Retinopathy Associated With Coxsackie B Virus Infection.

This case report describes a diagnosis of unilateral retinopathy secondary to coxsackie B virus in a male patient aged 41 years who presented with a central scotoma and blistering rash of the hands, feet, and mouth for 4 days.

A Novel Surgical Technique for Transscleral Gore-Tex Suture-Assisted Rescue of Dislocated CZ70BD Intraocular Lenses Using 27-Gauge Instrumentation.

PURPOSE: To report a novel technique for refixation of dislocated CZ70BD intraocular lens (IOL). METHODS: Vitrectomy trocars are placed along the horizontal meridian 5 mm apart. A CV-8 Gore-Tex suture is introduced through a bare sclerotomy into the midvitreous cavity. Under chandelier illumination, a 27 G broad platform forceps is threaded through the eyelet of the dislocated CZ70BD IOL. Using another pair of intraocular forceps, the free intraocular end of the Gore-Tex suture is fed to the broad platform forceps and externalized, thus repositioning the IOL. Particular attention is drawn to pass the suture in an over and under configuration to avoid IOL tilt. An identical procedure is repeated for the other eyelet if the IOL is completely dislocated. The 23 gauge or 25 gauge instruments should not be used for this technique because they do not fit loosely through the eyelets of the IOL. RESULTS: Three eyes were successfully operated on using this technique with at least 6 months of follow-up. There was significant improvement in best-corrected visual acuity after the operation. Postoperative IOL centration and alignment were satisfactory. CONCLUSION: The described surgical technique is effective for transscleral Gore-Tex-assisted refixation of dislocated CZ70BD IOL.

The retinal deep capillary plexus as a venous outflow system; insights from Sturge Weber Syndrome.

PURPOSE: To report on the venous abnormalities of a patient with Sturge-Weber syndrome (SWS). METHOD: Case report. PATIENT: A 29-year-old woman with a history of SWS since infancy was referred for evaluation of possible diffuse choroidal hemangioma. Multimodal imaging, including ultra-widefield fluorescein, indocyanine green, and optical coherence tomography-angiography (OCTA) were performed. RESULTS: Dilated fundus examination was remarkable for increased cupping of the optic disc in the right eye, venous tortuosity, and marked dilation of the choroidal vessels. Ultra-widefield fluorescein angiography confirmed marked venous tortuosity and dilation, as well as anastomoses of the retinal veins ipsilateral to the port wine stain. Indocyanine green angiography revealed marked engorgement of the vortex veins and choroidal vasculature. OCTA revealed dilated vascular channels in the deep capillary plexus (DCP) that were directly anastomosing to the superficial capillary plexus, but not the intermediate capillary plexus. Engorgement of the ampullae of the DCP vortex system was also observed. The normal contralateral eye was used as comparison for all imaging studies. CONCLUSION: These findings support the notion of generalized venous hypertension state in adult eyes with SWS and corroborate prior evidence that the deep capillary plexus acts as a venous outflow system.

27-GAUGE PARS PLANA/PLICATA VITRECTOMY FOR PEDIATRIC VITREORETINAL SURGERY.

PURPOSE: To report on the feasibility of 27-gauge (G) vitrectomy for pediatric patients. METHODS: This study is an international, multicenter, retrospective, interventional case series. Participants were patients 17 years or younger who underwent 27-G vitrectomy for various indications. RESULTS: The records of 56 eyes from 47 patients were reviewed. Mean age was 5.7 ± 5.2 years. Diagnoses included retinopathy of prematurity (Stages 3 with vitreous hemorrhage, 4A, 4B, and 5), Terson's syndrome, traumatic macular hole, posterior capsular opacification, endophthalmitis, and others. Instruments used were the 27-G infusion, 27-G vitreous cutter, 27-G light pipe, and 27-G internal limiting membrane forceps. Instrument bending was noted in one (1.8%) case. There were no cases with intraoperative complications, infusion issues, or postoperative endophthalmitis. There were 67/145 (46%) sclerotomies that required suturing, of which most (51/145) were sutured out of precaution. There were four cases (7.1%) that required conversion to a larger gauge and three cases (5.3%) that developed postoperative hypotony. Mean visual acuity improved from logarithm of the minimum angle of resolution 1.32 (20/420) to 0.72 (20/105), after a mean follow-up of 125.1 days (P = 0.01). Anatomic success was achieved in 96.4% of eyes after a single surgery. CONCLUSION: Twenty-seven-gauge vitrectomy was safe and feasible in selected pediatric vitreoretinopathies. Further studies are warranted to examine indications and outcomes.

The Prevalence of Retinal Disease and Associated CNS Disease in Young Patients with Incontinentia Pigmenti.

PURPOSE: To evaluate the prevalence of retinal disease on fluorescein angiography (FA) in patients with incontinentia pigmenti (IP) and to compare the severity of retinal disease in those with and without known central nervous system (CNS) disease. DESIGN: Multi-institutional consecutive retrospective case series. SUBJECTS: New patients with a diagnosis of IP were seen at the Casey Eye Institute at the Oregon Health and Science University (OHSU), Moran Eye Center, University of Utah, or Bascom Palmer Eye Institute, University of Miami from December 2011 to September 2018. METHODS: Detailed ophthalmoscopic examination and FA were recommended for all new patients and performed on every patient who had parental consent. Ophthalmoscopic findings and FA images were graded for severity by 2 masked graders on a 3-point scale: 0 = no disease, 1 = vascular abnormalities without leakage, 2 = leakage or neovascularization, and 3 = retinal detachment. The presence of known CNS disease was documented. Additional cases were obtained from a pediatric retina listserv for examples of phenotypic variation. MAIN OUTCOME MEASURES: The proportion of eyes noted to have disease on ophthalmoscopy compared with FA and the severity of retinal disease in those with and without known CNS disease. RESULTS: Retinal pathology was detected in 18 of 35 patients (51%) by indirect ophthalmoscopy and 26 of 35 patients (74%) by FA (P = 0.048) in a predominantly pediatric population (median age, 9 months). Ten patients (29%) had known CNS disease at the time of the eye examination. A Wilcoxon rank-sum test indicated that the retinal severity scores for patients with CNS disease (median, 2) were significantly higher than the retinal severity scores for patients without CNS disease (median, 1), z = -2.12, P = 0.034. CONCLUSIONS: Retinal disease is present in the majority of patients with IP, and ophthalmoscopic examination is less sensitive than FA for detection of disease. There may be a correlation between the severity of retinal and CNS disease.

Membranous Encapsulation of an Akreos IOL Following Scleral Fixation masquerading as opacification.

PURPOSE: To report of case of membranous encapsulation of a scleral-fixated Akreos intraocular lens (IOL) that was successfully treated with vitrectomy and membranectomy without IOL exchange or removal. METHODS: Case report. PATIENT: A 58-year-old woman with a history of bilateral chronic anterior and intermediate uveitis underwent scleral fixation of Akreos IOL for dislocated 3-piece IOL in the vitreous cavity. She presented one year later with progressively worsening vision (decreased to 20/200) and was found to have a complete encapsulation of the IOL. RESULTS: The patient underwent successful vitrectomy and membranectomy with return of vision to 20/25 within three months of surgery. CONCLUSION: There are numerous case reports of opacification after scleral fixation of Akreos IOL. To our knowledge, this is the first report of complete encapsulation of the IOL with a membrane of this type and character. The etiology for this patient's presentation is unclear, but was presumed to be related to her history of chronic intraocular inflammation.

Role of Optical Coherence Tomography Imaging in Predicting Progression of Age-Related Macular Disease: A Survey.

In developed countries, age-related macular degeneration (AMD), a retinal disease, is the main cause of vision loss in the elderly. Optical Coherence Tomography (OCT) is currently the gold standard for assessing individuals for initial AMD diagnosis. In this paper, we look at how OCT imaging can be used to diagnose AMD. Our main aim is to examine and compare automated computer-aided diagnostic (CAD) systems for diagnosing and grading of AMD. We provide a brief summary, outlining the main aspects of performance assessment and providing a basis for current research in AMD diagnosis. As a result, the only viable alternative is to prevent AMD and stop both this devastating eye condition and unwanted visual impairment. On the other hand, the grading of AMD is very important in order to detect early AMD and prevent patients from reaching advanced AMD disease. In light of this, we explore the remaining issues with automated systems for AMD detection based on OCT imaging, as well as potential directions for diagnosis and monitoring systems based on OCT imaging and telemedicine applications.

The Expanded Clinical Spectrum of Coxsackie Retinopathy.

The authors present two cases of coxsackie retinopathy that presented with the classic findings of acute outer retinal disruption that evolved to an outer retinal scar. In these two cases, the classic lesions displayed an extramacular distribution. The authors provide detailed multimodal imaging in both cases highlighting the nature of the lesions. It is important to consider coxsackie virus infection in the differential diagnosis of extramacular lesions with outer retinal disruption. The term "unilateral acute idiopathic maculopathy" should be replaced with "coxsackie retinopathy" to better align with the expanded clinical spectrum and the known etiology of this disorder. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:529-532.].

CRB1 related retinal degeneration with novel mutation.

PURPOSE: To describe novel and previously unreported genetic mutations in the CRB1 gene in a patient with retinal dystrophy. To increase the genotype-phenotype understanding of CRB1-related retinal degenerative diseases and describe patients' response to therapy. OBSERVATIONS: Patient was evaluated for progressive loss of central and peripheral vision. Fundus photography, fundus autofluorescence (FAF), fluorescein angiography (FA), and ocular-coherence tomography (OCT) were used in the evaluation. Genetic screening was performed to explore underlying mutations. Genetics revealed a previously reported, pathogenic variant in the CRB1 gene (c.2842+5G > A), and a novel mutation (c.4014T > A) whose clinical significance is uncertain due to the absence of conclusive evidence. This case is phenotypically unique in that CME was refractory to therapy, while CME in CRB1 related maculopathy typically responds well to treatment. CONCLUSIONS AND IMPORTANCE: This study adds a breadth of phenotypic understanding to genetic analysis in CRB1 related retinal degenerative conditions. The newly described CRB1 variant mutation c.4014T > A may portend a poor prognosis for CME responsiveness to therapy. Genetic testing in an otherwise unexplained CME event may be useful to identify underlying CRB1 variants and reveal genotype-phenotype correlations, which may alter the treatment plan and prognosis.

Degenerative Peripheral Retinoschisis: Observations From Ultra-Widefield Fundus Imaging.

BACKGROUND AND OBJECTIVE: To describe the ultra-widefield (UWF) imaging characteristics of patients with degenerative peripheral retinoschisis (DPR) using Optomap technology. PATIENTS AND METHODS: In this multicenter, retrospective, noncomparative, consecutive case series, eligible patients underwent detailed retinal examination including indirect ophthalmoscopy. UWF fundus imaging, including color fundus photography, autofluorescence, and angiography, was performed using standardized protocols and findings were recorded and reviewed and analyzed. RESULTS: A total of 35 patients (58 eyes) with DPR were identified who underwent 55 sessions of UWF imaging. Mean age was 65 years, and the inferotemporal quadrant was most commonly affected (74% of eyes). Of these patients, 31 underwent fluorescein angiography and 90% of these studies illustrated abnormalities in the area affected by the schisis. The most common finding was retinal vascular leakage originating from the deep capillary plexus observed in 29 eyes (93.5%). CONCLUSIONS: UWF imaging enables a more detailed identification of the clinical features associated with DPR and provides simple, practical, and noninvasive tools to monitor progression of disease. The breadth of retinal vascular complications identified with fluorescein angiography may suggest an important vascular component associated with the pathogenesis of this entity. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:557-564.].

Retinal Vascular Abnormalities in Phakomatosis Pigmentovascularis.

PURPOSE: To describe the spectrum of retinal vascular abnormalities in patients with phakomatosis pigmentovascularis (PPV). DESIGN: Multicenter, retrospective, noncomparative, consecutive case series. METHODS: Eligible patients underwent detailed retinal examination including indirect ophthalmoscopy. Ultra-widefield fundus imaging, including color fundus photography and angiography, was performed using standardized protocols, and findings were recorded and reviewed and analyzed. PARTICIPANTS: Three patients with a clinical diagnosis of PPV are presented. RESULTS: Evaluation of all patients (n = 6 eyes of 3 patients) with widefield fluorescein angiography showed several retinal vascular abnormalities, including peripheral retinal nonperfusion (n = 3 eyes), peripheral vascular leakage (n = 3 eyes), aberrant retinal vessels (n = 1 eyes), vascular tortuosity (n = 1 eyes), and disruption of the foveal avascular zone including fovea plana (n = 3 eyes). In addition, 2 eyes demonstrated peripheral retinal vascular straightening and leakage similar to the features of familial exudative vitreoretinopathy. One of the patients was a carrier of a somatic GNA11 R183C pathogenic variant that has been associated with PPV. CONCLUSIONS: Fluorescein angiography, especially with widefield capability, reveals numerous retinal vascular abnormalities in patients with PPV. Considering the association of GNA11 pathogenic variants with PPV and allied disorders, these observations may suggest a role of guanine-binding proteins (G-proteins) in retinal vascular development. Supplemental material available at www.ophthalmologyretina.org.

Spontaneous Hyaloidal Contraction and Complex Retinal Detachment in a Patient With Von Hippel-Lindau Syndrome.

The authors present an interventional case report of a patient with von Hippel-Lindau (VHL) syndrome who developed simultaneous exudative and combined tractional, rhegmatogenous, and exudative retinal detachment (RD) in the right and left eyes, respectively, following uneventful cryotherapy application of retinal capillary hemangioblastoma (RCH). After pars plana vitrectomy combined with encircling scleral buckling with radial element placement and silicone oil injection, complete retinal reattachment was achieved. The exudative RD of the fellow eye was managed initially with intense topical corticosteroid treatment and subsequent placement of a radial scleral buckle due to the presence of clinically apparent traction. Both eyes remained stable after successful surgical interventions, and long-term follow-up revealed no evidence of recurrent disease. The authors conclude that rapid worsening of vitreoretinal traction and exudation are complications that can occur after treatment of RCH in patients with VHL syndrome leading to complex retinal detachments. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:238-241.].

SURGICAL MANAGEMENT OF TRACTIONAL RETINOSCHISIS ASSOCIATED WITH VITREOUS HEMORRHAGE IN RETINOPATHY OF PREMATURITY.

PURPOSE: The tractional retinoschisis is a poorly understood, rare, and likely underappreciated entity in retinopathy of prematurity. The purpose of this article is to describe clinical findings and surgical management of tractional retinoschisis in retinopathy of prematurity, masquerading as Stage 4 retinopathy of prematurity retinal detachment. METHODS: A retrospective review of a single case with literature review and case discussion. RESULTS: In this report, we describe a child with retinopathy of prematurity and tractional schisis, who initially presented with vitreous hemorrhage and was effectively managed by vitrectomy and inner wall retinectomy. At 5 months after vitrectomy, the child demonstrated complete collapse of the retinoschisis with intact posterior pole and brisk light perception. CONCLUSION: Vitrectomy with or without inner wall retinectomy is effective in the management of tractional retinoschisis.

Outcomes of Anti-Vascular Endothelial Growth Factor Treatment for Choroidal Neovascularization in Fellow Eyes of Previously Treated Patients With Neovascular Age-Related Macular Degeneration.

Importance: Neovascular age-related macular degeneration (nvAMD) is a leading cause of vision loss. The optimal screening protocol to detect choroidal neovascularization (CNV) in fellow eyes of patients undergoing treatment for unilateral CNV has not been determined. Objective: To compare the visual outcomes of eyes with established, active nvAMD in index eyes with outcomes of fellow eyes that subsequently developed CNV during the management protocol. Design, Setting, and Participants: In this retrospective single-center case series conducted at a private vitreoretinal practice, data were collected for all patients treated for bilateral nvAMD between October 1, 2015, and October 1, 2016, for whom we could determine the date of index eye and fellow eye conversion to nvAMD (n = 1600). Per institutional protocol, patients were screened for new CNV in the fellow eye at every office visit. Patients were excluded if they had a condition that could result in marked asymmetric vision loss. Exposures: Development of nvAMD. Main Outcomes and Measures: Visual acuity (VA) at the time of diagnosis of nvAMD and at equivalent time points following conversion to nvAMD for both index eyes and fellow eyes. Results: A total of 264 patients met the inclusion criteria; 197 (74.6%) were women and 253 (95.8%) were white, and the mean (SD) age was 79.1 (8.2) years at time of index eye conversion to nvAMD and 80.6 (8.2) years at time of fellow eye conversion to nvAMD. Fellow eyes presented with better VA (mean VA, 20/50 [0.40 logMAR]) compared with index eyes (mean VA, 20/90 [0.67 logMAR]) at the time of conversion (difference, 14 letters [0.27 logMAR]; 95% CI, 10-17 [0.20-0.34]; P < .001). Index eyes did not achieve the same level of VA as fellow eyes after an equivalent postconversion follow-up of approximately 20 months (mean VA: index eye; 20/70 [0.56 logMAR]; fellow eye, 20/50 [0.40 logMAR]; difference, 8 letters [0.15 logMAR]; 95% CI, 4-11 [0.08-0.22]; P < .001). No difference was detected between the mean number of anti-vascular endothelial growth factor injections received by fellow eyes and index eyes (9.7 vs 10.0 injections, respectively). Conclusions and Relevance: This retrospective study suggests that fellow eyes of previously treated patients with nvAMD may achieve better VA than their index eye counterparts after an equivalent amount of follow-up. This may be because the CNV was detected and treated earlier and at a better level of VA, although it is unknown whether the frequent office visits, VA measurements, or optical coherence tomography testing was responsible for the detection at a better level of VA.

Embolization of a cavernous carotid fistula through the vein of Labbé: a new alternative transvenous access route.

Endovascular treatment of carotid cavernous fistulas (CCFs) via a transvenous approach is standard, but in rare cases this approach is challenging due to absence or thrombosis of the commonly used venous routes. A 61-year-old woman presented with a symptomatic CCF with all but one of the venous access routes to the CCF thrombosed, leaving an engorged superficial middle cerebral vein (SMCV) as the only venous outflow from the cavernous sinus. Access to the CCF was made possible after careful navigation of the sigmoid sinus, the vein of Labbé and the SMCV, bypassing the need for surgical access to the SMCV or for a direct transorbital puncture. The CCF was completely occluded by coiling and Onyx embolization. The patient made an uneventful recovery, with resolution of her symptoms. To the best of our knowledge, this access route has not been previously reported in the treatment of CCFs.

The Role of Microglia and Peripheral Monocytes in Retinal Damage after Corneal Chemical Injury.

Eyes that have experienced alkali burn to the surface are excessively susceptible to subsequent severe glaucoma and retinal ganglion cell loss, despite maximal efforts to prevent or slow down the disease. Recently, we have shown, in mice and rabbits, that such retinal damage is neither mediated by the alkali itself reaching the retina nor by intraocular pressure elevation. Rather, it is caused by the up-regulation of tumor necrosis factor-α (TNF-α), which rapidly diffuses posteriorly, causing retinal ganglion cell apoptosis and CD45+ cell activation. Herein, we investigated the involvement of peripheral blood monocytes and microglia in retinal damage. Using CX3CR1+/EGFP::CCR2+/RFP reporter mice and bone marrow chimeras, we show that peripheral CX3CR1+CD45hiCD11b+MHC-II+ monocytes infiltrate into the retina from the optic nerve at 24 hours after the burn and release further TNF-α. A secondary source of peripheral monocyte response originates from a rare population of patrolling myeloid CCR2+ cells of the retina that differentiate into CX3CR1+ macrophages within hours after the injury. As a result, CX3CR1+CD45loCD11b+ microglia become reactive at 7 days, causing further TNF-α release. Prompt TNF-α inhibition after corneal burn suppresses monocyte infiltration and microglia activation, and protects the retina. This study may prove relevant to other injuries of the central nervous system.

Vancomycin-Associated Hemorrhagic Occlusive Retinal Vasculitis: A Clinical-Pathophysiological Analysis.

PURPOSE: To derive novel insights into the pathophysiology of vancomycin-related hemorrhagic occlusive retinal vasculopathy (HORV) through a careful clinicopathologic correlation. METHODS: We retrospectively reviewed the clinical and pathologic course of 2 consecutive patients who developed HORV. The clinical history, multimodal imaging, ultrasound biomicroscopy (UBM), and intraoperative and histologic findings are reported. RESULTS: Both patients presented with decreased vision and eye pain within 1 week following otherwise uncomplicated cataract extraction and were diagnosed with HORV after endophthalmitis was ruled out. Both patients presented with significant ocular discomfort that progressively worsened, and both experienced a dismal visual outcome despite early aggressive medical and surgical therapy. One patient requested enucleation for a blind and painful eye. Upon histologic examination of this eye, the iris and ciliary body appeared to be infarcted with separation of the iris and ciliary epithelia from their adjacent stromal components. These findings were corroborated by UBM of the second patient. Histologic examination of the posterior segment demonstrated severe hemorrhagic necrosis of the neurosensory retina and an occlusive nonarteritic vasculopathy of the retina and choroid. The choroid was thickened by prominent nongranulomatous chronic inflammation accompanied by a glomeruloid proliferation of small vessels. The inflammatory infiltrate was almost exclusively confined to the choroid and consisted of predominantly T cells. There was conspicuous absence of inflammatory cells in the retina and no histologic evidence of leukocytoclastic vasculitis. CONCLUSIONS: HORV is a rare condition that can lead to profound vision loss. Significant ocular pain can be a presenting sign of HORV in cases with severe iris and ciliary body ischemia. Although it has been suggested that HORV is a form of leukocytoclastic retinal vasculitis, the histologic findings herein indicate that the pathophysiology is more complex. It is grounded in a necrotizing retinal vasculopathy in the absence of retinal vasculitis, chronic nongranulomatous choroiditis, and an unusual glomeruloid proliferation of endothelial cells in the choroid and elsewhere in the eye.

PROGRESSIVE RETINAL DETACHMENT IN INFANTS WITH RETINOPATHY OF PREMATURITY TREATED WITH INTRAVITREAL BEVACIZUMAB OR RANIBIZUMAB.

PURPOSE: Fibrovascular contraction and tractional retinal detachment (TRD) are recognized complications associated with the use of anti-vascular endothelial growth factor agents in vasoproliferative vitreoretinopathies. The authors characterize TRDs that developed after intravitreal bevacizumab or ranibizumab therapy for vascularly active retinopathy of prematurity. METHODS: This is an international, multicenter, interventional, retrospective, case series. Thirty-five eyes from 23 infants were included. Inclusion required anti-vascular endothelial growth factor treatment of Type 1 retinopathy of prematurity with progression to TRD. RESULTS: Mean gestational age was 26 ± 2 weeks, and mean birth weight was 873 ± 341 g. Mean postmenstrual age on the day of injection was 35 ± 2 weeks. Retinal detachment was noted a mean of 70 days (median, 34; range, 4-335) after injection. Eleven percent detached within 1 week, 23% within 2 weeks, and 49% within 4 weeks. The highest stage of retinopathy of prematurity noted was 4A in 29%, 4B in 37%, and 5 in 34% of eyes. Time to RD negatively correlated with postmenstrual age at the time of injection (Rho = -0.54; P < 0.01). Three TRD configurations were observed: 1) conventional peripheral elevated ridge or volcano-shaped Stage 5 detachment, 2) midperipheral detachment with tight circumferential vectors, and 3) very posterior detachment with prepapillary contraction. Full or partial reattachment was achieved with surgical intervention in 86% of eyes. CONCLUSION: Progressive atypical TRD may occur after anti-vascular endothelial growth factor injections for retinopathy of prematurity. The configuration of the detachment varies with the extent of primary retinal vascularization present at the time of treatment.