Prosthodontic Management of a Pediatric Patient with Christ-Siemens-Touraine Syndrome: A Case Report

Abstract

Ectodermal dysplasias (ED) are a group of rare genetic disorders characterized by congenital defects involving two or more ectodermal structures. Christ-Siemens-Touraine syndrome or hypohidrotic ectodermal dysplasia is the commonest form of ED. Hypohidrotic ectodermal dysplasia (HED) is an X-linked disorder characterized by excessively dry skin due to the absence of sweat glands (anhidrosis), sparse body hair especially on the scalp and eyebrows (hypotrichosis), brittle nails, absence of sebaceous glands (asteatosis) and malformed or absent teeth. Oral manifestations include oligodontia or complete anodontia, conical teeth, underdeveloped alveolar ridges, generalized spacing and delayed eruption of permanent teeth. This case report discusses a classical case of HED and the options for rehabilitation in a growing patient. A thorough knowledge about the clinical manifestations of ED will lead to proper diagnosis and appropriate treatment plan thereby leading to significant improvements in esthetics, phonetics and masticatory function in ED patients, which in turn leads to improved quality of life in these individuals.