Abstract
Background/Aim: The Rieger syndrome is a rare, autosomal dominant and phenotypically variable disorder, characterized by abnormalities of the anterior chamber of the eye, coincident with missing or misshapen teeth. Case report: This report features a case of the Rieger syndrome associated with bilateral cleft lip and palate and a severe open bite, findings not usually reported in association with this condition. Conclusions: The findings described in the present case of Rieger syndrome are unusual and expand the spectrum of manifestations of the condition.
Highlights
Rieger syndrome is characterized by hypodontia and primary mesodermal dysgenesis of the anterior chamber of the eye[1,2,3,4]
Dental and craniofacial defects help to distinguish the Rieger syndrome from other anterior chamber malformations (Axenfeld’s syndrome, Peters’ anomaly) or other syndromes in which goniodysgenesis is a component[10,17]
The purpose of this report is to present a case of the Rieger syndrome associated with bilateral cleft lip and palate and severe open bite, features not usually reported in association with this condition
Summary
SUMMARY Background/Aim: The Rieger syndrome is a rare, autosomal dominant and phenotypically variable disorder, characterized by abnormalities of the anterior chamber of the eye, coincident with missing or misshapen teeth. Case report: This report features a case of the Rieger syndrome associated with bilateral cleft lip and palate and a severe open bite, findings not usually reported in association with this condition. Conclusions: The findings described in the present case of Rieger syndrome are unusual and expand the spectrum of manifestations of the condition. Smaragda Kavadia[1], Konstantinos Antoniades[2], Eleni Markovitsi[1], Eleftherios G.
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