Dr.Jyoti Deswal

Dr. NEEBHA ANAND, Dr. CHAWLA URMIL, Dr. ASHOK KUMAR KHURANA

Abstract

A 31 yr old female presented with drooping of b/l upper eyelids since 5 years. She also had h/o squint surgery 10 yrs back for exotropia. On examination, BCVA was 6/12 (B/E). Patient had severe b/l ptosis, with fair LPS action. Exotropia of 10PD was present with restriction of ocular movements in all directions. Fundus showed salt and pepper appearance of retina. ERG showed B/E reduced photopic and scotopic responses. Diagnosis of Chronic Progressive External Ophthalmoplegia (CPEO) was made. Cardiological and neurological work-up was done to rule out any other associated disorders. Patient underwent bilateral sling surgery for correction of ptosis with slight undercorrection in view of poor Bell’s phenomenon. Post op patient was satisfied. Kearns-Sayre syndrome is a mitochondrial DNA (mtDNA) deletion syndrome. Our patient had typical signs of this syndrome. Treatment for this slowly progressive disorder is generally symptomatic and supportive.