Goldmann Visual Field Testing Research Articles

Leber’s Hereditary Optic Neuropathy Following Unilateral Syphilitic Optic Neuritis

This report describes a case of Leber’s hereditary optic neuropathy (LHON) following unilateral syphilitic optic neuritis. A 31-year-old man presented with a four-day history of decreased vision in his left eye. At the initial visit, the patient’s best corrected visual acuity (BCVA) was 1.2 and 0.02 in the right and left eye, respectively, along with a relative afferent pupillary defect in the left eye. Goldmann visual field testing revealed a central absolute scotoma and an enlarged blind spot in the left eye. Serological testing confirmed syphilis with rapid plasma reagin positivity. The patient was diagnosed with syphilitic optic neuritis and treated with intravenous penicillin G (24 million units daily) for two weeks. After two weeks of therapy, his left BCVA improved to 0.4 after two months. However, five months later, despite completing syphilis treatment, the patient developed acute painless visual loss in both eyes. His BCVA decreased to 0.8 and 0.05 in the right and left eyes, respectively. Goldmann visual field testing revealed a central absolute scotoma and an enlarged blind spot in both eyes. After two weeks, BCVA progressively declined to 0.4 in the right and 0.04 in the left eye. Furthermore, Goldmann visual field testing revealed an enlarged central absolute scotoma and blind spot in both eyes. Six months after the initial examination, his BCVA was counting fingers and 0.02 in the right and left eye, respectively. The optic discs appeared diffusely pale in both eyes. Genetic testing for LHON revealed a pathologic mtDNA 11778 point mutation. This case underscores the importance of considering the possibility of LHON if acute or subacute vision loss occurs subsequently or simultaneously in both eyes and remains unresponsive to treatment.

Visual Field Improvement in Non-Arteritic Posterior Ischemic Optic Neuropathy in a Patient Treated with Intravenous Steroids

A 50-year-old man presented with a nasal visual field defect in the left eye. His medical history was unremarkable. On ophthalmic examination, his best-corrected visual acuity (BCVA) was 1.2 in both eyes. Fundoscopy revealed no abnormalities in either eye. Goldmann visual field test showed a nasal visual field defect in the left eye. The patient was diagnosed with NPION of the left eye and was followed up without treatment. However, four days after the initial visit, his BCVA decreased to counting finger in the left eye. Goldmann visual field test showed a complete nasal visual field defect with central absolute scotoma. Following admission, the patient was treated for 3 days with intravenous methylprednisolone pulse therapy. Three months later, his BCVA was improved to 0.8 in the left eye, but central relative scotoma was remained. Six months later, his BCVA was maintained at 0.8, and central relative scotoma was disappeared. However, the optic disc looked diffusely pale in the left eye. This case highlights that steroid therapy is an option for severe cases with posterior ischemic optic neuropathy.

A Case of Nonarteritic Anterior Ischemic Optic Neuropathy that Recurred in the same Eye within a Month

A 78-year-old woman presented with an upper visual field defect in the left eye. On ophthalmic examination, her best-corrected visual acuity (BCVA) was 1.2 in both eyes. Fundoscopy revealed inferior optic disc swelling. Goldmann visual field test showed an upper visual field defect. The patient was diagnosed with nonarteritic anterior ischemic optic neuropathy (NAION) of the left eye and was followed up without treatment. One month after the initial visit, her BCVA decreased to 0.15 in the left eye. Fundoscopy revealed superior optic disc swelling. Goldmann visual field test showed an inferior visual field defect and central relative scotoma. Based on her clinical findings, she was diagnosed with recurrent NAION. Three months after the initial visit, her BCVA was 0.2 in the left eye, and the optic nerve developed atrophy. This case highlights the importance for clinicians to be aware of NAION recurred in the same eye within a short period of time.

Unilateral Syphilitic Optic Neuritis without Uveitis

This report describes a case of case of unilateral syphilitic optic neuritis without uveitis. A 56-year-old man presented with complaints of four days history of decreasing vision in his left eye. At the initial visit, the patient’s best corrected visual acuity was 1.2 and 0.03 in the right and left eye, respectively. A relative afferent pupillary defect was observed in the left eye. Slit-lamp examination revealed no cells, flare or keratic precipitates in the anterior segment either eye. Fundoscopy revealed diffuse optic disc swelling with peripapillary hemorrhage in the left eye. Goldmann visual field testing identified central absolute scotoma and an enlarged blind spot in the left eye. On serological evaluation for syphilis, serum rapid plasma reagin (RPR) and treponema pallidum hemagglutination assay (TPHA) were positive (RPR 52.5, TPHA 740.0). In addition, IgM fluorescent Treponemal antibody absorbance (FTA-ABS) was positive. In contrast, cerebrospinal fluid showed negative RPR and positive TPHA (TPHA 11.8). Cerebrospinal fluid (CSF) analysis showed lymphocytic pleocytosis and increased protein content and glucose. Magnetic resonance imaging showed a swollen enhanced lesion in the left optic nerve. The patient was diagnosed with syphilitic optic neuritis and received the currently recommended treatment with intravenous penicillin G (PCG). Twenty-four million units per day of PCG were administered for 2 weeks. After 2 weeks of therapy, his left visual acuity improved to 0.3 in 2 months. However, the optic disc looked diffusely pale in the left eye. At 6-month visit, the patient was clinically stable. RPR turned out negative and there was a reduction in serological TPHA reactivity. We consider that it is essential to perform serological tests for syphilis in every patient with optic neuritis, which makes it possible to initially treat syphilitic optic neuritis with PCG.

Mutational Profile and Retinal Phenotypes of PCARE-Related Cone-Rod Dystrophies in a Mexican Cohort.

The aim of the study is to describe the genotype and phenotype of a Mexican cohort with PCARE-related retinal disease. The study included 14 patients from 11 unrelated pedigrees with retinal dystrophies who were demonstrated to carry biallelic pathogenic variants in PCARE. Visual assessment methods included best corrected visual acuity, color fundus photography, Goldmann visual field test, kinetic perimetry, dark/light adapted chromatic perimetry, full-field electroretinography, autofluorescence imaging, and spectral domain-optical coherence tomography imaging. Genetic screening was performed either by gene panel sequencing or by exome sequencing. According to the results of multimodal imaging and functional tests, all 14 patients were diagnosed with cone-rod dystrophy. Six different PCARE pathogenic alleles were identified in our cohort, including three novel mutations: c.3048_3049del (p.Tyr1016∗), c.3314_3315del (p.Ser1105∗), and c.551A > G (p.His184Arg). Notably, alleles p.His184Arg, p.Arg613∗, and p.Arg984∗ were present in 18 of the 22 (82%) PCARE alleles from probands in our cohort. Our work expands the PCARE mutational profile by identifying three novel pathogenic variants causing retinal dystrophy. While phenotypic variations occurred among patients, a cone-rod dystrophy pattern was observed in all affected individuals.

Evaluation of Cup Disc Ratio and RNFL Thickness Based on Goldmann Visual Field Test

Introduction and Objective : To assess the relationship between the cup-disc ratio of the optic nerve head and peripapilarry RNFL thickness to the visual field loss in glaucoma patients. Methods : Visual field from Goldmann kinetic perimerty and Ocular Computed Tomography (OCT) records from Yap Eye Hospital, Yogyakarta are used to examine the figure of visual field loss in glaucoma patient. Result: Broad spectrum of glaucoma-related visual field defects were observed from 73 eyes. The most common visual field defects are arcuate defect (23.3%) and followed by general depression. Arcuate defects can already observable in some patients with cup-disk ratio of 0.5 (30%).Arcuate defect occurs in the average RNFL thickness of 69.90 ?m (46.93-118.77). It appears that the pinhole vision appeared on the average RNFL thickness of 44.23 ?m (25.33-63.13), and temporal RNFL thickness remnant occured at 48.64 ?m (46.22-51.06). RNFL thickness with normal visual field was on the thickness of 107.78 ?m (100.27-115.29). Conclusion: Visual field defect that may be observed in glaucoma with Goldmann kinetic perimetry are arcuate defect, and general visual field depression. RNFL thickness may be correlated longitudinally with the worsening of visual field defect.

Optic disc hemorrhage in a young female following mRNA coronavirus disease 2019 vaccination: a case report

Since the development of the coronavirus disease 2019 vaccine, there have been many reports of its adverse effects. While respiratory symptoms are common, many other symptoms in various organs have been reported. Herein, we report a case of optic disc and retinal hemorrhage that developed immediately after coronavirus disease 2019 vaccination. A healthy 18-year-old Japanese female noticed floater in the left eye 1day after the second vaccination for coronavirus disease 2019 (Pfizer Inc.). Her visual acuity was 20/20 in the left eye, and Goldmann visual field test showed a relative scotoma around blind spot and in the temporal lower quadrant. It was considered due to subretinal hemorrhage and optic disc swelling. Fundus examination revealed retinal and optic disc hemorrhage. Pupillary reflex was intact and central critical flicker was not impaired, indicating that optic nerve was not involved. There was no sign of inflammation, vascular abnormality, nor history of an intense Valsalva maneuver. The hemorrhage resolved spontaneously within 5months. This case expands the clinical presentation of coronavirus disease 2019 vaccination-associated ocular adverse events, and it should be kept in mind when patients with similar symptoms visit clinics. The case report will help clinicians avoid unnecessary and invasive examinations and treatment.

Detection of visual field defects using Eye Movement Pediatric Perimetry in children with intracranial lesions: feasibility and applicability

The study aimed at evaluating the feasibility of Eye Movement Pediatric Perimetry (EMPP) among children in detecting Visual Field Defects (VFDs) associated with Intracranial Lesions (IL). Healthy controls (n = 35) and patients diagnosed with IL (n = 19) underwent a comprehensive clinical evaluation followed by a Goldmann Visual Field (GVF) and a customised EMPP protocol. During EMPP, all the participants were encouraged to fixate on a central target and initiate Saccadic Eye Movement (SEM) responses towards randomly appearing peripheral stimuli. The SEM responses were recorded using an eye-tracking device and further inspected to calculate Performance Scores (PS), Saccadic Reaction Times (SRTs), and an EMPP Index (EMPI). The mean age (years) of the controls and cases were 7.3 (SD: 1.5) and 9.4 (SD: 2.4) respectively. Among the controls, the older children (≥7 years) showed statistically significantly faster SRTs (p = 0.008) compared to the younger group. The binocular EMPP measurements compared between the controls and the cases revealed no statistically significant differences in PS (p = 0.34) and SRT (p = 0.51). EMPP failed in 4 children because of data loss or unacceptably poor PS whereas GVF failed in 7 children due to unreliable subjective responses. Of the 16 reports, with regard to the central 30-degree VF, 63% of the outputs obtained from both methods were comparable. EMPP is a reliable method to estimate and characterise the central 30-degree VF in greater detail in children with IL. EMPP can supplement the conventional methods, especially in those children who fail to complete a long duration GVF test.

Clinical-genetic findings in a group of subjects with macular dystrophies due to mutations in rare inherited retinopathy genes.

To describe the results of clinical and molecular analyses in a group of patients suffering from inherited macular dystrophies, in which next-generation sequencing (NGS) efficiently detected rare causative mutations. A total of eight unrelated Mexican subjects with a clinical and multimodal imaging diagnosis of macular dystrophy were included. Visual assessment methods included best corrected visual acuity, color fundus photography, Goldmann visual field tests, kinetic perimetry, dark/light adapted chromatic perimetry, full-field electroretinography, autofluorescence imaging, and spectral domain-optical coherence tomography imaging. Genetic screening was performed by means of whole exome sequencing with subsequent Sanger sequencing validation of causal variants. All patients exhibited a predominantly macular or cone-dominant disease. Patients' ages ranged from 12 to 60years. Three cases had mutations in genes associated with autosomal dominant inheritance (UNC119 and PRPH2) while the remaining five cases had mutations in genes associated with autosomal recessive inheritance (CNGA3, POC1B, BEST1, CYP2U1, and PROM1). Of the total of 11 different pathogenic alleles identified, three were previously unreported disease-causing variants. Macular dystrophies can be caused by defects in genes that are not routinely analyzed or not included in NGS gene panels. In this group of patients, whole exome sequencing efficiently detected rare genetic causes of hereditary maculopathies, and our findings contribute to expanding the current knowledge of the clinical and mutational spectrum associated with these disorders.

Study of the Prevalence of Glaucoma in Kazakhstan.

Glaucoma is one of the leading causes of permanent visual disability around the world. However, the available literature lacks data on the prevalence of glaucoma in Central Asia, particularly in the Republic of Kazakhstan. The study was aimed at assessing the prevalence of glaucoma in the population of the Republic of Kazakhstan over 40 years old in 2019. A retrospective study was based on the analysis of the results of glaucoma screenings in 171 832 patients over 40 years old living in Kazakhstan (in 14 counties). Glaucoma cases were confirmed by Goldmann tonometry, fundus photography, and visual field testing. Demographic indicators, territorial differences, and hereditary predisposition were studied and analysed. In addition, blood pressure was measured. Of 171 832 patients examined, 452 with verified glaucoma were identified. The average age of the patients was 63.9 ± 9.4. In rural areas, the prevalence of glaucoma was higher compared to the urban population. The overall prevalence of glaucoma among people over 40 years old was 2.37 ± 0.17. The prevalence of glaucoma among women was higher than for men, with an indicator of 1.91 (95% CI relative risk 1.78 - 2.03) (p < 0.05). The highest prevalence was found in the 71 - 75 age group [equals to 14.2% (95% CI 11.7 - 19.9)], with a statistically significant difference (p < 0.05). The highest prevalence of glaucoma was observed in the group of people with a hereditary predisposition, with an indicator of 14.7% (95% CI 0.6 - 1.9) (p < 0.05). Among all patients with concomitant arterial hypertension (n = 90, 19.9%), women (60%) compared with men (40%) had a 2.4% higher risk of glaucoma morbidity (95% CI 1.2% - 3.8%). This study provides updated information on the prevalence of glaucoma in Kazakhstan. The results obtained confirm that the increase in the prevalence of glaucoma in Kazakhstan is directly proportional to the increase in the patients' age. These results showed the importance of screening for a timely diagnosis, especially for patients with high risk factors such as hereditary predisposition. Moreover, the results indicate that the early detection of systemic hypertension and increased intraocular pressure can be used for the prevention of undesirable outcomes such as an irreversible blindness.

Correlation between Intraocular Pressure and Visual Field Loss in Primary Open Angle and Primary Angle Closure Glaucoma: A Cross-Sectional Study

Introduction: Glaucoma is characterised by structural damage to optic nerve head with corresponding visual field defects and often associated with increased Intraocular Pressure (IOP). It may be broadly classified as Primary Angle Closure Glaucoma (PACG) and Primary Open Angle Glaucoma (POAG). It is one of the leading causes of global blindness, and a major proportion occurs in Indian population. Aim: To study the correlation between pretreatment IOP and extent of visual field loss in PACG and POAG. Materials and Methods: A cross-sectional observational study was carried out in Regional Institute of Ophthalmology, Trivandrum, Kerala, India from April 2016 to May 2017. Newly diagnosed cases of PACG (25 patients-13 males, 12 females, mean age 58.72±10.07 years) and POAG (85 patients- 45 males, 40 females, mean age 60.28±10.42 years) underwent a detailed glaucoma evaluation which included IOP measurement with Goldmann applanation tonometer and visual field testing using Humphrey Field Analysis (HFA) 24-2 pattern. Mean Deviation (MD), Pattern Standard Deviation (PSD) and Advanced Glaucoma Intervention Score (AGIS) score was calculated from reliable visual field test result. All data were coded and entered in to statistical software, Statistical Package for Social Sciences (SPSS) version 16.0 for analysis. The correlation between pretreatment IOP and visual field loss in patients with PACG and POAG was determined by Pearson Correlation of Coefficient. Results: Amongst the total 110 patients of this study, 25 patients were of PACG while POAG were in 85 patients. A significant correlation between pre treatment IOP and the extent of visual field loss in PACG was noted. There was no significant correlation in POAG. Linear regression analysis demonstrated a significant positive correlation between IOP and AGIS score in PACG (Pearson correlation coefficient(r)=0.805, p&lt;0.001), not in POAG (r=0.026, p=0.816). Correlation between IOP and MD is statistically significant in PACG (r=0.812, p&lt;0.001) but not in POAG (r=0.058, p=0.597). The correlation between IOP and PSD is not statistically significant in both groups (p-value &gt;0.450). Conclusion: A significant correlation between IOP and visual field loss in PACG indicates that extent of visual field damage can be controlled by controlling IOP alone in PACG. The correlation between the pretreatment IOP and visual field loss in POAG is not statistically significant which agrees with the current proposed pathophysiology of optic neuropathy in which multiple factors influence in addition to IOP.

Visual Field Impairment and Driver Fitness: A 1-Year Review of Crashes and Traffic Violations.

Occupational therapists frequently assess the fitness to drive of people with visual field impairment, but the relationship between these assessments and driving performance over time is not well understood. To determine traffic violation and crash incidence over a 1-yr period for drivers with visual field impairment. Retrospective review of medical and driving records. British Columbia, Canada. Participants (N = 445) were ages 26-74 yr with binocular, corrected visual acuity of ≥20/50. Goldmann visual field tests were reviewed to stratify participants on the basis of visual field impairment and whether impairments exceeded licensing standards. Traffic records were reviewed to determine group-specific traffic violations and crash incidence during the year after the visual field test. Of 445 participants, 292 held a valid driver's license during the designated period. Participants not meeting the licensing standards were less likely to become licensed than participants who met the standards. The results indicate that drivers with visual field impairment did not have a higher probability of crashes or violations than drivers without visual field impairment. Drivers with visual field impairment who retained a driver's license did not have an increased probability of crashes in the following year. A larger, prospective, mixed methods study of long-term driving behaviors among people with visual field impairment is recommended. This study provides preliminary data on the 1-yr incidence of traffic violations and crashes among people with visual field impairment.

Investigation of goldmann perimetry in evaluation of patients for upper eyelid blepharoplasty

ABSTRACTPurpose: To determine if preoperative Goldmann Visual Field (GVF) testing in patients with functional dermatochalasis accurately depicts the postoperative superior visual field (SVF) outcome.Methods: A prospective cohort study was done to compare preoperative and postoperative GVF field tests in patients undergoing upper eyelid blepharoplasty for treatment of dermatochalasis. This study was conducted in accordance with the Declaration of Helsinki and approved by the University of Arkansas for Medical Sciences institutional review board. A preoperative GVF was obtained with the eyelids in the natural position (untaped) and then again with excess skin elevated (taped). One month post-blepharoplasty, another GVF was conducted with eyelids untaped. The pre- and post GVF tests were analyzed to determine if preoperative testing accurately predicts the SVF improvement post-blepharoplasty.Results: Forty-six eyelids (23 patients) who underwent blepharoplasty for dermatochalasis were included. The preoperative testing underestimated 76% (35/46) of cases by a mean of 61%; and overestimated the final outcome in 24% (11/46) of cases by mean of 23%. Overall, the preoperative GVF testing underestimated the postoperative outcome by a mean of 35%.Conclusion: Improvement of the SVF after a blepharoplasty is typically greater than the preoperative GVF testing predicts.

Bilateral idiopathic retinal vasculitis following coxsackievirus A4 infection: a case report

BackgroundCoxsackieviruses are members of a group of viruses called the enteroviruses, which may cause respiratory and gastrointestinal symptoms, erythema, meningoencephalitis, myocarditis, pericarditis, and myositis. Unilateral acute idiopathic maculopathy caused by coxsackievirus A16 has been associated with hand, foot, and mouth disease, but only a few reports describe retinitis associated with coxsackievirus serotype B3 or B4. We report a case of bilateral multifocal obstructive retinal vasculitis that developed after coxsackievirus A4 infection.Case presentationA 60-year-old woman was referred to our department with bilateral visual disturbance that developed following flu-like symptoms. At the initial examination, best corrected visual acuity was 20/200 in the right eye and 20/50 in the left eye. The critical flicker frequency (CFF) was 23 Hz in the right eye and 27 Hz in the left eye. Fine white keratic precipitates with infiltrating cells were presented in the anterior chamber of both eyes, and multifocal retinal ischemic lesions were observed in the macula and posterior pole of both eyes. The retinal lesions corresponded with scotomas observed in Goldmann visual field test. On spectral domain-optical coherence tomography (SD-OCT), retinal lesions were depicted as hyper-reflective regions in the inner retina layers in both eyes, and disruption of ellipsoid line in the left eye., Fluorescein angiography exhibited findings indicative of multifocal obstructive retinal vasculitis. The patient had a history of current hypertension treated with oral therapy and glaucoma treated with latanoprost eye drops. Blood test for coxsackievirus antibody titers revealed that A4, A6, A9, B1, B2, B3, and B5 were positive (titers: 8–32). Abdominal skin biopsy of necrotic tissue suggested vascular damage caused by coxsackievirus. The general symptoms improved after 6 weeks, and the multifocal retinal ischemic lesions were partially resolved with residual slightly hard exudates. Only coxsackievirus A4 antibody titer increased from 4 to 32-fold after 14 months. However, hyper-reflective regions and disruption of the inner retinal layers on SD-OCT persisted especially in the right eye, and residual paracentral scotoma was observed in the right eye.ConclusionThe present case suggests that coxsackievirus A4 causes bilateral multifocal obstructive retinal vasculitis with irreversible inner retinal damage.

Tangent screen perimetry in the evaluation of visual field defects associated with ptosis and dermatochalasis.

PurposeTo determine if tangent visual fields gathered during assessment of superior visual field deficits caused by blepharoptosis and dermatochalasis offer good correlation to clinical exam in a time and cost efficient manner.MethodsProspective, observational case series. Subjects included all patients referred to a single surgeon (CCN) who underwent surgical correction of blepharoptosis and/or dermatochalasis. Preoperatively and postoperatively, upper margin-to-reflex distances were assessed. Tangent visual fields were performed in a timed fashion and analyzed for degrees of intact vision in the vertical meridian and degrees squared of area under the curve. Data were compared by Student t-tests and Pearson correlation coefficients.ResultsMean preoperative superior visual fields with the eyelid in the natural position measured 8° in the vertical meridian. Measurements in the vertical meridian and area under the curve showed excellent correlation (r = 0.87). Patients with ptosis showed strong correlation between margin-to-reflex distance and superior visual fields. Patients completed field testing faster than reported times for automated or Goldmann testing. Finally, tangent screens were the least expensive type of equipment to purchase.ConclusionsTangent visual fields are a rapid and inexpensive way to test for functional loss of superior visual field in patients with upper eyelid malposition. Our data revealed potential differences between tangent screen results and published results for automated or Goldmann visual field testing which warrants further studies.

THE PHYSIOLOGY OF THE RETINAL PIGMENT EPITHELIUM IN DANON DISEASE.

Danon disease is caused by mutations in the lysosome-associated membrane protein-2 gene (LAMP2). In the eye, LAMP2 is expressed only in the retinal pigment epithelium. This study aimed to investigate the previously unreported impact of LAMP2 mutations on the electrooculogram generated by the retinal pigment epithelium. Four members of a family with Danon disease were examined. All have mutations in c294G > A, of the LAMP2 gene on Xq24, by which no, or aberrant, protein will be formed. Electrooculograms to International Society for the Clinical Electrophysiology of Vision (ISCEV) standards were recorded with full-field electroretinography, Goldmann kinetic visual fields, and spectral optical coherence tomography with fundus autofluorescence imaging. Electrooculogram amplitude ratios of light rise:dark trough, the Arden index, fell at low-normal limits (range: 1.68-3.94) but misrepresent retinal pigment epithelium health, because the absolute dark trough voltages were abnormally low (median: 140 μV, range: 72-192 μV) as were the light rise amplitudes (median: 297 μV, range: 198-366 μV), and full-field electroretinograms were normal. Hyperfundus autofluorescence and hypofundus autofluorescence changes became more confluent and florid with increasing age of female patients. Goldmann visual field testing showed constriction of the central field. Low electrooculogram voltages indicate that the retinal pigment epithelium is unable to maintain its tight junctions in Danon disease.

Saccadic vector optokinetic perimetry in children with neurodisability or isolated visual pathway lesions: observational cohort study.

Children and adults with neurological impairments are often not able to access conventional perimetry; however, information about the visual field is valuable. A new technology, saccadic vector optokinetic perimetry (SVOP), may have improved accessibility, but its accuracy has not been evaluated. We aimed to explore accessibility, testability and accuracy of SVOP in children with neurodisability or isolated visual pathway deficits. Cohort study; recruitment October 2013-May 2014, at children's eye clinics at a tertiary referral centre and a regional Child Development Centre; full orthoptic assessment, SVOP (central 30° of the visual field) and confrontation visual fields (CVF). Group 1: age 1-16 years, neurodisability (n=16), group 2: age 10-16 years, confirmed or suspected visual field defect (n=21); group 2 also completed Goldmann visual field testing (GVFT). Group 1: testability with a full 40-point test protocol is 12.5%; with reduced test protocols, testability is 100%, but plots may be clinically meaningless. Children (44%) and parents/carers (62.5%) find the test easy. SVOP and CVF agree in 50%. Group 2: testability is 62% for the 40-point protocol, and 90.5% for reduced protocols. Corneal changes in childhood glaucoma interfere with SVOP testing. All children and parents/carers find SVOP easy. Overall agreement with GVFT is 64.7%. While SVOP is highly accessible to children, many cannot complete a full 40-point test. Agreement with current standard tests is moderate to poor. Abnormal saccades cause an apparent non-specific visual field defect. In children with glaucoma or nystagmus SVOP calibration often fails.

MULTIMODAL IMAGING OF A FAMILY WITH SPINOCEREBELLAR ATAXIA TYPE 7 DEMONSTRATING PHENOTYPIC VARIATION AND PROGRESSION OF RETINAL DEGENERATION

To report the variability and progression of clinical presentation in three family members with spinocerebellar ataxia Type 7 including early recognizable features on retinal imaging and magnetic resonance imaging. Retrospective case series. The proband, Patient 1 (mother) presented at age 26 with light perception vision. Initial examination was significant for optic disc pallor, vascular attenuation, and central macular atrophy. Two years later, her vision declined to no light perception, and fundus examination demonstrated marked progression of macular atrophy and peripheral bone spicule formation. Seven years after the onset of vision loss, neurologic examination demonstrated ataxia, dysarthria, and slowed saccades. Genetic testing of ATXN7 identified heterozygous 61-CAG trinucleotide repeat expansion confirming the diagnosis of spinocerebellar ataxia Type 7. Patient 2 (son) presented at age 11 with visual acuity of 20/300 bilaterally and decreased color vision. Funduscopic examination was notable for disc pallor, vascular attenuation, and peripheral pigmentary changes. Electroretinography demonstrated diminished rod and cone function, and Goldmann visual field testing revealed paracentral scotoma. Patient 3 (daughter) presented at age 14 with visual acuity of 20/50 bilaterally and minimal funduscopic changes. The only significant ophthalmic finding was retinal thinning with atrophy of the outer nuclear layer and subfoveal ellipsoid zone on optical coherence tomography. Early cerebellar volume loss was also noted on magnetic resonance imaging. The clinical presentation of spinocerebellar ataxia Type 7 can vary widely even within the same family. In individuals with vision loss and normal fundus examination, careful evaluation of optical coherence tomography and brain magnetic resonance imaging facilitates early diagnosis and genetic testing.

Surgical Indications of Exploring Optic Canal and Visual Prognostic Factors in Neurosurgical Treatment of Tuberculum Sellae Meningiomas.

Background:Tuberculum sellae meningiomas (TSMs) present a special symptom because of the adherence and compression to the optic nerve, optic artery, and the chiasm. A significant number of patients with TSMs appear visual deficits. This study aimed to investigate the surgical indications of exploring the optic canal and visual prognostic factors in the neurosurgical treatment of TSMs.Methods:Totally 21 patients with TSM, who were operated from September 2007 to August 2011 in the Department of Neurosurgery, Tongren Hospital were enrolled in this study. Results of orbital computed tomography (CT) and magnetic resonance imaging (MRI), visual acuity, Goldmann visual field test, orbital color Doppler flow imaging (CDI) test in these patients were retrospectively analyzed.Results:Visual deficit and optic canal involvement (OCI) were detected in all the 21 patients. Fourteen patients had bone proliferation within the area of the optic canal. After the operation, visual outcomes were improved in 13 patients, unchanged in 7 patients, and deteriorated in 1 patient. All the 21 patients performed orbital CDI test preoperatively, the results showed that if the peak systolic velocity (PSV) of central retinal artery (CRA) value was ≤8 cm/s, the visual outcome would be better.Conclusions:The surgical indications of exploring optic canal in TSM cases included: (1) The neuroimaging evidences of OCI (CT and/or MRI); (2) PSV of CRA in orbital CDI test was ≤8 cm/s; (3) visual acuity was below 0.1; (4) visual field deficit. The PSV of CRA in CDI test could be a prognostic factor for visual outcomes of TSMs.

Toxocara Optic Neuropathy after Ingestion of Raw Meat Products

To report Toxocara optic neuropathy in five men with histories of ingestion of raw meat, liver, or blood. Five men in their 30s to 60s presented with unilateral visual disturbance. All five had a history of ingesting raw meat, liver, or blood products. All five men presented with optic disc edema and three also showed granulomatous infiltration of the retina. With Goldmann or Humphrey visual field testing, two patients showed central scotomas and two exhibited arcuate defects. Serum eosinophil count and total IgE were elevated in four patients. Serum enzyme-linked immunosorbent assay testing was positive for Toxocara canis IgG in all five cases. This report shows the variable clinical presentations of Toxocara optic neuropathy and the importance of questioning for a history of eating raw meat and blood products as a risk factor.