Association of Health Insurance Status With Severity and Treatment Among Infants With Retinopathy of Prematurity.

This study describes retinopathy of prematurity treatment practices using Medicaid and commercial claims databases. Infants with Medicaid tend to be sicker overall and have higher rates of retinopathy of prematurity requiring treatment than those with commercial insurance. Among patients who required treatment, those with Medicaid were more likely to receive anti-vascular endothelial growth factor than laser treatment. [J Pediatr Ophthalmol Strabismus. 2023;60(6):e75-e78.].

Smartphone application links severity of retinopathy of prematurity to early motor behavior in a cohort of high-risk preterm infants.

PURPOSE: To evaluate the General Movement Assessment (GMA) with the Motor Optimality Score-Revised (MOS-R) as a neurodevelopmental marker in infants with retinopathy of prematurity (ROP). METHODS: Infants screened prospectively for ROP were evaluated at 3 months' post-term age using a smartphone application to complete the GMA and MOS-R. Results were analyzed by ROP severity. RESULTS: Of 105 enrolled infants, 83 completed the study. Of these, 54 (65%) had any ROP, 32 (39%) had severe ROP, and 13 (16%) had type 1 ROP. The proportion with aberrant GMA was significantly higher in infants with severe ROP (14/32 [44%]) compared with infants who had milder ROP (8/51 [16%]; P = 0.006). Of those with severe ROP, there was no significant difference comparing infants with type 1 ROP treated with bevacizumab (7/13 [54%]) to infants with type 2 ROP without treatment (7/19 [37%]; P = 0.47). Although the presence of any ROP, stage of ROP, and severe ROP each predicted lower MOS-R scores on univariate analyses, only severe bronchopulmonary dysplasia and markers of brain injury remained significant in the multivariate analysis. CONCLUSIONS: The GMA was a convenient, short-term method of data collection with low attrition. Although severe ROP initially appeared linked to poor early motor scores, this association is likely confounded by neurological and respiratory complications, which frequently accompany severe ROP.

Exudative Retinal Detachment Following Chimeric Antigen Receptor T-Cell Therapy in Relapsed B-Cell Acute Lymphoblastic Leukemia.

Chimeric antigen receptor (CAR) T-cell therapy has become a novel approach in the treatment of many hematologic malignancies. However, ocular adverse effects have not been well described. This report presents a case of a pediatric patient with relapsed B-cell acute lymphoblastic leukemia with ocular involvement treated with CAR T-cell therapy who developed an exudative retinal detachment likely secondary to an inflammatory response to CAR T-cell therapy. [Ophthalmic Surg Lasers Imaging Retina. 2022;53:113-115.].

Retinoblastoma presenting as pseudohypopyon and preserved visual acuity.

PURPOSE: To describe a Case of retinoblastoma that presented subtly as a pseudohypopyon in a child with preserved visual acuity. OBSERVATIONS: A 3-year-old male was referred for concern of hypopyon in the left eye. Initial examination revealed 20/30 vision, a pseudohypopyon, and a large white mass on fundoscopy. Examination under anesthesia revealed extensive retinoblastoma with vitreous seeds and anterior chamber involvement. Enucleation was performed and histology demonstrated retinoblastoma with tumor cells found within the ciliary body, iris, iridocorneal angle, and Schlemm canal. Based on the high-risk histopathology findings, adjuvant chemotherapy was performed. CONCLUSIONS AND IMPORTANCE: Retinoblastoma is the most common primary intraocular malignancy in children. Though the classic presentation is leukocoria and/or strabismus, it can present in a variety of ways. Physicians should be aware that retinoblastoma, even severe forms, can present subtly with pseudohypopyon and preserved vision. Adjuvant chemotherapy for anterior segment involvement remains controversial.

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.

Macular Sequelae Following Exudative Retinal Detachment After Laser Photocoagulation for Retinopathy of Prematurity.

BACKGROUND AND OBJECTIVE: To report a series of exudative retinal detachments (ERDs) following laser photocoagulation for retinopathy of prematurity (ROP). PATIENTS AND METHODS: Retrospective case series. RESULTS: Eleven eyes of seven infants were identified who developed ERD following laser. Median gestation age was 25 weeks (interquartile range [IQR]: 24-27 weeks), and median birth weight was 662 grams (IQR: 538-850 grams). Median postmenstrual age at time of laser was 35 weeks (IQR: 33-39 weeks). ERD was diagnosed at a median of 7 days (IQR: 5-7 days) after laser and was managed with steroids. Bevacizumab was also used for certain cases. Time to resolution ranged from 1 to 5 weeks. Macular pigment changes, atrophy, window defect on fluorescein angiography, and photoreceptor loss on optical coherence tomography were noted in some cases following ERD resolution. Excluding one patient who expired at 3 months, median length of follow-up was 10 years (IQR: 9-13.5 years). Overall, only one patient, who presented with less severe ERD, had normal vision. CONCLUSIONS: ERD is an uncommonly reported complication following laser for ROP. Macular changes following ERD resolution may have negative visual consequences. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:698-705.].

Two-Year Outcomes Comparing Anti-VEGF Injections to Laser for ROP Using a Commercial Claims Database.

BACKGROUND AND OBJECTIVE: To report ocular and neurodevelopmental outcomes among infants treated for retinopathy of prematurity (ROP) in a nationwide health insurance claims database. PATIENTS AND METHODS: Retrospective cohort study of 298 infants treated with laser or anti-vascular endothelial growth factor (VEGF) injection identified in the MarketScan database (2011-2017) with 2-year follow-up. RESULTS: A review of claims data found 298 patients with International Classification of Diseases and Common Procedural Technology codes for ROP treatment and 2 years of continuous insurance coverage. Of these, 63 infants received injections and 235 received laser. Overall, the anti-VEGF group had higher rates of underlying neurological comorbidities (35% vs. 23%; P = .05) and thrombocytopenia (17% vs. 8%; P = .02). Most ocular outcomes were similar, including retinal detachment (P = .87). There were higher rates of second procedures after injection (44% vs. 10%; P < .001). Rates of language, motor, and cognitive delays were similar. Rates of cerebral palsy were higher with injections but were not statistically significant after adjusting for comorbidities (odds ratio = 1.88; P = .10). CONCLUSIONS: The prevalence of retinal detachment after 2 years was similar comparing anti-VEGF to laser. Despite the higher rates of underlying neurologic comorbidity in the injection group, there were no differences in language, motor, or cognitive delays. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:486-493.].

Late visual outcomes in infants treated with primary bevacizumab for type 1 retinopathy of prematurity.

PURPOSE: To describe visual acuity findings after 4 years of age in infants treated with primary bevacizumab (IVB) for type 1 retinopathy of prematurity (ROP) and to correlate structural findings on fluorescein angiography (FA) with functional outcomes. METHODS: Infants born between January 2011 and January 2014 were identified by review of the medical records. Visual acuity was measured in clinic after 4 years of age. As described in the ET-ROP study, normal visual acuity was 20/40 (logMAR 0.3) or better. Examination under anesthesia with FA and prophylactic laser if necessary was recommended for all patients who received primary IVB. Vascular abnormalities were reviewed by 2 experts. RESULTS: A total of 23 infants (46 eyes) completed visual acuity testing. Median age was 6 years (IQR, 4-7). Median visual acuity was logMAR 0.18 (IQR, 0-0.3). Normal vision was recorded for 39/46 (85%) eyes. Twenty-one patients (42 eyes) completed an examination under anesthesia with FA. All eyes had some peripheral capillary abnormalities (shunts, tangles, or abnormal branching); most had peripheral nonperfusion (90%) and leakage (64%). CONCLUSIONS: Most eyes treated with IVB for type 1 ROP had normal visual acuity. Our results after IVB in this study compare favorably to 6-year visual outcomes in the ET-ROP study, in which 34.6% of early-treated eyes had normal visual acuity (20/40 or better). Nonetheless, a high percentage of eyes had abnormal vascular patterns on FA, which may be related to underlying ROP or to treatment. Peripheral vascular changes are common in eyes with ROP treated with IVB, but they do not preclude excellent visual acuity.

FEVR phenotype associated with septo-optic dysplasia.

Background: Septo-optic dysplasia, also known as de Morsier syndrome, is a disorder of brain development characterized by optic nerve hypoplasia, hypopituitarism, and midline brain defects.Materials and Methods: Single retrospective case report.Results: An infant born at 38 5/7 weeks gestation age weighing 3125 g developed respiratory distress shortly after birth. Systemic findings included myocardial dysfunction, hypopituitarism, feeding intolerance, microphallus, and dysmorphic features. Eye examination revealed tractional retinal detachments and optic nerve hypoplasia. In addition, peripheral non-perfusion and peripheral neovascularization were consistent with Familial Exudative Vitreoretinopathy (FEVR) phenotype. MRI showed hypoplastic optic nerves, ectopic posterior pituitary with hypoplastic pituitary infundibulum, and slightly thin corpus callosum, diagnostic of septo-optic dysplasia. Genetic testing revealed no pathogenic variants and two variants of uncertain significance.Conclusion: FEVR findings can be associated with septo-optic dysplasia and may point to an etiologic connection between neural development and subsequent vascular development.

Efficacy of Retinal Lesion Screening in Von Hippel-Lindau Patients With Widefield Color Fundus Imaging Versus Widefield FA.

BACKGROUND AND OBJECTIVE: Retinal hemangioblastoma is a retinal tumor in patients with Von Hippel-Lindau (VHL). The authors' objective was to determine whether widefield fluorescein angiography (FA) improves lesion detection. PATIENTS AND METHODS: Retrospective case series of VHL patients who underwent widefield fundus imaging and FA. Masked retina specialists graded fundus images as having: 1) definite retinal hemangioblastoma; 2) possible lesion; 3) no lesion. The number of lesions on FA was compared to widefield color imaging. RESULTS: One hundred six eyes of 55 patients were evaluated. A total of 94 lesions were identified on FA in 61.8% patients. Forty-three lesions (45.7%) were not identified on fundus images. Small lesion detection was significantly higher with FA compared to color imaging (P = .013). CONCLUSIONS: This study reports on improved detection of retinal hemangioblastomas with widefield FA compared to widefield fundus images. The authors recommend VHL monitoring guidelines to include periodic widefield FA to adequately screen for smaller lesions. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:e260-e265.].

Neurodevelopmental Outcomes Comparing Bevacizumab to Laser for Type 1 ROP.

BACKGROUND AND OBJECTIVE: To evaluate neurodevelopmental outcomes among infants treated for retinopathy of prematurity (ROP) at the authors' institution. PATIENTS AND METHODS: Before-and-after retrospective chart reviews identified 40 infants treated with laser and 46 treated with primary intravitreal bevacizumab (IVB). Primary outcomes were death, hearing loss, bilateral visual impairment (BVI), and cerebral palsy (CP); odds ratios (ORs) were calculated to determine factors associated with CP. Secondary outcomes were mean Bayley Scales of Infant and Toddler Development, Third Edition (BSID-III) scores. RESULTS: Overall, there were no significant differences in primary outcome measures by treatment group. However, adjusted odds of BVI were significantly higher with laser compared to IVB (OR = 13.1; P = .038). Although IVB was not associated with CP, both hydrocephalus and BVI were strongly correlated with CP. Mean Bayley-III scores were similar when comparing nine laser-treated infants to 13 IVB-treated infants. CONCLUSIONS: Visual outcomes are an important aspect of neurodevelopment. IVB was not associated with severe developmental disabilities and may protect against vision loss in this analysis. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:337-343.].

Foveal Development After Use of Bevacizumab for Aggressive Posterior Retinopathy of Prematurity.

Foveal development can occur after intravitreal bevacizumab (IVB) treatment for aggressive posterior retinopathy of prematurity (APROP). A 1,310 g male twin, born at 31 weeks, was diagnosed with APROP and undeveloped fovea at 33 weeks. IVB was injected in both eyes. Unfortunately, multiple surgical interventions were required to treat retinal detachment in the left eye, at which time, foveal development was studied in the right eye. Imaging revealed development of foveal capillary ring, avascular zone, and shallow pit. Although bevacizumab is an inhibitor of angiogenesis and delays vascular advancement, development of foveal capillary vascular network with foveal avascular zone and pit can proceed despite multiple treatments. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:e185-e187.].

Refractive outcomes comparing primary laser to primary bevacizumab with delayed laser for type 1 ROP.

PURPOSE: To compare the refractive outcomes of intravitreal bevacizumab (IVB) and delayed peripheral retinal photocoagulation (PRP) with primary PRP in infants treated for posterior type 1 ROP. METHODS: The medical records of 87 infants at a tertiary referral center treated for posterior type 1 ROP between 2006 and 2016 were reviewed retrospectively. Consecutive infants received primary PRP before and primary IVB after a change in treatment practice implemented in early 2011. In most cases primary IVB was supplemented with prophylactic laser treatment after 60 weeks' PMA (IVB-PRP). The main outcome was spherical equivalent (SE) in diopters, determined by cycloplegic refraction between 2 and 4 years. Infants treated with IVB-PRP were also compared to the those who received only IVB as monotherapy. RESULTS: The final analysis included 34 eyes of 19 infants in the primary PRP group and 40 eyes of 21 infants in the IVB-PRP group. Mean SE was -7.4 ± 5.2 D in the primary PRP group and -0.16 ± 2.2 D in the IVB-PRP group (P < 0.001). This relationship persisted after stratification by zone of ROP and the presence of aggressive posterior ROP. There was no statistically significant difference in mean SE between the IVB-PRP group and the 8 eyes of 4 infants who received IVB as monotherapy. Of 46 infants who received primary IVB, 37 completed an examination under anesthesia after 60 weeks' PMA. In these patients, 70% of eyes showed peripheral vascular leakage on fluorescein angiography. CONCLUSIONS: In our study cohort, infants treated with IVB-PRP were significantly less myopic than those treated with primary PRP. Delayed laser after 60 weeks' PMA, in hopes of reducing the risk of late reactivation with retinal detachment, did not negate the refractive benefits of primary IVB.

Late ROP Reactivation and Retinal Detachment in a Teenager.

Exudative and tractional retinal detachments have been reported as late sequelae of retinopathy of prematurity (ROP). The authors report a case of unilateral retinal detachment in a 19-year-old patient with history of peripheral ablation for ROP in the contralateral eye. This late reactivation is likely due to persistent avascular retina producing constant low levels of vascular endothelial growth factor. Therefore, close follow-up and ablation of peripheral avascular retina should be considered in patients with ROP, regardless of treatment history, to decrease lifelong risk of severe complications, including retinal detachment decades later. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:625-628.].

The importance of early diagnosis of Stickler syndrome: Finding opportunities for preventing blindness.

Stickler Syndrome (SS) is a significant cause of retinal blindness in children. The immediate cause of blindness is retina detachment from giant retinal tear (GRT). It is frequently diagnosed late and the giant retinal tear (GRT) may be complicated by high-grade proliferative vitreoretinopathy (PVR). The surgery for the combined GRT with PVR has limited structural results and the vision mainly remains impaired. In order to improve the visual outcomes, we propose an organized program oriented toward early diagnosis and surveillance. Adding an effective prophylaxis may maintain normal vision in a high percent of patients. The critical diagnostic moments for this program are prenatal and at birth. The tools include a directed history, general physical exam and advanced ophthalmologic exam looking for the particular features of SS. Some features may need advanced skills transfer, because they are not reliably taught in retina fellowships. Much of this program requires a partnership with obstetricians, pediatricians, neonatologists and geneticists. Finally, we review the evidence regarding prophylaxis and discuss our approach in the absence of guidance from a randomized clinical trial.

Reactivation of Retinopathy of Prematurity Three Years After Treatment With Bevacizumab.

Although intravitreal injection of anti-vascular endothelial growth factor agents (IVA) can induce rapid regression of retinopathy of prematurity (ROP), late reactivation of the ROP can occur in the form of tractional retinal detachment (TRD) from contracted recurrent extraretinal fibrovascular proliferation. The authors report a case of bilateral TRD for recurrent ROP in a 3-year-old, which is the latest-reported reactivation to date. The authors propose that persistent avascular retina in eyes that have undergone IVA for ROP receive laser ablation to prevent late recurrences. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:255-259.].

Immediate Sequential Bilateral Pediatric Vitreoretinal Surgery: An International Multicenter Study.

PURPOSE: To determine the feasibility and safety of bilateral simultaneous vitreoretinal surgery in pediatric patients. DESIGN: International, multicenter, interventional, retrospective case series. PARTICIPANTS: Patients 17 years of age or younger from 24 centers worldwide who underwent immediate sequential bilateral vitreoretinal surgery (ISBVS)-defined as vitrectomy, scleral buckle, or lensectomy using the vitreous cutter-performed in both eyes sequentially during the same anesthesia session. METHODS: Clinical history, surgical details and indications, time under anesthesia, and intraoperative and postoperative ophthalmic and systemic adverse events were reviewed. MAIN OUTCOME MEASURES: Ocular and systemic adverse events. RESULTS: A total of 344 surgeries from 172 ISBVS procedures in 167 patients were included in the study. The mean age of the cohort was 1.3±2.6 years. Nonexclusive indications for ISBVS were rapidly progressive disease (74.6%), systemic morbidity placing the child at high anesthesia risk (76.0%), and residence remote from surgery location (30.2%). The most common diagnoses were retinopathy of prematurity (ROP; 72.7% [P < 0.01]; stage 3, 4.8%; stage 4A, 44.4%; stage 4B, 22.4%; stage 5, 26.4%), familial exudative vitreoretinopathy (7.0%), abusive head trauma (4.1%), persistent fetal vasculature (3.5%), congenital cataract (1.7%), posterior capsular opacification (1.7%), rhegmatogenous retinal detachment (1.7%), congenital X-linked retinoschisis (1.2%), Norrie disease (2.3%), and viral retinitis (1.2%). Mean surgical time was 143±59 minutes for both eyes. Higher ROP stage correlated with longer surgical time (P = 0.02). There were no reported intraoperative ocular complications. During the immediate postoperative period, 2 eyes from different patients demonstrated unilateral vitreous hemorrhage (0.6%). No cases of endophthalmitis, choroidal hemorrhage, or hypotony occurred. Mean total anesthesia time was 203±87 minutes. There were no cases of anesthesia-related death, malignant hyperthermia, anaphylaxis, or cardiac event. There was 1 case of reintubation (0.6%) and 1 case of prolonged oxygen desaturation (0.6%). Mean follow-up after surgery was 103 weeks, and anatomic success and globe salvage rates were 89.8% and 98.0%, respectively. CONCLUSIONS: This study found ISBVS to be a feasible and safe treatment paradigm for pediatric patients with bilateral vitreoretinal pathologic features when repeated general anesthesia is undesirable or impractical.