Abstract

Study design-It is an observational study.
Purpose-A study of family presented with BPES.
Method-4cases of BPES syndrome a father with his three offsprings came to ophthalmology OPD with complain of diminution of vision.First case 64 years old male with visual acuity in RE- FC@3M LE- PL+PR +.Second case 40 years old female with visual acuity in RE- 6/60 LE- 6/24.Third case 38 years old male with visual acuity in RE- 6/18 LE- 6/24.Fourth case 30 years old female with visual acuity in RE- 6/24 LE- 6/24
All of them had complaints of drooping of both eye eyelids, on examination-blepharophymosis, epicathus inversus.Both females were nulligravida & had amenorrhoea s/o primary ovarian failure.
Result-All the 4 patients were suspected to have BPES.
CONCLUSION-BPES is a rare congenital oculofacial disorder inherited in an AD pattern .
Counselling of patient at the earliest can help to improve the visual outcome.Medial canthoplasty, ptosis correction & hormone replacement therapy.