Ebstein anomaly associated with retinal venular dilatation, migraine, and visual snow syndrome: a case report.

BACKGROUND: To present a case with Ebstein anomaly, a rare congenital heart disorder, with ophthalmological and neurophthalmological signs and symptoms. To date, retinal venous dilatation and visual snow syndrome have not been previously been published in this anomaly. CASE PRESENTATION: A 10-year-old white girl was diagnosed with Ebstein anomaly. From age 12, she regularly suffered from migraines with auras and photophobia. At age 16 she started to see short-term bouts of white snow, that after a year were present all day. At age 20, she was found to have a decreased retinal arteriovenous ratio. CONCLUSIONS: Retinal arterial tortuosity and venular dilatation are common in congenital heart disease but have not been described in Ebstein anomaly, nor has the visual snow syndrome.

Safety evaluation of subretinal injection of trypan blue in rats.

OBJECTIVE: To determine the appropriate concentration of trypan blue (TB) for subretinal injection in a rat model and to provide a safety profile that limits retinal toxicity while maintaining dye visibility. MATERIALS AND METHODS: Adult rats were subretinally injected with various concentrations of either TB or phosphate-buffered saline (PBS); rats which received sham injections served as an additional control. The injected areas were visualized under a surgical microscope. Electroretinography (ERG) was performed to measure retinal function. Animals were then sacrificed, and the eyes were sectioned and examined by light microscopy. Terminal deoxynucleotidy1 transferase dUTP nick-end labeling (TUNEL) was applied to determine retinal apoptosis. RESULTS: One day after the subretinal injection, TB stains were visible under the surgical microscope in the 0.2%, 0.08%, and 0.04% TB-injected groups, but not in the 0.02% TB-injected group. TB stain was detectable in the retina and sclera of the 0.2%, 0.08%, and 0.04% TB-injected groups for over 2 weeks after injection. However, the amplitudes of ERGa- and b-waves were affected and became significantly lower in the 0.2% TB-injected group than the amplitudes in the PBS-, or sham-injected group. Moreover, TUNEL+ cells appeared in the outer nuclear layer (ONL), ganglion cell layer (GCL), and retinal pigment epithelium (RPE) layer of the 0.2% and 0.08% TB-injected groups at 1 and 7 days after subretinal injection. In contrast, very few TUNEL+ cells were found in the 0.04% TB- or PBS-injected group. Two weeks after injection, the ONL was significantly thinner in the 0.2% TB-injected group than in the 0.04% TB-, PBS- or sham-injected group. CONCLUSIONS: TB injection induces a dose-dependent neurotoxic effect on retinal cells. Subretinal injection of 0.04% TB is relatively safe and effective for subretinal staining.