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Autosomal dominant neovascular inflammatory vitreoretinopathy (ADNIV) is a rare genetic (CAPN5) autoimmune condition typically diagnosed in adulthood and characterized by a triad of inflammation, retinal degeneration, and neovascularization. We report novel multimodal imaging findings in children and young adults with ADNIV, and early treatment response to short-duration local and systemic corticosteroids.

The article, titled "A Sleeper Wakes: The Cook Creek Ridge Fire," is an excerpt from the book Rampage by Firestorm. It describes a harrowing wildfire battle near Garden Valley in the Boise National Forest. The author, Robert W. Sisk, recounts his experience as a helitack crew pilot during a lightning-sparked forest fire that quickly escalated into a dangerous conflagration. The fire trapped a group of firefighters, who were eventually rescued by helicopter. The fire burned for a week and consumed over 700 acres of forest before being brought under control by a team of firefighters and aerial support. [Extracted from the article]

BACKGROUND AND OBJECTIVE:To compare duration and efficacy of the dexamethasone (DEX) intravitreal implant in vitrectomized (pars plana vitrectomy [PPV] group) and non-vitrectomized eyes (control group) with macular edema related to central retinal vein occlusion (CRVO).PATIENTS AND METHODS:Eyes that received the DEX implant for CRVO related to macular edema were included in a retrospective chart review. Outcomes measured were best-corrected visual acuity (BCVA) and central macular thickness (CMT)RESULTS:Fifteen eyes were included in the study. Eight of 15 eyes had prior vitrectomy. Mean BCVA was 20/160 in both groups. Baseline mean CMT was 550 microns (PPV group) and 556 microns (control group, Fifteen eyes were included in the study. Eight of 15 eyes had prior vitrectomy. Mean BCVA was 20/160 in both groups. Baseline mean CMT was 550 microns (PPV group) and 556 microns (control group, P= .70), and improved to 307.5 microns (PPV group) and 316.3 microns (control group) (P= .90) after implant. The BCVA was 20/125 (PPV group) and 20/200 (control group) (P= .60). Time to next procedure was similar in both groups.CONCLUSIONS:This retrospective study shows similar CMT reductions with DEX implant treatment for CRVO-related macular edema in vitrectomized and non-vitrectomized eyes.

Faricimab is a bispecific antibody that acts through dual inhibition of both angiopoietin-2 and vascular endothelial growth factor A. We report primary results of two phase 3 trials evaluating intravitreal faricimab with extension up to every 16 weeks for neovascular age-related macular degeneration (nAMD).

This retrospective, comparative case series assesses the 10-year outcomes of intravitreal anti-VEGF injections with and without verteporfin photodynamic therapy for the treatment of choroidal neovascularization secondary to presumed ocular histoplasmosis syndrome.

To explore the effect of patients’ age, baseline visual acuity (VA), and intraoperative foveal detachment on outcomes of subretinal voretigene neparvovec-rzyl (Luxturna) therapy and to assess patients’ perceptions of the treatment effect.

Serial anterior chamber paracentesis for sustained intraocular pressure elevation after intravitreal injections may be complicated by hypotony, choroidal detachment, intraocular lens dislocation, vitreous prolapse, and trauma to the penetrated ocular tissues. Complications may be limited by preventing hypotony and alternating wound location.

This retrospective, interventional, sequential, noncomparative case series evaluated surgical outcomes of eyes that underwent same-day combined phacoemulsification surgery for cataract with multifocal intraocular lens implantation and pars plana vitrectomy for symptomatic vitreous opacities.

Terson syndrome typically presents with bilateral hemorrhagic retinopathy associated with acute intracranial bleeding. The authors present a case of neonatal hemispheric ischemic stroke with vasogenic edema and increased intracranial pressure creating a unilateral Terson-like syndrome. Magnetic resonance imaging indicated congenital occlusion of the left internal carotid artery, among other vascular abnormalities. Chronic submacular, peripheral subretinal, and vitreous hemorrhage were observed, suggesting a multilaminar hemorrhagic process resembling Terson syndrome without frank intracranial hemorrhage. The patient underwent successful lens-sparing vitrectomy of the left eye. A unilateral Terson-like syndrome can result from severe cerebral edema following neonatal stroke in the setting of multiple congenital cerebrovascular abnormalities.[[Ophthalmic Surg Lasers Imaging Retina.2020;51:592–595.]

Purpose: Pediatric macular holes occur often related to trauma and rarely require surgical intervention. The purpose of the present study was to evaluate outcomes of microincisional vitrectomy surgery.Methods: A multicenter retrospective consecutive case series was conducted of pediatric patients undergoing surgery for macular hole repair.Results: A total of 31 eyes from 8 centers were included in this study. The mechanism of macular hole development was blunt trauma in 30 eyes (97%) and industrial laser in 1 eye (3%). The rate of anatomic closure after primary vitrectomy was 81% (25 eyes); the final anatomic closure rate after a secondary vitrectomy was 94% (29 eyes). There was a statistically significant improvement in visual acuity from initial presentation (20/164) to final follow-up (20/100) (P= .009).Conclusions: Microincisional vitrectomy surgery for pediatric macular holes results in substantial anatomic success and modest improvement in visual acuity.

BACKGROUND AND OBJECTIVE:Intravitreal triamcinolone acetonide (IVTA) used as a surgical adjuvant for pars plana vitrectomy (PPV) can stain cortical vitreous, control postoperative inflammation, and reduce retinal edema. Sterile endophthalmitis uncommonly complicates office-based IVTA injection. The authors report a new complication of IVTA depot injection at the end of PPV.PATIENTS AND METHODS:Retrospective records review of all patients treated at the Cincinnati Eye Institute with PPV between January 1, 2011, and December 31, 2017, who developed a triad of sterile endophthalmitis, atrophic retinal breaks under the depot IVTA in the inferior retina, and rhegmatogenous retinal detachment (RRD). Eyes with flap tears, solely superior breaks, or stretch holes from proliferative vitreoretinopathy causing RRD were excluded.RESULTS:Eight eyes of eight patients (four males and four females; mean age: 73.7 years) who received 4 mg or 8 mg IVTA depot at the end of PPV surgery presented at a mean of 23.5 days following PPV with RRD (one macula-involving, seven macula-sparing), requiring treatment with PPV in six eyes and laser retinopexy alone in two eyes. Seven eyes that underwent membrane peeling (MP) received IVT to prevent cystoid macular edema (CME) and one eye with prior MP was treated for CME. Mean vitreous inflammation was 2+ cell at 1 week postoperatively. Two patients had documented sterile endophthalmitis within the first week requiring vitreous cultures and antibiotics injections. Visual acuity (VA) for eyes requiring PPV for RD repair declined from 20/90 preoperatively to 20/212 at 6 months postoperatively. VA for eyes amenable to laser alone improved from 20/53 to 20/35. All eyes remained attached, with the exception of one patient who refused further treatment after developing recurrent detachment from PVR.CONCLUSIONS:Particle-induced sterile endophthalmitis from IVTA depot at the end of PPV surgery resulted in atrophic inferior retinal breaks and RRD as a newly described entity coined “erosive retinopathy.” All retinal detachment surgeries required silicone oil tamponade. Poor visual outcome at 6 months was common for eyes requiring PPV for RD repair. The authors recommend avoiding IVTA depot injection at the end of PPV.[[Ophthalmic Surg Lasers Imaging Retina.2019;50:613–619.]

BACKGROUND AND OBJECTIVE:To describe a novel, simple technique for surgically draining a bullous serous pigment epithelial detachment (PED).PATIENTS AND METHODS:Pars plana vitrectomy was performed with confirmed elevation of the hyaloid face. Proportional diathermy allowed stepwise entry into the PED superotemporally through an initially small, needle-point focus while providing control of any potential bleeding. Thick fluid was aspirated with a soft-tipped cannula, fluid-air exchange was performed, and intravitreal bevacizumab was injected before removing the cannulas.RESULTS:The PED was successfully completely drained intraoperatively and remained flat at 1 week postoperatively. However, the draining site ultimately closed, and continued exudation from choroidal neovascularization led to recurrent PED and eventual nonhemorrhagic retinal pigment epithelial tear despite aggressive treatment with aflibercept and photodynamic therapy. The early visual acuity benefit may relate to resolution of hyperopic shift.CONCLUSION:Serous PED can be surgically reduced without hemorrhagic complications, but long-term success depends upon control of the underlying choroidal neovascularization.[[Ophthalmic Surg Lasers Imaging Retina. 2019;50:510–513.]

BackgroundUsher syndrome (USH) is a neurosensory disorder characterised by deafness, variable vestibular areflexia and vision loss. The aim of the study was to identify the genetic defect in a Pakistani family (PKDF1051) segregating USH.MethodsGenome-wide linkage analysis was performed by using an Illumina linkage array followed by Sanger and exome sequencing. Heterologous cells and mouse organ of Corti explant-based transfection assays were used for functional evaluations. Detailed clinical evaluations were performed to characterise the USH phenotype.ResultsThrough homozygosity mapping, we genetically linked the USH phenotype segregating in family PKDF1051 to markers on chromosome 1p36.32-p36.22. The locus was designated USH1M. Using a combination of Sanger sequencing and exome sequencing, we identified a novel homozygous 18 base pair inframe deletion in ESPN.Variants of ESPN, encoding the actin-bundling protein espin, have been previously associated with deafness and vestibular areflexia in humans with no apparent visual deficits. Our functional studies in heterologous cells and in mouse organ of Corti explant cultures revealed that the six deleted residues in affected individuals of family PKDF1051 are essential for the actin bundling function of espin demonstrated by ultracentrifugation actin binding and bundling assays. Funduscopic examination of the affected individuals of family PKDF1051 revealed irregular retinal contour, temporal flecks and disc pallor in both eyes. ERG revealed diminished rod photoreceptor function among affected individuals.ConclusionOur study uncovers an additional USH gene, assigns the USH1 phenotype to a variant of ESPNand provides a 12th molecular component to the USH proteome.

BACKGROUND AND OBJECTIVE:To describe a new technique for closing challenging macular holes (MHs).PATIENTS AND METHODS:The technique involves vitrectomy with internal limiting membrane (ILM) peeling, isolating the macula under perfluorocarbon liquid, alternating scraping of the retina toward the MH, aspirating fluid from the MH until closure is achieved intraoperatively, and sealing the MH with an inverted ILM flap or autologous ILM free flap. Gas or oil tamponade is used to prevent rehydration of the MH.RESULTS:Covering the MH with ILM scaffolding by the inverted flap technique or autologous ILM free flap promoted closure of large MHs by photoreceptor layer migration rather than gliosis. Iatrogenic macular trauma from manipulation produced funduscopic and optical coherence tomography changes but did not preclude significant vision improvement.CONCLUSIONS:ILM scaffolding over the surgically reduced MH enhanced surgical closure by photoreceptor migration. Although anatomic success resulted in improvement in visual acuity, further study is required regarding long-term outcomes.[[Ophthalmic Surg Lasers Imaging Retina. 2019;50:450–452.]

The authors report a case of inadvertent sub retinal cannulation during a suture trabeculotomy surgery that resulted in serous macular detachment, which resolved spontaneously. The inadvertent complication demonstrated a potential pathway for the administration of therapeutics to the subretinal space.

Classify the appearance and quantify the growth rate of chorioretinal atrophy in patients who received voretigene neparvovec-rzyl (VN) for RPE65-mediated retinal degeneration.

To report the outcomes of combined cataract surgery with toric intraocular lens (IOL) implantation when performed in conjunction with transconjunctival sutureless pars plana vitrectomy.

To describe multimodal imaging and electrophysiologic characteristics of an unusual subset of patients with genetically confirmed autosomal recessive Stargardt disease (STGD1) who exhibited a central form of cone dysfunction resembling occult macular dystrophy that preceded the development of lipofuscin flecks, atrophy of retinal pigment epithelium (RPE), or full-field electroretinography abnormalities.

Purpose:To assess anatomic outcomes following repair of traumatic retinal detachment (RD) among a cohort of cognitively impaired adolescents with self-injurious behaviors.Methods:Main outcome measures in this retrospective, consecutive, interventional case series were retinal attachment and postoperative complications.Results:Complete retinal reattachment was initially achieved in all 9 eyes of 9 patients. Six (67%) eyes had chronic RD at initial presentation, and 7 (78%) patients had limited vision from inoperable RD in the fellow eye. Proliferative vitreoretinopathy led to recurrent RD in 4 (44%) eyes at a mean of 2.7 months. Final total or partial retinal attachment was achieved in 6 (67%) and 3 (33%) eyes, respectively, with a mean of 1.7 procedures over a mean follow-up of 22.4 months. Silicone oil emulsification led to progressive glaucoma in 4 (50%) of 8 eyes in which it was used, requiring silicone oil exchange (2 eyes) or removal (2 eyes). Recurrent RD developed in both eyes from which silicone oil was removed despite prior retinal anatomic stability for more than 6 months.Conclusion:Cognitively impaired adolescents with self-injurious behaviors typically presented with severe visual impairment from chronic traumatic RD in one or both eyes. Despite initial anatomic success, formation of proliferative vitreoretinopathy and further ocular trauma from continued self-injurious behaviors led to a high rate of recurrent RD. Long-term silicone oil tamponade may prevent recurrent RD in this challenging scenario but is associated with vision loss from progressive glaucoma and corneal decompensation.

BACKGROUND AND OBJECTIVE:To compare duration and efficacy of the dexamethasone (DEX) intravitreal implant in vitrectomized (pars plana vitrectomy [PPV] group) and non-vitrectomized eyes (control group) with macular edema related to central retinal vein occlusion (CRVO).PATIENTS AND METHODS:Eyes that received the DEX implant for CRVO related to macular edema were included in a retrospective chart review. Outcomes measured were best-corrected visual acuity (BCVA) and central macular thickness (CMT).RESULTS:Fifteen eyes were included in the study. Eight of 15 eyes had prior vitrectomy. Mean BCVA was 20/160 in both groups. Baseline mean CMT was 550 μm (PPV group) and 556 μm (control group, Fifteen eyes were included in the study. Eight of 15 eyes had prior vitrectomy. Mean BCVA was 20/160 in both groups. Baseline mean CMT was 550 μm (PPV group) and 556 μm (control group, P= .70), and improved to 307.5 μm (PPV group) and 316.3 μm (control group) (P= .90) after implant. The BCVA was 20/125 (PPV group) and 20/200 (control group) (P= .60). Time to next procedure was similar in both groups.CONCLUSIONS:This retrospective study shows similar CMT reductions with DEX implant treatment for CRVO-related macular edema in vitrectomized and non-vitrectomized eyes.

To report an anatomic change observed in a series of patients who underwent subretinal injection of voretigene neparvovec-rzyl (VN) for RPE65-mediated Leber congenital amaurosis.

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To describe a case of chronic endophthalmitis after cataract extraction caused by an unusual pathogen.

To report a new complication of intravitreal triamcinolone acetonide (TA) injection.

Purpose. To introduce a clinical sign on spectral domain optical coherence tomography (SDOCT), which may indicate high risk for full-thickness macular hole formation after internal limiting membrane (ILM) peeling. Methods. The preoperative SDOCT images of two patients—one with multilaminar hemorrhage from ruptured retinal artery macroaneurysm and one with serous retinal detachment and severe macular schisis from optic pit maculopathy—who developed full-thickness macular hole (FTMH) after ILM peeling were evaluated retrospectively. Results. On the preoperative SDOCT images of both patients there was a thin bridge of tissue on either side of the foveal center with an outer retinal defect. The photoreceptors were displaced laterally away from the foveal center to create an “omega-” shaped configuration of the remaining tissue. Conclusion. “Omega-” shaped configuration on SDOCT may represent a higher risk of FTMH following ILM peeling. Vitreoretinal surgeons may wish to consider this sign in the process of their surgical decision making.