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

Abstract

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.

Prosthetic rehabilitation with fixed prosthesis of a 5-year-old child with Hypohidrotic Ectodermal Dysplasia and Oligodontia: a case report

“…3 Upper and lower alginate impression used for the fabrication of the appliances dental home [6] and to explain to the parents the treatment stages required as the child grows. Pigno et al recommended use of a dental prosthesis before the child goes to school at around 3-4 years of age [7], which is in agreement with a systematic review by Schnabl et al, who found that the median age of prosthetic rehabilitation in patients with ED is 4 years [8]. Early prosthodontic rehabilitation of patients with ED helps to restore and normalize function of muscles of mastication and the skeletal growth pattern.…”

Ectodermal Dysplasia with Oligodontia: A Rare Case—Rehabilitation by Prosthetic Management

“…Molecular etiologies of HED consist of mutations in genes involved in ectodysplasin/NF -kappaB or Wnt/beta -catenin, both of which are involved in the development of ectodermal appendages [6]. The skin on most parts of the body can be abnormally thin, dry, and soft with an unusual absence of pigmentation (hypopigmentation).…”

Special-Needs Patients in Pediatric Dentistry: Progeroid Syndrome. A Case of Dental Management and Oral Rehabilitation

“…Cases of oral prosthetic rehabilitation in little children affected by genetic conditions, or after teeth extraction for trauma or early childhood caries are reported in literature. 7,8 Standardized protocols are not available; each solution is tailored according to the patient condition and the state of art of the prosthetic technology.…”

“…The prostheses will be updated according to the new techniques that will be developed, moreover, comfortable fixed solution using implants will be probably proposed in the future. 8…”