DR. PRITHVI CHANDRAKANTH

DR. VIRNA SHAH, DR. SHISHIR VERGHESE, DR. GEORGE J. MANAYATH

Abstract

A 16-year-old female presented to us with complaints of decreased vision in the right eye (RE) for 5 years and was associated with drooping of eyelids. Best corrected visual acuity in the RE was 6/36 and left eye (LE) was 6/12. Ocular adnexal examination revealed moderate ptosis along with external ophthalmoplegia. Fundus examination revealed atypical pigmentary retinopathy presenting in an asymmetric pattern with RE more affected than the LE, for which a multimodal imaging including an optical coherence tomography angiography was done. Following a complete systemic evaluation which revealed a complete heart block, cerebellar ataxia and was finally diagnosed with Kearns-Sayre syndrome – supportive therapy was advised. We describe the relatively new findings on multimodal imaging of atypical pigmentary retinopathy in KSS as well as its presentation in an asymmetric manner.