The usual manifestations of Moyamoya disease are bilateral carotid narrowing or occlusion at the carotid fork and abundant basal.vascular network with various carotid neurovascular symptoms. We report two unusual cases showing visual symptoms(bilateral cortical blindness, right homonymous hemianopsia, respectively) and angiographically vertebrobasilar and complete proximal internal carotid occlusion, with extensive parenchymal and transdural anastomosis in Moyamoya disease.
Blindness, Cortical ; Carotid Artery, Internal* ; Hemianopsia ; Moyamoya Disease*

The purpose of this study is to determine the prevalence and causes of visually impairment among children below 4 years old in two public tertiary referral hospitals in Selangor, Malaysia. In this cross-sectional retrospective study, the case files of all children aged below 4 years from the ophthalmology clinics in two separate public hospitals in Selangor, Malaysia were analysed over a 5-year period. The definition of visual impairment was based on the World Health Organization (WHO) classification, i.e. moderate visual impairment: visual acuity <6/18 to 6/60 in the better eye; severe visual impairment: <6/60 to 3/60 in the better eye; and blindness: visual acuity <3/60 in the better eye. Prevalence of visual impairment was based on the presenting visual acuity and then differentiated according to the cause of visual impairment diagnosed. A total of 243 (17.38%) children from a total of 1,398 children were classified as visually impaired. Most of these children (n=119, 49.0%) exhibited moderate visual impairment, 26 (10.7%) had severe visual impairment, while 98 (40.3%) were blind. Refractive errors (n=60, 24.7%) were the most common cause of vision impairment, mostly myopes (n=28, 46.7 %). This was followed by strabismus (n=31, 12.8%) and retinopathy of prematurity (n=29, 11.9%). In conclusion, vision impairment was prevalent in children aged below 4 years old presenting to two ophthalmology clinics in a highly urbanised region in Malaysia. High refractive errors were the major cause of vision impairment seen. Our study highlights the need and utmost importance of an early intervention programme to be implemented for these children since the main cause of vision impairment found in this study is an easily treated problem.
visual impairment ; prevalence ; children ; blindness

VEP is a gross electrical signal generated by the occipital region of the cortex in response to visual stimuli and it is useful in refraction, optic nerve disease, color blindness, amblyopia, field defect, macular disease, etc. We used Horizon computer with UTAS-E and normal VEP at different checksize was measured on 30 subjects(60 eyes) with visual acuity better than 1.0. The pattern reversal frequency was 2 Hz., analysis time was 250 msec., artifact reject threshold was 75 micro V., low-pass filter cut-off was 30Hz., high-pass filter cut-off was 1Hz. The check sizes used were 16 X 16(50min), 32 X 32(25min) and 64 X 64(12.5min). The results were as follows; 1. 16 X 16 (50min). amplitude: 8.18 +/- 3.55 micro V., latency: 106.78 +/- 4.28 msec. 2. 32 X 32(25min). amplitude: 8.48 +/- 5.99 micro V., latency: 106.68 +/- 3.82 msec. 3. 64 X 64(12.5min). amplitude: 7.79 +/- 3.68 micro V., latency: 109.73 +/- 5.15 msec. 4. The change of latency between 32 X 32 and 64 X 64 check-size was statistically significant(p<0.05). 5. The amplitude was largest in 32 X 32(25min.) check-size but statistically not significant(p>0.05). 6. The latency was most increased in 64 X 64(12.5min.) check-size and it was statistically significant(p<0.001).
Amblyopia ; Artifacts ; Color Vision Defects ; Optic Nerve Diseases ; Visual Acuity

PURPOSE: To report a case of posterior ischemic optic neuropathy accompanied by carotid artery plaque in a patient with retrobulbar optic neuritis. CASE SUMMARY: A 48-year-old man visited our clinic complaining of headache, decreasing visual acuity and defect of inferior visual field in his left eye for 3 days. The best corrected visual acuity was 1.0 in the right eye and 0.1 in the left eye. The anterior segment state, intraocular pressure, fundus examination and optical coherence tomography were normal in both eyes. Relative afferent pupillary defect, color vision deficiency and total scotoma were observed in his left eye. The results of the laboratory test and brain magnetic resonance imaging were normal. He was discharged from the hospital after 3 days of systemic steroid treatment on the basis of retrobulbar optic neuritis. A week later, fluorescent angiography and carotid ultrasonography were performed because of his history memory loss and left upper limb weakness before admission. A focal filling defect of the peripapillary area was found on fluorescent angiography. A plaque with a thickness of 1.9 mm and a length of 1.4 cm was found on carotid ultrasonography. After 6 months, the best corrected visual acuity was 0.4 in the left eye and the visual field showed a partially improved defect. CONCLUSIONS: Fluorescent angiography is recommended for potential posterior ischemic optic neuropathy in patients with retrobulbar optic neuritis, even though it is rare. Carotid ultrasonography is useful in finding atherosclerosis to prevent stroke or cardiovascular disease if ischemic cause is suspected on fluorescent angiography.
Angiography ; Atherosclerosis ; Brain ; Cardiovascular Diseases ; Carotid Arteries* ; Carotid Stenosis* ; Color Vision Defects ; Headache ; Humans ; Intraocular Pressure ; Magnetic Resonance Imaging ; Memory Disorders ; Middle Aged ; Optic Neuritis ; Optic Neuropathy, Ischemic* ; Pupil Disorders ; Scotoma ; Stroke ; Tomography, Optical Coherence ; Ultrasonography ; Upper Extremity ; Visual Acuity ; Visual Fields

PURPOSE: Joubert syndrome is a rare disorder which affects the cerebellum and the brain stem. Herein, we report a case of Joubert syndrome accompanied with retinal abnormality. CASE SUMMARY: A 9-year-old female visited our hospital with chief complaints of low vision in both eyes, nystagmus, and lack of gaze movement. The best-corrected visual acuity in her right eye was 20/80 and in the left 20/80 and heterotropia was not observed. She appeared to have incomplete total color blindness on the color vision test. The anterior segment test showed no abnormal findings other than diffuse pigmentation and degeneration of the peripheral retina, vascular attenuation, and pale optic disc in both eyes on fundus examination. The patient showed overall developmental delay and decreased muscle tension, but genetic and congenital metabolic disease tests were normal. The molar tooth sign of the midbrain, defect in the lower part of the cerebellum and dilatation of the fourth ventricle were observed on magnetic resonance imaging. CONCLUSIONS: Appropriate evaluation of retinitis pigmentosa and visual function should be performed in Joubert syndrome patients.
Brain Stem ; Cerebellum ; Child ; Color Vision ; Color Vision Defects ; Dilatation ; Female ; Fourth Ventricle ; Humans ; Magnetic Resonance Imaging ; Mesencephalon ; Metabolic Diseases ; Molar ; Muscle Tonus ; Pigmentation ; Retina ; Retinaldehyde* ; Retinitis Pigmentosa ; Tooth ; Vision, Low ; Visual Acuity

Objective: The primary goal of this study was to determine the effect of visual impairment (VI) on the quality of life (QoL) in children aged 3-7 years. Methodology: This was a cross-sectional study involving 138 parents or caregivers of children aged 3-7 years from Pediatric Ophthalmology and General Ophthalmology Clinics of a tertiary government hospital. The Filipino version of the Children’s Visual Function Questionnaire (CVFQ3plus), an instrument that measures the impact of visual impairment on the QoL of children aged 3-7 years and their families, was used. It has 6 subscales: general health, general vision, competence, personality, family impact, and treatment. T-test was used to compare the total index QoL score and subscale scores between children with VI (n=69) and the control group (n=69). The multivariate regression model based on the total index QoL score in children with VI was used to assess the effect of other demographic factors. Results: Children with VI had significantly lower total index QoL score (p=0.02), general vision (p=0.04), competence (p=0.00), and personality (p=0.02) subscale scores than the control group. The visual acuity of children with VI had a significant effect on the total index QoL score (p=0.04). Demographic factors like patient’s age and sex, and parental age, sex and educational attainment had no effect on total index QoL score. Conclusion Visual impairment has a negative effect on the quality of life of Filipino children aged 3-7 years as shown by the use of the CVFQ3plus.
Vision Disorders ; Quality of Life ; Vision, Low

A case or right occipital lobe infarction initially detected by the visual field examination and confirmed with the computerized tomography is presented. Reduction of significance of visual field test for identification of intracranial lesion as, the initial diagnostic procedure after the orientation of computerized tomography is unlikely. An occipital lobe lesion developed following posterior cerebral artery thrombosis has introd uced silgnificant ophthalmic symptom, namey, visual field defect, diplopia, ipsilateral ocular pain, visual hallucination, photophobia, and Anton's syndrom, etc. A characteristic feature of left homonymous hemianopsia with macular sparing due to right posterior cerebral artery thromb osis is demonstrated.
Diplopia ; Hallucinations ; Hemianopsia ; Infarction* ; Occipital Lobe* ; Photophobia ; Posterior Cerebral Artery ; Thrombosis ; Visual Field Tests ; Visual Fields

PURPOSE: To report a case of unilateral nasal hemianopsia caused by a large internal carotid artery aneurysm. CASE SUMMARY: A 56-year-old female presented with large cupping in the left optic nerve head detected incidentally during a routine check-up. She had no underlying systemic disease except hypertension. The best corrected visual acuity was 20/20 in both eyes and a slit-lamp examination showed no abnormal findings. Ophthalmoscopy showed cup/disc ratios of 0.6 in the right eye and 0.75 in the left eye. Relative afferent papillary defect or color vision defect was not observed. A Humphrey visual-field test indicated unilateral nasal hemianopsia in the left eye. Brain CT and angiography revealed a large 2.2-cm aneurysm on the left internal carotid artery. CONCLUSIONS: Internal carotid artery aneurysm should be considered as a possible cause of unilateral nasal hemianopsia in patients without intraocular lesion.
Aneurysm* ; Angiography ; Brain ; Carotid Artery, Internal* ; Color Vision Defects ; Female ; Hemianopsia* ; Humans ; Hypertension ; Middle Aged ; Ophthalmoscopy ; Optic Disk ; Visual Acuity

In present, clinically, the VEP has special value in the areas of refraction, infant acuity, diseases of the optic nerve, color blindness, amblyopia and field defects. VEP reflects the the activity of the visual system from the level of the photoreceptors to the occipital cortex, and it is more directly related to vision than retinoscopy. Authors studied the YEP change in refractive errors. We used Horizon computer with UTAS-E and the check sizes used were 16 X 16(50 min.), 32 X 32(25 min.), 64 X 64(12.5 min). One eye was occluded and then lenses of different power were successively placed before the other eye, and a seperate VEP was recorded for each lens power. The result were as follows: 1. Amplitude change according to change of check size in different trial lens powers. 1) +3, +4, +5D (64 X 64): statistically significant decrease. 2) -D(except -6D, 32 X 32): statistically not significant. 2. Latency change according to change of check size in different trial lens powers. 1) +D(64 X 64 and +4, +5D(32 X 32): statistically significant increase. 2) -D(64 X 64) and -10D(32 X 32): statistically significant increase. 3. Amplitude change according to change of trial lens power in different check sizes. 1) All check size(+4, +5D) and 64 X 64 size(+3D): statistically significant decrease. 2) All check size(-D): statistically significant decrease. 4. Latency change according to change of trial lens power in different check sizes. 1) 32 X 32 size(+4, +5D) and 64 X 64size(+3, +4, +5D): statistically significant increase. 2) 16 X 16 size(-6, -8, -10D) 32X32 size(-4, -6. -8, -10D) and 64X64 size (all D): statistically significant increase. 5. Change of amplitude and latency between each trial lens power in different check sizes. 1) +D(64 X 64), amplitude and Latency: statistically significant. 2) -D amplitude-all check size: statistically significant, latoncy-32 X 32 size: statistically significant.
Amblyopia ; Color Vision Defects ; Humans ; Infant ; Optic Nerve ; Refractive Errors* ; Retinoscopy

PURPOSE: To identify causes of conditions presenting with low vision without distinct abnormities in pediatric patients and to determine the appropriate diagnostic approach for those conditions. METHODS: We retrospectively reviewed medical records of pediatric patients with amblyopia, suspicious amblyopia or visual impairment of unknown origin referred by primary care providers. Patients were classified into 2 groups, amblyopia and visual impairment of unclear origin. In this study, we reviewed and analyzed the visual impairment of unclear origin. RESULTS: Of 152 patients, 94 patients were classified as amblyopia and 58 patients were classified as visual impairment of unclear origin. Among those with visual impairment of unclear origin, 26 patients (44.8%) were classified as functional visual loss, 23 patients (39.7%) as normal corrected visual acuity, 8 patients (13.8%) as organic disease and 1 (1.7%) patient could not be classified. Fundus examination revealed abnormal findings in all patients classified as organic disease. Six patients had optic atrophy and 2 had abnormalities on the macula. Ten patients had an orbital magnetic resonance imaging (MRI) scan. Only 1 of 10 MRI scans showed causative abnormality, however, the patient showed an optic atrophy on fundus examination before the MRI scan. CONCLUSIONS: Clinicians need to consider a high prevalence of functional visual loss and possibility of occult organic disorders when they evaluate pediatric patients presenting with decreased vision without distinct abnormities. MRI scan is recommended for only selected cases, when optic atrophy is not present.
Amblyopia ; Humans ; Magnetic Resonance Imaging ; Medical Records ; Optic Atrophy ; Orbit ; Prevalence ; Primary Health Care ; Retrospective Studies ; Vision Disorders ; Vision, Low ; Visual Acuity