Dr. Georey Joju, Dr. Anthrayos C V Kakkanatt, Dr. David Pudukadan, Dr. Ms. Minu Sasikumar
A 19 year old girl and 15 year old boy who are siblings were brought to the OP department with a history of reduced vision in both eyes since childhood.Both of them were having poor scholastic performances.On examination, the girl was obese with BMI of 32, had polydactyly and syndactyly and was having vision of 1/60 in both eyes and also had defective colour vision.Fundus examination showed bull’s eye maculopathy in both eyes. Th boy was also obese of BMI 30, had polydactyly, gynaecomastia and was having vision of 1/2 / 60 in both eyes and deffective colour vision. Fundus showed bull’s eye maculopathy. Bardet–Biedl syndrome (BBS) is a human genetic disorder that produces many effects and affects many body systems. It is characterized principally by obesity,retinitis pigmentosa(RP),polydactyly,hypogonadism and renal failure in some cases.Bull’s eye maculopathy is a rare form of RP.It is a rare cause of familial blindness of which ophthalmologists should be aware of for timely referal.