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

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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 1.6–21.9 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 patient’s 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.

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About the authors

Roman A. Rozov

Academician I.P. Pavlov First St. Petersburg State Medical University; City Dental Clinic No. 33

Author for correspondence.
Email: dds.rozov@gmail.com
ORCID iD: 0000-0001-5804-9497
SPIN-code: 1173-7870
Scopus Author ID: 57205048723
ResearcherId: E-3677-2019

MD, DDS, PhD, Associate Professor

Russian Federation, Saint Petersburg; 3/1 Koroleva av., Saint Petersburg, 197341

Vladimir N. Trezubov

Academician I.P. Pavlov First St. Petersburg State Medical University

Email: trezubovvn@mail.ru
ORCID iD: 0000-0003-0532-5632
SPIN-code: 2588-7283

MD, DDS, PhD, D.Sc., Professor

Russian Federation, Saint Petersburg

Aleksandr B. Gerasimov

City Dental Clinic No. 33

Email: onedoc@mail.ru
ORCID iD: 0000-0002-1101-7073
SPIN-code: 7384-7374


Russian Federation, 3/1 Koroleva av., Saint Petersburg, 197341

Leonid M. Emdin

City Dental Clinic No. 33

Email: dr.emdin@gmail.com
ORCID iD: 0000-0003-0374-4155
SPIN-code: 4710-9401


Russian Federation, 3/1 Koroleva av., Saint Petersburg, 197341


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Supplementary files

Supplementary Files
1. Fig. 1. Initial clinical presentation: (a) the patient’s appearance; (b) intraoral photography without a removable denture, the thinned mucous membrane of the alveolar ridges is determined; (c) intraoral photography with a removable denture, an inverse ratio of the anterior artificial and natural teeth

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2. Fig. 2. Results of X-ray examination of a patient with ectodermal dysplasia before treatment at the age of 17 years: (a) orthopantomogram with signs of oligodontia, 6 preserved primary teeth on the upper jaw and 12 preserved teeth on the lower jaw, including 2 permanent teeth (46 and 36); (b) profile teleroentgenogram; (c) computed cone-beam tomogram with signs of hypoplasia of the alveolar process of the upper jaw and the alveolar part of the lower jaw; (d) axial view of cone-beam computed tomography of the skull that shows the minimum sufficient width of the mandible body for the placement of intraosseous implants

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3. Fig. 3. Virtual diagnostic modeling of artificial dentition in the Exocad program

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4. Fig. 4. Computerized axiography: (a) appearance of the fixed extraoral part of the device on the head; (b) the results of recording the forward movement of the lower jaw

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5. Fig. 5. The technology of implantable dentures: (a) computer modeling of the frame of the implantable prosthesis; (b) zirconium dioxide frame of the lower jaw prosthesis; (c) ready implantation prostheses of the upper and lower jaws with an artificial ceramic gum; (d) implantation prostheses are compared outside the oral cavity in the position of central occlusion

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6. Fig. 6. Orthopantomogram after implant prosthetics

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7. Fig. 7. X-ray cephalometric analysis: (a) VTO analysis; (b) profile teleroentgenogram with preliminary implantation prostheses (with contrast material on the occlusal surface); (c) profile teleroentgenogram after prosthetics; (d) comparative results of the Sato analysis after and before rehabilitation

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8. Fig. 8. Results of the control surface electromyography of the masticatory muscles: a good level of their bioelectrical activity and sufficient symmetry of the indicators on the right and left are determined

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9. Fig. 9. The patient’s appearance (a) before and after the application of the implantable prosthesis of the upper jaw (b)

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Copyright (c) 2021 Rozov R.A., Trezubov V.N., Gerasimov A.B., Emdin L.M.

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This work is licensed under a Creative Commons Attribution 4.0 International License.

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