Structural changes of the choroid in sarcoid- and tuberculosis-related granulomatous uveitis.

AIM: The aim of this study is to characterise the choroidal features of patients diagnosed with sarcoid- and tuberculosis (TB)-associated granulomatous uveitis using spectral domain optical coherence tomography (OCT). METHODS: Twenty-seven patients (27 eyes) diagnosed with sarcoid- (13 eyes) and TB (14 eyes)-related uveitis were included in this retrospective, cross-sectional study. Over a six-month period, patients diagnosed with sarcoid and TB granulomatous uveitis were scanned using enhanced depth imaging OCT. Clinical and demographical characteristics were recorded, including the method of diagnosis, disease activity, site of inflammation (anterior or posterior), treatments, and visual acuity (VA). Manual segmentation of the choroidal layers was performed using custom image analysis software. RESULTS: The main outcome measure was OCT-derived thickness measurements of the choroid and choroidal sublayers (Haller's large vessel and Sattler's medium vessel layers) at the macula region. The ratio of Haller's large vessel to Sattler's medium vessel layer was significantly different at the total macula circle in eyes diagnosed with TB uveitis (1.47 (=140.71/95.72 µm)) compared with sarcoid uveitis (1.07 (=137.70/128.69 µm)) (P=0.001). A thinner choroid was observed in eyes with a VA ≥0.3 LogMAR (Snellen 6/12; 198.1 µm (interquartile range (IQR)=147.0-253.4 µm) compared with those with VA <0.3 LogMAR (292.4 µm (IQR=240.1-347.6 µm)) at the total macula circle (P=0.004). At the foveal central subfield, the median choroidal thickness was 336.8 µm (IQR=272.3-375.4 µm) in active compared with 239.3 µm (IQR=195.3-330.9 µm) in quiescent disease (P=0.04). CONCLUSION: A disproportionately enlarged Sattler's layer may indicate a diagnosis of sarcoid-related uveitis, and choroidal thickening may be a feature of active granulomatous uveitis.

Implementation of a nurse-delivered intravitreal injection service.

PURPOSE: The purpose of this study was to introduce nurse-delivered intravitreal injections to increase medical retina treatment capacity. METHODS: Indemnity, clinical governance, training, planning, and implementation issues were addressed. The outcome measures were patient safety, patient experience, and clinic capacity. RESULTS: No serious vision-threatening complications were recorded in a consecutive series of 4000 nurse-delivered intravitreal injections. A Mann-Whitney test showed a significant increase in intravitreal injections (P=0.003) in the medical retina service after introduction of nurse-delivered intravitreal injections. The majority of patients accepted and were satisfied with a nurse-delivered intravitreal injection. DISCUSSION: Nurse-delivered intravitreal injections appear safe, acceptable to patients, and are an effective means to increase intravitreal injection capacity in medical retina clinics.

Relationship between different fluorescein and indocyanine green angiography features in multiple evanescent white dot syndrome.

OBJECTIVES: To study the relationship between different fluorescein and indocyanine green angiographic features in a case series of multiple evanescent white dot syndrome (MEWDS) and to gain insight into its pathophysiological nature. METHODS: Retrospective review of seven patients (eight eyes) with MEWDS (selected based on clinical and angiographic manifestations of the disorder) examined using slit-lamp biomicroscopy and studied using fluorescein angiography (FA) and indocyanine green angiography (ICGA). RESULTS: In the clinically affected eyes, FA early phases revealed hyperfluorescence in three eyes and a combination of hyper- and hypofluorescence in five eyes; the following relationships between FA and ICGA were found: (1) areas of early hypo- and late hyperfluorescence (staining and leakage) on FA corresponding to areas of early hypo and late hypo-,iso or hyperfluorescence on ICGA; (2) early and late hyperfluorescence (staining but very faint, if any, leakage) on FA corresponding to normal early ICGA and intermediate-late hypofluorescence; (3) areas of hypo- and hyperfluorescence on ICGA without a clear counterpart on FA. CONCLUSIONS: Angiographic features may vary in eyes with similar clinical signs of MEWDS. Such variability could reflect the different anatomical structures involved during the natural evolution of the disease. Angiographic studies suggest that both, inner and outer choroid, are involved in this disorder. The final outcome is not affected by the initial angiographic presentation.

Presumed idiopathic outer lamellar defects of the fovea and chronic solar retinopathy: an OCT and fundus autofluorescence study.

AIM: To examine fundus autofluorescence (FAF) findings in eyes with presumed idiopathic outer lamellar defects (OLD) at the fovea and to discuss their pathogenesis. METHODS: Prospective observational case series of five eyes of four patients presenting with OLD at the fovea defined as discrete lesions of 50-100 mum in size located at the level of the outer retina on biomicroscopy and imaged on optical coherence tomography (OCT) as cylindrical, well-demarcated interruption of hyper-reflective bands corresponding to the inner/outer segments junction of photoreceptors and to the complex retinal pigment epithelium-choriocapillaris; none of the enrolled patients had any positive history for direct sungazing, welding-arc or sunbed exposure, whiplash injury, ocular trauma, macular oedema/detachment or evidence of vitreomacular traction. The corresponding FAF images were evaluated. RESULTS: In eyes with OLD, the neuroretina in the foveal region appeared to be thinner than in fellow, unaffected eyes. FAF revealed well-demarcated, hypoautofluorescent areas (corresponding in location to the OLD observed clinically and on OCT), surrounded by an irregular halo of relatively increased autofluorescence in the context of the greater hypoautofluorescent macular region. CONCLUSION: Biomicroscopy, OCT and FAF findings of presumed idiopathic OLD of the fovea strongly resemble those observed in association with chronic solar retinopathy. In association with OCT, FAF might represent a useful technique with which to detect subtle solar-induced injuries of the retina.

Intravitreal triamcinolone injection for unresponsive cystoid macular oedema in probable Behçet's disease as an additional therapy.

BACKGROUND: We present the description of a successful outcome of intravitreal triamcinolone injection (IVTA) in a patient with cystoid macular oedema (CME) and severe retinal vasculitis suggestive of ocular Behçet's disease. HISTORY AND SIGNS: A healthy 32 year-old Caucasian man presented initially with right retinal vasculitis with preserved vision. Despite initial systemic treatment with high dose steroids, he developed CME with a reduction in vision. THERAPY AND OUTCOME: IVTA injection was carried out, and resulted in a good anatomic and functional response. The same picture developed in his left eye 2 months later and a similar response occurred following IVTA. Six months after the initial episode and despite treatment with cyclosporine and azathioprine, CME reoccurred in his RE inducing a decrease of his VA from 6/5 to 6/24. IVTA injection was repeated and a week later his VA increased to 6/6. At this stage patient was referred for initiation of therapy with infliximab. CONCLUSION: IVTA injection appears to be very helpful for the resolution of acute CME and for visual recovery, but we would like to emphasise that even though this approach seems to be helpful in an acute situation, the effect is short-lived and proper immunosuppression is needed for long-term control of the disease and its complications.

Treatment of ocular inflammatory conditions with loteprednol etabonate.

Ocular inflammatory diseases impose a significant medical and economic burden on society. Corticosteroids are potent anti-inflammatory agents that have been used successfully to treat ocular inflammation. Topical corticosteroids provide maximal drug delivery, and are used to reduce the signs and symptoms of intraocular and ocular surface inflammation. However, side effects associated with topical corticosteroids-including increased intraocular pressure, risk of cataract formation after long-term use, and decreased resistance to infection-are concerns. Loteprednol etabonate (LE) is an ester corticosteroid with a high therapeutic index that contains an ester, rather than a ketone, at carbon-20 of the prednisolone core structure. LE blocks the release and action of inflammatory mediators and is clinically effective in the treatment of steroid-responsive inflammatory conditions including giant papillary conjunctivitis, seasonal (intermittent) allergic conjunctivitis and uveitis. LE relieves ocular surface and lacrimal gland inflammation associated with dry eye and is used in combination with ciclosporin A as a treatment of dry eye. LE is also effective in the treatment of postoperative ocular inflammation. Because of its rapid de-esterification to inactive metabolites, LE appears to have an improved safety profile compared with ketone corticosteroids, and may be more suitable than ketone corticosteroids for the treatment for ocular inflammatory conditions in which long-term therapy is necessary. However, further comparative safety studies are needed.

Surgically induced necrotising scleritis following three-port pars plana vitrectomy without scleral buckling: a series of three cases.

AIM: To document the presentation, management, and prognosis of three cases of surgically induced necrotising scleritis (SINS) following three-port-pars plana vitrectomy (PPV) without scleral buckling. To discuss potential pathogeneses and treatments. METHODS: An interventional case series of three patients presenting to a tertiary referral unit over a 6-month period. RESULTS: All cases were male, presenting within 6 weeks of PPV, with scleral necrosis around a sclerotomy site. Wound microbiology and systemic vasculitis screens were negative. Two cases had underlying systemic disease and had undergone previous ocular surgery. The necrosis resolved in all with aggressive immunosuppression. CONCLUSIONS: SINS following PPV is a rare occurrence requiring prompt and aggressive immunosuppression. Differentiation from an infective postoperative process is essential. The pathogenesis is poorly understood but may share features with classic SINS including hypersensitivity and ischaemia.

Mitigating selection in HLA-B27 not confirmed.

The existence of a small, discrete group of HLA-B27 promoters, potentially able to mitigate the deleterious effect of this allele, has not been confirmed.

The effect of pars plana vitrectomy on cystoid macular oedema associated with chronic uveitis: a randomised, controlled pilot study.

AIM: To evaluate the efficacy of pars plana vitrectomy (PPV) in the management of chronic uveitic cystoid macular oedema (CMO). METHODS: A prospective, interventional, randomised, controlled, pilot study. 23 eyes of 23 patients with CMO secondary to chronic intermediate or posterior uveitis unresponsive to medical treatment were randomised into a surgical (group S) or medical group (group M). 12 patients in group S underwent PPV as opposed to 11 patients in group M who received systemic corticosteroid and/or immunosuppressive treatment during the study period. The primary outcome measures of the study were change in visual acuity and angiographic appearance of CMO at 6 months. RESULTS: Mean visual acuity in group S improved significantly from 1.0 (0.62) at baseline to 0.55 (0.29) at 6 months following vitrectomy (p = 0.011), with five (42%) eyes reaching vision of 20/40 or better. Conversely, mean visual acuity in group M improved only marginally by 0.03 (0.27) (p = 0.785). CMO after vitrectomy was angiographically improved in four (33%) eyes, remained unchanged in seven (58%) eyes, and deteriorated in one (8%) eye. In the medical group, fluorescein leakage decreased in one eye, did not alter in four eyes, and deteriorated in two eyes. CONCLUSION: PPV for macular oedema secondary to chronic uveitis despite angiographic improvement in only one third of the patients, seems to have a significant beneficial effect on visual function. This study provides enough evidence to justify a large scale trial which would define the role of vitrectomy in uveitic macular oedema.

Unilateral electronegative ERG of non-vascular aetiology.

BACKGROUND: Full field and pattern electroretinograms (ERG, PERG) are performed to assess generalised retinal function and macular function, respectively. An (electro) negative full field ERG usually describes an ISCEV standard maximal response in which the b-wave is smaller than a normal or minimally reduced a-wave and indicates dysfunction that is post-phototransduction. The most common cause of a unilateral negative ERG is central retinal artery occlusion (CRAO) or birdshot chorioretinopathy (BCR). This study examines the clinical and electrophysiological features of patients with unilateral negative ERG who do not have CRAO or BCR. METHODS: 12 patients were ascertained with a unilateral negative ERG in whom a vascular aetiology and BCR were excluded. Most presented with symptoms of central retinal dysfunction. In 11 of the 12 patients additional long duration photopic stimuli were used to test cone system ON and OFF responses. RESULTS: All 12 patients had unilateral electronegative bright flash full field ERGs indicating total or relative preservation of rod photoreceptor function, but dysfunction post-phototransduction. Seven of these patients had non-specific inflammatory changes in the eye with the negative ERG. Six patients, including five with inflammatory signs, had involvement of the cone ON response with complete preservation of cone OFF responses. A further three patients showed evidence of cone ON response abnormality with less severe OFF response involvement. CONCLUSION: The ERGs in this heterogeneous group of patients predominantly showed post-phototransduction involvement of the ON pathways. Sparing of the cone OFF response was often observed. The majority of patients had signs of previous inflammation and it is speculated that these highly unusual unilateral changes may be mediated via an autoimmune mechanism.

The use of low dose methotrexate in children with chronic anterior and intermediate uveitis.

AIM: To assess the efficacy of low dose methotrexate (MTX) therapy for children with chronic anterior and intermediate uveitis. METHODS: A retrospective case review of 10 children who received MTX for chronic uveitis at a tertiary referral centre was performed. The following data were recorded for each patient: age, sex, race, duration of uveitis, primary diagnosis, anatomical localisation of uveitis, corticosteroid therapy, dose range of MTX, duration of MTX therapy, and side effects of MTX therapy. Several clinical parameters were evaluated to study the effect of MTX. These included visual acuity, anterior chamber inflammation, and topical and oral corticosteroid requirement. RESULTS: After MTX VA of 6/6 or better was present in 100% right eyes and 80% left eyes (p = 0.055 and p = 0.016, respectively). Anterior chamber inflammation decreased in 60% of children after MTX (p = 0.0168). The requirement of topical steroid decreased from a mean of 5.6 times a day before MTX to 1.5 times a day after MTX (p = 0.005). The dose of oral steroid decreased from a mean of 18 mg per day to 2.85 mg per day (p = 0.012). The most common adverse effect was nausea (20%). No patient required discontinuation of MTX because of side effects. CONCLUSION: MTX is effective and safe for chronic anterior and intermediate uveitis in children. An increase awareness of its efficacy is required among paediatricians and ophthalmologists to prevent sight threatening complication of chronic uveitis and its treatment with long term use of steroids.

The visual field in toxoplasmic retinochoroiditis.

AIMS: To document loss of central field in patients with scars from toxoplasmic retinochoroiditis close to the disc after resolution of disease. METHODS: Patients with a clinical diagnosis of toxoplasmic retinochoroiditis were enrolled from four centres. Automated central visual field testing was performed when their disease had settled and retinal photographs of the lesions were taken. The type of central field defect (whether absolute or relative) and whether it broke out to the periphery were correlated with the size of the retinochoroidal scar and its proximity to the optic nerve head. RESULTS: 69 eyes were enrolled; 16 (26%) were discarded because of poor field performance. Of the 53 remaining eyes, 31 showed absolute defects and 20 relative defects. Scars within one disc diameter of the disc were more likely to be associated with absolute defects breaking out to the periphery. CONCLUSION: The scarring induced by toxoplasmic retinochoroiditis is associated with considerable field loss when it occurs close to the optic nerve head. Current treatment is unlikely to ameliorate this situation. The degree of visual field loss should be an outcome measure for future trials of the efficacy of treatment trials for the disease.

Electrophysiological characterisation and monitoring in the management of birdshot chorioretinopathy.

AIMS: To characterise patients with birdshot chorioretinopathy (BCR) clinically and electrophysiologically in order to monitor changes in retinal function before and after treatment with corticosteroids and/or immunosuppression. METHODS: 18 patients with BCR were characterised clinically and electrophysiologically. Serial studies were performed on 14 patients in order to monitor changes in retinal function before and after treatment with corticosteroids and/or immunosuppression. RESULTS: Most patients presented with characteristic subretinal pale spots, were HLA-A29 positive, and had diverse signs of ocular inflammation. Various electrophysiological abnormalities were present. Moderately severe bilateral pattern electroretinogram (PERG) abnormalities at presentation were common, reflecting macular dysfunction. Cone mediated 30 Hz flicker electroretinograms (ERGs) were consistently delayed before treatment, and were the most sensitive parameter of retinal dysfunction. Scotopic maximal ERG responses were abnormal in 13 patients; 10 had an electronegative maximal ERG or a reduced b:a ratio in one or both eyes. Single flash photopic ERGs were less often and less severely affected. Photopic ON and OFF ERG responses often revealed predominant ON response b-wave abnormalities with relative OFF response preservation. ERGs improved in treated cases, sometimes preceding clinical signs of recovery. Pattern ERG improvements occurred, possibly reflecting the resolution of macular oedema. CONCLUSIONS: The ERG data confirm that BCR frequently affects inner retinal function of cone and rod systems. Clinical features were not reliable indicators of functional deterioration or recovery. Objective electrophysiological assessment of retinal function demonstrated improvement following treatment and provides a reliable method of monitoring treatment efficacy, enabling management decisions to be taken with greater confidence and allowing early initiation or modification of treatment.