FP2500 : CASE REPORT ON KEARNS SAYRE SYNDROME PRESENTING WITH PTOSIS, CPEOM, PIGMENT RETINOPATHY

Abstract

STUDY DESIGN:case report
BACKGROUND :Kearns Sayre Syndrome is a mitochondrial encephalomyopathy characterized by the presence of “ragged red fibers” seen in Trichrome stained skeletal muscle biopsies and has characteristic syndromic features which include: Chronic progressive external ophthalmoplegia (CPEO), bilateral atypical pigmentary retinopathy and cardiac conduction abnormalities.

PURPOSE: To report a rare case of kearns sayre syndrome.
METHOD: A 15 yr old male child presented with drooping of left upper eyelid since one year.
RESULTS: Ocular examination,BCVA was Log MAR 0.2 in BE. BE had ptosis with mild ptosis in right eye and a severe ptosis in the left eye with poor LPS function. There was restriction in extraocular movements of both eyes especially in elevation. Fundoscopy revealed a characteristic salt and pepper background.
CONCLUSION: Early recognition can help in early diagnosis and prompt intervention with pacemaker of the cardiac condition.

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