Jena Ashok Kumar, Kharbanda Om P
Centre for Dental Education and Research, All India Institute of Medical Sciences, New Delhi, India.
J Clin Pediatr Dent. 2005 Fall;30(1):83-8. doi: 10.17796/jcpd.30.1.v1732398454r0244.
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.
Axenfeld-Rieger综合征是一种罕见的常染色体显性疾病,其特征为各种眼内和眼外畸形。牙齿异常被认为是Rieger综合征诊断和与其他眼前房畸形相鉴别的决定性特征。本文描述了一例伴有明显牙齿和颅面异常的Rieger综合征病例。观察到的显著颅-牙-面表现为:牙根短且弯曲、系带增生和上颌骨发育不全。存在前牙反合、双侧后牙开合以及中度至重度前牙拥挤。