Intraocular solitary extramedullary plasmacytoma presenting as unilateral anterior and intermediate uveitis preceded by refractory glaucoma.

BACKGROUND: Solitary extramedullary plasmacytoma (SEP) is a localised proliferation of monoclonal plasma cells involving soft tissue with no or minimal bone marrow involvement and no other systemic evidence of multiple myeloma. Intraocular involvement is exceedingly rare. CASE PRESENTATION: We report a 78-year-old man who was referred with glaucoma in the right eye. He subsequently developed anterior chamber (AC) inflammation and refractory glaucoma then dense vitritis. A vitrectomy was performed with the biopsy revealing numerous plasma cells with atypical findings. In conjunction with the flow cytometry results, and a systemic work up excluding multiple myeloma, a diagnosis of SEP was made. The patient was treated with ocular external beam radiotherapy with resolution of the intraocular inflammation and control of the intraocular pressure. He remains well with no local recurrence and no development of multiple myeloma over a follow up period of 2.5 years. CONCLUSIONS: This is the first case report of SEP presenting as intraocular inflammation without a uveal tract mass.

Outcome measures in juvenile X-linked retinoschisis: A systematic review.

X-linked retinoschisis (XLRS) is a leading cause of hereditary juvenile macular degeneration in males resulting in significant vision impairment. Outcome measures to monitor disease progression or therapeutic interventions have evolved with technology. A systematic review was undertaken to evaluate outcome measures for XLRS. Inclusion criteria were all publications examining outcome measures for natural history studies or following an interventional approach for patients with XLRS. Studies which did not present follow-up data were excluded. We searched medical databases including CENTRAL, Ovid Medline, pre-Medline and ahead of Print up to February 2019. Two authors independently assessed the risk of bias. Twelve studies meet the inclusion criteria with four prospective and eight retrospective case series. Five series were natural history observational studies and seven were interventional series using either topical or systemic carbonic anhydrase inhibitors. Visual acuity (VA) declined very slowly in the natural history studies equivalent to 0.22-0.5 letters per year. Five of the six interventional studies showed an improvement in VA and four a reduction in spectral domain optical coherence tomography (SD-OCT) parameters for central macular thickness (CMT). The full-field electroretinogram identified the 30-Hz latency as a further parameter to monitor function. VA was the measure most likely to show a statistically significant outcome. How functionally meaningful this is, requires further evaluation. CMT SD-OCT outcomes are variable depending on cystic changes. More refined measures are required to better correlate structure with function.

TATTOO-ASSOCIATED UVEITIS WITH CHOROIDAL GRANULOMA: A RARE PRESENTATION OF SYSTEMIC SARCOIDOSIS.

PURPOSE: The authors report a rare case of systemic sarcoidosis manifesting as bilateral granulomatous panuveitis and multiple tattoo granulomas. METHODS: Case report. RESULTS: A 30-year-old man presented with headaches, ocular pain, photophobia, and blurred vision. He was found to have significant skin inflammation, predominantly involving areas of tattoos. Fundoscopic examination revealed bilateral granulomatous panuveitis with small focal choroidal lesions. Skin biopsy demonstrated granulomatous inflammation of the dermis with tattoo pigment evident in numerous histiocytes. He was diagnosed with systemic sarcoidosis. CONCLUSION: The authors report another rare case of tattoo-associated uveitis, in which inflammation is limited to tattooed skin and the uvea. This uncommon presentation may prove informative in elucidating the pathogenesis of systemic sarcoidosis.

Valacyclovir in the treatment of acute retinal necrosis.

BACKGROUND: To report the outcome of oral valacyclovir as the sole antiviral therapy for patients with acute retinal necrosis (ARN). METHODS: This study reports a retrospective, interventional case series of nine consecutive patients with ten eyes with newly diagnosed ARN treated with oral valacyclovir as the sole antiviral agent. Eight patients received oral valacyclovir 2 g tid (Valtrex, GlaxoSmithKline) and one patient with impaired renal function received oral 1 g tid. The main outcome measures were response to treatment, time to initial response to treatment, time to complete resolution of retinitis, best corrected visual acuity (BCVA) at final follow-up, retinal detachment and development of recurrent or second eye disease. RESULTS: Retinitis resolved in ten of ten (100%) affected eyes. The median time to initial detectable response was seven days and the median time to complete resolution was 21 days. A final BCVA of 20/40 or better was achieved in 6/10 (60%) of eyes. 3/10 eyes (30%) developed a retinal detachment. No patients developed either disease reactivation or second eye involvement over the course of the study (mean follow up 31 weeks, range 7 to 104 weeks). CONCLUSIONS: Treatment with oral valacyclovir as the sole antiviral therapy resulted in complete resolution of retinitis. Final BCVA and retinal detachment rate were comparable with previously reported outcomes for intravenous acyclovir.

Choroidal imaging in inherited retinal disease using the technique of enhanced depth imaging optical coherence tomography.

PURPOSE: The aim of this study is to image and describe the in vivo choroidal changes in various retinal dystrophies using the technique of enhanced depth imaging (EDI) optical coherence tomography (OCT) and to correlate these findings with the clinical appearance. Associations between choroidal change and genotype, visual acuity and results of retinal electrophysiology are also explored. DESIGN: Retrospective observational case series. METHODS: Twenty patients attending the medical retina clinics at Moorfields Eye Hospital underwent EDI OCT choroidal scans as part of the scanning protocol when they underwent OCT imaging with the Spectralis HRA and OCT. The choroidal images were obtained by moving the Spectralis camera close enough to obtain an inverted image of the retina. The scans were read by two experienced OCT readers assessing the choroidal thickness as well as the choroidal contour for focal areas of choroidal thinning corresponding to the areas of RPE/outer retinal atrophy. The spectrum of patients included those with Stargardt macular dystrophy, macular dystrophies secondary to known mutations such as peripherin/RDS, uncharacterised macular dystrophies, Best disease, bifocal chorioretinal atrophy, Bietti crystalline retinal dystrophy and choroideraemia. RESULTS: The choroidal appearance was symmetrical in all patients who had both eyes scanned. Ten patients showed no choroidal thinning, five had focal mild to moderate choroidal thinning, three had focal severe choroidal thinning, and two patients had diffuse severe choroidal thinning. There was no association between choroidal thinning and visual acuity [Fisher's exact test, p = 0.350 (right eye), p = 1.000 (left eye)], or extent of retinal dysfunction on electrophysiology (Fisher's exact test, p = 1.000). CONCLUSION: Enhanced depth imaging using spectral domain OCT can be used to identify choroidal changes in inherited retinal disease. The pattern of choroidal change correlates well with the clinical appearance. It appears that the extent and pattern of choroidal thinning is dependent on the stage of the disease in some cases, and in others the causative gene defect.

Antiviral selection in the management of acute retinal necrosis.

There is no consensus on the optimal antiviral regimen in the management of acute retinal necrosis, a disease caused by herpetic viruses with devastating consequences for the eye. The current gold standard is based on retrospective case series. Because the incidence of disease is low, few well-designed, randomized trials have evaluated treatment dosage and duration. Newer oral antiviral agents are emerging as alternatives to high-dose intravenous acyclovir, avoiding the need for inpatient intravenous treatment. Drug resistance is uncommon but may also be difficult to identify. Antiviral drugs have few side effects, but special attention needs to be paid to patients who have underlying renal disease, are pregnant or are immunocompromised.

Cataract surgery in high-risk age-related macular degeneration: a randomized controlled trial.

BACKGROUND: To investigate if cataract surgery causes progression, from high-risk early age-related macular degeneration (AMD) to choroidal neovascularization (CNV), in the postoperative period. METHODS: Randomized controlled trial. Patients, with visually significant cataract and fundus features of early AMD at high risk of progression to CNV, were randomized into two groups and were evaluated at baseline and 6 months. The study patients (n = 27) underwent immediate cataract surgery. The control group (n = 29) comprised patients who had cataract surgery deferred until after the 6-month visit. Assessment included visual acuity, quality of life (QoL) and fundus fluorescein angiography (FFA). RESULTS: Of 68 eligible eyes, 60 participated and 56 completed the study. Three referred eyes (3.2%) were ineligible on the basis of a pre-existing, unsuspected occult CNV that was detected by baseline FFA. All three cases had end-stage exudative AMD in the fellow eye. Of the study eyes in the immediate surgery arm (n = 27), one (3.7%) developed CNV compared with none (0/29) in the deferred arm (chi(2); P = 1.0) at 6 months. In the operated group, there was a 2.8-line improvement in logMAR visual acuity and 2.1-fold average gain in QoL at 6 months. CONCLUSIONS: No increased short-term risk of progression of AMD to CNV in high-risk fundi following uncomplicated phacoemulsification surgery was found. A low threshold for performing preoperative imaging in patients with AMD, especially in those with exudative AMD in the fellow eye, to exclude undetected CNV is recommended. Provided there is no CNV, there are distinct benefits of cataract surgery in people with early AMD.

Intraocular inflammation: its causes and investigations.

Intraocular inflammation, or uveitis, incorporates a diverse group of infectious and immune-mediated disorders. In addition, some conditions masquerade as uveitis. However, classifying uveitis according to anatomic location in adult and pediatric populations, and appreciating the effect of immune status and regional differences, refines the list of potential causes. In this way, a select few investigations can be performed, rather than a nondirected battery of tests.

Cardiovascular risk factors and age-related macular degeneration: the Los Angeles Latino Eye Study.

PURPOSE: To assess the association of cardiovascular risk factors and ocular perfusion pressure with early and advanced age-related macular degeneration (AMD) in Latinos. DESIGN: Population-based, cross-sectional study. METHODS: Data were collected from a population-based sample of self-identified adult Latinos using standardized protocols for assessing blood pressure and intraocular pressure (IOP) measurement and stereoscopic macular photography. Hypertension was defined as either a history of hypertension or systolic blood pressure (SBP) > 140 mm Hg +/- diastolic blood pressure (DBP) > or = 85 mm Hg. Ocular perfusion pressure (OPP) was defined as the difference between mean arterial blood pressure and IOP. AMD was diagnosed from photographic grading by masked trained graders. Logistic regression was used to assess associations. RESULTS: Gradable retinal photographs were available in 5,875 participants. After adjusting for age, gender, and cigarette smoking, higher DBP and uncontrolled diastolic hypertension were associated with exudative AMD (odds ratio [OR], 1.8; 95% confidence interval [CI], 1.1 to 2.8; and OR, 3.3; CI, 1.2 to 9.3, respectively). Higher OPP was associated with a decreased risk of geographic atrophy (GA) (OR, 0.4 per 10 mm Hg; CI, 0.3 to 0.5). Low pulse pressure was associated with a lower risk of exudative AMD (OR, 0.2; CI, 0.1 to 0.6). Obesity was associated with increased retinal pigment (OR, 1.6; CI, 1.0 to 2.3). CONCLUSIONS: These data suggest that in Latinos cardiovascular risk factors may play a role in advanced AMD. Given that Latinos have a high prevalence of cardiovascular risk factors, an intervention aimed at reducing these risk factors may also have a beneficial impact on the risk of having early and advanced AMD.

Impact of cataract surgery on quality of life in patients with early age-related macular degeneration.

PURPOSE: To investigate if cataract surgery improves overall and specific areas of quality of life (QoL) in patients with early age-related macular degeneration (AMD) using the impact of vision impairment (IVI) questionnaire. METHODS: Patients with visually significant cataract and early AMD, who were being considered for cataract surgery in the study eye, were recruited. Eligible patients were randomized to either "early surgery" or "standard surgery" (standard cataract surgery waiting time of 6 months) groups. The IVI, sociodemographic, and clinical data were collected. Rasch analysis was used to estimate QoL person measures at baseline and follow-up. The data were analyzed using repeated measures ANOVA. Effect sizes were calculated using Cohen's d coefficient. RESULTS: Fifty six patients (mean age = 78.5 years and visual acuity = 6/15) had one eye randomly allocated to either the early surgery (n = 29) or standard surgery (n = 27) groups. At follow-up, significant interaction effects were found for the overall IVI score [F(1,54) = 17.7; p < 0.001], the emotional well-being [F(1,54) = 13.4; p = 0.001], mobility and independence [F(1,54) = 13.4; p = 0.001], and reading and accessing information subscales [F(1,54) = 13.1; p = 0.001]. The standard surgery group systematically recorded worse scores at 6 months on all QoL measures whereas the early surgery group recorded significant gains (p < 0.001; Cohen's d = 0.66 to 0.91) on all of them. Visual acuity in the study eye significantly improved in the early surgery group only (Cohen's d = 1.1; p < 0.05) and improvement in log MAR lines read was identified as the single independent predictor of enhanced QoL explaining between 26 and 34% of the variance in the IVI scores. CONCLUSIONS: Cataract surgery is justified in patients with early AMD. It brings significant improvements in visual acuity, aspects of daily living, and overall QoL.

Management of submacular hemorrhage with intravitreal injection of tissue plasminogen activator and expansile gas.

PURPOSE: To evaluate the clinical outcome of intravitreal tissue plasminogen activator (tPA) and expansile gas injection as a minimally invasive treatment for submacular hemorrhage (SMH). METHODS: This study was a retrospective clinical case series examining 104 eyes that received an intravitreal injection of 30-100 mcg of tPA and expansile gas (SF6 or C3F8) for SMH. The main outcomes evaluated were visual acuities (VA), anatomic displacement of submacular blood, and surgical complications. RESULTS: : A total of 85, 77, and 81 eyes were available at 1 week, 3 months, and 12 months follow up, respectively. Postoperatively, > or = 2 Snellen lines improvement were achieved in 43/85 eyes (51%) at 1 week, 49/77 eyes (63%) at 3 months, and 52/81 eyes (64%) at 12 months. Postoperative VA improvement was significantly associated with preoperative VA, submacular blood displacement, and the underlying cause of SMH. Diagnostic postoperative angiogram and clinical examination were possible at 8.2 +/- 7.4 weeks and 9.5 +/- 7.4 weeks, respectively. The observed complications included breakthrough vitreous hemorrhage in 8 eyes (8%) and retinal detachment in 3 eyes (3%). CONCLUSIONS: In this retrospective series, intravitreal injection of tPA and expansile gas was shown to be a safe and effective technique that can improve VA in most eyes with SMH and assist in the diagnosis of the underlying cause.

Complicated hyphaema: think sickle.

Two cases of complicated hyphaema associated with sickle cell trait are presented. The pathophysiology, diagnosis and management of raised intraocular pressure in sickle cell trait are discussed.

New treatments in age-related macular degeneration.

Age-related macular degeneration (AMD) is the leading cause of legal blindness in individuals 50 years and older in the developed world. Choroidal neovascularization (CNV) in exudative AMD is responsible for the majority of severe vision loss. Until recently, laser photocoagulation was the only well-established and widely accepted treatment for CNV. However, it is beneficial only for a small subset of patients, has a high rate of CNV persistence and recurrence and results in iatrogenic, collateral damage to the overlying retina. These issues make it difficult to recommend in the case of subfoveal lesions. Consequently, numerous experimental therapeutic interventions are under investigation with the common objective of destroying the CNV but leaving the foveal neurosensory retina intact. Treatment modalities can be grouped into five major categories: photodynamic therapy; radiotherapy; transpupillary thermotherapy; anti-angiogenic and angiostatic agents; and surgical intervention. The present review aims to explain the rationale behind these new treatments, analyse the evidence for their safety and efficacy, determine their stage of development and indicate in which patients they are potentially useful.