Unclassified syndrome involving dental enamel, dentine and lack of tooth eruption

Abstract

BACKGROUND: Regional odontodysplasia, also described as ghost teeth, has been described on a number of occasions, in some cases generalized odontodysplasia affecting both the primary and the permanent dentition. However, generalised odontodysplasia is a very rare finding, involving large pulps and short roots of the whole dentition and in the cases previously reported the teeth finally erupt. In the case reported on herein this has not happened. CASE REPORT: A female patient, now aged 22-years-old, cared for in the TAKO-centre (Oslo) since she was 5 years old, presented with a continuing problem of absence of permanent teeth due to total lack of eruption. All her permanent teeth were either retained intra-alveolar or had been extracted following previous periods of pain and infections. There was no enamel on any of the permanent teeth and the dentine was softer than normal. In addition, there appeared to be very little potential for eruption. Hence, tooth development was affected in all aspects. Her skeletal height was much shorter than expected taking her tall parents into consideration. After all possible assessments, no specific diagnosis for the condition of this young woman has been determined. TREATMENT: Before the present series of dental care the patient was wearing full dentures in both jaws. In the autumn of 2007, implants were placed in her maxilla and an implant supported fixed prosthesis in porcelain was installed the following spring. Similarly, implants were placed in her mandible, partly in retained teeth, in the autumn of 2008, and an implant supported fixed prosthesis was inserted during the spring of 2009. FOLLOW-UP: after prosthetic treatment a dental hygienist has seen the patient regularly. No problems or signs of infections have occurred to date. CONCLUSION: This case report concerns a rare and apparently un-named syndrome affecting both primary and permanent teeth. This paper has been written with an aim of gathering the views as to the aetiology of her problem, hopefully finally to provide a definitive diagnosis.