Year : 2008 | Volume
: 26 | Issue : 2 | Page : 85--87
Dentinogenesis imperfecta: A case report
P Subramaniam, S Mathew, SN Sugnani
Department of Pedodontics, The Oxford Dental College and Hospital, Bangalore, India
S N Sugnani
Department of Pedodontics, The Oxford Dental College and Hospital, Bangalore
Dentinogenesis imperfecta is an autosomal dominant disorder of tooth development characterized by the presence of opalescent dentin, resulting in a dusky blue to brownish discoloration of the teeth. This condition is genetically and clinically heterogeneous; it may affect only the teeth or it may be associated with the osteogenesis imperfecta. Dentinogenesis imperfecta has been subdivided into three types: type I is associated with osteogenesis imperfecta; in type II there is no associated osteogenesis imperfecta; and when the condition is associated with the Brandywine triracial isolate and large pulp chambers it is classified as type III.
This report describes a 16-year-old female patient who showed the characteristic dental features of dentinogenesis imperfecta type II. The etiology and prevalence of the disorder, and a comprehensive treatment plan, will be briefly reviewed.
|How to cite this article:|
Subramaniam P, Mathew S, Sugnani S N. Dentinogenesis imperfecta: A case report.J Indian Soc Pedod Prev Dent 2008;26:85-87
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Subramaniam P, Mathew S, Sugnani S N. Dentinogenesis imperfecta: A case report. J Indian Soc Pedod Prev Dent [serial online] 2008 [cited 2020 Oct 31 ];26:85-87
Available from: https://www.jisppd.com/text.asp?2008/26/2/85/41624
Human dentition is subject to considerable variations in size, form, and number of teeth as well as in the structure of the dental tissues. Disorders of the teeth may be hereditory. Dentinogenesis imperfecta is one such disorder. 
It was probably first recognized by Barret in 1882. The first published report describing the disorder as an enamel defect was by Talbot as quoted by Witkop.  The term 'hereditary opalescent dentin' was first used by Skillen,  Finn  and Hodges  to describe the brown translucent teeth that have an opalescent sheen and are lacking in pulp chambers. Some authors suggest that the term 'hereditary opalescent dentin' should be preferred over 'dentinogenesis imperfecta' as it describes the general appearance better.
Dentinogenesis imperfecta is a localized mesodermal dysplasia affecting both the primary and permanent dentitions. The disease is inherited in an autosomal dominant fashion with high penetrance and a low mutation rate.  It is the most common dental genetic disease, affecting approximately 1 in 8000 births.  Witkop reported that it is the most common autosomal dominant disease affecting westerners. 
Clinically, both dentitions are affected. The color of the teeth varies from brown to blue, and is sometimes described as amber or gray. The enamel may show hypoplastic or hypocalcified defects in about one-third of the patients and in an affected person tends to crack away from the defective dentin. The exposed dentin may then undergo severe and rapid attrition. 
Radiographically, the teeth have bulbous crowns and constricted, short roots. Initially, the pulp chambers may be abnormally wide, giving the appearance of 'shell teeth', but they progressively get obliterated. 
Histologically the dentin is composed of irregular tubules, often with large areas of uncalcified matrix. The tubules tend to be larger in diameter and less numerous in a given volume of dentin than in normal teeth. 
A 16-year-old female patient reported to the Department of Pedodontics of the Oxford College and Hospital, Bangalore, India, with complaints of brown discolored teeth and the wearing away of all her teeth and hence desired to have them replaced by prosthesis.
She gave a history of similar discoloration and wearing away of her milk teeth, which had exfoliated uneventfully. Her parents had noticed that her permanent teeth were delayed in their eruption and were brown in color at the time of eruption. The patient also complained of having intermittent pain in the lower posterior tooth region since 1 month. She had visited a general hospital 4 years back with the complaint of having discolored teeth and had received root canal treatment of the upper central incisors and the lower incisors. During that period, she was diagnosed with trivial mitral and tricuspid regurgitation. Her family history revealed that she has two other siblings, an 18-year-old sister and a 12-year-old brother, both with similar dental problems. Her parents had had a consanguineous marriage.
Clinical examination revealed gingival recession in relation to 31 and 41. All teeth were yellowish brown in color and small in size. All teeth also showed attrition, with complete loss of enamel in most [Figure 1],[Figure 2]. The coronal height of the teeth was reduced to one-third of the normal. Pulpal exposures were seen in the maxillary anteriors; the mandibular left canine and maxillary central incisors and molars showed pulp polyps.
The patient's orthopantamogram and intraoral periapical radiographs revealed periapical unilocular radiolucencies in 34 (2 mm in diameter) and 46 (5 mm in diameter), with well-defined sclerotic borders; there were periapical unilocular radiolucencies in relation to 16, 36, 33, 43, and 44 (around 2 mm in diameter), with the borders merging with the surrounding cortical bone. The upper central incisors showed resorption of roots of up to half their lengths. Open apices were seen in the premolar teeth, and 18, 27, and 28 were missing. Short bulbous roots and radiopacities in the pulp chambers of a few teeth were also seen.
After a cardiologist had declared her fit to undergo the procedure, she was given appropriate antibiotic prophylaxis and was treated under general anesthesia. Treatment included cyst enucleations in 34 and 46 and extractions of 11, 12, 16, 21, 64, 33, 34, 36, 43, 44, and 46. Histologic examination revealed nonspecific chronic inflammatory changes. The rest of the treatment was given after 1 week, and included root canal treatment in the upper canines and left lateral incisor teeth, apexification in the upper right premolar, and placement of stainless steel crowns for all erupted molars and polycarbonate crowns for the premolars; metal-reinforced jacket crowns were cemented in place in the upper and lower anterior teeth following post and core preparations in all these teeth. Removable partial dentures were given in the upper and lower jaws for the remaining edentulous areas [Figure 3],[Figure 4],[Figure 5],[Figure 6].
The treatment rendered to the patient caused her no discomfort and she was pleased with the esthetic result as well as with the improvement in her ability to bite and chew.
The patient was advised to come for regular follow-up. Once her growth is complete, we plan to give more definitive treatment for all teeth with open apices.
One of the greatest challenges for the pediatric dentist is to provide adequate treatment to achieve functional and esthetic restoration in cases of diseases like dentinogenesis imperfecta. Early diagnosis and treatment are essential for obtaining a favorable prognosis, any delay in intervention making the treatment even more complex. 
Many treatment modalities have been suggested, eg, over-dentures, stainless steel crowns, jacket crowns, pin-retained cast-gold 'thimbles' under acrylic resin crowns, stainless steel crowns with acrylic facing, and simple removable appliances.  The use of a combination of partial dentures and prosthetic crowns on the anterior teeth has also been described. Orthodontic treatment has been successfully performed in patients with different degrees of dentinogenesis imperfecta. 
Steel crowns used to prevent attrition of the dental structure can be used in deciduous teeth and in young permanent posterior teeth, where esthetics is not an issue. According to Wei such a procedure must be undertaken as soon as the tooth erupts. 
Shafer et al . emphasize that restorations cannot be permanent owing to the diminished hardness of dentin.  Consequently, when fractures occur at the gingival level or below the gum, exodontia is indicated, as in the case of teeth that exhibit periapical rarefaction and root fracture. ,
When dealing with deciduous and young permanent anterior teeth, celluloid crowns are recommended; permanent molars should receive full cast crowns; metalloceramic restorations are recommended for premolars; and permanent anterior teeth should be restored with esthetic facets. 
In this case, the authors aimed at providing treatment, along with preventive measures, to achieve a good esthetic result and restore function. An efficient stomatognathic system was restored aiming at establishing a more favorable prognosis for such a complex anomaly.
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