Ectodermal Dysplasia - A Cephalometric Appraisal

Abstract

Ectodermal dysplasia (ED) is a rare genetic disturbance affecting the tissues of ectodermal origin with mutation carried by X chromosome with partial expression in females and major manifestation in male sex. It often presents problems of partial or total anodontia of one or both the series of dentition. Absence of complete dentition is rare. During a time span of 10 years, 4 cases (3 females & 1 male) affected with ectodermal dysplasia have been reported. Female subjects showed varying degree of anodontia while the male suffered from complete absence of both primary and permanent dentition. It is interesting to note a higher ratio of affected females, 3 females against 1 male. Females of course exhibited slight lesser manifestation of ED than observed in male. This is in agreement with phenomena of doses compensation of Lyons Hypothesis. Cephalometric angular and linear measurements revealed that ED subjects exhibit tendency for mild mandibular prognathism in the females. Linear dimension exhibit lesser dimensions with tendency to retruded maxilla and significant reduction in lower facial height due to hypodontia and absence or poor development of alveolar ridges. It is also interesting to note that only reported female members of their families were affected by ED and none of the parents or other members of their families were affected.