Andrew Q. McCormick

PERMANENT CORTICAL VISUAL IMPAIRMENT IN CHILDREN

Fifty patients with permanent cortical visual impairment were evaluated. They had a characteristic behaviour profile, usually with residual sight but poor visual attention. 30 of the 50 also had damage to the anterior visual pathway. Visual evoked potential mapping was shown to have a clear advantage over visual evoked responses, and using that in conjunction with CT and clinical data enabled several subgroups of cortical visual impairment to be identified. The diagnosis probably is more common than previously recognised, and should be suspected when there is greater delay in visual development in other areas and the degree of visual loss is unexplained by ocular findings. Using traditional criteria for cortical blindness may mean that many children are not diagnosed, which has serious implications for their rehabilitation.

CLINICAL SPECTRUM OF CONGENITAL OPTIC NERVE HVPOPLASIA: REVIEW OF 51 PATIENTS

Fifty‐one patients with congenital optic nerve hypoplasia (CONH) were reviewed. It was found tkat the risk of having an affected child is higher in an adolescent mother, and that maternal alcohol or drug abuse may be important factors. Frequently the disorder is associated with other neuropsychiatric handicaps, and with neuro‐endocrine abnormalities. The findings suggest that CONH probably is not a homogeneous group of disorders; some may be caused by primary failure of differentiation of the retinal ganglion cells, while others may be the product of an acquired transsynaptic degeneration of optic‐nerve fibres.

Permanent visual loss after shunt malfunction

Fourteen (1.8%) of more than 800 children evaluated in a Visually Impaired Program over 10 years became permanently blind during an episode of raised intracranial pressure secondary to shunt malfunction. Visual symptoms and papilledema were recognized in only three children at the onset of blindness. Clinical and radiologic findings suggested that the loss of sight was caused by a lesion in the pregeniculate pathway in nine patients, probably due to ischemia; five children in the postgeniculate group sustained infarcts of the occipital lobes. These cases highlight the importance of parent education and prompt treatment of raised intracranial pressure due to shunt malfunction.

Vasculopathy with renal artery stenosis in a child with sarcoidosis

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Eye-Pressing by Visually Impaired Children.

Many children with severely impaired sight exhibit stereotyped mannerisms. Visual self‐stimulation, e. g. eye‐pressing and light‐gazing, normally is restricted to the visually impaired; prolonged eye‐pressing is the most common. This behaviour depends on onset of visual impairment, age, degree and quality of residual light, type of ocular abnormality, the presence of additional handicaps, and the activities in which the child is involved. Children with bilateral optic‐nerve defects never press their eyes; those with retinal disorders tend to press vigorously. A possible physiological explanation is that self‐stimulation occurs when the demand of the brain for meaningful visual information is not adequately met.

THE UNEQUAL NYSTAGMUS TEST

In children who exhibit nystagmus secondary to visual impairment, the better eye determines the quality of the nystagmus. 40 children with different visual acuities and 10 with the same vision in each eye were studied. By inspecting the behaviour of the nystagmus in each eye, while covering the other, it was possible to identify which eye had better foveal vision. This co‐called ‘unequal nystagmus test’ is very simple to do and it offers valuable information.