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Case Report
Open Access Peer-reviewed

Dentine Dysplasia Type II: Report of a Rare Case with Radiographic Criteria of Type I

Hamdy AM Metwaly, Faraj Alotaiby
International Journal of Dental Sciences and Research. 2020, 8(4), 87-90. DOI: 10.12691/ijdsr-8-4-1
Received April 26, 2020; Revised May 28, 2020; Accepted June 04, 2020

Abstract

Dentin dysplasia (DD) is a hereditary malformation of dental tissues affecting the dentin during the process of development. The teeth appear clinically normal; however, radigraphically the pulp morphology is abnormal and may be large or obliterated. The roots are diminutive, blunted, deformed or even absent. Periapical radiolucencies may be present without an apparent cause. The affected teeth may exhibit great mobility and exfoliate early even with minor trauma. In this article, we reported clinical and radiographic findings of a rare case of DD that have radiographic features of both DD- type II and DD type I in a 24 years old male patient visited the Out-Patient Dental Clinic of Qassim University, KSA.

1. Introduction

Dentine Dysplasia (DD) is a rare autosomal dominant hereditary disease affecting primary or mixed dentitions. The incidence of this rare disorder is approximately 10 times less than that of dentinogenesis imperfecta (DI), with an affected patient in every 100,000 individuals 1, 2. This hereditary disease is characterized by disturbance in formation of dentin during tooth development, but does not affect enamel formation 3. In 1920, this condition was first described as rootless teeth by Ballschmiede 4 who described 7 cases in same family who have short blunt-rooted teeth, hypermobility and subsequent teeth exfoliation. The name DD was introduced by Rushton in 1939 5. The first classification of DD was done by Shield et al in 1973 into Typ1 DD (Dentine Dysplasia) and Type 2 (Anomalous dysplasia of dentin), which was confusing during that time 6.

The recent classification of DD was done by Witkop, in1974 who classified DD according to the affected part of the tooth into Radicular Dysplasia, DD type I (DD-I,) and Coronal Dysplasia, DD type II (DD-II) 7.

DD-I affects the roots of both deciduous and permanent dentitions. The roots in DD-I are short, blunt, conical, malformed or even absent. Periapical radiolucencies representing granuloma, abscess or cyst may be present. In permanent teeth, atypical growth of dentin in the pulp chamber leading to decreased pulpal cavity space and incomplete or total obliteration of the pulp chambers 4.

DD-II affects the coronal pulp and characterized by a large pulp chamber. It is described as ‘thistle-tube appearance’ which means a large pulpal chamber with some extension to a thin root canal. In addition, formation of multiple pulp stones in permanent teeth and obliteration of deciduous teeth are also documented in DD-II 8.

DD-I is further classified into 4 sub categories; DD-Ia, DD-Ib, DD-Ic, DD-Id based on distinctive criteria on radiograph 9. In DD-1a, there is complete obliteration of pulp chambers and no root development with many periapical radiolucent areas. In DD-1b, the pulp showed a horizontal, crescent shaped, radiolucent remnants and few millimeters of root development with many periapical radiolucent areas. DD-Ic has two horizontal, crescent shaped, radiolucent lines and significant but incomplete root development, with or without periapical radiolucent areas. In DD-Id pulp chambers present with oval pulp stones in the coronal third of the canal. Bulging of the canal around the stone in a normally formed root is usually discernible, with few, if any, periapical radiolucent areas.

Dentin dysplasia type III includes a combination of teeth of type I and type II in the same patient. It was proposed by Ciola et al. 10 as a third group of DD classification. The aim of this study is to report a rare case that possess features of both types of DD and shed highlights on clinical and radiographic findings of such condition.

2. Case Report

A 24 years old male patient visited the Out-Patient Dental Clinic of Qassim University, KSA seeking a construction of a fixed dental prosthesis for a missing anterior tooth for an esthetic reason. The medial history revealed that the patient was healthy and free from any systemic diseases.

On clinical examination, there were missing upper left central incisor, premolar and 1st molar teeth and upper right 1st molar tooth. The oral hygiene was bad with many carious teeth including upper left 2nd premolar, Upper canine and lower right 2nd molar (Figure 1). The crown of the teeth are proportionally normal in size except for the right central incisor which was larger than normal.

3. Radiographic Findings

Periapical and panoramic x-ray films were performed in order to evaluate the remaining teeth for construction of prosthesis. Radiographic examination of OPG revealed large pulp chambers (thistle shaped appearance) in some of teeth, which was evident in single rooted teeth. Other teeth were obliterated with radiopaque globules (denticles). The roots of some teeth are short, blunted or conical in shape, appearantly in lower incisors (Figure 2). In periapical radiographs of upper molars (Figure 3A) and lower molars (Figure 3B), there were large radiopaque globules obliterating the pulp chamber and decreasing the sized of root canals. In periapical radiograph of upper central and lateral incisors, the pulp chambers were large extending to radicular pulp giving the ‘thistle tube appearance’. Radiopaque globules were observed within the pulp chamber leaving remnants of pulp spaces (Figure 4). Based on the clinical and radiographic appearance, a diagnosis of DD-II with some features of DD-I was rendered.

4. Discussion

DD and DI are developmental autosomal dominant conditions affecting dentine formation. DD is a rare condition when compared to DI with an incidence one-tenth that of DI 1, 2, 11. The genetic mutations for DD- II and Non-syndromatic DI type II and III were reported in dentin sialophosphoprotein gene (DSPP) which is carried on chromosome 4q21.3 encoding the major non-collagenous proteins. This gene is involved in initiation and growth of crystals of hydroxyapatite and the process of mineralization 11. Genetic disturbance in DD-I have not been clarified yet 11, 12, 13.

It was reported in one study a genetic mutation in Sparc Related Modular Calcium Binding Protein-2 gene (SMOC-2) in 2 affected first cousin relatives’ in Turkish family who have DD-I associated with microdontia and malformed teeth 14.

Although DD-I is inherited as an autosomal dominant disorder, a rare cases of autosomal recessive mode of inheritance were reported in two separate studies 13, 14. The first study of DD-I was reported in sons and daughters of healthy parents who were relatives 14. The second study of an autosomal recessive case of DD-I was reported in 4 affected individuals born from a healthy Moroccan parents who were also relatives 15.

There are several theories in the previous dental literature regarding the pathogenesis of DD, however, the exact etiology need to be elucidated 16, 17. The theory proposed by Logan et al 16 suggested that foci of degenerative changes occur in dental papillae leading to delayed growth and abnormal calcification with subsequent obliteration of the pulp chamber. Another theory suggested by Wesley et al 17 proposed that DD is due to an abnormal differentiation and/or function of odontoblasts resulting from unusual interaction between odontoblasts and ameloblasts. Moreover, it was suggested that ectopic dentin is formed within the dental papilla either due to the occurrence of an earlier ingrowth of the radicular epithelial sheath 18 or due to displacement and proliferation of the internal cells of the developing dental organ in the dental papilla 7.

Microscopically, the enamel and the immediately subjacent dentin appear normal. Deeper layers of dentin, however, show atypical atubular patterns, with amorphous and irregular organization that lacks tubules. On the pulpal side of the normal-appearing mantle of dentin, globular or nodular masses of abnormal dentin are seen 1.

In the present case the teeth appeared clinically normal in shape and size except for the crown of upper 1st incisors which appears lager than normal. The radiographic findings include atypical pulp morphology that extended to radicular pulp. These changes were evident in single rooted teeth. Presence of radiopaque globules within the pulp chambers supports the diagnosis of DD-II (coronal type). However, other findings in the present study such as short, blunted or conical roots of lower incisors and obliteration of pulp chamber are features of DD-I (radicular dysplasia). Such findings supported our diagnosis of DD-II which also possess radiographic features of DD-I. To the best of our knowledge, this is the second case in English literature. The first case was reported in 1978 by Clio, et al, which was in accordance with our findings 10. This report suggested that DD type III may be considered for future classification of DD. Further studies are recommended in order to get more features of such cases.

References

[1]  Joseph AR, James JS, and Richard CKJ. Oral Pathology: Clinical Pathologic Correlations, 7th ed, Elsevier Saunders Publisher, Missouri 2017, 383-4.
In article      
 
[2]  Fulari SG, Tambake DP. Rootless teeth: Dentin dysplasia type I. Contemp Clin Dent 2013; 4:520-2.
In article      
 
[3]  Kumar N, Ansari N, Shrivastava H, Patsa S, Ray J. Dentin Dysplasia Type I: Case Series and Review Of The Literature. Int J Dent Health Sci 2015; 2: 198-202
In article      
 
[4]  Ballschmiede G. Dissertation, Berlin, 1920. Quoted in Herbst E, Apffelstaedt M, editors. Malformations of the Jaws and Teeth. New York: Oxford University Press; 1930.
In article      
 
[5]  Rushton MA. A case of dentinal dysplasia. Guy’s Hosp Rep 1939; 89: 369-73.
In article      
 
[6]  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: 543-553
In article      
 
[7]  Witkop CJ Jr. Hereditary defects of dentin. Dent Clin North Am 1975; 19: 25-45.
In article      
 
[8]  Brenneise CV, Conway KR. Dentin dysplasia, type II: report of 2 new families and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1999; 87: 752-5.
In article      
 
[9]  Carroll M.K., Duncan W.K., Perkins T.M. Dentin dysplasia: Review of the literature and a proposed subclassification based on radiographic findings. Oral Surg, Oral Med, Oral Pathol 1991; 72: 119-25.
In article      
 
[10]  Ciola B, Bahn SL, Goviea GL. Radiographic manifestations of an unusual combination types I and type II dentin dysplasia, Oral Surg Oral Med Oral Pathol 1978; 45: 317-22.
In article      
 
[11]  de La Dure-Molla M, Philippe Fournier B, Berdal A. Isolated dentinogenesis imperfecta and dentin dysplasia: revision of the classification. Eur J Hum Genet. 2015; 23: 445-51.
In article      
 
[12]  Song YL, Wang CN, Fan MW, Su B, Bian Z. Dentin phosphoprotein frameshift mutations in hereditary dentin disorders and their variation patterns in normal human population. J Med Genet. 2008; 45: 457-64.
In article      
 
[13]  Beattie ML, Kim J-W, Gong S-G, Murdoch-Kinch CA, Simmer JP, Hu JC-C. Phenotypic variation in dentinogenesis imperfecta/dentin dysplasia linked to 4q21. J Dent Res 2006; 85: 329-33.
In article      
 
[14]  Bloch-Zupan, X. Jamet, C. Etard, V. Laugel, J. Muller, et al. Homozygosity mapping and candidate prioritization identify mutations, missed by whole-exome sequencing, in SMOC2, causing major dental developmentaldefects, Am. J. Hum. Genet. 2011; 89: 773-81.
In article      
 
[15]  Cherkaoui JI, M. El Alloussi, F.Z. Laarabi, A. Bouhouche, etal. Inhabitual autosomal recessive form of dentin dysplasia type I in a large consanguineous Moroccan family Eur J Med Genet 2013; 56: 442-4.
In article      
 
[16]  Logan J, Becks H, Silverman S Jr, Pindborg JJ. Dentinal dysplasia. Oral Surg 1962; 15: 317-33.
In article      
 
[17]  Sauk JJ, Lyons HW, Trowbridge HO, Witkop CJ Jr. An electron optic analysis and explanation for the etiology of dentinal dysplasia. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 972; 33: 763-71.
In article      
 
[18]  Wesley RK, Wysocki GP, Mintz SM, et al. Dentin dysplasia type I. Oral Surg 1976; 41: 516-24.
In article      
 

Published with license by Science and Education Publishing, Copyright © 2020 Hamdy AM Metwaly and Faraj Alotaiby

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Cite this article:

Normal Style
Hamdy AM Metwaly, Faraj Alotaiby. Dentine Dysplasia Type II: Report of a Rare Case with Radiographic Criteria of Type I. International Journal of Dental Sciences and Research. Vol. 8, No. 4, 2020, pp 87-90. http://pubs.sciepub.com/ijdsr/8/4/1
MLA Style
Metwaly, Hamdy AM, and Faraj Alotaiby. "Dentine Dysplasia Type II: Report of a Rare Case with Radiographic Criteria of Type I." International Journal of Dental Sciences and Research 8.4 (2020): 87-90.
APA Style
Metwaly, H. A. , & Alotaiby, F. (2020). Dentine Dysplasia Type II: Report of a Rare Case with Radiographic Criteria of Type I. International Journal of Dental Sciences and Research, 8(4), 87-90.
Chicago Style
Metwaly, Hamdy AM, and Faraj Alotaiby. "Dentine Dysplasia Type II: Report of a Rare Case with Radiographic Criteria of Type I." International Journal of Dental Sciences and Research 8, no. 4 (2020): 87-90.
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  • Figure 3. Periapical radiograph of the A) upper left molars and B) lower right molars showing pulp chambers occluded with radiopaque globules and reduction of pulp size
  • Figure 4. A periapocal radiograph of upper central and lateral incisors showing large pulp chambers (thistle-tube appearance) containing oval shaped radiopaque pulp globules
[1]  Joseph AR, James JS, and Richard CKJ. Oral Pathology: Clinical Pathologic Correlations, 7th ed, Elsevier Saunders Publisher, Missouri 2017, 383-4.
In article      
 
[2]  Fulari SG, Tambake DP. Rootless teeth: Dentin dysplasia type I. Contemp Clin Dent 2013; 4:520-2.
In article      
 
[3]  Kumar N, Ansari N, Shrivastava H, Patsa S, Ray J. Dentin Dysplasia Type I: Case Series and Review Of The Literature. Int J Dent Health Sci 2015; 2: 198-202
In article      
 
[4]  Ballschmiede G. Dissertation, Berlin, 1920. Quoted in Herbst E, Apffelstaedt M, editors. Malformations of the Jaws and Teeth. New York: Oxford University Press; 1930.
In article      
 
[5]  Rushton MA. A case of dentinal dysplasia. Guy’s Hosp Rep 1939; 89: 369-73.
In article      
 
[6]  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: 543-553
In article      
 
[7]  Witkop CJ Jr. Hereditary defects of dentin. Dent Clin North Am 1975; 19: 25-45.
In article      
 
[8]  Brenneise CV, Conway KR. Dentin dysplasia, type II: report of 2 new families and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1999; 87: 752-5.
In article      
 
[9]  Carroll M.K., Duncan W.K., Perkins T.M. Dentin dysplasia: Review of the literature and a proposed subclassification based on radiographic findings. Oral Surg, Oral Med, Oral Pathol 1991; 72: 119-25.
In article      
 
[10]  Ciola B, Bahn SL, Goviea GL. Radiographic manifestations of an unusual combination types I and type II dentin dysplasia, Oral Surg Oral Med Oral Pathol 1978; 45: 317-22.
In article      
 
[11]  de La Dure-Molla M, Philippe Fournier B, Berdal A. Isolated dentinogenesis imperfecta and dentin dysplasia: revision of the classification. Eur J Hum Genet. 2015; 23: 445-51.
In article      
 
[12]  Song YL, Wang CN, Fan MW, Su B, Bian Z. Dentin phosphoprotein frameshift mutations in hereditary dentin disorders and their variation patterns in normal human population. J Med Genet. 2008; 45: 457-64.
In article      
 
[13]  Beattie ML, Kim J-W, Gong S-G, Murdoch-Kinch CA, Simmer JP, Hu JC-C. Phenotypic variation in dentinogenesis imperfecta/dentin dysplasia linked to 4q21. J Dent Res 2006; 85: 329-33.
In article      
 
[14]  Bloch-Zupan, X. Jamet, C. Etard, V. Laugel, J. Muller, et al. Homozygosity mapping and candidate prioritization identify mutations, missed by whole-exome sequencing, in SMOC2, causing major dental developmentaldefects, Am. J. Hum. Genet. 2011; 89: 773-81.
In article      
 
[15]  Cherkaoui JI, M. El Alloussi, F.Z. Laarabi, A. Bouhouche, etal. Inhabitual autosomal recessive form of dentin dysplasia type I in a large consanguineous Moroccan family Eur J Med Genet 2013; 56: 442-4.
In article      
 
[16]  Logan J, Becks H, Silverman S Jr, Pindborg JJ. Dentinal dysplasia. Oral Surg 1962; 15: 317-33.
In article      
 
[17]  Sauk JJ, Lyons HW, Trowbridge HO, Witkop CJ Jr. An electron optic analysis and explanation for the etiology of dentinal dysplasia. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 972; 33: 763-71.
In article      
 
[18]  Wesley RK, Wysocki GP, Mintz SM, et al. Dentin dysplasia type I. Oral Surg 1976; 41: 516-24.
In article