Abstract
BACKGROUND: No more than half of patients with neurofibromatosis type 1 (NF1)-associated optic pathway gliomas (OPGs) develop vision loss. Prospectively identifying those who will require therapy remains challenging, because no reliable factors have yet been identified that predict future vision loss. To determine whether brain tissue microstructure is associated with visual acuity loss, we examined diffusion tensor imaging (DTI) and ophthalmologic evaluations in children with NF1-associated OPG. METHODS: We retrospectively reviewed ophthalmology records and concurrent DTI measurements of the optic nerves, tracts, and radiations from 50 children with NF1-associated OPGs. Multivariate linear regression measured the association between fiber trajectory quantity and white matter integrity on visual acuity measured by the logarithm of the minimal angle of resolution (logMAR). RESULTS: In multivariate analysis, fractional anisotropy (FA) of the optic radiations was associated with visual acuity loss (adjusted coefficient = -6.081 logMAR/FA; P = .006) after adjusting for age, extent of tumor, DTI acquisition type, prior chemotherapy, and fundus examination findings. The association remained after eliminating tumors involving the optic radiations. In an evaluation of 15 subjects with paired ophthalmologic examination and DTI a year apart, initial FA of the optic radiation was associated with a trend toward change in visual acuity a year later (coefficient = -2.652 logMAR/FA; P = .069). CONCLUSIONS: A decrease in FA of the optic radiations is associated with abnormal visual acuity in NF1-associated OPGs and may be predictive of visual acuity loss during the following year.