Current role of intravitreal injections in Irvine Gass syndrome-CRIIG study.

OBJECTIVE: To analyze the role of intravitreal anti-vascular endothelial growth factor (anti-VEGF) or steroid injection for the management of Irvine Gass syndrome. METHODS: It is an interventional, retrospective, multicenter study. One hundred and thirty-two injections were given in 79 eyes of 72 patients with Irvine Gass syndrome. Patients were treated with at least one intravitreal injection of either anti-VEGF or steroid. Outcomes were measured at 12 months (± 1 week). [Ranibizumab (Lucentis; Genentech, South San Francisco, CA) (Razumab; Intas Pharmaceutical Ltd, Ahmedabad, India) Bevacizumab (Avastin; Genentech, South San Francisco, CA) or Aflibercept (Eylea; Regeneron, Tarrytown, NY)] or steroids [Dexamethasone implant (Ozurdex, Allergan Inc, Irvine, CA) or intravitreal triamcinolone)]. RESULTS: Intravitreal injections were initiated in (67.6%) of eyes within 14 weeks of diagnosis. Intravitreal dexamethasone implant was used as the initial intravitreal therapy in (73.4%) of eyes. More than fifty percent (54.5%) of the patients were switched from anti-VEGF to Intravitreal dexamethasone implant. Reduction in the mean CMT was 336.7 ± 191.7 and 160.1 ± 153.1 microns in eyes treated within four weeks and more than 14 weeks from diagnosis (p = 0.005). Mean ETDRS letter gain was 16.7 ± 12.9 and 5.2 ± 9.2 in eyes treated within 4 weeks and more than 14 weeks from diagnosis (p = 0.004). Three eyes injected with intravitreal dexamethasone implant reported an intraocular pressure spike of > 25 mmHg which was controlled with topical medications. No other ocular or systemic adverse events were observed. CONCLUSION: Study results suggest that physicians tend to introduce intravitreal therapy within 14 weeks of diagnosis. The most common therapy at initiation and for the switch is intravitreal dexamethasone implant. Patients treated early (within 4 weeks) respond better in terms of structure and function.

Impact of COVID-19-related lifestyle changes on diabetic macular edema.

AIM: To assess diabetic macular edema (DME) progression during the early phases of the COVID-19 pandemic, when severe societal restrictions raised the concern of possible deterioration of health in patients with systemic conditions, particularly those requiring frequent office visits. METHODS: This is a multicenter retrospective chart review of 370 patients (724 eyes) with an established diagnosis of DME seen on 3 separate visits between January 2019 and July 2021. Period 1 was January 2019 to February 2020 (considered pre-COVID-19), period 2 was March 2020 to December 2020 (considered the height of the pandemic; highest level of pandemic-related clinical and societal regulations) and period 3 was January 2021 to July 2021 (re-adjustment to the new "pandemic norms"). Main outcome measures included visual acuity, body mass index (BMI), blood pressure (BP), hemoglobin A1c (HbA1c), macular thickness, patient adherence to scheduled ophthalmology visits, and DME treatment(s) received at each visit. To facilitate measurement of macular thickness, each macula was divided into 9 Early Treatment Diabetic Retinopathy Study (ETDRS)-defined macular sectors as measured by OCT imaging. RESULTS: There was no change of BMI, systolic BP, and diastolic BP between any of the time periods. HbA1c showed a very small increase from period 1 (7.6%) to period 2 (7.8%, P=0.015) and decreased back to 7.6% at period 3 (P=0.12). Macular thickness decreased for 100% of macular regions. The central macular thickness decreased across all 3 periods from 329.5 to 316.6 µm (P=0.0045). After analysis of multiple variables including HbA1c, BMI, adherence to scheduled appointments, different clinic centers, and treatment interventions, there was no easily identifiable subgroup of patients that experienced the increase in DME. CONCLUSION: DME doesn't worsen during the COVID-19 pandemic, instead sustaining a very small but statistically significant improvement. While identifying a mechanism behind our findings is beyond the scope of this study, potential explanations may include a delay in retinal changes beyond our study period, an unexpected increase in treatment frequency despite pandemic restrictions, and an unanticipated pandemic-related improvement in some lifestyle factors that may have had a positive impact on DME.

Clinical Characteristics and Surgical Outcomes of Patients Undergoing Pars Plana Vitrectomy for Proliferative Diabetic Retinopathy.

OBJECTIVE: To evaluate clinical characteristics impacting surgical outcomes of patients undergoing pars plana vitrectomy (PPV) for complications of proliferative diabetic retinopathy (PDR). DESIGN: Retrospective consecutive observational case series of patients at a large county hospital in Dallas, Texas, from 2014 to 2019. SUBJECTS: Seven hundred thirty-two patients (933 eyes) undergoing PPV for PDR complications. METHODS: Collected data included demographics, surgical indication, adjuvant therapies, intraoperative course, complications, and best corrected visual acuity (BCVA). Patients with < 6 months of follow-up were excluded. Best corrected visual acuity was converted to logarithm of the minimum angle of resolution for analysis. Statistics performed included t test, analysis of variance, and multivariate analyses. MAIN OUTCOME MEASURES: Postoperative BCVA, primary anatomic success rate, and postoperative complications. RESULTS: Three hundred ninety-three patients were male (509 eyes; 54.5%) with an average age of 52 years. Postoperative BCVA at 6 months was significantly different among surgical indications: 0.79 versus 0.77 versus 1.20 (P < 0.0001) for vitreous hemorrhage (VH), vitreomacular interface abnormalities, and tractional retinal detachment (TRD), respectively. Adjuvant preoperative therapy with panretinal photocoagulation (PRP) versus no PRP (0.95 vs. 1.25; P < 0.001) and insulin versus no insulin (0.99 vs. 1.17; P < 0.01) were associated with improved vision. Iatrogenic breaks were associated with decreased postoperative vision (1.40 vs. 0.88; P < 0.001). The primary anatomic success rate for TRD was 85% (495 eyes). Combined TRD/RRD (tractional and rhegmatogenous retinal detachment) was associated with a lower success rate compared with macula-on/macula-off TRD, with odds ratios of 0.36, 0.46, and 0.53, respectively. Patients experiencing recurrent detachment postsurgery had worse preoperative visual acuity (VA) (1.93 vs. 1.63; P < 0.01) and were younger (47.6 vs. 50.0; P = 0.02). Postoperative complications occurred in 699 eyes (75%), with VH (498 eyes, 53%), cataract (465, 50%), and elevated intraocular pressure (149, 16%) being the most common. Two hundred thirty-six eyes (25%) required a second PPV operation. Endophthalmitis (1 eye; <1%) and choroidal detachment (5 eyes; <1%) were rare. CONCLUSIONS: In this retrospective series analyzing surgical outcomes among patients with complications from PDR, vitrectomy led to improved vision on average, with a meaningful proportion of patients receiving additional surgical intervention. Surgical indication, presenting VA, age, and adjuvant therapies appeared to impact outcomes. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Retinal neovascularization in the setting of CALR-mutation positive essential thrombocythemia.

Patients with Calreticulin (CALR) mutation positive essential thrombocythemia are often thought of as having a "low-risk" of thrombotic complications. This report examines a case of a patient with CALR-mutation positive essential thrombocythemia presenting with peripheral retinal ischemia and subsequent retinal neovascularization. This patient had a 2-year documented history of CALR-mutation positive essential thrombocythemia with a maximum platelet count of over 800,000 cells/µL. Fluorescein angiogram showed significant leakage in the areas of the vascular lesions with peripheral vascular nonperfusion consistent with neovascularization. The patient was treated with photocoagulation to the areas of avascular retina. At 6-month follow-up, some regression of neovascularization was noted with no signs of progression in the retinopathy. Furthermore, essential thrombocythemia patients can present with neovascularization secondary to retinal ischemia due to microvascular thrombotic events. Identification of these changes is important for guiding medical and procedural interventions in order to preserve vision.

Evaluation of the Clinical Course of Endogenous Endophthalmitis.

Purpose: To evaluate the presentations, clinical course, treatments, and outcomes of endogenous endophthalmitis cases. Methods: Consecutive medical records from 2016 to 2021 of a county hospital and an academic, private hospital in Dallas, Texas were retrospectively reviewed. This study comprised 37 eyes of 31 patients with endogenous endophthalmitis. Collected data included demographic factors, identifiable risk factors, medical history, presenting symptoms, infectious data, complications, and best-corrected visual acuity (VA) throughout the clinical course. Results: Twenty-two eyes had bacterial endophthalmitis, 7 had fungal endophthalmitis, and 8 had infections that could not be classified. Of the bacterial cases, 5 eyes had panophthalmitis with associated cellulitis. The most common organisms were Staphylococcus aureus, Candida albicans, and Pseudomonas aeruginosa. The most common presenting symptoms were decreased vision (70%), eye redness (41%), and eye pain (38%). Among all cases, there was no significant difference in the presenting logMAR VA (1.86) before treatment and the most recent logMAR VA (1.75; P = .70) after treatment. However, fungal infections demonstrated better logMAR VA than bacterial infections 6 months after diagnosis (mean logMAR VA, 0.93 vs 2.54, respectively; P = .016) and at most recent follow-up (mean logMAR VA, 0.76 vs 2.3, respectively; P = .004). There was also a strong correlation between presenting VA and most recent VA (r2 = 0.81; P < .01). Conclusions: Visual outcomes of endogenous endophthalmitis cases were poor. Our study found 2 components to be predictive of final VA: (1) whether the infecting organism was bacterial or fungal and (2) a patient's presenting VA.

Pediatric Rhegmatogenous Retinal Detachments: Etiologies, Clinical Course, and Surgical Outcomes.

Purpose: To describe the predisposing factors, clinical course, and surgical methods of pediatric rhegmatogenous retinal detachment (RRD) and determine which factors affect anatomic success. Methods: Data of patients 18 years or younger who had surgical repair for RRD from January 1, 2004, to June 31, 2020, with a minimum of 6 months of follow-up were retrospectively analyzed. Results: The study evaluated 101 eyes of 94 patients. Of the eyes, 90% had at least 1 predisposing factor to pediatric RRD, including trauma (46%), myopia (41%), prior intraocular surgery (26%), and congenital anomaly (23%); 81% had macula-off detachments and 34% had proliferative vitreoretinopathy (PVR) grade C or worse at presentation. The presence of PVR grade C or worse (P = .0002), total RRD (P = .014), and vitrectomy alone at first surgery (P = .0093) were associated with worse outcomes. Patients who had scleral buckle (SB) alone at the first surgery had statistically higher rates of anatomic success than those who had vitrectomy alone or combined with SB (P = .0002). After the final surgery, 74% of patients achieved anatomic success. Discussion: The majority of cases in this study were associated with 1 of the 4 risk factors predisposing to pediatric RRD. These patients often present late with macula-off detachments and PVR grade C or worse. The majority of patients achieved anatomic success after surgical repair using SB, vitrectomy, or a combination.

CMV retinitis in patients on mycophenolate immunosuppression: a report of two cases.

BACKGROUND: The rate of cytomegalovirus (CMV) retinitis is increasing, likely secondary to aggressive immunosuppressive regimens for a variety of diseases. Transplant and rheumatological literature show growing evidence suggesting a unique relationship between CMV infection and mycophenolate in particular. This study reports two cases of CMV retinitis infection in patients on mycophenolate immunosuppression. CASE PRESENTATION: Case A was a 39-year-old African American woman with systemic lupus erythematosus (SLE) with stage IV lupus nephritis who presented for bilateral retinal detachments with areas of moth-eaten and thin retina concerning for prior viral retinitis. Case B was a 53-year-old man who presented with floaters in the right eye status-post heart transplant since 2008 on immunosuppressive therapy. Fundoscopic examination of the right eye showed frosted branch angiitis with intraretinal hemorrhage and inner retinal thickening and disorganization, consistent with CMV retinitis infection. Both patients were on mycophenolate immunosuppression with the recommendation to reduce or discontinue mycophenolate. CONCLUSION: Patients on mycophenolate immunosuppression may be more vulnerable to cytomegalovirus infection, including CMV retinitis. Ophthalmologists should be aware of this increased risk and consider reducing or discontinuing mycophenolate to promote viral clearance in these susceptible patients, in conjunction with the patient's transplant or rheumatology teams.

SMALL-GAUGE TECHNIQUES FOR REMOVING A FLUOCINOLONE ACETONIDE IMPLANT.

PURPOSE: Long-acting injectable fluocinolone releasing implants are used in clinical practice. Although limited in scope, situations may arise where removal of the implant is warranted. We set out to describe possible explantation techniques and to determine whether these implants can be safely removed from a standard sclerotomy or eliminated using a vitrectomy system. METHODS: A vitreoretinal surgery system was designed using a porcine eye model. A fluocinolone implant was injected into the vitreous cavity. Pars plana vitrectomy was performed and the vitreous cavity was infused with balanced salt solution. The injected implants were removed from 23-Gauge (G) and 25-Gauge (G) vitrectomy cannulas with 27-G forceps. The implants were examined under the microscope for induced defects. Implants were injected into the eye model and eliminated using a 23-G and 25-G vitrector system. RESULTS: The implant was removed from both the 23-G and 25-G vitrectomy cannulas with only mild structural damage to the implant. During implant extraction through the 25-G sclerotomy, the cannula was dislodged from the incision along with the implant. The most technically challenging portion involved aligning the implant coaxially to allow for removal en bloc through the sclerotomy site. Implants could be eliminated using both the 23-G and 25-G vitrector using a low-cut rate. CONCLUSION: The fluocinolone implant was removed safely via standard 23-G or 25-G vitrectomy systems. It is unknown whether intraocular manipulation will affect pharmacokinetics of drug delivery if the implant is not explanted.

Comparison of Surgical Outcomes for Uncomplicated Primary Retinal Detachment Repair.

Purpose: To compare the outcomes of primary uncomplicated rhegmatogenous retinal detachment (RRD) repair using pars plana vitrectomy (PPV), scleral buckling (SB), or combined scleral buckling with vitrectomy (SB/PPV). Patients and Methods: Single-institution, retrospective, observational study of 179 patients with primary RRD managed at a large academic hospital system. We excluded patients with less than 6 months of follow-up, previous vitrectomy or buckle, giant retinal tears, aphakia, recurrent forms of RRD, or extensive proliferative vitreoretinopathy (Grade C or worse) documented on exam or requiring membrane peel. Outcome measures included primary anatomical success at 6 months, functional success defined as BCVA ≥ 20/200, and best corrected visual acuity (BCVA) using logMAR scoring. Subgroup analysis was performed in the following patient groups: phakic, pseudophakic, inferior detachments, and prior pneumatic retinopexy. Results: Primary anatomical success was achieved in 145 of 179 eyes (81.0%), with SB/PPV showing a significantly greater success rate (p = 0.046) when compared to SB and PPV. Functional success was achieved in 137 of the 145 anatomically successful eyes (94.5%), with values ranging between 92% and 97% amongst the interventions (p = 0.552). No difference was found in final BCVA (p = 0.367). Patients with inferior detachment had an odds ratio of 2.15 for primary anatomic failure. Prior pneumatic retinopexy did not significantly affect any of the primary outcomes. Conclusion: SB/PPV yielded a significantly better primary anatomical success rate when compared to SB and PPV. Functional success and final BCVA was similar amongst the interventions. Inferior detachments were associated with worse primary anatomic outcomes. Prior pneumatic retinopexy did not significantly affect surgical outcomes.

Orbital compartment syndrome following primary scleral buckle surgery.

PURPOSE: To illustrate a patient with orbital compartment syndrome following scleral buckle placement that was successfully treated with canthotomy and cantholysis. METHODS: Observational case report. RESULTS: A 26-year-old male underwent a primary scleral buckle repair for a chronic rhegmatogenous retinal detachment. On post-operative day four, the patient presented to the emergency room with pain and increased intraocular pressure (IOP). Initial treatment with conservative IOP lowering agents was unsuccessful. The patient was diagnosed with delayed orbital compartment syndrome and was successfully managed with lateral canthotomy and inferior cantholysis in addition to aggressive steroid and antibiotic medical management. CONCLUSION: Following scleral buckle placement with sub-tenon's anesthesia block, there may be a delayed presentation of orbital compartment syndrome. Recognition and management of this rare complication is important for preventing irreversible blindness.

Unexplained Vision Loss Associated With Intraocular Silicone Oil Tamponade in Rhegmatogenous Retinal Detachment Repair.

Purpose: To evaluate the visual outcomes with unexplained vision loss during or after silicone oil (SO) tamponade. Methods: This multicenter retrospective case series comprised patients with unexplained vision loss associated with SO tamponade or its removal. Eyes with other clear secondary identifiable causes of vision loss were excluded. Results: Twenty-nine eyes of 28 patients (64% male) were identified. The mean age was 50 ± 13 years (range, 13-78 years). The mean duration of SO tamponade was 148 ± 38 days. Eighteen eyes (62%) developed unexplained vision loss while under SO; 11 (38%) had vision loss after SO removal. The most common optical coherence tomography (OCT) finding was ganglion cell layer (GCL) thinning (55%). Eyes with vision loss after SO removal had a mean logMAR best-corrected visual acuity (BCVA) of 0.6 ± 0.7 (Snellen 20/85) before SO tamponade and 1.2 ± 0.4 (20/340) before SO removal. By the last follow-up after SO removal, the BCVA had improved to 1.1 ± 0.4 (20/235). In eyes with vision loss after SO removal, the BCVA before SO removal was 0.7 ± 0.7 (20/104), which deteriorated to 1.4 ± 0.4 (20/458) 1 month after SO removal. By the last follow-up, the BCVA had improved to 1.0 ± 0.5 (20/219). Conclusions: Unexplained vision loss can occur during SO tamponade or after SO removal. Vision loss was associated with 1000-centistoke and 5000-centistoke oil and occurred in macula-off and macula-on retinal detachments. The duration of tamponade was 3 months or longer in the majority of eyes. Most eyes had GCL thinning on OCT. Gradual visual recovery can occur yet is often incomplete.

Parafoveal acute middle maculopathy (PAMM) in sickle cell disease after discontinuation of hydroxyurea.

Purpose: Paracentral acute middle maculopathy (PAMM) is a rare ophthalmologic emergency involving the intermediate and deep retinal capillary plexus that supply the retina's middle layers. This case report describes an episode of PAMM in a patient with sickle cell disease (SCD) to demonstrate the importance of early diagnosis, review potential pathophysiologic mechanisms, and finally discuss appropriate management in this patient population. Observations: A 33-year-old black female with SCD, who had recently discontinued disease-modifying therapy with hydroxyurea, presented with a central scotoma of the left eye. Examination showed superficial opacification and whitening of the temporal perifoveal macula. After an initial diagnosis of central retinal artery occlusion she was admitted for a stroke workup. MRI was negative for stroke, and the patient was discharged after undergoing a red blood cell exchange (RBCX). Follow-up exam and optical coherence tomography (OCT) findings were more consistent with PAMM. Conclusions and Importance: To our knowledge, this is the first report of PAMM after discontinuation of hydroxyurea in preparation for pregnancy. It highlights the importance of a multidisciplinary approach when treating peripartum patients with SCD and the need for further research regarding vaso-occlusive prophylactic agents and their effects in pregnancy to minimize morbidity during family planning.

Multimodal Imaging in Ocular Siderosis.

Purpose: This report aims to characterize ocular changes in a case of ocular siderosis with iron toxicity using multimodal imaging and electroretinography. Methods: A 34-year-old woman presented with ocular siderosis of the left eye following penetrating injury with an iron-containing foreign body. The patient's uncorrected visual acuities were 20/60 and 20/150 in the right and left eye, respectively, with abnormal pupillary function and presence of a cataract in the left eye. She underwent successful intraocular foreign body removal and cataract surgery with no postoperative complications. Cone contrast threshold (CCT), full-field electroretinogram, spectral-domain optical coherence tomography (OCT), and OCT angiography (OCTA) were used to characterize ocular alterations preoperatively and postoperatively. Results: CCT color vision testing showed abnormal color vision, and OCTA revealed increased vascular flow density associated with the foreign body. Conclusions: CCT color vision testing, OCTA, OCT, and full-field electroretinogram can characterize retinal changes in cases of ocular siderosis.

Klebsiella pneumoniae endogenous endophthalmitis presenting as orbital cellulitis.

This report describes the first documented case of Klebsiella pneumoniae endogenous endophthalmitis with concurrent orbital cellulitis in a patient without a liver abscess. A 34-year-old, caucasian male with a history of intravenous drug abuse and type 1 diabetes mellitus was transferred from an outside hospital for progressively worsening pain and swelling in the right eye. Careful history, physical examination, and imaging were consistent with a diagnosis of endogenous endophthalmitis with concurrent orbital cellulitis. Vitreous cultures were positive for Klebsiella pneumoniae. Despite aggressive and early intervention with antibiotics and vitrectomy, the patient's condition continued to worsen and evisceration was required to adequately control the infection. Klebsiella pneumoniae endogenous endophthalmitis with concurrent orbital cellulitis is a rare and often blinding infection, despite appropriately aggressive intervention.

TWO CASES OF ACUTE RETINAL NECROSIS DUE TO VARICELLA ZOSTER DESPITE PRIOR SHINGLES VACCINATION.

PURPOSE: To review two cases of acute retinal necrosis in adults due to varicella zoster virus despite prior shingles vaccination. METHODS: Retrospective chart review. PATIENTS: A 62-year-old man and a 64-year-old man with unilateral acute retinal necrosis who had shingles vaccinations 16 and 7 months before their ocular symptoms. RESULTS: Polymerase chain reaction confirmation of varicella zoster virus in the anterior chambers of both patients. Both patients had good responses to oral antiviral therapy and topical and/or oral steroids. DISCUSSION: These two cases demonstrate that singles vaccination is not an absolute protection against varicella zoster virus-related acute retinal necrosis. However, such vaccination may reduce the severity of the acute retinal necrosis.

Studies on the Histopathology of Temporal Arteritis.

AIMS: The aim of this paper was to identify the location and to grade the severity of most significant inflammation within positive temporal artery biopsies along with other key clinical and histologic characteristics. METHODS: Charts and pathology slides for 70 patients diagnosed with temporal arteritis at the University of Wisconsin (UW) Hospital and Clinics from 1989 to 2015 were reviewed. A subset of 48 specimens was immunostained for CD68 and graded on a scale from 0 to +++; the location of staining was recorded. RESULTS: The most severe granulomatous inflammation was in the media and adventitia in 13% (9/70) of the biopsies; the remaining had uniform full thickness inflammation. Of the slides that were stained with CD68, 94% (45/48) were positive. In 42% (19/45), the stained cells were found mainly in the muscularis and adventitia. Seven percent (3/45) of the slides had staining solely around the internal elastic lamina, and 2% (1/45) had staining limited to the intima. CONCLUSIONS: With a few exceptions, granulomatous inflammation in positive temporal artery biopsies is most evident at the media and adventitia or is uniform throughout the layers of the artery. Our study lends support to the theory that the muscularis and adventitia may play an inciting role in the pathogenesis of temporal arteritis.

Use of Topical Insulin to Treat Refractory Neurotrophic Corneal Ulcers.

PURPOSE: To report the clinical course of 6 patients with refractory neurotrophic corneal ulcers that were treated with topical insulin drops. METHODS: Retrospective chart review of patients who had neurotrophic corneal ulcers or epithelial defects refractory to standard medical and surgical treatment. Insulin drops, prepared by mixing regular insulin in artificial tears with a polyethylene glycol and propylene glycol base at a concentration of 1 unit per milliliter, were prescribed 2 to 3 times daily. RESULTS: Six patients, aged 2 to 73 years, developed neurotrophic corneal ulcers refractory to a range of medical and surgical treatments, including bandage contact lens, amniotic membrane grafting, and permanent tarsorrhaphy. Each patient was administered topical insulin drops with complete corneal reepithelialization within 7 to 25 days. CONCLUSIONS: Topical insulin may be a simple and effective treatment for refractory neurotrophic corneal ulcers. Further study is required to determine the clinical efficacy and side effect profile of insulin drops.

Prevalence of Antiretinal Antibodies in Acute Zonal Occult Outer Retinopathy: A Comprehensive Review of 25 Cases.

PURPOSE: To perform a comprehensive review and to investigate the presence and role of autoimmune antibodies in 25 cases of acute zonal occult outer retinopathy (AZOOR) identified using the classification originally proposed by J. Donald Gass. DESIGN: Observational case series. METHODS: Setting: Institutional. STUDY POPULATION: Twenty-five patients were identified by characteristic symptoms (abrupt onset of photopsias, followed by large scotomata at or connected to the blind spot), ocular findings (paucity of pigmentary changes with no sign of vitreous inflammation and abnormal electroretinogram in at least 1 eye), and a negative family history for retinitis pigmentosa. OBSERVATION PROCEDURES: Patients underwent a full comprehensive ophthalmologic examination, fundus retinography, Goldmann kinetic visual field (GVF), and full-field electroretinogram (ffERG). Blood samples were also obtained to verify for the presence of antiretinal antibodies by Western blot analysis. MainOutcome Measures: Clinical presentation, best-corrected visual acuity (BCVA), fundus abnormalities, visual field defects, ffERG changes, and presence of antiretinal antibodies. RESULTS: Sixteen patients (64%) presented with photopsias, 56% (14/25) with night blindness, and 56% (14/25) with loss of peripheral vision. Sixty-four percent (16/25) of cases were bilateral. All patients demonstrated retinal vascular attenuation, optic nerve head pallor, and mottling of retinal pigment epithelium. The most common visual field changes included enlargement and expansion of the blind spot extending into large pericentral or other types of scotomata (64%). Both scotopic and photopic ffERG values were abnormal and affected to a similar degree in our patients. Nine patients (36%) had a greater than 20% asymmetry in ERG values between the 2 eyes. All patients had antiretinal antibodies on Western blot with an average of 6.6 bands. CONCLUSION: Evidence suggests that AZOOR is a unique form of autoimmune retinopathy and retinal manifestation suggests possible antiretinal antibody leakage from the disc margin with spread of immune products under the retina, resulting in large scotomata that connect to the optic nerve head.