Oral Rehabilitation of Patients with Hypohidrotic Ectodermal Dysplasia: When, Why and How to Intervene

Ηypohidrotic ectodermal dysplasia (HED) patients present absence of many permanent teeth, alveolar deficiency and severely affected maxillofacial skeleton. The management commonly includes a series of removable dentures and orthodontic appliances, adapted constantly to the child's growth and development. The dental literature lacks a protocol to elucidate when, why and how to intervene for the modification of jaw growth pattern and for the treatment of dental and occlusal problems to maximize the clinical therapeutic outcome. The aim of the present review was to propose and redefine the treatment plan for the orofacial rehabilitation of patients with HED.

Normal craniofacial growth is described, followed by a review of published findings about facial growth in HED patients. Subsequently, the treatment strategy is presented, focusing on the stages of physical growth and dental development: (a) infancy and early childhood (primary dentition years), (b) late childhood (mixed dentition years), (c) adolescence (early permanent dentition years) and (d) adulthood (permanent dentition years), and to the special characteristics related to each developmental stage. Finally, the treatment possibilities and restrictions are considered, and a protocol defining when, why and how to intervene is proposed.