Dentin dysplasia is a rare inherited autosomal dominant disorder characterized by rootless teeth. We hereby report a case of dentin dysplasia type 1b with typical radiographic findings: short and blunt roots, pulpal obliteration, horizontal/crescent shaped radiolucencies in pulp chambers, and multiple periapical radiolucencies. However, the present case did not show the autosomal dominant pattern of inheritance and the patient also exhibited concurrent dental fluorosis, transposition of 13 and 14, and multiple cusps in maxillary first molars. Moreover, on careful review of previously documented cases of radiographs of dentin dysplasia, the horizontal/crescent shaped radiolucencies in pulp chambers are a rare finding, which is characteristically seen in the present case.
Dentin dysplasia (DD) is an autosomal dominant hereditary disturbance in dentin formation, which may present with either mobile teeth or pain associated with spontaneous dental abscesses or cysts. It is a rare anomaly of unknown etiology that affects approximately one patient in every 100,000 [
The present case is dentin dysplasia type 1b with typical radiographic findings: short and blunt roots, pulpal obliteration, horizontal/crescent shaped radiolucencies in pulp chambers, and multiple periapical radiolucencies.
Though dentin dysplasia is transmitted as autosomal dominant disorder, the present case did not show the autosomal dominant pattern of inheritance and the patient also exhibited concurrent dental fluorosis, transposition of 13 and 14, and multiple cusps in maxillary first molars. Moreover, on careful review of previously documented cases of radiographs of dentin dysplasia, the horizontal/crescent shaped radiolucencies in pulp chambers are a rare finding, which is characteristically seen in the present case.
A 16-year-old male patient presented with a chief complaint of partial edentulousness due to early shedding of teeth. Patient did not give any history of trauma. Extraoral examination did not reveal any abnormality. On intraoral examination, teeth present were 17 16 15 13 14 22 23 24 25 26 27 47 46 45 43 33 34 35 36 37
The majority of the teeth exhibited grade I mobility except for 35 which had grade II mobility. The crowns of teeth showed brownish yellow discoloration with white chalky areas, corresponding to dental fluorosis. The maxillary first molars showed multiple cusps and there was transposition of 13 and 14 (Figure
Intraoral photograph showing transposition between 13 and 14 and multiple cusps in occlusal surface of the 1st maxillary molars (black arrows).
Cropped panoramic radiograph showing generalized short and tapered roots, pulpal obliteration, horizontal/crescent shaped pulpal remnants in pulp chambers, and multiple periapical radiolucencies (white arrows).
Intraoral periapical radiographs showing horizontal/crescent shaped pulpal remnants in pulp chambers (black arrows).
Dentin dysplasia is a rare disorder which is thought to arise due to abnormal differentiation or function of the ectomesenchymally derived odontoblasts [
Dentin dysplasia is inherited as an autosomal dominant trait. However, in this case none of the family members exhibited radiographic features of dentin dysplasia. This indicated that the patient is a first generation sufferer. Such similar incidence was also reported by Toomarian et al. [
Carroll et al. [
The present case exhibited classical radiographic features of type 1b. Though the previous literature has documented considerable number of cases of dentin dysplasia type 1, very few of them had successfully demonstrated the horizontal/crescent shaped pulp in their radiographs. Our case clearly demonstrates this feature in addition to other radiographic findings like short roots, pulpal obliterations, and multiple periapical radiolucencies.
The present case also showed other associated clinical findings like dental fluorosis, transposition of 13 and 14, and multiple cusps in maxillary first molars. Dental fluorosis in the present case is attributed to the fact that the patient belonged to the geographical area which is endemic to fluorosis.
Management of dentin dysplasia has always been a challenging task to the dentists. Followup and routine conservative treatment are often advised. Maintenance of periodontal health through adherence to oral hygiene instructions is vital to the survival of mobile teeth. Endodontic treatment is contraindicated in teeth with total obliteration of root canals and pulp chambers [
In conclusion, dentin dysplasia is a rare genetic disorder. The present case exhibited characteristic radiographic findings along with other associated clinical findings like dental fluorosis, transposition of 13 and 14, and multiple cusps in maxillary first molars. Also this case did not show the expected pattern of inheritance. Cases of dentin dysplasia have to be treated conservatively to prevent premature exfoliation of teeth.