Delhi Journal of Ophthalmology

Axenfeld-Rieger Syndrome: A Rare Case

Neeti Gupta,  Shrinkhal, Kirti Aggarwal, Rimpi Rana, Manisha Sharma, Sanjeev Kumar Mittal 
Department of Ophthalmology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India

Editor-in-Chief, Delhi Journal of Ophthalmology, Dr R.P.Centre, AIIMS.


Corresponding Author:

SHRINKHAL (MBBS, MS, DNB, MNAMS)
Department Of Ophthalmology, 
AIIMS, Rishikesh, Uttrakhand
E-mail ID: shrinkhalbhu@gmail.com


Received: 18-NOV-2019 Accepted: 28-FEB-2020 Published Online: 09-OCT-2020
DOI: https://dx.doi.org/10.7869/djo.571

Abstract
A typical case of Axenfeld-Rieger syndrome (ARS) with clinical features of bilateral posterior embryotoxon, correctopia, pseudopolycoria and segmental iris hypoplasia is presented. Gonioscopy revealed anteriorly displaced Schwalbe’s line and bands of iris extending across the iridocorneal angle to the trabecular meshwork. Fundus was within normal limits in both the eyes. Systemic examination revealed hypodontia, microdontia, prominent lower lip and redundant peri-umbilical skin. There was no associated cardiovascular or other associated systemic association. Axenfeld-Rieger syndrome is a multi-system anomaly requiring a multi-disciplinary approach to management. The patient needs to be regularly followed-up for development of glaucoma

Keywords :Axenfeld-Rieger syndrome, microdontia, pseudopolycoria, redundant peri-umbilical skin