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 Table of Contents  
CASE REPORT
Year : 2013  |  Volume : 3  |  Issue : 1  |  Page : 57-60

Dentin dysplasia type I: A rare case report


Department of Oral Medicine and Radiology, M M's N.G. Halgekar Institute of Dental Sciences and Research Centre, Belgaum, Karnataka, India

Date of Web Publication26-Nov-2013

Correspondence Address:
Sujata Mohan Byahatti
Plot No 49, Sector #9, Malmaruti Extn, Belgaum - 590 016, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2231-6027.122128

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  Abstract 

Dentin dysplasia (DD) type I is an inherited autosomal dominant genetic defect affecting the dentin formation. Here, the teeth are characterized by normal appearing crowns, with 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 where little is known about the specific treatment of this disorder and management of patients with DD. This report documents one such rarity of DD type I in a 16-year-old female and the clinical and radiographical findings of this condition.

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


How to cite this article:
Byahatti SM. Dentin dysplasia type I: A rare case report. Int J Oral Health Sci 2013;3:57-60

How to cite this URL:
Byahatti SM. Dentin dysplasia type I: A rare case report. Int J Oral Health Sci [serial online] 2013 [cited 2017 Mar 23];3:57-60. Available from: http://www.ijohsjournal.org/text.asp?2013/3/1/57/122128


  Introduction Top


The dentin dysplasias (DD), is a rare autosomal dominant inherited abnormality affecting the primary and permanent dentition. [1] It is characterized by defective dentin formation with abnormal pulpal morphology with clinically normal appearing crowns with severe hypermobility of teeth and spontaneous dental abscesses or cysts. [2] It is a rare anomaly of unknown etiology that affects approximately one patient in every 1,00,000 [3] individuals. The condition was first described by Ballschmiede. [4] However, it was Rushton [5] who first described this condition as a "rootless teeth" later he preferred to use the term "dentinal dysplasia." In 1972, Witkop [6] 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. [7] Owing to shortened roots observed in this anomaly, teeth may exhibit extreme mobility and exfoliate prematurely. [8],[9] DD type II, which is also called coronal DD, 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 a color, similar to those seen in dentinogenesis imperfecta and the pulp chambers are completely obliterated, but these findings are not present in the permanent teeth. [11] Management of this condition varies according to the age of the patient, severity of the problem and the presenting complaint. [12]

This following paper describes one such uncommon case of DD type I in a 16-year-old girl, describing the clinical and radiological variations of the defect in detail.


  Case Report Top


A 16-year-old girl referred to the Department of Oral medicine and Radiology, complaining of irregularly placed teeth. None of the family members had a similar complaint. Patient was apparently healthy, moderately built and nourished with no other clinical deformities extraorally. One of the main clinical finding encountered in DD I are short roots associated with tooth mobility and premature exfoliation. In this patient, submerged 36, retained 73, transposition with 33, 32, Grade 1 mobility with 11, 21, 22, Grade 2 mobility with 32, and Grade 3 mobility with 42 were noted [Figure 1], [Figure 2] and [Figure 3]. All other teeth presented normal crown clinically and on radiographic appearance.
Figure 1: Intraoral photograph showing upper jaw

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Figure 2: Intraoral photograph showing lower jaw

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Figure 3: Intraoral photograph showing anterior open bite

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Panoramic radiographic [Figure 4] examination confirmed that teeth 12 and 42 were missing. The roots were completely absent for 11, 16, 21, 22, 26, 32, 33, 36, 46, More than half of root noted in 13, 14, 15, 17, 24, 25, 27, 34, 35, 36, 43, 44, 45, 47, complete root formed with 13, 43 and taurodontism with 17,37. With above history, clinical findings and radiographic findings provisional diagnosis of DD type I was done. DD type I should be differentiated from DD type II, dentinogenesis imperfecta and odontodysplasia. In our patient, the wide pulp chambers, rootless teeth, are characteristic findings for the diagnosis of DD type I.
Figure 4: Panoramic radiographs exhibiting the absence of root development

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The progression of this case was under continuing evaluation. At each follow-up visit scheduled on a 3-month basis and for professional control of oral hygiene, instructions on oral home-care are reinforced and the affected regions are examined both clinically and radiographically.


  Discussion Top


DD I is characterized clinically by nearly normal appearing crowns and hypermobility of the teeth. [13],[14] The female patient in this report has clear features of DD I such as clinically normal crowns, mobility of the teeth, taurodontism and short, tapering roots. One of the main clinical problems encountered in DD I are short, tapering roots associated with tooth mobility and premature exfoliation. [15] In this patient, submerged 36, Retained 73, Transposition with 33,32, Grade 1 mobility with 11, 21, 22, Grade 2 mobility with 32, and Grade 3 mobility with 42 are noted. The root lengths were excessively shortened except for 13, 43.

The pathogenesis of DD is unknown in most of the dental literature. Logan et al. [13] proposed that it is the dentinal papilla that is responsible for the abnormalities in root development. They suggested that multiple degenerative foci within the papilla become calcified, leading to reduced growth and final obliteration of the pulp space. Wesley et al. [16] 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 is usually an autosomal dominant condition, [3] but in this patient, there was no familial history of the disease, so she is considered to be a first generation to suffer. Teeth with radiographic or histologic features of DD occur in a number of disorders such as calcinosis,  Ehlers-Danlos syndrome More Details and the brachioskeletogenital syndrome. [17] Some association has also been reported between dentine dysplasia and osseous changes in addition to sclerotic bone formation, [18] but our patient had no signs of other pathologic conditions.

The intrinsic abnormalities of the cementum are not consistent features [19],[20] of DD, it is likely that loss of periodontal attachment may be adequately prevented through effective oral hygiene. In addition, abnormal extensions of the pulp occlusally may cause pulp exposures from minimal dental caries. [21] Thus, effective preventive measures for dental caries such as fissure sealants and topical fluoride are important aspects of patient management. Prophylactic stainless steel crowns may be considered for patients with accompanying enamel hypoplasia, but care must be taken to prevent periodontal inflammation, which may exacerbate the likelihood of early exfoliation. In the primary dentition, extraction of involved teeth and space maintenance are the obvious treatments for pulpal infections. However, the present patient was kept under observation and recalled after every 6 months for the follow-up. [22]

Management of patients with dentinal dysplasia has presented dentists with many problems. 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 the treatment plan in DD. [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. [24],[25] Since these patients usually have early exfoliation of the teeth and consequently, maxillomandibular bony atrophy, treatment with a combination of onlay bone grafting and a sinus lift technique to accomplish implant placement can be used successfully. [26]

 
  References Top

1.Sekerci AE, Etoz M, Sahman H, Yildiray S, Nazlim S. A rare condition affecting the primary and permanent dentition: Dentin dysplasia type I. J Oral Maxillofac Radiol 2013;1:13-6.  Back to cited text no. 1
    
2.Shafer WG. Hine MK, Levy BM. Developmental disturbances of oral and paraoral structures. In: A Text Book of Oral Pathology. 4 th ed. Philadelphia: Elsevier Science, Saunders Co.; 2003. p. 2-85.  Back to cited text no. 2
    
3.Kim JW, Simmer JP. Hereditary dentin defects. J Dent Res 2007;86:392-9.  Back to cited text no. 3
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4.Ballschmiede G. Dissertation. Berlin. In: Herbst E, Istaedt MA, editors. Malformations of the Jaws and Teeth. New York: Oxford University Press; 1930. p. 286.  Back to cited text no. 4
    
5.Rushton MA. A case of dentinal dysplasia. Guys Hosp Rep 1939;89:369-73.  Back to cited text no. 5
    
6.Witkop CJ Jr. Hereditary defects of dentin. Dent Clin North Am 1975;19:25-45.  Back to cited text no. 6
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7.Toomarian L, Mashhadiabbas F, Mirkarimi M, Mehrdad L. Dentin dysplasia type I: A case report and review of the literature. J Med Case Rep 2010;4:1.  Back to cited text no. 7
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8.Depprich RA, Ommerborn MA, Handschel JG, Naujoks CD, Meyer U, Kübler NR. Dentin dysplasia type I: A challenge for treatment with dental implants. Head Face Med 2007;3:31.  Back to cited text no. 8
    
9.Carroll MK, Duncan WK, Perkins TM. Dentin dysplasia: Review of the literature and a proposed subclassification based on radiographic findings. Oral Surg Oral Med Oral Pathol 1991;72:119-25.  Back to cited text no. 9
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10.Duncan WK, Perkins TM, O Carroll MK, Hill WJ. Type I dentin dysplasia: Report of two cases. Ann Dent 1991;50:18-21.  Back to cited text no. 10
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11.Ravanshad S, Khayat A. Endodontic therapy on a dentition exhibiting multiple periapical radiolucencies associated with dentinal dysplasia Type 1. Aust Endod J 2006;32:40-2.  Back to cited text no. 11
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12.Barron MJ, McDonnell ST, Mackie I, Dixon MJ. Hereditary dentine disorders: Dentinogenesis imperfecta and dentine dysplasia. Orphanet J Rare Dis 2008;3:31.  Back to cited text no. 12
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13.Logan J, Becks H, Silverman S Jr, Pindborg JJ. Dentinal dysplasia. Oral Surg Oral Med Oral Pathol 1962;15:317-33.  Back to cited text no. 13
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14.Sauk JJ Jr, Lyon HW, Trowbridge HO, Witkop CJ Jr. An electron optic analysis and explanation for the etiology of dentinal dysplasia. Oral Surg Oral Med Oral Pathol 1972;33:763-71.  Back to cited text no. 14
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15.Scola SM, Watts PG. Dentinal dysplasia type I. A subclassification. Br J Orthod 1987;14:175-9.  Back to cited text no. 15
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16.Wesley RK, Wysoki GP, Mintz SM, Jackson J. Dentin dysplasia type I. Clinical, morphologic, and genetic studies of a case. Oral Surg Oral Med Oral Pathol 1976;41:516-24.  Back to cited text no. 16
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17.Perl T, Farman AG. Radicular (type 1) dentin dysplasia. Oral Surg Oral Med Oral Pathol 1977;43:746-53.  Back to cited text no. 17
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18.Morris ME, Augsburger RH. Dentine dysplasia with sclerotic bone and skeletal anomalies inherited as an autosomal dominant trait. A new syndrome. Oral Surg Oral Med Oral Pathol 1977;43:267-83.  Back to cited text no. 18
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19.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 1980;50:335-40.  Back to cited text no. 19
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20.Jasmin JR, Clergeau-Guerithault S. A scanning electron microscopic study of dentin dysplasia type II in primary dentition. Oral Surg Oral Med Oral Pathol 1984;58:57-63.  Back to cited text no. 20
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21.Steidler NE, Radden BG, Reade PC. Dentinal dysplasia: A clinicopathological study of eight cases and review of the literature. Br J Oral Maxillofac Surg 1984;22:274-86.  Back to cited text no. 21
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22.Coke JM, Del Rosso G, Remeikis N, Van Cura JE. Dentinal dysplasia, Type I. Report of a case with endodontic therapy. Oral Surg Oral Med Oral Pathol 1979;48:262-8.  Back to cited text no. 22
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23.Elzay RP, Robinson CT. Dentinal dysplasia. Report of a case. Oral Surg Oral Med Oral Pathol 1967;23:338-42.  Back to cited text no. 23
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24.Ozer L, Karasu H, Aras K, Tokman B, Ersoy E. Dentin dysplasia type I: Report of atypical cases in the permanent and mixed dentitions. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2004;98:85-90.  Back to cited text no. 24
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25.Ansari G, Reid JS. Dentinal dysplasia type I: Review of the literature and report of a family. ASDC J Dent Child 1997;64:429-34.  Back to cited text no. 25
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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
    


    Figures

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



 

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