In children with INS, some early-onset retinal dystrophies can be differentiated through ERGs, also with skin electrodes.

Background: Childhood vision screening is aimed at the detection of reduced vision due to amblyopia, thus enabling early diagnosis and timely intervention. The purpose of the study was to review the demographics and visual parameters of children referred to the ophthalmologist at Community Health Centre Ljubljana from Slovenian community-based vision screening program and define the visual outcome after treatment in children with amblyopia. Methods: Retrospective medical records review of children referred from community-based vision screening program for further assessment. Medical records were reviewed to determine findings from ophthalmic assessments, treatment received, and visual acuity at the final visit. The main outcome measures were the cause of visual impairment and the visual acuity at the final follow-up visit. Results: From 439 children (mean age 7.3 +/- 3.7 years) referred from community-based vision screening program, 75 children (17%; mean age 5.3 +/- 2.6 years) had amblyopia and received treatment. They had amblyogenic refractive error (3.67 +/- 2.44 diopters of sphere and 1.86 +/- 1.23 diopters of astigmatism) with uncorrected visual acuity on average 0.32 +/- 0.28 logMAR in the worse eye. Visual outcomes after treatment were good with a visual acuity of 0.04 +/- 0.07 logMAR in the worse eye, 60% of them had a visual acuity of 0.00 logMAR (40/40). Conclusion: Children with amblyopia in the presented clinical sample were mostly diagnosed and treated before the school-age. These children showed significant improvement in visual acuity in the amblyopic eye.

Amblyopia is a reduction of vision in one or both eyes due to a failure of normal neural development in the immature visual system.Amblyopia occurs due to two basic conditions – abnormal binocular interaction (e.g., strabismus) and blurring or distortion of visual image due to uncorrected refractive errors or media opacities. Best-corrected visual acuities in amblyopic eyes range from mild deficits to severe vision loss.The principle of treating amblyopia involves clearing any image blur and encouraging use of the amblyopic eye with occlusion of the better-seeing eye. Paediatric Eye Disease Investigator Group (PEDIG) studies show that both part-time and full-time occlusions can produce similar results in the eye with severe amblyopia and occlusion can be prescribed initially at 2 hours per day for the moderate amblyope. Studies show that daily atropine and patching for 6 hours/day can be equivalent treatment options and that if pharmacologic blurring is used for treatment, initial treatment can begin with just weekend use of atropine for moderate amblyopia.Amblyopia can lead to permanent loss of vision; therefore vision screening is strongly recommended over the course of childhood to detect amblyopia early enough to allow successful treatment.

Purpose: To study electroretinograms in infantile nystagmus syndrome associated with idiopathic infantile nystagmus, optic nerve hypoplasia, and albinism. Methods: A total of 30 children with idiopathic infantile nystagmus, 18 with optic nerve hypoplasia, and 18 with albinism were studied. Three electroretinogram protocols were applied according to child’s age: 58 (mean: 2.0 years) were recorded with skin electrode to Great Ormond Street Hospital protocol, 11 (mean: 5.3 years) with skin electrode to International Society for Clinical Electrophysiology of Vision protocol, and 7 children (mean: 12.2 years) with HK electrode to International Society for Clinical Electrophysiology of Vision protocol. The electroretinograms were compared to those of age-matched controls. Results: Electroretinogram waveforms in idiopathic infantile nystagmus, optic nerve hypoplasia, and albinism were comparable to controls in all protocols. Electroretinogram amplitudes in idiopathic infantile nystagmus group showed increased white scotopic and photopic electroretinograms in 26 children (skin electrode to Great Ormond Street Hospital protocol), no difference to the controls in 3 children (skin electrode to International Society for Clinical Electrophysiology of Vision protocol), and increased rod electroretinogram in 3 children (HK electrode to International Society for Clinical Electrophysiology of Vision protocol). Optic nerve hypoplasia group showed increased white scotopic, photopic, and blue electroretinograms in 15 children (skin electrode to Great Ormond Street Hospital protocol); increased 30-Hz electroretinogram in 3 children (HK electrode to International Society for Clinical Electrophysiology of Vision protocol); and reduced combined rod-cone, cone, and 30-Hz electroretinograms in 3 children (skin electrode to International Society for Clinical Electrophysiology of Vision protocol). Albinism group showed increased white scotopic, photopic, and 30-Hz electroretinograms in 17 children (skin electrode to Great Ormond Street Hospital protocol), while it showed reduced cone and 30-Hz electroretinograms in 5 children (skin electrode to International Society for Clinical Electrophysiology of Vision protocol). Implicit times were shorter in albinism. Conclusion: Electroretinogram waveforms in idiopathic infantile nystagmus, optic nerve hypoplasia, and albinism were normal with mostly increased electroretinograms, while reduced electroretinograms did not show a specific pattern as in early-onset retinal dystrophies.

The correction of compensatory head posture in a congenital nystagmus involves surgical treatment that includes recession and resection of extraocular muscles to move the eccentric null zone to a primary position. A 39-year-old patient presented with an impaired visual acuity and nystagmus that was present since childhood. She had a left head turn with permanent neck pain. At the examination, best corrected visual acuity was 0.4 in both eyes with her glasses. Correction in the right eye was -6.50-1.50/180° and in the left eye -5.50-2.50/180°. Measured objective and subjective angles of squint were +4°, fusion from -3° to +29°, with the presence of stereo vision. During the cover test the nystagmus was present and it enhanced while covering the eye. Ocular motility was not limited. Fundus examination revealed myopic changes in both eyes. Prisms were prescribed, which were well tolerated by the patient. Also, no apparent head turn was noticed while wearing the prisms. Nine months later, the patient underwent a Kestenbaum procedure. Retroposition of the lateral rectus muscle with resection of the medial rectus muscle in the right eye and retroposition of the medial rectus muscle with resection of the lateral rectus muscle in the left eye were performed. After the procedure nystagmus dampened the most in the minimal left position, the head was in a straight position. Two years after the procedure nystagmus dampened the most in the primary position, the head was in a straight position. Nine years after surgery and refractive correction with contact lenses, the visual acuity was 0.8-0.9p in both eyes. The presented case showed that adequate functional and surgical treatment led to a good morphological outcome with improved visual acuity in a patient with congenital nystagmus and a compensatory head posture even in adulthood.