Non syndromic familial hypodontia - a case series

Abstract

Aim: The aim of this case report is to discuss the features of non-syndromic familial hypodontia, which was noted in three siblings who had an unremarkable medical history.

Summary: Congenital absence of teeth is a common dental anomaly referring to teeth that fail to erupt in the oral cavity and remain invisible in radiographs. Congenital tooth agenesis is defined by the number of missing teeth, excluding the third molars; hypodontia refers to the absence of less than six teeth, while oligodontia is the absence of six or more teeth. Tooth agenesis can occur as part of a genetic syndrome such as the cleft lip and palate, ectodermal dysplasia and Trisomy 21 (syndromic hypodontia) or an isolated trait (nonsyndromic hypodontia). Non-syndromic hypodontia is the most common form and can be familial or sporadic. The most supported etiological theory suggests that tooth agenesis may be due to a combination of genetic and environmental factors, which lead to disturbances in the tooth germ during the initial formation stages. This case series reports bilateral agenesis of the maxillary canines in siblings, which is quite rare in occurrence. The management of these anomalies involves a multidisciplinary approach in order to restore esthetics and function. An early diagnosis can lead to effective treatment planning and satisfactory results.

Key learning points: The clinical manifestations as well as the short and long term management of this condition are discussed.