Hypohidrotic Ectodermal Dysplasia: Prosthetic and Endodontic Management

Abstract

Individuals affected by ectodermal dysplasia syndromes have abnormalities of the glands, tooth buds, hair follicles, and nail development. Oral finding in ectodermal dysplasia patient are significant and can include multiple abnormalities of the dentition such as anodontia, hy-podontia or malformed and widely spaced peg like teeth, loss of occlusal vertical dimension, protuberant lips and lack of normal alveolar ridge development. This clinical report describes a combined surgical, pedodontic, and prosthodontic approach for the treatment of a patient with hypohidrotic ectodermal dysplasia.

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

“…Fifteen case reports and two case series reported on prosthetic rehabilitation of 17 teenagers (13 males, 4 females) aged 10‐17 years with HED (Table ). At the time of prosthetic rehabilitation, teenagers were 13 (10/16.4) years.…”

“…Primary or permanent teeth considered of little prosthetic value may be extracted in the course of rehabilitation by means of implants . The extraction of firm, non‐carious teeth and the subsequent provision of a full denture, as described by Jain et al 2010, does not appear to be a warrantable procedure to the authors. A consistent attendance comprising oral hygiene, denture adaptation and renewal, and a stepwise approach to treatment goals, serves as a basis for treatment success. Renewal of full‐ or overdentures has to be expected after 1‐3 years, and renewal of partial dentures after one to 6 years …”

“…Primary or permanent teeth considered of little prosthetic value may be extracted in the course of rehabilitation by means of implants. 77,82 The extraction of firm, non-carious teeth and the subsequent provision of a full denture, as described by Jain et al 2010, 71 does not appear to be a warrantable procedure to the authors.…”

Christ Siemens Touraine syndrome: Two case reports and felicitous approaches to prosthetic management

“…[ 2 ] Ectodermal dysplasia is rare with their incidence at 1 in 10,000–1 in 100,000 births. [ 3 ] The most common ectodermal dysplasia is X-linked recessive hypohidrotic ectodermal dysplasia (Christ Siemens Touraine syndrome) and hydrotic ectodermal dysplasia (Clouston syndrome). [ 4 5 ] In this case report, we are describing two different management approaches of two Christ Siemen Touraine syndrome cases.…”

Ectodermal Dysplasia Syndrome with Orthodontic Treatment and Oral Finds: A Case Report

“…Patients who suffering from ectodermal dysplasia can be characterize variably. Multiple ectodermal derivatives can be affected, including teeth, hair, nails, sweat gland, etc [1]. The first case was described by Thurman in 1848 [2].…”