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Given the prevalence of poor adherence to therapy and the biases of self-reporting across healthcare, we hypothesized that an engaging, personalized therapy may improve adherence and treatment outcomes in the home. We tested this hypothesis in the initial indication of amblyopia, a neurodevelopmental disorder for which available treatments are limited by low adherence. We designed a novel digital therapeutic that modifies patient-selected cinematic content in real-time into therapeutic visual input, while objectively monitoring adherence. The therapeutic design integrated a custom-designed headset that delivers precise visual input to each eye, computational algorithms that apply real-time therapeutic modifications to source content, a cloud-based content management system that enables treatment in the home, and a broad library of licensed content. In a proof-of-concept human study on the therapeutic, we found that amblyopic eye vision improved significantly after 12 weeks of treatment, with higher adherence than that of available treatments. These initial results support the utility of personalized therapy in amblyopia and may have broader relevance for improving treatment outcomes in additional indications.

To determine whether the fellow eye of children who have undergone unilateral cataract extraction in the first year of life are at increased risk of injury and vision loss, the 10.5-year data on 109 of 114 children enrolled in the Infant Aphakia Treatment Study were examined. Based on this limited data, it was estimated that the fellow eye is at greater risk of injury than the operated eye. Our data do not support the risk being higher in children with the worst vision in the treated eye.

PURPOSE: To determine whether age at surgery is associated with surgical outcome of intermittent exotropia (IXT) at 3 years. DESIGN: Secondary analysis of pooled data from a randomized trial METHODS: 197 children 3 to

The effectiveness of amblyopia therapy can be limited by poor adherence. Dichoptic therapies are a new approach, but recent trials have demonstrated difficulty maintaining high adherence over extended periods of at-home treatment. We evaluated the efficacy and adherence of Luminopia One-a dichoptic treatment that applies therapeutic modifications to streaming content chosen by the patient.This single-arm, multicenter prospective pilot study enrolled children aged 4-12 with anisometropic, strabismic, or mixed amblyopia at 10 pediatric ophthalmic and optometric practices across the United States. The therapeutic was prescribed for 1 hour/day, 6 days/week for 12 weeks of at-home use. The primary endpoint was best-corrected visual acuity (BCVA) at the 12-week follow-up visit.In total, 90 participants (mean age, 6.7 ± 2.0 years) were enrolled, and 73/90 participants (81%) had prior treatment beyond refractive correction. For those who completed the 12-week visit, mean amblyopic eye BCVA improved from 0.50 logMAR to 0.35 logMAR (1.5 logMAR lines; 95% CI, 1.2-1.8 lines; P 0.0001). Mean stereoacuity improved by 0.28 log arcsec (95% CI, 0.14-0.42 log arcsec; P 0.0001). Median adherence was 86% (interquartile range, 70%-97%).In our study cohort, adherence over the 12-week study period was high, and participants demonstrated clinically and statistically significant improvements in visual acuity and stereoacuity.

Purpose To determine whether stereopsis of infants treated for monocular cataracts varies with the type of optical correction used. Design Randomized prospective clinical trial. Methods The Infant Aphakia Treatment Study randomized 114 patients with unilateral cataracts at age 1–7 months to either primary intraocular lens (IOL) or contact lens correction. At 4.5 years of age a masked examiner assessed stereopsis on these patients using 3 different tests: (1) Frisby; (2) Randot Preschool; and (3) Titmus Fly. Results Twenty-eight patients (25%) had a positive response to at least 1 of the stereopsis tests. There was no statistically significant difference in stereopsis between the 2 treatment groups: Frisby (contact lens, 6 [11%]; IOL, 7 [13%]; P = .99), Randot (contact lens, 3 [6%]; IOL, 1 [2%]; P = .62), or Titmus (contact lens, 8 [15%]; IOL, 13 [23%]; P = .34). The median age at surgery for patients with stereopsis was younger than for those without stereopsis (1.2 vs 2.4 months; P = .002). The median visual acuity for patients with stereopsis was better than for those without stereopsis (20/40 vs 20/252; P = .0003). Conclusion The type of optical correction did not influence stereopsis outcomes. However, 2 other factors did: age at surgery and visual acuity in the treated eye at age 4.5 years. Early surgery for unilateral congenital cataract and the presence of visual acuity better than or equal to 20/40 appear to be more important than the type of initial optical correction used for the development of stereopsis.

The purpose of this report was to present a case of congenital alacrima in a patient with blepharophimosis-ptosis-epicanthus inversus syndrome (BPES). A 9-month-old boy presented with characteristic clinical findings of BPES confirmed by genetic testing. On further history taking and evaluation, the patient was noted to have no tear production, despite clinically present palpebral lobes of the lacrimal glands. BPES is an autosomal dominant condition characterized by narrowed horizontal palpebral fissures, severe bilateral symmetric ptosis, epicanthus inversus, and telecanthus. To the authors' knowledge, this represents the second reported case of congenital alacrima in this syndrome. The first case described in the literature was in a 9-month-old girl who had congenital absence of the lacrimal glands. BPES may present with alacrima requiring vigilant lifelong lubrication and careful consideration in decisions for eyelid surgery including ptosis repair.

Objective To evaluate the safety and efficacy of the tumor necrosis factor fusion protein etanercept in children with treatment-resistant uveitis. Methods Ten children with chronic active uveitis (7 girls and 3 boys, mean age 7.5 years [range 3–12 years]) were enrolled in this prospective study. In 7 children, uveitis was associated with pauciarticular juvenile rheumatoid arthritis. Five children were antinuclear antibody positive. All patients had failed previous therapy with topical steroids and methotrexate and/or cyclosporine. All were treated with etanercept at a dosage of 0.4 mg/kg twice weekly for the first 3 months, and then, if eyes did not improve, with 25 mg twice weekly (mean 1.1 mg/kg) for at least 3 additional months. Results At the beginning of the trial, uveitis affected 18 eyes in the 10 children. Within 3 months, 10 of 16 affected eyes (63%; P = 0.017) showed a rapid decrease in anterior chamber cell density, including remission of uveitis in 4 eyes. In children with visual acuity of less than 20/25, 4 of 10 eyes (40%) improved. An exacerbation of uveitis during etanercept therapy occurred in only 1 child (1 of 14 eyes [7%]). Other ocular outcome parameters, such as intraocular pressure, synechia formation, and lens clarity, remained unchanged. Following a dosage increase to an average of 1.1 mg/kg after 3 months in 7 children, no further improvement was noted. Conclusion Our data suggest that etanercept injected subcutaneously twice a week has a beneficial effect on treatment-resistant chronic uveitis in children. Further controlled studies with etanercept in systemic or topical form are necessary to confirm its efficacy and optimal mode of administration.

Purpose To describe a rarely reported complication of strabismus surgery. Design Observational case series. Methods A review of four eyes in three patients with orbital cysts following strabismus surgery. Results Each patient had either a symptomatic strabismus or visible mass that brought them to medical attention many years, often decades after surgery (mean 34 years). All had some degree of incomitancy. During surgery, all cysts were found to be associated with the involved rectus muscle. Conclusions Orbital cysts are a rarely recognized complication of strabismus surgery. However, it should be considered in the differential of orbital cysts after strabismus surgery because of the risk of muscle damage during surgical excision.

Blindness from pediatric cataracts has been largely curtailed in the last few decades with the aid of routine pediatric screening, early diagnosis, prevention of congenital rubella, improved surgical techniques, and visual rehabilitation through contact lenses, and now, lens implantation. Unfortunately aphakic patients are prone to glaucoma, and management of this disease in children is complicated by the difficulty in monitoring young patients for glaucomatous change. Because of longevity, children are also at greater risk for a gradual long-term pressure rise in spite of medical or surgical intervention. Advances in glaucoma medications and surgery in the past few decades have offered new treatment, but as in all disease states, a continued search for prevention may be most effective.

Cerebrotendinous Xanthomatosis (CTX) is an autosomal recessive disorder of bile acid synthesis1. At least 50 different causative mutations have been identified in the CYP27A1 gene encoding for a sterol 27-hydroxylase important in bile acid synthesis2. Sterol 27-hydroxylase deficiency leads to 5α-cholestanol accumulation in the blood and tissues of affected patients1, including brain, often leading to severe neurological dysfunction that can incapacitate patients by the 4th–5th decade of life1. CTX generally presents clinically in the 2nd–3rd decade; childhood signs and symptoms can include chronic diarrhea, juvenile cataracts, school failure1,3, and potentially xanthomas1. Eighty-five percent of CTX patients develop cataracts of multiple types2, reported to occur as young as age 5–6 years1, with cataracts and chronic diarrhea commonly presenting before neurologic disease3. For this reason, and since diarrhea is non-specific, the clinician may not consider a diagnosis of CTX when cataracts are identified. Treatment for CTX is available in the form of chenodeoxycholic acid (CDCA)1,4. The FDA recently reapproved CDCA (Manchester Pharmaceuticals, Inc). As treatment of CTX from the preclinical stage prevents the onset of disease complications4, the value of early diagnosis for this disorder cannot be overstated. We describe two cases of CTX affected children enrolled in an IRB approved study at OHSU with bilateral cataracts as presenting signs of disease. Case 1 A 9 year old female was referred to an ophthalmologist for decreased vision over several months. Past ocular history was unremarkable; full ocular exam 2 years prior was normal. Family history was unremarkable. Past medical history was significant for diarrhea and nonverbal learning disorder. On examination uncorrected distance visual acuity was 20/80 and uncorrected near acuity was 20/50 in each eye. Manifest refraction was 0.75+0.50X90 in each eye, with corrected distance acuity of 20/60 in the right and 20/80 in the left. Complete dilated exam was remarkable for the presence of cataract in each eye and described as a diffuse nuclear haze on slit lamp examination, with mild posterior capsular opacification (figure 1). Retroillumination highlighted posterior capsular findings (figure 2). Sequential bilateral cataract surgery with intraocular lens placement resulted in a corrected visual acuity of 20/20 in each eye. A metabolic workup indicated plasma 5α-cholestanol was markedly elevated at 3.7 mg/dl (normal < 0.2 mg/dl1), suggestive of CTX. Urine testing confirmed CTX with elevated bile alcohol glucuronides present (30.1 μg/ml). CDCA treatment was initiated and diarrhea resolved within one month, with accompanying weight gain. Plasma cholestanol dropped to 0.5 mg/dl within 7 months and after 6 years of treatment is currently stable at

Objective To compare the use of contact lenses and intraocular lenses (IOLs) for the optical correction of unilateral aphakia during infancy. Methods In a randomized, multicenter (12 sites) clinical trial, 114 infants with unilateral congenital cataracts were assigned to undergo cataract surgery with or without IOL implantation. Children randomized to IOL treatment had their residual refractive error corrected with spectacles. Children randomized to no IOL treatment had their aphakia treated with a contact lens. Main outcome measures Grating acuity at 12 months of age and HOTV visual acuity at 4 1/2 years of age. Application to clinical practice This study should determine whether either treatment for an infant with a visually significant unilateral congenital cataract results in a better visual outcome. Results Enrollment began December 23, 2004, and was completed January 16, 2009. The median age at the time of cataract surgery was 1.8 months. Fifty patients were 4 to 6 weeks of age at the time of enrollment; 32, 7 weeks to 3 months of age; and the remaining 32, more than 3 to less than 7 months of age. Fifty-seven children were randomized to each treatment group. Eyes with cataracts had shorter axial lengths and steeper corneas on average than the fellow eyes. Conclusions The optimal optical treatment of aphakia in infants is unknown. However, the Infant Aphakia Treatment Study was designed to provide empirical evidence of whether optical treatment with an IOL or a contact lens after unilateral cataract surgery during infancy is associated with a better visual outcome.

Importance The Intermittent Exotropia Questionnaire (IXTQ) is a patient, proxy, and parental report of quality of life specific to children with intermittent exotropia. We refine the IXTQ using Rasch analysis to improve reliability and validity. Observation Rasch analysis was performed on responses of 575 patients with intermittent exotropia enrolled from May 15, 2008, through July 24, 2013, and their parents from each of the 4 IXTQ health-related quality-of-life questionnaires (child 5 through 7 years of age and child 8 through 17 years of age, proxy, and parent questionnaires). Questionnaire performance and structure were confirmed in a separate cohort of 379 patients with intermittent exotropia. One item was removed from the 12-item child and proxy questionnaires, and response options in the 8- to 17-year-old child IXTQ and proxy IXTQ were combined into 3 response options for both questionnaires. Targeting was relatively poor for the child and proxy questionnaires. For the parent questionnaire, 3 subscales (psychosocial, function, and surgery) were evident. One item was removed from the psychosocial subscale. Resulting subscales had appropriate targeting. Conclusions and Relevance The Rasch-revised IXTQ may be a useful instrument for determining how intermittent exotropia affects health-related quality of life of children with intermittent exotropia and their parents, particularly for cohort studies.

Objective: To determine whether earlier treatment of high-risk, prethreshold retinopathy of prematurity (ROP) improves retinal structural outcome at 2 years of age. Methods: Infants with bilateral high-risk prethreshold ROP had one eye randomly assigned to treatment with peripheral retinal ablation. The fellow eye was managed conventionally, and either treated at threshold ROP or observed if threshold was never reached. In patients with asymmetrical disease, the high-risk, prethreshold eye was randomised to earlier treatment or to conventional management. At 2 years of age, children were examined comprehensively by certified ophthalmologists to determine structural outcomes for their eyes. For the purposes of this study, an unfavourable structural outcome was defined as (1) a posterior retinal fold involving the macula, (2) a retinal detachment involving the macula or (3) retrolental tissue or "mass" obscuring the view of the posterior pole. Results of the 2-year examination were compared with those from the 9 months examination. Results: Data were available on 339 of 374 (90.6%) surviving children. Unfavourable structural outcomes were reduced from 15.4% in conventionally managed eyes to 9.1% in earlier-treated eyes (p = 0.002) at 2 years of age. Ophthalmic side effects (excluding retinal structure) from the ROP or its treatment were similar in the earlier-treated eyes and the conventionally managed eyes. Conclusion: The benefit of earlier treatment of high-risk prethreshold ROP on retinal structure endures to 2 years of age, and is not counterbalanced by any known side effect caused by earlier intervention. Earlier treatment improves the chance for long-term favourable retinal structural outcome in eyes with high-risk prethreshold ROP. Long-term follow-up is planned to determine structural and functional outcomes at 6 years of age., link_to_OA_fulltext

In the nonpediatric eye clinic, the physician often views the presence of a small child in an examining lane with some anxiety, if not dread. Examination of a child is quite different from that of the adult. The history is largely from a source other than the patient, and the examination requires patience and talent. There are several tricks to make the visit go as smoothly and efficiently as possible (see the box on the following page).

Purpose To compare rates and severity of complications between infants undergoing cataract surgery with and without intraocular lens (IOL) implantation. Design Prospective randomized clinical trial. Methods A total of 114 infants were enrolled in the Infant Aphakia Treatment Study, a randomized, multi-center (12) clinical trial comparing the treatment of unilateral aphakia in patients under 7 months of age with a primary IOL implant or contact lens. The rate, character, and severity of intraoperative complications, adverse events, and additional intraocular surgeries during the first 5 postoperative years in the 2 groups were examined. Results There were more patients with intraoperative complications (28% vs 11%, P = .031), adverse events (81% vs 56%, P = .008), and more additional intraocular surgeries (72% vs 16%, P Conclusion The increased rate of complications, adverse events, and additional intraocular surgeries associated with IOL implantation in infants

Because there is a broad spectrum of visual dysfunction in eyes with optic nerve hypoplasia, we determined whether any clinical observations, including optic disk measurements, could predict visual outcome in eyes with hypoplastic optic nerves.We prospectively studied the eyes of 19 children (eight consecutive and 11 randomly selected), ages 7 to 92 months, in whom a diagnosis of optic nerve hypoplasia was made by an ophthalmologist. Clinical assessment included visual acuity, pupillary responses, and nystagmus. The ratio of the horizontal disk diameter to the disk-macula distance was calculated from fundus photographs. The amplitude and latency of the flash visual-evoked potential as well as the threshold response to pattern reversal checks were recorded.Visual acuity was associated with pupil reactivity, nystagmus, the ratio of the horizontal disk diameter to the disk-macula distance, pattern visual-evoked potential threshold, and flash visual-evoked potential amplitude (P.001 for all parameters). All eyes with a ratio of the horizontal disk diameter to the disk-macula distance greater than .30 had good visual acuity, and all eyes with a ratio of the horizontal disk diameter to the disk-macula distance less than .30 had poor visual acuity. No eye with a ratio of the horizontal disk diameter to the disk-macula distance less than or equal to .15 had visual acuity better than light perception.Visual-evoked potential and photogrammetric measurement of relative disk size can be used to approximate visual acuity in eyes with optic nerve hypoplasia. This finding could lead to a diagnostic criterion or a severity classification for optic nerve hypoplasia.

Eyelid retraction in an adult occurs usually secondary to Graves' ophthalmopathy, but in children the diagnosis is often obscure. To our knowledge, there are no reports reviewing the relative frequency of the various etiologies of eyelid retraction in children. We reviewed the records of our Pediatric Service from 1976 to 1991 and identified 16 children with eyelid retraction. The eyelid retractions were unilateral in 11 cases and bilateral in five cases, and were the result of five different identifiable causes. Unilateral eyelid retraction was associated with congenital aberrant innervation of the third nerve (3), levator fibrosis (2), hemangioma (1), hyperthyroidism (1), craniosynostosis (1) and Down syndrome (1). Bilateral eyelid retraction was associated with either hyperthyroidism (2) or bilateral optic nerve anomalies with vertical nystagmus (2). Seven of our 11 patients with unilateral retraction had an identifiable etiology, and the remaining four patients had incomplete evaluations. Imaging studies were diagnostic in four of nine patients so studied. Based on our findings, we recommend thyroid studies in all patients with eyelid retraction without aberrant innervation, and neuroimaging if thyroid function is normal.

Extracorporeal membrane oxygenation (ECMO) is a modified, prolonged cardiopulmonary bypass procedure used to treat newborns who have reversible cardiac or respiratory failure. The venoarterial bypass technique requires cannulation of both the right carotid artery and the internal jugular vein, and after decannulation these vessels are permanently ligated. Left-sided retinal vascular changes after ECMO have been reported, and were attributed to ligation of these vessels. A retrospective review of the results of ocular examinations of 86 infants who had undergone ECMO therapy at Childrens Hospital in Los Angeles between March, 1987 and May, 1991 was conducted. Normal findings were noted in 73 infants. One infant had bilateral retinal vascular tortuosity, and 12 infants had incidental ocular findings, but there was no evidence of left-sided retinal hemorrhage, venous congestion, or tortuosity. Our results suggest that left-sided retinal vascular changes after ECMO do not occur, occur only rarely, or clear rapidly and result in no permanent retinal damage. To unequivocally rule out the possibility that ECMO can cause transitory retinal vascular changes, we recommend further prospective studies, with ocular examinations performed before, during, and after ECMO.

This Issue of Seminars in Ophthalmology deals with the rare and the common ends of the pediatric ophthalmology spectrum. Most general ophthalmologists have at least occasionally been faced with a child who requires vision assessment or has strabismus, and more burden may be placed on the generalist with increasing capitation and managed care. The article on evaluation of vision in the preverbal child by Drs Hall and Donahue provides a good overview of available tools for this age group. Despite awareness of its existence for centuries, amblyopia remains a leading cause of visual loss, and early detection and treatment results in the best visual outcome. Patching for amblyopia is still the mainstay of therapy and Drs Olson and Scott have provided a good algorithm for treatment of amblyopia once detected.