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Journal of Oral Medicine, Oral Surgery, Oral Pathology and Oral Radiology


A non-syndromic hereditary oligodontia- a rare case report


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Author Details: Raj Rathore, Deepshikha Rathore, Piyush Arora, Don Varghese, Sumita Kaswan

Volume : 2

Issue : 3

Online ISSN : 2395-6194

Print ISSN : 2395-6186

Article First Page : 180

Article End Page : 183


Abstract

Tooth agenesis is a condition where teeth are missing due to developmental failure. It is often seen in one-quarter of general population making it the most common of human developmental anomalies and it may or may not be associated with syndromes. Oligodontia is a rare hereditary or developmental anomaly showing absence of 6 or more teeth with the incidence varying from 0.08% to 0.16%. The genes expressed in embryonic tissues associated with non-syndromic tooth agenesis are found to be MSX1 and PAX9 genes. These genes play a key role in tooth development, where PAX9 and MSX1 are responsible for odontogenesis in multicuspids and incisors respectively.
This case report describes a rare familial presentation of non-syndromic hereditary oligodontia in a 20-year-old female, showing absence of ten teeth involving first and second molars in all four quadrants and mandibular second premolar in third and fourth quadrant. Subject’s father showed missing second molars in all four quadrants along with missing right mandibular first molar. Her sister had missing first and second molars in all quadrants while her brother had missing first and second molars, mandibular incisors and second premolars.
The consequences of missing teeth are numerous as causing inconvenience in mastication, speech, etc. Considering the level of complexity in the management of oligodontia patients, treatment should begin with early diagnosis to prevent future functional and esthetic problems.

Keywords:
Agenesis, Non-syndromic, Hereditary, Oligodontia