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J Clin Pediatr Dent. 2005 Fall;30(1):83-8.

Axenfeld-Rieger syndrome: report on dental and craniofacial findings.

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Centre for Dental Education and Research, All India Institute of Medical Sciences, New Delhi, India.


Axenfeld-Rieger syndrome is a rare autosomal dominant disorder characterized by various ocular and extraocular malformations. Dental abnormalities are considered as definitive features for the diagnosis and differentiation of Rieger syndrome from other anterior chamber of the eye malformations. A case of Rieger syndrome with distinct dental and craniofacial anomalies is described. Significant cranio-dento-facial findings that have been observed are, teeth with short and dilacerated roots, hyperplastic frenums and underdeveloped maxilla. There was an anterior crossbite, bilateral posterior open-bite and moderate to severe anterior crowding.

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