Correlation of visual acuity with foveal hypoplasia grading by optical coherence tomography in albinism.

PURPOSE: To develop a new foveal hypoplasia grading system using optical coherence tomography (OCT) and to determine the correlations of visual acuity to the iris transillumination, macular transparency, and foveal hypoplasia grades, respectively, in young albinism patients.

DESIGN: Observational case series.

PARTICIPANTS: Thirteen patients who were clinically diagnosed with oculocutaneous albinism.

METHODS: The authors obtained refraction and correction for participants, who then underwent anterior segment photography and fundus photography. Foveal hypoplasia was evaluated using a fast macular cross-hair scan with StratusOCT (Carl Zeiss Meditec, Dublin, CA). The authors developed a new grading system based on foveal hyporeflectivity, the degree of choroidal transillumination, the presence of the tram-tract sign, and foveal depression as determined from OCT images. Correlations of Snellen visual acuity to the iris transillumination, macular transparency, and foveal hypoplasia grades were calculated, and areas under receiver operating characteristic curves (AROCs) were measured to evaluate the predictive value of foveal hypoplasia grading using OCT.

MAIN OUTCOME MEASURES: The AROCs of foveal hypoplasia grading by OCT.

RESULTS: Thirteen patients were enrolled. Correlations of visual acuity to the iris transillumination (P<0.001), macular transparency (P = 0.014), and foveal hypoplasia (P = 0.004) grades were significant. The AROC values for foveal hypoplasia measurement by OCT, iris transillumination, and macular transparency measurements were 0.98, 0.91, and 0.87, respectively.

CONCLUSIONS: The prognostic value of foveal hypoplasia as assessed by OCT in young patients with albinism is superior to grades based on iris transillumination or macular transparency.