Natural course of visual functions in the Bardet-Biedl syndrome
A. B. Fulton, R. M. Hansen and R. J. Glynn
Department of Ophthalmology, Children's Hospital, Boston, Mass.
OBJECTIVE: To determine the course of visual functions in patients with
Bardet-Biedl syndrome. PATIENTS AND METHODS: The 21 patients with
Bardet-Biedl syndrome seen at Children's Hospital, Boston, Mass, had
optotype and grating acuities and dark-adapted thresholds measured over
time. Their ages at first visit ranged from 2 weeks to 23 years (median
age, 6 years). The courses of the visual functions were analyzed with a
random-effects model. RESULTS: Substantial declines in visual functions
were found. On average, grating and optotype acuities declined 0.09 log
units (roughly 1 line) per year, and thresholds increased about 0.19 log
units per year. The rates at which these visual functions were lost and the
predicted level of the visual functions at ages 11 to 12 years (the mean
ages of measurement) varied among individuals. CONCLUSIONS: The visual
prognosis for children with Bardet-Biedl syndrome is poor. The course of
both central and peripheral visual functions is variable.
