Case series: Treatment considerations in x-linked hypohidrotic ectodermal dysplasia.

AIMS: To describe our treatment considerations obtained from the dental care of several boys affected with X-linked hypohidrotic ectodermal dysplasia (HED) and to discuss the different problems that may arise in connection with these kinds of treatments.

STUDY

DESIGN

The subject group included 10 males affected with X-linked HED, treated at the department of Paediatric Dentistry and Clinical Genetics, Copenhagen School of Dentistry. All patients were treated, after a period of adaptation, with removable prostheses and whenever necessary, composite restoration of the conical crown morphology of the maxillary incisors. Furthermore, some of the patients received an orthodontic treatment for closure/reduction of the medial diastema.

RESULTS

The mean number of appointments at the department was 32 with a range from 7 to 59 appointments. In 7 out of 10 patients, the treatment was a success according to the patient, parents and dentist. In 3 out of 10 patients the treatment was not a success from the dentist's point of view: primarily due to lack of cooperation between the patient, parents and dentist. In half of the patients the deviated maxillary incisors morphology was restored and 5 out of 10 patients received orthodontic treatment for closure of the medial diastema in the maxilla.

CONCLUSION

It is of utmost importance to take the motivation of the patient and parents as well as the patient's acceptance for dental treatment into consideration. Furthermore, the treatment is rather time-consuming and should involve different areas of specialisation. Finally it is vital to remember that the parents may also have undergone a prolonged dental treatment, which might reduce their patience regarding their children's treatments.