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Authors: Dr. Ghosh Tuhina, Dr. Sarkar Subir, Dr. Saha Subrata


Dentin dysplasia (DD) is an autosomal dominant inherited abnormality of dentin formation, characterized by defective dentin development with clinically normal appearing crowns, severe hypermobility of teeth and spontaneous dental abscesses or cysts.1 It is a rare anomaly of unknown etiology affecting either the primary or both the dentitions in approximately one patient in every 1, 00,000 individuals.2
The condition was first described by Ballschmeide 3 in 1920 as ‘rootless teeth’ but it was Rushton 4 who coined the term Dentinal dysplasia in1939. In 1972, Witkop 5 classified DD classified into type I: Radicular DD and type II: Coronal DD. Dentin Dysplasia type1(DD1) is characterized by the presence of primary and permanent teeth with normal appearing crowns, but no or only rudimentary root development, aberrant growth of dentin in the pulp chamber leading to reduced pulp space in permanent teeth, incomplete or total obliteration of the pulp chamber, periapical radiolucent areas or cysts. Owing to shortened roots, there may be extreme mobility and premature exfoliation of teeth, either spontaneously or with minimum trauma. 6
This case report highlights a rare case of DD type 1 describing the clinical and radiological variations of the defect and the importance of preventive care in this anomaly.

Figure 1. Frontal and lateral profile showing midface deficiency, competent lips and flattening of bridge of nose. Figure 2. Intraoral view showing missing mandibular centrals and carious maxillary right central incisor.
Figure 3. Maxillary occlusal view. Figure 4. Mandibular occlusal view.


A 12 year old girl reported with a chief complaint of spontaneous loss of teeth in lower front region of the mouth. Extraoral examination showed class ӏӏӏ profile with midface deficiency, competent lips and flattening of bridge of nose [Figure 1]. Intraoral examination showed satisfactory oral hygiene with no signs of gingivitis and periodontitis. Clinical examination showed missing lower central incisors, grade II mobility in mandibular laterals with caries present on right maxillary central incisor [Figure 2]. The patient was having crowding in maxillary and mandibular anterior region [Figure 3 and 4]. Panoramic radiograph showed normal appearance of crowns, obliterated pulp chambers in most of the teeth with rudimentary root formation. Bone below the teeth showed well defined margins and periapical radiolucencies were not seen [Figure 5].

Patient’s medical history revealed no disturbance in general health. Clinical and radiographic examination of the patient’s parents revealed no such disorder or any previous familial history of such condition; therefore she was considered a first generation sufferer. History, clinical and radiographic findings revealed this case as ‘DD type I’. Considering the age of the patient, treatment was aimed in maintaining the health of existing teeth. Treatment strategy consisted of instructing the patient in oral hygiene and dietary measures with fluoride supplements.

Restoration of the carious lesion and removable partial dentures were advised to meet the patient’s functional, phonation, and aesthetic requirements until a more definitive treatment plan in form of dental implants or fixed partial dentures can be considered once her growth is completed.
Figure 5. Panoramic radiograph.


The etiology of DD1 remains speculative. Logan et al 7 suggested that abnormal calcification of the pulp is related to degeneration of the dental papilla, whereas Sauk and coworkers 8 postulated that premature invagination of the epithelial root sheath is associated with abnormal dentin deposition. Wesley et al 9 proposed that the condition is caused by an abnormal interaction of odontoblasts with ameloblasts leading to abnormal differentiation and/or function of these odontoblasts.

DD type I should be differentiated from DD type II, dentinogenesis imperfecta, and odontodysplasia. The present case exhibited defined features of DDI including clinically normal crowns, radiographic obliteration of pulp chambers along with short blunted roots. Teeth with radiographic or histologic features of DD occur in a number of disorders such as calcinosis, osteogenesis imperfecta, Ehlers Danlos syndrome, Goldblatt syndrome, Schimke immuno‑osseous dysplasia, and Brachio‑skeleto‑genital syndrome but our patient had no signs of other pathological conditions.

DD is usually an autosomal dominant condition but in this patient, there was no familial history of the disease. She had no siblings and radiographs of her parents’ dentition appeared normal, therefore she was considered a first generation sufferer. Premature loss of teeth and loose teeth as a result of rudimentary or absence of roots has presented dentists with many problems in management of patients with DD I. Preventive care is of foremost importance for patients with dentin dysplasia type I. Although not identified in the present case, periapical granulomas and apical cysts present another important management problem. These are likely to have resulted from "spontaneous" pulp infections associated with severe pulpal obliteration. In addition pulp vascular channels extend close to the dentino-enamel junction. Therefore, even shallow occlusal restorations can result in pulpal necrosis. Thus, effective preventive measures for dental caries such as fissure sealants and topical fluoride are important aspects of patient management. Obliteration of the pulp chambers and root canals in teeth that develop abscesses makes endodontic therapy difficult. If conventional endodontic therapy is not possible, periapical curettage and retrograde filling is another alternative but not recommended in teeth with too short roots. [10]

Extraction has been suggested as a treatment alternative for teeth with pulp necrosis and periapical abscess. In DDI, because of the shortened roots, early tooth loss from periodontitis is frequent. For the maintenance of periodontal health of the mobile teeth, oral hygiene must be established and maintained. In this case, follow-up and routine conservative treatment plan was preferred for the patient, because the teeth had no mobility and the patient’s oral hygiene was good.

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