Swedish Oral Disability Center Focusing on Dental Symptoms in Ectodermal Dysplasia

December 2007
By Birgitta Bergendal, DDS, Head, National Oral Disability Centre, Jönköping, Sweden

The National Oral Disability Centre for rare disorders in Jönköping, Sweden, was established in 1999 and is one out of six centres in Scandinavia focusing on dental symptoms in rare disorders. In our center at the Institute for Postgraduate Dental Education (www.lj.se/oi), which is our clinical and research affiliation, we have worked with different projects on diagnosis and treatment in ED for a long time. In 1998, we arranged a consensus conference on ED where a care program was worked out and in the year 2000, we arranged the first European conference on ED for the dental profession.

It has recently been claimed that a majority of individuals with oligodontia (missing six or more permanent teeth) also have other ectodermal symptoms and thus an ED syndrome with a mild expression. In order to validate these findings a population study was conducted in children born 1981–1994 in an area with a million inhabitants. 162 individuals with oligodontia were found, out of which 123 were clinically examined. Based on answers to a structured interview and clinical saliva testing, half of the individuals had one or more sign or symptom from other ectodermal organs than their teeth, including salivary function. One in three had low salivary secretion rates. Only one in ten individuals with oligodontia had subjective symptoms from hair, nails, and/or sweat glands which are the organs included in the clinical criteria for an ED syndrome used today. In order to validate the results against the genes known to cause oligodontia or ectodermal dysplasia a genetic study is conducted analysing six different mutations from this clinical material.

In a recent evaluation of early placement of dental implants in small children with hypohidrotic ED in Sweden, a high rate of failed implants was found. This calls for a closer monitoring of patients by quality registries and preferably centralisation of treatment to a few centres.

A screening of orofacial function in ED was conducted last year at the NFED family conference in St. Louis and at the annual meeting of the Swedish ED Society using the Nordic Orofacial Test (NOT-S). Individuals with ED scored higher on orofacial dysfunction than healthy controls.

Our mission is to help patients and families to get information about their diagnosis, help to plan their treatment, and to find the best possible dental management. We work to increase the knowledge base on dental aspects of rare disorders and publish our findings in order to make them accessible for professionals worldwide.

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