Approaches to Retinal Detachment Prophylaxis among Patients with Stickler Syndrome

Stickler syndrome is the most common cause of pediatric rhegmatogenous retinal detachments. Given the dramatic long term visual impact and difficult surgical management of these detachments, there is increasing interest in determining whether prophylactic treatment can be used to prevent retinal detachments in this population. However, severity of ocular findings in Stickler syndrome can vary by subtype. Three commonly used modalities to provide prophylactic treatment against retinal detachments in patients with Stickler syndrome include scleral buckle, laser retinopexy, and cryotherapy. While laser retinopexy is the most common approach to prophylactic treatment, treatment settings can vary by specialist. In addition, the decision to treat and manage Stickler syndrome is nuanced and requires careful consideration of the individual patient. After reviewing the literature on prophylactic treatment approaches, this chapter will also over guidelines in management of this complex patient population

Vitreoretinopathy-Associated Pediatric Retinal Detachment Treatment Outcomes

Purpose: To determine the treatment patterns and outcomes of pediatric retinal detachments (RDs) associated with hereditary vitreoretinopathies. Design: Retrospective cohort analysis using IRIS® Registry (Intelligent Research in Sight) database. Participants: Patients < 18 years old with a rhegmatogenous RD and a systemic disorder associated with vitreoretinal degeneration (e.g., Stickler syndrome) or other malformation of the vitreous from 2013-2019. Methods: Cases were identified using International Classification of Diseases, Ninth and Tenth Revisions (ICD-9, ICD-10) diagnostic codes from the IRIS® Registry cohort. Other hereditary vitreoretinopathies that are not encoded by specific ICD code(s) were captured by text search. Nonspecific vitreous abnormality ICD codes were also included. Exclusion criteria included traumatic retinal detachments using ICD codes for ocular trauma and serous or exudative retinal detachment. Surgical procedures were identified using Current Procedural Terminology (CPT) codes for repair of retinal detachment. Baseline demographic information collected included age, gender, race/ethnicity, geographic region of the provider location, and health insurance status. Main Outcome Measures: Main outcomes measured in this study were average time to first surgery, number of eyes presenting with bilateral detachments, and choice of initial surgical procedure. Results: A total of 2115 eyes of 1722 patients were identified (mean age, 10.4 years; 58% male). The median time to first surgery was 7 days (interquartile range, 40 days). One thousand four hundred seven eyes of 1134 patients had ≥ 1 year of follow-up, with 506 eyes (36%) developing a fellow eye RD. Thirty-three percent of patients presenting with bilateral detachments, and 349 eyes had initial RD surgery within 1 year of the index date documented by CPT code. Fellow eye detachment occurred a mean of 32 days after initial presentation. The mean number of surgeries per eye within 1 year was 1.68. Best-corrected visual acuity did not improve from a baseline 20/54 to 20/62. The initial procedure was most commonly complex RD repair (n = 176), followed by scleral buckle (n = 102), pars plana vitrectomy (n = 89), laser (n = 59), cryotherapy (n = 5), and pneumatic retinopexy (n = 5). There were 51 new diagnoses of glaucoma and 37 new diagnoses of aphakia within 1 year after the surgical procedure. Conclusions: IRIS Registry data provide insight into rare pediatric vitreoretinopathy-associated RDs, which have a high rate of reoperation and fellow eye involvement. Financial Disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article

Longitudinal Assessment of Retinopathy of Prematurity (LONGROP) Study: Impacts of Viewing Time and Ability to Compare on Detection of Change

Purpose: This study compared two imaging grading techiques to assess the utility of longitudinal image-based analysis in retinopathy of prematurity (ROP) screening: 1) time-limited without image comparison (a proxy for bedside indirect ophthalmoscopy, termed sBIO) and time-unlimited with image comparison (for telemedicine grading, termed TELE) screening. We tested two hypotheses: 1) H1: TELE was superior to sBIO for the detection of change (Tempo)–same, better, or worse–and, 2) H2: granular data of change (e.g. at the image and feature level) is integrated by graders to achieve the Tempo assessment. Design: Prospective reliability analysis. Methods. Gold standard reference (GS) was a published curated ROP image database consisting of both Tempo and granular level changes (image and components) from 40 patients in 2 sets. Graders were divided into 2 cohorts. There were two screening techniques–1) sBIO with time limited review of 10 minutes/patient, access to prior notes and drawings and 2) TELE with unlimited review time, access to prior weeks’ images, notes and schematics. Graders switched techniques and sets after 6 weeks. H1 outcome was comparison of graders’ weekly Tempo scores to GS-Gestalt and for H2 was Tempo score compared to GS-View and GS-Component. Results: H1 demonstrated no difference–accuracy of sBIO and TELE compared to GS was 51.7% and 51.9% respectively (p=0.95). Highest agreement occurred when all exams exhibited no change (91.5% sBIO vs. 93.5% TELE, p=0.46) and worst agreement was when exams always demonstrated worsening (46.5% sBIO vs. 47.1% TELE, p=0.93). Both sets of graders did worse in weeks 7-12, irrespective of technique. H2 demonstrated that Tempo assessment did not correlate with granular data changes in the GS for View level and Component level assessments–overall agreement dropped to 31.4% for Tempo vs GS-VIEW (31.2% for sBIO, 31.5% for TELE) and 4.6% for Tempo vs GS-COMPONENT (4.9% for sBIO, 4.3% for TELE). Conclusions: Detection of ROP Tempo was independent of screening technique by expert pediatric retina graders. Both groups did significantly better in the first half of the study, indicative of a fatigue factor. This is the first study in ROP history to demonstrate that graders integrate image and retinal features in various ways that can be in contradiction of their assessment of overall disease progression

The impact of the COVID-19 lockdown on retinopathy of prematurity screening and management in the United States: a multicenter study

To study the effect of the pandemic-related lockdown (physical distance measures and movement restrictions) on the characteristics and management of retinopathy of prematurity (ROP). In this controlled, multicenter cohort study, the medical records of patients born prematurely and screened for ROP in the neonatal intensive care unit during four time periods were reviewed retrospectively: (1) November 1, 2018, to March 15, 2019; (2) March 16, 2019, to August 2, 2019 (lockdown control period); (3) November 1, 2019, to March 15, 2020; and (4) March 16, 2020-August 2, 2020. A total of 1,645 patients met inclusion criteria. Among the 1,633 patients with complete data, mean gestational age (GA) at birth was 28.2, 28.4, 28.0, and 28.3 weeks across time periods 1 to 4, respectively (P = 0.16). The mean birth weight of all patients was 1079.1 ± 378.60 g, with no significant variation across time periods (P = 0.08). There were fewer patients screened during the lockdown period (n = 411) compared with the period immediately before (n = 491) and the same period in the prior year (n = 533). Significantly more patients were screened using indirect ophthalmoscopy, compared to digital imaging (telemedicine), during the lockdown (P < 0.01). There were 11.7%, 7.7%, 9.0%, and 8.8% of patients requiring treatment in each time period, respectively (P = 0.42), with a median postmenstrual age at initial treatment of 37.2, 36.45, 37.1, and 36.3 weeks, respectively (P = 0.32). We recorded a decrease in the number of infants meeting criteria for ROP screening during the lockdown. The GA at birth and birth weight did not differ. Significantly more infants were screened with indirect ophthalmoscopy, compared to digital imaging, during the lockdown

Referable Macular Hemorrhage-A Clinically Meaningful Screening Target in Newborn Infants. Position Statement of the Association of Pediatric Retina Surgeons

Universal newborn eye screening facilitates early diagnosis of ocular abnormalities and mitigates vision loss. "Referral warranted" eye disease is present at birth in about 5.5% of term infants, with "macular hemorrhage impinging on the fovea" representing about 50% of referral warranted disease. The Association of Pediatric Retina Surgeons held a symposium on February 9, 2021 that culminated in a position statement on "referable macular hemorrhage" (RMH) in newborn infants. RMH is meaningful in that in can cause amblyopia through deprivation, can be readily captured with wide-angle photography in a safe and efficient manner, and may lead to early intervention with mitigation of vision loss

Late-Onset Retinal Findings and Complications in Untreated Retinopathy of Prematurity

PURPOSE: 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. DESIGN: Retrospective, nonconsecutive, noncomparative, multicenter case series. PARTICIPANTS: Three hundred sixty-three eyes of 186 patients. METHODS: 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. MAIN OUTCOME MEASURES: Rate of RDs and factors conferring a higher risk of RDs. RESULTS: 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 (P \u3c 0.05) and in eyes with furthest vascularization to posterior zone 2 eyes compared with zone 3 eyes (P = 0.009). CONCLUSIONS: 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