Dental Management of Ectodermal Dysplasia Syndrome at an Early Age: A Case Report

Ectodermal dysplasia (ED) is a relatively common sex-linked dermatitis characterized by congenital dysplasia of one or more ectodermal structures and their accessory appendages. Common manifestations include fragile skin and nails, defective teeth and salivary glands, frontal bossing with prominent supra orbital ridges, nasal bridge depression and protuberant lips. Teeth are often few in number (hypodontia or oligodontia) and have a conical form that results in generalized spacing. In extreme cases, both deciduous and the permanent dentition may fail to form (anodontia) and consequently, hypoplasia of the jaws may happen. This article reports a case of ED with its management protocol.
A 4 year-old boy with hypohydrotic ED was referred to Dental School of shahid Beheshti University of Medical Sciences. Clinical examination revealed classical features of ED, with only a few teeth. He had fine scanty hair, dry skin and depressed nasal bridge. Removable denture was made with particular limitations for his lower jaw to restore esthetics and masticatory function. The existing upper teeth were initially reshaped using composite resin restoration material.
Preventive treatments in ED patients are very important to save the existing teeth. In patients with oligodontia, removable dentures can be used as a costbenefit and pleasant intermediate treatment to restore function and esthetics and improve patient‟s psychological status.