Ectodermal Dysplasia, a Case Report: a Challenge for Prosthodontic Solution

Abstract

Introduction: Ectodermal dysplasia (ED) is a hereditary disorder characterized by developmental disturbances of two or more tissues and structures (skin, hair, nails, sweat glands, teeth structures, eye lenses, parts of the inner ear and nerves) of ectodermal embryonic origin. Hypodontia or anodontia of the primary and permanent dentition, poorly developed alveolar ridges and improper мaxillo - mandibular relations are the most common oral manifestations. Management of patients with ED requires a multidisciplinary team approach.

Case report: A 6-year-old girl with ectodermal dysplasia having a normal intellectual development has typical features of ED: square and bossing forehead, prominent supraorbital ridges, slightly pigmented and wrinkled eyelids, prominent and pointed ears, wide nose with anteverted nostrils, thin lips, and pointed chin. Hypotrichosis is characterized by almost no eyebrows, eyelashes and skin hairs; the sculp is covered with a wig. The skin is dry (hypohidrosis), thin, rough and shiny. Finger and toe nails are short, thick, striated and slow-growing. Decreased lower facial height, together with deep mentolabial and nasolabial folds contributed to an old-looking facial expression. Complete bilateral cleft palate was partially solved on 1.5 year of age, with remained oro-nasal communication and a presence of soft palate only. An alveolar ridge in the mandible is atrophic with deciduous canines destroyed by caries and carious deciduous and permanent molar teeth in the upper jaw (severe hypodontia). The gingiva is rather swollen, with dark red color especially around the teeth. Several functions of the oro-facial system such as mastication, swallowing and phonetics are disrupted.

Treatment procedure: According to the patient’s age and clinical findings, removable complete over-denture prosthesis was the treatment of choice. Preliminary impressions were taken with appropriate stock trays and irreversible hydrocolloid material. Border molding was done with a thermoplastic material while the functional impressions were made with light body polyvinyl siloxane impression material using the custom trays. Maxillo-mandibular relations were recorded using temporary bases with wax rims. Artificial teeth, reshaped considering the child’s age, were arranged according to a balanced occlusion. Final trial was taken to verify vertical and centric relations, occlusion, phonetics and aesthetics. The maxillar and mandibular prosthesis were produced in the heat cure acrylic resin. The facial frontal and profile expression improved significantly with complete dentures.

Conclusion: Correct maxillo - mandibular relations and normal function of the dento-facial system in patients with ED, can be established by the acrylic dentures. Prosthodontic treatment has a major impact not only on the aesthetics and functions, but on the psychological development and emotional condition as well.

Keywords

Cleft palate, Ectodermal Dysplasia, Hypodontia, Removable dentures