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CASE REPORT
Year : 2018  |  Volume : 36  |  Issue : 2  |  Page : 213-215
 

Dentin dysplasia type 1 - clinical management dilemmas: A case report of first-generation sufferers


1 Department of Pedodontics and Preventive Dentistry, GDC, Amritsar, Punjab, India
2 Department of Genetics, Guru Nanak Dev University, Amritsar, Punjab, India

Date of Web Publication2-Jul-2018

Correspondence Address:
Samita Gumber
Department of Pedodontics and Preventive Dentistry, GDC, S. S. S. S. Chownk, Amritsar - 143 001, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JISPPD.JISPPD_1127_17

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   Abstract 


Dentine Dysplasia is a rare genetic condition. The treatment options and dilemmas associated with the condition remain undiscovered so far. This article highlights the variations in traits and challenges faced in the treatment of the cases.


Keywords: Clinical management, dentin dysplasia, dilemmas


How to cite this article:
Grewal N, Gumber S, Kaur A, Kaur N. Dentin dysplasia type 1 - clinical management dilemmas: A case report of first-generation sufferers. J Indian Soc Pedod Prev Dent 2018;36:213-5

How to cite this URL:
Grewal N, Gumber S, Kaur A, Kaur N. Dentin dysplasia type 1 - clinical management dilemmas: A case report of first-generation sufferers. J Indian Soc Pedod Prev Dent [serial online] 2018 [cited 2019 Jul 20];36:213-5. Available from: http://www.jisppd.com/text.asp?2018/36/2/213/235671





   Introduction Top


Dentin dysplasia is a rare developmental disorder affecting the dentin formation. Due to the rare occurrence with the prevalence rate of 1:100,000[1],[2] and lack of literature, the dilemmas to treat such cases are still unfolded which makes this condition a real challenge for dentist to treat.[3],[4],[5],[6],[7] The undermentioned cases highlight the variations in clinical and radiographic features and management dilemmas faced in treating such patients.


   Case Reports Top


Case 1 (proband)

A patient aged 17 years/male reported to the Department of Pedodontics and Preventive Dentistry, GDC, Amritsar, with the chief complaint of unesthetic appearance and history of early loss of primary teeth and rapid attrition of permanent teeth. Family history revealed that three more children in the family were affected with the similar condition. Intraoral examination of proband revealed oligodontia, attrition of 13, 12, 11, 21, 22, 23, 33, 32, 31, 41, 42, and 43, and mobility of 12.41 (Miller's Grade 1). Radiographic examination (orthopantomogram [OPG]) [Figure 1] revealed multiple unerupted teeth and wide variations in root morphology. Multiple periapical radiolucencies were present with respect to 12, 21, 23, 31, 32, and 41. No extra oral abnormality was detected.
Figure 1: Orthopantomogram showing the radiographic findings of Case 1

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Case 2 - sibling (16 years/female)

The case presented with similar features as proband. Hypodontia (16, 21, 26, 31, 32, 36, 37, 41, 42, 45, 46, 47, 53, 54), attrition (16, 53, 54, 63, 65, 26, 31, 32, 36, 41, 42, 46), and an intraoral sinus with respect to 21 were present intraorally. OPG [Figure 2] revealed tooth buds without root formation with respect to all unerupted teeth and periapical radiolucencies with respect to 21, 31, 32, 41 and 42.
Figure 2: Orthopantomogram showing the radiographic findings of Case 2

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Case 3 - sibling (14 years/male)

This case presented with wider variations. Hypodontia (16, 11, 26, 36, 32, 32, 41, 42, 46, 53, 55, and 63), attrited (55, 53, 63, 31, 32, 41, and 42), Angle's Class 3 molar relation with collapsed anterior bite, and severe bone resorption in mandibular posterior region were present. OPG [Figure 3] revealed tooth buds present with respect to all unerupted teeth and multiple periapical radiolucencies.
Figure 3: Orthopantomogram showing the radiographic findings of Case 3

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Case 4 - cousin (14 years/female)

Hypodontia 16, 11, 21, 26, 31, 32, 36, 41, 42, 43, 46, 64, 74, and 75, attrition (11, 31, 32, 41, 42, and 43), Angle's Class 3 molar relation with collapsed anterior bite, and severe bone resorption in mandibular posterior region. OPG [Figure 4] revealed tooth buds present with respect to all unerupted teeth and complete root formation was seen with respect to all erupted teeth which increased the prognosis of the treatment. Rapid changes in the eruption of tooth buds were seen in this case.
Figure 4: Orthopantomogram showing the radiographic findings of Case 4

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Patients were referred to orthopedician, endocrinologist, and a physician to rule out any syndromic association. No positive findings were reported by any of the specialists.

A pedigree chart was prepared with the help of a geneticist which revealed that the patients were maternal as well as paternal cousins and no other family member of the earlier generation was affected. Hence, the patients became the “ first-generation sufferers.”

Chromosomal analysis

No abnormality in the size, shape, and count of chromosomes was reported by the geneticist.

Histopathological analysis

Histopathological analysis of ground sections of extracted permanent teeth revealed the presence of patchy stained dentin with irregularly formed dentinal tubules and osteodentin with entrapped odontoblasts [Figure 5].
Figure 5: Histopathological picture of the affected permanent tooth (21) of Case 2

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Based on clinical, radiographic, histological, karyotype findings and pedigree analysis, the condition was diagnosed as dentin dysplasia Type 1.

Treatment and dilemmas

The ultimate treatment in such cases will be implants. However, the option of implants was restricted due to the age of the patients. Multiple interim treatments were performed so as to preserve the bone as much as possible and hence attempts were made to preserve the natural teeth but the teeth with poor prognosis were extracted. Root canal treatments and interim restorations were done. The biomechanical preparation had to be done with great caution to avoid iatrogenic errors of roots with abnormal morphology. Crown lengthening was done in mandibualr anteriors as their crowns were severally attrited. Since their prognosis could not be predicted, acrylic crowns were chosen as an interim treatment. Stainless steel crowns were cemented to prevent further attrition of mandibular molars followed by interim conventional removable partial dentures with respect to unerupted premolars. The erratic eruption of teeth was unpredictable. The conventional dentures created interference in the path of eruption of teeth and the bony bulges created by the erupting teeth lead to pain in the insertion of dentures. Hence, new interim flexible removable partial dentures were made for the patients later on.


   Discussion Top


Dentin dysplasia may vary in clinical expression, either in isolation or with other congenital anomalies, presented in such severe form as in this case report is a challenge to maintain the form and function of teeth and meet long-term need of patients. Morris and Augsburger described dentin dysplasia (type 1) to be associated with sclerotic bone which could be an explanation to the delayed eruption of the tooth buds. However, the erratic eruption of these buds could only be explained on the basis of early loss of gubernacular cord and any other physiological guidance to these buds.

All the cases are on a constant follow-up of every 3 months for the adjustment of dentures according to the eruption status of teeth in the interim period. Root canal treated teeth and prosthesis are clinically and radiographically asymptomatic till date. Under the limitations of the treatment provided to the patients, more options will be explored such as whole ridge augmentation with bone grafts followed by implants after completion of their growth.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Kim JW, Simmer JP. Hereditary dentin defects. J Dent Res 2007;86:392-9.  Back to cited text no. 1
[PUBMED]    
2.
Rushton MA. A case of dentinal dysplasia. Guys Hosp Rep 1939;89:369-73.  Back to cited text no. 2
    
3.
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. 3
[PUBMED]    
4.
Witkop CJ Jr. Hereditary defects of dentin. Dent Clin North Am 1975;19:25-45.  Back to cited text no. 4
[PUBMED]    
5.
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. 5
    
6.
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. 6
    
7.
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. 7
[PUBMED]    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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