1Consultant, Dept. of Oculoplast and Cataract, Rotary Narayana Nethralaya, Kolkata
2Consultant, Ispat General Hospital, Rourkela, Odisha, India
*Corresponding Author: Madhusmita Behera, Email: madhusmita.behera@rediffmail.com
Online published on 14 January, 2019.
Bardet-Biedl syndrome (BBS) is a rare autosomal recessive disorder with wide spectrum of clinical features. BBs is distinguished from the much rarer Laurence-moon syndrome, in which retinal pigmentary degeneration, mental retardation, and hypogonadism occur in association with progressive spastic paraparesis and distal muscle weakness, but without polydactyly. Most common feature of BBS is retinal dystrophy. The visual prognosis for children with Bardet-Biedl syndrome is poor.
Mental retardation, Pigmentary retinopathy, Polydactyly
