with organic material, similar to blood vessel–like inclusions.

During the patient’s follow-up, the pulp chamber and root canals underwent progressive obliteration. The permanent teeth became more opaque, with a yellow-brown discoloration mainly on the mandibular incisors, which may require esthetic procedures in the future. Nonetheless, a patient with DI should receive professional care and instructions to avoid enamel loss, since it protects dentin from attrition and, thus, obviates a reduction in the vertical dimension, as well as decreases the risk of developing periapical diseases.

In recent years, the development of new restorative techniques and adhesive systems has improved the treatment of teeth with structural anomalies.4,14,29,30 For the patient in our case report, we were able to maintain esthetics and occlusion with resin-based composite restorations because of her age at the beginning of the treatment, because we frequently examined the patient, and because the permanent teeth had more favorable esthetic conditions than did the primary teeth. Frequent dental appointments in which dentists provide specific instructions regarding patients’ diets such as avoiding hard foods, as well as frequent prophylaxis and applications of topical fluoride, help prevent carious lesions in adulthood.

The patient in the case we reported had fractured at least 11 bones by the time she was 15 years old. Currently, her clinical signs of OI are barely evident and do not adversely affect her general health. Identifying the disorder and its correct treatment aided in several ways, helping the patient avoid bone fractures and preventing osteoporosis.

Although fragility fractures occur frequently in children with OI, the fracture rate decreases after adolescence owing to the influences of sex hormones and maturation.31 Conversely, the hormone level reduction that occurs during menopause can aggravate the clinical manifestations of OI.12 Several therapies have been proposed to reinforce bone structure and reduce OI symptoms. These treatments reduce bone resorption through bisphosphonate therapy, stimulate bone formation with growth hormones or both.32–35 Medical therapies that used bisphosphonates reduced fracture risk and bone pain, mainly in children with severe types of OI.13 A nitrogen-containing bisphosphonate such as alendronate generally is accepted as a safe, effective and well-tolerated treatment for postmenopausal osteoporosis and has been used in adult patients with OI.31,36

Early diagnosis of OI is important because the genetic disturbance can have a dominant auto-somal character, which can affect patients’ descendants with great variability in the severity of how it manifests clinically.11,23 Diagnosing OI can be difficult if no family members are affected, especially when it has discreet manifestations and when bone fragility is not associated with extraskeletal abnormalities.19 There have been case reports of OI that was first diagnosed as osteoporosis.12,14,31 Although DI dental alterations occur in only 50 percent of OI cases, they often are the most visible clinical manifestation of OI,11 and, even in cases in which permanent teeth were barely affected by DI, the pulp was radiographically or microscopically altered.7,28,37 Furthermore, the obliteration of the pulp chamber and root canals should alert dental professionals to the possibility of OI. Frequent observation allows for endodontic intervention when necessary while the patient is young and the canals still are accessible. When endodontic procedures have not been performed at the right time, the dental prognosis has been affected.5


   CONCLUSIONS
TOP
 ABSTRACT
 CASE REPORT
 DISCUSSION
 CONCLUSIONS
REFERENCES
 
In the case we have reported here, early and appropriate dental care led to improved control of oral disease, function and esthetics. Also, this case report emphasized the importance of making a correct diagnosis to associate DI with OI. In patients with mild forms of DI, a careful clinical and radiographic analysis may be needed to accurately diagnose OI. Therefore, when dental professionals are verifying DI, they should take a detailed medical history and be aware of the potential for OI involvement.


   FOOTNOTES
 

Dr. Teixeira is a doctoral student, School of Dentistry, University of Ribeirão Preto, Ribeirão Preto, São Paulo, Brazil, and a professor, Dental School, Federal University of Santa Catarina, Florianópolis, SC, Brazil.


Dr. Mara Cristina Santos Felippe is an adjunct professor, Dental School, Federal University of Santa Catarina, Florianópolis, SC, Brazil.


Dr. Wilson Tadeu Felippe is an adjunct professor, Dental School, Federal University of Santa Catarina, Florianópolis, SC, Brazil.


Dr. Silva-Sousa is the dean, Postgraduate Endodontics Program, and titular professor, School of Dentistry, University of Ribeirão Preto, São Paulo, Brazil.


Dr. Sousa-Neto is an associate professor, Restorative Dentistry Department, School of Dentistry, University of São Paulo, Rua Célia de Oliveira Meirelles 350, Ribeirão Preto, SP, 14024-070, Brazil, e-mail "sousanet{at}forp.usp.br". Address reprint requests to Dr. Sousa-Neto.


Disclosure. None of the authors reported any disclosures.


   REFERENCES
TOP
 ABSTRACT
 CASE REPORT
 DISCUSSION
 CONCLUSIONS
 REFERENCES
 

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