Dental Management of Ectodermal Dysplasia: A Report of Two cases.

Abstract

Ectodermal dysplasia (ED) is a rare genetic disorder recognized by failure of development of two or more ectodermal structures mainly skin, hair, nails, exocrine glands and teeth. The main 2 types of ED are ahydrotic or hypohidrotic and hidrotic ED. It is characterized by 3 “Hâ€: Hypotrichosis,  Hypohidrosis, Hypodontia. Dental problems include a partial or complete absence of primary and/or permanent dentition. Dental management of affected individuals is a very complex issue and a multidisciplinary approach is recommended. The unaesthetic appearance, poor self image, school/job related discrimination has a negative psychological effect on the patient. Therefore an early treatment of craniofacial features is recommended. The management of the orofacial disfigurement provides the patient confidence and improved psychosocial behaviour with positive feedback.  Dental management of two hypohydrotic type ED with hypodontia is illustrated. Both the patients had no familial history of ED. Considering the age of the patients oral rehabilitation with complete dentures was done and follow up plan was explained to the parents. Classification, clinical features, etiology, diagnosis and treatment modalities are also discussed.