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CASE REPORT |
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Year : 2013 | Volume
: 2
| Issue : 2 | Page : 127-129 |
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Oligodontia: A rare case report and literature review
Vijaykumar G Biradar, Santosh I Hugar, Surekha V Biradar
Department of Oral Pathology, MIDSR Dental College, Hospital and Research Centre, Latur, Maharashtra, India
Date of Web Publication | 10-Sep-2013 |
Correspondence Address: Vijaykumar G Biradar Department of Oral Pathology, MIDSR Dental College, Hospital and Research Centre, Latur - 413 512, Maharashtra India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/2278-0521.117918
Dental agenesis is the most common developmental anomaly in humans which can occur in an isolated fashion, or as a part of syndrome. Isolated cases of missing teeth can be familial or sporadic in nature. In the literature, many terms are used to describe missing teeth like oligodontia, anodontia, aplasia of teeth, congenitally missing teeth, absence of teeth, agenesis of teeth and lack of teeth. Oligodontia is defined as the developmental absence of six teeth or more, excluding third molars. It can be isolated or as a part of a syndrome such as in ectodermal dysplasia. The present case report describes agenesis of lower anterior permanent teeth which are non-familial and with no apparent systemic abnormalities. Keywords: Agenesis of teeth, non-familial, oligodontia
How to cite this article: Biradar VG, Hugar SI, Biradar SV. Oligodontia: A rare case report and literature review. Saudi J Health Sci 2013;2:127-9 |
Introduction | |  |
Tooth agenesis (MIM# 106600), the congenital absence of one or more permanent teeth, is a common human anomaly (Pemberton, et al., 2005). In most populations, the reported prevalence of permanent tooth agenesis, excluding third molars, varies from 2.2-10.1% (Polder, et al., 2004). In the majority of cases, persons are missing only one tooth (Daugaard-Jensen, et al., 1997). The prevalence becomes progressively smaller as the number of missing teeth increases. Agenesis of more than two teeth occurs in approximately 1% of the population (Polder, et al., 2004). [1] The purpose of this article is to report a rare case of congenitally missing all lower anterior teeth and review the literature.
Case Report | |  |
A 21 year old female visited to the department of oral and maxillofacial pathology along with her parents for seeking treatment for unpleasant smile and replacement of missing teeth. On examination, the patient gave no significant past medical and dental history, neither there was any abnormality detected on general examination suggestive of any syndromes. The family history did not reveal any multiple missing teeth. Soft tissues were normal. Patient was born to non-consanguineous parents. There was no history of any infection or trauma to the anterior region. Patient mother gave a history of presence of milk teeth in the lower anterior region but after their exfoliation, permanent teeth did not erupt. Patient was healthy with no relevant medical and family history. Suspecting the congenital absence of permanent teeth panoramic radiograph was taken which showed presence of 18 teeth. Teeth present were 13-15, 17, 23-27, 34, 35, 37, 38, 44-48 [Figure 1], [Figure 2] and [Figure 3]. Queries revealed that missing lower 6 teeth like 31-33, 41-43 were not extracted, were absent since childhood. The other missing teeth like 11, 12, 16, 21, 22, 36 were extracted due to caries. Third molar tooth buds in maxillary arch were absent in comparison to mandibular arch. Based on clinical and radiological examinations, diagnosis of nonsyndromic oligodontia was made. The condition was explained to the patient and treatment plan was discussed. | Figure 1: Clinical photograph showing mandibular and maxillary missing teeth
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 | Figure 3: Orthopantomograph showing congenitally missing 31, 32, -33, 41, 42-43
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Discussion | |  |
According to a 1996 consensus conference on oral implants in young patients, the following definitions are used; Hypodontia is defined as the absence of one to five permanent teeth, while the term oligodontia refers to the absence of six or more permanent teeth and 'anodontia' to the absence of all permanent teeth. [2] Oligodontia as well as hypodontia (lack of one or more permanent teeth) are highly heritable conditions associated with mutations in the AXIN2, MSX1, PAX9, EDA, and EDAR genes.[3] The incidence of oligodontia is reported to vary from 0.08-0.16%. The pattern of tooth absence is influenced by the gene affected, as well as the type of mutations within the specific gene. [4] Although several potential and verified environmental factors in tooth agenesis have been identified, genetic defects play a major role in the etiology (Graber, 1978; Burzynski and Escobar, 1983; Vastardis, 2000). So far, researchers have identified genetic defects that cause tooth agenesis either as a sole anomaly (isolated or non-syndromic) or as a part of multiple congenital anomalies (syndromic). One gene associated with syndromic tooth agenesis is the EDA gene (MIM# 300451), which underlies X-linked hypohidrotic ectodermal dysplasia (XLHED; MIM# 305100). Non-syndromic tooth agenesis has wide phenotypic heterogeneity and is classified as either sporadic or familial, which can be inherited in an autosomal-dominant, autosomal-recessive, or X-linked mode (Burzynski and Escobar, 1983). [1] Abnormal gene function may also disrupt specific signaling pathways involved in tooth development, resulting not only in abnormal tooth number but also in abnormal tooth size and or shape. Several genes and disruptions in the molecular pathways are suggested in causing defects affecting all teeth in a majority of cases presenting as oligodontia or anodontia. List of the genes and molecular pathway involved in tooth agenesis are shown in [Table 1]. [5] | Table 1: List of the genes and molecular pathway involved in tooth agenesis are
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The present case reported absence of lower anterior permanent teeth and the use of panoramic radiography was recommended, together with clinical examination for the detection or confirmation of dental development and performing the diagnosis of oligodontia. In the reported case, panoramic radiography revealed absence of twelve permanent teeth. Out of which lower six anterior teeth were congenitally missing with no other associated dental anomalies like delayed formation and eruption of teeth, reduction in tooth size and form, ectopic eruption of teeth, rotation of teeth except for enamel hypoplasia. Congenital missing teeth may create dental and facial disfigurement, which can lead to social withdrawal, especially in adolescent years. [6] Prosthodontic treatment of oligodontia patients is, therefore, important for functional, esthetic and psychological reasons. [7] Treatment of such patients requires a fully integrated interdisciplinary approach of orthodontists, oral and maxillofacial surgeons and prosthodontist. [8] A number of factors must be taken into account at the time of treatment planning, which include age of the patient, number and condition of retained teeth, number of missing teeth, condition of supporting tissues, the occlusion and interocclusal space. [9] Common methods of treatment employed include, pre-restorative orthodontics which is frequently required to move teeth to a favorable position, restoration with removable and fixed partial dentures and restoration with implant supported prosthesis. Increased experience with oral implants and supplementary augmentation techniques has created new option for treatment of patients with oligodontia. [2],[7],[9] In the present case, impression of mandibular arch was made and the patient was asked to come back in the next visit. Unfortunately the patient did not turn up. Such an approach becomes a major hindrance to successful treatment of such cases.
Conclusion | |  |
Current understanding of rare conditions like oligodontia may be enhanced by reporting of such cases. Patients of oligodontia generally require a multidisciplinary approach. Thus, the cases should be evaluated carefully by clinicians and early diagnosis and treatment planning should be made for appropriate treatment modalities and to minimize the complication of these dental anomalies.
References | |  |
1. | Suda N, Ogawa T, Kojima T, Saito C, Moriyama K. Non- syndromic oligodontia with a novel mutation of PAX9. J Dent Res 2011;90:382-6.  [PUBMED] |
2. | Durstberger G, Celar A, Watzek G. Implant-surgical and prosthetic rehabilitation of patients with multiple dental aplasia: A clinical report. Int J Oral Maxillofac Implants 1999;14:417-23.  [PUBMED] |
3. | Bergendal B, Klar J, Stecksén-Blicks C, Norderyd J, Dahl N. Isolated oligodontia associated with mutations in EDARADD, AXIN2, MSX1 and PAX9 genes. Am J Med Genet A 2011;155A: 1616-22.  |
4. | Singer SL, Henry PJ, Lander ID. A treatment planning classification for oligodontia. Int J Prosthodont 2010;23:99-106.  [PUBMED] |
5. | De Coster PJ, Marks LA, Martens LC, Huysseune A. Dental agenesis: Genetic and clinical perspectives. J Oral Pathol Med 2009;38:1-17.  [PUBMED] |
6. | Giray B, Akça K, Iplikçioðlu H, Akça E. Two-year follow-up of a patient with oligodontia treated with implant and tooth-supported fixed partial dentures: A case report. Int J Oral Maxillofac Implants 2003;18:905-11.  |
7. | Finnema KJ, Raghoebar GM, Meijer HJ, Vissink A. Oral rehabilitation with dental implants in oligodontia patients. Int J Prosthodont 2005;18:203-9.  [PUBMED] |
8. | Worsaae N, Jensen BN, Holm B, Holsko J. Treatment of severe hypodontia-oligodontia-an interdisciplinary concept. Int J Oral Maxillofac Surg 2007;36:473-80.  [PUBMED] |
9. | Dhanrajani PJ. Hypodontia: Etiology, clinical patterns, and management. Quintessence Int 2002;33:294-302.  [PUBMED] |
[Figure 1], [Figure 2], [Figure 3]
[Table 1]
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