Management of ptosis in Kearns Sayre syndrome (KSS): a case report and Literature review on Management of ptosis in KSS

Abstract

Kearns Sayre syndrome (KSS) is a rare mitochondrial disease that affects young adults, due to a deletion of mitochondrial DNA and characterized by the triad: age of onset lower than 20 years, chronic progressive external ophthalmoplegia and an atypical pigmentary retinopathy. It’s also characterized by other endocrine, neurological and especially cardiac impairment with a very high risk of cardiac complications during surgical procedures under all types of anesthesia. We report a case of KSS revealed by sever bilateral ptosis and confirmed by a muscle biopsy with “ragged red fibers”. The ptosis was surgically managed by cautious Frontal suspension under local anesthesia “Frontal nerve block”. Through this case we discuss challenges in the management of KSS patients.