Título

ATYPICAL PRESENTATION OF CHANDLER SYNDROME: A CASE REPORT

Introdução

 Iridocorneal endothelial (ICE) syndrome encompasses a group of diseases that affect the corneal endothelium, often accompanied by iris abnormalities and glaucoma in a single eye.1 ICE is subdivided into three subtypes (1) Chandler syndrome (CS), (2) progressive iris atrophy and (3) Cogan-Reese syndrome.2 The present report depicts a rare case of a middle-aged man presenting unilateral corneal edema with findings suggestive of Iridocorneal endothelial (ICE) syndrome in its variant Chandler.

Métodos

Case report of a male adult with a recent complaint of low visual acuity and blurred vision accompanied by unilateral corneal edema

Resultados

A.P.P.A., a sixty-eight-year-old man referred redness and tearing in his left eye with several relapsing episodes of blurred vision, without pain, that resolved spontaneously over the past 6 years. He was taking oral acyclovir and 1% Prednisolone drops q.d for the past 2 weeks. In his medical record, there was a presumed diagnosis of Fuchs corneal endothelial dystrophy. The routine ophthalmologic exam showed an inferior paracentral focal stromal edema in the left cornea, The visual acuity was 20/60 in the left eye, and 20/20 in the other eye. The fundus exam revealed an increased cup/disc ratio that did not respect the ISNT rule  Under gonioscopy of the left eye, there was an appositional contact between the iris and pigmentaryt rabeculum inferiorly, without synechias or other abnormalities. Biomicroscopy, gonioscopy and fundus exam of the fellow eye were otherwise normal.  The pachymetry map obtained with the scheimpflug was consistent with biomicroscopy, showing an increased corneal thickness paracentral in the left eye (700-740 µm). The specular microscopy showed a homogeneous and normocytic endothelial monolayer of the right eye with cell counts above 2.000 cells/mm2, while in the left eye, adjacent to the edematous area it was possible to identify enlarged polygonal cells at the level of the endothelium. Besides, the cell counts were between 800-1.400 cells/ mm2 presented with disrupted and irregular cell border patterns with a reversal of the light/dark color of the cytoplasm/cell wall. 

Conclusões

Chandler syndrome is a rare condition which is often challenging to manage due to the multiple comorbidities associated with it. That might interfere with the therapeutical success. Proper diagnosis is warranted to establish a therapeutial plan and avoid complications

Palavras Chave

chandler, cornea, edema, man, keratoplasty

Arquivos

Área

Patologia Clínica