Region Jnkpings ln Odontologiska Institutionen
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Signs and symptoms of ectodermal dysplasia in young individuals with oligodontia

Bergendal B*, Bågesund M, Holst A, Norderyd J
 

Introduction

Individuals with oligodontia (six or more congenitally missing teeth) often need extensive oral habilitation including prosthetic treatment. Recent studies by Nordgarden (2001) have indicated that more than half of individuals with oligodontia also have symptoms from other organs of ectodermal origin such as low salivary secretion, decreased sweating capacity, sparse hair and dry skin, and thus fulfil the criteria for an ectodermal dysplasia syndrome. Tooth agenesis is usually the only symptom that requires treatment. Therefore it is the responsibility of the dentist performing the oral habilitation to establish a diagnosis.

Aim

The aim of the present study was to improve the clinical diagnostics of hypohidrotic ectodermal dysplasia in order to give patients relevant information about their diagnosis.

Materials and Methods

An appeal was made to dentists working as specialists in paediatric dentistry, orthodontics, and prosthetic dentistry and to public dental clinics in three Swedish county councils to report all individuals with oligodontia among children and adolescents born 1981–1994. The reported individuals were invited to participate in a clinical study comprising a structured interview focused on signs and symptoms from ectodermal organs, clinical photographs, evaluation of salivary function, and collection of blood samples for later genetic analysis. The study design and protocols were approved by an ethical committee.

Results

A total of 164 individuals with oligodontia were reported. Of these, 2 were excluded because they had undergone radiation therapy in early childhood, 33 declined to participate, 2 could not cooperate to the examination, and 4 had moved from the area. This left 123 children and adolescents in the study (71 girls, 52 boys). Oligodontia was part of a known syndrome or a rare disorder in 11 individuals (9 %). Reduced salivary secretion rates (≤0.5 ml/min) were found in 11 %. The examiners anticipated that 15 % of the children had signs and symptoms of an ectodermal dysplasia syndrome.

Conclusions

The prevalence of oligodontia in this population study was 0.09 %. Salivary secretion rates and other signs and symptoms from ectodermal organs should be evaluated in persons with oligodontia.

Uppdaterad: 2013-04-03
Anna Thofelt, Avdelningen för odontologisk radiologi Jönköping, Folktandvården