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To characterize the progression of structural and functional changes in the retinas of a small cohort of unrelated patients with early late-onset retinal degeneration and evaluate these changes as potential biomarkers for future treatment trials.Best-corrected visual acuity, contrast sensitivity, Goldman visual fields, retinal sensitivity measurement by mesopic microperimetry, extent of ellipsoid zone disruption using spectral domain optical coherence tomography, and fundus autofluorescence imaging were performed at each biennial visit.Three unrelated patients with molecularly confirmed late-onset retinal degeneration (S163R mutation in C1QTNF5 ) were prospectively followed for 4 years.The patient's ages were 44, 54, and 62 at baseline. Over the 4-year follow-up period, one patient demonstrated a significant reduction in best-corrected visual acuity (6 Early Treatment of Diabetic Retinopathy Study letters), whereas two patients suffered a significant reduction in contrast sensitivity. Early in the disease, there was a close relationship between ellipsoid zone disruption and a loss in retinal sensitivity. Later in the course of the disease, there were areas outside the zones of ellipsoid zone disruption that also suffered progressive loss of retinal sensitivity, suggesting that ellipsoid zone loss was not the only factor responsible for the loss of retinal sensitivity. Changes in fundus autofluorescence and Goldman visual field loss were not closely related to changes in ellipsoid zone disruption or retinal sensitivity loss.This study has found that the monitoring of the progression of ellipsoid zone disruption and changes in mesopic microperimetry may be useful biomarkers in future clinical trials in patients with late-onset retinal degeneration.

Purpose The authors describe a case of new bilateral epiretinal membranes and vitreous cells following intravitreal and subTenon's injections of bone marrow-derived stem cells. Methods Case report of a 43-year-old man with OPA-1 autosomal dominant optic atrophy who self-enrolled in a stem cell therapy clinical trial and received simultaneous bilateral intravitreal and subTenon's injections of bone marrow-derived stem cells. Results Within one month of receiving the injections, the patient developed epiretinal membranes and vitreous cells in both eyes, seen with optical coherence tomography. Conclusion Stem cell therapy has been gaining popularity as a potential intervention for progressive retinal and optic nerve diseases; however, the mechanism of action of bone marrow-derived stem cells is still not well understood and may result in unintended cell differentiation.

To present immunocytochemical characterization of a surgically excised central posterior lens capsule (PLC) that was transplanted to close a secondary refractory full-thickness macular hole (FTMH) as an epiretinal flap. For comparison, tissue of both an unaffected internal limiting membrane and unexposed PLC was processed.Clinical-pathological case report.We report of a 38-year-old patient who underwent pars plana vitrectomy (PPV) with PLC tissue for patching secondary FTMH and silicone oil tamponade after tractional retinal detachment. The PLC was peeled off during a vitrectomy 1 year after positioning. For immunocytochemistry, the removed PLC was prepared as flat mount and showed a positive immunofluorescence of the Müller cells marker glutamine synthetase and for vimentin. The microglia marker IBA and the neuroprotective neurotrophic marker glia cell-derived neurotrophic factor were tested positive too. There was no immunoreactivity of cellular retinaldehyde-binding protein and glial fibrillary acidic protein. In comparison, tissue of a control internal limiting membrane that was obtained during standard FTMH surgery showed few single cells that were likewise positive for glutamine synthetase, glia cell-derived neurotrophic factor, and IBA. The control specimen of unexposed PLC showed rarely cells that were without positive immunostaining for the tested markers.Our analysis revealed positive immunoreactivity of macroglia and microglia cells of the PLC tissue that was used to patch a refractory FTMH. Similar immunostaining of PLC material and internal limiting membrane suggests the PLC transplantation as an alternative treatment option for refractory FTMH.

To report a case of nonleaking cystoid macular edema (CME) associated with pentosan polysulfate sodium (PPS)-induced pigmentary maculopathy.Multimodal imaging, including optical coherence tomography, fundus photography, autofluorescence, and fluorescein angiography, was used to substantiate our diagnosis, further characterize the cystoid macular edema showed by our patient and to monitor the response to treatment.A 59-year-old woman was referred for decreased visual acuity and bilateral macular edema. She had been treated for interstitial cystitis with PPS for 10 years. Multimodal imaging showed the characteristic features of PPS-induced pigmentary maculopathy. Moreover, fluorescein angiogram showed nonleaking cystoid macular edema in both eyes. She was treated successfully with intravitreal injections of bevacizumab.To our knowledge, this report is the first to demonstrate that PPS-associated cystoid macular edema can be nonleaking on fluorescein angiography and responds well to intravitreal anti-vascular endothelial growth factor injections.

Purpose To describe a novel clinical and imaging finding in patients with tubercular posterior uveitis. Methods A retrospective review of 3 cases presented at a tertiary referral eye centre in North India between June 2016 to March 2019 was performed. All the patients had received an initial diagnosis of non-infective etiologies (sympathetic ophthalmia, necrotizing scleritis and lymphoma). Fundus photography, fluorescein angiography (FA), fundus autofluorescence (FAF), and enhanced-depth imaging optical coherence tomography (EDI-OCT) were reviewed. Results Three patients (all Asian Indian females: aged 18, 49 and 52 years) diagnosed with panuveitis were investigated for various etiologies based on the initial clinical suspicion. During the course of therapy, all the patients developed peripheral yellow sub-retinal pigment epithelim (RPE) deposits (YSRPE) which appeared hypo-autofluorescent on FAF, and initially hypofluorescent with late hyperfluorescence on FA. The patients were subjected to detailed systemic evaluation and laboratory tests. All the patients showed acid fast bacilli on invasive tissue biopsies. After initiation of anti-tubercular therapy, the lesions resolved in all eyes. Conclusions YSRPE deposits represent a novel and important diagnostic sign of tubercular posterior uveitis.

PURPOSE We describe a case of endophthalmitis caused by streptococcus salivarius presenting as a retinal vasculitis less than twenty-four hours after intravitreal injection. METHODS Case report RESULTS:: The patient progressed from a hemorrhagic retinal vasculitis to severe endophthalmitis with no view to the posterior segment within 24 hours. Visual acuity progressed from 20/20 pre-injection to light perception within less than 48 after injection. Vitreous tap and injection was performed within 24 hours of intravitreal aflibercept injection, followed by pars plana vitrectomy the next day. Final visual acuity was hand motions. CONCLUSIONS Streptococcus salivarius is a virulent organism that may cause an endophthalmitis early after intravitreal injection with an unusual presentation of hemorrhagic retinal vasculitis. A high index of suspicion for infectious endophthalmitis should be maintained for all patients presenting with ocular inflammation and worsening vision in the days following intravitreal injections.

To describe a patient with a choroidal osteoma treated with photodynamic therapy to prevent tumor growth in whom choroidal neovascularization (CNV) developed after being treated with photodynamic therapy.Case report.A 5-year-old Hispanic woman presented with an asymptomatic choroidal osteoma, temporal to the macula of her right eye. According to the patient's mother, her medical, surgical, and family history was unremarkable. At examination, best-corrected visual acuity was 20/30 in both eyes. After 11 months of follow-up, signs of tumor growth toward the fovea without any signs of CNV was noted. Photodynamic therapy was performed to prevent invasion of the foveola. Two months thereafter, the patient developed CNV in the macula region in the right eye, decreasing visual acuity to 20/200. The patient was treated with four total intravitreal injections of 1.25 mg of bevacizumab over 24 weeks, which resulted in inactivation of the CNV and improved visual acuity to 20/20. Choroidal neovascularization had been never reported in her past history and her follow-up visits over 7 years. In addition, no evidence of recurrent neovascular activity or tumor growth was reported.Choroidal osteoma is a benign tumor that can result in vision-threatening complications, caused by tumor growth and tumor decalcification. Photodynamic therapy is an effective modality in inducing choroidal osteoma decalcification and stabilization; however, CNV due to reperfusion following photodynamic therapy can be seen in the retina.

To report a sporadic case of a familial adenomatous polyposis (FAP) discovered in a patient with bilateral retinal pigment epithelial lesions.Case report.A 30-year-old Asian woman presented for evaluation of bilateral pigmented lesions at the level of the retinal pigment epithelium. She had no personal or family history of colonic polyps or colon cancer. Colonoscopy revealed innumerable adenomatous polyps and genetic testing revealed a mutation in the adenomatous polyposis coli gene consistent with FAP. She subsequently underwent prophylactic total colectomy.The pigmented retinal pigment epithelium lesions of FAP have a characteristic appearance and it is vital for the retinal specialist to be familiar with them. De novo mutations in the APC gene are responsible for 20% to 30% of FAP cases. In the presence of the characteristic retinal pigment epithelium lesions, it is important to send the patient for work up of FAP even in the absence of family history of FAP.

Purpose To report two cases of cytomegalovirus (CMV) retinitis in renal transplant patients after conversion to belatacept immunosuppression. Methods Case report. Results Case A was a 41-year-old African American man with no ocular history, chronic hepatitis B infection on entecavir, status-post living relative renal transplant for end-stage renal disease secondary to IgA nephropathy presented with a chief complaint of "blurry vision" in his right eye. Fundoscopic examination of the right eye revealed perivascular retinitis of the superior arcade extending temporally and a lesion nasal to the optic disk. The patient was diagnosed with CMV retinitis with macular involvement. Case B was a 52-year-old woman with an ocular history significant for bilateral vein occlusions treated with laser, and a medical history of hypertension, hyperlipidemia, and end-stage renal disease secondary to hypoplastic kidney status-post deceased donor renal transplant presented with one week of blurred vision in the right eye. Fundoscopic examination of the right eye revealed macular star configuration of exudates, as well as subretinal fibrosis temporal to the macula. Fundoscopic examination of the left eye showed Grade 4 vitreous haze and multiple areas of retinal whitening and hemorrhage, consistent with CMV retinitis infection. The patient was diagnosed with bilateral CMV retinitis, with macular involvement in the right eye. Conclusion Renal transplant patients converted to belatacept immunosuppression may be at an increased risk for herpes virus infection, and thus herpes virus retinitis. Although the degree of risk remains uncharacterized, patients on belatacept therapy who are at the high risk of CMV infection (i.e., donor-positive/recipient-negative patients) should be counseled on the presenting signs and symptoms of CMV retinitis. In these cases, clinicians should also consider regular monitoring of serum CMV titers or continuation of antiviral prophylaxis.

PURPOSE We report a transgender patient with branch retinal artery occlusion who had secondary polycythemia from unregulated testosterone injections and review the literature on the mechanisms of supraphysiologic and standard doses of testosterone causing a hypercoagulable state. METHODS Case report. RESULTS A 45-year-old Chinese transgender man with no medical history presented with a 1-week history of a scotoma in his left eye vision. Ophthalmologic examination revealed retinal pallor and edema along the superotemporal arteriole in the left eye. Optical coherence tomography showed increased thickness of the inner retinal layers of the superotemporal retina. Fluorescein angiography demonstrated an arm-retina time of 1 minute and 43 seconds, with no vascular sheathing and capillary fallout. A diagnosis of left superotemporal branch retinal artery occlusion was made. Initial blood tests revealed a hemoglobin level of 19.3 g/dL (11.8-14.6 g/dL), hematocrit of 62% (34.3-43.0%), and erythrocytes of 6.56 × 10/L (3.7-4.8 × 10/L). He revealed later that he had been on weekly testosterone injections (testosterone enanthate 250-mg depot injection) since 2011. He was also exposed to a moderately high altitude, when his symptoms occurred, raising the possibility of worsening hypercoagulability resulting in his thrombotic event. CONCLUSION To the best of our knowledge, this is the first documented case of a trans man who developed branch retinal artery occlusion after self-administering supraphysiological doses of testosterone. In a young patient with no history of cardiovascular risk factors who develops retinal arterial occlusion, other causes such as hypercoagulable syndromes must be excluded. This case warns of the dangers of unregulated testosterone use, especially at supraphysiologic doses, and the risks of thrombotic events from secondary polycythemia.

Purpose To present a case of endophthalmitis caused by Abiotrophia defectiva after repeated injections of dexamethasone intravitreal implant (Ozurdex), in a patient already on systemic immunosuppressive therapy, and discuss the management of this challenging situation. Methods Case report and review of literature. Results A 70-year-old female patient with history of idiopathic pulmonary fibrosis presented for urgent evaluation due to left eye vision loss and mild discomfort 12 days after receiving her seventh dexamethasone implant injection. Ocular history was pertinent for recurrent macular edema resulting from a remote branch vein occlusion. Twelve days after her last injection, visual acuity decreased from 20/30 to counting fingers at 5 feet. Slit-lamp examination showed anterior chamber cell without fibrinoid membranes or hypopyon. Within hours, the patient's clinical appearance progressed. A vitreous tap followed by intravitreal injection of vancomycin (1 mg/0.1 mL) and ceftazidime (2.25 mg/0.1 mL) was performed. The culture from the vitreous sample revealed heavy growth of A. defectiva. Owing to limited improvement, patient was taken to the operating room, and the presence of a full-thickness scleral defect at the site of most recent dexamethasone implant injection was confirmed. Vitrectomy with removal of implant, closure of all sclerotomies, including nonhealing full-thickness scleral defect, and repeated intravitreal antibiotic injection were performed. At the 3-month follow-up, no inflammation was observed, but visual acuity remained poor. Conclusion Intravitreal dexamethasone implant-associated endophthalmitis in the setting of systemic immunosuppression is a rare and challenging situation. Both local and systemic immunosuppression may delay wound healing, predisposing to wound leakage and consequent endophthalmitis. Despite repeated intravitreal antimicrobial injection and vitrectomy with implant removal, A. defectiva endophthalmitis carried a poor visual outcome.

Background/purpose To describe a patient with visually symptomatic circumscribed choroidal hemangioma (CCH) treated successfully with intravitreal beta-blocker. Methods This is an interventional single case report of a 63 year-old man with a juxtafoveal CCH and extensive subretinal fluid (SRF) unsuccessfully treated with intravitreal anti-VEGF. Off-label intravitreal use of metoprolol (50μg/0.05 ml) was then performed. Main outcome measures were resolution or decreased subretinal fluid on OCT, visual stability or improvement, lack of retinal/ocular toxicity. Results Following 2 intravitreal injections of metoprolol (1 month apart), significant response was observed with decrease of SRF and visual improvement to 20/400 during a 9-week follow-up after the injections. Conclusion These preliminary findings suggest that intravitreal metoprolol can be a safe alternative treatment for patients with CCH. This off-label therapy could represent another option for patients with this condition.

PURPOSE To report two cases of cat-scratch fever with atypical posterior segment manifestations. METHODS Two cases were retrospectively reviewed. RESULTS A 27-year-old woman presented with painless blurring of central vision in her left eye. Clinical examination revealed a small focal area of retinitis within the macula associated with a subtle macular star. Spectral-domain optical coherence tomography showed a hyper-reflective inner retinal lesion in addition to subretinal and intraretinal fluid as well as hyperreflective foci within the outer plexiform layer. Serology was positive for anti-B. henselae IgM (titer 1:32). A 34-year-old woman presented with painless loss of vision in both eyes associated with headaches and pain with extraocular movement. Spectral-domain optical coherence tomography depicted subretinal fluid, intraretinal fluid, and hyperreflective deposits within the outer plexiform layer. A focal collection of vitreous cell was observed overlying the optic nerve in the left eye. Bilateral disk leakage was identified on fluorescein angiography. Serology revealed high-titer anti-B. henselae antibodies (IgM titers 1:32, IgG titers 1:256). CONCLUSION Our cases highlight the necessity of recognizing more unusual posterior segment presentations of ocular bartonellosis. Multimodal retinal imaging including spectral-domain optical coherence tomography may help better characterize lesions.

Purpose To describe a transient positive scotoma and corresponding optical coherence tomography (OCT) structural and angiographic findings. Methods The patient was evaluated with a comprehensive ophthalmic examination to include OCT structural and angiographic imaging with two different instruments, the Zeiss Plex Elite and the Optovue RTVue XR Avanti. Results A 45-year-old man had a sudden onset of a positive scotoma in the visual field of the left eye. No abnormalities were noted by ophthalmoscopy or fundus photography. Optical coherence tomography angiography was performed to evaluate the macular perfusion status. With each instrument, a small hyperreflective area, 175 μm in diameter, was imaged in the inner nuclear layer. The OCT angiographic images suggested a small area of decreased perfusion in the deep capillary plexus. Except for the diminutive size, the lesion had an appearance suggestive of paracentral acute middle maculopathy. The symptoms lessened rapidly, and when examined 4 days later, the lesion was less hyperreflective. Two weeks after presentation, the positive scotoma was not present and there was no longer any hyperreflectivity in the inner nuclear layer. Conclusion Detection of the lesion was aided by using OCT angiographic scans, which have a much higher scan density than conventional OCT evaluations. The diminutive abnormality was consistent with a paracentral acute middle maculopathy lesion, although smaller than those previously reported. Micro-paracentral acute middle maculopathy lesions should be considered in the differential diagnosis of positive scotomas.

Purpose To describe the clinical course of acute syphilitic posterior placoid chorioretinitis (ASPPC) in the preplacoid stage, placoid stage, and after treatment with penicillin. Method A retrospective case report of serial multimodal imaging and electrophysiology studies of a patient with ASPPC, with 18 months of follow-up. Results A 47-year-old man presented with bilateral panuveitis. The patient defaulted follow-up and returned when his vision deteriorated. Tests for neurosyphilis and retroviral disease were positive, and treatment was initiated. The earliest change on serial optical coherence tomography was loss of the signal from the reflective band corresponding to the ellipsoid zone. In the placoid stage, there was nodular thickening of the retinal pigment epithelium. The ellipsoid zone signals reappeared after treatment. Fundus fluorescein angiogram at presentation showed peripapillary vasculitis and disk leakage; indocyanine green angiography revealed multiple hypofluorescent spots in the peripapillary region and posterior pole that was not visible clinically. The angiographic abnormalities resolved after treatment. Electrophysiology demonstrated bilateral maculopathy and reduction of both a- and b-waves from dark-adapted and light-adapted responses at presentation. The b-waves (inner retina) recovered partially with treatment. Conclusion To the best of our knowledge, this is the first case report of the multimodal imaging and electrophysiology findings in a patient with acute syphilitic posterior placoid chorioretinitis, before the development of the classic placoid lesion. Improvement of structural and functional pathology after systemic treatment is demonstrated.

PURPOSE: To describe in detail the phenotype of a patient with enhanced S-cone syndrome. METHODS: We describe a 13-year-old boy who presented with blurred vision, vitreous cells, cystoid macular edema refractory to steroid treatment, and a negative uveitic workup. The patient underwent a complete ophthalmic examination, full-field electroretinograms (ffERG), automatic static perimetry and multimodal imaging with spectral domain optical coherence tomography, and adaptive optics scanning laser ophthalmoscopy (AOSLO). RESULTS: Spectral domain optical coherence tomography demonstrated cystoid macular edema and a hyperthick, delaminated midperipheral retina. Fluorescein angiography did not demonstrate macular leakage. Rod-mediated ffERGs were undetectable, and there was a supernormal response to short-wavelength stimuli compared with photopically matched longer wavelengths of light consistent with enhanced S-cone syndrome. Gene screening was positive for compound heterozygous mutations NR2E3: a known (c.119-2 A>C) and a novel (c.119-1G>A) mutation. By perimetry, sensitivities were normal or above normal for short-wavelength stimuli; there was no detectable rod-mediated vision. AOSLO demonstrated higher than normal cone densities in the perifoveal retina and evidence for smaller outer segment cone diameters. CONCLUSION: Evidence for supernumerary cones (at least twice the normal complement) by AOSLO and spectral domain optical coherence tomography was associated with supernormal S-cone sensitivities and electroretinogram responses confirming previous in vivo findings in postmortem human specimens. Smaller than normal cones in enhanced S-cone syndrome may represent “hybrid” photoreceptors analogous to the rd7/rd7 murine model of the disease.

Purpose To describe a novel surgical technique to remove retained subfoveal perfluorocarbon liquid (PFCL). Methods After setting up for 23-G pars plana vitrectomy, a 38-G flexible-tip macular hydrodissection cannula connected to the automated viscous fluid infusion kit was used to create a small retinotomy approximately 700 μm to 800 μm inferior to the fovea and induce macular detachment involving the retained PFCL bubble. The flexible cannula was bent at its junction with the shaft and was carefully advanced through the same retinotomy into the subretinal space to access and directly aspirate the retained subfoveal PFCL bubble. Fluid-air exchange was then performed, and surgery was concluded. Results The retained subfoveal PFCL bubble was successfully removed with restoration of normal foveal architecture on optical coherence tomography and with objective and subjective improvement of central vision. Conclusion We report a novel surgical technique combining macular detachment with direct aspiration of the retained subfoveal PFCL without direct perforation of the foveal center. This technique may provide an alternative approach to manage this difficult complication.

PURPOSE To report the outcomes of macular add-on intraocular lens implantation in improving reading vision in patients with bilateral advanced diabetic maculopathy. METHODS In this retrospective study, a supplementary bifocal sulcus intraocular lens (Scharioth Macular Lens) was implanted in the better-seeing eye of five patients. Baseline-corrected distance vision, corrected near visual acuity, a preoperative simulation test, and multimodal imaging were collected. The primary outcome was the uncorrected near visual acuity at a working distance of 15 cm, at a 12-month follow-up. RESULTS Study patients included 3 cases of refractory subfoveal exudation and 2 cases of diabetic macular ischemia. A preoperative test to assess the potential gain in near vision showed an improvement of ≥2 paragraphs on the RADNER reading chart in all patients. At 12 months, median reading vision (corrected near visual acuity at 15 cm) significantly improved from 20/125 (range 20/50-20/200) preoperatively to uncorrected near visual acuity (at 15 cm) of 20/50 (range 20/40-20/80) (P = 0.042; Wilcoxon signed-ranks test). Distance vision remained unchanged in four patients. All patients were able to achieve the size of newsprint (20/50 Snellen equivalent), within the first 3 months. CONCLUSION The macular add-on intraocular lens improves reading vision in visually impaired patients due to end-stage diabetic macular disease.

Purpose To report a case of failed endolaser retinopexy in patient taking pazopanib and possible association with impaired retinal remodeling. Methods Case report. Results A 68-year-old man with a history of radiation-induced high-grade metastatic mediastinal leiomyosarcoma, on pazopanib 600 mg daily, presented with a superior retinal tear 2 months after initiating pazopanib. The patient was treated with pars plana vitrectomy and endolaser retinopexy without any immediate complications. One month postoperatively, the patient was found to have a new superior macula-sparing rhegmatogenous retinal detachment extending through the laser barricade nasally. A large proportion of the laser lesions in this area had notably remained chalk-white. The patient underwent pars plana vitrectomy with scleral buckling, and repeat endolaser retinopexy. Pazopanib therapy was stopped 1 week later in consultation with the oncology team and was reinitiated after the retina seemed stable under silicone oil for several weeks. The scars from the patient's repeat endolaser retinopexy remained pale white at his clinic visit 2 months after the procedure, and at that time, his initial laser scars had only just begun to darken with pigment. Four and a half months after the initial vitrectomy and endolaser retinopexy, and three and a half months after his repeat vitrectomy and endolaser retinopexy, all the patient's laser scars have developed increased pigment, and his retina remains completely flat under silicone oil tamponade. Conclusion Pazopanib may have had an adverse effect on retinal wound healing after vitrectomy with endolaser retinopexy, leading to persistence of pale laser lesions many weeks after laser application. It is possible that this may have impaired the typical development of chorioretinal laser scar formation and led to subsequent retinal detachment.

Supplemental Digital Content is Available in the Text. The largest case series to date describing uveitis occurring secondary to systemic treatments for malignant melanoma. Twenty-two cases are described. Uveitis occurred after treatment with checkpoint, BRAF, and mitogen-activated protein kinase inhibitors., Background/Purpose: To report the largest case series to date of uveitis occurring in association with immunomodulatory therapy for malignant melanoma. Methods: A retrospective multicenter case review. Twenty-two patients with uveitis occurring in association with either immunotherapy or targeted immune therapy for malignant melanoma were identified. Results: Of 22 patients, 11 had anterior uveitis in isolation. The remainder showed a variety of clinical features including panuveitis, ocular hypotony, papillitis, cystoid macular edema, and melanoma-associated retinopathy. Most patients responded well to treatment. Conclusion: We report the largest case series to date of patients with uveitis secondary to drug treatment for malignant melanoma. These cases are likely to increase in number in the future as newer immunomodulatory therapies for cancers are developed and the indications for these drugs increase. A dilemma arises when patients respond well to these drugs but develop vision-threatening side effects.

Background/purpose To report vitreous and epiretinal seeding of choroidal melanoma after transvitreal fine-needle aspiration biopsy (FNAB). Methods A 74-year-old Caucasian man with a medium-sized, dome-shaped choroidal melanoma without Bruch membrane rupture, retinal invasion, or vitreous seeding. Transvitreal FNAB was performed for cytopathologic evaluation using a long 27-gauge needle attached to a 10-mL syringe by connector tubing. After passing through the pars plana, vitreous cavity, neurosensory retina, and Bruch membrane, the needle was inserted into the tumor and aspiration was performed. Results Cytopathologic evaluation revealed spindle B melanoma cells, and the tumor was subsequently treated with an 18-mm round Iodine-125 radioactive plaque delivering a tumor apex radiation dose of 7,000 cGy. Transscleral FNAB for cytogenetic evaluation performed at the time of plaque application disclosed chromosome three monosomy and chromosome six and eight disomy. Vitreous pigmented cells were first noted 14 months after brachytherapy. Funduscopy and optical coherence tomography at 24 months after plaque radiotherapy showed extensive vitreous and preretinal pigmented seeding that completely covered the optic disk. Fine-needle aspiration biopsy of the vitreous confirmed the presence of malignant melanoma cells, and the right eye was enucleated. Histopathologic evaluation showed a totally necrotic choroidal melanoma and an extensive malignant epiretinal membrane that covered and extended into the substance of the optic nerve head and focally invaded the neurosensory retina. The patient developed liver metastasis 3 years after the initial presentation and died 10 months later. Conclusion This case demonstrates the rare development of extensive vitreous and preretinal seeding with retinal and optic nerve invasion after transvitreal FNAB of choroidal melanoma.

The purpose of this study was to describe a new 33-gauge hypodermic needle-guided iris suturing technique for repair of iris defects and its adaptation for use to address a wide range of iris pathology in complex anterior segment reconstruction cases. This approach uses a 33-gauge hypodermic needle loaded with a 10-0 Prolene suture within its lumen, which is then used to directly introduce and manipulate the suture within the anterior chamber. Eliminating the need for a needle holder, this technique provides surgeons improved ergonomics and minimizes risk for trauma to adjacent tissues. Improving on and addressing limitations of the original sewing machine technique described by Safran in 1995, iris repair using this technique has been applied successfully in 15 patients to achieve satisfactory visual and cosmetic outcomes without complications. Multiple iris suturing strategies can be combined on a given case to attain complex repair with this new approach.

Purpose To describe two patients with giant cell arteritis who presented with macular lesions mimicking persistent placoid maculopathy. Methods Retrospective case reports. Patient Two patients, a 74-year-old man and an 85-year-old woman both were diagnosed with biopsy-confirmed giant cell arteritis with vision loss in one eye due to arteritic ischemic optic neuropathy. Both presented many years later with acute vision loss in the remaining eye. Results Both patients had bilateral retinal whitening in the parafovea, and the second also had sectoral optic disk swelling. On fluorescein angiography, these areas of retinal whitening in both patients were hypofluorescent early with late hyperfluorescence staining. On indocyanine green angiography, there were areas of foveal hypocyanescence bilaterally in both patients that persisted throughout the imaging and were more apparent late. These findings can be seen in patients with bilateral persistent placoid maculopathy. Conclusion Giant cell arteritis should be included in the differential diagnosis of persistent placoid maculopathy.

PURPOSE To illustrate the features of unilateral retinal pigment epithelium dysgenesis (URPED) in an African-American male patient. METHODS Case report. RESULTS A 47-year-old asymptomatic African-American man was referred for an atypical subretinal pigmented mass in the left eye. On examination, visual acuity was 20/20 in both eyes. The right eye was unremarkable. The left eye revealed a darkly pigmented grey-black lesion at the level of the RPE with irregular, unraveled fringe-like margins, consistent with URPED. The lesion measured 5 mm in basal dimension and was located 400 μm from the foveola. The dark portion of the lesion was grey-black and demonstrated homogeneous hypoautofluorescence, particularly at the site of grey-white peripheral fringe of fibrous metaplasia. By contrast, there was an additional, subtle lacey arrangement of normal-appearing RPE traversing over the entire lesion demonstrating isoautofluorescence. On fluorescein angiography, the lesion was generally hypofluorescent, particularly in the dark portion of the lesion, but the peripheral fringe of fibrous metaplasia displayed angiographic hyperfluorescent staining, and the subtle lacey normal RPE showed isofluorescence. Optical coherence tomography demonstrated RPE hyperplasia and shallow RPE detachment interspersed with normal-appearing RPE and thinning of outer retina and preservation of the foveola and choroid. CONCLUSION In this case, URPED demonstrated biphasic autofluorescence implying RPE dysfunction in the hypoautofluorescent area and partial RPE function in the lacey isoautofluorescent region.

PURPOSE To describe a novel fluorescein angiographic finding in patients with von Hippel-Lindau disease. METHODS Retrospective case series of patients diagnosed with retinal capillary hemangioblastoma (RCH) in the setting of von Hippel-Lindau. RESULTS We identified six eyes of three patients with von Hippel-Lindau and leaky retinal vessels. All eyes showed segmental diffuse vascular leakage (SDVL) that was seen in the late phase of the angiogram and that originated from third order and more peripheral retinal veins and adjacent capillaries. These vessels did not drain from the RCHs. Segmental diffuse vascular leakage was mainly seen in the mid and far periphery. In some cases, it was located near the RCHs, while in other cases, it was remote. Segmental diffuse vascular leakage was also seen in one eye without RCHs. On follow-up, the extent and intensity of segmental diffuse vascular leakage did not change after the RCHs were treated with laser or cryotherapy. CONCLUSION Diffuse vascular leakage from retinal venules around and away from RCHs in patients with von Hippel-Lindau disease is seen, but the clinical and prognostic importance of this finding is uncertain.

Purpose To describe a case of symptomatic outer retinal disruption in a patient heterozygous for the p.Leu154Pro interphotoreceptor matrix proteoglycan-1 (IMPG1) mutation implicated in adult-onset foveomacular vitelliform dystrophy. Methods Observational case report. Results We describe a case of a 25-year-old female patient with symptomatic scotoma and vision decrease who exhibited bilateral small foveal yellow spots. Optical coherence tomography revealed disorganization and decreased reflectance of the foveal ellipsoid and interdigitation zones in the left eye more than in the right eye. Fundus autofluorescence imaging showed minimal findings, and dye angiography was unrevealing. Multifocal electroretinogram revealed slightly decreased retinal sensitivity in the central retina of the left eye. Genetic testing identified a heterozygous p.Leu154Pro mutation in the IMPG1 gene. Conclusion The p.Leu154Pro IMPG1 mutation may cause symptomatic outer retinal disturbance in the heterozygous state. Further studies are necessary.

Purpose To report patients who demonstrated an alteration in the clinical and optical coherence tomography features of neovascular age-related macular degeneration after resolution of endophthalmitis. Methods Retrospective case series of the subsequent changes in the macula and need for anti-vascular endothelial growth factor therapy in patients with neovascular age-related macular degeneration who developed endophthalmitis after intravitreal injection. Results The study included seven eyes of seven patients with follow-up ranging between 3 months and 11 years. The vitreous cultures (n = 7) before intravitreal antibiotic injection were the following: culture-negative (4) and coagulase-negative Staphylococcus (3). Initial treatment included vitreous tap and injection (4) and pars plana vitrectomy (3). In 5/7 eyes, the optical coherence tomography showed resolution of subretinal fluid and serous pigment epithelial detachment, and there was no additional anti-vascular endothelial growth factor treatment administered. Conclusion After successful treatment of endophthalmitis in patients with neovascular age-related macular degeneration, there was relative involution of the maculopathy and reduced anti-vascular endothelial growth factor treatment burden in this series.

Purpose To report a new family with North Carolina macular dystrophy including a patient with choroidal neovascularization (CNV). Methods Diagnostic modalities included fundus imaging, fluorescein angiography, optical coherence tomography, and genetic testing. The CNV was treated with intravitreal anti-vascular endothelial growth factor according to a treat-and-extend protocol in both eyes. Results A 60-year-old man presented with North Carolina macular dystrophy with decreasing vision in the left eye and persistently deceased central vision in the right eye. Optical coherence tomography examination showed intraretinal and subretinal fluid consistent with CNV. Genetic testing was performed. Examination of family members showed no signs of CNV. The visual acuity improved from 20/400 to 20/150 in the right eye and from 20/100 to 20/40 in the left eye after intravitreal bevacizumab treatment for CNV. Molecular analysis of the PRDM13 gene revealed a pathogenic heterozygous point mutation. Conclusion Recognition and treatment of CNV in North Carolina macular dystrophy can result in improved vision. Genetic testing of the PRDM13 gene can confirm a molecular diagnosis for North Carolina macular dystrophy.

PURPOSE To describe the safety and efficacy of rhegmatogenous retinal detachment (RRD) repair with external drainage of subretinal fluid using a 28-gauge External Drainage and Depression device (Vortex Surgical, Chesterfield, MO). METHODS Retrospective review of patients who underwent primary rhegmatogenous retinal detachment repair with scleral buckle, pars plana vitrectomy, or scleral buckle/pars plana vitrectomy using the drainage device from August 2018 through March 2020, performed by four surgeons at two vitreoretinal practices. RESULTS Eighty-three eyes of 83 patients were included. At presentation, 28% had proliferative vitreoretinopathy. Surgery included 65% scleral buckle/pars plana vitrectomy, 33% pars plana vitrectomy, and 2% scleral buckle. There were no cases of retinal incarceration and two subretinal hemorrhages at the drainage site (both < 2 DD), 2 cases of recurrent RD with proliferative vitreoretinopathy (1 had proliferative vitreoretinopathy at presentation), and 6 (10%) new epiretinal membranes (3 were mild). There were no other complications. Mean follow-up was 274 days. Single operation success rate for those with ≥ 6-month follow-up was 97% (57/59). CONCLUSION External drainage of subretinal fluid during rhegmatogenous retinal detachment repair demonstrated a favorable safety profile with a high single operation success rate. Further study of the role of external drainage in rhegmatogenous retinal detachment repair is warranted.

PURPOSE To describe a case of choroidal neovascularization (CNV) and chorioretinal scarring in a patient with melanoma-associated retinopathy after ipilimumab/nivolumab combination immune therapy for malignant melanoma. METHODS Retrospective case report with fundus photography, fluorescein angiography, optical coherence tomography, and electroretinography. RESULTS A 65-year-old woman presented with symptoms of photopsia and visual field loss. She had previously undergone ipilimumab/nivolumab combination chemotherapy treatment for malignant melanoma 14 months earlier coinciding with the onset of her visual symptoms. Fundus photography showed bilateral atrophic chorioretinal lesions and peripheral retinal pigment epithelial changes. Fluorescein angiography revealed retinovascular leakage in both eyes with CNV in the right eye. Optical coherence tomography showed a pigment epithelial detachment with subretinal fluid and subretinal hyperreflective material consistent with occult CNV. Visual field testing showed generalized visual field loss in both eyes. Bloodwork discovered an elevated angiotensin-converting enzyme. Electroretinography revealed abnormal peripheral rod and cone function with impairment of the photoreceptor and inner nuclear layer. Serum Western blot was positive for 60 kDa antiretinal autoantibody. After a single bevacizumab injection in the right eye, CNV resolved and visual acuity improved from 20/50 before the injection to 20/25 3 months after the injection. Visual acuity in the left eye deteriorated for months to counting fingers but then improved to 20/100 on follow-up examinations. CONCLUSION Ipilimumab and nivolumab have been associated with immune-related ocular adverse effects. We report a case of combination therapy presenting with chorioretinal scarring and subsequent CNV in a patient with melanoma-associated retinopathy, a rare yet important adverse effect.

Purpose To report three middle-aged cases with choroidal neovascularization (CNV) associated with early-onset drusen documented with optical coherence tomography angiography (OCTA). Methods Three patients with bilateral early-onset drusen were referred to our hospital. Fundus examination, fluorescein angiography, indocyanine green angiography, OCTA, and other multimodal imaging were performed. Results Case 1 involved a 47-year-old woman who presented with sudden unilateral anorthopia. She had no previous systemic pathologies. Funduscopic examination and fluorescein angiography revealed bilateral large colloid drusen accompanied by unilateral mild subretinal hemorrhage. Indocyanine green angiography revealed CNV, although it was unclear in fluorescein angiography. Optical coherence tomography angiography also showed interconnecting CNV beneath the retinal pigment epithelium. Case 2 involved a 40-year-old woman with membranoproliferative glomerulonephritis Type 3 who presented with unilateral anorthopia. On fluorescein angiography, cuticular drusen secondary to membranoproliferative glomerulonephritis were seen in both eyes. An interconnecting vascular network was revealed with OCTA and indocyanine green angiography indicating Type 1 CNV in the affected eye. Case 3 involved a 47-year-old man without any medical or family history. Predominant large colloid drusen associated with cuticular drusen were seen in both eyes. Unilateral mild serosanguinous changes were accompanied in the macula, where Type 1 CNV was detected with OCTA. Conclusion All our cases with early-onset drusen showed Type 1 CNV that was detected by indocyanine green angiography or OCTA. Optical coherence tomography angiography has a potential to help noninvasively diagnose CNV in the cases of EOD.

Purpose To describe relaxing nasal retinotomy as a technique to achieve closure of a recurrent macular hole. Methods A patient with juxtafoveal telangiectasia presented with recurrent macular hole after primary vitrectomy and internal limiting membrane peel. During repeat surgery, a superonasal retinotomy was performed to allow for release of tangential traction contributing to the macular hole. Results Intraoperative ocular coherence tomography demonstrated decrease in size of the macular hole immediately after retinotomy. At follow-up, both the macular hole and retinotomy site had closed completely. Conclusion In patients with small macular holes that fail primary vitrectomy, nasal relaxing retinotomy can contribute to successful hole closure.

Purpose Prosthetic iris devices have recently been used to improve cosmesis and reduce glare in aniridia. There is currently no consensus on which prosthetic iris device or which surgical approach is preferred for managing large iris defects. Methods A novel surgical approach with Gore-Tex polytetrafluoroethylene sutures was used to achieve scleral fixation of an intraocular lens and artificial iris complex in a 19-year-old Caucasian female patient with aniridia, nystagmus, cataracts, and ectopia lentis. Results Six weeks postoperatively, the intraocular lens-artificial iris complex remained well centered, and the vision in the left eye improved from 20/400 to 20/70. Two years after prosthetic iris device implantation, there have been no complications. Conclusion This case demonstrates a promising proof-of-concept for long-term management of complicated aniridia cases using an intraocular lens and artificial iris complex prosthetic iris devices. Gore-Tex sutures may be preferable to conventional polypropylene sutures because of their improved durability.

Purpose To document by optical coherence tomography angiography the presence of a choroidal neovascularization (CNV) secondary to unilateral retinal pigment epithelium dysgenesis and describe its changes after treatment with intravitreal bevacizumab. Methods Case report. Results A 51-year-old woman referred to our ocular oncology service for a possible choroidal osteoma in her left eye was found to have a unilateral retinal pigment epithelium dysgenesis complicated by CNV. Best-corrected visual acuity at baseline was 20/200. A complete ophthalmological evaluation including color fundus photography, fundus autofluorescence, fluorescein angiography, indocyanine green angiography, optical coherence tomography, and optical coherence tomography angiography was performed. The CNV appeared as a tangled vascular network on optical coherence tomography angiography. After 2 intravitreal injections of bevacizumab, the best-corrected visual acuity improved to 20/50, and optical coherence tomography angiography showed a progressive contraction of the CNV. Unilateral retinal pigment epithelium dysgenesis margins remained stable at consecutive follow-up. Conclusion Optical coherence tomography angiography is a useful imaging technique for the diagnosis and follow-up of CNVs secondary to unilateral retinal pigment epithelium dysgenesis. Anti-vascular endothelial growth factor agents represent an effective therapy for the treatment of CNV in patients with this disease.

Purpose Intraocular gases are commonly used in vitreoretinal surgery. Patients are routinely advised against air travel before the complete absorption of intraocular gas. Consequently, reports on air travel in patients with large intraocular gas bubbles are highly unusual. Here, we report the intraocular pressure changes of a patient ascending to an altitude of 2,600 feet in a helicopter with a 50% fill perfluoropropane (C3F8) gas bubble in his left eye. Methods Case report and literature review. Results A 61-year-old male patient underwent pars plana vitrectomy for a rhegmatogenous retinal detachment, with fluid-gas exchange using 16% C3F8. With a 50% fill bubble in the left eye, the patient took a short helicopter trip ascending to a maximum altitude of 2,600 feet. Before take-off, intraocular pressure in the operated eye was 14 mmHg. The average increase in intraocular pressure was 10.8 mmHg per 1,000 feet of ascent, with a maximum recorded intraocular pressure of 42 mmHg. The patient denied both ocular pain and loss of vision but did report changes in the appearance of the gas bubble meniscus at 2,100 feet. Conclusion Short-term low-altitude air travel may be tolerated by some patients with intraocular gas in situ. Further studies are required to define the conditions by which patients with gas bubbles may fly safely.

Purpose To describe a case of congenital retinal macrovessel complicated by cystoid macular edema associated with contralateral myelinated retinal nerve fibers and retinal vascular abnormalities studied with optical coherence tomography angiography (OCTA). Methods Case report. Results A healthy 25-year-old woman with decreased vision in her right eye was found to have a congenital retinal venous macrovessel in the macula associated with cystoid edema. In the contralateral amblyopic eye, the examination revealed a tuft of myelinated retinal nerve fibers along the superotemporal vascular arcade associated with superficial vascular abnormalities. A complete multi-imaging examination was obtained, including fundus color photography, fluorescein angiography, indocyanine green angiography, optical coherence tomography (OCT), and optical coherence tomography angiography. At 1-week follow-up, the optical coherence tomography displayed spontaneous resolution of the edema that remained stable at consecutive 1-month follow-up. Conclusion Congenital retinal macrovessels can be associated with other ocular developmental anomalies. Vascular complications can occur, leading to macular edema and retinal ischemia. Optical coherence tomography angiography can be useful for the diagnosis and follow-up of this condition.

PURPOSE To describe a unique presentation of multifocal paracentral acute middle maculopathy in a young patient with juvenile dermatomyositis including optical coherence tomography angiography findings. METHODS Retrospective single case report. PATIENT Seventeen-year-old male patient with a history of juvenile dermatomyositis presented for routine hydroxychloroquine screening. RESULTS Optical coherence tomography revealed undulations of the outer retinal layers and retinal thinning near the fovea in both eyes. Optical coherence tomography angiography revealed loss of flow void densities in the superficial and deep capillary plexi in both eyes. These findings were consistent with a diagnosis of paracentral acute middle maculopathy. CONCLUSION To the authors' knowledge, this is the first report of the optical coherence tomography angiography findings in paracentral acute middle maculopathy associated with juvenile dermatomyositis. The defects were located in the superficial and deep capillary plexi and likely due to the known occlusive retinopathy associated with juvenile dermatomyositis.

Purpose To report a case of a woman diagnosed with choroidal melanoma during pregnancy, as well as review similar cases published in the literature. The prevalence, risk factors, treatment, prognosis, and outcomes of choroidal melanoma in pregnancy are addressed. Methods An extensive search of the literature was conducted by accessing PubMed, MEDLINE, and Scopus databases in January 2018 to identify cases of choroidal or uveal melanoma diagnosed during pregnancy. Patient A 37-year-old white woman pregnant with twins and diagnosed with choroidal melanoma. Results Choroidal melanoma in pregnancy has a prevalence ranging from 4.3% to 7.5% among women of childbearing age with the disease. Pregnancy has been linked to increased incidence of choroidal melanoma, but female hormones have not been shown to play a role in the pathogenesis of the disease. Other factors such as melanocyte-stimulation hormone and fetal-induced angiogenesis may be implicated. Choroidal melanoma in pregnancy has not resulted in a worse maternal or fetal outcome. Conclusion Although rare, choroidal melanoma in pregnancy is a clinical challenge. Management of these patients should be tailored to maternal and fetal needs.

Treating neovascular age-related macular degeneration with intravitreal aflibercept treat-and-extend (TE) can reduce treatment burden. ARIES assessed whether intravitreal aflibercept early-start TE was noninferior to late-start TE.A randomized, open-label, Phase 3b/4 study that included treatment-naïve patients aged ≥50 years with the best-corrected visual acuity 73-25 Early Treatment Diabetic Retinopathy Study letters and active choroidal neovascularization secondary to AMD. Patients received 2 mg intravitreal aflibercept at Week (W) 0, W4, W8, and W16. At W16, patients were randomized 1:1 to early-start (2W interval adjustments) or late-start TE (8W intervals until W48 then 2W interval adjustments). Primary endpoint: the best-corrected visual acuity change from randomization to W104.Two-hundred seventy-one patients were randomized. The mean (SD) best-corrected visual acuity at baseline was 60.2 (12.1; early-TE) and 61.3 (10.8; late-TE) letters. The mean (SD) best-corrected visual acuity change (W16-104) was -2.1 (11.4) versus -0.4 (8.4) letters (early-TE vs. late-TE; least-squares mean difference: -2.0; 95% confidence interval: -4.75 to 0.71; P = 0.0162 for noninferior); +4.3 (13.4) versus +7.9 (11.9) letters (W0-104). The mean (SD) number of injections was 12.0 (2.3) versus 13.0 (1.8). From baseline to W104, 93.4% and 96.2% maintained best-corrected visual acuity; the mean (SD) central retinal thickness change was -161.6 (135.6) µm and -158.6 (125.1) µm. The last injection interval (W104) was ≥12W for 47.2% and 51.9% of patients.Outcomes were similar between patients with neovascular age-related macular degeneration treated with an intravitreal aflibercept early-TE or late-TE regimen after initial dosing, with one injection difference over 2 years.ClinicalTrials.gov Identifier: NCT02581891 https://clinicaltrials.gov/ct2/show/NCT02581891. Supplemental Digital Contents (files 1 http://links.lww.com/IAE/B419).

To describe endogenous endophthalmitis in the setting of COVID-19 pneumonia.Patients recovering from COVID-19 pneumonia who presented to our department with any or all of the following complaints: pain, watering, redness, and decreased vision were identified. All relevant data were collected for analysis.Three patients with endogenous endophthalmitis were identified. All patients had been treated for COVID-19 pneumonia and therefore had received remdesivir and systemic steroids; 2 of the 3 patients received tocilizumab. All patients received vitreous biopsy, vitrectomy, and intraocular antibiotic injection. Patient 1 demonstrated Klebsiella pneumoniae in blood culture, K. pneumoniae and Escherichia coli in urine culture, and K. pneumoniae in vitreous fluid, whereas Patients 2 and 3 demonstrated Stenotrophomonas maltophilia and methicillin-resistant Staphylococcus aureus in the blood and nasopharyngeal culture, respectively. Correspondingly, the same organism was cultured from vitreous in Patients 2 and 3. The visual acuity at the last follow-up in Patients 1 to 3 was 20/100, 20/80, and 20/40, respectively. The probable source of infection was identified in each as renal calculi, dental caries, and the pharynx, respectively. Real-time polymerase chain reaction demonstrated the presence of Severe Acute Respiratory Syndrome Coronavirus 2 in the vitreous fluid of Patient 1.We report good outcomes of early intervention for endogenous endophthalmitis in the setting of COVID-19 infection. We also document the presence of SARS-CoV-2 in vitreous.

Characterization of the degradation of scotopic electroretinography recordings from patients in various stages of ADNIV reveal photoreceptors may sustain early insult and reveals a unique time frame for potential therapeutic intervention. PURPOSE: To characterize the changes found in the electroretinography (ERG) recordings of Autosomal Dominant Neovascular Inflammatory Vitreoretinopathy (ADNIV) patients and correlate with clinical stages of the disease. METHODS: Retrospective chart review. Bright and dim flash full-field scotopic, photopic, and 30 Hz flicker ERGs were obtained according to international standards. The scotopic ERGs were further processed to analyze the oscillatory potential (OP). The patient described in the case report underwent full ERG testing; five patients composed the archival case series data and included scotopic ERG recordings. RESULTS: Stage I ADNIV is characterized by a decrease in the b-wave amplitude on scotopic flash ERG and the disappearance of late OPs; however, the a-wave amplitude is normal. In stage II, attenuation of early OPs and the c-wave are observed in scotopic ERG recordings, but both a- and b-wave amplitudes are unchanged. For patients in stage III, there is a continued decline of both a- and b-wave amplitudes in scotopic ERG recordings. There was a loss of recordable scotopic ERG response in patients with stage IV disease. CONCLUSIONS: ERG may be valuable in determining optimal timing for therapeutic intervention and response prior to loss of recordable retinal function in CAPN5 vitreoretinopathy.

Background/purpose To describe the use of topical mitomycin-C in sclerostomy revision for recalcitrant idiopathic uveal effusion syndrome. Methods A 50-year-old healthy man presented with painless, gradual vision loss in the right eye. He underwent multimodal retinal imaging with wide-field fundus photography, spectral domain optical coherence tomography, and B-scan and A-scan ultrasonography. He was found to have idiopathic (non-nanophthalmic) uveal effusion syndrome with choroidal and serous retinal detachments in the right eye and a peripheral choroidal detachment in the left eye. Central vision became threatened in the right eye. Medical treatment with oral corticosteroids and surgical treatment with choroidal drainage through sclerostomies and sclerostomy revision were administered. Results Initial treatment with systemic corticosteroids was ineffective. Subsequent choroidal drainage through sclerostomies only partially resolved the effusion. Later sclerostomy revision with application of topical mitomycin-C led to complete resolution with anatomical stability maintained after at least 42 months of follow-up. Conclusion Successful use of topical mitomycin-C in sclerostomy revision has not previously been reported in idiopathic (non-nanophthalmic) uveal effusion syndrome. We propose that topical mitomycin-C may be considered as a potential therapeutic adjunct in the treatment of refractory idiopathic uveal effusion syndrome before further sclerostomy procedures are attempted in additional quadrants of the eye.

PURPOSE The Revo NX is a new optical biometer, based on spectral-domain optical coherence tomography and able to obtain high-definition scans of both the anterior and posterior segment of the eye. A previous study found that its measurements of axial length (AL) were not interchangeable with those provided by a validated optical biometer; so, the manufacturer updated the instrument to improve agreement of AL values. This study aimed to prospectively compare the measurements by the updated Revo NX (version 9.5.0, biometry module) with those by 2 validated devices, the IOLMaster 700 and Lenstar LS-900. SETTING Optopol Technologies, Zawiercie, Poland. DESIGN Prospective evaluation of diagnostic test. METHODS Comparison between the devices was performed using repeated measures analysis of variance (ANOVA) with Bonferroni posttest, correlation coefficients, and the Bland-Altman method. RESULTS The investigation evaluated the results of 63 patients. For AL, anterior chamber depth (ACD), and lens thickness (LT), the differences were not clinically significant because they were less than 0.01 mm. Repeated measures ANOVA, however, detected a statistically significant difference for AL (P < .0001) and central corneal thickness (P < .0001) but not for ACD (P = .0630) or LT (P = .2667). The results obtained with all 3 biometers manifested a high level of agreement in the Bland-Altman analysis and very strong correlation. CONCLUSIONS The measurements by the updated Revo NX had high agreement with the other optical biometers; a clear improvement was detected than the previous analysis between the original Revo NX (version 8.0.3) and the Lenstar LS-900.

PURPOSE To report the multimodal imaging findings of immune recovery uveitis mimicking recurrent T-cell lymphoma after autologous bone marrow transplant therapy. METHODS A 71-year-old man presented with posterior uveitis 6 weeks after chemotherapy and autologous bone marrow transplant for angioimmunoblastic T-cell lymphoma. Multimodal imaging included fluorescein angiography, fundus autofluorescence, and optical coherence tomography. Diagnostic testing included ocular polymerase chain reaction and diagnostic vitrectomy. RESULTS Clinical examination demonstrated vitritis and perivascular deep retinal whitening. Imaging of the retinal whitening showed late hyperfluorescence on fluorescein angiography, hyperautofluorescence on fundus autofluorescence, and ellipsoid zone loss on optical coherence tomography without infiltrative lesions. Testing was negative for syphilis, herpes simplex virus, varicella-zoster virus, and cytomegalovirus. After no clinical improvement with valacyclovir and intravitreal foscarnet treatment, diagnostic vitrectomy was performed. Bacterial and fungal cultures were negative, and herpes simplex virus, varicella-zoster virus, and cytomegalovirus were not detected by polymerase chain reaction. Cytopathology showed mature small nonneoplastic lymphocytes, macrophages, and monocytes. Flow cytometry demonstrated a reactive T-cell population. The patient demonstrated clinical improvement over time with spontaneous resolution of all retinal findings. CONCLUSION This case most likely represents immune recovery uveitis-like syndrome. Diagnostic vitrectomy is highly valuable when the differential includes inflammatory, infectious, and neoplastic processes.

Falls are the leading cause of death due to unintentional injuries in the older adult population, and fall-related death rates among older adults are escalating annually. Visual deficits are underrecognized and underdiagnosed, which increases fall risk. The purpose of this article is to provide a review of the common types of visual impairment, their etiology, and treatment and to present strategies to reduce falls among older adults with visual impairments. Both traditional home safety interventions and emerging technology-based interventions to reduce falls are described. Appropriate use of both traditional and emerging fall prevention interventions may reduce fall risk and falls among older adult home healthcare patients.

PURPOSE To report a rare case of multiple fundal colobomata and to review the possible etiopathogenic factors involved in its genesis. METHOD Case report. RESULT A 21-year-old myopic lady was noted to have 2 fundal colobomata-one in the inferior and the other in superior hemisphere of globe in a mirror-image fashion resembling an "hourglass" in the right eye and a typical Type 3 coloboma in the left eye. The iris was normal in both the eye. Multiple fundal colobomata have not been described in the literature. The presence of "accessory embryonic fissure" in the developing eyeball is the only probable mechanism that can explain this presentation. This case is an evidence for the possibility of presence of accessory embryonic fissure in developing eyeball in human. The eponym "hourglass coloboma" or "mirror-image colobomata" best describes this condition. CONCLUSION This report of multiple fundal colobomata is the first of its kind. This case is an evidence for possibility of presence of accessory embryonic fissure in developing eyeball in human.

Purpose To evaluate visual, refractive, aberrometric and patient-reported outcomes of wavefront-guided (WFG) myopic laser in situ keratomileusis (LASIK) using a high-resolution Hartmann-Shack aberrometer (iDesign Advanced WaveScan system, Johnson & Johnson Vision, Santa Ana, CA) with a new nomogram. The study was designed to determine if the new nomogram resolved the mild undercorrection that occurs with the manufacturer's default settings. Setting Three private LASIK practices. Design Prospective, open label, non-comparative, multicenter study. Methods One hundred ninety eyes of 95 patients underwent bilateral WFG LASIK for the correction of myopia or myopic astigmatism. A new nomogram was used, which effectively adjusted the wavefront-measured refraction sphere up or down to equal the manifest refraction sphere. Patients were followed for six months. Results Eighty-four patients completed final follow up. At 6 months, 96.4% (162/168) of eyes achieved monocular uncorrected distance visual acuity of 20/20 or better. No eye lost two or more lines of corrected distance visual acuity. The safety and efficacy indices were 1.12 and 1.09, respectively. 98% of eyes (164/168) had manifest refraction spherical equivalent within ±0.5 D of emmetropia and 92% (154/168) had residual manifest refractive astigmatism ≤0.5 D. Fewer patients experienced burning, stinging, soreness and irritation postoperatively than preoperatively. 96% (81/84) of patients reported improved quality of life. Conclusions WFG myopic LASIK using a high-resolution Hartmann Shack aberrometer and a new nomogram resolved the undercorrection with the manufacturer's default settings. The treatment was safe and effective with excellent visual and refractive outcomes, high patient satisfaction and improved quality of life.

The multiple radial stromal deep corneal incisions placed in radial keratotomy (RK) lead to higher-order aberrations and pose a surgical challenge to performing any further corrective procedure on the cornea because of fear of inducing an incisional dehiscence. A method to perform pinhole pupilloplasty (PPP) in the setting of previous RK is presented. Application of pinhole optics by performing PPP leads to significant improvement in image quality and helps to optimize visual potential in post-RK cases.

PURPOSE Visual outcomes after postinjection endophthalmitis have been well-studied, but the effect of endophthalmitis on the underlying exudative disease process remains unclear. We investigate the need for continued anti-vascular endothelial growth factor injections after endophthalmitis. METHODS Eyes that developed endophthalmitis after intravitreal injection of anti-vascular endothelial growth factor between January 1, 2016, and May 31, 2018, at a single academic retina practice were identified. Retrospective chart review was performed to determine 1) the proportion of eyes without recurrence of macular edema or subretinal fluid after endophthalmitis and 2) the proportion achieving a 12-week or greater interval between anti-vascular endothelial growth factor injections or exudation after endophthalmitis compared with internal controls before endophthalmitis. RESULTS Of 50 eyes with endophthalmitis, seven (14.0%) had no fluid recurrence at a mean of 98.1 week. Of 43 eyes with recurrence, 48.0% achieved a >12-week recurrence-free interval after endophthalmitis (vs. 8.3% before endophthalmitis; P < 0.0001). Eyes with compared to those without choroidal neovascularization were more likely to achieve this interval (60.5% vs. 8.3%, respectively; P = 0.002). CONCLUSION Endophthalmitis after anti-vascular endothelial growth factor injection is associated with relative stability of the underlying exudation. Further research is necessary to elucidate the mechanism, which may be useful in developing strategies and targets for the treatment of exudative macular diseases.

PURPOSE To characterize the true position of in-the-bag intraocular lenses (IOLs) relative to the limbus using ultrasound biomicroscopy and estimate scleral-sutured IOL positioning. METHODS This prospective single-center study included 70 eyes of 41 patients with in-the-bag posterior chamber IOLs. Four vertical ultrasound biomicroscopy captures were performed in each eye in the superior, inferior, nasal, and temporal quadrants. Postoperative biometric data were collected. The primary outcome was the vertical distance of the in-the-bag IOL from the sclerocorneal limbus. Secondary outcomes included anterior shift and refractive change of a theoretical scleral-sutured IOL using sclerotomies at 2.5 mm and 3 mm posterior to the limbus. RESULTS A total of 265 ultrasound biomicroscopy images were analyzed, including 64 superior, 69 inferior, 66 nasal, and 66 temporal. The true in-the-bag IOL position measured as distance posterior to the sclerocorneal limbus was 4.23 ± 0.56 mm superiorly, 4.22 ± 0.46 mm inferiorly, 3.95 ± 0.48 mm nasally, and 3.86 ± 0.52 mm temporally. The anterior shift of a theoretical scleral-sutured IOL was 0.60 mm for a 3-mm sclerotomy and 0.93 mm for a 2.5-mm sclerotomy, resulting in a theoretical myopic shift of 0.45 diopter (D) and 0.79 D, respectively, assuming a 15-D IOL. Larger biometric measurements correlated with a more posterior in-the-bag position. CONCLUSION True in-the-bag IOL position was found to be more posterior than estimates of scleral-sutured IOLs. Additional corrections in scleral-sutured IOL calculations may improve refractive outcomes.