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.

Aggressive Onset of a Progressive FEVR Phenotype in a Child With Novel Mutations in LRP5 and TSPAN12.

Purpose: To describe a patient with familial exudative vitreoretinopathy (FEVR) and the treatment course. Methods: A case was evaluated. Results: A 3-year-old boy presented with severe onset of FEVR, with a subhyaloid hemorrhage in 1 eye and tractional retinal detachment (TRD) in the fellow eye. Aggressive treatment with retinal photocoagulation and repeated injections of intravitreal bevacizumab resulted in stability of the retinal disease. Lens-sparing vitrectomy was performed for the TRD. The treatment effect was durable, and the patient retained useful vision in the better eye at 19 years of age. A subsequent genetic analysis showed 2 novel heterozygous missense mutations in LRP5 and TSPAN12. Conclusions: The presence of 2 novel mutations associated with severe FEVR identified in our patient is in agreement with in vitro studies showing that a more severe reduction in Norrin/ß-catenin signal activity occurs with the combination of 2 mutations.

Peripheral nonperfusion and tractional retinal detachment associated with congenital optic nerve anomalies.

PURPOSE: To report an association of congenital optic nerve anomalies with peripheral retina nonperfusion and to describe the clinical manifestations and treatment. DESIGN: Retrospective, observational case series. PARTICIPANTS: Fifteen patients with congenital optic nerve anomalies referred for pediatric retina consultation were studied. Sixteen eyes of 9 patients with optic nerve hypoplasia and 8 eyes of 6 patients with other congenital optic nerve anomalies, including optic nerve coloboma, morning glory disc, and peripapillary staphyloma, were included. METHODS: All patients underwent examinations under anesthesia. Wide-angle retina photographs and fluorescein angiograms were reviewed. The severity of nonperfusion was graded. The presence of fibrovascular proliferation (FP), vitreous hemorrhage (VH), and tractional retinal detachment (TRD) were documented. Anatomic outcome after treatment was recorded. MAIN OUTCOME MEASURES: Severity of nonperfusion, occurrence of secondary complications, and the anatomic outcome of patients who underwent laser treatment. RESULTS: In patients with optic nerve hypoplasia, 12 of 16 eyes (75%) had severe peripheral nonperfusion, 12 of 16 eyes (75%) had FP, 3 of 16 eyes (19%) had VH, and 10 of 16 eyes (63%) had TRD. Six of these eyes with severe nonperfusion received laser photocoagulation to the nonperfused retina; laser-treated retinas remained attached in all 6 eyes. In patients with the other optic nerve anomalies, 7 of 8 eyes (88%) had mild to moderate nonperfusion, 2 of 8 eyes (25%) had FP, 1 of 8 eyes (12%) had VH, and 2 of 8 eyes (25%) had TRD. Six of 9 patients (67%) with optic nerve hypoplasia and 1 of 6 patients (17%) with other anomalies had a coexisting congenital brain disease. CONCLUSIONS: Congenital optic nerve anomalies may be associated with peripheral retina nonperfusion and the secondary complications of FP, VH, and TRD. In this select group of patients, the nonperfusion associated with optic nerve hypoplasia seemed to be more severe and associated more frequently with secondary complications. Peripheral retina examination in eyes with optic nerve anomalies may identify nonperfusion or FP. Laser treatment of the avascular retina may have helped prevent complications from proliferative retinopathy in eyes clinically observed to have progressed or considered at risk for progression to proliferative retinopathy.

Peripheral retinal nonperfusion in fellow eyes in coats disease.

PURPOSE: To measure peripheral nonperfusion and describe bilateral vascular abnormalities in patients with Coats disease, emphasizing subtle findings in the contralateral eyes of patients diagnosed with unilateral disease. METHODS: Clinical records, including retina drawings and fluorescein angiography images obtained under anesthesia, were retrospectively reviewed from consecutive pediatric patients with Coats disease. The main outcome measure was disk diameters of peripheral nonperfusion and proportion of Coats disease patients with bilateral findings. RESULTS: Twenty-two of 32 patients with Coats disease had bilateral abnormal peripheral vasculature. In Group 1 (classic Coats disease: presumed unilateral, ocular only disease), 15 of 24 patients had peripheral nonperfusion >2 disk diameters in their contralateral eyes, 5 with telangiectasis and/or microaneurysms. Seven of 8 patients in Group 2 (Coats-like disease: systemic and/or clinically bilateral disease) had bilateral vascular anomalies. CONCLUSION: Bilateral vascular abnormalities are more common in Coats disease than previously reported. This observation supports a systemic and/or genetic association with Coats disease. Patients with Coats disease should have careful evaluation of the periphery of the less affected eye, preferably with fluorescein angiography, to identify vascular changes not visible clinically. Both eyes warrant surveillance over the lifetime of the patient for potential progression to exudative disease that would warrant treatment.

Exudative retinal detachment after laser in retinopathy of prematurity.

Treatment of retinopathy of prematurity with laser photocoagulation can be very effective in preventing future blindness, but its complications should be well understood by the ophthalmologists performing the treatment. We present the case of a 4-month-old girl in whom laser photocoagulation led to an exudative retinal detachment in both eyes. The fluid eventually resolved after treatment with topical and systemic steroids, but the effects of persistent fluid led to permanent photoreceptor loss. Optical coherence tomography can be useful in diagnosing the complication of exudative retinal detachment after laser photocoagulation and monitoring treatment response.

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.

Retinopathy of Prematurity in the 21st Century and the Complex Impact of Supplemental Oxygen.

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).

Late Vitreoretinal Complications of Regressed Retinopathy of Prematurity: Retinal Break, Vitreous Hemorrhage, and Retinal Detachment.

PURPOSE: To investigate late vitreoretinal complications and visual outcomes in patients with regressed retinopathy of prematurity (ROP) with or without prior treatment. DESIGN: International, multicenter, noncomparative retrospective case series. PARTICIPANTS: We analyzed 264 eyes of 238 patients from 13 centers worldwide who developed vitreoretinal complications (retinal detachment [RD], vitreous hemorrhage [VH], or retinal break) ≥ 2 years after resolution of acute ROP. METHODS: Each participant was assigned to 1 of 3 groups (the RD, VH, and retinal break groups) according to their primary diagnosis. The average age at presentation, visual acuities, refractive error, axial length, gestational age, birth weight, acute ROP classification, prior treatments for acute ROP, postoperative visual acuity (VA), and concomitant eye conditions in the 3 groups were documented and compared. MAIN OUTCOME MEASURES: Clinical features and visual outcomes of late vitreoretinal complications in patients with regressed ROP. RESULTS: A total of 264 eyes of 238 patients were included. The prior acute ROP status was comparable among the 3 groups, except that the VH group had a higher proportion of patients with type 1 ROP (P = 0.03) and prior treatment (P < 0.001) than the other groups. The average age at presentation was earlier in the RD (20.3 ± 15.5 years) and VH (21.4 ± 18.9 years) groups than in the retinal break group (31.9 ± 18.2 years; P < 0.001). The retinal break group had the best presenting best-corrected VA, followed by the RD and VH groups (P < 0.001). Surgical intervention improved VA in both the RD and VH groups (both P < 0.05). The overall trend of VA was the most favorable in the retinal break group, followed by that in the VH and RD groups. Cicatricial changes in the fellow retina were observed in > 90% of patients with unilateral involvement. CONCLUSIONS: Infants with acute ROP remain at a high risk of vision-threatening complications throughout childhood and adulthood. Continual follow-up of patients with ROP is important. When severe complications, such as RD or VH, are detected, timely surgical intervention is necessary to ensure favorable visual outcomes in these patients.

En face spectral-domain optical coherence tomography outer retinal analysis and relation to visual acuity.

PURPOSE: To describe a method of en face visualization and quantification of the photoreceptor inner segment/outer segment junction area, using spectral-domain optical coherence tomography, and association with visual acuity. METHODS: Case series of 74 eyes in 53 patients. Central 1-mm and 400-µm en face areas were analyzed with a computer algorithm. RESULTS: The presence or absence of inner segment/outer segment junction was visible on both spectral-domain optical coherence tomography en face and retinal cross sections. Thirty eyes (40.6%) had no retinal pathology and an average logMAR visual acuity of 0.116. Twenty-five eyes (33.8%) had intraretinal edema, with visual acuity of 0.494. Nineteen eyes had nonneovascular age-related macular degeneration (dry age-related macular degeneration, 25.6%), with visual acuity of 0.392. In all eyes, central 1-mm and 400-µm en face areas were 58.3 ± 25.0% and 56.4 ± 26.0%, which showed significant correlation with visual acuity (Pearson correlation, r = -0.66 and -0.56, both P < 0.001). This correlation was greater than correlation of visual acuity with central subfield thickness (r = 0.39, P < 0.001), macular volume (r = 0.36, P = 0.002), and average macular thickness (r = 0.37, P = 0.001). However, no variables were significantly correlated with dry age-related macular degeneration eyes. CONCLUSION: Central en face inner segment/outer segment junction areas are significantly correlated with visual acuity in most eyes. This may correlate better with visual acuity than other spectral-domain optical coherence tomography values, as a reflection of photoreceptor integrity. Dry age-related macular degeneration may disrupt the plane used to formulate the en face display. Advancements in spectral-domain optical coherence tomography may provide routine en face visualization analysis.

Laser Prophylaxis in Patients with Stickler Syndrome.

PURPOSE: To evaluate the association among laser prophylaxis treatment, retinal detachment (RD), and visual acuity (VA) in patients with Stickler syndrome (SS). DESIGN: Retrospective comparative case series. PARTICIPANTS: Patients with SS. METHODS: Patients received extended vitreous base laser (EVBL), nonprotocol laser (NPL), or no laser prophylaxis treatment of any kind. MAIN OUTCOME MEASURES: The 2 main outcome measures that were examined in these patients were rates of RD and VA. RESULTS: In this study, 230 eyes of 115 patients were included. Fifty-nine patients were women (51%). The median age at the time of laser prophylaxis treatment was 9.5 years (interquartile range [IQR], 6-13 years), and the median age of patients with RD was 11 years (IQR, 7-18 years). Of the 230 eyes, 92 did not undergo any laser treatment, 9 received NPL treatment, and 129 received EVBL treatment. Of the 129 eyes that underwent EVBL treatment, 4 (3%) had RD, compared with 74 eyes (73%) that had RD and did not receive laser or NPL treatment (P < 0.001). Eyes that received EVBL treatment had approximately 8 lines better vision, on average, compared with those that did not receive laser or NPL treatment (-0.86 logarithm of the minimum angle of resolution; 95% confidence interval,-1.1 to -0.64; P < 0.001). CONCLUSIONS: Treatment with EVBL seems to reduce the rate of subsequent RD and is associated with better VA in patients with SS.

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.].

A loss of function mutation in the COL9A2 gene causes autosomal recessive Stickler syndrome.

Stickler syndrome is characterized by ocular, auditory, skeletal, and orofacial abnormalities. We describe a family with autosomal recessive Stickler syndrome. The main clinical findings consisted of high myopia, vitreoretinal degeneration, retinal detachment, hearing loss, and short stature. Affected family members were found to have a homozygous loss-of-function mutation in COL9A2, c.843_c.846 + 4del8. A family with autosomal recessive Stickler syndrome was previously described and found to have a homozygous loss-of-function mutation in COL9A1. COL9A1, COL9A2, and COL9A3 code for collagen IX. All three collagen IX α chains, α1, α2, and α3, are needed for formation of functional collagen IX molecule. In dogs, two causative loci have been identified in autosomal recessive oculoskeletal dysplasia. This dysplasia resembles Stickler syndrome. Recently, homozygous loss-of-function mutations in COL9A2 and COL9A3 were found to co-segregate with the loci. Together the data from the present study and the previous studies suggest that loss-of-function mutations in any of the collagen IX genes can cause autosomal recessive Stickler syndrome.

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.