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Purpose: To characterize and compare residual retinal changes of spontaneously regressing retinopathy of prematurity (ROP) at termination of acute-phase retinal screening examinations. Methods: A retrospective chart review of 521 infants screened for ROP from 2016 to 2019 was performed. Data were collected on patients with at least stage 1 ROP, but who were not treated. The primary outcome measure was presence of residual fibrotic retinal tissue at termination of acute ROP screening examinations. Secondary outcomes included: type of residual fibrotic retinal tissue, persistent avascular retina, vitreous hemorrhage, and retinal detachment. A novel grading system was used to categorize findings. Comparisons between groups were made using Fisher exact tests. Results: A total of 255 eyes met inclusion criteria. A total of 206 of 255 eyes (81%) showed persistent avascular retina, whereas 57 of 255 eyes (22%) showed residual fibrotic retinal tissue at termination of acute screening. All eyes with previous stage 3 ROP showed residual fibrotic retinal tissue (8 of 8 eyes, 100%). Eyes whose highest stage of ROP was stage 2 showed more residual fibrotic retinal tissue at termination of acute screening (40 of 73 eyes, 55%) compared to eyes whose highest stage was stage 1 (9 of 174, 5%) ( P < .0001). Stage 3 eyes were excluded from this comparison due to the small sample size. One eye had vitreous hemorrhage at post-menstrual age 44 1/7 weeks. No eyes had retinal detachment. Conclusions: Despite meeting at least one of the criteria for termination of acute ROP screening, residual fibrotic retinal tissue and persistent avascular retina are common after spontaneous regression of ROP. Further investigation into the long-term clinical significance of these residual changes is warranted. [ J Pediatr Ophthalmol. 20XX;X(X):XX–XX.]

Purpose: We aim to compare the incidence of corneal epithelial defects after laser for retinopathy of prematurity (ROP) with and without the use of postoperative erythromycin ointment. Methods: In this retrospective observational cohort study, a total of 100 infants (200 eyes) consecutively treated with laser for ROP between 2012 and 2018. The primary outcome was presence or absence of corneal epithelial defect using fluorescein on bedside examination within the first week following laser for ROP. Additional data assessed included: the use of postoperative prophylactic erythromycin ointment for 1 week, postoperative day on which examination using fluorescein occurred, presence of corneal opacity, gender, birth weight, and gestation age. The presence or absence of postoperative corneal epithelial defects was compared between eyes receiving postoperative erythromycin ointment or not using a Fisher's exact test. Results: Postoperative corneal epithelial defects were more common in eyes which did not receive postoperative erythromycin (7 of 40 eyes; 17.5%), compared to eyes which did receive erythromycin (1 of 160 eyes; 0.6%; P < 0.0001). Postoperative bedside examinations with fluorescein were performed within 2 days of surgery on 136 of 200 of eyes (68%). Corneal opacities were noted in 3 of 200 eyes (1.5%). Conclusion: We observed less corneal epithelial defects in eyes which received postoperative erythromycin ointment for 1 week after laser for ROP than in those which did not. While multiple variables may influence the presence or absence of postoperative corneal epithelial defects following laser for ROP, consideration for postoperative lubricating ointment following laser for ROP seems reasonable.

To compare characteristics of laser treatment for high-risk type 1 retinopathy of prematurity (ROP) in eyes treated with primary laser versus laser after an initial treatment with intravitreal anti-vascular endothelial growth factor (anti-VEGF).The medical records of consecutive patients at a single academic institution treated for type 1 ROP before 36 weeks' postmenstrual age with primary laser versus laser after initial treatment with anti-VEGF were reviewed retrospectively. Outcome measures were laser spot number, mean laser power, total laser energy (Joules), and retinal vascularization to the nasal ora at time of laser treatment.Compared with the 46 eyes treated with primary laser, the 46 eyes treated with laser after anti-VEGF required fewer spots (mean, 775 vs 1418 [P0.01]), less power (182 vs 223 mW [P0.01]), and less total energy (27 vs 61 Joules [P0.01]), and showed greater vascularization to the nasal ora at the time of laser treatment (47.8% vs 6.5% [P0.01]).In our study cohort, laser after initial anti-VEGF treatment may have allowed for greater retinal vascularization and been less destructive than primary laser for high-risk type 1 ROP.

To compare rates of short-term retinal detachment (RD) of infants treated for type 1 retinopathy of prematurity (ROP) with intravitreal anti-vascular endothelial growth factor (VEGF) therapy with infants treated with laser therapy. The choice between these 2 treatments remains controversial. Comparative data are limited and describe re-treatment rates rather than retinal structural outcomes predictive of long-term vision. Anti-vascular endothelial growth factor acts faster than laser therapy, which may be beneficial for more aggressive ROP.Nonrandomized, comparative cohort study.The study included 1167 eyes of 640 infants treated for type 1 ROP. Among these, 164 eyes received anti-VEGF therapy and 1003 eyes received laser therapy.Pretreatment and posttreatment examinations and treatments were completed by ophthalmologists with expertise in ROP. The study was a secondary analysis of data from the retrospective Postnatal Growth and Retinopathy of Prematurity Study (G-ROP) 1 study (2006-2012) and the prospective G-ROP 2 study (2015-2017).Rate of RD (ROP stages 4A, 4B, or 5) within 8 weeks of initial treatment, an end point predictive of poor long-term vision. The results were stratified by postmenstrual age (PMA) at treatment as occurring before versus at or after 36 weeks and 0 days, because earlier disease may be considered more aggressive.Among 458 eyes treated before PMA 36 weeks and 0 days, the short-term RD rate was higher after laser therapy (29/368 eyes [7.9%]) than after anti-VEGF therapy (0/90 eyes [0%]; P0.001). Of 709 eyes treated at or after PMA 36 weeks and 0 days, short-term RD risk did not differ between groups (laser [20/635 eyes], 3.1%; anti-VEGF [1/74 eyes], 1.4%; P = 0.27).Anti-vascular endothelial growth factor therapy results in better short-term structural outcomes than laser therapy when type 1 ROP is treated before 36 weeks' PMA. After this age, both treatments have very low rates of short-term RD. The faster action of anti-VEGF agents likely is responsible for these findings.

Purpose Spasmus nutans is an acquired form of nystagmus that is classically associated with torticollis and titubations of the head, often presenting in the first year of life and spontaneously resolving within the next 2 years. The purpose of our study was to record and characterize the long-term prognosis of children diagnosed with spasmus nutans. Methods All patients under the age of 21 years with a diagnosis of spasmus nutans were included. Parameters of age, nystagmus symmetry and quality, presence of titubations, torticollis, and strabismus, and findings on CT or MRI were recorded. Results Our 22 patients had an average age of onset of 9.8 and a follow-up of 62.6 months. Nystagmus was unilateral in 5 children, asymmetric in 5, symmetric in 10, and inapparent in 2. Titubations were identified in 10 and torticollis in 7. Nystagmus persisted in 16 of 20 children, titubations in 3, and torticollis in 6. Neuroimaging, performed on 17 of the 22 children, was negative for any space-occupying lesions. Conclusion In conclusion, we are reluctant to assure parents of children who have even the most typical spasmus nutans that their child will be normal after a predictable interval. Instead, we advise them that many children do well, assuming normal imaging, but that nystagmus, torticollis, and even titubations may persist. Follow-up, especially for strabismus and amblyopia, should be continued throughout childhood.

Purpose To compare respiratory outcomes after treatment of retinopathy of prematurity (ROP) between infants treated with laser therapy under general anesthesia and infants treated with intravitreal bevacizumab under bedside sedation. Design Retrospective cohort study. Participants One hundred thirty-eight consecutive infants treated for ROP from September 2010 through September 2018 at 1 institution. Methods Retrospective medical, procedural, and ophthalmologic data were collected, including preprocedure (baseline) and postprocedure (24 hours, 48 hours, 7 days, and 28 days) respiratory status, birth weight, gestational age, gender, ROP treatment method, postmenstrual age at treatment, and coincident nonocular procedures during anesthesia. Respiratory outcomes at 48 hours were compared between infants treated with laser therapy under general anesthesia and infants treated with intravitreal bevacizumab under local sedation using multivariate logistic regression analysis to control for potentially confounding factors. Main Outcome Measures Proportion of infants who had returned to their respiratory baseline by 48 hours after ROP treatment. Results Return to respiratory baseline was significantly less common among 119 infants initially treated with laser therapy compared with 19 infants initially treated with bevacizumab at 24 hours (40% vs. 74%; P = 0.0115), 48 hours (53% vs. 79%; P = 0.0453), and 7 days (79% vs. 100%; P = 0.0242). In a multivariate logistic regression analysis, infants treated with laser therapy were less likely to return to respiratory baseline at 48 hours (odds ratio, 0.14; 95% confidence interval, 0.04–0.54). At 28 days, no difference was found between groups (laser, 97%; bevacizumab, 100%; P > 0.99). Conclusions Infants treated with intravitreal bevacizumab using bedside sedation returned to their preprocedure respiratory baseline faster than infants treated with laser under general anesthesia, with the differences persisting at least to 7 days or more after the procedure.

Purpose To perform a stratified comparison of the short-term risk of retinal detachment after treatment of type 1 retinopathy of prematurity treated with panretinal photocoagulation laser versus intravitreal bevacizumab. Methods The medical records of consecutive infants treated for type 1 ROP between 2010 and 2018 were retrospectively reviewed. An a priori decision was made to divide infants into two groups, those treated before postmenstrual age (PMA) of 36 0/7 weeks and those treated at or after PMA of 36 0/7 weeks. The primary outcome was presence of any retinal detachment (stage 4A, 4B, or 5) during the 8 weeks following treatment. Results A total of 222 eyes of 115 infants were included. In eyes treated before 36 0/7 weeks' PMA, retinal detachment occurred in 0 of 34 eyes treated initially with bevacizumab compared with 9 of 56 (16%) treated with laser (P = 0.0112); in eyes treated at or after 36 0/7 weeks, in 0 of 2 eyes treated with bevacizumab and 1 of 130 eyes (0.8%) treated with laser. Conclusions The short-term risk of retinal detachment among infants requiring treatment for type 1 ROP prior to 36 0/7 weeks' PMA was lower in eyes treated with intravitreal bevacizumab than in eyes treated with laser, presumably due to the faster effect of bevacizumab in eyes that have more aggressive ROP.

We report 2 cases of unilateral retinal arteriovenous malformation (AVM) with previously unreported anomalies of the inner retinal layers detected on spectral domain optical coherence tomography (SD-OCT): a 5-year-old girl with a large unilateral retinal AVM, ipsilateral visual acuity of 20/200, and ipsilateral intracranial AVM; and a 10-year-old boy with a large unilateral retinal AVM, ipsilateral visual acuity of 20/20, ipsilateral temporal visual field defects, and no intracranial AVM. Both macular SD-OCT findings showed multiple large inner retinal vessels that created a prominent shadowing artifact, retinal thickening, and speckling and heterogeneity of inner retinal layers.

Purpose: Many children present with excessive blinking. Categorization, associated conditions, and prognosis are controversial. Methods: All children with excessive blinking were reviewed, excluding those with known uveitis, glaucoma, or obvious eyelid abnormalities. Parents were telephoned for follow-up. Results: No ocular pathology was identified in 31 of 34 children with excessive blinking (91%). Parents were able to report a specific cause of blinking in 7 (21%). In 24 of 34 (71%), parents reported complete resolution of excessive blinking. No new ophthalmologic diagnoses were uncovered on follow-up. Conclusions: Episodes of excessive blinking rarely indicate neurologic disorders and frequently resolve spontaneously. [ J Pediatr Ophthalmol Strabismus. 2016;53(1):22–24.]

Purpose: Although much literature has focused on various techniques to repair epiblepharon, no study has addressed how frequently surgical intervention is required. Methods: The authors tabulated data from all patients with epiblepharon seen over the past 15 years. Results: Eighty-nine patients were included, 61 (69%) with tearing, discharge, conjunctival injection, or eye rubbing. Trichiasis was present in 15 cases (17%), 6 (7%) with corneal staining. In all cases, the initial treatment was conservative: antibiotic ointment or tear substitutes in 73 cases with trichiasis, symptoms of irritation, or corneal changes and observation in the remaining 16 cases. Three children (3%) were referred for eyelid surgery because of persistent symptoms. No patient had corneal scarring or long-term complications. Conclusions: Although vision-threatening complications can result, a trial of topical antibiotic ointment and/or ocular lubricants was effective in nearly all patients. Most resolved with minimal symptoms. The few eventually requiring eyelid surgery suffered no long-term complications. The authors recommend a trial of conservative treatment before eyelid surgery is undertaken. [ J Pediatr Ophthalmol Strabismus . 2017;54(1):15–16.]

Purpose: To report a case of a child with Pfeiffer syndrome, unique ocular anterior segment findings and a mutation in FGFR2 (Trp290Cys).Methods: Case Report.Results: We describe a patient with Pfeiffer syndrome with a unique constellation of ocular anterior segment anomalies including microcornea, limbal scleralization, corectopia and glaucoma. Genomic DNA extraction was heterozygous for a G to T mutation at nucleotide 870 of the fibroblast growth factor receptor 2 gene (FGFR2) which changes tryptophan (TGG) to cysteine (TGT) at amino acid position 290 (Trp290Cys).Conclusion: This case supports the association between Pfeiffer syndrome and severe ocular anterior segment anomalies, including glaucoma, and underscores the possible role that FGFR2 has in development of the anterior segment of the eye.

Infants with osteopetrosis may present early for ophthalmologic examination because of threatened visual impairment, primarily related to optic nerve compression. The authors describe two children with osteopetrosis who had narrow optic canals on orbital computed tomography. These canals were statistically narrower than those of newly developed, age-matched controls. Optic canal diameters, in addition to visual evoked potential data, may be helpful in determining prognosis.

To determine the effectiveness of a series of policy changes designed to increase the attendance rate for outpatient retinopathy of prematurity (ROP) screening examinations.We retrospectively reviewed the records of consecutive neonatal intensive care unit patients before and after the implementation of policy changes. Policy changes included parent education forms, streamlined scheduling, and creation of a log for all patients seen. The primary outcome measure was attendance rates for the first outpatient appointment after discharge. The Fisher exact test was used to compare rates between the two groups.Before the policy was implemented, 22 of 52 (42%) neonates and their caregivers attended their first outpatient ROP screening examination on the recommended date. This rate improved significantly after policy implementation, when 46 of 57 (81%) neonates and their caregivers were seen on the recommended date (P0.01). The number of patients who ultimately met the criteria for conclusion of acute retinal screening examinations also significantly improved, from 47 of 52 (90%) of neonates in the pre-implementation group to 57 of 57 (100%) in the post-implementation group (P = 0.02).The attendance rates for initial outpatient ROP examinations and the number of patients who ultimately met criteria for conclusion of acute retinal screening examinations significantly improved after the implementation of new policies.

The authors present the youngest reported child with proliferative sickle retinopathy. A 3-year, 6-month-old boy presented with leukocoria in the left eye, left esotropia, and dense free-floating white vitreous cells obscuring the fundus, suspicious for endophytic retinoblastoma. Ultrasonography depicted dense debris in the vitreous with no distinct calcific echo or retinal mass. Transcorneal, transzonular fine-needle aspiration biopsy of the vitreous confirmed the presence of dehemoglobinized vitreous red blood cells without tumor. The opposite eye showed peripheral retinal pigment epithelial proliferation and fibrosis with angiographic peripheral ischemia. Hemoglobin electrophoresis disclosed sickle trait (AS). The findings were classified as proliferative sickle cell retinopathy with vitreous hemorrhage in the left eye and non-proliferative sickle cell retinopathy in the right eye. Leukocoria generally raises warnings for retinoblastoma, but can also reflect chronic vitreous hemorrhage.

Purpose To compare the performance of patients with strabismus to that of age-matched controls in a validated surgical training module. Methods A prospective experimental study was conducted of 14 adult patients with strabismus since childhood and absent stereopsis and of 14 age-matched controls with normal stereopsis. Each participant received instruction in the task of peg transfer on a validated surgical training device and then completed 10 consecutive timed trials. The means of the best 5 scores were compared using the 2-sample Wilcoxon rank-sum test. Results The average age of cases was 34.8 years (range, 15-51 years) compared with 37.8 years (range, 14-56 years) for controls. The scores for the strabismic patients ranged from 50.8 to 151.4 seconds, with a mean of 82.5 ± 26.7 seconds. Controls ranged from 43.2 to 129 seconds, with a mean of 64.7 ± 23.9 seconds. The Wilcoxon rank-sum test showed significantly better performance among controls (p = 0.022). Conclusions Patients with strabismus performed more poorly than did age-matched controls in this model of hand-eye coordination. However, there was significant overlap between groups and several patients with strabismus performed better than the mean of the control group. Further investigation is required to elucidate the impact of strabismus on surgical performance.