Oral rehabilitation of a patient with hidrotic ectodermal dysplasia (Clouston syndrome)

Abstract

BACKGROUND: Ectodermal dysplasia is a rare genetic condition affecting the development and homeostasis of two or more ectodermal structures, including hairs, teeth, nails, and some glandular organs. The prevalence is 1.621.9 per 100000. The teeth are affected in 79% of cases. Patients with ectodermal dysplasia have a low level of quality of life affected by unemployment, utilization of removable dentures, and a constant dry mouth condition. The main rehabilitation method of the masticatory-phonic elements for the patient with ectodermal dysplasia is conventional removable prosthodontics. The main treatment objective for these patients is to restore the missing elements of the masticatory-phonic apparatus for normalizing mastication, speech, deglutition, and creating optimal aesthetics. This approach can lead to restoring social activity by the increase of a patients quality of life. A clinical case report of patient M., 20 years old, is presented in this article. Treatment was completed one year ago. CLINICAL CASE: The rehabilitation strategy included producing screw-retained full ceramic opposing prostheses supported by six implants in the upper and lower jaws. DISCUSSION: The choice restoration method of the masticatory-phonic elements for patients with ectodermal dysplasia is prosthodontic treatment with the fabrication of ordinary removable dentures. Early attempts of implant treatment of patients with such conditions led to a low percentage of implant survival. In most works dedicated to treating patients with ectodermal dysplasia, the fabrication of implants supported by a metal resin hybrid prosthesis with acrylic teeth sets is the final rehabilitation. We decided to produce an implant supported by a zirconia ceramic prosthesis with partial layering for patient M. It was feasible because, first of all, his growth was finished, and also this decision gives us the possibility to fabricate a high quality implant supported by a prosthesis enabling optimum function with an unlimited service period. After finishing treatment, 100% of implants and prostheses last one year. CONCLUSION: It is necessary to engage all available clinic diagnostic, planning, therapeutic, and preventive resources to achieve a high aesthetic and functional result of the dental rehabilitation of patients with ectodermal dysplasia. Implementing implant supported prosthetic rehabilitation of such patients after reaching 17 years of age has to be the treatment of choice that could help achieve stable and reliable results. It might also foster social adaptation that would enable high quality of life.