Oral rehabilitation of adult twins with severe lack of bone due to hypohidrotic ectodermal dysplasia--a 12-month follow-up.
OBJECTIVE
Patients with x-linked hypohidrotic ectodermal dysplasia (XLHED, OMIM 305100) often present with exceptional oral conditions, because alveolar ridges can remain severely hypotrophic due to oligo- or anodontia in the primary and/or permanent dentition. This can seriously disable a young person physically and psychologically, and lead to sociopsychological isolation.


METHODS
The oral rehabilitation of fraternal male twins with XLHED, one with severe hypodontia and the other with anodontia is described. The treatment of both patients comprised augmentation of the jaws using bicortical corticocancellous bone blocks from the medial aspect of the anterior hip, delayed implantation, and restoration of the occlusion with bar-retained overdentures. The multidisciplinary team approach included an oral and maxillofacial surgeon, a prosthodontist, and a speech therapist.


RESULTS
After uneventful healing, the 12-month follow-up showed that sufficient osseointegration of the implants for optimized function was obtained by creating adequate de novo bone volume in the underdeveloped jaw regions. Full regular masticatory function was achieved, and esthetics were improved considerably in both patients.


CONCLUSIONS
Augmentation and implant placement prior to prosthetic rehabilitation appears to be a suitable treatment option for adult patients with XLHED.
Kirmeier
Robert
R
Department of Oral Surgery and Radiology, School of Dental Medicine, Medical University of Graz, Austria.
Gluhak
Christine
C
Marada
Peter
P
Wegscheider
Walther A
WA
Eskici
Antranik
A
Jakse
Norbert
N
D000293:Adolescent; D016301:Alveolar Bone Loss / Q000209:etiology / Q000628:therapy*; D000540:Alveolar Ridge Augmentation / Q000379:methods*; D000848:Anodontia / Q000150:complications* / Q000628:therapy; D016025:Bone Transplantation / Q000379:methods; D000044:Dental Abutments; D003758:Dental Implantation, Endosseous / Q000379:methods; D015921:Dental Implants; D019094:Dental Prosthesis, Implant-Supported; D003779:Denture Design; D003781:Denture Retention / Q000379:methods*; D003828:Denture, Overlay; D053358:Ectodermal Dysplasia 1, Anhidrotic / Q000150:complications*; D005500:Follow-Up Studies; D006801:Humans; D007575:Jaw, Edentulous / Q000209:etiology / Q000534:rehabilitation; D008297:Male; D008439:Maxillary Diseases / Q000150:complications / Q000628:therapy; D013516:Oral Surgical Procedures, Preprosthetic / Q000379:methods; D016896:Treatment Outcome; D014429:Twins, Dizygotic
D015921:Dental Implants