Temporal Macular Atrophy as a Predictor of Neovascularization in Sickle Cell Retinopathy.

BACKGROUND AND OBJECTIVE: To evaluate the association between temporal macular atrophy and the presence of neovascularization in eyes with sickle cell disease (SCD). PATIENTS AND METHODS: Retrospective, case-controlled study identifying 64 eyes from 38 consecutive patients with SCD. Dilated funduscopic examination and wide-field fluorescein angiography were used to identify the Goldberg stage of proliferative sickle cell retinopathy. Spectral-domain optical coherence tomography images were analyzed for the presence of temporal macular atrophy. The association between temporal macular atrophy and neovascularization was then evaluated. RESULTS: Temporal macular atrophy had a sensitivity of 27%, a specificity of 67%, a positive predictive value of 83%, and a negative predictive value of 13% for identifying neovascularization. CONCLUSION: Although the presence of temporal macular atrophy is not sensitive enough to be used as a screening test, if seen in a patient with SCD, the physician should be alerted to the strong possibility that peripheral neovascularization may be present.

25-gauge pars plana vitrectomy with medium-term postoperative perfluoro-n-octane for the repair of giant retinal tears.

PURPOSE: The purpose of this study was to describe the treatment of giant retinal tears (GRTs) with 25-gauge pars plana vitrectomy (PPV) and medium-term postoperative perfluoro-n-octane (MT-PFO). METHODS: The study was a retrospective interventional case series of consecutive patients with GRTs treated with 25-gauge PPV and postoperative MT-PFO for a period of 2-3 weeks. A second, staged procedure was performed in all patients for PFO removal. RESULTS: Twenty-three eyes of 22 patients were studied, with a mean follow-up of 33.04 ± 19.74 months. Successful reattachment was achieved in 91.3 % of eyes (21/23) after MT-PFO. Retinal re-detachment occurred in five eyes, which was caused by proliferative vitreoretinopathy. Additional complications included cataract progression (n = 10), foreign body response (30.4 %, 7/23), and transient intraocular pressure (IOP) elevation (8/23, 34.8 %). Transient IOP elevation was associated with worse visual outcome (p = 0.01). CONCLUSIONS: MT-PFO was found to be an effective and safe technique for operative management of GRTs. In the majority of patients, retinas remained attached without further surgical intervention. Cataract progression, intraocular inflammation, and associated increased intraocular pressure are potential complications of MT-PFO.

Circulating Autoantibodies in Age-Related Macular Degeneration Recognize Human Macular Tissue Antigens Implicated in Autophagy, Immunomodulation, and Protection from Oxidative Stress and Apoptosis.

BACKGROUND: We investigated sera from elderly subjects with and without age-related macular degeneration (AMD) for presence of autoantibodies (AAbs) against human macular antigens and characterized their identity. METHODS: Sera were collected from participants in the Age-Related Maculopathy Ancillary (ARMA) Study, a cross-sectional investigation ancillary to the Health ABC Study, enriched with participants from the general population. The resulting sample (mean age: 79.2±3.9 years old) included subjects with early to advanced AMD (n = 131) and controls (n = 231). Sera were tested by Western blots for immunoreactive bands against human donor macular tissue homogenates. Immunoreactive bands were identified and graded, and odds ratios (OR) calculated. Based on these findings, sera were immunoprecipitated, and subjected to 2D gel electrophoresis (GE). Liquid chromatography-tandem mass spectrometry (LC-MS/MS) was used to identify the targets recognized by circulating AAbs seen on 2D-GE, followed by ELISAs with recombinant proteins to confirm LC-MS/MS results, and quantify autoreactivities. RESULTS: In AMD, 11 immunoreactive bands were significantly more frequent and 13 were significantly stronger than in controls. Nine of the more frequent bands also showed stronger reactivity. OR estimates ranged between 4.06 and 1.93, and all clearly excluded the null value. Following immunoprecipitation, 2D-GE and LC-MS/MS, five of the possible autoreactivity targets were conclusively identified: two members of the heat shock protein 70 (HSP70) family, HSPA8 and HSPA9; another member of the HSP family, HSPB4, also known as alpha-crystallin A chain (CRYAA); Annexin A5 (ANXA5); and Protein S100-A9, also known as calgranulin B that, when complexed with S100A8, forms calprotectin. ELISA testing with recombinant proteins confirmed, on average, significantly higher reactivities against all targets in AMD samples compared to controls. CONCLUSIONS: Consistent with other evidence supporting the role of inflammation and the immune system in AMD pathogenesis, AAbs were identified in AMD sera, including early-stage disease. Identified targets may be mechanistically linked to AMD pathogenesis because the identified proteins are implicated in autophagy, immunomodulation, and protection from oxidative stress and apoptosis. In particular, a role in autophagy activation is shared by all five autoantigens, raising the possibility that the detected AAbs may play a role in AMD via autophagy compromise and downstream activation of the inflammasome. Thus, we propose that the detected AAbs provide further insight into AMD pathogenesis and have the potential to contribute to disease biogenesis and progression.

Ocular histoplasmosis syndrome.

Ocular histoplasmosis syndrome (OHS) is a chorioretinal disorder with a distinct fundus appearance that is commonly found in regions endemic for Histoplasma capsulatum. Choroidal neovascularization (CNV) secondary to OHS is considered one of the principal causes of central vision loss among young adults in endemic areas. Although there is no consensus regarding its pathogenesis, evidence points to Histoplasma capsulatum as the most probable etiology. Once considered an intractable hemorrhagic maculopathy, CNVs are now treatable. Extrafoveal CNVs are successfully treated with laser photocoagulation. Subfoveal and juxtafoveal CNVs are managed with anti-vascular endothelial growth factor therapy, photodynamic therapy, or a combination of both. Modern imaging technologies such as spectral-domain optical coherence tomography have improved our diagnostic abilities, making it easier to monitor disease activity and CNV regression. We review the epidemiology, pathogenesis, clinical manifestations, differential diagnosis, and current treatment of this disease.

Proliferative ischemic retinopathy after arteriovenous malformation embolization in a child with hereditary hemorrhagic telangiectasia.

PURPOSE: To describe a case of hereditary hemorrhagic telangiectasia, presenting with multiple branch retinal artery occlusions, retinal ischemia, neovascularization, and vitreous hemorrhage after cerebral arteriovenous malformation embolization. METHODS: The authors report a 7-year-old patient with decreased vision in his left eye after embolization of a pineal arteriovenous malformation secondary to hereditary hemorrhagic telangiectasia. Ophthalmic evaluation, fundus photography, fluorescein angiography, spectral domain optical coherence tomography, electroretinogram, examination under anesthesia, and pars plana vitrectomy (PPV) were performed. RESULTS: Fundus examination of the left eye revealed extensive posterior segment ischemia, vascular tortuosity, and vitreous hemorrhage. Fluorescein angiography was remarkable for partial obstruction of retinal arteries, midperipheral nonperfusion, and associated leakage from multiple areas of neovascularization. Spectral domain optical coherence tomography was normal. Electroretinogram demonstrated decreased b-wave amplitude. The patient was subsequently treated with 25-gauge pars plana vitrectomy, panretinal endophotocoagulation, and intravitreal bevacizumab. Five weeks after surgery, best-corrected visual acuity had improved to 20/40, and examination showed resolution of vitreous hemorrhage and neovascularization. CONCLUSION: Retinal vascular abnormalities, posterior segment ischemia, and vitreous hemorrhage suggested a combination of retinal involvement of hereditary hemorrhagic telangiectasia complicated by nontarget embolization.

Outcomes of treatment of pediatric choroidal neovascularization with intravitreal antiangiogenic agents: the results of the KKESH International Collaborative Retina Study Group.

PURPOSE: To evaluate safety and clinical results of intravitreal antiangiogenic agents for choroidal neovascularization in pediatric patients. METHODS: Retrospective, multicenter, interventional case series. A total of 45 eyes of 39 pediatric patients with choroidal neovascularization of various etiologies were treated with intravitreal injection of antiangiogenic agents (1.25 mg per 0.05 mL of bevacizumab or 0.5 mg per 0.05 mL of ranibizumab). RESULTS: There were 24 girls and 15 boys with group median age of 13 years (range, 3-17 years). Mean follow-up period was 12.8 months (range, 3-60 months). Median visual acuity in terms of logarithm of the minimum angle of resolution at presentation and last follow-up was 0.87 and 0.7, respectively (P = 0.0003). Mean and median number of injections received over the follow-up period was 2.2 and 1, respectively. At the last follow-up, 22 eyes (48%) gained more than 3 lines of vision and 27 eyes (60%) had final visual acuity 20/50 or better. Nine eyes (20%) did not improve and had severe vision loss (20/200 or worse). CONCLUSION: Intravitreal antiangiogenic therapy for choroidal neovascularization in pediatric patients seems temporarily safe and effective in majority of affected eyes. Because of the rarity and character of this condition, it is unlikely that any clinical trials will soon take place to study this or other treatment option.

Intraoperative grasp site correlation with morphologic changes in retinal nerve fiber layer after internal limiting membrane peeling.

BACKGROUND AND OBJECTIVE: Recent reports have demonstrated inner retinal changes after internal limiting membrane (ILM) peeling, but the mechanism responsible for these findings remains poorly understood. The purpose of this report is to establish a correlation between ILM removal and postoperative morphologic changes. PATIENTS AND METHODS: Prospective, observational case series of eight consecutive patients undergoing pars plana vitrectomy with ILM peeling for macular hole or epimacular membrane. Intraoperatively, all grasp sites were recorded and subsequently superimposed on postoperative infrared and spectral-domain optical coherence tomography (SD-OCT) images. Repeat examination and imaging were performed at regular postoperative intervals. RESULTS: Infrared fundus photography revealed well-defined, hyporeflective arcuate striations in all patients during the early postoperative period. These defects followed the course of axonal pathways from the grasp site to the optic nerve. SD-OCT images on all patients revealed early focal nerve fiber layer swelling directly corresponding to grasp sites, with eventual atrophy. CONCLUSION: A dynamic process takes place within the inner retina following surgical removal of ILM. Inadvertent surgical trauma induced by ILM forceps may be the mechanism responsible for nerve fiber layer morphologic changes after ILM peeling.

Subacute exogenous Rhizoctonia solani endophthalmitis following cataract extraction with intraocular lens implantation.

UNLABELLED: Seven weeks after uneventful cataract extraction with intraocular lens (IOL) implantation, a 64-year-old man presented to his cataract surgeon with decreased vision and photophobia. The subacute presentation with anterior uveitis prompted initial therapy with topical and periocular glucocorticoids. One month later, the patient presented to the vitreoretinal service with counting fingers visual acuity, prominent anterior chamber reaction, a 2.5 mm hypopyon, and inflammatory deposits over the IOL. Cultures grew Rhizoctonia solani. The inflammation was successfully treated with pars plana vitrectomy, IOL explantation, and intravitreal voriconazole. Rhizoctonia should be included in the differential diagnosis of subacute exogenous endophthalmitis, especially in the context of fibrillar white IOL plaques. FINANCIAL DISCLOSURE: No author has a financial or proprietary interest in any material or method mentioned.