Early prosthetic treatment of patients with ectodermal dysplasia: A clinical report

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Hypohidrotic ectodermal dysplasia is a hereditary disorder of ectodermal origin. The early orthodontic treatment of 2 young boys suffering from hypohidrotic ectodermal dysplasia with partial maxillary and complete mandibular anodontia of the primary dentition is described. Both were treated with removable maxillary partial and mandibular complete dentures with individualized occlusion and age-appropriate artificial teeth. The results were significant improvements in speech, masticatory function, and facial esthetics, contributing to the development of normal dietary habits, and the improved and more rapid social integration of these children.

Section snippets

Patient 1

A 3-year, 11-month-old male patient was referred to the Department of Pediatric Dentistry, Semmelweis University, Budapest, Hungary for examination, evaluation, and treatment. The child exhibited the classical features of HED including diffusely sparse hair, eyelashes and eyebrows, severe hypohidrosis, and subsequent problems with thermoregulation and dry skin. The facial profile showed a sunken nasal bridge (so-called “saddle nose”), with prominent forehead and everted lips. Cephalometric

Discussion

Prosthetic treatment modes using RPDs or complete dentures and dental implants are the primary treatment alternatives for the clinical management of young patients with severe hypodontia. Oligodontia or anodontia totalis associated with ED is often characterized by underdeveloped alveolar bone structures with missing or reduced alveolar ridges. This results in less volume of bone for support of conventional prosthetic dentures, and it can also affect the bone volume available for the placement

Summary

Young children with HED, associated with a partial or total absence of the primary dentition, suffer not only from the difficulties in alimentation and speech but also from poor esthetics. Prosthetic treatment is recommended beginning at the age of 3 years, as part of a team approach with psychologists and speech therapists. Apart from fabrication difficulties, early prosthetic treatment should be provided to encourage a normal physiological development and to improve the function of the

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      Ectodermal dysplasia is a hereditary disorder occurring as a consequence of disturbances in the ectoderm of the developing embryo. The triad of alopecia (scanty hair on the scalp and eyebrows), nails dystrophy (onychodysplasia) and palmoplantar hyperkeratosis, is associated with lack of sweat glands and hypodontia/anodontia of primary and/or permanent dentition.1 There are two major types ED depending on the number and functionality of the sweat glands: (1) X-linked anhidrotic or hypohidrotic, where sweat glands are either absent or significantly reduced in number (Christ-Siemens-Touraine syndrome) and (2) hidrotic, where sweat glands are normal and the condition is inherited as autosomal dominant (Clouston's syndrome).4

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