Axenfeld-Rieger Syndrome: Rare Case Presentation and Overview.

• 发表时间: Sep 2020
• 来源期刊: J Maxillofac Oral Surg
• DOI: 10.1007/s12663-019-01307-9
• PMID: 32801529
• 文章类型: Case Reports
• 作者列表: Agarwal P,Jain K,Sandesh S,Chopra S

关键词: Anodontia; Axenfeld–Rieger syndrome; Craniofacial abnormalities; Oligodontia; Telecanthus; Tooth abnormalities

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.