Taurodontism in oral-facial-digital syndrome

Abstract

Oro-facial-digital syndrome (OFDS) is one of the rare hereditary conditions involving various developmental anomalies of the oral cavity, face as well as digits. Oral-facial-digital syndrome, Type I (OFDS I), a variant of OFDS, is a X-linked dominant condition showing malformations involving the oral cavity, face, and digits, predominantly affecting the females and lethal in males. Taurodontism is a clinical condition rarely encountered in OFDS I. It is a tooth developmental anomaly caused due to the failure of Hertwig’s epithelial sheath to invaginate horizontally at proper level, thereby resulting in an enlarged pulpal chamber and apical displacement of the pulp floor. A case of OFDS I, with typical clinical findings along with a unique feature of taurodontism, is presented here. Performing root canal treatment in taurodontism is a challenge. An early diagnosis and treatment will help the patient to lead a normal and healthy life.