Eight-Year Outcomes of Bilateral Lateral Rectus Recessions versus Unilateral Recession-Resection in Childhood Basic-Type Intermittent Exotropia.

PURPOSE: To report 8-year outcomes from a randomized controlled trial (RCT) comparing bilateral lateral rectus muscle recession (BLRc) with unilateral recession-resection (R&R) for childhood intermittent exotropia (IXT). DESIGN: Eight-year follow-up of RCT cohort. PARTICIPANTS: Of 197 randomized participants, 123 agreed to continue follow-up after the 3-year outcome visit (baseline age, 3-< 11 years; basic-type IXT, 15-40 prism diopters [Δ] by prism and alternate cover test [PACT]; baseline stereoacuity, ≤ 400 arcsec; no prior surgery). METHODS: After the RCT primary outcome at 3 years, annual follow-up from 4 through 8 years with treatment at investigator discretion. MAIN OUTCOME MEASURES: Suboptimal surgical outcome by 8 years after randomization, defined as any of the following at any visit: exotropia of 10 Δ or more by simultaneous prism cover test (SPCT) at distance or near, constant esotropia (ET) of 6 Δ or more by SPCT at distance or near, loss of near stereoacuity by 0.6 log arcsec or more from baseline, or reoperation. Secondary outcomes included (1) reoperation by 8 years and (2) complete or near-complete resolution at 8 years, defined as exodeviation of less than 10 Δ by SPCT and PACT at distance and near and 10 Δ or more reduction from baseline by PACT at distance and near, ET of less than 6 Δ at distance and near, no decrease in stereoacuity by 0.6 log arcsec or more from baseline, and no reoperation or nonsurgical treatment for IXT. RESULTS: The Kaplan-Meier cumulative probability of suboptimal surgical outcome through 8 years was 68% (55 events among 101 at risk) for BLRc and 53% (42 events among 96 at risk) for R&R (difference, 15%; 95% confidence interval [CI], -2% to 32%; P = 0.08). Complete or near-complete resolution at 8 years occurred in 15% (7/46) for BLRc and 37% (16/43) for R&R (difference, -22%; 95% CI, -44% to -0.1%; P = 0.049). The cumulative probability of reoperation was 30% for BLRc and 11% for R&R (difference, 19%; 95% CI, 2%-36%; P = 0.049). CONCLUSIONS: Despite no significant difference for the primary outcome, the 95% CI did not exclude a moderate benefit of R&R, which together with secondary outcomes suggests that unilateral R&R followed by usual care may yield better long-term outcomes than BLRc followed by usual care for basic-type childhood IXT using these surgical doses. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Variable Phenotype of Congenital Corneal Opacities in Biallelic CYP1B1 Pathogenic Variants.

PURPOSE: The aim of this study is to describe the variable phenotype of congenital corneal opacities occurring in patients with biallelic CYP1B1 pathogenic variants. METHODS: A retrospective chart review was conducted to identify patients with congenital corneal opacities and CYP1B1 pathogenic variants seen at UPMC Children's Hospital of Pittsburgh. Ophthalmic examination, high-frequency ultrasound, anterior segment optical coherence tomography, histopathologic images, and details of genetic testing were reviewed. RESULTS: Three children were identified. All presented with raised intraocular pressure. Two patients showed bilateral limbus-to-limbus avascular corneal opacification that did not resolve with intraocular pressure control; 1 showed unilateral avascular corneal opacity with a crescent of clear cornea, iridocorneal adhesions, iridolenticular adhesions, and classical features of congenital glaucoma in the fellow eye (enlarged corneal diameter, Haab striae, and clearing of the corneal clouding with appropriate intraocular pressure control). The first 2 patients were visually rehabilitated with penetrating keratoplasty. Histopathology revealed distinct features: a variably keratinized epithelium; a thick but discontinuous Bowman-like layer with areas of disruption and abnormal cellularity; Descemet membrane, when observed, showed reduced endothelial cells; and no pathological changes of Haab striae were identified. Two patients had compound heterozygous pathogenic variants in CYP1B1 causing premature stop codons, whereas 1 was homozygous for a pathogenic missense variant. CONCLUSIONS: Congenital corneal opacities seen in biallelic CYP1B1 pathogenic variants have a variable phenotype. One is that commonly termed as Peters anomaly type 1 (with iridocorneal adhesions, with or without iridolenticular adhesions) and the other is a limbus-to-limbus opacity, termed CYP1B1 cytopathy. Clinicians should be aware of this phenotypic variability.

Glaucoma Following Cataract Surgery: Pearls for Success Manuscript from the 2022 AOC/AACO/AAO Sunday Symposium.

Glaucoma Following Cataract Surgery (GFCS) remains a menace, so parents must be counseled prior to cataract removal in children. Age less than 7 months at the time of surgery increases this risk, and IOL placement has no effect. To lower IOP in GFCS, start with drops and before you escalate to surgery, consider phospholine iodide. Then, proceed cautiously with angle surgery and shunts, mixing in cycloablative procedures where appropriate in your hands. With patient-centered models for access, follow-up & adherence to treatment, GFCS can be controlled. Partnering with our certified orthoptist colleagues, we can achieve excellent results for the "whole patient" spanning cataracts, glaucoma, strabismus, and amblyopia.

Dupilumab-associated ectropion and punctal stenosis treated with tacrolimus ointment (0.03%) in a 15-year-old girl.

A 15-year-old Asian girl with severe atopic dermatitis was referred for dupilumab-associated blepharoconjunctivitis. Medical history was significant for severe atopic dermatitis. She was started on prednisolone acetate 1% ophthalmic suspension three times daily, and dupilumab injections were withheld after the initial visit. The patient was noted to have right lower eyelid ectropion, cicatricial occlusion, and severe punctal stenosis 6 weeks later. She was started on 0.03% tacrolimus ointment to the eyelid margin. Resolution of ectropion and restoration of punctal patency with residual stenosis were observed 4 weeks later. This is the first reported adolescent case of dupilumab-associated ectropion and punctal stenosis successfully treated with topical tacrolimus ointment.

Unilateral Horizontal Rectus Muscle Recessions for Pediatric Comitant Strabismus.

INTRODUCTION: Compared to bilateral horizontal rectus muscle recessions (BHR), a unilateral horizontal rectus muscle recession (UHR) confers shorter operating time and anesthesia exposure, limits complication risks to one eye, and requires less recovery from the patient. METHODS: Retrospective chart review of pediatric patients who underwent unilateral medial rectus recession (UMR) for esotropia (ET), or unilateral lateral rectus recession (ULR) for exotropia (XT). Primary successes were defined as maximum distance deviation at post-operative month 3: -12 to +5 for ET, -5 to +12 for XT. Rates of postoperative horizontal incomitance (HI) >5 PD and success for small medium, and large (in mm, respectively, ET: <5, 5-6 mm, >6; XT: <8, 8-10, >10) recessions were analyzed. RESULTS: Seventeen ETs and 40 XTs were analyzed. Overall primary success was 71.9% (p = .02). Significantly, 80% (95% CI: 67.60,92.40) XTs succeeded. ETs were equally likely to succeed (53.9%) or fail (47.1%) (p = .22). For patients without significant preoperative HI, average postoperative HI was 3.90 PD (95% CI: 0.20, 7.60) for ETs; 5.48 PD (95% CI: 3.65, 7.32) for XTs. CONCLUSIONS: UHR was 71.9% successful in treating pediatric comitant strabismus. ULR for XT, particularly small to medium deviations, was most likely to succeed. In contrast to prior reports, large UMR and ULR were less likely to succeed and post-operative incomitance was frequent but rarely clinically significant.

Follow-up Adherence and Barriers to Care for Pediatric Glaucomas at a Tertiary Care Center.

PURPOSE: To determine the percent adherence to follow-up for patients with pediatric glaucomas seen at a tertiary care center and to elucidate risk factors. DESIGN: Retrospective cohort study. METHODS: Patients with pediatric glaucomas seen at the University of Minnesota over 8.5 years were classified as adherent, nonadherent, or lost to tertiary follow-up if they followed up within 0-30 days, between 31 and 180 days, or later than 180 days of the recommended appointment time or never, respectively. RESULTS: Of 176 patients analyzed, 95 (54%) were adherent (51% male; mean [standard deviation (SD)] age: 56.1 [59.8] months), 5 (3%) were nonadherent (20% male; mean [SD] age: 25.0 [35.8] months), and 76 (43%) were lost to tertiary follow-up (55% male; mean [SD] age: 58.9 [53.1] months). Multiple logistic regression analysis of variables that were significant in isolation revealed that only race (white: odds ratio, 3.58; 95% confidence interval, 1.42-9.05; P = .007) and distance from the eye clinic (per 50 miles: odds ratio, 0.79; 95% confidence interval, 0.67-0.92; P = .003) significantly impacted adherence. CONCLUSIONS: This is the first study of adherence to follow-up recommendations for patients with pediatric glaucomas. Percent adherence to follow-up appointments was alarmingly low, and decreased adherence was observed with non-white race and increased distance to the eye clinic. Physicians should consider these risk factors when risk-stratifying patients with pediatric glaucomas for nonadherence to follow-up. Additional studies to improve adherence through interventions that reduce biases and barriers to follow-up are needed.

Your eye doctor will virtually see you now: synchronous patient-to-provider virtual visits in pediatric tele-ophthalmology.

Community transmission of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2 or COVID-19) in the United States on February 26, 2020, and the rapid spread that followed forced patients, providers, payors, and policy makers to adapt to an unprecedented, nearly instant, and enormous demand for virtual care. Although few US ophthalmology practices incorporated telemedicine prior to COVID-19, its use has now become the norm. Regarding the use of synchronous patient-to-provider virtual visits (SPPVV) in pediatric ophthalmology, we have pooled our collective experience at three academic practices across the country to describe initial workflows, technology solutions, use cases, and barriers to care.

Anatomic and visual outcomes of corneal transplantation during infancy.

PURPOSE: To determine the effect of age at penetrating keratoplasty (PKP) on graft survival and visual outcome in children with corneal opacities transplanted during infancy. METHODS: In this two-center retrospective consecutive cohort study, the medical records of infants who underwent unilateral or bilateral PKP during the first year of life between 2004 and 2011 were reviewed retrospectively. PKP was categorized as early (age 0-90 days) or late (age 91-365 days). Main outcome measures were graft survival and vision (classified as poor, fair, or good, considering both testing method and age norms). RESULTS: A total of 62 eyes of 52 infants were included: 19 eyes underwent early PKP; 43 eyes, late PKP. Of the 62 eyes, 61 had central congenital corneal opacities; 1 was acquired. Median follow-up was 38.1 months (range, 12.2-150.5 months). Kaplan-Meier graft survival estimates were 0.92 at 1 year (95% CI, 0.81-0.96) and 0.61 at 5 years (0.44-0.74). Graft survival (early PKP, 73.7%; late PKP, 65.1% [P = 0.57]) did not differ between groups. Of the 55 eyes with recorded visual acuities, no significant difference existed in proportion with ambulatory or better vision at latest follow-up between early and late PKP (42.1% vs 55.6%; P = 0.61). CONCLUSIONS: Visual outcomes were better for PKP performed during infancy compared to results of prior reports of late PKP; however, clearing of congenital opacities in the first 3 months of life did not improve visual outcomes compared to later PKP. One-half of grafts survived >5 years. Early PKP did not worsen graft survival, but PKP may be technically easier to perform later in infancy.

Ocular Injury Presenting to a Level-III Pediatric Trauma Center.

The purpose of this study is to describe pediatric ocular injuries presenting to a level-III pediatric trauma center and emergency department. METHODS: We performed a retrospective study and identified children from January 1, 2011, to January 1, 2016. Charts were reviewed for any subject, age from newborn to younger than 18 years, based on International Classification of Diseases, ninth and tenth revision, codes for any ocular injury. Data abstraction included age, sex, means of arrival, eye involved, mechanism of injury, type of ocular injury, imaging studies obtained, procedures performed, location of definitive repair (in the operating room or emergency department), and subspecialty services involved. RESULTS: In the 5-year period, we describe 356 injuries among 278 children. Males had a slightly higher rate of presentation than females (156 and 122, respectively). Forty-three children (15.46%) required repair in the operating room. Dog bites comprised of 7.19% children with outpatient follow-up, one patient (0.36%) eventually developed anophthalmia, and 30 children (10.79%) had long-term ophthalmological sequelae (ie, glaucoma and blindness). CONCLUSIONS: At our institution, a level-III trauma center, we evaluated and managed approximately 1 ocular injury case per week and children required surgical repair in the operating room at a higher rate than higher-level trauma centers. Injuries secondary to dog bites remain a clinically significant etiology.

Trabeculotomy Ab Interno With the Trab360 Device for Childhood Glaucomas.

PURPOSE: To report outcomes and complications of trabeculotomy ab interno using the Trab360 device (Trab360; Sight Sciences, Menlo Park, California, USA) in eyes with childhood glaucomas. DESIGN: Multicenter retrospective interventional case series. METHODS: Eyes with childhood glaucomas that underwent Trab360 with at least 3 months follow-up were evaluated. Postoperative intraocular pressure (IOP) less than or equal to 24 mm Hg with or without medications and no additional surgery defined success. RESULTS: Forty-six eyes of 41 patients were included. Median age at surgery was 12 months (range 1-325 months, mean 71 months); 54% prior to 20 months. A total of 48% were right eyes; 48% were male. Mean treatment was 290°. Median follow-up was 14.5 months (range 6-34 months, mean 16.2 months). Median preoperative IOP was 30 mm Hg (range 18-49 mm Hg, mean 30.9 mm Hg); median postoperative IOP was 18 mm Hg (range 5-40 mm Hg, mean 20.3 mm Hg]. Median number of preoperative glaucoma medications was 2.5 (range 0-5, mean 2.6); median number postoperatively was 1 (range 0-4, mean 1.6). Success was achieved in 67.4% (95% CI: 51.9%-80.0%) of eyes. Among 40 eyes for which Trab360 was the first glaucoma surgery, success rate was 70% (95% CI 53.3%-82.9%). Success was achieved in 81% (95% CI 57.4%-93.7%) of primary congenital glaucoma (PCG) eyes. Among 18 PCG eyes for which Trab360 was the first glaucoma surgery, success rate was 83.3% (95% CI 57.7%-95.6%). Two eyes (4.3%) suffered cyclodialysis. There were no other significant complications. CONCLUSIONS: Trab360 success resembles literature on other angle surgeries for childhood glaucomas. Good surgical technique and caution in high-risk angles is imperative to avoid cyclodialysis. Our study is limited by the imperfections inherent in any retrospective analysis. Single-incision ab interno trabeculotomy with the Trab360 device is effective and safe for treating childhood glaucomas, especially PCG.

Strabismus Patterns in Patients with Intrauterine Drug Exposure.

Purpose: This retrospective study evaluates the patterns of strabismus, the incidence of amblyopia, and prevalence of anatomical ocular pathology in patients with intrauterine drug exposure (IDE) presenting to a tertiary referral center for an ophthalmic evaluation.Methods: We reviewed charts of patients who presented to our pediatric ophthalmology clinic from 8/2017-10/2018 with reported IDE. The mean age at presentation was 29 months. We documented the type of exposure, birth history, social history, and past medical and surgical history. Visual acuity, motility, fundus exam, and cycloplegic refraction were reviewed.Results: Thirty-eight patients met the criteria. Motility exam showed that 23 patients were exotropic, 10 were esotropic, and 5 were orthophoric. Average angle of the exotropic deviation was 27ΔXT (range X 10 to XT 50). Amblyopia, nystagmus, and anatomical ocular anomalies also occurred frequently.Conclusion: Strabismus and other eye diseases are common in patients with IDE. Exodeviations were the most common finding and occurred in 60.5% of our cohort. Patients with IDE are at a higher risk for treatable conditions such as strabismus and amblyopia. Possible history of IDE should be considered in infants who present with congenital strabismus, particularly exotropia.

Childhood glaucoma in association with congenital disorder of glycosylation caused by mutations in fucosyltransferase 8.

A rare form of congenital disorder of glycosylation (CDG) was recently discovered in individuals with biallelic mutations in fucosyltransferase 8 (FUT8). The clinical characteristics of patients with FUT8-CDG include intrauterine growth retardation, feeding difficulties, hypotonia, microcephaly, seizures, short stature, developmental delay, and respiratory abnormalities. We report the first case of glaucoma in an infant with FUT8-CGD and hypothesize a pathogenesis for glaucoma.

Pigment dispersion syndrome and response to laser peripheral iridotomies in a child with Marfan syndrome.

Pigment dispersion syndrome (PDS) and pigmentary glaucoma have rarely been reported in Marfan syndrome and have never been reported in a child with Marfan syndrome. We report the clinical and ultrasound biomicroscopic findings of PDS in a 14-year-old girl with Marfan syndrome and its favorable response to bilateral laser peripheral iridotomy.

A Cluster of Cyclic Esotropia: White Matter Changes on MRI and Surgical Outcomes.

PURPOSE: To report a series of patients with cyclic esotropia, their surgical outcomes, and incidental findings. METHODS: The medical records of five patients with cyclic esotropia presenting over 17 months were reviewed. Age at onset, ocular and motility examinations, brain magnetic resonance imaging (MRI), acetylcholine receptor antibodies, thyroid hormone levels and antibodies, calendars documenting phases, surgical treatments, postoperative alignment, and fusion were documented. RESULTS: Three boys and two girls presented at 3 to 4 years old in 2015-2016. Typical periodicity followed a 48-hour cycle. Duration of cycling varied from 1 to 9 weeks. Mean maximum deviation was 37 prism diopters (PD) of esotropia (range: 35 to 40 PD of esotropia). All patients had normal laboratory studies. MRIs showed an abnormal white matter signal in the frontal lobes in 2 patients and were normal in the others. Bilateral medial rectus recessions for the maximum angle were successful in 4 patients at a minimum follow-up of 13 months; the other patient required reoperation for a residual esotropia. Fusion was present in 4 patients preoperatively and all postoperatively. Stereopsis was stable or improved in all postoperatively. CONCLUSIONS: This is the first report of frontal white matter changes occurring in the setting of cyclic esotropia. MRI with attention to frontal lobe white matter might be considered in the work-up of cyclic esotropia to determine if this is a common finding. Bilateral medial rectus recessions can restore fusion in these patients. [J Pediatr Ophthalmol Strabismus. 2019;56(3):178-182.].

Endothelial dysfunction in a child with Pearson marrow-pancreas syndrome managed with Descemet stripping automated endothelial keratoplasty using a suture pull-through technique.

A 4-year-old girl with a history of Pearson marrow-pancreas syndrome presenting with severe, progressive photophobia was found to have bilateral, diffuse corneal thickening and peripheral pigmentary retinopathy. She underwent Descemet stripping automated endothelial keratoplasty (DSAEK) surgery in both eyes using a modified suture pull-through technique. Postoperatively there was no evidence of cataract formation or graft detachment; her corneas thinned, and her photophobia improved dramatically.

Chronic inflammatory granulomata after use of GenTeal gel during strabismus surgery.

Subconjunctival granulomata developed in 2 patients after use of GenTeal gel to lubricate the cornea during strabismus repair. We report the clinical findings and histopathology of the subconjunctival nodules. These lesions are similar to those previously reported following use of GenTeal gel in retinal surgery and use of similar large polymers in nonocular surgeries. Copious irrigation should be considered when conjunctival wounds are exposed to GenTeal gel, and alternative corneal wetting agents should be considered for use during strabismus surgery.

Ectropion Improvement with Topical Tazarotene in Children with Lamellar Ichthyosis.

BACKGROUND/OBJECTIVES: Lamellar ichthyosis (LI) is a well-described phenotypic subtype of autosomal recessive congenital ichthyosis (ARCI). The condition typically presents at birth with collodion membrane and leads to thick, plate-like scaling of the skin throughout the body, alopecia, and prominent ocular manifestations. Ocular complications include bilateral cicatricial ectropion and lagophthalmos. These ocular complications can lead to chronic exposure keratitis and in some cases corneal ulceration and blindness. No cure for ichthyosis exists. Treatment of ocular complications in LI includes surgical correction, systemic retinoids, and a variety of topical therapies such as emollients, keratolytics, and retinoids. METHODS: Five children with LI cared for at our institution were identified and included. Patient age at the start of therapy ranged from 2 weeks to 9 years. Electronic medical records were reviewed and data from pediatric dermatologist and pediatric ophthalmologist visits were obtained. Data were collected before and after treatment of daily or twice-daily 0.05% to 0.1% tazarotene cream applied to the face and eyelids. RESULTS: All patients had improvement in the degree of ectropion, with complete resolution in two of the five patients. The two patients with lagophthalmos at the time of tazarotene initiation experienced complete resolution. No adverse effects were reported. CONCLUSIONS: Tazarotene cream appears to be effective in the management of ectropion and lagophthalmos in the setting of LI in children, even in the neonatal period.

Ocular HSV-1 latency, reactivation and recurrent disease.

Ocular infection with HSV-1 continues to be a serious clinical problem despite the availability of effective antivirals. Primary infection with HSV-1 can involve ocular and adenaxial sites and can manifest as blepharitis, conjunctivitis, or corneal epithelial keratitis. After initial ocular infection, HSV-1 can establish latent infection in the trigeminal ganglia for the lifetime of the host. During latency, the viral genome is retained in the neuron without producing viral proteins. However, abundant transcription occurs at the region encoding the latency-associated transcript, which may play significant roles in the maintenance of latency as well as neuronal reactivation. Many host and viral factors are involved in HSV-1 reactivation from latency. HSV-1 DNA is shed into tears and saliva of most adults, but in most cases this does not result in lesions. Recurrent disease occurs as HSV-1 is carried by anterograde transport to the original site of infection, or any other site innervated by the latently infected ganglia, and can reinfect the ocular tissues. Recurrent corneal disease can lead to corneal scarring, thinning, stromal opacity and neovascularization and, eventually, blindness. In spite of intensive antiviral and anti-inflammatory therapy, a significant percentage of patients do not respond to chemotherapy for herpetic necrotizing stromal keratitis. Therefore, the development of therapies that would reduce asymptomatic viral shedding and lower the risks of recurrent disease and transmission of the virus is key to decreasing the morbidity of ocular herpetic disease. This review will highlight basic HSV-1 virology, and will compare the animal models of latency, reactivation, and recurrent ocular disease to the current clinical data.