Results
During the study period, 228 patients with OPG were identified. Of these, 134 received treatment, 38 of whom received BBT. Eleven patients were excluded due to incomplete ophthalmologic records, and 6 were excluded due to early discontinuation of BBT secondary to intolerance. An additional 4 were excluded due to young age at treatment initiation and inability to complete quantifiable VA assessments. The remaining 17 patients were included in the study analysis.
Demographic and clinical information for each patient is displayed in Table 1. Median age was 5.6 years (IQR 2.6-8.8 years) at OPG diagnosis and 7.9 years (IQR 3.8-12.8 years) at BBT initiation. Fourteen had sporadic OPG and 3 had NF1. All but one patient had prior treatment failure, and 9 had multiple prior treatment failures. Indications for BBT included radiographic progression alone (N=11), vision decline alone (N=2), and both radiographic progression and vision decline (N=4). Median follow-up time at last vision assessment was 12.9 months (IQR 11.0-16.6 months).
At baseline, 15 of 17 patients had impairment in VA, VF, or both (displayed by-eye in Figure 1). Ten of 17 patients had at least moderate VA impairment and 8 of 9 patients with evaluable VF had defects at baseline.
Following BBT, 14 of 17 (82%) had stable or improved VA and 6 of 9 (67%) had stable or improved VF. Among the 6 patients with vision decline as the indication for BBT, all but one experienced vision stabilization or improvement in both VA and VF (Table 1). VA and VF outcomes were discordant in 2 patients. Eight (47%) patients had radiographic progression during BBT, 5 of whom had stable vision at time of progression. Vision and radiographic outcome were discordant in 7/17 (41%).