ORAL MANIFESTATIONS OF THE AXENFELD-RIEGER SYNDROME

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Journal of the Korean Academy of Pediatric Dentistry 2003;30(3):510-514.

Axenfeld-Rieger 증후군의 치과적 소견

강태성, 최병재, 김성오, 이제호

연세대학교 치과대학 소아치과학교실.구강과학연구소

Abstract

The Axenfeld-Rieger syndrome is a rare autosomal dominant disorder characterized by dental and ocular abnormalities. The essential ocular features include partial or complete bilateral hypoplasia of the iris stroma, abnormalities of the angle structures with congenital iris adhesions, and anterior displacement of Schwalbe's corpuscles. Common oral findings are hypodontia(especially in anterior maxillary segment), microdontia, misshaped teeth, delayed eruption of the teeth. Additionally, other systemic symptoms can be seen and early detection by the pedodontist through dental diagnosis should prevent visual impairment.

Key Words: Axenfeld-Rieger syndrome, Axenfeld anomaly, Rieger anomaly, Rieger syndrome, glaucoma