The electroretinogram in children.

PURPOSE OF REVIEW: The electroretinogram is an essential tool in the evaluation of the functional integrity of the retina, which may be especially useful in preverbal children. The present review describes the modifications needed for performing an electroretinogram in young children. Also, we present electroretinogram studies that have added to our understanding of the maturation of the visual system, the development of retinopathy of prematurity, and ametropia. RECENT FINDINGS: The selected articles were grouped into four subjects: the technical modifications for performing a pediatric electroretinogram, including the possible need for sedation, the patient's position, and the recording electrodes; the maturation of the electroretinogram in preterm infants and in the first years of life; advancements in the understanding of retinopathy of prematurity; and understanding the development of ametropia based on the electroretinogram findings. SUMMARY: Although the technique for performing an electroretinogram must be modified for young children, when needed, it can and should be performed early in life. This will yield an early diagnosis that may be useful for planning family and personal life.

Eye manifestations of intrauterine infections and their impact on childhood blindness.

Intrauterine infections are important causes of childhood blindness in both developed and developing countries. Chorioretinal scars are the most characteristic eye manifestation of a congenital or prenatal infection. The various ocular manifestations of congenital infections, summarized by the mnemonic TORCH, and recent additions to the "other" category (lymphocytic choriomeningitis virus and West Nile virus) are discussed.

The natural history of retinal degeneration in association with cobalamin C (cbl C) disease.

PURPOSE: To describe the association between retinal degeneration and cobalamin C (cblC) disease and to review previously published ophthalmic data regarding cblC disease. METHODS: Descriptive case series of three patients and compilation of all previously reported cases of cblC disease in the ophthalmic literature. RESULTS: All three new cases presented with macular pigmentary changes and showed attenuation of electroretinographic responses. Sequential ERG (electroretinogram) testing in Case 1 demonstrated ERGs that began at the lower limits of normal and became progressively attenuated over time. CONCLUSIONS: Cobalamin C disease results in progressive retinal degeneration with its onset in the first few months of life and progressing rapidly over the first few years of life.

Eye findings of diffuse unilateral subacute neuroretinitis and multiple choroidal infiltrates associated with neural larva migrans due to Bbaylisascaris procyonis.

PURPOSE: To report childhood infection with Baylisascaris procyonis (raccoon round worm) manifesting as diffuse unilateral subacute neuroretinitis (DUSN) and choroidal infiltrates in association with neurologic disease (neural larva migrans). METHOD: Observational case series, one with eye manifestations of DUSN, the other with choroidal infiltrates, both with severe neurologic degeneration. RESULTS: Indirect immunofluorescence assays on serum and cerebrospinal fluid were positive for B. procyonis in one and serially positive and increasing in the other. Both children had a history of pica and raccoon exposure. CONCLUSIONS: Baylisascaris procyonis infection is associated with two cases of severe neurologic degeneration with ocular lesions: DUSN and choroidal infiltrates. Although B. procyonis is known to cause DUSN, these cases indicate that concomitant ocular migration may accompany neural larva migrans. These are the third and forth cases in the US literature of neural larva migrans due to B. procyonis with eye findings.