Dentinogenesis Imperfecta Type II: Case Report

Alessandra Castro ALVES
Alexandre Rezende VIEIRA
Faculdade de Odontologia, Universidade Federal do Rio de Janeiro Rio de Janeiro, RJ, Brasil

Braz Dent J (1996) 7(1): 47-52 ISSN 0103-6440

| Introduction | Review of the Literature | Case Report | Discussion | References |

A case of dentinogenesis imperfecta type II is described. The authors also present a brief literature review and focus on the difficulty in treating such cases.

Key Words: dentinogenesis, dentinogenesis imperfecta.


Significant hereditary anomalies have been diagnosed over the years, among which dentinogenesis imperfecta (DI) stands out. DI is described as a localized form of mesodermal dysplasia, observed in histodifferentiation, and which corresponds to a congenital hereditary change, involving deciduous and permanent teeth (Nayar et al., 1981).

One of the great challenges to pediatric dentists when facing such an anomaly is adequate treatment to achieve functional and esthetic restoration (Posnick, 1976). Early diagnosis and treatment are therefore fundamental, aiming at obtaining a favorable prognosis, since late intervention makes treatment even more complex.

The objective of this paper is to present a case of DI Type II, dealing with its different aspects, its synonyms, classification, clinical and radiographic characteristics, and possible treatment.

Review of the Literature

Dentinogenesis imperfecta has been reported using several different names, the most common of which are hereditary opalescent dentin, owing to the refractory property of the dentin, and odontogenesis imperfecta (Roberts and Schour, 1939; Finn, 1976; Shafer et al., 1985). The variability of expression of the various cases of DI has made it clear that there are apparently several forms of the disease. In order to distinguish among them, Shields et al. (1973) have suggested a classification into three types: type I, which occurs in families with osteogenesis imperfecta, segregated with an autosomal dominant trait, with variable expression, and which may be recessive; type II, the most common, which is not associated to osteogenesis imperfecta and which is an autosomal dominant disorder; and type III, the rarest, corresponding to the Brandywine type, which occurs in a racial segregate in Maryland, USA, and which is also an autosomal dominant disorder.

In the clinical picture of DI type II, primary and secondary dentitions have a typical amberlike translucency or opalescence against reflected light, and can vary from greyish purple to purple-brown or yellowish-brown (Roberts and Schour, 1939; Hursey Jr. et al., 1956; Bixler et al., 1969; Stafne and Gibilisco, 1982; Shafer et al., 1985; Guedes-Pinto, 1988). After the primary dentition is complete, the enamel tends to chip away from the incisal rim of the anterior teeth and from the occlusal surface of posterior teeth. The exposed dentin, which is soft, undergoes quick abrasion, to such an extent that the dentinal surface occasionally becomes smooth and continuous with the gingival tissue. The permanent dentition, on the other hand, which is of better quality is not destroyed to such an extent, and can have a normal clinical appearance (Roberts and Shour, 1936; Hursey et al., 1956; Stafne and Gibilisco, 1982; Shafer et al., 1985; Guedes-Pinto, 1988; Gage et al., 1991; McDonald and Avery, 1994).

According to Shafer et al. (1985), DI type II presents an unusual pathognomonic radiographic aspect. Bell-shaped bulbous crowns and short, thin, blunt roots are present, with normal enamel, cementum, pericementum, peridontium and alveolar bone (Hursey Jr. et al., 1956; Bixler et al., 1969; Gage et al., 1991; McDonald and Avery, 1994). Other authors (Finn, 1976; Stafne and Gibilisco, 1982; Shafer et al., 1985; Guedes-Pinto, 1988; McDonald and Avery, 1994) report that the most remarkable characteristic of DI Type II is the complete or partial obliteration of the pulp and root canals owing to the continuous formation of dentin, with occasional periapical rarefaction of deciduous teeth, with the absence of pulpal exposure or pulpal necrosis.

Several types of treatment of DI have been described in the literature (Shafer et al., 1985; Gage et al., 1991; McDonald and Avery, 1994), changing according to each case. Steel crowns, employed as a preventive device for the abrasion of the dental structure, can be used in deciduous and young permanent posterior teeth which do not require esthetics. According to Wei (1988) such a procedure must be undertaken as soon as the tooth erupts. When dealing with deciduous and young permanent anterior teeth, celluloid crowns are recommended; permanent molars should receive full cast crowns; metallo-ceramic restorations are recommended for pre-molars, and permanent anterior teeth should be restored with esthetic facets (McDonald and Avery, 1994).

Shafer et al. (1985) emphasize that restorations cannot be permanent, owing to the low hardness of the dentin. Consequently, when fractures occur at the gingival level or below the gum, exodontia is indicated, as in the case of teeth which exhibit periapical rarefaction and root fracture (Wei, 1988; McDonald and Avery, 1994). Should patients need a removable, partial or full prosthesis, care should be taken in preparing the teeth and in using the prosthesis, since the pressure it exerts on the teeth may cause the roots to fracture easily (Wei, 1988).

Case Report

A 3-year old white female was examined at the School of Dentistry of the Federal University of Rio de Janeiro with the complaint of esthetic dissatisfaction. Her medical history revealed allergic bronchitis and the common childhood diseases.

Upon examination, the face and soft tissue were normal, showing a Baume arch type I and deciduous second molars with straight terminal plane. There were no caries but brownish-yellow teeth were present, with an acute loss of enamel at the incisal and occlusal edges, and loss of the vertical dimension (Figure 1). The exposed dentin was hardened. The family history revealed that the condition was present in the mother, grandmother, 3 great-aunts, great-grandfather, 4 uncles and 7 maternal cousins, with no relation to osteogenesis imperfecta, which led to a diagnosis of DI type II.

Radiographs showed deciduous teeth with bulbous crowns, short, thin, blunt roots, with normal enamel, cementum, pericementum, periodontium and alveolar bone associated to a partial obliteration of the pulpal chamber and root canals. The germs of permanent teeth exhibited abnormal development, suggesting DI.

After establishing the treatment plan and the basic preventive procedures (instruc-tion in oral hygiene and rational use of fluorides) restorations were carried out with stainless steel crowns in all posterior teeth, and celluloid crowns in the superior and inferior incisors and superior canines, while the need to use behavioral control techniques was emphasized throughout the treatment (Figure 2).

The patient was examined at three-month intervals for re-evaluation of the restorations, to carry out preventive procedures and to monitor the growth and development of the dentition, the efficacy of the treatment under-taken being confirmed, as well as the normality of the biogenesis of the dentition (Figure 3).

Figure 1 - Intra-oral view showing serious loss of enamel and of the vertical dimension.

Figure 2 - A, Clinical aspect with celluloid crowns on superior and inferior incisors. B, Panoramic radiograph showing oral rehabilitation of the superior and inferior arches.

Figure 3 - Clinical control of the superior and inferior arches three years after rehabilitation, showing the efficacy of the treatment and normal development of the dentition.


DI was first reported in 1887 when the case of an 18-year-old boy was described who had dark brown stains on his teeth, which had worn to the gum line (Nayar et al., 1981). Roberts (1935) reported a family in which the deciduous and permanent teeth of certain members were soft and wore away rapidly. Roberts and Schour (1939) traced a family history in which 22 of its 40 members were affected. In the clinical case under study, DI occurred in a large number of the family members (18), which corroborates studies cited previously (Roberts, 1935; Roberts and Schour, 1939; Nayar et al., 1981).

Finn (1976) emphasizes that the large destruction of the crowns of involved teeth simulates cases of rampant caries. Finn (1976), Nayar et al. (1981) and Hodge et al. (1936) attribute the fracture of the enamel to the deficient support offered by the abnormal dentin and to the lack of the typical scalloping of the dentino-enamel junction, which helps the union mechanically.

McDonald and Avery (1994) state that caries have been observed in teeth affected by DI. However, the carious process seems to develop slowly and to be influenced by the abrasion of the dentinal surface. According to Finn (1976), Hursey Jr. et al. (1956) and Shafer et al. (1985), teeth affected by DI do not seem to be more susceptible to caries than normal teeth, and can exhibit some resistance, owing to the absence of dentinal tubules and to the inability of caries to develop in a surface where enamel is rapidly being lost to abrasion and fracture. The case presented here confirms this with its absence of carious lesions.

Treatment of DI is very difficult (McDonald and Avery, 1994). Its main purpose is to prevent loss of enamel and dentin to abrasion (Roberts and Schour, 1939; Shafer et al., 1985). Treatment of the case reported here was very difficult, not only because of the complexity of the situation associated with the presence of an abnormal dentition with fracture of the enamel, but also owing to the young age of the patient, and to her lack of cooperation during treatment.

Permanent teeth, in general, seem to be of better quality, and suffer less destruction. Sometimes, they have a normal clinical aspect (Komorowska et al., 1989; McDonald and Avery, 1994). In the case described, permanent incisors and first molars erupted with normal characteristics (Figure 3).

The authors provided adequate treatment, with a preventive approach to establish an esthetic result and restore function. Vertical dimension, which is frequently lost in such patients, and an efficient stomatognathic system were restored aiming at establishing a more favorable prognosis for such a complex anomaly, and to insure the integrity of the erupting dentition.


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Correspondence: Adriana Modesto, Faculdade de Odontologia, Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, Brasil.

Accepted October 17, 1995
Electronic publication: September, 1996