Cerebral visual impairment (CVI), a neurological disorder of childhood caused by damage to the retrochiasmatic visual pathways in the absence of any major ocular disease, is the main cause of visual deficit in children in the developed world. Although clinical findings are extremely variable, reduced visual acuity, of variable entity but always associated with some residual visual function, is the principal and most frequent neurophthalmological sign. We set out to describe the spectrum of neurophthalmological and ophthalmological features in a sample of patients with CVI (of varying aetiology), diagnosed according to Good, who underwent a protocol including neurological and neurophthalmological assessment (functional visual assessment, ophthalmological and electrophysiological examination, brain MRI). We report our findings in the most severely affected children (30 patients in whom visual acuity was not quantifiable using standardized measures), paying particular attention to their residual visual function, documented through the presence of neurobehavioral adaptations, also defined as direct and indirect signs of visual perception. Neurobehavioral adaptations were documented in all the subjects, confirming the principle that some residual visual function is always conserved, even in the most severely affected children. Early and careful assessment of these children is essential for a correct diagnosis and for the development of personalized rehabilitation programmes
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