CATASTROPHIC, BILATERAL RETINAL VASCULAR OCCLUSION AFTER INTRAVITREAL BEVACIZUMAB INJECTION.

PURPOSE: To describe two cases of catastrophic, bilateral retinal vascular occlusion after intravitreal (IVT) bevacizumab injection. METHODS: Case series. Main outcome measures included clinical and fluorescein angiography findings. RESULTS: Case 1-A 65-year-old woman with calcinosis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasis syndrome developed acute, severe, bilateral visual loss 2 weeks after bilateral IVT bevacizumab injection for proliferative diabetic retinopathy. Examination and fluorescein angiography revealed moderate anterior chamber inflammation, bilateral perivascular retinal hemorrhages, and near total retinal vascular occlusion. Extensive testing revealed moderately elevated anti-B2 glycoprotein (antiphospholipid) antibodies. Case 2-An 85-year-old man with polymyalgia rheumatica and left eye exudative age-related macular degeneration experienced severe, bilateral, sequential visual loss in the left eye and then right eye approximately 3 weeks after IVT bevacizumab left eye injection. Examination revealed bilateral panuveitis, diffuse perivascular exudates, and intraretinal hemorrhages. Fluorescein angiography showed diffuse venous leakage. Extensive testing revealed an elevated antinuclear antibody and mildly elevated anticardiolipin antibody. CONCLUSION: Patients with underlying retinal vascular vulnerabilities may be at increased risk of catastrophic, bilateral retinal vascular occlusion after treatment with IVT bevacizumab. The moderate-to-severe intraocular inflammation in both cases and the contralateral involvement after unilateral IVT injection in Case 2 suggest a possible delayed immune-mediated mechanism.

NIVOLUMAB-INDUCED HARADA-LIKE UVEITIS WITH BACILLARY DETACHMENT MIMICKING CHOROIDAL METASTASIS.

PURPOSE: To describe a patient with metastatic clear cell renal cell carcinoma in remission on maintenance nivolumab therapy who developed late-onset ocular toxicity manifesting as creamy chorioretinal lesions with exudative retinal detachment concerning for choroidal metastasis. METHOD: Case report. Main outcome measures include ophthalmoscopic examination, fundus photography, fundus autofluorescence, fluorescein angiography, indocyanine green angiography, spectral domain optical coherence tomography, and B-scan ultrasonography. RESULTS: A 49-year-old woman with a medical history of metastatic clear cell renal cell carcinoma in remission for two years after immunotherapy with four cycles of ipilimumab and nivolumab followed by maintenance nivolumab infusions developed lesions concerning for choroidal metastases in her right eye. Optical coherence tomography of the lesions revealed a bacillary layer detachment containing possible fibrinous exudate organized into layers and underlying choroidal thickening with chorioretinal folds. Later, choroidal thickening and chorioretinal folds also occurred in the left eye. Given that pan imaging detected no metastasis and the posterior segment abnormalities resolved after cessation of nivolumab and treatment with systemic corticosteroids, the patient was diagnosed with nivolumab-induced Vogt-Koyanagi-Harada-like uveitis. CONCLUSION: This case expands on the clinical spectrum of nivolumab-induced Vogt-Koyanagi-Harada-like uveitis, a condition that can also present with bacillary layer detachment mimicking an early choroidal metastasis, manifest asymmetrically in each eye, and develop after long-standing treatment.

VOGT-KOYANAGI-HARADA-LIKE UVEITIS FOLLOWED BY MELANOMA-ASSOCIATED RETINOPATHY WITH FOCAL CHORIORETINAL ATROPHY AND CHOROIDAL NEOVASCULARIZATION IN A PATIENT WITH METASTATIC CUTANEOUS MELANOMA.

PURPOSE: To report a case of Vogt-Koyanagi-Harada-like uveitis followed by melanoma-associated retinopathy with focal chorioretinal atrophy and subsequent choroidal neovascularization in a patient with metastatic cutaneous melanoma. METHOD: Case report. Main outcome measures include external photography, anterior segment photography, ophthalmoscopic examination, fundus photography, fluorescein angiography, indocyanine green angiography, spectral domain optical coherence tomography, optical coherence tomography angiography, and electroretinography. RESULTS: A 68-year-old man with a history cutaneous melanoma presented with Vogt-Koyanagi-Harada-like uveitis. Work-up revealed a pelvic mass, which was excised and found to be metastatic melanoma. Two years later, the patient developed melanoma-associated retinopathy with focal chorioretinal atrophy and adjacent choroidal neovascularization. CONCLUSION: Patients with metastatic cutaneous melanoma can develop distinct and sequential paraneoplastic ocular complications. Onset of a Vogt-Koyanagi-Harada-like uveitis may be a good prognostic factor for survival in patients with metastatic cutaneous melanoma.

BARTONELLA HENSELAE -ASSOCIATED OPTIC NEUROPATHY PRESENTING AS A CENTRAL SCOTOMA IN THE ABSENCE OF OVERT PAPILLITIS: A MULTIMODAL IMAGING STUDY.

BACKGROUND/PURPOSE: The purpose of this report was to describe the use of multimodal imaging to establish the diagnosis of Bartonella henselae -associated optic neuropathy in a patient who presented with a central scotoma without overt evidence of optic nerve involvement. METHODS: This was a case report. Main outcome measures included clinical, optical coherence tomography, and fluorescein angiography findings. OBSERVATIONS: A 72-year-old woman presented with a 3-day history of central scotoma in the left eye. Her examination was remarkable for faint exudation in the nasal macula of the left eye but was otherwise normal for her age. Spectral domain optical coherence tomography of the macula revealed mild thickening of the papillomacular bundle with scattered small cystoid spaces and several intraretinal exudates, none of which were visible clinically. Fluorescein angiography revealed localized leakage of the inferotemporal optic disc. When prompted, the patient recalled being scratched multiple times by her two pet kittens. Serial testing showed rising anti- B. henselae ( B. henselae ) immunoglobulin G antibody titers to 1:1,280, confirming the suspected diagnosis of B. henselae -associated optic neuropathy. CONCLUSION: Bartonella -associated optic nerve involvement can occur without overt evidence of optic disc swelling. Multimodal imaging can be used to suggest the diagnosis and support appropriate serologic testing.

Bartonella henselae-associated recurrent, bilateral segmental periphlebitis.

Purpose: To describe a patient with atypical Bartonella henselae (B. henselae)-associated ocular inflammation that manifested with recurrent, bilateral segmental periphlebitis. Observations: A 32-year-old White man presented with multiple paracentral scotomata in each eye. Examination revealed mild vitreous cell, segmental sheathing of the retinal veins, and inflammation of the paravenous retina in each eye. Multimodal imaging, including optical coherence tomography as well as widefield fundus autofluorescence, fluorescein angiography, and indocyanine green angiography, was consistent with bilateral, segmental retinal periphlebitis with paravenous inflammation and retinochoroidal scarring. Serology showed elevated B. henselae antibody titers, but was otherwise unrevealing, and the patient was diagnosed with presumed B. henselae-associated ocular inflammation. Treatment with systemic doxycycline (100 mg PO BID) for four weeks improved the patient's symptoms and posterior uveitis. However, after an asymptomatic period of nearly one year, his bilateral pericentral scotomata recurred and posterior segment examination confirmed new foci of retinal periphlebitis in each eye. Re-treatment with doxycycline (100 mg PO BID) for four weeks again yielded improvement, but one month after completing his antibiotic course, his visual symptoms recurred, and we observed additional areas of periphlebitis and paravenous retinitis with associated branch retinal vein occlusions in each eye. This time a dual antibiotic regimen of doxycycline (100 mg PO BID) and rifampin (300 mg PO BID) was administered for three months, with improvement. Over the next eight years, the patient experienced no further disease relapse, and the previous sites of retinal periphlebitis eventually developed perivenous fibrosis with paravenous retinochoroidal scarring. Conclusion: Rarely, patients with B. henselae-associated ocular inflammation develop segmental retinal periphlebitis with or without retinal vein occlusion. This form of ocular bartonellosis can recur, requiring multiple courses of antimicrobial therapy.

Optic disc edema with peripapillary serous retinal detachment as the presenting sign of necrotizing herpetic retinitis.

PURPOSE: To describe an atypical case of necrotizing herpetic retinitis (NHR) that presented initially with mild anterior uveitis, optic disc swelling, and peripapillary serous retinal detachment (SRD). OBSERVATIONS: A 48-year-old Asian Indian man presented with blurred central vision and pain in his left eye. Examination revealed mild anterior chamber inflammation, optic disc swelling, and a peripapillary SRD in the affected eye. Multimodal imaging, including widefield fluorescein angiography, showed optic disc leakage and confirmed the presence of a peripapillary SRD, but was otherwise unremarkable with no evidence of retinitis. The patient was diagnosed with presumed Vogt-Koyanagi-Harada disease and was treated with systemic corticosteroids. While there was objective visual improvement initially, the patient subsequently noted peripheral vision loss and was found to have peripheral necrotizing retinitis and occlusive retinal vasculitis in affected eye. Polymerase chain reaction-based testing of aqueous humor detected varicella zoster virus DNA, confirming the diagnosis of NHR. CONCLUSION AND IMPORTANCE: Optic disc edema and peripapillary SRD may precede the development of overt retinitis in some patients with NHR.

OCULAR INJURIES ASSOCIATED WITH ELASTIC EXERCISE BANDS.

PURPOSE: To discuss the mechanism of injury and characterize the clinical features of ocular trauma associated with elastic cord exercise equipment band injuries in a consecutive series of patients seen at a single vitreoretinal surgery practice. METHODS: We performed a retrospective review of all patients who were treated for blunt trauma from 2013 to 2020 at a single vitreoretinal practice. RESULTS: Thirteen eyes from 11 patients met the inclusion criteria of possessing ocular trauma secondary to recoil from exercise bands. Presenting visual acuity ranged from 20/16 to HM (median: 20/32). The most frequently observed anterior segment pathologies were traumatic iritis (54%) and angle recession (31%). The most common posterior segment findings were vitreous hemorrhage (54%) and peripheral commotio retinae (54%). Three eyes (23%) required surgical intervention. Follow-up intervals ranged from 0 to 10 months (median: 1.75 months). Visual acuity at last examination ranged from 20/13 to 20/400 (median: 20/40). CONCLUSION: A wide spectrum of serious ocular injuries requiring medical and surgical intervention can result from this form of blunt ocular trauma. The frequency of this event would be decreased by the use of sports goggles and careful inspection of equipment for wear and over use.

Occlusive retinal vasculitis associated with systemic sclerosis and antiphospholipid antibodies.

PURPOSE: To report a series of patients with occlusive retinal vasculitis associated with systemic sclerosis (SSc) and elevated antiphospholipid antibody titers. METHOD: Case series. Main outcome measures included clinical and fluorescein angiographic findings at presentation and over time. OBSERVATIONS: Case 1 - A 61-year-old woman initially diagnosed with idiopathic, bilateral panuveitis and retinal vasculitis causing peripheral nonperfusion was subsequently diagnosed with limited cutaneous systemic sclerosis (lcSSc). Her ocular inflammation and retinal vasculitis were controlled with topical and periocular corticosteroids, but she eventually developed peripheral retinal vascular occlusion that progressed to macular ischemia 11 years after presentation. Repeat serologic evaluation detected interval development of antiphospholipid antibodies. Case 2 - A 58-year-old woman was found to have bilateral peripheral nonperfusion and retinal neovascularization in her right eye. Given her elevated hemoglobin A1c of 8.5%, she was diagnosed with presumed proliferative diabetic retinopathy. Three years after initial presentation, she was diagnosed with lcSSc. Subsequent serum workup detected elevated B2-glycoprotein antibody titers. Her peripheral nonperfusion progressed despite adequate glycemic control, resulting in further neovascularization in each eye. Case 3 - A 40-year-old woman with diffuse cutaneous systemic sclerosis (dcSSc) and elevated titers of anti-cardiolipin antibodies developed multiple branch retinal artery occlusions with subsequent neovascularization of the retina, optic disc, and angle in the right eye. CONCLUSION AND IMPORTANCE: Vision-threatening occlusive retinal vasculitis may develop in select patients with SSc. The presence of elevated anti-phospholipid antibody titers may confer increased risk for this vision-threatening complication.

Rituximab for non-infectious Uveitis and Scleritis.

PURPOSE: To provide a comprehensive review of rituximab use for the treatment of non-infectious uveitis and scleritis. METHODS: Review of literature through December 2020. RESULTS: Individual data was available for 229 patients with refractory non-infectious uveitis (n = 108) or scleritis (n = 121) who received treatment with rituximab (RTX). Rituximab was generally utilized as third-line or later treatment (uveitis: 67/90, 74.4%; scleritis: 90/96, 93.8%) at a mean of 33.5 months following the diagnosis of uveitis (range = 0 to 168.0 months; median = 24.0 months) and 39.4 months after diagnosis of scleritis (range = 1.0 to 168.0 months; median = 21.0 months). Patients with non-infectious uveitis and scleritis either received prior treatment with corticosteroids only (uveitis: 18/90, 20%; scleritis: 4/94, 4.3%), or with one (uveitis: 19/90, 21.1%; scleritis: 30/94, 31.9%), two (uveitis: 11/90, 12.2%; scleritis 27/94, 28.7%), or three or more (uveitis: 37/90, 41.1%; scleritis: 31/94, 33.0%) corticosteroid-sparing immunosuppressive agents with or without corticosteroids before initiation of RTX treatment. The rheumatologic protocol (two infusions of 1 gram of RTX separated by 14 days) was utilized most frequently (uveitis: 45/87, 51.7%; scleritis: 87/114, 76.3%), followed by the Foster protocol (eight weekly infusions of 375 mg/m2 RTX; uveitis: 18/87, 20.7%; scleritis: 10/114, 8.8%), and the oncologic protocol (four weekly infusions of 375 mg/m2 RTX; uveitis: 5/87, 5.7%; scleritis: 6/114, 5.3%). Various other off-label regimens were used infrequently (uveitis: 19/87, 21.8%; scleritis 11/114, 9.6%). Rituximab treatments resulted in a positive therapeutic response for the majority of patients with non-infectious uveitis (81/97, 83.5%). Commonly treated uveitic diagnoses included non-paraneoplastic autoimmune retinopathy (30/107, 28.0%), juvenile idiopathic arthritis (21/107, 19.6%), Vogt-Koyanagi-Harada disease (12/107, 11.2%), and Behçet disease (11/107, 10.3%). Cases of non-infectious scleritis were most commonly attributed to granulomatosis with polyangiitis (75/121, 62.0%) and rheumatoid arthritis (15/121, 12.4%), and showed an even greater rate of positive therapeutic response (112/120, 93.3%) following RTX treatment. No side effects were reported in 76.3% (74/97) of uveitis and 85.5% (71/83) scleritis cases. Of those cases associated with RTX-induced adverse events, the most common were infusion reactions of various severity (11/35, 31.4%). CONCLUSIONS: Overall, RTX appeared to be both effective and well-tolerated as second or third-line therapy for patients with non-infectious uveitis and scleritis.

Rituximab for treatment of non-infectious and non-malignant orbital inflammatory disease.

PURPOSE: To provide a comprehensive review of rituximab use for the treatment of non-infectious/non-malignant orbital inflammation. METHODS: Review of literature through January 2021. RESULTS: Individual data was available for 167 patients with refractory non-infectious/non-malignant orbital inflammation who received treatment with rituximab (RTX). Rituximab was generally utilized as third-line or later treatment (108/149, 72.5%) at a mean of 44.6 months following the diagnosis of orbital inflammation (range = 0 to 360 months; median = 13.7 months). Patients with non-infectious/non-malignant orbital inflammation either received prior treatment with corticosteroids only (27/122, 22.1%), or with one (31/122, 25.4%), two (25/122, 20.5%), or three or more (25/122, 20.5%) corticosteroid-sparing immunosuppressive agents with or without corticosteroids before initiation of RTX treatment. The rheumatologic protocol (two infusions of 1 gram of RTX separated by 14 days) was utilized most frequently (80/144, 55.6%), followed by the oncologic protocol (four weekly infusions of 375 mg/m2 RTX; 51/144, 35.4%). Various other off-label regimens were used infrequently (13/144, 9.0%). Rituximab treatments resulted in a positive therapeutic response for the majority of patients with orbital inflammation (146/166, 88.0%). Commonly treated diagnoses included granulomatosis with polyangiitis (99/167, 59.3%), IgG-4 related disease (36/167, 21.6%), and orbital inflammation of indeterminate cause (25/167, 15.0%). No side effects were reported in 83.3% (55/66) of cases. The most common RTX-induced adverse event was an infusion-related temporary exacerbation of orbital disease (4/66, 6.1%), which occurred prior to the routine use of systemic corticosteroids as pre-conditioning. CONCLUSIONS: Overall, RTX appears to be both efficacious and well-tolerated as second- or third-line therapy for patients with non-infectious/non-malignant orbital inflammation.

Massive Delayed Hemorrhagic Choroidal Detachment and Giant Retinal Tear Detachment Following Scleral-Fixated Akreos IOL.

A 78-year-old woman underwent 25-gauge pars plana vitrectomy and scleral fixation for a dislocated intraocular lens in the left eye under general anesthesia. She developed massive hemorrhagic choroidal detachment 1 day later. A giant retinal tear detachment and proliferative vitreoretinopathy was noted at postoperative Week 7 and repaired with choroidal drainage, 240° peripheral retinectomy, perfluorocarbon liquid, endolaser, and silicone oil. Hypotony associated with the 25-gauge sclerotomies used for suture fixation was the suspected etiology for this complication. Use of 27-gauge instrumentation or application of fibrin glue to superior sclerotomies may help reduce the incidence of postoperative hypotony. [Ophthalmic Surg Lasers Imaging Retina. 2021;52:288-292.].

Cardiopulmonary Resuscitation Retinopathy in an Adult.

A man developed vision loss and bilateral subhyaloid, outer plexiform, and subretinal hemorrhages after 2 minutes of chest compressions. Several potential mechanisms, including venous transmission of pressure and elevated intracranial pressure (ICP), could explain these findings.

FINGERPRINT SIGN OF THE HENLE FIBER LAYER.

PURPOSE: To describe the appearance of concentric, fingerprint-like waves within the Henle fiber layer (HFL) using en face optical coherence tomography in patients with tractional pathologies of the retina. METHODS: Retrospective analysis of six eyes of six patients imaged by optical coherence tomography with volumetric slabs positioned at the level of the HFL. RESULTS: Optical coherence tomography data from six patients with tractional vitreoretinal pathology were reviewed. Concentric, fingerprint-like microwaves were visualized through en face optical coherence tomography in all six study eyes at the level of the HFL. This finding resembled the finding of HFL waves previously noted histopathologically from force exerted on this layer. CONCLUSION: In retinal pathologies in which specific physical forces act on the retina, volumetric optical coherence tomography may permit visualization of en face concentric, fingerprint-like hyperreflective rings within the HFL. This "fingerprint sign" may represent a biomechanical consequence of traction on the retina and allow clinical decision making based on improved recognition of the existence of such traction.

Clinical course of von Szily reaction: Case report and comprehensive review of the literature.

PURPOSE: To describe a rare case of von Szily reaction (VSR) accompanied by a comprehensive review of the literature. OBSERVATIONS: A 57-year-old woman with herpes zoster ophthalmicus (HZO) associated with ipsilateral sectoral scleritis and anterior uveitis (sclerouveitis) subsequently developed contralateral necrotizing retinitis, leading to a diagnosis of VSR. A literature review revealed 10 additional cases of VSR. The full VSR cohort of 11 subjects included six women and five men, had a median age of 39 years (range 21-78 years), and most presented with HZO (n = 7, 63.6%), often associated with either ipsilateral anterior uveitis (n = 5; 45.5%) or keratitis (n = 4; 36.4%). All 11 cases developed necrotizing retinitis in the fellow eye, at a median of six weeks following onset in the sentinel eye. The most frequently implicated agent was varicella zoster virus (VZV; n = 8, 72.7%). A high proportion of the eight patients with VZV-associated VSR were identified as having increased risk of VZV reactivation, including age of 50 years or greater (n = 5, 62.5%), an underlying malignancy (n = 3, 37.5%), and/or use of immunosuppressive medication (n = 2, 25.0%). CONCLUSION: This was the first reported case of VSR presenting as HZO-associated with sclerouveitis. A comprehensive literature review revealed that most previously reported cases presented with HZO associated with isolated anterior uveitis and/or keratitis, and that all reported cases of VSR developed necrotizing retinitis in the fellow eye, typically within two months of initial presentation. Patients with VZV-associated VSR often had known risk factors for VZV reactivation.

Multiple evanescent white dot syndrome following influenza immunization - A multimodal imaging study.

PURPOSE: To describe the multimodal imaging (MMI) findings and clinical course of a case of Multiple Evanescent White Dot Syndrome (MEWDS) following immunization with inactivated intra-dermal influenza virus, and to explore whether similarities exist with other, previously reported cases. OBSERVATIONS: A 34-year-old Caucasian man presented with unilateral onset of para-central scotomata, photopsias, and dyschromatopsia two weeks after administration of an influenza vaccine. Clinical examination and MMI were indicative of MEWDS. The patient's MMI abnormalities and symptoms resolved spontaneously after four weeks. CONCLUSION AND IMPORTANCE: This is the first reported case of MMI of post-influenza vaccination-associated MEWDS. Comparison with eight previously reported cases of MEWDS following various immunizations revealed that subjects tended to be healthy, young to middle age women with a median time to onset of two weeks. Vision tended to recover spontaneously over one to three months.

Heterozygous CRX R90W mutation-associated adult-onset macular dystrophy with phenotype analogous to benign concentric annular macular dystrophy.

Background Historically, CRX mutations have been associated with cone-rod dystrophy, cone dystrophy, Leber's congenital amaurosis, and retinitis pigmentosa. There is recent emerging evidence of an adult-onset macular dystrophy phenotype. We review the published literature and discuss the first case of heterozygous CRX R90 W macular dystrophy. Materials and Methods The patient received serial ophthalmic examination and imaging. Genetic testing was performed by MyRetinaTracker with the use of a retinal dystrophy panel. Results A 55-year-old Caucasian male patient without a prior medical history presented for evaluation of decreased vision in the right eye. Visual acuity was 20/32 both eyes, and his fundus examination was notable for an incomplete ring-shaped macular atrophy with foveolar sparing in both eyes. Fundus autofluorescence was notable for hypo-autofluorescence of the ring and fluorescein angiography for transmission hyperfluorescence. Full-field ERG and EOG were normal, while mfERG showed central depression. His lesion was clinically diagnosed as benign concentric annular macular dystrophy, but genetic testing revealed a heterozygous mutation in CRX (c.268 C > T, p.R90 W). A three-generation family tree did not reveal other members with known macular dystrophy. Given the lack of documentable autosomal dominant inheritance and the presence of a CRX mutation, the patient's diagnosis was revised to adult-onset macular dystrophy. Conclusions We believe this to be the first case of adult onset macular dystrophy associated with heterozygous CRX R90 W mutation.

Detection of uric acid stones in the ureter using low- and conventional-dose computed tomography.

OBJECTIVE: To determine the ability of low- and conventional-dose computed tomography (CT) in identification of uric acid stones, which are of lower density than calcium oxalate stones. MATERIALS AND METHODS: Uric acid stones (3, 5, and 7 mm) were randomly placed in human cadaveric ureters and scanned using conventional 140-mAs and low-dose 70-, 50-, 30-, 15-, 7.5-, and 5-mAs settings. A single-blinded radiologist reviewed a total of 523 scanned stone images. Sensitivity and specificity were compared among different settings and stone sizes. RESULTS: Imaging using 140-, 70-, 50-, 30-, 15-, 7.5-, and 5-mAs settings resulted in 97%, 97%, 96%, 93%, 83%, 83%, and 69% sensitivity and 92%, 92%, 91%, 89%, 88%, 91%, and 94% specificity, respectively. There was a significant difference in sensitivity between 140 mAs and 15, 7.5, and 5 mAs (P = .011, P = .011, and P <.001, respectively). Sensitivity for 3-, 5-, and 7-mm stones was 83%, 90%, and 93%, respectively. At ≤ 15 mAs, 3-mm stones had a higher rate of false negatives (P <.001). CONCLUSION: Both low- and conventional-dose CTs demonstrate excellent sensitivity and specificity for the detection of ureteral uric acid stones. However, low-dose CT at ≤ 15 mAs resulted in reduced detection of uric acid stones.