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Case Report

A rare condition affecting the primary and permanent dentition: Dentin dysplasia type I
Ahmet Ercan Sekerci, Meryem Etoz, Halil Sahman1, Yldray Sisman, Sinan Nazlm2
Department of Oral and Maxillofacial Radiology, Faculty of Dentistry, Erciyes University, Kayseri, 1Department of Oral and Maxillofacial Radiology, Faculty of Dentistry, Abant Izzet Baysal University, University, Bolu, 2Department of Pathology, Faculty of Medicine, Erciyes University, Kayseri, Turkey

A B S T R A C T
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 specic 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 ndings of this condition. Key words: Dentin dysplasia, pulp obliterations, permanent dentition, rootless teeth

INTRODUCTION
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 rst described by Ballschmiede[3] but it was Rushton[4] who rst used the term dentinal dysplasia. This condition is rarely encountered in dental practice. In 1972, Witkop[5] classied 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
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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 ndings 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

DOI: 10.4103/2321-3841.111346

Corresponding Author: Assistant Professor, Ahmet Ercan Sekerci, Department of Maxillofacial Radiology, Faculty of Dentistry, Erciyes University, 38039, Kayseri, Turkey. E-mail: aercansekerci@hotmail.com Journal of Oral and Maxillofacial Radiology / January-April 2013 / Vol 1 | Issue 1 13

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Sekerci, et al.: Dentin dysplasia type I

appearing mantle dentin and globular or nodular masses of abnormal dentin are seen.[6] Management of this condition presents the dentist with difculty, 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 conrmed by histopathological examination.

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 welldened 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. The maxillary primary central incisors, right lateral incisor, mandibular primary right canine, rst 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 supercial dentin of the crown appeared normal, but the pulp chamber was obliterated by an unusual type of calcied 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,

CASE REPORT
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 patients 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 patients 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 patients father revealed no evidence similar condition. The patients 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 1a]. 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 stule on the buccal region of the maxillary right primary central incisor Periapical radiolucencies were present at the permanent upper central incisors. The panoramic radiograph revealed features of dentine
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Figure 1: Intraoral view of the patient with dentin displasia type I (a-c)

Figure 2: Panoramic radiographs exhibiting the absence of root development, obliterated pulps of teeth affecting the primary and permanent dentition

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Sekerci, et al.: Dentin dysplasia type I

Figure 3: Some extracted teeth show normal coronal size, shape, color, and no root development

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 patients older brother, mother, and grandfather had similar condition. Although from the patients 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 difcult, 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
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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)

remaining deciduous teeth were removed under local anesthesia due to excessive mobility. Overdenture-style removable dentures were advised to meet the patients 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.

DISCUSSION
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 calcied pulp chambers, rootless teeth, periapical radiolucent areas, and the nature of the periapical lesion were characteristic ndings for the diagnosis of DD

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Sekerci, et al.: Dentin dysplasia type I

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 lling, 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.

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Cite this article as: 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. Source of Support: Nil. Conict of Interest: No.

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