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Year : 2013  |  Volume : 1  |  Issue : 1  |  Page : 13-16

A rare condition affecting the primary and permanent dentition: Dentin dysplasia type I

1 Department of Oral and Maxillofacial Radiology, Faculty of Dentistry, Erciyes University, Kayseri, Turkey
2 Department of Oral and Maxillofacial Radiology, Faculty of Dentistry, Abant Izzet Baysal University, University, Bolu, Turkey
3 Department of Pathology, Faculty of Medicine, Erciyes University, Kayseri, Turkey

Date of Web Publication3-May-2013

Correspondence Address:
Ahmet Ercan Sekerci
Department of Maxillofacial Radiology, Faculty of Dentistry, Erciyes University, 38039, Kayseri
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Dentin dysplasia type I is a genetic defect of dentin formation, which is inherited as an autosomal dominant trait. In this anomaly, the teeth generally appear unremarkable with normal crowns; and it is characterized by normal appearing crowns, absence or severe restriction of root formation, obliterated pulp chambers, and periapical radiolucencies without an obvious cause. Clinically, the teeth can be poorly aligned, drifting and mobile, and can prematurely exfoliate due to abnormal root formation. This condition is rarely encountered in dental practice. Little is known about the specific treatment of this disorder and management of patients with dentin dysplasia has presented with problems. This report documents a rare case of DD type I in a 7-year-old girl and the clinical, radiographical and histopathological findings of this condition.

Keywords: Dentin dysplasia, pulp obliterations, permanent dentition, rootless teeth

How to cite this article:
Sekerci AE, Etoz M, Sahman H, Sisman Y, Nazlim S. A rare condition affecting the primary and permanent dentition: Dentin dysplasia type I. J Oral Maxillofac Radiol 2013;1:13-6

How to cite this URL:
Sekerci AE, Etoz M, Sahman H, Sisman Y, Nazlim S. A rare condition affecting the primary and permanent dentition: Dentin dysplasia type I. J Oral Maxillofac Radiol [serial online] 2013 [cited 2023 Mar 24];1:13-6. Available from: https://www.joomr.org/text.asp?2013/1/1/13/111346

  Introduction Top

Dentin dysplasia (DD) is a rare hereditary disturbance of dentin formation characterized by defective dentin development with clinically normal appearing crowns, severe hypermobility of teeth, and spontaneous dental abscesses or cysts, but atypical dentin with abnormal pulpal morphology. [1] It is a rare anomaly of unknown etiology that affects approximately 1 patient in every 100,000. [2] The condition was first described by Ballschmiede [3] but it was Rushton [4] who first used the term "dentinal dysplasia." This condition is rarely encountered in dental practice. In 1972, Witkop [5] classified it into type I and type II which affect both dentitions. DD Type I is by far the more common. Both types include generalized/multiple involvement of the primary and permanent dentitions. [6] Combinations of both types have also been described in the literature. [7] also called radicular dentin dysplasia, is characterized by the presence of primary and permanent teeth with normal appearing crowns, but no or only rudimentary root development, incomplete or total obliteration of the pulp chamber, a horizontal radiolucent line at the dentinoenamel junction and periapical radiolucent areas or cysts. This condition is sometimes said characterized by "rootless teeth" because of the shortened roots observed. In this anomaly, teeth may exhibit extreme mobility and exfoliate prematurely. [8],[9] DD type II, also called coronal dentin dysplasia, is characterized by partial pulpal obliteration with the amorphous dentin often forming pulp stones, thistle-tube-shaped coronal pulp chambers, thread-like root canals, and usually the absence of periapical radiolucencies. [9],[10] In this type of anomaly, teeth roots are of normal shape and contour. Clinically, the primary teeth are brown or opalescent blue in color, similar to those seen in dentinogenesis imperfect and the pulp chambers are completely obliterated, but these findings are not present in the permanent teeth. [11]

Histopathologically, the enamel and the immediately subjacent dentin appear normal. Deeper layers of dentin show an atypical tubular pattern with an amorphous, atubular area, and irregular organization. Pulpally to normal appearing mantle dentin and globular or nodular masses of abnormal dentin are seen. [6]

Management of this condition presents the dentist with difficulty, the teeth being lost early through periapical abscesses, cysts or spontaneous exfoliation. [12] Treatment varies according to the age of the patient, severity of the problem and the presenting complaint. [13]

This paper describes an uncommon case of DD type I in a 7-year-old girl, highlighting the clinical and radiographic variations of the defect as confirmed by histopathological examination.

  Case Report Top

A 7-year-old girl was referred to the Department of Oral and Maxillofacial Radiology, Dentistry Faculty, Erciyes University complaining mobile teeth and a swelling in the maxillary deciduous right incisor area. According to the patient's father, the patient had lost her mandibular central incisors teeth by the age of 3 and mandibular left primary second molar by the age of 5. Her older brother had a similar condition. Furthermore, the patient's mother reportedly became edentulous at an early age and had required full maxillary and mandibular dentures. The grandfather of the girl stated that he too had a history of delayed eruption. He further stated that he had lost most of his teeth at an early age because of spontaneous exfoliation, which had necessitated a full denture in the maxilla and a partial denture in the mandible by the end of his adolescence. Dental examination of the patient's father revealed no evidence similar condition.

The patient's medical history revealed no evidence of disturbance in general health. The patient was having class III malocclusion with spacing between maxillary and mandibular anteriors. Clinical examination revealed an anterior open bite and mandibular prognatie [Figure 1]a. The intraoral examination revealed the following teeth, which were of normal size, morphology and color, to be present: 55, 54, 53, 52, 51, 61, 63, 64, 65, 74, 73, 31, 41, 83, 84, 85. The dentition was free of caries [Figure 1] b-d. There was grade I mobility of 31 and 41, grade II mobility of 55, 54, 53, 63, 64, 65 and grade III mobility with 51, 52, 61,83,84,85. All the teeth were normal in shape and size. There was a painful fistule on the buccal region of the maxillary right primary central incisor Periapical radiolucencies were present at the permanent upper central incisors.
Figure 1: Intraoral view of the patient with dentin displasia type I (a-c)

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The panoramic radiograph revealed features of dentine dysplasia type I with normal appearance of crown but no root development in relation to 51, 52, 54, 55, 64, 65, 73, 74, 83, 84, 85, 16, 26, 36, 46, 11 and 21, slight root formation in relation to 31,41,53 and 63. Pulp chambers were obliterated and bone below the teeth showed well-defined margins, and periapical radiolucencies were not seen. All permanent teeth were present except third molars [Figure 2]. On the basis of the clinical and radiographic appearance, a diagnosis of DD type I was suspected.
Figure 2: Panoramic radiographs exhibiting the absence of root development, obliterated pulps of teeth affecting the primary and permanent dentition

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The maxillary primary central incisors, right lateral incisor, mandibular primary right canine, first and second molars mandibular left second premolar was extracted owing to extensive mobility [Figure 3]. Extracted teeth were sent to an oral pathologist for histopathological examination. The ground section was examined with a stereomicroscope; the superficial dentin of the crown appeared normal, but the pulp chamber was obliterated by an unusual type of calcified material consisting of dentin, and the deeper layers of dentin had an atypical tubular pattern [Figure 4]. These features are consistent with the clinical diagnosis of DD type I. On the basis of the clinical, radiographical and histopathological examinations, the patient was diagnosed with DD type I.

In a follow-up visit 2 months after the initial examination, remaining deciduous teeth were removed under local anesthesia due to excessive mobility. Overdenture-style removable dentures were advised to meet the patient's functional, phonational, and esthetic requirements but both the patient and her father refused this treatment plan. The patient was observed with an excellent in oral hygiene. The patient continues to be followed up by our department.
Figure 3: Some extracted teeth show normal coronal size, shape, color, and no root development

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Figure 4: Histologic examination of the pulp chambers are revealed to be obliterated with nodular. These nodular structures composed of ill-organized bundles containing (HE X40)

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  Discussion Top

DD type I is a rare condition affecting the primary and permanent dentition, in which teeth present with normal appearing crowns but no or only rudimentary root development, incomplete or total obliteration of the pulp chamber and periapical radiolucent areas or cysts. [14],[15] In the present case, the calcified pulp chambers, rootless teeth, periapical radiolucent areas, and the nature of the periapical lesion were characteristic findings for the diagnosis of DD type I. The etiology of DD is still unknown; however, several theories have been proposed in the dental literature. Logan et al. [16] suggested that the dentinal papilla is responsible for the abnormalities in root development. Sauk et al. in a scanning electron microscope study, postulated an invagination of the epithelial root sheath resulting in abnormal dentin formation. Wesley et al. disagreed with this suggestion and proposed that the condition is caused by an abnormal interaction of odontoblasts with ameloblasts leading to abnormal differentiation and/or function of these odontoblasts. Clearly, the precise nature of the defect has yet to be determined. The cause of periapical radiolucencies in DD type I is not understood. Steidler et al. [17]

suggest that they are the result of pulpal necrosis, occurring either secondary to caries or spontaneously. DD type I should be differentiated from DD type II, dentinogenesis imperfecta and odontodysplasia [6] Flynn and High [18] reported an interesting case. In that study, teeth had some of the radiographic features of DD type I, as well as some of DD type II and of dentinogenesis imperfecta. DD is recognized as a genetic disorder and is thought possibly to be a single gene mutation. [10] When multiple family members have a similar pattern of pathosis, this supports a diagnosis of a hereditary condition. [19] An attempt to identify a familial history in our case, the patient's older brother, mother, and grandfather had similar condition. Although from the patient's description, it was not possible to examine them to reach any conclusive diagnosis.

Histologically, the immediately subjacent dentin and the enamel appear normal. Deeper layers of dentin with demonstrate an atypical tubular pattern with amorphous, atubular globular, or nodular masses of abnormal dentin are seen. [20] The periapical radiolucent areas seen in most cases of dentinal dysplasia have been interpreted as radicular cysts; however, in some cases, a diagnosis of periapical granuloma has been reported. [16],[21] Teeth with histological or radiographical characteristics of DD occur in a number of disorders such as Ehlers-Danlos syndrome, calcinosis, brachioskeletogenital syndrome, [22] rheumatoid arthritis, and vitaminosis D [5] Some association has also been reported between DD and osseous changes in addition to sclerotic bone formation [23] but our patient had no signs of other pathologic conditions.

The management of patients with DD is difficult, and various treatment strategies have been proposed. Extraction has been suggested as a treatment alternative for teeth with pulp necrosis and periapical abscess. Follow-up and routine conservative treatment is another choice of treatment plan in DD [24] 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. [23] Another approach for the treatment of teeth with DD has included periapical surgery and retrograde filling, which is recommended in teeth with long roots. [11],[24] Since these patients usually have early exfoliation of the teeth and consequently, maxilla-mandibular bony atrophy, treatment with a combination of onlay bone grafting and a sinus lift operation to accomplish implant placement can be used successfully. [23] Though a malalignment of the arch is one of the most common characteristics of this disorder, orthodontic correction should not be applied in a routine fashion. The short roots characteristic of DD type I might not be able to resist the applied orthodontic forces, which would result in further resorption of the roots, loosening of teeth, and premature exfoliation. [11] In the majority of cases, despite early diagnosis and the provision of regular dental care, teeth are lost because of spontaneous abscess formation. There is no treatment for severe cases of DD type I other than extraction of symptomatic teeth. Even for special cases, onlay autogenous bone grafting and sinus lift technique are well-tested methods of augmentation. [23]

Therapy, including extraction of all teeth, curettage of cystic alteration, and functional rehabilitation by the insertion of a conventional complete denture, has been presented previously by Neumann et al.[25] Munoz-Guerra et al. [26] reported successful treatment of a 24-year old female after onlay bone grafting and sinus augmentation. It is anticipated that a lot of permanent teeth may be lost due to severe mobility and may undergo spontaneous pulpal necrosis. The possibility of endosseous implants is being explored for when the patient reaches her late teens and growth is complete.

Though many cases of DD type I have been described some clinical aspects and theoretic issues remain uncertain. Early diagnosis of the condition is important for the initiation of effective preventive treatment. Follow-up studies on patients with DD type I might provide insights into treatment strategies. Future molecular genetic research may determine precise information about the gene locus responsible for DD type I.

  References Top

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13.Barron MJ, McDonnell ST, Mackie I, Dixon MJ. Hereditary dentine disorders: Dentinogenesis imperfecta and dentine dysplasia. Orphanet J Rare Dis. 2008;20;3:31.  Back to cited text no. 13
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15.Shields ED, Bixler D, el-Kafrawy AM. A proposed classification for heritable human dentine defects with a description of a new entity. Arch Oral Biol 1973;18:543-53.  Back to cited text no. 15
16.Logan J, Becks H, Silverman S, Pinnborg JJ. Dentinal dysplasia. Oral surg. 1962;15:317-33.   Back to cited text no. 16
17.Steidler NE, Radden BG. Reade PC. Dentinal dysplasia: A clinicopathological study of eight cases and review of the literature.Br J Oral Maxillofacl Surg 1984;22:274-86.  Back to cited text no. 17
18.Flynn CM, High AS. The diagnosis of dentinal dysplasia Type 1. Dentomaxillofac Radiol 1993;22:43.  Back to cited text no. 18
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20.Melnick M, Levin LS, Brady J. Dentin dysplasia type I: A scanning electron microscopic analysis of the primary dentition. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1980;50:335-40.  Back to cited text no. 20
21.Coke JM, Del Russo G, Remeikie N, VanCura JE. Dentinal dysplasia type I. Report of a case with endodontic therapy. Oral Surg 1979;48:262-8.  Back to cited text no. 21
22.Perl T, Farman AG, Elizabeth P, Town C. Radicular (Type I) dentin dysplasia. Oral Surg Oral Med Oral Pathol 1977;43:746-53.  Back to cited text no. 22
23.Morris ME, Augsburger RH. Dentine dysplasia with sclerotic bone and skeletal anomalies inherited as an autosomal dominant trait. Oral Surg Oral Med Oral Pathol 1977;43:267-83.  Back to cited text no. 23
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26.Muñoz-Guerra MF, Naval-Gías L, Escorial V, Sastre-Pérez J. Dentin dysplasia type I treated with onlay bone grafting, sinus augmentation, and osseointegrated implants. Implant Dent 2006;15:248-53.  Back to cited text no. 26


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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