Retinal Screening of Patients Suffering from Bardet – Biedl Syndrome Using Electroretinography

Bardet-Biedl Syndrome (BBS) is a complex genetic disorder that affects various systems in the body, including the visual system. The aim of this study is to investigate the adverse effects of BBS on the retina using Electroretinography (ERG).

In this case-control study, 10 BBS patients (6 males and 4 females) aged between 12 and 20 years were selected as the case group. Additionally, 10 age- and sex-matched healthy individuals with normal retinal function were included as the control group. ERG recordings were performed to measure the amplitude (μV) and latency (msec) of the ERG b-wave in both groups.

The mean age of the case group was 15.4 ± 3.06 years, and it was 15 ± 2.78 years in the control group. The mean visual acuity in the case group was 0.49 ± 0.14 (LogMAR), significantly different from the control group with a mean visual acuity of 0 ± 0 (LogMAR). The difference in visual acuity was statistically significant between the two groups. Furthermore, the case group showed significantly lower amplitude and higher latency of the ERG b-wave peak compared to the control group (p < 0.001).

Bardet-Biedl Syndrome has adverse effects on the visual system, particularly the retina, resulting in a decrease in the amplitude and an increase in the latency of the ERG b-wave. These findings indicate impaired retinal function in BBS patients, highlighting the importance of early detection and management of retinal abnormalities in individuals affected by this rare genetic disorder.