Search

BACKGROUND Retinitis pigmentosa (RP) comprises a group of hereditary eye diseases characterized by progressive degeneration of retinal photoreceptors. It results in severe visual loss that may lead to blindness. Symptoms may become manifest during childhood or adulthood which include poor night vision (nyctalopia) and constriction of peripheral vision (visual field loss). Visual field loss is progressive and affects central vision later in the disease course. The worldwide prevalence of RP is approximately 1 in 4000, with 100,000 individuals affected in the USA. At this time, there is no proven therapy for RP. OBJECTIVES The objective of this review was to synthesize the best available evidence regarding the effectiveness and safety of vitamin A and fish oils (docosahexaenoic acid (DHA)) in preventing the progression of RP. SEARCH METHODS We searched the Cochrane Central Register of Controlled Trials (CENTRAL), which contains the Cochrane Eyes and Vision Trials Register (2020, Issue 2); Ovid MEDLINE; Embase.com; PubMed; Latin American and Caribbean Health Sciences Literature Database (LILACS); ClinicalTrials.gov; the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP); and OpenGrey. We did not use any date or language restrictions in the electronic searches for trials. We last searched the electronic databases on 7 February 2020. SELECTION CRITERIA We included randomized controlled trials that enrolled participants of any age diagnosed with any degree of severity or type of RP, and evaluated the effectiveness of vitamin A, fish oils (DHA), or both compared to placebo, vitamins (other than vitamin A), or no therapy, as a treatment for RP. We excluded cluster-randomized trials and cross-over trials. DATA COLLECTION AND ANALYSIS We prespecified the following outcomes: mean change from baseline visual field, mean change from baseline electroretinogram (ERG) amplitudes, and anatomic changes as measured by optical coherence tomography (OCT), at one-year follow-up, and mean change in visual acuity, at five-year follow-up. Two review authors independently extracted data and evaluated risk of bias for all included trials. We also contacted study investigators for further information when necessary. MAIN RESULTS In addition to three trials from the previous version of this review, we included a total of four trials with 944 participants aged 4 to 55 years. Two trials included only participants with X-linked RP and the other two included participants with RP of all forms of genetic predisposition. Two trials evaluated the effect of DHA alone; one trial evaluated vitamin A alone; and one trial evaluated DHA and vitamin A versus vitamin A alone. Two trials recruited participants from the USA, and the other two recruited from the USA and Canada. All trials were at low risk of bias for most domains. We did not perform meta-analysis due to clinical heterogeneity. Four trials assessed visual field sensitivity. Investigators found no evidence of a difference in mean values between the groups. However, one trial found that the annual rate of change of visual field sensitivity over four years favored the DHA group in foveal (-0.02 ± 0.55 (standard error (SE)) dB versus -0.47 ± 0.03 dB, P = 0.039), macular (-0.42 ± 0.05 dB versus -0.85 ± 0.03 dB, P = 0.031), peripheral (-0.39 ± 0.02 versus -0.86 ± 0.02 dB, P < 0.001), and total visual field sensitivity (-0.39 ± 0.02 versus -0.86 ± 0.02 dB, P < 0.001). The certainty of the evidence was very low. The four trials evaluated visual acuity (LogMAR scale) at a follow-up of four to six years. In one trial (208 participants), investigators found no evidence of a difference between the two groups, as both groups lost 0.7 letters of the Early Treatment Diabetic Retinopathy Study (ETDRS) visual acuity per year. In another trial (41 participants), DHA showed no evidence of effect on visual acuity (mean difference -0.01 logMAR units (95% confidence interval -0.14 to 0.12; one letter difference between the two groups; very low-certainty evidence). In the third trial (60 participants), annual change in mean number of letters correct was -0.8 (DHA) and 1.4 letters (placebo), with no evidence of between-group difference. In the fourth trial (572 participants), which evaluated (vitamin A + vitamin E trace) compared with (vitamin A trace + vitamin E trace), decline in ETDRS visual acuity was 1.1 versus 0.9 letters per year, respectively. All four trials reported electroretinography (ERG). Investigators of two trials found no evidence of a difference between the DHA and placebo group in yearly rates of change in 31 Hz cone ERG amplitude (mean ± SE) (-0.028 ± 0.001 log μV versus -0.022 ± 0.002 log μV; P = 0.30); rod ERG amplitude (mean ± SE) (-0.010 ± 0.001 log μV versus -0.023 ± 0.001 log μV; P = 0.27); and maximal ERG amplitude (mean ± SE) (-0.042 ± 0.001 log μV versus -0.036 ± 0.001 log μV; P = 0.65). In another trial, a slight difference (6.1% versus 7.1%) in decline of ERG per year favored vitamin A (P = 0.01). The certainty of the evidence was very low. One trial (51 participants) that assessed optical coherence tomography found no evidence of a difference in ellipsoid zone constriction (P = 0.87) over two years, with very low-certainty evidence. The other three trials did not report this outcome. Only one trial reported adverse events, which found that 27/60 participants experienced 42 treatment-related emergent adverse events (22 in DHA group, 20 in placebo group). The certainty of evidence was very low. The rest of the trials reported no adverse events, and no study reported any evidence of benefit of vitamin supplementation on the progression of visual acuity loss. AUTHORS' CONCLUSIONS Based on the results of four studies, it is uncertain if there is a benefit of treatment with vitamin A or DHA, or both for people with RP. Future trials should also take into account the changes observed in ERG amplitudes and other outcome measures from trials included in this review.

A 74-year-old man had reproducible superior and inferior arcuate visual field defects in the left eye only that were initially believed to be caused by primary open-angle glaucoma. Diagnostic evaluation with the aid of optical coherence tomography revealed extrafoveal vitreomacular traction (VMT) with secondary retinal thickening and schisis. We discuss the evaluation of non-glaucomatous visual field defects and review the literature on the pathogenesis, clinical manifestations, and treatment of VMT syndrome.

A 39-year-old white man presented with intractable headaches and papilledema. The initial workup, with normal MRI and MRV but elevated cerebrospinal fluid protein raised concerns about the putative diagnosis of idiopathic intracranial hypertension, and his condition remained refractory to maximum medical treatment. Angiography revealed cerebral venous sinus stenosis, thought to represent chronic thrombosis. The diagnosis and treatment of cerebral venous sinus stenosis and thrombosis are discussed.

Rosai-Dorfman disease (RDD) is a rare histiocytic disorder most often characterized by painless cervical lymphadenopathy, but it may also present with orbital disease. The clinical course of RDD is variable; it can be either relapsing-remitting or progressive, and the outcome relates to clinical location and treatment response. Orbital RDD can have an insidious onset and similar presentation to other ophthalmic conditions; this can result in a delayed diagnosis. Nearly all cases of orbital RDD cause visual disturbances and require treatment. Because orbital RDD is an uncommon presentation, a variety of interventions have been employed, including surgery, immunotherapy, chemotherapy, and radiotherapy. We present a case of salvage radiotherapy for progressive orbital RDD refractory to surgery and chemotherapy in a pediatric patient.

A case of progressive optic neuropathy in a woman with a history of breast cancer is presented. Differential diagnoses including optic neuritis, infiltrative optic neuropathy, carcinomatous meningitis, and toxic optic neuropathies are discussed. Risk factors for metastatic brain lesions are also discussed.

BACKGROUND: Retinitis pigmentosa (RP) comprises a group of hereditary eye diseases characterized by progressive degeneration of retinal photoreceptors. It results in severe visual loss that may lead to blindness. Symptoms may become manifest during childhood or adulthood which include poor night vision (nyctalopia) and constriction of peripheral vision (visual field loss). Visual field loss is progressive and affects central vision later in the disease course. The worldwide prevalence of RP is approximately 1 in 4000, with 100,000 individuals affected in the USA. At this time, there is no proven therapy for RP. OBJECTIVES: The objective of this review was to synthesize the best available evidence regarding the effectiveness and safety of vitamin A and fish oils (docosahexaenoic acid (DHA)) in preventing the progression of RP. SEARCH METHODS: We searched the Cochrane Central Register of Controlled Trials (CENTRAL), which contains the Cochrane Eyes and Vision Trials Register (2020, Issue 2); Ovid MEDLINE; Embase.com; PubMed; Latin American and Caribbean Health Sciences Literature Database (LILACS); ClinicalTrials.gov; the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP); and OpenGrey. We did not use any date or language restrictions in the electronic searches for trials. We last searched the electronic databases on 7 February 2020. SELECTION CRITERIA: We included randomized controlled trials that enrolled participants of any age diagnosed with any degree of severity or type of RP, and evaluated the effectiveness of vitamin A, fish oils (DHA), or both compared to placebo, vitamins (other than vitamin A), or no therapy, as a treatment for RP. We excluded cluster‐randomized trials and cross‐over trials. DATA COLLECTION AND ANALYSIS: We prespecified the following outcomes: mean change from baseline visual field, mean change from baseline electroretinogram (ERG) amplitudes, and anatomic changes as measured by optical coherence tomography (OCT), at one‐year follow‐up, and mean change in visual acuity, at five‐year follow‐up. Two review authors independently extracted data and evaluated risk of bias for all included trials. We also contacted study investigators for further information when necessary. MAIN RESULTS: In addition to three trials from the previous version of this review, we included a total of four trials with 944 participants aged 4 to 55 years. Two trials included only participants with X‐linked RP and the other two included participants with RP of all forms of genetic predisposition. Two trials evaluated the effect of DHA alone; one trial evaluated vitamin A alone; and one trial evaluated DHA and vitamin A versus vitamin A alone. Two trials recruited participants from the USA, and the other two recruited from the USA and Canada. All trials were at low risk of bias for most domains. We did not perform meta‐analysis due to clinical heterogeneity. Four trials assessed visual field sensitivity. Investigators found no evidence of a difference in mean values between the groups. However, one trial found that the annual rate of change of visual field sensitivity over four years favored the DHA group in foveal (−0.02 ± 0.55 (standard error (SE)) dB versus −0.47 ± 0.03 dB, P = 0.039), macular (−0.42 ± 0.05 dB versus −0.85 ± 0.03 dB, P = 0.031), peripheral (−0.39 ± 0.02 versus −0.86 ± 0.02 dB, P < 0.001), and total visual field sensitivity (−0.39 ± 0.02 versus −0.86 ± 0.02 dB, P < 0.001). The certainty of the evidence was very low. The four trials evaluated visual acuity (LogMAR scale) at a follow‐up of four to six years. In one trial (208 participants), investigators found no evidence of a difference between the two groups, as both groups lost 0.7 letters of the Early Treatment Diabetic Retinopathy Study (ETDRS) visual acuity per year. In another trial (41 participants), DHA showed no evidence of effect on visual acuity (mean difference −0.01 logMAR units (95% confidence interval −0.14 to 0.12; one letter difference between the two groups; very low‐certainty evidence). In the third trial (60 participants), annual change in mean number of letters correct was −0.8 (DHA) and 1.4 letters (placebo), with no evidence of between‐group difference. In the fourth trial (572 participants), which evaluated (vitamin A + vitamin E trace) compared with (vitamin A trace + vitamin E trace), decline in ETDRS visual acuity was 1.1 versus 0.9 letters per year, respectively. All four trials reported electroretinography (ERG). Investigators of two trials found no evidence of a difference between the DHA and placebo group in yearly rates of change in 31 Hz cone ERG amplitude (mean ± SE) (−0.028 ± 0.001 log μV versus −0.022 ± 0.002 log μV; P = 0.30); rod ERG amplitude (mean ± SE) (−0.010 ± 0.001 log μV versus −0.023 ± 0.001 log μV; P = 0.27); and maximal ERG amplitude (mean ± SE) (−0.042 ± 0.001 log μV versus −0.036 ± 0.001 log μV; P = 0.65). In another trial, a slight difference (6.1% versus 7.1%) in decline of ERG per year favored vitamin A (P = 0.01). The certainty of the evidence was very low. One trial (51 participants) that assessed optical coherence tomography found no evidence of a difference in ellipsoid zone constriction (P = 0.87) over two years, with very low‐certainty evidence. The other three trials did not report this outcome. Only one trial reported adverse events, which found that 27/60 participants experienced 42 treatment‐related emergent adverse events (22 in DHA group, 20 in placebo group). The certainty of evidence was very low. The rest of the trials reported no adverse events, and no study reported any evidence of benefit of vitamin supplementation on the progression of visual acuity loss. AUTHORS' CONCLUSIONS: Based on the results of four studies, it is uncertain if there is a benefit of treatment with vitamin A or DHA, or both for people with RP. Future trials should also take into account the changes observed in ERG amplitudes and other outcome measures from trials included in this review.

A 55-year-old HIV-positive man presented with acute vision loss in the right eye and altered mental status. Ophthalmic evaluation revealed light perception vision OD with a right relative afferent pupillary defect, conjunctival chemosis, large mutton-fat keratitic precipitates, and diffuse cream-colored vitreous cells. Magnetic resonance imaging of the brain and orbit with and without contrast with fat saturation showed choroidal thickening OD, multifocal deep periventricular and deep ganglionic enhancing lesions, and a suprasellar mass. Brain biopsy showed diffuse large B-cell lymphoma. Intrathecal chemotherapy with methotrexate and cytarabine and whole brain radiation therapy failed. His mental status deteriorated. He developed pancytopenia, neutropenic fever, and septic shock and subsequently expired under palliative care.

Background: Optic disc elevation associated with Down syndrome is an uncommon phenomenon and raises the suspicion of an intracranial space-occupying lesion, thus necessitating the consideration of invasive and noninvasive investigations. Method of study: Four patients with Down syndrome and optic disc elevation without an underlying intracranial pathology are reported. Thorough ophthalmological and neuroradiological investigations were performed on each patient. Mild hyperopia occurred in three patients and myopia in one. Conclusions: We believe that in most Down syndrome patients with disc elevation, fluorescein angiography and/or clinical follow-up may be sufficient.

Purpose To study the course and visual outcome of Vogt-Koyanagi-Harada (VKH) syndrome in children. Methods The medical records of 97 consecutive patients with VKH syndrome were reviewed. Patients had been seen at King Khaled Eye Specialist Hospital in Riyadh, Saudi Arabia. The records were divided into two groups: those in whom the onset of the disease occurred at the age of 14 years or younger (pediatric group) and those in whom the disease manifested after the age of 14 years (adult group). Results Eight (61%) of the 13 children required cataract surgery compared to 14(17%) of 84 adult patients. Despite medical and surgical therapy, eight (61%) of the pediatric group had a final visual acuity of 20/200 or worse compared with 22 (26%) of the 84 adult patients (p=0.0103). Conclusion The course of early-onset VKH (before the age of 14 years) appears to be aggressive. The ocular complications are more severe in children than in adults, leading to rapid deterioration in vision. Despite therapy, the final visual outcome of VKH in children is poor.

A 39 year-old Behcet's patient had demyelination of retinal myelinated nerve fibers after recurrent papillitis and vitritis. Oral Prednisolone, cyclosporine, and later periocular corticosteroids and oral colchicine were used but demyelination continued over a 5 year-period. A pseudobulbar palsy with urinary incontinence and pyramidal tract signs developed and azathioprine and corticosteroids were used. Demyelination of retinal nerve fibers stopped and while treatment was underway, the central nervous system (CNS) signs were stable. While the ocular pathology of Behcet's can mirror the CNS signs, indeed optic nerve ischemic demyelination may signal the potential for CNS involvement; azathioprine with prednisone may be more effective in the long-term for optic nerve and CNS involvement than cyclosporine with prednisone.

To assess the outcome and complications of cataract extraction in patients with Behçet's disease.Thirty-six Behçet's disease patients underwent cataract surgery without intraocular lens implantation. Twenty-six patients had phacoemulsification or extracapsular cataract extraction, and ten patients had pars plana lensectomy/vitrectomy. The follow-up period ranged from 24 to 84 months (mean, 38 months).There was no statistically significant difference in the follow-up period of patients who underwent phacoemulsification, extracapsular cataract extraction, or pars plana lensectomy/vitrectomy. Sixteen (62%) of 26 patients who had extracapsular cataract extraction or phacoemulsification had a visual acuity of 20/200 or better compared with none of the 10 patients who had pars plana lensectomy/vitrectomy. Three (30%) of ten eyes which underwent pars plana lensectomy/vitrectomy eventuated in phthisis bulbi while none of the eyes that underwent extracapsular cataract extraction or phacoemulsification developed phthisis bulbi.Extracapsular cataract extraction or phacoemulsification had a better visual outcome and fewer postoperative complications than pars plana lensectomy/vitrectomy in patients with Behçet's disease.

Federico da Montefeltro (1422–1482), the Duke of Urbino, was a well-known historical figure during the Italian Renaissance. He is the subject of a famous painting by Piero della Francesca (1416–1492), which displays the Duke from the left and highlights his oddly shaped nose. The Duke is known to have lost his right eye due to an injury sustained during a jousting tournament, which is why the painting portrays him from the left. Some historians teach that the Duke subsequently underwent nasal surgery to remove tissue from the bridge of his nose in order to expand his visual field in an attempt to compensate for the lost eye. In theory, removal of a piece of the nose may have expanded the nasal visual field, especially the “eye motion visual field” that encompasses eye movements. In addition, removing part of the nose may have reduced some of the effects of ocular parallax. Finally, shifting of the visual egocenter may have occurred, although this seems likely unrelated to the proposed nasal surgery. Whether or not the Duke actually underwent the surgery cannot be proven, but it seems unlikely that this would have substantially improved his visual function.

A 64-year-old woman presented with bilateral optic neuropathy leading to a diagnosis of Sjogren syndrome. She improved with high-dose corticosteroids and oral azathioprine and was subsequently found to have asymptomatic bilateral iridocyclitis. Although central nervous system manifestations of Sjogren syndrome are documented in the literature, they are not widely recognized in clinical practice. Associated optic neuritis often mimics demyelinating disease such as multiple sclerosis. Treatment of CNS disease related to Sjogren syndrome is highly controversial. Uveitis is an uncommon finding associated with Sjogren syndrome.

In a prospective, open clinical trial, we studied long-term effects of cyclosporine (CsA) on the optic nerve and retinal vasculitis in 14 Behçet's disease patients. Patients were treated with CsA and corticosteroids for a mean period of 42 months, with a range of 36 to 52 months. They received an initial CsA dosage of 7 mg/kg/day for three days, followed by 5 mg/kg/day, and prednisone 1 mg/kg/day for three to five days, tapered to 0.4 mg/kg/day. CsA was tapered when clinical response was noted. Improvement occurred in visual acuity and visual field defects secondary to papillitis, optic neuritis, macular neuroretinitis, and retinal phlebitis, but not with retinal arteritis. Despite a 12/14 (85%) exacerbation rate, no permanent liver or renal lab tests abnormalities were noted. Intermittent, low-dose CsA therapy may be considered in treatment of acute retinal and optic nerve vasculitis assisted with Behçet's disease.

A 58-year-old woman presented with a problem with her peripheral vision. Computed tomography scan showed an occipital hemorrhagic stroke. She subsequently suffered gastrointestinal bleeding and at surgery biopsy of a portion of the middle colic artery aneurysm revealed changes consistent with polyarteritis nodosa.

Two months following an Epstein-Barr virus infection, a 17-year-old white female presented with seizures, intermittent visual changes, and altered mental status. Magnetic resonance imaging showed white matter changes of acute disseminated encephalomyelitis with a predilection for posterior cerebral artery distributions but without radiological evidence of arteritis. Epstein-Barr virus titers and polymerase chain reaction analysis results for the virus were consistent with postinfectious acute disseminated encephalomyelitis. The symptoms and signs improved following treatment with high-dose corticosteroids and intravenous immunoglobulin. Although Epstein-Barr virus can cause acute viral encephalomyelitis, the authors report a case of acute disseminated encephalomyelitis months after acute Epstein-Barr virus infection.

Objective This study aimed to determine whether heparin surface-modified (HSM) intraocular lenses (IOLs) with a hydrophilic surface would reduce cell adherence and other postoperative changes compared with the conventional polymethylmethacrylate (PMMA) IOLs in patients with either diabetes mellitus or inactive uveitis. Design The study design was a randomized, double-masked, clinical trial. Participants Twenty-five patients with bilateral cataracts, 14 with inactive anterior uveitis and 11 with diabetes, with an age range of 11 to 81 years (mean, 52.8 years) participated. Intervention Bilateral cataract extraction with posterior chamber IOL implantation was measured, each patient receiving an HSM lens in one eye and a PMMA lens in the other. Pharmacia one-piece HSM and PMMA IOLs were used. Postoperative ocular changes were evaluated at regular intervals for 24 months in patients with inactive uveitis and for 6 months in patients with diabetes. Patients and physicians alike were unaware of which eye contained which lens until postoperative results were compiled. Records were kept by a study coordinator. Main outcome measures Comparisons of posterior synechiae, IOL cellular deposits, and posterior capsular fibrosis between PMMA and HSM IOLs were measured. Results Using the chi-square test, no statistically significant difference was found between the HSM and PMMA IOLs in the number of cellular deposits found on the anterior IOL surface, the number of adhesions between the iris and IOL, or the incidence of capsular opacification. Conclusion The HSM and PMMA IOLs showed similar postoperative results in patients with inactive uveitis or diabetes mellitus.

In the absence of obvious pulmonary or disseminated tuberculosis, ocular and central nervous system (CNS) tuberculosis may represent a significant diagnostic challenge. Refinements in polymerase chain reaction techniques and neuroimaging have strengthened the battery of tests used to diagnose CNS and ocular tuberculosis, yet in many cases, the diagnosis remains one of exclusion; it may ultimately be determined through exacerbation by anti-inflammatory therapy with subsequent improvement by antitubercular medication treatment. Because of emerging drug resistance, at least a two-drug regimen is required for therapeutic testing and treatment of isolated ocular tuberculosis. If pulmonary or miliary disease coexists, a 6-month, four-drug regimen with isoniazid, rifampin, pyrazinamide, and ethambutol is required for treatment. Tubercular meningitis is treated with the same four-drug regimen for at least 9 to 12 months. Burden of therapeutic compliance rests on the treating physician and public health sector. Best compliance is realized with directly observed therapy.

Background A young woman with a history of controlled hypertension noted a suddenly decreased peripheral temporal field in the left eye. This occurred after moderate peripartum hypertension. Method A monocular peripheral temporal crescentic defect could be plotted on Goldmann visual fields despite a normal dilated peripheral retinal examination and normal disc appearance. Result A dilated parieto-occipital sulcus could be seen on computed tomography, and magnetic resonance imaging showed changes consistent with atrophy and gliosis in the cuneus, precuneus, and anterior calcarine cortex surrounding the parieto-occipital sulcus. Conclusion By magnetic resonance imaging, this can be seen to comprise less than 10% of the visual cortex, as suggested by the Horton and Hoyt revised Holmes map. The temporal crescent syndrome is a rare monocular retrochiasmatic visual field defect that can be correlated to a lesion along the parieto-occipital sulcus.

The present study was undertaken because of the recent report of an association between familial multiple sclerosis (MS) and prior ownership of a small pet. Forty-five patients with sporadic MS were retrospectively compared with 45 closely matched controls and were found to have owned a significantly greater number of indoor dogs prior to the onset of MS. Importantly, the MS patients had also had more exposure to neurologically ill dogs in the five years before the onset of symptoms. These new data further support the hypothesis that house dogs may serve as an animal vector in MS.