Aesthetic and functional rehabilitation of a girl with Hypohidrotic Ectodermal Dysplasia. Case report

Abstract

A case of a 5-year-old girl diagnosed with hypohidrotic ectodermal dysplasia (HED) is presented. This X-linked autosomal recessive genetic disease is characterized by a failure in the development of two or more structures derived from the ectoderm. The patient is referred to the Comprehensive Children's Dentistry Chair of Mendoza University for a dental evaluation. Clinical examination shows dry skin, with wrinkles and hyperpigmentation; sparse hair, eyebrows and eyelashes, and decreased vertical dimension. Intraoral examination reveals oligodontia and erupted temporary teeth with altered shape. Xerostomia is also observed. The mother reports that the girl has problems relating to other children of her age and does not smile. These types of patients need oral rehabilitation from an early age with functional appliances and their continuous adjustment during growth. In a first stage, it was decided to design functional space maintainers for the upper maxilla and mandible, aiming at improving aesthetics and favoring the development of language and chewing function. After placement, controls are carried out once a month for the first three months, then twice a year. After two years of control, it was decided to replace the maintainers with a flexible partial prosthesis, due to the fact that they frequently loosened. Within three years, the patient adapted to the treatment and the proposed aesthetic and functional objectives were achieved.