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CASE REPORT
Year : 2015  |  Volume : 4  |  Issue : 4  |  Page : 282-285

Radicular dentin dysplasia: A case report


Department of Pedodontics, Government Dental College and Hospital, Hyderabad, Telangana, India

Date of Web Publication14-Dec-2015

Correspondence Address:
I Hemachandrika
Asst. Professor, Department of Pedodontics, Govt. Dental College and Hospital, Afzalgunj, Hyderabad
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2277-8632.171748

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  Abstract 

Dentin dysplasias form a rare group of hereditary dentin malformations characterized clinically by normal appearing crowns but extreme mobility of teeth due to defective root formation. Both deciduous and permanent teeth may be affected. Delayed eruption and malocclusion may be associated features. This disorder is broadly classified into two types: Type I (radicular dentin dysplasia) and Type II (coronal dentin dysplasia). Type I is more common and is known by the term "rootless teeth." The difficulties encountered with rootless teeth are obvious: there is no anchorage, there is compromised blood supply, deranged homeostatic mechanisms of the tooth as well as overload of masticatory and other stresses on the crowns leading to their early exfoliation. This paper discusses a case report of Type I dentin dysplasia and attempts to project the existing data in a concise form.

Keywords: Complete denture, dentin dysplasia, rootless teeth


How to cite this article:
Hemachandrika I, Reddy J S, Suhasini K, Tarasingh P. Radicular dentin dysplasia: A case report. J NTR Univ Health Sci 2015;4:282-5

How to cite this URL:
Hemachandrika I, Reddy J S, Suhasini K, Tarasingh P. Radicular dentin dysplasia: A case report. J NTR Univ Health Sci [serial online] 2015 [cited 2020 Apr 4];4:282-5. Available from: http://www.jdrntruhs.org/text.asp?2015/4/4/282/171748


  Introduction Top


Dentin dysplasia is a rare disturbance of dentin formation characterized by a normal enamel but atypical dentin formation with abnormal pulpal morphology. It has been described as "an apparently hereditary disorder of dentin formation, marked by normal appearance of coronal dentin, associated with pulpal obliteration, faulty root formation, and a tendency for peripheral lesions without obvious cause." [1]

This disorder was first described in 1920 under the term "rootless teeth." Rushton [1] termed it "dentin dysplasia." Shields et al. (1973) [2] divided inherited dentin defects into five types: three types of dentinogenesis imperfecta and two types of dentin dysplasias. They also classified dentin dysplasias into two main classes based on clinical and radiographic appearances:

Type I - Dentin dysplasia

Type II - Anomalous dysplasia of the dentin

Witkop [3] later termed Type I as "radicular dentin dysplasia" and Type II as "coronal dentin dysplasia" to indicate the parts of the teeth that are primarily involved. Scola and Watts [4] suggested a subclassification of Type I into total and subtotal types while Carrol et al. [5] provided an extensive classification based on radiographic findings into Types Ia, Ib, Ic, and Id. However, a simpler subclassification into either mild or severe may be more clinically relevant.

Both the types have an autosomal dominant mode of inheritance. [2],[3] The prevalence is said to be 1 in a population of 100,000. [6]


  Case Report Top


A 14-year-old male patient reported to our department complaining of missing teeth and mobility of his remaining teeth. The medical history did not reveal any evidence of bone disease or unusual bone fracture. Dental history revealed that the patient's teeth were frequently becoming mobile and shedding off on their own since they started erupting at around 7 years of age. His primary dentition was apparently normal. Family history was also nonsignificant and there was no history of consanguinity. On oral examination [Figure 1], the patient had 15 teeth present. All the teeth exhibited mobility to a minimum grade II. The upper right first premolar was rotated and there was a generalized mottling of the enamel. A panoramic radiograph [Figure 2] showed that the erupted teeth had absolutely no evidence of root formation. The erupting third molars also showed no evidence of root formation. Only a shell of coronal dentin was evident in all the teeth, thus appearing as floating teeth. Pulp chambers of erupted teeth were rudimentary or obliterated [Figure 3] and [Figure 4]. Based on the clinical and radiographic findings, a diagnosis of Type I or radicular dentin dysplasia was made. All the teeth were extracted [Figure 5] and the patient was advised to come at weekly intervals for monitoring the healing of the arches. As the teeth were rootless, healing of the arches occurred within 1 week [Figure 6] and [Figure 7]. A step-by-step procedure for denture fabrication from preliminary impression to try-in was followed and a complete denture was delivered to the patient [Figure 8].
Figure 1: Preoperative photographs showing missing teeth and teeth present

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Figure 2: OPG showing rootless permanent teeth

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Figure 3: IOPA showing rootless and pulpless shell-like 34, 35, 36

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Figure 4: IOPA showing rotated 14 and rootless 14, 16

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Figure 5: Photograph of extracted teeth

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Figure 6: Postoperative photograph showing healed upper arch after extraction

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Figure 7: Postoperative photograph showing healed lower arch after extraction

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Figure 8: Postoperative photograph with complete denture

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  Discussion Top


The present case depicts the most severe form of Type I dentin dysplasia. Though the family history was not significant, the clinical and radiographic features related to those existing in the literature for Type I dentin dysplasia, i.e., clinically normal crowns, short, conical, or absent roots leading to abnormal mobility and early exfoliation of teeth, total obliteration of pulp chambers except for a thin crescent-shaped pulpal remnant parallel to the cementoenamel junctions, and malposition of the teeth. [7]

The differential diagnosis for this disorder includes dentinogenesis imperfecta, syndromes such as Ellis-van Creveld syndrome, osteogenesis imperfecta associated with dentinogenesis imperfecta, systemic conditions such as tumoral calcinosis and brachio-skeleto-genital syndrome. [8]

The etiology of Type I dentin dysplasia remains a mystery. Sauk et al. [9] postulated that there was a defect in the epithelial component in which there is a premature invagination of the epithelial root sheath, and then a sequence of futile attempts to correct itself, resulting in a stunted root form, with an unusual whorl-like pattern of the dentin obliterating the pulp chamber.

Type II dentin dysplasia and Types II and III dentinogenesis imperfecta have been linked to defects in dentin sialophosphoprotein (DSPP), a gene encoding the major noncollagenous proteins of the dentin. They may represent increasing levels of severity of a single disease. (Beattie et al. 2006). [10] No genetic link could be established likewise for Type I dentin dysplasia.

There is no effective cure for this disorder and preventive care is of great importance. Increased mobility of teeth necessitates extraction, as patients experience difficulty in mastication but care should be taken to prevent the fracture of teeth during extraction.


  Conclusion Top


The psychological and social stigma faced by such patients is enormous, overriding the difficulty faced due to mobility of teeth. Hence, future dental research should focus more on the etiologic aspect of such diseases. It is hoped that advances in prenatal diagnosis and genetic engineering will prevent such disorders from occurring before birth itself.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Shafer WG, Hine MK, Levy BM. Textbook of Oral Pathology. 4 th ed. Philadelphia, USA: WB Saunders Company; 1993. p. 61-3.  Back to cited text no. 1
    
2.
Shields ED, Bixler D, el-kafrawy AM. A proposed classification or heritable human dentin defects with a description of a new entity. Arch Oral Biol 1973;18:543-53.  Back to cited text no. 2
[PUBMED]    
3.
Witcop Jr CJ. Hereditary defects of dentin. Dent Clin North Am 1975; 19:25-45.  Back to cited text no. 3
    
4.
Scola SM, Watts PG. Dentinal dysplasia type I. A subclassification. Br J Orthod 1987;14:175-9.  Back to cited text no. 4
[PUBMED]    
5.
O Carroll MK, Duncan WK, Perkins TM. Dentin dysplasia: Review of literature and a proposed subclassification based on radiographic findings. Oral Surg Oral Med Oral Pathol 1991;72:119-25.  Back to cited text no. 5
    
6.
Stewart RE, Prescott GH. Textbook of Oral Facial Genetics. St. Louis: CV Mosby Company; 1976. p. 237-9.  Back to cited text no. 6
    
7.
Seow WK, Shusterman S. Spectrum of dentin dysplasia in a family: Case report and literature review. Pediatr Dent 1994;16: 437-42.  Back to cited text no. 7
    
8.
Ghassem A, Reid JS. Dentinal dysplasia type I: Review of literature and report of a family. ASDC J Dent Child 1997;64:429-34.  Back to cited text no. 8
    
9.
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. 9
[PUBMED]    
10.
Beattie ML, Kim JW, Gong SG, Murdoch-Kinch CA, Simmer JP, Hu JC. Phenotypic variation in dentinogenesis imperfecta/dentin dysplasia linked to 4q21. J Dent Res 2006;85:329-33.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

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Case Report
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