Fungal panophthalmitis presenting as severe posterior scleritis.

Purpose: To report a highly unusual and fulminant case of infectious fungal panophthalmitis that initially presented as angle closure in the setting of posterior scleritis, culminating in the loss of the affected eye. Observations: A 57-year-old woman with a history of poorly controlled diabetes mellitus and autoimmune disease presented with a unilateral flat anterior chamber, highly elevated intraocular pressure (50-65 mmHg) and severe chemosis of the right eye. Initial VA was NLP in the affected eye. An ultrasound B-scan revealed a very pronounced T-sign and severely thickened posterior sclera and choroid indicative of posterior scleritis. Bloodwork showed elevation of WBC count to 18 K/µL and broad spectrum antibiotics were initiated. However, a comprehensive infectious workup including fungal cultures were persistently negative. After three days of IV NSAIDs and antibiotics, WBC count normalized and pain had mildly improved. After consultation with a multidisciplinary team that included the Glaucoma, Retina/Uveitis, Infectious Disease, Rheumatology and Internal Medicine services, high dose IV methylprednisolone was started. Despite the initial improvement, corneoscleral decompensation and paralimbal perforation of the globe occurred. The eye was enucleated, and pathologic examination revealed a dense focus of budding yeast in the vitreous cavity. Conclusions and importance: Scleritis is a rare entity, with posterior scleritis, infectious scleritis, and fungal scleritis representing increasingly rare subtypes. However, fungal scleritis may be underdiagnosed due to a number of factors including culture negativity, a lack of clinical suspicion, as well as the disease's propensity to masquerade as other pathologies such as angle closure or malignant glaucoma. Fungal scleritis should be considered in cases that present with possible infectious etiology, worsen with systemic corticosteroid treatment, or worsen despite broad-spectrum antibiotic coverage. When treating patients with underlying risk factors such as uncontrolled diabetes mellitus, recent antibiotic use, use of total parenteral nutrition, or immunosuppression, a higher level of suspicion for fungal etiology is also appropriate. In the outpatient setting, fungal eye infections do not always present with critical systemic illness or culture positivity. If there is suspicion for fungal involvement, early aqueous or vitreous tap may improve diagnostic yield.

Severe Multifocal Placoid Chorioretinitis Associated with Immune Checkpoint Inhibitor Therapy.

PURPOSE: To report a case of severe bilateral multifocal placoid chorioretinitis in a patient receiving ipilimumab and nivolumab therapy for metastatic melanoma. METHODS: Retrospective, observational case report. RESULTS: A 31-year-old woman on ipilimumab and nivolumab for metastatic melanoma developed severe multifocal placoid chorioretinitis in both eyes. The patient was started on topical and systemic corticosteroid therapy and immune checkpoint inhibitor therapy was paused. Following resolution of ocular inflammation, the patient was restarted on immune checkpoint inhibitor therapy without return of ocular symptoms. CONCLUSION: Extensive multifocal placoid chorioretinitis may occur in patients undergoing immune checkpoint inhibitor (ICPI) therapy. Some patients with ICPI-related uveitis may successfully resume ICPI therapy under close collaboration with the treating oncologist.

Hemorrhagic Occlusive Retinal Vasculitis Associated With Triamcinolone-Moxifloxacin Use During Uncomplicated Cataract Surgery.

This case series describes the outcomes of cataract extraction with intraocular lens placement in 2 patients.

Metformin suppresses pro-inflammatory cytokines in vitreous of diabetes patients and human retinal vascular endothelium.

Metformin is a traditional anti-hyperglycemic medication that has recently been shown to benefit vascular complications of diabetes via an anti-inflammatory mechanism other than glycemic control. This study aims to test the hypothesis that metformin suppresses diabetic retinopathy (DR) associated intraocular inflammation. Human vitreous from control and proliferative diabetic retinopathy (PDR) patients with or without long-term metformin treatment (> 5 years) were collected for multiple inflammatory cytokines measurements with a cytokine array kit. The vast majority of the measurable cytokines in PDR vitreous has a lower level in metformin group than non-metformin group. Although the p values are not significant due to a relatively small sample size and large deviations, the 95% confidence interval (CI) for the mean difference between the two groups shows some difference in the true values should not be neglected. Using quantitative ELISA, soluble intercellular adhesion molecule -1 (ICAM-1) and monocyte chemoattractant protein -1 (MCP-1) presented with significantly lower concentrations in metformin group versus non-metformin group. Metformin group also has significantly less up-regulated cytokines and diminished positive correlations among the cytokines when compared to non-metformin group. Possible role of AMP-activated protein kinase (AMPK) and nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κB) in metformin's anti-inflammatory effects were studied in human retinal vascular endothelial cells (hRVECs) cultured in normal glucose (NG) and high glucose (HG) conditions. Metformin inhibited HG-induced ICAM-1, IL-8, and MCP-1 via AMPK activation, whereas pharmacological AMPK inhibition had no effect on its inhibition of NF-κB p65, sICAM-1, and tumor necrosis factor-α (TNF-α). Metformin-induced suppression of the inflammatory cytokines could also be mediated through its direct inhibition of NF-κB, independent of AMPK pathway. This is a proof-of-concept study that found metformin treatment was associated with reduced inflammatory responses in vitreous of diabetes patients and retinal vascular endothelial cells, supporting the rationale for using metformin to treat DR at an early stage.

Pyramidal Inflammatory Deposits of the Retinal Pigment Epithelium and Outer Retina in Ocular Syphilis.

PURPOSE: To evaluate the role of OCT in the diagnosis of uveitis secondary to syphilis. DESIGN: Consecutive, retrospective case series. PARTICIPANTS: All patients 18 years of age or older with ocular syphilis from 2 tertiary referral centers. METHODS: All patients who were diagnosed with intermediate uveitis, posterior uveitis, or panuveitis secondary to syphilis were included in the study (40 patients representing a total of 62 eyes) to identify important imaging features to aid in diagnosis. Patients underwent confirmatory serologic testing, OCT imaging, and dilated examination by a uveitis specialist. MAIN OUTCOME MEASURE: Hyperreflective retinal lesions on OCT. RESULTS: The mean age of the study population was 42.9 ± 12.16 years. Forty-five percent of the eyes included in this study harbored hyperreflective pyramidal lesions of the outer retina and retinal pigment epithelium on OCT. Fifty-four percent of eyes with these imaging findings did not show a placoid retinal lesion on examination. Sixty-eighty percent of the described outer retinal lesions on OCT resolved after treatment for syphilis. Visual acuity ranged from normal (20/20) to no light perception, with a mean of 20/43 at diagnosis, and improved significantly to a mean visual acuity of 20/26 after treatment (P < 0.05). Vision-threatening complications were seen in less than 5% of eyes and included both treatable and irreversible causes of vision loss, including retinal detachment, cystoid macular edema, and optic neuropathy. CONCLUSIONS: Patients treated for uveitis secondary to syphilis achieve good visual recoveries. Outer retinal lesions seen on OCT are common and can serve as an additional imaging finding of the disease.

Posterior scleritis and acute posterior multifocal placoid pigment epitheliopathy: A case of painful chorioretinitis and review of the current literature.

PURPOSE: To describe a patient who developed concurrent acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and posterior scleritis. OBSERVATIONS: We describe a middle-aged woman that developed eye pain and photopsia. She was found to have a "T-sign" on ultrasound of the right eye and multiple, nearly confluent, ill-defined subretinal whitish lesions in both eyes. After an extensive laboratory evaluation and neuroimaging, her photopsia, pain with eye movements, and subretinal lesions began to regress on high dose systemic corticosteroids. CONCLUSIONS AND IMPORTANCE: This is the first reported case of bilateral APMPPE and concurrent posterior scleritis. Our case highlights the importance of performing a full review of systems, specifically eliciting neurological changes, and dilated eye examination in all new uveitis cases.

Temporally independent association of multiple evanescent white dot syndrome and optic neuritis.

PURPOSE: To describe three patients that developed temporally distinct episodes of optic neuritis and multiple evanescent white dot syndrome (MEWDS). METHODS: We retrospectively reviewed the medical records and imaging studies of three women evaluated at a tertiary referral center for both optic neuritis and MEWDS. RESULTS: Three otherwise healthy women, aged 17, 36, and 41, developed temporally separated episodes of optic neuritis and MEWDS. The time periods between the two events were 3, 48, and 60 months, and in two of the three cases, the optic neuritis event preceded the episode of MEWDS. No patient endorsed prodromal flu-like symptoms prior to developing vision loss. The mean presenting visual acuities were better with the optic neuritis episode (LogMAR 0.360, Snellen 20/46) than with retinal event (LogMAR 0.684, Snellen 20/97). All three patients had improvement in vision, with mean visual acuity of 20/29 (LogMAR 0.165) at last follow-up. One patient later developed idiopathic noninfectious posterior uveitis and another developed multiple sclerosis requiring treatment. CONCLUSION: While a rare association, patients can develop both optic neuritis and MEWDS within the same eye at different time points. It is unknown whether such patients are at even higher risk of developing systemic autoimmune disease than are patients with either MEWDS or optic neuritis alone.

RETINAL NEOVASCULARIZATION FROM A PATIENT WITH CUTIS MARMORATA TELANGIECTATICA CONGENITA.

PURPOSE: To report a rare case of peripheral retinal neovascularization in a patient diagnosed with cutis marmorata telangiectatica congenita (CMTC). METHODS: Observational case report. RESULTS: A 16-year-old girl was referred to clinic for retinal evaluation. The patient had a clinical diagnosis of CMTC later confirmed by skin biopsy. Examination revealed temporal peripheral retinal sheathing, as well as lattice degeneration in both eyes. Wide-field fluorescein angiogram showed substantive peripheral retinal nonperfusion with evidence of vascular leakage from areas of presumed retinal neovascularization. The patient subsequently had pan retinal photocoagulation laser treatment to each eye without complication. DISCUSSION: Cutis marmorata telangiectatica congenita is a rare vascular condition known to affect multiple organ systems including the eyes. Although ocular manifestations of CMTC are rare, instances of congenital glaucoma, suprachoroidal hemorrhage, and bilateral total retinal detachments resulting in secondary neovascular glaucoma have been reported. Our patient demonstrates the first reported findings of peripheral nonperfusion and retinal neovascularization related to CMTC in a 16-year-old girl. We propose early retinal examination, wide-field fluorescein angiogram, and early pan retinal photocoagulation laser treatment in patients with peripheral nonperfusion and retinal neovascularization from CMTC.

BILATERAL ENDOGENOUS ENDOPHTHALMITIS FROM STREPTOCOCCUS PNEUMONIAE.

PURPOSE: To report a case of bilateral endogenous endophthalmitis from Streptococcus pneumoniae with devastating sequelae. METHODS: Interventional case report. RESULTS: A 56-year-old man presented with acute bilateral blurred vision and floaters with fever and hemodynamic instability. Previously, he was diagnosed with acute otitis externa and reported manually extracting several of his own teeth. He underwent a vitreous tap and intravitreal antibiotic injections. Blood and vitreous cultures were positive for S. pneumoniae. The patient later developed life-threatening medical sequelae. His final visual acuity was no light perception in the right eye and 20/25 in the left eye. CONCLUSION: Diagnosing endogenous endophthalmitis early is essential to initiating a systemic evaluation for potentially life-threatening medical conditions, including sepsis, endocarditis, and osteomyelitis. A high degree of suspicion, expeditious treatment, and interdisciplinary collaboration are essential to maximizing patient outcomes.

Ciliary Body Mass as a Feature of Ocular Sarcoidosis.

Importance: Ocular sarcoidosis may be the primary and only clinical manifestation of sarcoidosis, and irreversible loss of visual acuity may occur from delayed treatment. Thus, early diagnosis is imperative to preserve visual acuity. Establishing the diagnosis of sarcoidosis in cases manifesting only in the eye may require a diagnostic biopsy, which may prove challenging in the absence of available ocular surface, lacrimal gland, and systemic tissue for biopsy. Objective: To describe diffuse or segmental ciliary body mass as a clinical sign and tissue source for biopsy in the diagnosis of ocular sarcoidosis. Design, Setting, and Participants: This case series captures 3 cases from a university-based ocular oncology and uveitis practice. All 3 patients presented with uveitis and a diffuse or segmental ciliary body mass, which was identified via ultrasonographic biomicroscopy. Main Outcomes and Measures: Clinical, ancillary, and histopathological ocular findings in the 3 patients. Results: The patients were a 52-year-old white woman, a 42-year-old African American man, and an 81-year-old white man. Two individuals had unilateral involvement, and 1 had bilateral involvement. All 3 individuals presented with uveitis. Diffuse, circumferential ciliary body masses were observed in 2 patients. A localized, nodular ciliary body mass on the temporal quadrant with superior and inferior extensions was observed in 1 patient. Moderate to high internal reflectivity was observed on ultrasonographic biomicroscopy in all 3 individuals. Biopsies of the ciliary body masses were diagnosed as sarcoidosis in all 3 patients. Conclusions and Relevance: These findings support that diffuse or segmental ciliary body mass may serve as a clinical sign and tissue source for biopsy in the diagnosis of ocular sarcoidosis, although the frequency of this cannot be determined from 3 cases. To our knowledge, such cases all demonstrate a uveitic component; whether a ciliary body mass caused by sarcoidosis can occur in the absence of uveitis remains unknown.

Survey of Intravitreal Injection Practice Patterns Among Retina Specialists.

Purpose: Intravitreal injection therapy (IVT) is the most performed procedure in ophthalmology. This study was conducted to determine current trends in IVT delivery. Methods: An online, 31-question, multiple-choice survey was sent to 1677 retina specialists. The survey consisted of 3 sections: general questions, procedure technique, and postprocedure technique. Results: A total of 264 (16%) retina specialists completed the survey. The use of povidone-iodine (100%) and small-gauge needles (97%) was common, whereas ocular anesthesia was split among lidocaine gel (31%), lidocaine drops (25%), subconjunctival lidocaine (28%), and lidocaine-soaked pledgets (15%). More than 85% indicated povidone-iodine contributes to post-IVT corneal toxicity, and 12% reported that a needlestick injury to physician or staff occurred during IVT. Conclusions: Key areas for IVT improvement include optimized ocular anesthesia, development of a guarded needle for ocular drug delivery, and formulation of a less toxic ocular antiseptic.

Prefilled syringes for intravitreal drug delivery.

Intravitreal injections of anti-vascular endothelial growth factor (VEGF) medications play an increasingly critical role in numerous retinal vascular diseases. Initially, anti-VEGF medications came in vials that had to be drawn up by the physician into a syringe for administration. In 2018, the US Food and Drug Administration (US FDA) approved the ranibizumab 0.3 mg prefilled syringe (PFS), and in October 2016, the US FDA approved the ranibizumab 0.5 mg PFS. This article discusses the advantages of the PFS, including reduced injection time, possible reduced risk of endophthalmitis, reduction in intraocular air bubbles and silicone oil droplets, and improved precision in the volume and dose of intravitreal ranibizumab administered, along with possible disadvantages. Implications of the innovation of the PFS on intravitreal injection technique and clinical practice pattern are discussed and reviewed.

Chorioretinal Perfusion in Persistent Placoid Maculopathy.

Persistent placoid maculopathy (PPM) is a rare clinical entity that has previously been reported in the literature with a characteristic appearance on multimodal imaging, including hypofluorescence observed on fluorescein angiogram (FA) and indocyanine green angiography (ICGA). The leading mechanisms proposed for this hypofluorescence include impaired choroidal vasculature with nonperfusion of the choriocapillaris and/or blockage of choroidal fluorescence by inflammatory deposits. This report demonstrates previously unreported characteristics of chorioretinal perfusion in a case of acute onset PPM in a 58-year-old woman. Optical coherence tomography angiography confirmed hypoperfusion of the choriocapillaris corresponding to the hypoperfusion seen on FA and ICGA. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:1026-1031.].

BASAL CELL NEVUS SYNDROME PRESENTING AS EPIRETINAL MEMBRANE AND MYELINATED NERVE FIBER LAYER.

PURPOSE: To report a case of epiretinal membrane and myelinated nerve fiber layer, which preceded the diagnosis of basal cell nevus syndrome, in a young girl. METHODS: Observational case report. RESULTS: A 12-year-old girl was referred for an asymptomatic epiretinal membrane. Examination revealed epiretinal membrane in the right eye without posterior vitreous separation or vitreous abnormality and bilateral myelinated nerve fiber layer. Subsequent workup yielded pathologic diagnosis of multiple skin basal cell carcinoma and odontogenic keratocysts in the jaw. Genetic testing revealed a frameshift mutation in the PTCH1 gene. CONCLUSION: Basal cell nevus syndrome is a rare autosomal dominant disease that affects multiple organ systems, including the eyes. Recognition of common ocular findings in children with basal cell nevus syndrome can lead to systemic diagnosis. Early diagnosis is critical to initiate early screening for known neoplastic associations and lifelong minimization of sun exposure to reduce the incidence and severity of basal cell carcinoma.