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阿克森费尔德-里格尔综合征:罕见病例报告及概述

Axenfeld-Rieger Syndrome: Rare Case Presentation and Overview.

作者信息

Agarwal Padmanidhi, Jain Kanav, Sandesh Shubham, Chopra Sakshi

机构信息

Department of Dentistry and Maxillofacial Surgery, All India Institute of Medical Sciences (AIIMS), Rishikesh, India.

Teerthanker Mahaveer Dental College, Moradabad, India.

出版信息

J Maxillofac Oral Surg. 2020 Sep;19(3):364-369. doi: 10.1007/s12663-019-01307-9. Epub 2019 Nov 23.

Abstract

Axenfeld-Rieger syndrome (ARS) is an extremely rare autosomal dominant disorder characterized by ocular, craniofacial, dental and periumbilical abnormalities. We present a case of a 10-year-old boy. Its awareness among oral surgeons is essential for timely diagnosis and subsequent prevention of ophthalmic and systemic complications as craniofacial and dental features constitute the early recognizable symptoms of this syndrome. Systematic ophthalmic surgeries aid in relieving vision abnormalities, while symptomatic dental treatment should be provided for masticatory and esthetic rehabilitation.

摘要

阿克森费尔德-里格尔综合征(ARS)是一种极为罕见的常染色体显性疾病,其特征为眼部、颅面部、牙齿及脐周异常。我们报告一例10岁男孩的病例。由于颅面部和牙齿特征是该综合征早期可识别的症状,口腔外科医生了解该综合征对于及时诊断及随后预防眼部和全身并发症至关重要。系统性眼科手术有助于缓解视力异常,而对症牙科治疗则应提供以进行咀嚼和美观修复。

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引用本文的文献

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Dental and Maxillofacial Manifestations of Axenfeld-Rieger Syndrome: Presentation of a Case in a 5-Year-Old Girl.
Case Rep Dent. 2022 Aug 2;2022:4348264. doi: 10.1155/2022/4348264. eCollection 2022.

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