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  • Title: Prosthetic rehabilitation of a child with X-linked hypohidrotic ectodermal dysplasia: a case report and 12-month follow-up.
    Author: Cassol DV, Viera TI, Souza IPR, Pomarico L.
    Journal: Gen Dent; 2019; 67(4):e1-e6. PubMed ID: 31355772.
    Abstract:
    The hereditary condition known as ectodermal dysplasia (ED) is characterized by the absence of or a defect in 2 or more ectodermally derived structures such as skin, nails, hair, sweat glands, or teeth. Patients with this disorder usually present with reduced salivary gland function and absence of some or all teeth, which compromises orofacial function and development. In addition, children with ED usually experience difficulty in social interactions because of their appearance. This article reviews previously published case reports pertaining to ED and describes oral rehabilitation with removable partial dentures (RPDs) in a 5-year-old girl diagnosed with X-linked hypohidrotic ED, which presented as hypodontia. An orthodontic expander screw was inserted in the maxillary prosthesis to correct the patient's crossbite, and periodic recall examinations were scheduled to monitor the effects of the patient's growth on occlusion and fit of the prosthesis. The child was monitored for 12 months, during which she exhibited significant improvement in physiologic function, appearance, and social behavior. Because negative esthetic, functional, and psychological consequences are associated with this condition, dentists must be knowledgeable about its common oral manifestations.
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