Spontaneous Resolution of a Combined Hamartoma in a One-Year-Old Child.

The combined hamartoma of the retina and the retinal pigment epithelium (CHR-RPE) is a rare but histologically benign tumor of which no clear consensus exists regarding its appropriate management. Most commonly CHR-RPE presents unilaterally in children as an elevated, pigmented lesion associated with vascular tortuosity and an epiretinal membrane (ERM). Patients may have decreased visual acuity (VA) in the affected eye, notably if the overlying ERM extends into the macula. For this reason, surgical intervention in the form of ERM removal has been pursued with notable success. Still, those who pursue observation cite the interwoven nature of the overlying glial membrane within the dysplastic retina, complicating surgery and accounting for variability in post-operative VA success. Given the rarity of the tumor coupled with the scarcity of data on its natural evolution, clinicians who pursue observation cannot know for sure whether withholding surgical management is predisposing their patients to worse visual outcomes as compared to those patients who undergo ERM peel as first-line therapy. This case report will discuss an infant with clinically diagnosed CHR-RPE whose lesion showed significant regression after 6 months of observation. This success story should cause Ophthalmologists to reconsider the management options at their disposal when faced with a case of CHR-RPE, and to call for further research to better define the risks of observation over surgical intervention. [Ophthalmic Surg Lasers Imaging Retina 2024;55:XX-XX.].

Bilateral Combined Rhegmatogenous and Tractional Retinal Detachments Due to Persistent High-Titer Syphilis.

A 23-year-old man developed bilateral rhegmatogenous retinal detachments secondary to high-titer ocular syphilis. The patient's titer increased four-fold after completing a 14-day course of intravenous penicillin (IVP). He underwent bilateral pars plana vitrectomy with silicone oil fill in both eyes. In this article, the authors propose an updated treatment method for patients with advanced ocular syphilis that includes oral doxycycline for 30 days following 14 days of IVP to optimally minimize the patient's infectious burden. Following surgery and this new treatment regime, this patient's best-corrected visual acuity 10 weeks postoperatively measured 20/50 in the right eye and 20/30 in the left eye. This case highlights a rare but devastating complication of ocular syphilis. We suggest the addition of oral doxycycline to IVP for patients with syphilis titers ≥ 1:256, HIV co-infection, and presence of posterior retinitis. [Ophthalmic Surg Lasers Imaging Retina 2024;55:46-50.].

Potential application of foveal structural measurements in treatment decision for retinopathy of prematurity: an OCT-based study.

PURPOSE: To investigate foveal changes in infants with ROP not requiring treatment(nROP) and ROP infants needing treatment (tROP) using a handheld SD-OCT device. METHOD: We performed horizontal SD-OCT scans through the fovea in 156 eyes of 81 infants diagnosed with ROP. Foveal immaturity indices including the presence of inner retinal layers (IRL), absence of foveal outer nuclear layers widening (ONL) and attenuation of hyperreflective outer segment layers (OS), presence and type of cystoid macular edema (CME), epiretinal membrane (ERM), foveal pit depth (FPD), foveal pit width (FPW) and central foveal thickness (CFT) were calculated. The multivariate logistic regression model was used to predict the need for treatment based on OCT measurements. RESULTS: The shape of the foveolar pit was not significantly different among tROP and nROP groups (P-value = 0.287, Chi-square test). IRL extrusion was incomplete in both tROP and nROP groups (P-value = 0.0.41, Chi-square test). Nevertheless, the presence of thicker IRL was more frequent in the nROP group in comparison with the tROP group (100% vs.64.8%, P-value = 0.001). CME was observed in 29% of eyes in the tROP group and 40% of eyes in the nROP group; however, this difference was not statistically significant (P-value = 0.32, Chi-square test). ERM was detected in 15 (75%) and 84 (65.6%) eyes in the nROP and tROP groups, respectively (P-value = 0.39, Chi-square test). Multivariate logistic regression analyses showed that the need for treatment was significantly associated with gestational age (GA), CFT and FPD (P-values 0.001 and 0.002 respectively). CONCLUSIONS: This study demonstrated GA, foveal pit depth and the central foveal thickness could accurately predict the need for treatment with sensitivity, specificity, and diagnostic accuracy of 97%, 65% and 91.7% respectively.

Comparison in Retreatments between Bevacizumab and Ranibizumab Intravitreal Injections for Retinopathy of Prematurity: A Multicenter Study.

PURPOSE: To compare the types and dosages of anti-vascular endothelial growth factors (VEGFs) to ascertain whether specific dosages or types of injection were associated with retreatment in clinical practice in the United States. DESIGN: Multicenter, retrospective, consecutive series. PARTICIPANTS: Patients with retinopathy of prematurity (ROP) treated with anti-VEGF injections from 2007 to 2021. METHODS: Sixteen sites from the United States participated. Data collected included demographics, birth characteristics, examination findings, type and dose of anti-VEGF treatment, retreatment rates, and time to retreatment. Comparisons of retreatment rates between bevacizumab and ranibizumab intravitreal injections were made. MAIN OUTCOME MEASURES: Relative rate of retreatment between varying types of anti-VEGF therapy, including bevacizumab and ranibizumab, and the various dosages used for each. RESULTS: Data from 873 eyes of 661 patients (61% male and 39% female) were collected. After exclusion of 40 eyes treated with laser before anti-VEGF injection and 266 eyes re-treated with laser at or beyond 8 weeks after the initial anti-VEGF treatment, 567 eyes of 307 patients (63% male and 37% female) remained and were included in the primary analysis. There was no difference between the no retreatment and retreatment groups in terms of birthweight, gestational age, age at first injection, ROP stages, or number of involved clock hours. The retreatment group had a larger percentage of aggressive ROP (34% vs. 18%, P < 0.001) and greater percentage of zone 1 ROP (49 vs. 34%, P = 0.001) than the no retreatment group. Ranibizumab use was associated with a higher rate of retreatment than bevacizumab use (58% vs. 37%, P < 0.001), whereas the rate of retreatment was not associated with a specific dose of ranibizumab (R2 = 0.67, P = 0.32). Meanwhile, lower doses of bevacizumab were associated with higher rates of retreatment compared with the higher doses (R2 = 0.84, P = 0.01). There was a dose-specific trend with higher doses trending toward lower retreatments for bevacizumab. CONCLUSIONS: In a multicenter study of ROP patients initially treated with anti-VEGF therapy, ranibizumab and lower-dose bevacizumab use were associated with an increased rate of retreatment when compared with higher-dose bevacizumab. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

Practice Patterns and Outcomes of Intravitreal Anti-VEGF Injection for Retinopathy of Prematurity: An International Multicenter Study.

PURPOSE: To report practice patterns of intravitreal injections of anti-VEGF for retinopathy of prematurity (ROP) and outcomes data with a focus on retreatments and complications. DESIGN: Multicenter, international, retrospective, consecutive series. SUBJECTS: Patients with ROP treated with anti-VEGF injections from 2007 to 2021. METHODS: Twenfty-three sites (16 United States [US] and 7 non-US) participated. Data collected included demographics, birth characteristics, examination findings, and methods of injections. Comparisons between US and non-US sites were made. MAIN OUTCOME MEASURES: Primary outcomes included number and types of retreatments as well as complications. Secondary outcomes included specifics of the injection protocols, including types of medication, doses, distance from limbus, use of antibiotics, and quadrants where injections were delivered. RESULTS: A total of 1677 eyes of 918 patients (43% female, 57% male) were included. Mean gestational age was 25.7 weeks (range, 21.2-41.5 weeks), and mean birth weight was 787 g (range, 300-2700 g). Overall, a 30-gauge needle was most commonly used (51%), and the quadrant injected was most frequently the inferior-temporal (51.3%). The distance from the limbus ranged from 0.75 to 2 mm, with 1 mm being the most common (65%). Bevacizumab was the most common anti-VEGF (71.4%), with a dose of 0.625 mg in 64% of cases. Overall, 604 (36%) eyes required retreatment. Of those, 79.8% were retreated with laser alone, 10.6% with anti-VEGF injection alone, and 9.6% with combined laser and injection. Complications after anti-VEGF injections occurred in 15 (0.9%) eyes, and no cases of endophthalmitis were reported. Patients in the United States had lower birth weights and gestational ages (665.6 g and 24.5 weeks, respectively) compared with non-US patients (912.7 g and 26.9 weeks, respectively) (P < 0.0001). Retreatment with reinjection and laser was significantly more common in the US compared with the non-US group (8.5% vs. 4.7% [P = 0.0016] and 55% vs. 7.2% [P < 0.001], respectively). There was no difference in the incidence of complications between the 2 geographic subgroups. CONCLUSIONS: Anti-VEGF injections for ROP were safe and well tolerated despite a variance in practice patterns. Infants with ROP receiving injections in the US tended to be younger and smaller, and they were treated earlier with more retreatments than non-US neonates with ROP.

Retinopathy of Prematurity Screening Examination and Changes in Vital Signs.

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.].

Self-Induced Bilateral Retinal Detachments and Traumatic Cataracts in a Patient With Bohring-Opitz Syndrome.

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.].

Very late-onset reactivation of retinopathy of prematurity post anti-VEGF bevacizumab treatment for type 1 ROP: a case report.

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.

CHOROIDAL NEOVASCULARIZATION AND CHORIORETINAL ATROPHY IN A PATIENT WITH MELANOMA-ASSOCIATED RETINOPATHY AFTER IPILIMUMAB/NIVOLUMAB COMBINATION THERAPY.

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.

Traumatic Retinal Detachment in Patients with Self-Injurious Behavior: An International Multicenter Study.

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 < 0.001). 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.

Playing With Fire.

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.].

SAFER-ROP: Updated Protocol for Anti-VEGF Injections for Retinopathy of Prematurity.

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.].

Central serous retinopathy associated with topical oral corticosteroid use: a case report.

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.

Longitudinal Examination of Fellow-Eye Vascular Anomalies in Coats' Disease With Widefield Fluorescein Angiography: A Multicenter Study.

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.].

FLUORESCEIN ANGIOGRAPHIC EVALUATION OF PERIPHERAL RETINAL VASCULATURE AFTER PRIMARY INTRAVITREAL RANIBIZUMAB FOR RETINOPATHY OF PREMATURITY.

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.

Nonleaking cystoid macular edema in Cohen syndrome.

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.

Histopathologic Characterization of the Expression of Vascular Endothelial Growth Factor in a Case of Retinopathy of Prematurity Treated With Ranibizumab.

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.