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PURPOSE: To evaluate the prevalence of retinal disease on fluorescein angiography (FA) in patients with incontinentia pigmenti (IP) and to compare the severity of retinal disease in those with and without known central nervous system (CNS) disease. DESIGN: Multi-institutional consecutive retrospective case series. SUBJECTS: New patients with a diagnosis of IP were seen at the Casey Eye Institute at the Oregon Health and Science University (OHSU), Moran Eye Center, University of Utah, or Bascom Palmer Eye Institute, University of Miami from December 2011 to September 2018. METHODS: Detailed ophthalmoscopic examination and FA were recommended for all new patients and performed on every patient who had parental consent. Ophthalmoscopic findings and FA images were graded for severity by 2 masked graders on a 3-point scale: 0 = no disease, 1 = vascular abnormalities without leakage, 2 = leakage or neovascularization, and 3 = retinal detachment. The presence of known CNS disease was documented. Additional cases were obtained from a pediatric retina listserv for examples of phenotypic variation. MAIN OUTCOME MEASURES: The proportion of eyes noted to have disease on ophthalmoscopy compared with FA and the severity of retinal disease in those with and without known CNS disease. RESULTS: Retinal pathology was detected in 18 of 35 patients (51%) by indirect ophthalmoscopy and 26 of 35 patients (74%) by FA (P = 0.048) in a predominantly pediatric population (median age, 9 months). Ten patients (29%) had known CNS disease at the time of the eye examination. A Wilcoxon rank-sum test indicated that the retinal severity scores for patients with CNS disease (median, 2) were significantly higher than the retinal severity scores for patients without CNS disease (median, 1), z = −2.12, P = 0.034. CONCLUSIONS: Retinal disease is present in the majority of patients with IP, and ophthalmoscopic examination is less sensitive than FA for detection of disease. There may be a correlation between the severity of retinal and CNS disease.

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

BACKGROUND AND OBJECTIVE: Dilation and examination during retinopathy of prematurity (ROP) screening can cause systemic complications and even life-threatening adverse events (AEs). The main objective of this study is to assess the prevalence and significance of AEs and changes in vital signs during ROP screenings, and to correlate birth weight, gestational age, and length of exam as possible predictors of these changes. PATIENTS AND METHODS: This is a prospective, observational study that includes 1,360 screening exams from 680 premature infants in two neonatal intensive care units. Systolic blood pressure, diastolic blood pressure, heart rate, respiratory rate, and oxygen saturation were monitored from the time that dilating drops were administered until 4 hours after the examination was completed. Any clinical deterioration or complication was documented. RESULTS: Of 1,360 infant screening exams, 153 (11%) screening exams resulted in at least one change in vital sign that alerted the monitor. There was one serious AE requiring bag valve mask resuscitation 30 minutes after dilating drops were placed. Gestational age, birth weight, and length of ROP exam were not significant predictors of AEs. There were statistically significant changes when comparing pre- and post-vital signs following dilation and funduscopy exam. CONCLUSIONS: Although ROP screening exams are essential for at-risk neonates, mydriatic drops and retinal exams can result in changes to vital signs and AEs. It is important to closely monitor neonates during these examinations. [ Ophthalmic Surg Lasers Imaging Retina . 2021;52:458–463.]

Purpose To describe the clinical characteristics, surgical outcomes, and management recommendations in patients with traumatic rhegmatogenous retinal detachment (RRD) resulting from self-injurious behavior (SIB). Design International, multicenter, retrospective, interventional case series. Participants Patients with SIB from 23 centers with RRD in at least 1 eye. Methods Clinical histories, preoperative assessment, surgical details, postoperative management, behavioral intervention, and follow-up examination findings were reviewed. Main Outcome Measures The rate of single-surgery anatomic success (SSAS) was the primary outcome. Other outcomes included new RRD in formerly attached eyes, final retinal reattachment, and final visual acuity. Results One hundred seven eyes with RRDs were included from 78 patients. Fifty-four percent of patients had bilateral RRD or phthisis bulbi in the fellow eye at final follow-up. The most common systemic diagnoses were autism spectrum disorder (35.9%) and trisomy 21 (21.8%) and the most common behavior was face hitting (74.4%). The average follow-up time was 3.3 ± 2.8 years, and surgical outcomes for operable eyes were restricted to patients with at least 3 months of follow-up (81 eyes). Primary initial surgeries were vitrectomy alone (33.3%), primary scleral buckle (SB; 26.9%), and vitrectomy with SB (39.7%), and 5 prophylactic SBs were placed. Twenty-three eyes (21.5%) with RRDs were inoperable. The SSAS was 23.1% without tamponade (37.2% if including silicone oil), and final reattachment was attained in 80% (36.3% without silicone oil tamponade). Funnel-configured RRD (P = 0.006) and the presence of grade C proliferative vitreoretinopathy (P = 0.002) correlated with re-detachment. The use of an SB predicted the final attachment rate during the initial surgery (P = 0.005) or at any surgery (P = 0.008. These associations held if restricting to 64 patients with ≥12 months followup. Anatomic reattachment correlated with better visual acuity (P Conclusions RRD resulting from SIB poses therapeutic challenges because of limited patient cooperation, bilateral involvement, chronicity, and ongoing trauma in vulnerable and neglected patients. The surgical success rates were some of the lowest in the modern retinal detachment literature. The use of an SB may result in better outcomes, and visual function can be restored in some patients.

We report the case of 10-year-old girl who developed retinal neovascularization and vitreous hemorrhage 10 years after successful treatment of type 1 retinopathy of prematurity infant with intravitreal bevacizumab without laser ablation therapy.

A 9-year-old female with a history of Bohring-Opitz syndrome (BOS), Down syndrome, and autism initially presented with bilateral cataracts and a total retinal detachment in her left eye secondary to chronic self-injurious behavior. The authors report the first case of self-induced retinal detachment and traumatic cataracts in a patient with BOS. For patients who present with self-injurious behavior, the authors advocate for behavioral modifications at home, including the use of “no-no's,” supplemental medication if necessary, and behavioral therapy to reduce the risk of self-induced visual injury. The authors also suggest the use of 25-gauge vitrectomy with silicone oil for retinal detachment repair. Finally, given the high risk of irreversible vision loss from amblyopia and recurrent retinal detachments in children with BOS and self-injurious behavior, the authors recommend regular 2-month interval ophthalmic follow-up. [ Ophthalmic Surg Lasers Imaging Retina . 2021;52:400–402.]

To investigate late retinal findings and complications of eyes with a history of retinopathy of prematurity (ROP) that did not meet treatment criteria and did not receive treatment during infancy.Retrospective, nonconsecutive, noncomparative, multicenter case series.Three hundred sixty-three eyes of 186 patients.Data were requested from multiple providers on premature patients with a history of ROP and no treatment during infancy who demonstrated late retinal findings or complications and included age, gender, gestational age and weight, zone and stage at infancy, visual acuity, current retina vascularization status, vitreous character, presence of peripheral retinal findings such as lattice retinal tears and detachments (RDs), retinoschisis, and fluorescein findings.Rate of RDs and factors conferring a higher risk of RDs.The average age was 34.5 years (range, 7-76 years), average gestational age was 26.6 weeks (range, 23-34 weeks), and average birth weight was 875 g (range, 425-1590 g). Findings included lattice in 196 eyes (54.0%), atrophic holes in 126 eyes (34.7%), retinal tears in 111 eyes (30.6%), RDs in 140 eyes (38.6 %), tractional retinoschisis in 44 eyes (11.9%), and visible vitreous condensation ridge-like interface in 112 eyes (30.5%). Fluorescein angiography (FA) was performed in 113 eyes, of which 59 eyes (52.2%) showed leakage and 16 eyes (14.2%) showed neovascularization. Incomplete vascularization posterior to zone 3 was common (71.6% of eyes). Retinal detachments were more likely in patients with a gestational age of 29 weeks or less (P0.05) and in eyes with furthest vascularization to posterior zone 2 eyes compared with zone 3 eyes (P = 0.009).Eyes with ROP not meeting the treatment threshold during infancy showed various late retinal findings and complications, of which RDs were the most concerning. Complications were seen in all age groups, including patients born after the Early Treatment for Retinopathy of Prematurity Study. Contributing factors to RDs included atrophic holes within peripheral avascular retina, visible vitreous condensation ridge-like interface with residual traction, and premature vitreous syneresis. We recommend regular examinations and consideration of ultra-widefield FA examinations. Prospective studies are needed to explore the frequency of complications and benefit of prophylactic treatment and if eyes treated with anti-vascular endothelial growth factor therapy are at risk of similar findings and complications.

Retinopathy of prematurity (ROP) is a leading cause of childhood blindness. Not only do the epidemiologic determinants and distributions of patients with ROP vary worldwide, but clinical differences have also been described. The Third Edition of the International Classification of ROP (ICROP3) acknowledges that aggressive ROP (AROP) can occur in larger preterm infants and involve areas of the more anterior retina, particularly in low-resource settings with unmonitored oxygen supplementation. As sub-specialty training programs are underway to address an epidemic of ROP in sub-Saharan Africa, recognizing characteristic retinal pathology in preterm infants exposed to unmonitored supplemental oxygen is important to proper diagnosis and treatment. This paper describes specific features associated with various ROP presentations: oxygen-induced retinopathy in animal models, traditional ROP seen in high-income countries with modern oxygen management, and ROP related to excessive oxygen supplementation in low- and middle-income countries: oxygen-associated ROP (OA-ROP).

Background Oral topical corticosteroid gels are widely used in dental medicine. Case studies of central serous retinopathy have been reported following administration of corticosteroids, but none so far coinciding with the use of topical fluocinonide gel. This case report further contributes to the database of potential risks of corticosteroid use. Case presentation A 40-year-old South Asian woman presented with decreased vision, pigment epithelial detachments, and serous retinal detachments in both eyes 1 month after starting treatment with topical fluocinonide 0.05%, a topical oral corticosteroid gel. Her condition resolved 6 months after discontinuing the use of the steroid. Conclusions To the best of our knowledge, this is the first case of idiopathic central serous retinopathy associated with the use of oral fluocinonide gel. Discontinuing the use of the steroid may result in resolution of the serous retinal detachment and improvement of visual symptoms. Patients and their doctors who prescribe this medication should be aware of this association.

To describe the clinical characteristics, surgical outcomes, and management recommendations in patients with traumatic rhegmatogenous retinal detachment (RRD) resulting from self-injurious behavior (SIB).International, multicenter, retrospective, interventional case series.Patients with SIB from 23 centers with RRD in at least 1 eye.Clinical histories, preoperative assessment, surgical details, postoperative management, behavioral intervention, and follow-up examination findings were reviewed.The rate of single-surgery anatomic success (SSAS) was the primary outcome. Other outcomes included new RRD in formerly attached eyes, final retinal reattachment, and final visual acuity.One hundred seven eyes with RRDs were included from 78 patients. Fifty-four percent of patients had bilateral RRD or phthisis bulbi in the fellow eye at final follow-up. The most common systemic diagnoses were autism spectrum disorder (35.9%) and trisomy 21 (21.8%) and the most common behavior was face hitting (74.4%). The average follow-up time was 3.3 ± 2.8 years, and surgical outcomes for operable eyes were restricted to patients with at least 3 months of follow-up (81 eyes). Primary initial surgeries were vitrectomy alone (33.3%), primary scleral buckle (SB; 26.9%), and vitrectomy with SB (39.7%), and 5 prophylactic SBs were placed. Twenty-three eyes (21.5%) with RRDs were inoperable. The SSAS was 23.1% without tamponade (37.2% if including silicone oil), and final reattachment was attained in 80% (36.3% without silicone oil tamponade). Funnel-configured RRD (P = 0.006) and the presence of grade C proliferative vitreoretinopathy (P = 0.002) correlated with re-detachment. The use of an SB predicted the final attachment rate during the initial surgery (P = 0.005) or at any surgery (P = 0.008. These associations held if restricting to 64 patients with ≥12 months followup. Anatomic reattachment correlated with better visual acuity (P0.001).RRD resulting from SIB poses therapeutic challenges because of limited patient cooperation, bilateral involvement, chronicity, and ongoing trauma in vulnerable and neglected patients. The surgical success rates were some of the lowest in the modern retinal detachment literature. The use of an SB may result in better outcomes, and visual function can be restored in some patients.

The authors present their concerns surrounding data presented in studies from 2018 and 2020 regarding very low dose bevacizumab for the treatment of retinopathy of prematurity. [ Ophthalmic Surg Lasers Imaging Retina . 2020;51:542–544.]

BACKGROUND AND OBJECTIVE: To describe a safe and dependable protocol for intravitreal injections for the treatment of retinopathy of prematurity (ROP). MATERIALS AND METHODS: SAFER is an acronym used to describe the injection protocol and includes (S)hort needle (4-mm length), (A)ntiseptic/antibiotic (5% to 10% topical betadine), (F)ollowup (48 to 72 hours post-injection), (E)xtra attention to detail (clean environment, injection site 0.75 mm to 1.0 mm posterior to limbus), and (R)echeck (1 to 2 weeks following injection and until mature vascularization or laser). RESULTS: No cases of cataract formation, endophthalmitis, or vitreous hemorrhage using this technique were reported in a recent retrospective chart review. CONCLUSION: This protocol is a safe way to inject anti-vascular endothelial growth factor and to monitor ROP progression following injection. [ Ophthalmic Surg Lasers Imaging Retina. 2020;51:402–406.]

To develop a nomogram based on age and disease type for sclerotomy placement in pars plana vitrectomy for infants and children with congenital and acquired vitreoretinopathies.A retrospective, single-center, single-surgeon comparative case series.A total of 171 eyes of 93 patients ranging in age from a postmenstrual age (PMA) of 34 weeks to a chronological age of 23 years with congenital and acquired vitreoretinopathies.The corneal white-to-white diameter and the distance between the ora serrata and surgical limbus (ora-limbus distance) in the nasal, temporal, inferior, and superior quadrants were measured externally in vivo under general anesthesia prior to planned pars plana vitrectomy or laser procedure. The average ora-limbus distance of the quadrants was calculated for each eye (mean ora-limbus distance); and mean ora-limbus distance was evaluated as a function of age and compared among age-matched subjects from control and disease groups. Results were used to create a nomogram for safe sclerotomy placement based on age and disease type.The main outcome measure was distance (millimeters) from the ora serrata to the surgical limbus; a secondary measurement was corneal white-to-white distance (millimeters).Among all subjects, the ora-limbus distance was greatest in the superior quadrant, followed by the temporal, inferior, and nasal quadrants. A positive logarithmic relationship between ora-limbus distance and age was demonstrated in all groups. Whereas age-adjusted mean ora-limbus distances were similar among the familial exudative vitreoretinopathy and control groups, growth trends differed in patients with coloboma, persistent fetal vasculature, Stickler syndrome and/or myopia, and retinopathy of prematurity (ROP). In ROP, when subjects12 months of age were considered separately, a negative correlation was found between the ora-limbus distance and PMA, with infants of lesser gestational age exhibiting greater restriction in ora-limbus distance.Placement of sclerotomies for pars plana vitrectomy in children requires consideration of ocular development in congenital and acquired vitreoretinopathies; we present an age-based nomogram for sclerotomy placement in infants and children based on analyses of ora-limbus distance in several pediatric ocular conditions.

BACKGROUND AND OBJECTIVE: Retinovascular anomalies in the fellow eyes of patients with Coats' disease have been described, but the clinical significance is unknown, as well as whether these lesions progress over time. PATIENTS AND METHODS: This is an international, multicenter, retrospective, observational cohort study of fellow-eye abnormalities on widefield fluorescein angiography in patients with Coats' disease. RESULTS: Three hundred fifty eyes of 175 patients with Coats' disease were analyzed. A total of 33 patients (18.8%) demonstrated abnormal fellow-eye findings: 14 (42.4%) telangiectasias, 18 (54.5%) aneurysms, six (18.2%) segmental non-perfusion, six (18.2%) leakage, and two (6.0%) vascular tortuosity. All eyes were asymptomatic, and none of the lesions progressed over time. There was no association between fellow-eye findings with severity of Coats' disease ( P = .16), patient age ( P = .16), or presence of systemic vascular disease ( P = .16). CONCLUSIONS: The vascular abnormalities in fellow eyes of patients with Coats' disease did not progress over time. Observation is a reasonable initial management strategy. [ Ophthalmic Surg Lasers Imaging Retina . 2019;50:221–227.]

An 11-year-old girl with a history of neutropenia, developmental delay, hypotonia, and intellectual disability was diagnosed with Cohen syndrome after genetic testing discovered homozygous mutation in the VPS13B gene. She was referred to a retinal specialist with a chief complaint of decreased peripheral vision. On examination, decreased visual acuity, pigmentary changes, and nonleaking cystoid macular edema were present in both eyes.

PURPOSE To evaluate angiographic findings in neonates up to 150 weeks postmenstrual age who received intravitreal ranibizumab for primary treatment of Type 1 retinopathy of prematurity. METHODS Retrospective evaluation of fluorescein angiogram findings was completed for 30 eyes of 16 neonates who received intravitreal ranibizumab as primary treatment for Type 1 retinopathy of prematurity between April 2013 and January 2015. Outcome measures included maturity to Zone III, vascular blunting, vascular loops, vascular dilatation, capillary dropout, and vascular fluorescein leakage. RESULTS Mean gestational age was 24 weeks and mean postmenstrual age at time of intravitreal ranibizumab treatment was 35 weeks. Fluorescein angiograms performed at 44 weeks to 150 weeks postmenstrual age showed only 50% of eyes reached vascularization to Zone III; 40% had persistent vascular leakage; and ≥90% exhibited vascular blunting, vascular dilatation, and/or capillary dropout. CONCLUSION Although intravitreal ranibizumab is effective in initial cessation of Type 1 retinopathy of prematurity, vascularization to Zone III was only achieved in 50% of eyes in our series and most eyes had fluorescein angiography evidence of vascular anomalies. If future studies are performed comparing treatment with laser photocoagulation to anti-vascular endothelial growth factor, fluorescein angiographic studies should be considered to assess the status of the peripheral retinal vasculature to determine treatment effect.

Purpose To characterize the expression of vascular endothelial growth factor (VEGF) in a patient with retinopathy of prematurity (ROP) treated with ranibizumab (Case 1) and compare it with a case of ROP without treatment (Case 2), a case of a premature baby without ROP (Case 3), and a case of a baby without history of ROP or prematurity (Case 4). Design Observational case series. Methods The eyes of the deceased babies were removed postmortem and were sent to the Florida Lions Ocular Pathology Laboratory, where they were processed. The specimens were immunostained using an antibody against VEGF. Results All eyes except for the eyes in Case 4 disclosed positive VEGF staining. Positive staining was present within the nerve fiber layer, inner plexiform layer, and inner and outer nuclear layers and within the spindle-shaped cell population in the vanguard in Case 1. In the posterior pole, positive staining was only observed at the level of the nerve fiber layer. This case also demonstrated less positive staining when compared with Case 2, where positive staining was found within all layers of the retina. Conclusion Less VEGF staining was observed within the retina of the eyes treated with ranibizumab when compared with the VEGF staining in Case 2. This supports the idea that anti-VEGF agents are effective in reducing the amount of VEGF present in the retina. Furthermore, the fact that some expression of VEGF remains in the immature retina after injection supports the idea that anti-VEGF agents can suppress uncontrolled neovascularization without completely blocking the vascular drive for the vascularization of the immature retina.

Extended release of immunosuppressive drugs and sustained drug levels are desirable for the treatment of corneal graft rejection and other ocular immune disorders. This experiment was conducted with biodegradable microspheres containing cyclosporine A to assess their suitability for achieving this goal. Microspheres containing cyclosporine A were prepared using a solvent evaporation process. A mixture of poly(lactic) and poly(glycolic) acid polymers (50:50) and cyclosporine A was dissolved in a mixture of chloroform and acetone. The solution was then emulsified in an aqueous solution of polyvinyl alcohol and stirred for 24 hours to evaporate the organic solvent. The final assayed concentration of cyclosporine A was 15.38 mg/mL. A 0.13 mL aliquot (2.0 mg) of the suspension of the microspheres was injected subconjunctivally in 24 eyes of white New Zealand rabbits. The concentration of cyclosporine A in the aqueous and cornea was measured at 6, 12, 24, 48, 72, and 144 hours after injection (n = 4 for each group). Corneal levels of cyclosporine A ranged from 2392 +/- 70 ng/mL at 6 hours to 1297 +/- 459 ng/mL at 144 hours. The aqueous levels ranged from 110 +/- 0 ng/mL at 6 hours to 62 +/- 11 ng/mL at 144 hours. These data indicate that a microsphere drug delivery system is an effective means of delivering cyclosporine A to the cornea and anterior chamber, and may provide an alternative for the treatment of ocular immune disorders.