Evolution of Autoimmune Retinopathy in Stiff Person Syndrome: A Case Report.

PURPOSE: To report a case of autoimmune retinopathy (AIR) as the presenting feature of Stiff Person Syndrome (SPS) and assess its evolution. OBSERVATIONS: A 35-year-old man presented with progressive, chronic, vision loss. On initial examination, visual acuity measured 20/20 OD and 20/50 OS. Humphrey Visual Field testing (HVF) demonstrated decreased foveal threshold OU. Mild subfoveal ellipsoid zone loss was noted on Optical Coherence Tomography (OCT). Five years later the patient presented with painful lower extremity muscle spasms and stiffness and complained of increasing vision loss with difficulty distinguishing colors. OCT showed marked progression of ellipsoid zone loss. Scotoma were demonstrated on HVF and electroretinography demonstrated reduced responses consistent with bilateral severe maculopathy. Serum testing showed autoantibodies to the glutamic acid decarboxylase 65-kilodalton isoform (GAD65) at a high titer and a diagnosis of AIR in the setting of SPS was made. A systemic workup for malignancy was negative. The patient was treated with IVIG and transitioned to rituximab with improvement in systemic symptoms. CONCLUSIONS: and Importance: Unlike previous cases of AIR in the setting of SPS, vision symptoms and OCT changes presented years before the onset of muscle spasms. Etiologies such as SPS should be on the differential of unexplained retinopathy, even in the absence of systemic symptoms, especially when paraneoplastic etiologies are ruled out.

Google Search Trends to assess public interest in and concern about Vuity for treating presbyopia.

PURPOSE: To assess public awareness, interest, and concerns regarding Vuity (1.25% pilocarpine hydrochloride ophthalmic solution), an eye drop for the treatment of presbyopia, based on Google Trends. METHODS: We used Google Trends that provides a relative search volume for queried terms, to evaluate searches for Vuity from June 30, 2021, to June 30, 2022, in the United States. The data for this study were downloaded on June 30, 2022. Main outcome measures were changes in relative search volumes for the terms "Vuity," "Eye drops for reading," "Eye drops for near vision," "Presbyopia," "Pilocarpine," and related popular search terms, such as "Vuity side effects," and "Vuity retinal detachment". RESULTS: Since the approval of Vuity on October 29, 2021, notable increases in the relative search volumes occurred for Vuity in October 2021, December 2021, and from March to April 2022, which coincided with its approval, availability, and subsequent direct-to-consumer advertising based on positive results of clinical trials. The direct-to-consumer advertising had the greatest impact on the search volume for Vuity. Specific interests included Vuity cost, where to buy it, and its side effects. Retinal detachment was the most highly searched Vuity side effect. Geographic variation was evident, with the relative search volumes highest for "Vuity" in Wyoming, followed by North Dakota. CONCLUSION: Google Trends is a useful tool for monitoring increases in public interest in Vuity for presbyopia. Public concerns regarding side effects warrant further real-world investigations of the causal relationship between Vuity and retinal detachment.

PSEUDOMONAS ENDOPHTHALMITIS AFTER 0.59 MG FLUOCINOLONE ACETONIDE SURGICAL IMPLANT IN A PATIENT WITH A LONG-STANDING CRAWFORD TUBE.

PURPOSE: The purpose of this study was to report a case of Pseudomonas aeruginosa endophthalmitis after surgical 0.59 mg fluocinolone acetonide implant in a patient with a long-standing Crawford tube. METHODS: This was a retrospective case review. RESULTS: A 52-year-old woman with a history of bilateral sarcoid-associated panuveitis and nasolacrimal duct obstructions treated with dacryocystorhinostomies and long-standing Crawford tubes underwent placement of a surgical fluocinolone acetonide implant. The Crawford tube was visible throughout the surgery and notably exhibited small amounts of rotation and prolapse with manipulation of the eye. On postoperative Day 4, the patient presented urgently with pain and decreased visual acuity. Endophthalmitis was suspected, and a vitreous tap and intravitreal injections of vancomycin and amikacin were performed. Cultures grew P. aeruginosa. Initially she responded to treatment with no evidence of intraocular infection or inflammation by postoperative Week 3. However, at postoperative Week 4, the patient returned with a yellow purulent subconjunctival nodule and surrounding scleral injection. A second nodule appeared 2 weeks later. The patient was treated with topical and systemic antibiotics. The nodules responded well to treatment showing notable consolidation and revealing an area of scleral thinning as they regressed. CONCLUSION: We present a case of P. aeruginosa endophthalmitis and presumed scleritis after the surgical fluocinolone acetonide implant placement in an eye with a Crawford nasolacrimal tube effectively treated with topical, intravitreal, and systemic antibiotics. Long-standing nasolacrimal duct hardware may allow reflux of nasopharyngeal and nasolacrimal bacteria, contaminating the ocular surface during surgery.

Primary Vitreoretinal Lymphoma Masquerading as Post-Operative Endophthalmitis in a Pregnant Patient with Longstanding Idiopathic Panuveitis.

PURPOSE: To report primary vitreoretinal lymphoma after surgical 0.59 mg fluocinolone acetonide implant (FAi) exchange in a patient treated with adalimumab for idiopathic bilateral panuveitis. METHODS: Retrospective case review. RESULTS: A 37-year-old woman with bilateral idiopathic panuveitis, who had favorable responses to previous FAi surgical implants, presented with right eye recurrent intraocular inflammation and cystoid macular edema that partially responded to systemic adalimumab. Her FAi was replaced, given her previous favorable response. She developed post-operative ocular inflammation transiently responsive to two serial vitreous taps and injections of intravitreal antimicrobials and then worsening inflammation and new layered flocculant material. Diagnostic vitrectomy showed a few atypical lymphocytes and cultures were negative. At post-diagnostic vitrectomy month 1, flocculant material recurred. Aqueous cytology and flow cytometry revealed large CD45 positive B-cells suspicious for lymphoma. Post-operatively, she revealed that she was pregnant. She was treated with 8 monthly intravitreal methotrexate injections and post-partum consolidation radiotherapy. Subsequent repeat cytology, flow cytometry, and corneal pathology revealed large B-cells that were CD20 positive, and next generation sequencing detected a dominant monoclonal B-cell population, diagnostic of PVRL. 19 months after FAi exchange, she developed an area of enhancement in the lateral aspect of the right frontal lobe on brain MRI, consistent with central nervous system involvement. CONCLUSION: We present a unique case of PVRL masquerading as post-operative endophthalmitis after FAi exchange in an eye with chronic panuveitis treated with adalimumab immunosuppressive therapy. We hypothesize that there may be a causal relationship between adalimumab and PVRL.

0.18 MG FLUOCINOLONE ACETONIDE INSERT FOR THE TREATMENT OF CHRONIC POSTOPERATIVE PSEUDOPHAKIC CYSTOID MACULAR EDEMA.

PURPOSE: To report the outcomes of the 0.18 mg fluocinolone acetonide insert (FAi) in the treatment of chronic (>6 months) postoperative cystoid macular edema after cataract surgery. METHODS: This was a retrospective consecutive case series of eyes with chronic postoperative cystoid macular edema treated with the FAi. Visual acuity, intraocular pressure, optical coherence tomography metrics, and supplemental therapies were extracted from the charts before and at 3, 6, 12, 18, and 21 months after FAi placement, when available. RESULTS: Nineteen eyes of 13 patients with chronic postoperative cystoid macular edema after cataract surgery underwent FAi placement with an average follow-up of 15.4 months. Ten eyes (52.6%) had a ≥2-line gain in visual acuity. Sixteen eyes (84.2%) had a ≥20% reduction in optical coherence tomography central subfield thickness. Eight eyes (42.1%) had complete resolution of CME. Improvements in central subfield thickness and visual acuity were sustained throughout individual follow-up. Compared with 18 eyes (94.7%) requiring local corticosteroid supplementation before FAi, only six eyes (31.6%) required supplementation after FAi. Similarly, of the 12 eyes (63.2%) that were on corticosteroid drops before FAi, only 3 (15.8%) required drops after FAi. CONCLUSION: Eyes with chronic postoperative cystoid macular edema after cataract surgery treated with the FAi had improved and sustained visual acuity and optical coherence tomography metrics, along with a reduction in supplemental treatment burden.

A Unique Case of Syphilitic Hypopyon Panophthalmitis.

Purpose: This work reports a unique case of syphilitic hypopyon panophthalmitis. Methods: A case report is presented. Results: A 25-year-old man with a history of HIV and intravenous (IV) drug use presented to an outside hospital with blurred vision and swelling of the right eye. Computed tomography was concerning for orbital cellulitis. On examination, he had limited extraocular motility, relative exophthalmos, periocular edema, 4+ anterior chamber cell, an irregular layering hypopyon, and no view of the fundus. Magnetic resonance imaging showed enhancement of the sclera, lateral rectus muscle, and lacrimal gland concerning for infectious or inflammatory panophthalmitis. The patient's history and presentation were concerning for bacterial or fungal etiologies from an endogenous source. He began antimicrobial therapy. Diagnostic vitrectomy was unrevealing. Syphilis testing was positive. The patient showed improvement with IV antiluetic therapy. Conclusions: We present a case of syphilitic hypopyon panophthalmitis, a new constellation of findings in syphilitic ocular disease.

Update on Retinal Drug Toxicities.

PURPOSE OF REVIEW: This review aims to provide an update on the clinical presentations and diagnostic findings of drug-induced retinal toxicities. RECENT FINDINGS: Several newly FDA-approved medications have been associated with acute retinal toxicities, including brolucizumab, MEK inhibitors, ulixertinib, and FGFR inhibitors. Additionally, as previously believed-to-be well-tolerated medications, such as pentosan sulfate sodium, anti-retroviral therapies, and certain intraoperative ocular medications, are used more frequently or for longer periods of time, associated toxic retinopathies and inflammatory reactions have been reported. Finally, advances in ocular imaging have revealed novel findings in hydroxychloroquine and tamoxifen maculopathies. SUMMARY: Discovery of new medications, increased frequency of use, and longer-term use have led to increased reports of retinal toxicities. Advances in retinal imaging have allowed for earlier detection of subclinical changes associated with these medications, which may help prevent progression of disease. However, more research is needed to determine the point at which vision loss becomes irreversible. Risks and benefits must be assessed prior to discontinuation of the offending, but potentially lifesaving, therapy.

Bilateral infectious scleritis from Histoplasma capsulatum in an immunosuppressed uveitis patient.

PURPOSE: To describe a case of bilateral infectious scleritis secondary to Histoplasma capsulatum in the setting of a locally and systemically immunosuppressed patient. OBSERVATIONS: A 45-year-old man with HLA-B27 associated ankylosing spondylitis and anterior uveitis on systemic secokinumab, underwent bilateral cataract extraction which required extensive peri-operative steroids, including intravitreal triamcinolone, topical prednisolone, and oral prednisone. Six weeks after cataract surgery, the patient presented with mild eye irritation and was found to have bilateral subconjunctival purulence and necrosis. Histoplasma capsulatum was identified on fungal cultures and confirmed by DNA probe. The patient was treated with fortified amphotericin drops and oral itraconazole with complete resolution of scleritis. CONCLUSION AND IMPORTANCE: H. capsulatum is a rare cause of infectious scleritis that must be considered in our immunosuppressed and post-surgical patients.

Clinical and Multimodal Imaging Findings in Disseminated Mycobacterium Chimaera.

PURPOSE: To characterize the ophthalmic clinical and multimodal imaging findings of disseminated Mycobacterium chimaera infection after cardiothoracic surgery. DESIGN: Observational case series. PARTICIPANTS: Four patients (8 eyes) with disseminated M. chimaera infection. METHODS: Patients were evaluated with biomicroscopy, OCT and OCT angiography, fundus autofluorescence, and fluorescein and indocyanine green angiography. MAIN OUTCOME MEASURES: Clinical and multimodal imaging findings of patients with disseminated M. chimaera infection. RESULTS: All 4 patients were white men with a mean age of 65.5 years (range, 60-75 years) who had aortic valve or root infection, or both, with M. chimaera diagnosed by culture, gene sequencing, or both. All 4 patients demonstrated bilateral choroidal lesions on funduscopy and evidence of osteomyelitis by imaging, culture analysis, or both at the time of ocular diagnosis. Indocyanine green and OCT angiography revealed numerous additional subclinical choroidal lesions and were used to track disease response to therapy. Fluorescein angiography and fundus autofluorescence were useful in determining lesion age and activity. All patients were treated with 3- or 4-drug antimycobacterial therapy. Three underwent revision of cardiothoracic surgery with removal of infected graft. One patient went on to demonstrate progressive ocular disease that was noted before each of his surgical revisions. Two patients showed improvement in ocular and systemic disease, however one of them developed a choroidal neovascular membrane. The final patient was a single encounter whose clinical and imaging findings showed longstanding inactive disease. CONCLUSIONS: Ophthalmologists should be aware of the systemic and ocular findings of this rare life-threatening disease. Multimodal imaging is useful in corroborating a diagnosis of ocular M. chimaera and particularly in evaluating patient response to therapy, because choroidal activity seems to mimic systemic activity. Treating physicians should be aware of the co-occurrence of choroiditis and osteomyelitis. Choroidal neovascular membrane can also be a late complication of this disease.

Recurrence Rates of Inflammation after Switching from the Originator Infliximab to Biosimilar Infliximab-abda for Noninfectious Uveitis.

PURPOSE: To describe the frequency of ocular flares in patients with noninfectious uveitis who were switched from the originator infliximab to a biosimilar infliximab. DESIGN: Retrospective case series. METHODS: All patients with noninfectious uveitis who were switched from the originator infliximab to biosimilar infliximab-abda for nonmedical reasons were reviewed. Patients were excluded if they had less than 3 months of follow-up on either drug. Data included patient demographics, infliximab dosage information, additional immunosuppression medications, and numbers of and times to flares. The main study outcome was frequency of flares, defined as new or worsening inflammatory activity on examination or imaging. RESULTS: A total of 17 patients met the inclusion criteria. There were no statistical differences between the duration of follow-up while on the originator and the duration while on the biosimilar infliximab (12.0 vs. 10.1 months, respectively; P = .307). Patients experienced more flares per person-years after switching to infliximab-abda (.92), than on the originator infliximab (0.19; P = .028). Four of the 6 patients (66.7%) who experienced flare after switching to infliximab-abda did so within 90 days. Only 1 patient had flares while on originator infliximab went on to develop a single flare on infliximab-abda. The final normalized dosage for patients who flared and remained on infliximab-abda (1.301 mg/kg/week) was higher than that for those who did not flare (1.186 mg/kg/week) but was not statistically significant (P = .417). CONCLUSIONS: Patients who were switched to biosimilar infliximab-abda experience more flares than when previously treated with the originator infliximab. Providers should closely observe patients who switch to biosimilar infliximab, especially within the first 90 days. Patients who do have flares after switching may achieve quiescence with increased biosimilar dosage.

Acute retinal necrosis following recombinant subunit varicella-zoster virus vaccine.

PURPOSE: Previously, secondary prevention of herpes zoster required live-attenuated vaccination, which is contraindicated in immunocompromised populations. More recently, a recombinant subunit vaccine (Shingrix, GlaxoSmithKline, Research Triangle Park, North Carolina) was approved by the Food and Drug Administration. Iatrogenic varicella-zoster virus (VZV) infection is theoretically impossible as it does not contain a live virus. We present a case of acute retinal necrosis (ARN) and disseminated zoster after receiving the recombinant subunit vaccine. OBSERVATIONS: A 65-year-old woman with past medical history of multiple myeloma treated with a previous autologous hematopoietic stem cell transplant and now with daratumumab and pomalidomide developed disseminated zoster and subsequently acute retinal necrosis weeks after receiving the zoster subunit vaccine. Molecular testing confirmed the presence of VZV, and the absence of herpes simplex virus, cytomegalovirus, and toxoplasmosis. The VZV was found to be genotypically wildtype and not related to the Oka strain used in the live-attenuated zoster vaccine. She was treated with systemic valacyclovir and intravitreal foscarnet. CONCLUSIONS AND IMPORTANCE: This is the first report of VZV infection following the zoster subunit vaccine. The Advisory Committee on Immunization Practices (ACIP) has recommended the recombinant subunit vaccine over the live-attenuated vaccine due to its superior efficacy. The off-label use of the subunit vaccine in immunocompromised populations has been supported up to this point by studies demonstrating its relative safety. Though post-vaccination VZV infection or reactivation appears to be rare, clinicians should be aware of this potential complication to the recombinant subunit vaccine.

Orbital Cellulitis Following Uncomplicated Glaucoma Drainage Device Surgery: Case Report and Review of Literature.

PURPOSE: Orbital cellulitis (OC) is a rare postoperative complication of glaucoma drainage device (GDD) implantation. To date, there have only been 10 reported cases of OC following GDD implantation. CASE REPORT: Here, we report a case of OC in a 57-year-old man who developed pain, proptosis, and limited extraocular motility two days after uneventful Ahmed FP7 implantation in the right eye. Contrast-enhanced computed tomography of the orbits demonstrated fat stranding and a small fluid collection, consistent with OC. He had minimal improvement with intravenous antibiotics and ultimately underwent GDD explantation. A systematic review of the literature showed that the development of OC following GDD implantation can occur in the early or late postoperative period. Immediate hospitalization with intravenous administration of broad-spectrum antibiotics is recommended. Explantation of the infected GDD is often required for source control. CONCLUSION: OC is a rare postoperative complication of GDD implantation. Prompt evaluation and treatment are required, often combined with GDD explantation.

Visual recovery and vascular reperfusion after vaso-occlusive retinopathy from anti-phospholipid syndrome associated with systemic lupus erythematosus.

PURPOSE: To report a case of visual recovery and vascular reperfusion after vaso-occlusive retinopathy from anti-phospholipid syndrome associated with systemic lupus erythematosus. OBSERVATIONS: A 15-year-old boy with a known diagnosis of systemic lupus erythematosus and a clinically significant anti-phospholipid panel presented with sudden vision loss in the left eye. Examination and ocular imaging revealed signs of vaso-occlusive retinopathy. The patient was immediately started on high dose intravenous steroids, followed by mycophenolate mofetil. He remained on aspirin. After showing no improvement in retinal arteriole and capillary perfusion he was started on therapeutic anti-coagulation with enoxaparin. He regained 20/20 vision. Intravenous fluorescein angiography demonstrated reperfusion of retinal arterioles. Optical coherence tomography angiography showed return of flow in the capillary networks. CONCLUSIONS: We present a case of vaso-occlusive retinopathy in a patient with known systemic lupus erythematosus and a clinically significant anti-phospholipid panel, thus meeting criteria for anti-phospholipid syndrome. He was treated with intravenous methylprednisolone, mycophenolate motefil, aspirin, and enoxaparin. The patient not only had great recovery of visual acuity, but also demonstrated reperfusion of arterioles and reconstitution of flow in the retinal capillary network. These findings suggest that the vaso-occlusive disease is reversible if the diagnosis is made promptly and intensive therapy is initiated. IMPORTANCE: Currently there are no reported cases of vaso-occlusive retinopathy from APLS and SLE with visual recovery, reperfusion, and return of capillary flow.

PPV, Retinectomy, and Silicone Oil Without Scleral Buckle for Recurrent RRD From Proliferative Vitreoretinopathy.

BACKGROUND AND OBJECTIVE: To analyze the anatomic success rate of pars plana vitrectomy (PPV), retinectomy, and silicone oil (SO) tamponade without scleral buckle (SB) for repair of recurrent rhegmatogenous retinal detachment (RRD) associated with proliferative vitreoretinopathy (PVR). PATIENTS AND METHODS: Retrospective, consecutive, single-surgeon case series of 28 eyes of 28 patients with PVR-associated RRD repaired with PPV, retinectomy, and SO tamponade without SB. RESULTS: The single-procedure anatomic success rate was 85.2% at 3 months and 82.1% at 12 months. Final reattachment rate was 100.0%. There were no preoperative factors that predicted single procedure anatomic success. Mean logarithm of the minimal angle of resolution visual acuity (VA) was improved at 3 months (1.61 to 1.51, P = .732) and at 12 months (1.61 to 1.41; P = .271). VA outcome was related to preoperative macula and lens status. CONCLUSION: The single-procedure anatomic success rate of PPV, retinectomy, and SO tamponade without SB for PVR-related recurrent RRD is comparable to prior reports of similar surgery incorporating SB. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:e278-e287.].

Pneumatic Retinopexy Experience and Outcomes of Vitreoretinal Fellows in the United States: A Multicenter Study.

Objective: To evaluate the outcomes of patients undergoing pneumatic retinopexy (PR) performed by vitreoretinal fellows at 6 academic centers in the United States. Design: Retrospective, multicenter, consecutive case series. Participants: 483 patients with primary retinal detachments who underwent PR by 49 vitreoretinal fellows from 6 U.S. training sites between 2002 and 2016. Methods: We reviewed medical records of patients and recorded baseline clinical characteristics (age, sex, baseline visual acuity, lens status, presence of lattice degeneration, presence of vitreous hemorrhage, location of retinal breaks, macular status, and size of detachment), visual and anatomic outcomes at 3-months after PR, as well as training level and PR experience of the fellow at the time of the procedure. Main Outcome Measures: Single-procedure anatomic success and visual acuity at 3-months follow-up, and association with clinical and training-related factors. Results: Vitreoretinal fellows performed a variable number of PR, with a median of 7 cases per fellow (range 1-24). Single-procedure anatomic success was 66.8%, and mean LogMAR visual outcome was 0.43 (Snellen equivalent 20/54) at 3 months. Factors that were independently associated with single-procedure success include phakic lens status (P = 0.01), smaller size of retinal detachment (P = 0.02), and the fellow's procedure experience (P = 0.01). The only factor associated with worse visual outcome was baseline visual acuity (P < 0.001). Conclusion: Vitreoretinal fellows perform variably few pneumatic retinopexies but have outcomes comparable to reported rates by experienced specialists. Procedure experience of individual fellows may impact anatomic outcomes.

Outcomes of Pars Plana Vitrectomy for Epiretinal Membrane in Eyes With Coexisting Dry Age-related Macular Degeneration.

PURPOSE: There has been limited evidence on the benefits of pars plana vitrectomy with membrane peel (PPV-MP) for epiretinal membrane (ERM) in eyes with dry age-related macular degeneration (AMD). We sought to assess anatomic and functional outcomes of PPV-MP for ERM in this subset of eyes. DESIGN: A retrospective cohort study. PARTICIPANTS: Patients with dry AMD who underwent PPV-MP for ERM from January 1, 2010, to December 1, 2016. METHODS: Visual acuity (VA) and central foveal thickness (CFT) as measured on spectral-domain OCT were recorded and analyzed for the preoperative, 6-month, and final follow-up visits. The presence of cystoid macular edema (CME) and ellipsoid zone (EZ) integrity were recorded and compared with postoperative imaging. Conversion to neovascular AMD in eyes for which at least 2 years of follow-up were available, as confirmed by either OCT and/or fluorescein angiography and documentation of treatment with intravitreal antivascular endothelial growth factor, was recorded and compared between case eyes that underwent PPV-MP versus fellow control eyes. MAIN OUTCOME MEASURES: Postoperative VA. RESULTS: A total of 38 eyes from 38 patients met the study criteria. There was a significant improvement in the median (interquartile range, [IQR]) logarithm of the minimum angle of resolution [logMAR] VA from 0.60 (IQR 0.46-1.00) (20/80, Snellen equivalent) at the preoperative visit, to 0.48 (IQR 0.30-0.70) (20/60, Snellen equivalent) at the 6-month follow-up visit (P = 0.04), and to 0.48 (IQR 0.30-0.70) (20/60, Snellen equivalent) at the final visit (P = 0.01). There was a significant median decrease in CFT at the final visit (P < 0.001) compared with the preoperative CFT. Only eyes with either CME or an intact EZ showed significant improvement in median logMAR VA at the final visit compared with the preoperative visit (P = 0.01 and P = 0.004, respectively). In a subgroup analysis of eyes for which a minimum of 2 years of follow-up were available, 4 of 25 (16.0%) vitrectomized eyes and 1 of 25 (4.0%) fellow control eyes progressed to neovascular AMD (P = 0.16). CONCLUSIONS: PPV-MP appears to confer anatomic and functional improvement in eyes with ERM and coexisting dry AMD. Moreover, greater preoperative CFT, the presence of CME, and an intact EZ were predictors of VA improvement in these eyes.