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| CASE REPORT |
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| Year : 2015 | Volume
: 8
| Issue : 3 | Page : 337-339 |
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Nonsyandromic oligodontia: A rare case report
Mayank Chaturvedi1, Jigar Thakkar1, Rekha Thaddanee2, Ajeet Kumar Khilnani3
1 Department of Dentistry, GMERS Medical College and Hospital, Dharpur, Patan, Gujarat, India
2 Department of Pediatrics, GMERS Medical College and Hospital, Dharpur, Patan, Gujarat, India
3 Department of ENT, GMERS Medical College and Hospital, Dharpur, Patan, Gujarat, India
| Date of Web Publication | 15-May-2015 |
Correspondence Address:
Ajeet Kumar Khilnani
GMERS Medical College and Hospital, Dharpur, Patan, Gujarat
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0975-2870.157079
Dental agenesis is the most common developmental anomaly in humans, which can be either in the form of anodontia, oligodontia, or hypodontia. Oligodontia can occur either as an isolated finding or as part of a syndrome. Nonsyndromic oligodontia is rare. Management requires integrated multidisciplinary approach. Prompt intervention improves quality of life. Here, we report a case of isolated, nonsyndromic oligodontia in a 13-year-old female who allegedly had complete set of primary teeth but failed to develop complete permanent dentition. No other clinical feature, except oligodontia, was present that could suggest any syndromic association. Keywords: Anodontia, dental agenesis, hypodontia, oligodontia
How to cite this article:
Chaturvedi M, Thakkar J, Thaddanee R, Khilnani AK. Nonsyandromic oligodontia: A rare case report. Med J DY Patil Univ 2015;8:337-9 |
| Introduction | |  |
Oligodontia refers to a condition where more than five permanent teeth are missing, excluding third molars or wisdom teeth. Usually oligodontia is a part of a syndrome called ectodermal dysplasia, where other skin appendages such as nails, teeth, and skin are also affected. 150 variants of ectodermal dysplasia are known. [1] Nonsyndromic oligodontia is a rare occurrence, and very few cases are reported in the literature. One missing teeth is the most common anomaly of tooth agenesis, seen in 80% of total cases, 1-5 missing teeth is seen in <10% of cases while more than 5 missing teeth is seen in <1% cases. [2] Diagnosis is based on history, clinical examination and radiology. Orthopantomograph and lateral skull radiograph are useful to view the development of primary and permanent tooth buds and jaw bones. A multidisciplinary approach is required for complete oral rehabilitation, which includes orthodontic treatment, surgical intervention, prosthodontic rehabilitation and psychological counseling.
| Case Report | | |
A 13-year-old female patient visited the Department of Dentistry with the complaints of missing teeth and inability to chew. She was born with low birth weight and had two elder sisters which had no similar or any other complaints. Patient gave the history of early shedding of her primary teeth without the eruption of permanent teeth. Intraoral examination revealed few discolored retained deciduous teeth and one permanent canine and reduced alveolar ridge height [Figure 1]. Extra-oral examination revealed concave facial profile with hypoplastic maxilla and relative prognathic mandible [Figure 2].  | Figure 2: Lateral view showing hypoplastic maxilla and relative prognathic mandible
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Orthopantomograph showed presence of two permanent maxillary molars present on both sides and one canine (23) and few deciduous teeth (52, 53, 62 and 71) [Figure 3]. Lateral skull radiograph revealed decreased alveolar height of maxilla and mandible and complete absence of rest of permanent teeth [Figure 4]. Skin, hair, and nails were normal. No other family member suffered from a similar condition. Based on the above findings clinical diagnosis of nonsyndromic oligodontia was made. Total prosthodontic rehabilitation was planned and explained to the patient and impressions of maxillary, and mandibular ridges were made on first visit. However, the patient did not turn up for completion of treatment. | Figure 3: Orthopantomograph showing reduced alveolar ridge and unerrupted permanent teeth
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| Discussion | | |
Isolated oligodontia is a rare disorder, which represents the absence of six or more permanent dentitions. [3] It is sometimes referred to as partial anodontia or severe hypodontia. [4] The prevalence of oligodontia has been reported as 0.3%. [5] Although oligodontia is genetically conditioned, the presence of external and internal factors, such as X-ray therapy, particular medications (anticancer chemotherapeutic agents), infectious diseases (rubella, candida), endocrine disorders (hypoparathyroidism and pseudohypoparathyroidism) and intrauterine disorders cannot be excluded. [6] Nonsyndromic oligodontia has been shown to be inherited as an autosomal dominant trait. [7] At present, the list of genes involved in human nonsyndromic oligodontia includes not only those encoding a signaling molecule (transforming growth factor-alpha) and transcription factors (MSX1 and PAX9) that play critical roles during early craniofacial development, but also genes coding for a protein involved in canonical Wnt signaling (AXIN2), and a transmembrane receptor of fibroblast growth factors receptor 1. [8] Oligodontia is often associated with conical teeth, microdontia, increased freeway space and retention of deciduous teeth, as was in our case.
Hypodontia can also be seen in people with cleft lip and palate. Missing teeth can lead to functional disability as well as social maladjustments, especially in adolescent years. [9] Hence, a prompt prosthodontic treatment can lead to improved functional, cosmetic and psychological development of the patient. Treatment of oligodontia requires an integrated multidisciplinary approach involving orthodontists, maxillofacial surgeons, and prosthodontists. Treatment options depend on the severity of oligodontia, condition of the patient, condition of retained teeth and number of missing teeth. Various treatment options include prerestorative orthodontics, restoration with removable and fixed partial dentures and restoration with implant supported prosthesis. There is increasing evidence that the implant supported prostheses are the preferred choice for patients with oligodontia. Orthodontic treatment should be aimed at improving the position of erupted teeth, guiding erupting permanent teeth and reducing the overbite. [10]
| Conclusion | | |
Every case of oligodontia should be reported so as to enhance the understanding of this rare entity. Oligodontia can lead to functional, esthetic and social loss to a patient; hence a prompt treatment can lead to improved quality of life.
| References | | |
| 1. | Pinheiro M, Freire-Maia N. Ectodermal dysplasias: A clinical classification and a causal review. Am J Med Genet 1994;53:153-62.  |
| 2. | De Coster PJ, Marks LA, Martens LC, Huysseune A. Dental agenesis: Genetic and clinical perspectives. J Oral Pathol Med 2009;38:1-17. |
| 3. | 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. |
| 4. | Goya HA, Tanaka S, Maeda T, Akimoto Y. An orthopantomographic study of hypodontia in permanent teeth of Japanese pediatric patients. J Oral Sci 2008;50:143-50. |
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| 6. | Créton MA, Cune MS, Verhoeven W, Meijer GJ. Patterns of missing teeth in a population of oligodontia patients. Int J Prosthodont 2007;20:409-13. |
| 7. | Klein ML, Nieminen P, Lammi L, Niebuhr E, Kreiborg S. Novel mutation of the initiation codon of PAX9 causes oligodontia. J Dent Res 2005;84:43-7. |
| 8. | Sharma S, Bedi S. Dystrophic epidermolysis bullosa associated with non-syndromic hypodontia. Indian Dermatol Online J 2013;4:296-9. [ PUBMED]  |
| 9. | Giray B, Akça K, Iplikçioglu 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. |
| 10. | Jepson NJ, Nohl FS, Carter NE, Gillgrass TJ, Meechan JG, Hobson RS, et al. The interdisciplinary management of hypodontia: Restorative dentistry. Br Dent J 2003;194:299-304. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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