Oral manifestations of the Rieger syndrome: report of case.

The Rieger syndrome is a rare, autosomal dominant disorder. It is characterized by defects of the anterior chamber of the eyes as well as developmental malformations of the dentition. A case is described that shows classic findings with emphasis on dental management. Oral abnormalities in the pedigree may also suggest subtle manifestations of the syndrome. Recognition of the dental anomalies may result in early diagnosis of the syndrome and prevent progressive visual loss.