Dr.Jyoti Gontia

Dr.Preeti Rawat, Dr. shruti chaudhary, Dr.Shivati Sahu

Abstract

INTRODUCTION: Blepharophimosis Ptosis Epicanthus Inversus Syndrome(BPES) ,inherited eyelid syndrome with telecanthus, epicanthus inversus & ptosis, inheritance is AD & sporadic. BPES caused by mutation in the FOXL2 gene on Chr 3.

CASE DISCUSSION:17 year male presented bilateral small eyes,drooping of upper eyelids since birth,associated with DOV, watering. Past h/o of oculoplasty surgery (V-Yplasty with MCL tightening) elsewhere. Ocular examination: vision BE 6/12, anterior segment examination :severe ptosis present, head slightly tilted backwards, IPD 60 mm, Intercanthal distance 40 mm. Systemic examination WNL. managed by Mustarde (double Z plasty) under LA done for epicanthus and telecanthus correction. Ptosis surgery is planned for future

CONCLUSION: BPES treatment initially includes correction of epicanthus and telecanthus followed by ptosis correction.It is also important as it improves patient’s vision and quality of life.