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.

Retinal angiomatous proliferation with chorioretinal anastomosis in childhood Coats disease: a reappraisal of macular fibrosis using multimodal imaging.

PURPOSE: To describe the structural characteristics of retinal angiomatous proliferation and chorioretinal anastomosis in childhood Coats disease and redefine the previously described macular fibrosis. METHODS: Prospective observational case series of consecutive patients with Coats disease examined over a 1-year study period. Multimodal imaging, including color fundus photography, wide-field fluorescein angiography, and spectral domain optical coherence tomography, was used to identify the features of macular retinal angiomatous proliferation and chorioretinal anastomosis. RESULTS: Retinal angiomatous proliferation and chorioretinal anastomosis were present in 5 of 21 patients with Coats disease (24%). The lesions appeared as well demarcated, nodular retinal pigment epithelial detachments surrounded by exudate, with retinal vessels continuous with the underlying choroidal neovascularization. CONCLUSION: Retinal angiomatous proliferation and chorioretinal anastomoses are features observed in a number of children (24% in the present series) with Coats disease and macular involvement. This lesion represents a distinct macular variant of Coats disease that underlies at least a proportion (all in the present series) of the previously described "macular fibrosis" and "subretinal mounds."

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.

25-gauge pars plana lensectomy with vitrectomy.

BACKGROUND AND OBJECTIVE: To describe a technique of 25-gauge pars plana lensectomy with primary posterior capsulotomy and sparing of the anterior lens capsule that is suitable for all lens densities. PATIENTS AND METHODS: The authors describe a technique they routinely employ for pars plana vitrectomy using primarily a 25-gauge, three-port approach with intraoperative lens density assessment and possible fragmatome use for dense lenses. An analysis of the ability to achieve surgical goals is provided. RESULTS: Surgical goals were achieved in all 68 cases performed during an 18-month period. Anterior lens capsule was consistently spared, and 25-gauge fluidics functioned well even in the presence of a fragmatome and single 20-gauge sclerotomy. CONCLUSION: 25-gauge pars plana lensectomy with vitrectomy with and without fragmatome incision is an efficacious technique for lensectomy.

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.

Anatomical and visual outcomes after two-port pars plana vitrectomy reoperation under silicone oil for epimacular membrane or recurrent retinal detachment.

PURPOSE: To describe the anatomical and visual outcomes in a series of patients undergoing two-port pars plana vitrectomy reoperation under silicone oil for recurrent retinal detachment (RD) due to proliferative vitreoretinopathy or epimacular membrane (EMM) after RD repair. METHODS: This study is a prospective, consecutive, interventional case series of patients presenting with recurrent RD or EMM under silicone oil. Two-port 25-gauge pars plana vitrectomy reoperation without an infusion port was performed in all cases. RESULTS: Thirty-nine patients were included. Reoperation pathology included recurrent RD with proliferative vitreoretinopathy (n = 33) and EMM alone (n = 6). The mean number of previous retinal surgeries was 2.4 ± 1.1 (range, 1-5). The mean overall follow-up was 24 ± 3.7 months. The mean visual acuity change from baseline at final follow-up was an improvement of 0.74 ± 0.63. Macular reattachment was achieved in 29 of 33 patients with RD, and EMMs were successfully removed in all patients. CONCLUSION: Two-port pars plana vitrectomy reoperation is an efficacious method for repair of consecutive RD due to proliferative vitreoretinopathy or EMM in patients with previous RD repair with silicone oil. Significant visual improvement with a low complication rate may be achieved in patients with advanced proliferative vitreoretinopathy or EMM under silicone oil.

Comparison of morphologic features of macular proliferative vitreoretinopathy and idiopathic epimacular membrane.

BACKGROUND: To compare the spectral-domain optical coherence tomography morphologic features and visual characteristics of a series of patients with epimacular membrane with and without a history of retinal breaks. METHODS: Prospective, comparative case series of patients with epimacular membrane. All patients were evaluated with spectral-domain optical coherence tomography and detailed peripheral retinal examination. Symptomatic patients were treated with pars plana vitrectomy and epimacular membrane removal based on standard visual criteria. RESULTS: Macular proliferative vitreoretinopathy was present in 21 of 50 patients (42%). Approximately 18 of 21 patients had a previous retinal break, 5 of which were untreated before the initial examination. No retinal breaks were observed in the idiopathic group. Macular proliferative vitreoretinopathy was highly associated with a history of retinal breaks (P < 0.001). Presenting visual acuity was significantly worse (mean, 0.86 ± 0.44) for macular proliferative vitreoretinopathy than for the idiopathic group (mean, 0.44 ± 0.36). CONCLUSION: Epimacular membrane occurring in the context of previous retinal breaks or macular proliferative vitreoretinopathy has a characteristic morphologic feature in spectral-domain optical coherence tomography. Surgical removal typically results in significant visual improvement.

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.

Current management of Coats disease.

Since its original description in 1908, Coats disease has been recognized as an idiopathic cause of severe vision loss with a remarkable diversity in clinical presentation and morphology. Key clinical and imaging variables are helpful in differentiating Coats disease from life-threatening malignancies, and proper management revolves around a thorough knowledge of the differential diagnosis. Despite significant advancement in scientific understanding of the disease process and clinical spectrum, the underlying etiology remains obscure, and both primary and secondary forms are recognized. With the development of anti-VEGF therapy, vitreoretinal specialists have a new, effective adjunct to the clinical management of exudates, macular edema, and serous retinal detachment. We highlight the history, diagnostic challenges, evolving clinical spectrum, and current management of Coats disease.

Submacular choroidal varix simulating chorioretinal folds with metamorphopsia.

A novel case description of an isolated unilateral submacular choroidal varix simulating chorioretinal folds and inducing metamorphopsia in a 74-year-old phakic Caucasian man. Posterior segment examination revealed focal choroidal elevations corresponding to a deep, large choroidal vessel in the inferotemporal subparafoveal region. Fundus photography demonstrated a pigment epithelial detachment-like elevation inferior to the fovea, continuous with the course of a large choroidal vessel. Angiographic, indocyanine green (ICG), and spectral-domain optical coherence tomography (SD-OCT) imaging revealed an isolated unilateral submacular choroidal varix with direct connection to an inferior vortex vein. The diagnosis of submacular choroidal varix should be considered when the presence of metamorphopsia and corresponding choroidal elevations that remain unchanged through gaze direction or contact lens ophthalmoscopy occur. If present, angiographic, ICG, and SD-OCT imaging are recommended for proper evaluation of submacular choroidal varix that may enhance the characteristics of this lesion.

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.

Arcuate retinotomy for the repair of large macular holes.

BACKGROUND AND OBJECTIVE: To present a case series to elucidate a novel technique that involves the creation of an arcuate retinotomy in the treatment of large macular holes after failed primary repair. PATIENTS AND METHODS: retrospective chart review. Six eyes (six patients) with large macular holes, all of which had failed primary repair, underwent 25 gauge pars plana vitrectomy revision coupled with full thickness arcuate retinotomy temporal to the macular hole and fluid-gas exchange. The main outcome measure was anatomic macular hole closure based on optical coherence tomography (OCT), with visual acuity and visual field evaluation as secondary outcome measures. RESULTS: Five of the six patients (83%) had successful hole closure with three of the six patients (50%) exhibiting improvement in visual acuity. CONCLUSION: Arcuate retinotomy is a new approach that may aide in the repair of large macular holes not otherwise amenable to closure with traditional techniques.

25-gauge pars plana vitrectomy with medium-term postoperative perfluoro-n-octane tamponade for inferior retinal detachment.

BACKGROUND AND OBJECTIVE: To describe a series of patients with inferior retinal detachments managed with primary 25-gauge pars plana vitrectomy (PPV), 2 to 3 weeks of postoperative perfluoro-n-octane (MT-PFO) tamponade, and upright positioning followed by secondary PPV and PFO removal. PATIENTS AND METHODS: Interventional case series of 157 patients with inferior retinal detachments with and without proliferative vitreoretinopathy. Eyes were treated with 25-gauge PPV, endophotocoagulation, and MT-PFO tamponade. Patients underwent postoperative upright positioning followed by repeat PPV and PFO removal in a planned, staged procedure. RESULTS: Mean follow-up was 32 ± 4.6 months. Successful reattachment was achieved in 87.5% of 159 eyes. Main initial postoperative complications were persistent intraocular pressure (IOP) elevation in 34% (n = 54), excessive inflammation in 27% (n = 43), and PFO in the anterior chamber in 21% (n = 34). Additional complications occurring after PFO removal included cataract surgery in 16% (n = 22) of initially phakic eyes, redetachment in 13% (n = 21), and need for filtering surgery in 6% (n = 10). Logistic regression analysis revealed macula status (P = .003) and progression to filtering surgery (P = .001) as significant factors predicting visual outcome at 1-year follow-up. CONCLUSION: MT-PFO tamponade and upright head positioning may be efficacious for inferior retinal detachment repair in patients unable to assume face-down postoperative positioning. Anatomic and visual outcomes are similar to previously described reattachment procedures. A characteristic granulomatous inflammatory reaction presents in some patients, but does not appear to leave long-term visual or anatomic sequelae. Persistent IOP elevation and progression to filtering surgery may occur in a small percentage of patients and portends a worse visual outcome.

Incidence, morphology, and classification of epimacular membrane rip.

PURPOSE: To describe the incidence, associated factors, morphology, and visual characteristics of a series of patients with tears within an epimacular membrane (EMM). METHODS: Consecutive, prospective, observational case series of patients with EMM rip. Patients were evaluated with spectral domain optical coherence tomography, infrared photography, and fundus autofluorescence. Symptomatic patients were treated with pars plana vitrectomy and EMM removal. Follow-up imaging evaluation was performed at 3-month intervals. RESULTS: A total of 761 patients presented with EMM. Thirty-four eyes of 34 patients (4.5%) developed EMM rip. Frequent associated features included history of cataract extraction, diabetes mellitus, and retinal tear treated with photocoagulation. Morphologic characteristics included a scrolled torn edge of internal limiting membrane/EMM. Rip patterns include horseshoe shapes and patchy confluent striae and appeared as dark lines on infrared or fundus autofluorescence imaging. The EMM rips were classified as Type 1 if the rip occurred within 500 µm of the foveola on spectral domain optical coherence tomography and Type 2 if extrafoveal. Foveal involvement was associated with worse presenting vision (P = 0.002) and visual outcome after EMM removal (P = 0.012). Previous retinal tear was associated with worse visual outcome. CONCLUSION: The EMM rip occurs in a small but significant minority of patients with EMM. Foveal involvement leads to increased visual deficit and may indicate a worse visual outcome at presentation and after intervention. Fundus autofluorescence and infrared imaging are useful in identifying EMM rip patterns.