We report on three cases of visual loss due to nonarteritic anterior ischemic optic neuropathy that developed during the course of refractory anemia, a subtype of myelodysplastic syndrome. Patients underwent fundus, visual field examination, and fluorescein angiography. A thrombophilic tendency investigation including prothrombin time, partial thromboplastin time, protein C, free ...

PURPOSE: Consecutive exotropia is commonly the result of a surgical correction of a primary esotropia but may develop spontaneously. This study examines the clinical characteristics of patients who have developed a spontaneous consecutive exotropia and relates the key features to previously reported findings. METHODS: Patients were identified by routine clinical ...

To investigate six cases of annular cyclitis. All patients with impairment of visual acuity underwent complete ophthalmologic examination, color fundus photography, laboratory tests and fluorescein angiography. Indocyanine green (ICG) angiography and B-scan ultrasonography were also performed in three cases in order to diagnose the disease. All patients presented a unilateral ...

PURPOSE: To report three cases of idiopathic Creutzfeldt-Jakob disease (CJD) with visual symptoms. CASES: Patient 1 initially visited our ophthalmology department complaining of blurred vision. Electro-retinography (ERG) showed a selective reduction of the b wave before she was diagnosed with CJD. Patient 2 had normal ERG results even with severely ...

PURPOSE: Great variation exists in the age of onset of symptoms and the severity of disease at a given age in patients with retinitis pigmentosa (RP). The final pathway for this disease may involve apoptotic photoreceptor cell death. Telomere length is associated with biologic aging, senescence, and apoptosis. We evaluated ...

AIM: Our purpose was to determine the vitreous erythropoietin (EPO) level in patients with rhegmatogenous retinal detachment (RRD) and proliferative vitreoretinopathy (PVR). METHODS: The levels of EPO in vitreous were measured using a sandwich enzyme-linked immunosorbent assay kit from 64 patients, of whom 40 had RRD, 13 had PVR and ...

Frequency doubling technology (FDT) perimetry measures contrast sensitivity. The magnocellular component of ganglion cells in human retina is isolated as a whole by the FDT stimulus. The aim of this study is to investigate the role of Humphrey Matrix threshold testing in the detection of early functional retinal impairment in ...

The clinical manifestations and histopathologic and neuroimaging findings in 4 Indian patients with a variant form of mucolipidosis IV are described. The presenting symptoms were psychomotor delay, spastic paraplegia, and mild mental retardation. One patient also had visual deterioration due to optic atrophy. None had corneal or retinal abnormalities. Magnetic ...

To describe vision loss associated with the use or removal of silicone oil retinal tamponade. Records were reviewed of all patients with a decrease in visual acuity of at least 3 Snellen lines from best acuity with 5000 centistoke silicone oil in place or after removal of silicone oil at ...

To evaluate retrospectively the features, treatment, and outcome of idiopathic intracranial hypertension (IIH) in children. Nine patients, 15 years and younger, diagnosed with IIH. Inclusion criteria were papilledema, normal brain computer tomography or magnetic resonance imaging, cerebrospinal fluid pressure greater than 250 mm H(2)O, normal cerebrospinal fluid content, and a ...

PURPOSE: It has recently been reported that the anti-aquaporin-4 antibody (AQP4-Ab) can be a specific marker of neuromyelitis optica. We present three cases of optic neuritis (ON) where the patients tested positive for AQP4-Ab, but showed no neurological signs. METHODS: Sera were obtained from 32 Japanese patients with ON and ...

AIMS: Optic nerve sheath meningiomas typically present with unilateral visual deterioration. Here, a single centre's experience with radiotherapy aimed at local control and visual stabilisation is presented. MATERIALS AND METHODS: The meningioma database within the Radiation Oncology Department, Prince of Wales Hospital was audited for patients whose meningiomas took origin ...

To better understand the effects of retinitis pigmentosa (RP) on post-receptor anatomy, the thicknesses of the receptor, inner nuclear, retinal ganglion cell (RGC), and retinal nerve fiber layers (RNFL) were measured with frequency-domain optical coherence tomography (fdOCT). FdOCT scans were obtained from the horizontal midline in 30 patients with RP ...

There is evidence that some visual information in blind regions may still be processed in patients with hemifield defects after cerebral lesions ("blindsight"). We tested the hypothesis that, in the absence of retinogeniculostriate processing, residual retinotectal processing may still be detected as modifications of saccades to seen targets by irrelevant ...

The arteriole-to-venule ratio (AVR) is widely used for investigating subclinical cerebral microangiopathy. The possible occurrence of retinal vascular caliber changes was investigated in a population of hyperhomocysteinaemic (plasma total homocysteine (tHcy) >13 micromol/l) adult epileptic patients. Retinal photographs of cases and controls were evaluated for generalized narrowing of the retinal ...

Optic neuropathy is common in mitochondrial disorders, but poorly characterized in Friedreich's ataxia (FRDA), a recessive condition caused by lack of the mitochondrial protein frataxin. We investigated 26 molecularly confirmed FRDA patients by studying both anterior and posterior sections of the visual pathway using a new, integrated approach. This included ...

This article describes nine Chinese patients with Bietti's crystalline dystrophy, including two families, one consisting of three siblings and the other a pair of sisters. All patients had the classic refractile deposits located in all layers of the retina, with varying degrees of pigment epithelium atrophy. However, paralimbal crystals were ...

PURPOSE: To assess and compare longitudinal visual function and retinal morphology in patients with methylmalonic aciduria with homocystinuria, cobalamin C type (cblC), and identified mutations in the MMACHC gene. METHODS: Vision function, anterior segment, and fundi were evaluated in patients with homozygous or compound heterozygous MMACHC mutations. Best-corrected visual acuity, ...

To compare melanin-related near-infrared fundus autofluorescence (FAF; NIA, excitation 787 nm, emission >800 nm) with lipofuscin-related FAF (excitation 488 nm, emission >500 nm) in retinitis pigmentosa (RP). Thirty-three consecutive RP patients with different modes of inheritance were diagnosed clinically, with full-field ERG, and if possible with molecular genetic methods. FAF ...

To investigate retinal microstructure of patients affected with malattia leventinese (MLVT) and mutation in the EFEMP1 gene using high-resolution optical coherence tomography (OCT). Patients diagnosed with MLVT received a comprehensive eye exam, full-field and multifocal electroretinogram testing and imaging with a high-resolution Fourier domain OCT (Fd-OCT, UC Davis Medical Center, ...

BACKGROUND: Juxtapapillary capillary hemangiomas can occur as isolated clinical abnormalities or a manifestation of von Hippel-Lindau (VHL) disease. Because of the location of these vascular tumors on or adjacent to the optic disc, any therapeutic treatment option involves risk. Management typically includes close monitoring, with treatment initiation as the tumor ...

PURPOSE: Aspergillus species is found worldwide and does not normally cause disease. However, when the immune system is compromised, it can invade many organs and be responsible for severe disease. The authors present cases with both classical and atypical features of ophthalmic aspergillosis. METHODS: Case series of three patients. RESULTS: ...

In a recent study, we found high levels of erythropoietin (EPO) in patients with diabetic macular oedema (DME), suggesting a role of EPO in the pathogenesis of this condition. To investigate a possible relationship between EPO and other diseases causing macular oedema, we determined vitreous levels of this peptide in ...

PURPOSE: To estimate the mean rates of ocular function loss in patients with autosomal recessive retinitis pigmentosa due to USH2A mutations. METHODS: In 125 patients with USH2A mutations, longitudinal regression was used to estimate mean rates of change in Snellen visual acuity, Goldmann visual field area (V4e white test light), ...

AIMS: To describe the clinical and radiological features of orbital apex syndromes caused by presumed tuberculosis (TB). METHODS: A review of the discharge summaries of all patients seen in the Medical Eye Unit (MEU), St Thomas' Hospital between 1975 and 2006 identified seven patients with a diagnosis of orbital apex ...

PURPOSE: To describe diverse and atypical presentations of the most common masquerader in neoplastic masquerade syndromes. METHODS: Retrospective interventional case series. The authors identified three patients who presented with atypical and diagnostically challenging masquerading manifestations. These patients were eventually found to have primary intraocular lymphoma (PIOL). Their case histories, presenting ...

PURPOSE: To assess components of the fibrinolytic system in the vitreous humour and serum of patients with vitreoretinal disorders. METHODS: Forty-three samples of vitreous humour and plasma of 43 patients undergoing pars plana vitrectomy for macular hole, macular pucker, retinal detachment or proliferative vitreoretinopathy were evaluated for their content of ...

AIM: To report a case of spontaneous resolution of diabetic maculopathy with cystoid macular oedema in a diabetic patient after discontinuation of rosiglitazone, documented with serial ocular computed tomography (OCT) images. METHODS: A 59-year-old male with a 6-year history of well-controlled diabetes mellitus presented with reduced visual acuity (VA) attributed ...

BACKGROUND: Neuromyelitis optica (NMO) is an inflammatory disease with combined features of optic neuritis and myelitis. This pathologic entity may induce severe disability, including visual loss and paraplegia. Other than clinical follow-up, there is no marker for severity of the disease. OBJECTIVES: To evaluate the use of optical coherence tomography ...

PURPOSE: Bilateral visual field constriction has been reported following the use of the antiepileptic drug (AED) vigabatrin. The incidence of retinal toxicity is variable and there are limited data in Asian populations. The authors report the results of ophthalmologic examination in Chinese patients taking this drug. METHODS: The authors identified ...

BACKGROUND: Drusen-like lesions beneath the retina in patients with partial lipodystrophy and type II mesangiocapillary glomerulonephritis (MCGN) were first reported in 1989. This study reports the long-term follow-up of this original cohort of patients more than 10 years later. METHODS: Three patients had undergone renal transplantation. Retinopathy was graded semiquantitatively ...

INTRODUCTION: Lyme disease is a multisystemic disease with protean ocular manifestations. We describe the occurrence of inflammatory choroidal neovascular membrane (CNVM) in two patients suffering from presumed Lyme disease. METHODS: Descriptive review of the clinical records of two patients. RESULTS: Patient 1: 16-year-old healthy male presenting with a visual acuity ...

BACKGROUND AND PURPOSE: Our study aims to evaluate retinal function and neural conduction in post-retinal visual pathways of patients with non-arteritic ischaemic optic neuropathy (NION). METHODS: Twenty patients (mean age: 63.7 +/- 5.96 year) with NION and 20 age-similar control subjects were enrolled. Simultaneous recording of pattern electroretinograms (PERGs) and ...

PURPOSE: To evaluate the patterns of choroidal watershed zones (WZs) in exudative age-related macular degeneration (AMD) and to describe their relationship with choroidal neovascularization (CNV). METHODS: We retrospectively evaluated 50 digital indocyanine green video-angiograms of 50 patients with exudative AMD demonstrating one or more WZs. In addition, the relationship between ...

BACKGROUND: Emerging evidences suggest that circulating hematopoietic stem cells (HSCs) affect the pathogenesis of choroidal neovascularization (CNV), however, the roles of HSCs in CNV remain unclear in human population. The current study was designed to investigate the role of HSCs in the pathogenesis of CNV secondary to pathologic myopia (PM). ...

PURPOSE: A retrospective case-matched study designed to compare patients with diabetic retinopathy (DR) and other ocular diseases, managed in a low-vision clinic, in four different types of functional vision. METHODS: Reading, mobility, visual motor, and visual information processing were measured in the patients (n = 114) and compared with those ...

PURPOSE: To demonstrate efficacy and safety of the implantation of neural retinal progenitor cell layers (sheets) with its retinal pigment epithelium (RPE) in retinitis pigmentosa (RP) and dry age-related macular degeneration (AMD) patients with 20/200 or worse vision in the surgery eye. DESIGN: Interventional nonrandomized clinical trial. METHODS: Ten patients ...

To study the topography of photoreceptor loss early in the course of Leber congenital amaurosis (LCA) caused by RPE65 mutations. Young patients with RPE65-LCA (n = 9; ages, 6-17 years) were studied with optical coherence tomography (OCT) in a wide region of central retina. Outer nuclear layer (ONL) thickness was ...

The present study was aimed to investigate optic nerve involvement by computerized perimetry in 40 (29 women, 11 men) consecutive GO patients not showing definite dysthyroid optic neuropathy (DON). All patients presenting visual acuity defects, pallor or swelling of the optic nerve, concomitant eye disease, evidence of apical crowding or ...

PURPOSE: To investigate the ability of a technique employing pupillometry and functionally-shaped stimuli to assess loss of visual function due to glaucomatous optic neuropathy. METHODS: Pairs of large stimuli, mirror images about the horizontal meridian, were displayed alternately in the upper and lower visual field. Pupil diameter was recorded and ...

PURPOSE: The goal of this study was to investigate the ultrastructural changes of tendon axonal profiles of medial recti in patients with intermittent exotropia at different ages. In addition, we compared the patterns of degeneration with those of secondary exotropia over time. METHODS: Thirteen patients, with different ages, with exotropia ...

PURPOSE: To characterize the clinical, psychophysical, and electrophysiological phenotype of 19 patients with enhanced S-cone syndrome (ESCS) and relate the phenotype to the underlying genetic mutation. METHODS: Patients underwent ophthalmic examination and functional testing including pattern ERG, full-field ERG, and long-duration and short-wavelength stimulation. Further tests were performed in some ...

OBJECTIVE: To assess the role of low vision devices in visual rehabilitation of patients with Stargardt's disease. STUDY DESIGN: Descriptive study. PLACE AND DURATION OF STUDY: The Khyber Institute of Ophthalmic Medical Sciences, Hayatabad Medical Complex, Peshawar, Pakistan, from June 2003 to June 2005. PATIENTS AND METHODS: Patients diagnosed as ...

Three patients with recent central retinal vein occlusion underwent vitrectomy, fluid-air exchange, and a 50-microg tissue plasminogen activator intravitreal injection. After a mean follow-up period of 38.7 weeks, no patient's vision improved and one patient required an additional vitrectomy. Post-vitrectomy tissue plasminogen activator injection did not improve the course of ...

PURPOSE: In acute retinal necrosis, which is caused by herpes virus, urgent treatment is essential. DESIGN AND METHODS: Here we present a 47 years old male patient, who developed an acute retinal necrosis in his left eye. Therapy was initiated with systemic aciclovir and corticosteroids. RESULTS: During the course of ...

The aim of this study was to establish the causes of leukocoria in pediatric patients in a tertiary hospital environment. All patients younger than 10 years who presented with leukocoria between January 1, 1999, and December 31, 2000, were included in the study; 39 (55%) patients were boys and 32 ...

PURPOSE: To calculate the reliability of an eye-dedicated triaging system named Rome Eye Scoring System for Urgency and Emergency (RESCUE). METHODS: There were four coding parameters: pain, redness, loss of vision, and risk of open globe. Each parameter is assigned a score, the sum of which allows color coding. There ...

Early-onset, severe retinal dystrophy caused by mutations in the gene encoding retinal pigment epithelium-specific 65-kD protein (RPE65) is associated with poor vision at birth and complete loss of vision in early adulthood. We administered to three young adult patients subretinal injections of recombinant adeno-associated virus vector 2/2 expressing RPE65 complementary ...

Leber's congenital amaurosis (LCA) is a group of inherited blinding diseases with onset during childhood. One form of the disease, LCA2, is caused by mutations in the retinal pigment epithelium-specific 65-kDa protein gene (RPE65). We investigated the safety of subretinal delivery of a recombinant adeno-associated virus (AAV) carrying RPE65 complementary ...

To determine the prevalence of cystoid macular oedema (CME) by optical coherence tomography (OCT) in retinitis pigmentosa (RP) patients with no evidence of cystic macular lesions on fundus examination. We included 63 RP patients with no evidence of cystic-appearing macular changes on fundus examination. All patients underwent a complete ocular ...