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 Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 27  |  Issue : 3  |  Page : 437-440

Familial non-syndromic oligodontia


Department of Oral Medicine and Radiology, SGT Dental College, Hospital and Research Institute, Gurgaon, Haryana, India

Date of Submission09-Feb-2015
Date of Acceptance19-Oct-2015
Date of Web Publication25-Nov-2015

Correspondence Address:
Seema Basoya
Department of Oral Medicine and Radiology, SGT Dental College, Hospital and Research Institute, Near Sultanpur Bird Sanctuary, Village Budhera, Gurgaon - 122 505, Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-1363.170489

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   Abstract 

Oligodontia, a rare genetic disorder, represents the congenital absence of more than six teeth in primary, permanent, or both dentitions and is usually the feature of a syndrome. Its occurrence as an isolated entity is even rarer. This article reports non-syndromic oligodontia in two siblings. One case also had concurrent maxillary canine transmigration as one of the rarest associated entities.

Keywords: Hypodontia, oligodontia, transmigration


How to cite this article:
Basoya S, Koduri S, Gupta I, Chandar VV. Familial non-syndromic oligodontia. J Indian Acad Oral Med Radiol 2015;27:437-40

How to cite this URL:
Basoya S, Koduri S, Gupta I, Chandar VV. Familial non-syndromic oligodontia. J Indian Acad Oral Med Radiol [serial online] 2015 [cited 2022 Jan 21];27:437-40. Available from: https://www.jiaomr.in/text.asp?2015/27/3/437/170489


   Introduction Top


Tooth agenesis is a common dental anomaly posing a significant clinical problem. It is classified based on the number of missing permanent teeth, excluding the third molars. [1] Anodontia is defined as complete absence of teeth, hypodontia is missing of one to five teeth, and oligodontia is six or more teeth missing. [2] The incidence of missing permanent teeth has been reported to vary from 2.6 to 11.3% depending on the demographic and geographic profiles. [3] It may occur as part of a syndrome or as an isolated condition that has been linked to mutations of the genes. [2],[4] Non-syndromic oligodontia is a relatively rare condition, with the incidence being as low as 0.08-0.16%. [2] We report here two cases of non-syndromic oligodontia in the same family, of which one case showed concurrent maxillary canine transmigration, which is a rare entity.


   Case Reports Top


Case 1

A 25-year-old male patient reported with the complaint of missing lower front teeth since birth with history of difficulty in chewing food and speaking the alphabets "f" and "v." There was no history of extraction of any tooth; family history revealed non-consanguineous marriage with normal delivery and similar history of missing teeth present in the patient's younger brother. On extraoral examination, the patient's skin and hair were found to be normal in texture and appearance, with deepening of the mentolabial sulcus [Figure 1]. Intraoral examination revealed 13 teeth. The right and left maxillary lateral incisors, canine, premolars, first molars and mandibular anteriors, and premolars were found to be missing. Deciduous maxillary right and left canines were retained. The patient was wearing a faulty prosthesis with respect to the maxillary right and left premolar region [Figure 2] [Figure 3] [Figure 4]. Orthopantomograph (OPG) of the patient showed totally 13 teeth present. No impacted teeth were seen in the OPG [Figure 4]. Based on the history and clinical findings, diagnosis of non-syndromic oligodontia was made. Removable partial denture in the mandibular arch was made and delivered to the patient [Figure 5] and [Figure 6]. Further treatment planned for the patient was removal of the faulty prosthesis and extraction of the retained teeth.
Figure 1: Profi le picture of Case 1

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Figure 2: Maxillary arch (Case 1)- Cross-sectional view

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Figure 3: Mandibular arch (Case 1)- Cross-sectional view

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Figure 4: Case 1- OPG

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Figure 5: Case 1- Before treatment

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Figure 6: Case 1- After (removable partial denture in mandibular arch)

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Case 2

Patient's younger brother, aged 17 years, was found to have missing teeth in the right upper back and lower front regions of the jaws since birth. There was no history of dryness, loss of hair, weight loss, or any other symptom. On extraoral examination, no abnormality was detected in the skin; hair was normal in texture and appearance [Figure 7]. Intraoral examination revealed the presence of 16 teeth. Bilateral maxillary lateral incisors, premolars, and mandibular central and lateral incisors were missing. Deciduous maxillary right lateral incisor, bilateral canine, left maxillary second molar, and mandibular canines were retained. Other findings were conical-shaped mandibular canines, midline diastema, spacing distal to left maxillary central incisor, and increased overbite [Figure 8] and [Figure 9]. OPG of the patient revealed impacted right maxillary canine crossing the midline and 11 missing permanent teeth [Figure 10]. Treatment planned for the patient's brother was removal of the deciduous retained teeth, orthodontic treatment, followed by rehabilitation of missing teeth.
Figure 7: Profi le picture of Case 2

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Figure 8: Maxillary arch (Case 2)- Cross-sectional view

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Figure 9: Mandibular arch (Case 2)- Cross-sectional view

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Figure 10: Case 2- OPG

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   Discussion Top


Oligodontia has variety of manifestations, and may be associated with growth disturbances of the maxillofacial skeleton leading to altered facial appearance of the individual. Deepening of mentolabial sulcus was seen in both the patients and could be attributed to the missing lower permanent incisors. Oligodontia is seen as an isolated trait or a part of syndrome. [5] Several factors have been proposed for the etiology of oligodontia. Environmental factors like infection, trauma, drugs, chemotherapy, or radiotherapy at a young age have been implicated; a strong genetic component has also been suggested. Gene mutation in PAX9, MSX1, AXIN2, and He-Zhao deficiency have been suggested. [6]

A single missing tooth is common, but prevalence becomes progressively smaller as the number of missing teeth increase. Oligodontia is a rare anomaly having an overall prevalence of 0.14%. [7] The most common missing teeth are mandibular second premolars followed by maxillary second premolar and maxillary lateral incisors (excluding third molars). [8] Whereas the rare missing teeth are maxillary central incisor, maxillary and mandibular first molars, and canines. Interestingly, in our cases, bilateral second molars were missing, which account for about 0.7-0.8% of missing teeth as per earlier studies, along with bilateral mandibular central incisors (2.2%) and mandibular lateral incisors (1.1%) in Case 1. [4] In Case 2, the missing teeth were bilateral mandibular central incisors (2.2%) and mandibular lateral incisors (1.1%). The maxillary right canine was seen crossing the midline, i.e. transmigration. [9] This is a rare case of maxillary canine transmigration seen along with oligodontia.

The unesthetic appearance, missing teeth, and overclosure in patients with oligodontia may cause depression and psychosocial problems; therefore, the goal of treatment is to improve esthetics, mastication, and speech. Sequel associated with hypodontia includes abnormal spacing of teeth, delayed tooth formation, delayed deciduous tooth exfoliation, delayed permanent tooth eruption, and altered dimension of associated gnathic regions. [8] Both the familial cases presented here had spacing present in the dentition and multiple retained deciduous teeth. There were 19 teeth missing in the elder brother and 11 teeth missing in the younger one (excluding the third molars).

The timing of diagnosis is important for choosing an appropriate treatment plan and also for obtaining successful treatment results. The missing teeth, abnormal occlusion, or altered facial appearance may cause psychological distress in some or all patients. Multidisciplinary team approach of pedodontists, orthodontists, oral and maxillofacial surgeons, and prosthodontists is mandatory not only to improve esthetics, but also to restore function and psychological aspects, depending on the severity of the condition and patient's perceived need for care. The treatment not only improves speech and masticatory function, but also has psychological implications that may greatly help in regaining self-confidence and social perception. [10] The prosthetic treatment of oligodontia varies and includes removable partial dentures, fixed partial dentures, cast partial denture, overdenture, and implants. The choice is dependent on the condition of the remaining teeth and alveolar bone. Treatment costs should also be considered. In our case, the patient was more worried about the esthetics; so, we first delivered removable partial denture.


   Conclusion Top


The cases reported here exemplify the rare occurrence of familial oligodontia along with transmigration of maxillary canine. Cases of oligodontia should be evaluated carefully by clinicians, as early diagnosis aids in proper treatment planning.

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.

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.

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.
De Coster PJ, Marks LA, Martens LC, Huysseune A. Dental agenesis: Genetic and clinical perspectives. J Oral Pathol Med 2009;38:1-17.  Back to cited text no. 1
    
2.
Singer SL, Henry PJ, Lander ID. A treatment planning classification for oligodontia. Int J Prosthodont 2010;23:99-106.  Back to cited text no. 2
    
3.
Cobourne MT. Familial human hypodontia - Is it all in the genes? Br Dent J 2007;230:203-8.  Back to cited text no. 3
    
4.
Tangade P, Batra M. Non syndromic oligodontia: Case report. Ethiop J Health Sci 2012;22:219-21.  Back to cited text no. 4
    
5.
Gorlin RJ, Cohen MM, Levin LS. Syndromes of the Head and Neck. 3 rd ed. New York: Oxford University Press; 1990. p. 1119-23.  Back to cited text no. 5
    
6.
Neville BW, Damm DD, Allen CM, Bouquot JE. Oral and Maxillofacial Pathology. 3 rd ed. Philadelphia: Saunders; 2009. p. 77-80.  Back to cited text no. 6
    
7.
Polder BJ, Van't Hof MA, Van der Linden FP, Kuijpers-Jagtman AM. A meta-analysis of the prevalence of dental agenesis of permanent teeth. Community Dent Oral Epidemiol 2004;32: 217-26.  Back to cited text no. 7
    
8.
Rajendran R, Sivapathasundaram B. Shafer's Textbook of Oral Pathology. 6 th ed. New Delhi, India: Elsevier; 2008. p. 45-6.  Back to cited text no. 8
    
9.
Ando S, Aizawa K, Nakashima T, Sanka Y, Shimbo K, Kiyokawa K. Transmigration process of the impacted mandibular cuspid. J Nihon Univ Sch Dent 1964;6:66-71.  Back to cited text no. 9
    
10.
Dali M, Singh R, Naulakha D. Idiopathic nonsyndromic tooth agenesis: A report of rare three. J Interdiscip Dentistry 2012;2: 190-4.  Back to cited text no. 10
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10]



 

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