Abstract
Purpose :
Cerebral palsy (CP) is characterized by perinatal onset neurologic damage with global physical and neurologic dysfunction. We have previously shown that optic nerve head (ONH) cupping and pallor can serve as biomarkers for motor deficit and ambulatory status. The main objective of this exploratory cross-sectional study was to evaluate the association of ONH changes (hypoplasia, retinal nerve fiber layer (RNFL) loss and cupping) and visual perception in CP.
Methods :
We studied 9 consecutive patients (5 females) with perinatal onset CP. Patients with media opacity or preexisting diagnosis of glaucoma were excluded. Measurements included visual acuity, intraocular pressure (IOP-ICare, Finland), OCT imaging (Heidelberg, Germany), fundus photographs (Optos®, USA), and the Test of Visual Perception Skills (TVPS version 4).2 masked ophthalmologists independently reviewed fundus photographs to document cup-disc ratio (CDR). A subject was classified as “large cup” if both observers labelled the CDR ≥0.5. ONH was labelled hypoplastic if the horizontal disc diameter to the horizontal disc to macula distance ratio was <0.3.
Results :
Mean age was 15 years (range 11.8-21.2). All the children were ambulatory with or without assistance. The mean best corrected visual acuity in the worse eye was 0.09 log MAR units (range 0-0.3). The mean population percentile score on the TVPS was 31±20. No subject had hypoplastic discs. CDR≥ 0.5 was observed in 7/9 (77%) patients with a mean CDR of 0.7±0.15 in the worse eye. Inter rater reliability was good with a disagreement on cupping between the observers in one case. Only 1 subject had IOP>21 mm Hg but had no signs of glaucoma (CDR<0.5). The mean RNFL thickness in the worse eye was 90 µ (range 40-123). The percentile TVPS score correlated well with the CDR (r2 =0.6) [Figure].
Conclusions :
ONH cupping, RNFL loss and visual perception abnormalities are very common in CP and may reflect global hypoxic neuronal damage. CP should be recognized as a cause of pseudo-glaucomatous cupping and RNFL loss in children to limit unnecessary examinations under anesthesia. We propose that ONH cupping is a marker for visuospatial cognitive deficits in children with CP. CP patients with ONH cupping should have visual perception testing to guide rehabilitation.
This is a 2020 ARVO Annual Meeting abstract.