Simultaneous occurrence of unusual odontodysplasia and oligodontia in the permanent dentition: report of a case

Abstract

Odontodysplasia is an uncommon clinicopathological condition with a variety of expressions. Although it is generally recognized as a localized disorder of dental tissue, its aetiology has not yet been well explained. In the present case, odontodysplasia with oligodontia in the permanent dentition is reported. The patient was in good health with normal stature and no other physical abnormalities. His parents and siblings were dentally and medically normal. The primary teeth appeared to be normal except for the primary second molars, where the enamel was malformed. However, the permanent incisors that had erupted into the oral cavity showed rough and hypoplastic enamel. An orthopantomogram showed 17 congenitally missing permanent teeth and malformation of the other 11 permanent teeth and tooth-germs. Because these findings were caused by developmental disturbances of both the mesodermal and ectodermal dental components, we diagnosed the present case as odontodysplasia accompanied by oligodontia in the permanent dentition.

A cocktail of abnormalities (atypical dental dysplasia associated with agenesis, extreme microdontia and segmental ankylosis)

“…In regional odontodysplasia (RO), caused by a malformation of dentin, enamel, and cementum, the teeth are hypoplastic and hypocalcified, and therefore have a ("ghost" teeth aspect), where the enamel and the dentin cannot be clearly distinguished 16,18 . RO is not caused by genetics, and in the same way as the patient's case, it affects only one of the four dental quadrants 1,6,9,10,11 . It is worrying that a form of RO was associated with perceptive deafness, grouped under the term Robinson syndrome.…”

Regional Odontodysplasia: A Systematic Review of Case Reports

“…Koskinen et al [12] described a 6.5-year-old girl, whose mother and two sisters (all of the siblings) had partially missing teeth with no other symptoms. On the contrary, there are also cases highlighting the absence of a positive familial history in the affected patients [13,14]. Even though many authors suggest the potential cause, no general conclusions have been made.…”