Syndromes of the Head and Neck

Hypohidrotic ectodermal dysplasia (HED) comprises a large group of inherited disorders of ectodermal structures, characterised by hypo- or anhidrosis, hypotrichosis and hypo- or oligo- or anodontia. We aimed to systematically assess the spectrum of prosthodontic approaches with regard to the patients' age and to provide clinical implications for practicing dentists. An electronic and manual search was conducted in four databases (Medline, LIVIVO, Cochrane Library, Web of Science Core Collection). Publications of multiple study designs written in English or German without data restrictions, reporting on prosthodontic treatment of patients diagnosed with HED and afflicted with oligo- or anodontia, were included. In total, 75 articles on 146 patients were analysed according to the patients' age. In children aged 2-17 years, removable full or partial (over)dentures represented standard treatment. In the mandible, implant-supported removable dentures on two interforaminal implants presented an alternative, already in young childhood. In cases with more than six teeth per jaw, also fixed (resin) bridges were used, frequently after orthodontic treatment. In adults, fixed or removable reconstructions with the help of up to eight implants per jaw, usually placed after bone augmentation procedures, were standard. Ten case reports/series with long-term follow-up illustrated the need for consistent maintenance including denture renewals. Prosthodontic rehabilitation should start in early childhood and needs to be revised in accordance with the patients' growth. Treatment should be carried out by a multidisciplinary team addressing variable demands in different age groups.

https://onlinelibrary.wiley.com/doi/pdf/10.1111/joor.12638

Prosthetic rehabilitation of patients with hypohidrotic ectodermal dysplasia: A systematic review.

Ectodermal dysplasia (ED) represents a disorder group characterised by abnormal development of the ectodermal derivatives. Removable partial dentures (RPD), complete dentures (CD) or overdentures (OD) are most often the treatment of choice for young affected patients. Prosthetic intervention is of utmost importance in the management of ED patients, as it resolves problems associated with functional, aesthetic, and psychological issues, and improves a patient’s quality of life. However, few studies present the principles and guidelines that can assist in the decision-making process of the most appropriate removable prosthesis. The purpose of this study was to suggest a simple treatment decision-making algorithm for selecting an effective and individualised rehabilitative treatment plan, considering different parameters. The cases and treatment of two young ED patients are described and each one was treated with either RPDs or ODs. Periodic recalls were employed to manage problems, and monitor the changes associated with occlusion and fit of the prostheses in relation to each patient’s growth. Both patients were followed up for more than 2 years and reported significant improvement in their appearance, masticatory function, and social behaviour as a result of the prosthetic rehabilitation. The main factors guiding the decision process towards the choice of an RPD or an OD are the presence of posterior natural teeth, facial aesthetics, lip support, number and size of existing natural teeth, and the occlusal vertical dimension.

/pdf/removable-partial-dentures-vs-overdentures-in-children-with-t2h0sxrd4r.pdf

Removable partial dentures vs overdentures in children with ectodermal dysplasia: two case reports.

Hypohidrotic ectodermal dysplasia is a hereditary disorder of two or more ectodermal structures. It may include hypodontia, hypotrichosis and hypohidrosis. Patient with this disease often needs complex prosthetic treatment. The option for a definitive treatment plan may include removable, fixed or implant supported prosthesis, single or in combination. The present review focuses on the classification, genetic aspects, and clinical manifestation along with various treatment modalities.

/pdf/prosthodontic-management-of-children-with-ectodermal-5brg4u1e3x.pdf

Prosthodontic Management of Children with Ectodermal Dysplasia: Review of Literature

. The Ectodermal Dysplasia (ED) is a disease that affects ectodermal structures. Statistically ED affects 7 out of every 100,000 births in the United States, and 7 out of 9 people affected with this disease are men. Several options of rehabilitation are possible, always making emphasis on the search for functional and aesthetic improvements and increased self-esteem of the patient in order to achieve the proper and expected growth and development. A case of ectodermal dysplasia in a 3 year old girl who attends the Clinic of Pediatric Dentistry, Faculty of Dentistry, Catholic University of Montevideo, Uruguay, is presented. A treatment plan tailored to the needs of the patient was performed: control of caries, paradencial disease, measures of local and systemic reinforcement with fluoride, in order to perform the rehabilitation, aesthetic, functional and psychological, composites resins (CR) and a set of chrome-cobalt Removable Prosthodontic patial (RPP) to respecting the principles of growth and development were made, with regular checks.

Tratamiento integral de un paciente niño con displasia ectodérmica. Utilización de resinas compuestas y prótesis parcial removible. Reporte de caso.

This clinical report describes the 5-year follow-up treatment of an 11-year-old boy with ectodermal dysplasia. The dentition was initially restored with a combination of maxillary anterior-bonded composite restorations and a mandibular complete denture. This individualized approach allowed successful management of the patient.

Five‐Year Follow‐Up Treatment of an Ectodermal Dysplasia Patient with Maxillary Anterior Composites and Mandibular Denture: A Clinical Report

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Five‐Year Follow‐Up Treatment of an Ectodermal Dysplasia Patient with Maxillary Anterior Composites and Mandibular Denture: A Clinical Report