Vitreous fluorophotometry in carriers of choroideremia and X-linked retinitis pigmentosa
M. M. Rusin, G. A. Fishman, J. A. Larson and L. D. Gilbert
Department of Ophthalmology, Eye and Ear Infirmary, University of Illinois College of Medicine, Chicago 60612.
The status of the blood-retinal barrier (BRB) in carriers of choroideremia
and X-linked retinitis pigmentosa (XLRP) was determined by vitreous
fluorophotometry (VF) and compared with that in female control subjects.
Electroretinographic (ERG) amplitudes were measured to determine the
overall functional integrity of retinal rods and cones. Comparison of the
VF results showed an abnormal BRB in at least some carriers of XLRP,
particularly those with peripheral fundus pigmentary changes, but not in
carriers of choroideremia with even moderately extensive pigmentary
changes. The abnormal BRB in XLRP carriers, with or without peripheral
fundus pigmentary changes, was associated with at least moderate to
moderately extensive reduction in scotopic ERG amplitudes, while the normal
VF results in choroideremia carriers were associated with normal scotopic
ERG amplitudes. However, in XLRP carriers, mild to modest reductions in ERG
scotopic responses were seen in the presence of normal VF findings.