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Axenfeld-Rieger syndrome: case report

Poster Details

First Author: A.Gonçalves BRAZIL

Co Author(s):    B. Negreiros   T. Valadao                 

Abstract Details


To report the case of a patient with probable diagnosis of ocular axenfeld-rieger syndrome.


Ophtalmology Group at Corneal Department of the Federal Hospital of Bonsucesso situated in Rio de Janeiro – Brazil.


The pacient was referred to corneal department complaining of low visual acuity since birth. We performed complete ophthalmologic evaluations o, including a slit-lamp examination her, fundus examination and photography, anterior segment photography, gonioscopic examination and photography and visual field test (Humphrey Visual Fields Analyzer 24-2; Carl Zeiss, Jena, Germany).


A 19-year-old white female patient with low visual acuity and multiple pupils in both eyes since birth. It presents antecedent of bronchial asthma and some teeth in palate already extracted and hypertelorism. Visual acuity of 20/50 and 20/400 without improvement with correction. Biomicroscopy: polycystic and uveal ectropion and corectopia and anterior synechiae in both eyes. Fundus optic discs were pale with an atrophic appearance of raised lips and constricted vasculature. Gonioscopy: tissue bands from peripheral iris to a prominent line of the Schwalbe line. Intraocular pressures were 18 and 15mmHg. Specular microscopy revealed an endothelial count of 2734 and 2775.


This study may help to understand clinical findings and prognosis for patients with Axenfeld-Rieger syndrome.

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