Larger Tumor Volume is Associated with Visual Acuity Loss and Axonal Degeneration in Children with Optic Pathway Gliomas Secondary to Neurofibromatosis Type 1

Abstract

Abstract BACKGROUND We investigated whether volumetric magnetic resonance imaging (MRI) measures of the anterior visual pathway (AVP) in optic pathway gliomas secondary to Neurofibromatosis type 1 (NF1-OPG) are associated with visual acuity (VA) loss and axonal loss, as measured using optical coherence tomography (OCT). METHODS Children with NF1-OPGs enrolled in a prospective study of VA and OCT measures of the circumpapillary retinal nerve fiber layer (cpRNFL) thickness were eligible if they had undergone 3-Tesla MRI that included a T1-weighted volumetric sequence. The linear dimension and volume of the optic nerves, chiasm and optic tracts were measured using our semi-automated algorithm. The combined volume of these components comprising the proximal AVP was used as a surrogate of total tumor burden. Regression models for VA and cpRNFL thickness were reported on a per-eye basis. RESULTS Fifty-two study eyes (26 children, mean 7.1 years) met inclusion criteria, of which 40% (N=21 eyes) had abnormal VA. In regression analysis, only total AVP volume demonstrated a significant relationship to axonal loss, such that for every 1 ml increase in AVP volume, cpRNFL declined by 5.4 microns (p = 0.01). Total AVP volume > 1.75 ml predicted both axonal loss and abnormal VA (positive predictive value of 83.3% and 70.8%, respectively; negative predictive value of 80.0% and 88.0%, respectively). CONCLUSIONS Volumetric measures of NF1-OPGs identified children with VA loss and axonal degeneration. Total tumor burden, as measured by AVP volume, had the strongest relationship with axonal injury. NF1-OPG volumetric measures may be helpful in making treatment decisions.