Posters

Corneal confocal microscopy in a case of Kearns-Sayre syndrome

Poster Details

First Author: M. Shoja IRAN, ISLAMIC REPUBLIC OF

Co Author(s):    S. Salimpoor   M. Mahdavi                 

Abstract Details

Purpose:

A case of Kearns-Sayre syndrome (KSS) with corneal endothelial abnormality is reported

Setting:

A 31-year-old woman was referred to our clinic with history of bilateral visual loss, nyctalopia, drooping of the upper lids, and progressive limitation in eye movements

Methods:

The patient underwent uneventful bilateral cataract surgery. Three years later, ophthalmic examination disclosed bolus keratopathy in left eye while in the right eye, no obvious corneal edema was observed

Results:

Ocular and systemic examination revealed retinitis pigmentosa, progressive external ophthalmoplegia, bilateral posterior sub capsular cataract, conductive heart block, muscle weakness, and short stature. Clinical diagnosis of KSS was confirmed by muscle biopsy appearance of ragged red fibers..The corneal endothelium examined by confocal microscope showed marked decrease in cell density, severe polymegathism, and pleomorphism in both eyes while severity was more prominent in the left eye.

Conclusions:

The fact that abnormal mitochondrial dysfunction ,the main pathogenicity of this disorder,preferentially affects cells with high energy demands including corneal endothelium may explain increased apoptosis in these cells.Consideration of this susceptibility of Corneal endothelial to decompensation is important specially when an intraocular procedure is scheduled

Financial Disclosure:

NONE