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Quintessence Int 34 (2003), No. 9 1. Oct. 2003
Quintessence Int 34 (2003), No. 9 (01.10.2003)
The Rieger syndrome: Orofacial manifestations. Case report of a rare condition
Singh, Jagjit / Pannu, Karneev / Lehl, Gurvanit
Rieger syndrome is a rare, autosomal dominant disorder due to developmental arrest of tissues derived from neural crest ectoderm in the third trimester. It is characterized by abnormalities of the anterior chamber of the eye coincident with dental and craniofacial anomalies. Early recognition of dental anomalies aids in detection of the syndrome and prevents progressive loss of vision. This case report discusses the ocular, dental, and craniofacial findings and the overall management of the patient.