Systemic lupus erythematosus‐associated optic neuritis: clinical experience and literature review

Purpose: This study aimed to evaluate the visual outcome of optic neuritis in patients with systemic lupus erythematosus (SLE). Methods: A retrospective, case‐observational study was conducted by reviewing eight patients with SLE‐associated optic neuritis from January 1986 to October 2004. The demographic data, clinical manifestations, laboratory data, medical management and disease courses of these eight patients were retrospectively analysed. Main outcome measurements included final visual acuity (VA) and relapse of optic neuritis. Statistical analyses were made using the chi‐square test and a linear regression model. The English‐language literature on SLE‐associated optic neuritis was reviewed. Results: Initial visual loss was severe in SLE‐associated optic neuritis. Seven patients (87%) had VA < 20/200 at onset. All patients received steroid pulse therapy followed by oral steroid tapering. Final visual outcome was highly variable, ranging from the complete recovery of VA in four patients, to partial recovery in one and poor recovery in three. Better visual recovery occurred in patients who received earlier treatment (within 10 days). However, longer duration of steroid administration was found to have no significant benefit on visual outcome. Conclusions: Systemic lupus erythematosus‐associated optic neuritis is not common. However, it is important that ophthalmologists differentiate SLE‐associated optic neuritis from idiopathic optic neuritis because of the severe visual impairment and steroid dependence associated with the former. Early diagnosis and prompt treatment are important for restoring visual function in these patients.     

Pseudophakic bullous keratopathy

We reviewed the records of all patients with pseudophakic bullous keratopathy (271 eyes, 251 patients) seen during a six-month period to determine predisposing factors, associated problems, current management, and visual outcome. Pseudophakic bullous keratopathy was associated most frequently with anterior chamber intraocular lenses in general (155 of 271), and with Leiske style lenses in particular (100 of 271). It was associated with a visual acuity of 20/200 or less in 206 eyes and a visual acuity of counting fingers or less in 129 of the eyes at the initial examination. Penetrating keratoplasties had been performed in 189 of the eyes. After penetrating keratoplasty, 108 of 189 of the eyes had a visual acuity of 20/200 or less (mean follow-up, 15 months). Visual acuity improved with longer follow-up, and among patients with a minimum follow-up of two years, 23 of 36 eyes had a visual acuity of 20/100 or better. Most grafts were clear (145 of 189). Pseudophakic bullous keratopathy was associated with marked visual loss, which was permanent despite clear grafts in 29 of 92 eyes followed-up for one year or longer.     

Converting visual acuity to utilities

BACKGROUND: Utility theory can be used to quantify dysfunction associated with various diseases and thus can represent a "hard" measure of quality of life. By determining utility values, one can compare the quality of life of patients with ocular disease to that of patients with non-ophthalmic problems. We performed a study to determine whether utility values from patients with ocular disease are associated with clinical variables, including visual acuity in the better-seeing eye, and to develop a mathematical method for converting visual acuity to utility value, if there is an association between the two. METHODS: Cross-sectional study. A total of 239 patients from a tertiary care retinal practice with various ocular conditions, including macular degeneration, cataract, glaucoma and diabetic retinopathy, were interviewed under standardized conditions to determine their utility values by the time trade-off technique. Visual acuity, duration of visual loss and number of concomitant conditions were also determined. Multiple linear regression was performed to determine which variables were associated with utility values. RESULTS: The mean acuity in the better-seeing eye was 0.479 (near 20/40 vision). The mean utility value was 0.72. Accordingly, the average patient in our series was willing to trade 2.8 of every 10 remaining years of life to obtain perfect vision in both eyes. Utility value was significantly associated with visual acuity in the better-seeing eye (F = 69.1, p < 0.001). Other variables were not significantly associated with utility value. The association with duration of visual loss approached statistical significance (p = 0.075). Utility values (U) for patients with ocular disease can be derived from the following formula: U = (0.374)(visual acuity in better-seeing eye) + 0.514. INTERPRETATION: Utility values from patients with ocular disease were strongly associated with visual acuity and could be estimated mathematically.     

Asymmetry of the optokinetic nystagmus in lesion of the central nervous system

BACKGROUND: Unilateral lesions of the central nervous system (CNS) may be associated with a reduction of the optokinetic nystagmus (OKN) slow component in the direction of the lesion. The aim of our study was to assess the role played in these cases by the direct injury of the OKN pathways and/or by a possible associated visual field defect. METHODS AND PATIENTS: Monocular OKN was elicited with black and white stripes moving temporally-to-nasally (TN) or nasally-to-temporally (NT) at velocities of 15, 30, 45 and 60 degrees /s. Patients with cortical or chiasmal lesions associated with visual field defects were investigated. OKN was considered asymmetrical if the gain difference between TN and NT stimulation was not within 2 standard deviations of an age-matched control group (n = 86). RESULTS: We examined 12 patients with cortical lesions and 4 patients with chiasmal lesions. Asymmetric OKN gain was measured in 7 patients with cortical lesions associated with a visual field defect, and in 2 patients with chiasmal compression and bitemporal hemianopia. In 2 patients with isolated occipital lesions, OKN asymmetry was explained by the associated visual field defect. CONCLUSION: The interpretation of OKN asymmetry in patients with CNS lesions should not only consider a direct lesion of the OKN pathways but also a sensory deficit due to a visual field defect.     

Cortical visual loss in posterior reversible encephalopathy syndrome in late postpartum eclampsia: Case series

The purpose of this study was to determine the prevalence of visual disturbances in patients with posterior reversible encephalopathy syndrome (PRES) associated with late postpartum eclampsia. We retrospectively reviewed the clinical records of late postpartum eclampsia patients with features of PRES for the presence of visual disturbances and location of radiological abnormalities. We found a higher prevalence of cortical visual loss in patients with PRES associated with late postpartum eclampsia. Bilateral symmetrical vasogenic edema of the parieto-occipital lobe was the most common magnetic resonance imaging (MRI) abnormality noted. No significant differences were observed in the extent of edema in patients with and without visual loss.     

Cortical visual impairment in children with infantile spasms.

BACKGROUND: Infantile spasms are often associated with cortical visual impairment. METHOD: We report a series of 10 patients with infantile spasms, hypsarrhythmic electroencephalograms, and developmental delay, (ie, West syndrome), who presented with severe visual inattention despite a normal ocular examination. RESULTS: At follow-up (14 months to 6 years), 5 patients (50%) had no improvement in their visual behaviour. Although some degree of improvement was observed in the others, their visual function remained abnormal. All patients had moderate or severe mental retardation. CONCLUSIONS: The clinical features of West syndrome are reviewed, and the cause of the associated cortical visual impairment is discussed. Cortical visual impairment with infantile spasms is an important association, which pediatric ophthalmologists should recognize.     

Optic neuritis associated with multiple sclerosis in Koreans

There have been few reports on the visual prognosis of optic neuritis (ON) associated with multiple sclerosis (MS) in Asians, although previous reports have suggested a racial effect. Therefore, in this study we focused on the assessment of visual prognosis of ON associated with MS in Koreans. Sixteen Korean patients who were diagnosed with ON associated with MS between 1992 and 1998 were enrolled in this study. Two groups were identified: clinically diagnosed MS (CMS) and laboratory-supported MS (LSMS). Of the 16 patients, nine were CMS and seven were LSMS. All patients experienced recurrent ON; the patients with CMS tended towards more recurrent ON than those with LSMS (4.1:2.8). Visual acuity at the final follow-up was 20/40 or better in 33% of CMS and 70% of LSMS. Final visual acuity in Korean patients with ON associated with MS was worse than the result in other studies to date. One risk factor influencing visual outcome in ON associated with MS may be the recurrence of ON. These results have important clinical implications for the assessment of ON associated with MS in Asians.     

Visual perception during phacoemulsification cataract surgery under subtenons anaesthesia

AIM: The aim of this study was to record the subjective visual experience of patients during phacoemulsification and intraocular lens implantation under subtenons anaesthesia. METHODS: Prospective, nonrandomised, cohort, postoperative questionnaire based study. Patients selected underwent routine phacoemulsification and intraocular lens implantation under regional subtenons anaesthesia. chi(2) and Fisher's exact tests (two-tail) were used to evaluate the data. RESULTS: A total of 104 patients were selected, 38 (36.5%) were male and 66 (63.5%) were female. The mean age of patients was 74.0+/-8.8 years. In all, 87.5% saw light during the operation with 9.6% finding this painful. Photophobia was highest among younger patients (P=0.002). Coloured lights were seen by 56.7% and 13.5% found the visual experience frightening. Frightening visual experiences were significantly associated with the perception of colour (P=0.005) and photophobia (P=0.003). A volume of anaesthetic greater than 4 m was associated with a significant reduction in anxiety as a result of the visual experience (P=0.003). None of the other visual phenomena recorded were associated with a frightening visual experience. CONCLUSIONS: Patients undergoing regional anaesthesia experience a wide variety of visual sensations during cataract surgery. Perception of colour and volumes of anaesthetic less than 4 m appear to be associated with the visual experience being more frightening. Detailed preoperative counselling is mandatory. It should include comprehensive information about visual perception during the procedure relieving the patients from unnecessary distress.     

Anterior ischemic optic neuropathy associated with Vogt–Koyanagi–Harada disease

Background

Optic disc swelling is a common finding associated with Vogt–Koyanagi–Harada disease (VKH); however, visual field loss from optic disc involvement is uncommon. This reports report presents recent findings regarding unusual patients with visual field defects from optic disc involvement, thus suggesting the presence of anterior ischemic optic neuropathy (AION) in the acute phase of VKH.

Methods

Observational case series. A consecutive series of 52 patients with VKH (6 complete VKH, 46 incomplete VKH) was reviewed. Fifteen patients in this series had optic disc swelling, and six of them developed irreversible visual field defects in the acute phase of VKH. The clinical features of these six patients were analyzed.

Results

The patients with associated visual field loss were four males and two females between 54 to 79 years of age. They had bilateral panuveitis associated with meningismus. All of the patients had bilateral optic disc swelling and fluorescein angiography showed both a filling delay and late leakage of the optic disc. Visual field examinations revealed various degrees of visual field defects in 11 eyes. They were treated with high-dose corticosteroid therapy, and several weeks later, both the uveal inflammation and optic disc swelling disappeared. The visual fields showed some improvement as the retinal detachments and disc swelling resolved, but the visual field defects remained in ten eyes. The small and localized visual field defects were asymptomatic. Subsequently, optic disc pallor developed in nine eyes and retinal nerve fiber layer thickness, measured by optical coherence tomography, was decreased in six eyes. The fundus gradually showed various degrees of hypopigmentation, but did not show any chorioretinal atrophy causing visual field loss. Four patients had risk factors for AION, including diabetes mellitus or a relatively small optic nerve head.

Conclusions

In this consecutive series of patients with VKH, 15 out of 52 patients were found to have disc swelling, and six patients, who were mostly elderly, had associated visual field loss, which is probably due to AION associated with VKH.     

MACULAR OEDEMA AND RETINAL BRANCH VEIN OCCLUSION

The visual prognosis of patients suffering from retinal branch vein occlusion complicated by macular oedema is assessed in relation to the degress of capillary fallout. Extensive retinal capillary fallout associated with macular oedema was found to confer a poor visual prognosis in a series of 89 patients followed for an average time of 14 months. If the degree of capillary fallout associated with marular oedema was minimal, the visual prognosis was considerably improved.     

Surgical removal of massive subretinal hemorrhage associated with age-related macular degeneration

The authors report on 11 patients with age-related macular degeneration associated with massive subretinal hemorrhage, who were treated with surgical removal of the hemorrhage and associated fibrosis. Preoperative visual acuity ranged from 20/400 to hand motions. Postoperative visual acuity ranged from 20/200 to light perception with a minimum of 3 months of follow-up. Complications included partial or total retinal detachment in four patients (36%) and cataract in four patients (36%). Four of 11 patients showed some improvement in vision (36%). All four of these patients had surgery within 1 week of the onset of severe visual loss. Although surgical removal of large subretinal hemorrhages is technically feasible, visual recovery is limited, even in uncomplicated cases, by macular degenerative changes.     

Traumatic wound dehiscence after cataract extraction

PURPOSE: To study the visual outcome and factors associated with it in patients with traumatic dehiscence of a cataract surgery wound. SETTING: Department of Ophthalmology and the Brown School of Public Health and Community Medicine, The Hebrew University-Hadassah Medical School, Jerusalem, Israel. METHODS: A retrospective observational study of 37 eyes of 37 consecutive patients with traumatic wound dehiscence of a cataract surgery wound was conducted. A complete ophthalmic evaluation was performed in all patients. Statistical analysis was done to identify factors associated with the best corrected visual acuity at the end of follow-up. RESULTS: Patients had extracapsular cataract extraction (n = 29), intracapsular cataract extraction (n = 4), or lensectomy (n = 4). No patient had phacoemulsification via a small incision. A univariate analysis showed that factors associated with a worse visual outcome included the presence at presentation of hyphema (P = .05), intraocular lens dislocation or loss (P = .006), vitreous hemorrhage (P = .0002), scleral rupture (P = .001), a long interval from surgery to trauma (P < .0001), and fall as the cause of trauma (P < .0001). In a multivariate model, only a surgery-to-trauma interval longer than 8 weeks was associated with a worse visual outcome (P < .0001). Visual acuity immediately after trauma was a poor predictor of final visual acuity. CONCLUSIONS: Visual outcome after traumatic wound dehiscence of a cataract surgery wound was strongly associated with the interval from surgery to trauma. Other factors were less reliable predictors of visual outcome. Traumatic wound dehiscence only moderately affected visual outcome after cataract surgery in most cases.     

Clinical spectrum of posterior ischemic optic neuropathy.

PURPOSE: To describe the systemic and visual characteristics and prognosis in patients with posterior ischemic optic neuropathy (PION). DESIGN: Observational case series. METHODS: Retrospective chart review in a multicenter setting. Seventy-two patients (98 eyes) with a clinical diagnosis of PION. Co-morbid systemic diseases and visual function were recorded at both initial presentation and after mean visual follow-up of 4.1 years and systemic follow-up of 5.4 years. RESULTS: PION occurred in three main settings: in the perioperative period following a variety of surgical procedures (28 patients), associated with giant cell (temporal) arteritis (6 patients), and associated with nonarteritic systemic vascular disease (38 patients). Patients with perioperative and arteritic PION were more likely to have severe, bilateral visual loss that did not improve. Among eyes with nonarteritic PION, 34% experienced improvement in vision, 28% remained stable, and 38% worsened. Among patients with nonarteritic PION, carotid artery disease and a history of stroke (with or without carotid artery disease) were both associated with a statistically significant increased risk of poor final visual outcome. CONCLUSIONS: There are three distinct subtypes of PION: perioperative, arteritic, and nonarteritic. Patients with PION that is unassociated with surgery should undergo an evaluation for systemic vascular diseases, including giant cell arteritis, that may or may not be apparent at the time of vision loss. The visual prognosis for patients with perioperative or arteritic PION is poor, whereas that for nonarteritic PION is similar to that for patients with nonarteritic AION.     

Short-wavelength automated perimetry

Short-wavelength automated perimetry (SWAP) is a visual field test designed to assess the short-wavelength sensitive color system by isolating the blue-yellow pathway. SWAP is a powerful clinical tool able to detect visual field deficits 3 to 5 years before standard automated perimetry (white-on-white) in most glaucoma patients, and progression of visual field defects up to 3 years earlier. SWAP deficits are predictive of the onset and location of future visual field loss, and they correlate well with structural damage associated with glaucoma. The main disadvantage of SWAP remains the longer testing time required. In the clinic, it is recommended that SWAP be performed on patients who are at higher risk for glaucoma. Although SWAP was originally developed to detect visual loss in glaucoma patients, it is also useful for patients with diabetic retinopathy and maculopathy, optic neuropathies, vision loss associated with HIV, migraine, and multiple sclerosis. More sensitive psychophysical tests of visual function, such as SWAP, can significantly shorten clinical trials and aid in the validation of new therapeutic approaches.     

Visual hallucinations in patients with retinal disease

PURPOSE: To investigate the prevalence and phenomenologic nature of visual hallucinations among patients with retinal disease and to investigate whether presence of hallucinations is a significant predictor of functional status, quality of life, and/or emotional distress after adjusting for visual acuity. DESIGN: Cross-sectional study. METHODS: Eighty-six consecutive patients at the Wilmer Ophthalmologic Institute Retinal Vascular Center were interviewed using the Sickness Impact Profile, Community Disability Scale, General Health Questionnaire, Visual Phenomena Interview, Eysenck Personality Questionnaire, and Telephone Interview for Cognitive Status. RESULTS: The prevalence of visual hallucinations was 15.1%. Most were formed hallucinations in clear consciousness that lasted for seconds to minutes. The majority of patients had been experiencing visual hallucinations for less than 1 year (61.5%) or for 1 to 2 years (23.1%). Only two of the 13 patients with hallucinations had informed a physician of their hallucinations. Univariate analyses revealed that variables significantly associated with experiencing hallucinations were female sex, worse visual acuity, bilateral visual impairment, emotional distress, decreased functional status, and decreased quality of life. Regression analysis demonstrated that among patients with relatively good vision, those who experienced hallucinations were more emotionally distressed and had a lower quality of life than patients without hallucinations. CONCLUSIONS: Visual hallucinations among patients with retinal disease are common, underdiagnosed, and not associated with cognitive deficits, abnormal personality traits, or a family or personal history of psychiatric morbidity. Among patients with relatively good vision, hallucinations are associated with increased emotional distress and decreased quality of life.     

Surgical posterior vitreous detachment combined with gas/air tamponade for treating macular edema associated with branch retinal vein occlusion: retinal tomography and visual outcome

PURPOSE: To assess the effectiveness of surgical posterior vitreous detachment (PVD) together with gas/air tamponade in treating visual impairment from macular edema associated with branch retinal vein occlusion (BRVO-macular edema). METHODS: A cohort study was conducted. To treat visual disturbance caused by BRVO-macular edema in 19 consecutive patients at a University Hospital, phacoemulsification, intraocular lens implantation, and vitrectomy were performed, together with gas/air tamponade. Patients were followed up postoperatively for 3-18 months. Foveal structure was defined using optical coherence tomography (OCT). Preoperative visual acuity, central retinal thickness, and interval between BRVO onset and operation were compared between patients with postoperative visual improvement and those without improvement. RESULTS: Ten patients recovered normal or near-normal foveal configuration, while nine patients did not. Mean postoperative visual acuity in the former group of patients was significantly higher than in the latter. Mean foveal retinal thickness decreased significantly after the operation. The interval from onset of BRVO until operation was significantly shorter in patients with improved postoperative vision than in other patients, and patients operated on within 11 months had significantly increased postoperative visual acuity. CONCLUSIONS: Surgical PVD and gas/air tamponade appears effective in treating BRVO-macular edema, although relatively short duration from disease onset until operation is critical for improvement of vision. When a good postoperative foveal contour is seen, it seems to be associated with better visual outcome.     

Abnormalities of retinal function (light sense) following idiopathic central serous chorioretinopathy and diffuse retinal epitheliopathy

Although central serous retinopathy is considered a "benign" condition, it is associated in healed forms with frequent and troublesome impairment of visual function. The abnormalities are even more severe after diffuse retinal pigment epitheliopathy. Since Snellen visual acuity testing is a relatively imprecise index of visual function, it does not enable the subtle visual disturbances experienced by these patients to be fully appreciated. The authors evaluated visual function disorders by static automated perimetry and contrast sensitivity in 30 patients with healed central serous retinopathy and in five patients with diffuse epitheliopathy at a cicatricial stage. The results of this study are examined and discussed.     

Patients' expectation and experience of visual sensations during phacoemulsification under topical anaesthesia

PURPOSE: To describe what patients expect to see and the visual sensations they actually experience during phacoemulsification under topical anaesthesia. We also sought to determine if patients find their intraoperative visual experience frightening and the factors associated with this. MATERIALS AND METHODS: Ninety-eight patients who underwent phacoemulsification and intraocular lens implantation under topical anaesthesia were interviewed preoperatively on what they expected to see with their operated eye during surgery and again postoperatively on what they actually saw. No patient received counselling about possible intraoperative visual sensations. A logistic (multivariate) regression model was used for statistical analysis. RESULTS: Preoperatively, 36 patients (36.7%) expected at least light perception, 38 (38.8%) expected no light perception, and 24 (24.5%) were unsure what to expect. Some patients also expected a variety of different visual sensations. Postoperatively, all patients (100%) reported seeing light intraoperatively and many experienced various other visual sensations. Nineteen patients (19.4%) found their visual experience frightening. The following factors were statistically associated with a frightening visual experience: preoperative anxiety, previous cataract surgery in the fellow eye, experiencing an intraoperative increase in clarity, not seeing movement intraoperatively, and not knowing what to expect. CONCLUSIONS: The majority of patients in this study either expected that they would see nothing at all during the surgery or were unsure of what to expect. All patients subsequently saw at least some light, and many perceived various other visual sensations that were frightening to nearly one in five patients. Preoperative counselling should inform about possible intraoperative visual experience.     

Retinal detachment and herpesvirus retinitis in patients with AIDS.

BACKGROUND--The prolongation of survival of patients with herpesvirus retinitis and AIDS has been associated with a rise in the incidence of retinal detachment. In such cases, however, retinal reattachment may be difficult to achieve, and postoperative visual acuity may be poor despite anatomically successful surgery. METHODS--In order to examine factors affecting the visual outcome of surgery, a retrospective review of 29 patients with retinal detachment, herpesvirus retinitis, and AIDS was performed. Retinal reattachment surgery (32 procedures) or prophylactic laser demarcation (five procedures) was performed in 28 eyes of 23 patients. RESULTS--The macula was attached in 23/28 (82%) eyes at the last outpatient visit. Best postoperative visual acuity (median 6/18, range 6/6-hand movements) was significantly greater than final postoperative acuity (median counting fingers, range 6/6-no perception of light) (Wilcoxon sign rank test, p = 0.003), and was retained for a median of 3 months (1-91 weeks) after surgery. Poor visual outcome as evidenced by submedian final visual acuity was invariably associated with persistence of macular detachment, and significantly associated with the occurrence of optic atrophy (odds ratio = 5, p = 0.02). CONCLUSION--Retinal reattachment surgery appears justified in patients with herpesvirus retinitis and AIDS, but postoperative visual deterioration may occur in association with optic atrophy.     

Visual function in prolactinoma patients treated with bromocriptine

Ten patients with prolactin-secreting pituitary adenomas (prolactinoma) and visual dysfunction were treated primarily with bromocriptine. Nine patients had improvement of visual field or acuity associated with computed tomography (CT) evidence of a decrease in tumor size and reduction of serum prolactin level (SPL). Visual improvement usually began within days of commencing bromocriptine therapy. One patient with initial improvement had a worsening of visual fields at 11 months despite a normal SPL and a stable CT appearance of tumor size. This patient and the single patient without visual improvement underwent trans-sphenoidal hypophysectomy (TSH), but neither patient had postoperative visual improvement. Two other patients underwent TSH for nonvisual reasons. The remaining six patients have maintained excellent visual function at last follow-up (average of 18 months on bromocriptine therapy). This study shows that bromocriptine is an effective short- and long-term therapy for most patients with visual dysfunction due to prolactinoma.