Axenfeld-Rieger syndrome: a case report

Abstract

Axenfeld-Rieger syndrome (ARS) is a rare autosomal dominant condition manifesting as a heterogeneous group of features. Of particular note are the ocular and craniofacial anomalies and dental features such as hypodontia, microdontia, taurodontism, enamel hypoplasia, conical-shaped teeth, shortened roots and delayed eruption. To treat cases with ARS effectively, a multidisciplinary approach is required, and this report describes the complex and long-term management of a case with input from Paediatric Dentistry, Orthodontics, Restorative Dentistry, Speech and Language Therapy, Oral and Maxillofacial Surgery and Radiology.

Keywords: Axenfeld Rieger syndrome; multidisciplinary care; orthognathic surgery; severe hypodontia.