Showing papers in "Journal of Aapos in 2009"

The critical period for surgical treatment of dense congenital bilateral cataracts.

Abstract: Introduction We examined the critical period for deprivation amblyopia in a cohort of patients with dense bilateral congenital cataracts to investigate the optimum timing for surgical treatment. Methods Thirty-seven infants with dense bilateral congenital cataracts that were extracted by 31 weeks of age were enrolled prospectively. Visual acuity outcome was assessed at ≥5 years of age. We statistically evaluated which of 4 models provided the best fit to the data: (1) no change in visual acuity outcome with delay in surgery, (2) linear decline of outcome with delay, (3) a bilinear model in which a critical age exists after which outcome depends on delay, and (4) a bilinear model in which a critical age exists before which outcome depends on delay. In addition, we reviewed medical records for associated adverse outcomes, including strabismus, nystagmus, secondary membrane formation, and glaucoma. Results A bilinear model with a critical age of 14 weeks fit the data better than a linear model (χ 2 = 14.7; p Conclusions We did not find a latent period for the treatment of children with dense bilateral congenital cataracts. Deprivation amblyopia may be minimized with early surgery for bilateral cataracts.

Leber congenital amaurosis: clinical correlations with genotypes, gene therapy trials update, and future directions.

Abstract: Leber congenital amaurosis comprises a group of early onset childhood retinal dystrophies, characterized by vision loss, nystagmus, and severe retinal dysfunction. To date, 15 causative genes have been identified that account for the heterogeneous presentation and clinical course. Knowledge of key aspects of phenotype and clinical course can contribute to the determination of a precise genetic etiology using genetic testing. Gene-based therapies are emerging, and knowledge of a patient's genotype is essential. A review of clinical presentation and disease course, their correlation to specific genotypes, and underlying physiological mechanisms, coupled with the latest results of human gene therapy trials, will assist the clinician in patient diagnosis and counseling.

"Heavy Eye" syndrome in the absence of high myopia: A connective tissue degeneration in elderly strabismic patients.

Abstract: Purpose In axial high myopes with “heavy eye” syndrome, orbital MRI can be use to demonstrate degeneration of the lateral rectus–superior rectus (LR-SR) band, with the result that the lateral rectus muscle slips inferiorly and causes esotropia and hypotropia. We investigated whether this degeneration might also cause strabismus in nonmyopic elderly patients. Methods Three elderly patients with strabismus, 3 strabismic high myopes, and 12 orthotropic elderly subjects underwent ophthalmic examinations and orbital MRI. The lateral rectus muscle position was determined relative to globe center from quasicoronal images and correlated with LR-SR band structure. MRI scans were compared with histology of 4 cadaveric orbits ranging in age from 17 months to 93 years. Results Two strabismic patients exhibited hypotropia; one exhibited esotropia. Mean axial length was 24.1 ± 0.8 mm (mean ± SD), compared with 31.6 ± 1.4 mm for myopes. The lateral rectus muscle position of elderly strabismic subjects averaged 4.6 ± 1.7 mm inferior to globe center, which was significantly lower than that of orthotropic elderly subjects (2.1 ± 1.9 mm; p = 0.01) and similar to that of high myopes (5.1 ± 3.2 mm). On MRI scanning, 100% of strabismic elderly orbits, 67% of strabismic myopic orbits, and 12.5% of control elderly orbits showed LR-SR band thinning, discontinuity, or displacement. LR-SR band degeneration was present histologically only in older cadavers. Conclusions Age-related LR-SR band degeneration permits the lateral rectus muscle to slip inferiorly in elderly nonmyopes, a mechanism of strabismus similar to myopic “heavy eye” syndrome. Imaging may assist in diagnosing this mechanical cause of age-related strabismus.

Postoperative outcomes in children with intermittent exotropia from a population-based cohort

Abstract: Purpose To describe the long-term surgical outcomes in a population-based cohort of children with intermittent exotropia. Methods The medical records of all children ( Results Of 184 patients with intermittent exotropia, 61 (33%) underwent surgery at a mean age of 7.6 years (range, 3.2 to 23 years). Twelve of the 61 children (19.7%) underwent a second surgery (10 for recurrent exotropia and 2 for consecutive esotropia), and no patient received 3 or more surgeries during a mean follow-up of 10 years from the first surgery. The final postoperative measurements were recorded in 56 of 61 patients (92%) at a mean of 7.4 years (range, 0 to 18 years) after the first surgery: 31 of the 56 (55%) were within 9 Δ of orthotropia at distance and 25 of 55 (45%) had better than 60 seconds of stereopsis. The Kaplan-Meier rate of developing ≥10 Δ of misalignment after the first surgery was 54% by 5 years, 76% by 10 years, and 86% by 15 years. Conclusions In this population-based study of surgery in children with intermittent exotropia, although only 1 in 5 received a second surgery, after a mean follow-up of 8 years, approximately half were successfully aligned and 45% had high-grade stereopsis.

Treatment of severe amblyopia with weekend atropine: results from 2 randomized clinical trials.

Abstract: Purpose To determine the effectiveness of weekend atropine for severe amblyopia from strabismus, anisometropia, or both combined among children 3 to 12 years of age. Methods We enrolled children into 2 prospective, randomized multicenter clinical trials of amblyopia therapy. Herein we report the results for severe amblyopia, 20/125 to 20/400. In Trial 1, 60 children 3 to 6 years of age (mean, 4.4 years) were randomized to weekend atropine plus a plano lens or weekend atropine plus full spectacle correction for the sound eye. In Trial 2, 40 children 7 to 12 years of age (mean, 9.3 years) were randomized to weekend atropine or 2 hours of daily patching. The visual acuity outcome was assessed at 18 weeks in Trial 1 and 17 weeks in Trial 2. Results In Trial 1, visual acuity improved by an average of 4.5 lines in the atropine plus correction group (95% CI, 3.2-5.8 lines) and 5.1 lines in the atropine plus plano lens group (95% CI, 3.7-6.4 lines). In Trial 2, visual acuity improved by an average of 1.5 lines in the atropine group (95% CI, 0.5-2.5 lines) and 1.8 lines in the patching group (95% CI, 1.1-2.6 lines). Conclusions Weekend atropine can improve visual acuity in children 3 to 12 years of age with severe amblyopia. Improvement may be greater in younger children.

Odds of abuse associated with retinal hemorrhages in children suspected of child abuse.

Abstract: Purpose To describe the prevalence of retinal hemorrhages in children being evaluated for abusive head trauma and quantify the association between the likelihood of abuse and the presence and severity of retinal hemorrhages. Methods Retrospective cross-sectional study of 110 children aged 15 months or younger who were evaluated for abusive head trauma and received an ophthalmological examination. The child abuse specialist's diagnosis was categorized as definite accident, probable accident, probable abuse, or definite abuse, according to an algorithm that excluded eye findings. Retinal hemorrhage severity was scored on a 12-point scale (6 points per eye) based on type, size, location, and extent. Higher scores indicated greater severity of eye findings. Results Seventy-four percent of children were younger than 6 months old. Forty-five percent of cases were definite abuse and 37% were definite accident. The prevalence of retinal hemorrhages was 32%. Across all subjects, the presence of retinal hemorrhage was highly associated with definite or probable abuse versus definite or probable accident (age-adjusted odds ratio 5.4 [95% CI, 2.1-13.6]). The odds ratio in children younger than 6 months (n = 81) was 11.7 (95% CI, 2.9-66.8). Retinal hemorrhage severity was higher in abuse versus accident ( p r = 0.406, p Conclusions Retinal hemorrhages are highly associated with abusive head trauma, particularly in children younger than 6 months of age. Increasing retinal hemorrhage severity is correlated with increasing likelihood of abuse.

An evidence-based update on myopia and interventions to retard its progression

Abstract: Myopia is the most common human eye disorder. With its increasing prevalence and earlier age-of-onset in recent birth cohorts, myopia now affects almost 33% of adults in the United States, and epidemic proportions of 85% to 90% adults in Asian cities. Unlike children in Western populations, where the prevalence of myopia is very low (less than 5%), Asian children have prevalences as high as 29% in 7-year-olds. In addition to the direct economic and social burdens of myopia, associated ocular complications may lead to substantial vision loss. This workshop summarizes the current literature regarding myopia epidemiology, genetics, animal model studies, risk factors, and clinical treatments. Published treatment strategies to retard the progression of myopia in children, such as pharmacologic agents, progressive addition lenses, and neural adaptation programs, are outlined.

Unilateral amblyopia: An optical coherence tomography study.

Abstract: Purpose To determine whether retinal nerve fiber layer thickness (RNFLT), macular thickness (MT), and foveal volume (FV) in patients with unilateral amblyopia differ between the amblyopic and the sound eye. Methods A Humphrey-Zeiss Stratus (OCT3) with software 4.0.3.1 was used to evaluate 40 patients (17 male, 23 female; mean age, 15.2 years; range, 5–56 years) with unilateral amblyopia. Patients were divided into 2 groups: 20 strabismic and 20 anisometropic. Maps of macular thickness and RNFL thickness (3.46) created by the use of optical coherence tomography were applied to calculate FV and MT and RNFLT. Results Mean best-corrected visual acuity was +0.3 LogMAR (range, +0.2 to +1.0) in the amblyopic eye. Mean RNFL thickness was not significantly different between eyes in either group. In strabismic amblyopia, mean MT and FV were 5% lower in the sound eye than in the amblyopic eye (MT, 267 μm vs 253 μm, p = 0.005; FV, 2.57 mm 3 vs 2.43 mm 3 , p = 0.001). In anisometropic amblyopia, there was no significant difference between eyes in either MT or FV. Conclusions In patients with strabismic amblyopia, the MT and FV were slightly but significantly lower in the sound eye than in the amblyopic eye. The clinical importance of this difference is not known. No such difference was observed in patients with anisometropic amblyopia.

Finite element model of ocular injury in abusive head trauma.

Abstract: Purpose To develop a finite element analysis of the eye and orbit that can be subjected to virtual shaking forces. Methods LS-DYNA computer software was used to design a finite element model of the human infant eye, including orbit, fat, sclera, retina, vitreous, and muscles. The orbit was modeled as a rigid solid; the sclera and retina as elastic shells; the vitreous as viscoelastic solid or Newtonian fluid; and fat as elastic or viscoelastic solid. Muscles were modeled as spring-damper systems. Orbit-fat, fat-sclera, sclera-retina, and vitreous nodes-retina interfaces were defined with the use of the tied surface-surface function in LS-DYNA. The model was subjected to angular acceleration pulses obtained from shaking tests of a biofidelic doll (Aprica 2.5 kg dummy). Parametric studies were conducted to evaluate the effect of varying the material properties of vitreous/fat on maximum stress and stress distribution. Results With the vitreous modeled as a Newtonian fluid, the repeated acceleration-deceleration oscillatory motion characteristic of abusive head trauma (AHT) causes cumulative increases in the forces experienced at the vitreoretinal interface. Under these vitreous conditions, retinal stress maximums occur at the posterior pole and peripheral retina, where AHT retinal hemorrhage is most often found. Conclusions Our model offers an improvement on dummy and animal models in allowing analysis of the effect of shaking on ocular tissues. It can be used under certain material conditions to demonstrate progressive "stacking" of intraocular stresses in locations corresponding to typical AHT injury patterns, allowing a better understanding of the mechanisms of retinal hemorrhage patterns.

The effect of induced monocular blur on measures of stereoacuity

Abstract: Purpose To determine the effect of induced monocular blur on stereoacuity measured with real depth and random dot tests. Methods Monocular visual acuity deficits (range, 20/15 to 20/1600) were induced with 7 different Bangerter filters ( 60 and ≤200 arcsec), or “coarse/nil” (>200 arcsec to nil) stereo. Results Across visual acuity deficits, stereoacuity was more severely degraded with random dot (PSR, DR) than with real depth (Frisby, FD2) tests. Degradation to worse-than-fine stereoacuity consistently occurred at 0.7 logMAR (20/100) or worse for Frisby, 0.1 logMAR (20/25) or worse for PSR, and 0.1 logMAR (20/25) or worse for FD2. There was no meaningful threshold for the DR because worse-than-fine stereoacuity was associated with −0.1 logMAR (20/15). Course/nil stereoacuity was consistently associated with 1.2 logMAR (20/320) or worse for Frisby, 0.8 logMAR (20/125) or worse for PSR, 1.1 logMAR (20/250) or worse for FD2, and 0.5 logMAR (20/63) or worse for DR. Conclusions Stereoacuity thresholds are more easily degraded by reduced monocular visual acuity with the use of random dot tests (PSR and DR) than real depth tests (Frisby and FD2). We have defined levels of monocular visual acuity degradation associated with fine and nil stereoacuity. These findings have important implications for testing stereoacuity in clinical populations.

Comparison of botulinum toxin with surgery as primary treatment for infantile esotropia

Abstract: Purpose To compare bilateral medial rectus muscle injection of botulinum toxin with surgery as primary treatment for infantile esotropia. Methods A single-center, prospective, nonrandomized comparative study was undertaken of botulinum toxin versus surgery in children who presented by age 36 months with esotropia onset before 12 months. Successful outcome was defined as ocular alignment within 10 Δ of orthotropia after one surgery or 1 to 3 bilateral botulinum injections. Results Of 442 subjects, 322 received botulinum toxin (1 injection, 49%; 2, 41%; 3, 10%); 120 had surgery. Motor success was achieved in 66% of surgery patients, compared with 45% of botulinum patients ( p 30 Δ , surgery achieved 69% success versus 36% with botulinum toxin (relative risk, 1.95; 95% CI, 1.53-2.49). At deviations ≤30 Δ , there was no difference (surgery, 60%; botulinum toxin, 59%; relative risk, 1.03; 95% CI, 0.78-1.35). There were no statistically significant differences in mean pretreatment deviation (botulinum toxin, 38.8 Δ ; surgery, 38.2 Δ ) or mean follow-up (botulinum toxin, 22.6 months; surgery, 20.7). Surgery occurred later than botulinum injection (mean age at treatment, 27.0 vs. 16.7 months; p p Conclusions In this large, nonrandomized prospective cohort, surgery was more successful than botulinum toxin in the treatment of large-angle esotropia. Botulinum toxin appeared most effective for esotropia Δ to 35 Δ , with a success rate comparable with surgery. Botulinum toxin may be an alternative to surgery in children with small- to moderate-angle infantile esotropia.

Bilateral response following unilateral intravitreal bevacizumab injection in a child with uveitic cystoid macular edema.

Abstract: Untreated cystoid macular edema (CME) is a major cause for visual loss in intermediate and posterior forms of uveitis. Conventional treatments for inflammatory CME include steroids, nonsteroidal antiinflammatory agents, and carbonic anhydrase inhibitors; however, not all patients respond, even after quieting of the uveitis. Intravitreal injection of bevacizumab, a monoclonal antibody to vascular endothelial growth factor, has recently been suggested as a short-term treatment for inflammatory CME in adults. Because unilaterally injected bevacizumab can reach the contralateral eye via the systemic circulation there may be a contralateral clinical effect; however, the few reports that evaluate this in adults are conflicting. The purpose of this report is to document bilateral reduction of uveitic CME following unilateral intravitreal bevacizumab injection in an 8-year-old girl.

The visual status of children ages 3 to 6 years in the vision screening program in Taiwan.

Abstract: Purpose To investigate the current status of myopia, amblyopia, and strabismus of preschoolers in Taiwan. Methods The records of a vision screening performed in 2005 at 4 preschools by a tertiary referral medical center in Kaohsiung, Taiwan were reviewed. Cycloplegic refraction, visual acuity by E chart, stereopsis by Titmus and National Taiwan University tests, and ocular alignment were analyzed. Results Most (97.7%) of the children (n = 618) were able to perform visual acuity testing. There was good correlation between the results of cycloplegic spherical equivalent obtained by autorefraction and by retinoscopy ( r = 0.934, p p = 0.028, χ 2 test for trend). The prevalence of myopia was 3.0%, 4.2%, 4.7%, and 12.2% at ages 3, 4, 5, and 6 years, respectively. There was a trend of increased myopia with increased age ( p = 0.021, χ 2 test for trend). Conclusions The prevalence of myopia in children ages 3 to 6 years in Taiwan was high compared with the prevalence in other countries. Criteria for amblyopia based on age difference and on the types of visual acuity test are recommended.

Field testing of the plusoptiX S04 photoscreener.

Abstract: Purpose To evaluate the diagnostic accuracy of the plusoptiX S04 digital photoscreener in a school screening program. Methods Between 2006 and 2007, 1343 information pamphlets/consent forms were sent to all junior kindergarten students in a local school district. Assistants from a local public health unit photographed 307 children. Of these, 271 children received an independent ophthalmic examination by a physician. Photographic results were compared with the those of the ophthalmic examination. Amblyopia risk factors were defined as anisometropia >1 D (sphere or cylinder), astigmatism >1.25 D, myopia >3 D, hyperopia >3.5 D, any manifest strabismus, and any media opacity. Results Photographic and examination results agreed in 94% of cases. Sensitivity in detecting amblyopia risk factors was 83%; specificity was 95%. The positive and negative predictive values were 73% and 97%, respectively. The untestable/unusable rate was 1%. Conclusions These results compare favorably with a previously reported (but no longer available) digital photoscreening camera and are superior to results obtained with other off-axis photoscreening devices that require human interpretation. On the basis of these results, in a real-world screening program, the camera would falsely refer 4% of those screened and would fail to correctly refer 2%. The accuracy of the plusoptiX S04 camera in detecting amblyopia risk factors appears sufficiently high to consider its further deployment in a widespread school screening program.

Long-term follow-up of congenital esotropia in a population-based cohort

Abstract: Purpose To report the long-term outcomes of a population-based cohort of children diagnosed with congenital esotropia during a 30-year period. Methods The medical records of all patients diagnosed with congenital esotropia as residents of Olmsted County, MN, from January 1, 1965, through December 31, 1994, were retrospectively reviewed. Results A total of 130 children were diagnosed during the 30-year period at a median age of 7.4 months with a mean deviation of 30 Δ . During a median follow-up of 11.9 years, 126 patients underwent a mean of 1.8 strabismus surgeries. The risk for undergoing a second surgery was significantly greater in patients with a larger presenting angle ( p = 0.017) and a younger age at first surgery ( p = 0.006). The Kaplan-Meier rate of having a second surgery was 51% at 10 years and 66% at 20 years. For those with 6 weeks or more of follow-up from the final surgery, last examined at a mean age of 15.1 years, 42 of 94 (45%) were within 8 Δ of orthotropia and 30 of 98 had some level of stereopsis (≤3000 arcsec). Conclusions In this population-based study of children with congenital esotropia, a second surgery was necessary in half the patients after 10 years and was more likely in those patients with a larger presenting angle and a younger age at first surgery. Approximately half of the patients were within 8 Δ of orthotropia and one-third had measurable stereopsis after a mean of 10.9 years of follow-up.

Longitudinal follow-up of hypermetropic children identified during preschool vision screening.

Abstract: Purpose Early childhood hypermetropia is an important risk factor for the development of amblyopia and esotropia. Understanding the natural history of these complications aids in management decisions. Methods A retrospective observational review was undertaken of 149 patients referred from a preschool photoscreening program who were determined to have hypermetropia of ≥+3.75 D spherical equivalent on criterion standard examination and were treated/followed by one group of academic pediatric ophthalmologists. The prevalence and incidence of accommodative esotropia and amblyopia were determined. Results At presentation 19% of hypermetropic children had amblyopia, 32% had esotropia, and 13% had both. Follow-up data of 108 patients during a mean of 40 months showed that 20 (24%) of 83 initially nonamblyopic patients developed amblyopia and that 22 (33%) of 67 initially nonstrabismic patients developed accommodative esotropia. Of patients initially managed with observation, 38% (6 of 16) developed amblyopia, and 31% (5 of 16) developed accommodative esotropia as compared with 21% (14 of 67) and 33% (17 of 51), respectively, for those given full or partial refractive correction. For patients without amblyopia or strabismus at presentation, only 20% developed amblyopia and 35% esotropia. Strabismic patients responded well to treatment, with no cases developing partially accommodative strabismus requiring surgery during follow-up. Conclusions In this case series we found a high prevalence of amblyopia and strabismus. The results support the importance of early preschool vision screening and spectacle correction of moderate to high hypermetropia (>+3.50 D) to reduce the risk of amblyopia, although more research is needed.

Port-wine vascular malformations and glaucoma risk in Sturge-Weber syndrome

Abstract: Purpose Treatment of the capillary vascular malformation (port-wine stain) in Sturge-Weber syndrome with the use of a laser is helpful cosmetically. However, concerns have been raised that laser obliteration of port-wine stains may result in ocular hypertension. The aim of this study was to review clinical features and management of ocular complications of SWS and assess the effects of dermatological laser treatment on the incidence of glaucoma or ocular hypertension. Methods This retrospective cohort study was conducted in an institutional setting. All patients had involvement of the face. Patients who underwent skin laser to the port-wine vascular malformation were analyzed further. Ocular involvement, glaucoma, and skin laser treatment and the relationship to ocular hypertension/glaucoma were observed. Results Forty-one Sturge-Weber syndrome patients with port-wine vascular malformation were analyzed. Glaucoma was observed in 24 patients (58.5%) at mean age of 2.9 years (range, 0.0−16.5). Of these, 18 (75.0%) were treated with medical therapy, and 10 (41.7%) required trabeculectomy, with 2 of these requiring Seton implant. Of the 41 patients, 28 (68.3%) underwent laser to face/forehead. Mean age of laser commencement was 5 years (range, 0.4−16.5). Thirteen did not undergo laser treatment. Fourteen of the 28 and 10 of the 13 developed ocular hypertension/glaucoma. Conclusions This retrospective review did not find evidence to suggest that laser treatment of port-wine vascular malformations causes glaucoma or that it can worsen a preexisting ocular hypertension or glaucoma. Statistical analysis was inconclusive.

Treatment of unilateral coronal synostosis by endoscopic strip craniectomy or fronto-orbital advancement: Ophthalmologic findings.

Abstract: Background Unilateral coronal synostosis results in ipsilateral retrusion of the forehead and superior orbital rim, shortening the elevation of the orbital roof and contralateral frontal bossing and orbital roof depression. This craniosynostosis is associated with the development of V-pattern strabismus and aniso-astigmatism. Since 2004 endoscopic strip craniectomy performed in patients by 3 months of age has been offered as an alternative to fronto-orbital advancement at 9 to 11 months of age. We compare the incidence and severity of V-pattern strabismus and aniso-astigmatism in children treated by these 2 procedures. Methods A retrospective review identified 37 children with unilateral coronal synostosis treated with either fronto-orbital advancement or endoscopic strip craniectomy. Incidence and severity of V-pattern strabismus, fundus excyclotorsion, and aniso-astigmatism was recorded for an "early" examination (between 2 and 14 months of age) and a "late" examination (between 14 and 45 months of age). Results Early examination revealed no statistical difference in severity of V-pattern strabismus or aniso-astigmatism between the 2 groups. At late examination there was a trend toward greater severity of V-pattern strabismus, an increase in excyclotorsion, and a statistically significant increase in the standard deviation of aniso-astigmatism in the cohort of children treated by fronto-orbital advancement. Conclusions Children with unilateral coronal synostosis treated by early endoscopic strip craniectomy may develop less severe V-pattern strabismus, excyclotorsion, and range of aniso-astigmatism than those treated by later fronto-orbital advancement. This is an early, retrospective, nonrandomized study with a short follow-up period; longer follow-up is necessary to confirm these results.

Adult strabismus and social phobia: a case-controlled study.

Abstract: Purpose To determine the social phobia rate, social anxiety level, severity of depressive symptoms, and disease-related disability in adult strabismus patients. Methods Forty-nine strabismus patients and 46 control subjects from 15 to 65 years of age were evaluated. A psychiatric interview focusing on social phobia and the Liebowitz Social Anxiety Scale (LSAS) were administered to each participant. All participants completed the Hospital Anxiety and Depression Scale (HADS), a psychiatric symptom checklist (Symptom Checklist 90-Revised; SCL-90R), and the Sheehan Disability Scale. Results Social phobia was diagnosed in 26 of the 49 strabismus patients and in 8 of the 46 control subjects ( p Conclusions In adult strabismus patients, social phobia is a frequent psychiatric comorbidity. Adult patients with strabismus need to be carefully evaluated for social phobia for an appropriate referral and treatment.

Cyclophotocoagulation versus sequential tube shunt as a secondary intervention following primary tube shunt failure in pediatric glaucoma.

Abstract: Purpose To examine the efficacy of a sequential tube shunt versus transscleral diode cyclophotocoagulation following failure of an initial tube shunt on maximal medical therapy in treatment of refractory childhood glaucoma. Methods A nonrandomized retrospective chart review was conducted of 17 eyes of 14 pediatric patients (less than 18 years old) with refractory glaucoma treated with either sequential tube shunt (Group A) or diode cyclophotocoagulation (Group B) following initial failed tube shunt. Success was defined as an intraocular pressure ≤22 mm Hg on medical therapy, no visually devastating complications, and no further glaucoma surgery performed or recommended. Results Of the 17 eyes, 8 had a sequential tube shunt and 9 underwent diode cyclophotocoagulation as a secondary procedure. Kaplan-Meier analysis demonstrated a successful outcome of 75% and 62.5% at 12 months and 24 months, respectively, for Group A, and 66.7% at both 12 months and 24 months for Group B ( p = 0.48). Corneal decompensation or graft failure was noted in 3/8 eyes (38%) in Group A. Cataract surgery was performed in 2/5 phakic eyes (40%) in Group B. One eye in each group progressed to no light perception. Conclusions Diode cyclophotocoagulation and sequential tube shunt following primary tube shunt failure in childhood glaucoma showed similar efficacy and complication rates. However, the small sample size of this study warrants further evaluation of these 2 procedures following failure of a tube shunt device in pediatric glaucoma.

Long-term visual outcome of penetrating keratoplasty in infants and children with Peters anomaly

Abstract: Purpose To determine the long-term visual outcome of penetrating keratoplasty for Peters anomaly and to identify prognostic factors affecting final vision. Methods The records of children 12 years of age or younger who underwent penetrating keratoplasty for Peters anomaly between January 1, 1971 and December 31, 1992 at Emory University were reviewed. Characteristics of the recipient, eye, donor, and surgical procedure were examined with the use of multivariate analyses. Results One hundred forty-four keratoplasties in 72 eyes of 47 children who were followed for a minimum of 3 years from the date of first keratoplasty (median, 11.1 years) were reviewed. Visual acuities ranged from 20/25 to no light perception. Twenty-nine percent of eyes achieved 20/400 or better visual acuities, whereas 38% had light perception or no light perception. Stromal vessels ( p p Conclusions Less than one-third of eyes with Peters anomaly undergoing keratoplasty achieved a visual acuity of 20/400 or better. Stromal vessels and large corneal grafts (≥8 mm) were the only independent predictors of a poor visual outcome.

Sensitivity of papilledema as a sign of shunt failure in children.

Abstract: Purpose Papilledema is considered one of the cardinal ophthalmologic signs of shunt failure. However, the prevalence of papilledema in children with shunt malfunction has not been systematically investigated. The purpose of this study is to determine the sensitivity of papilledema as a sign of shunt failure in children. Methods A prospective evaluation was undertaken of all children (n = 29; Results Twenty-nine patients had surgically confirmed shunt failure. Patient's age ranged from 36 days to 18 years, 8 months. Four patients (14%) had papilledema, and 1 patient had severe optic disk pallor. The remaining 24 patients had flat optic disks. Five patients had flat optic disks despite an intracranial pressure ≥300 mm H 2 O. Conclusions Papilledema is not a sensitive sign of shunt failure. Even children with severe elevations in intracranial pressure from shunt malfunction may have flat optic disks. Therefore, physicians that evaluate children with shunts should be aware that a normal optic disk does not preclude shunt malfunction.

Performance of the plusoptiX S04 photoscreener for the detection of amblyopia risk factors in children aged 3 to 5.

Abstract: Purpose Our previous study of the plusoptiX S04 photoscreener included children ages 6 months to 16 years and allowed us to modify the referral criteria retrospectively to improve specificity and sensitivity. In this study we used the new referral criteria and tested children ages 3 to 5 years, a more appropriate range for a pediatric vision screening program. Methods A total of 153 children were examined consecutively. Each patient was screened with the plusoptiX S04 photoscreener on the same day as part of a comprehensive pediatric ophthalmology examination. Results Fifty-three percent of the patients were found to have amblyopia or amblyopia risk factors during the pediatric ophthalmology examination. By using these results as our standard, we calculated that the plusoptiX S04 photoscreener offered a sensitivity of 99%, specificity of 82%, false-positive rate of 18%, and false-negative rate of 1.2%. These findings were similar to the results we achieved in our previous study with a broader age range. Conclusions The plusoptiX S04 is an automated photoscreening device that takes multiple images in less than 30 seconds, determining a noncycloplegic refraction. Sensitivity and specificity suggests that testing the plusoptiX S04 for applications in a general screening environment should be considered.

Management and outcome of unilateral retinoblastoma.

Abstract: Background Two-thirds of retinoblastoma cases are unilateral, with most presenting with an advanced stage of disease. Primary enucleation is usually the preferred treatment. Conservative therapy may save less involved eyes. Methods We retrospectively reviewed the presentation, age at diagnosis, classification, genetic status, treatment, and long-term outcome of 130 patients with unilateral retinoblastoma (1988–2008). Results The mean age at presentation was 26 months. Based on retinoblastoma gene ( RB1 ) status in tumors, germ-line status was defined in 92% of patients; 13% had a germ-line mutation. The primary treatment of 106 patients was enucleation. Severe disease at presentation (International Intraocular Retinoblastoma Classification [IIRC] group E) was significantly ( p Conclusions Chemotherapy/focal therapy can save selected eyes, but primary enucleation is preferred for advanced unilateral retinoblastoma. "Conservative" treatment is an option when there is good potential for useful vision without prolonged, costly therapy with potential side effects. Simple enucleation reduces the risk of masking high-risk pathology and promotes early return to normal life.

The psychosocial aspects of strabismus: correlation between the AS-20 and DAS59 quality-of-life questionnaires

Abstract: Purpose To compare two quality-of-life (QoL) scales and subscales: the Derriford Appearance Scale 59 (DAS59) and the Adult Strabismus-20 (AS-20) scale in a series of strabismic and nonstrabismic patients and to illustrate the differences in results between strabismic and nonstrabismic patients. Methods The DAS59 is a self-report QoL questionnaire generating an assessment of distress caused by problems of appearance. The AS-20 is a newly developed strabismus specific QoL scale. A prospective, noninterventional study of a series of consecutive adult patients undergoing strabismus surgery and a control group was performed. Patients were given both questionnaires to complete before surgery. The underlying condition, previous number of surgeries, and deviation measurements were recorded. Results Thirty-four patients and 30 controls correctly completed both questionnaires. In the strabismus group, the mean AS-20 score was 50.5. The mean DAS59 score was 122.5 (out of 269). There is strong correlation between the scales ( r = −0.813; 95% CI, −0.90 to −0.66; p t = 12.9 [ p t = −4.642 [ p Conclusions The AS-20 shows strong correlation to the widely used DAS59. Strabismus patients show significantly more psychosocial distress than controls. The AS-20 is specific to strabismus patients and the result is not influenced by any other systemic factors that can impinge on the more general DAS59 scale.

Improving postoperative follow-up of children receiving surgery for congenital or developmental cataracts in Africa.

Abstract: Purpose Access to cataract surgery for children has improved in recent years, and there are a number of tertiary facilities for children's eye health in sub-Saharan Africa. However, surgery alone will have limited value if postoperative follow-up is poor. We have developed a program to improve follow-up after pediatric cataract surgery and compare the follow-up rates before and after implementation. Methods Baseline information was collected from 2003 to 2004. Program activities were implemented in 2005. Postintervention information was collected from 2006. During both periods all children younger than 16 who had cataract surgery at Kilimanjaro Christian Medical Centre Hospital were included. Follow-up, defined at both 2 weeks and 10 weeks, was recorded. Results Baseline data showed that 67% and 43% of children, respectively, came for 2-week and 10-week postoperative follow-up. After the intervention, 89% came for 2-week follow-up and 83% came for 10-week follow-up. Gender inequity, documented in the baseline data, was eliminated in the postintervention period. Conclusion Follow-up of children who have had cataract surgery can be greatly enhanced in Africa. Improved follow-up enables children to receive the spectacles and low vision devices necessary for visual rehabilitation. Multiple strategies will probably be needed to improve follow-up.

Management of vertical deviations after vertical rectus transposition surgery.

Abstract: Purpose To describe options for the management of vertical deviations after vertical rectus muscle transposition surgery (VRT). Methods Retrospective case series including 7 children who underwent VRT for esotropic Duane syndrome and developed the complication of a vertical deviation. Results Eighty-two consecutive children underwent VRT for Duane syndrome. Seven eyes of 7 patients (4 boys, 3 girls) were found to have induced vertical deviations postoperatively. Average length of follow-up was 12.7 months (range, 3-28 months). Median induced vertical deviation was 10.1 Δ (range, 8-12). All patients required only one additional surgery to ameliorate the vertical deviation. At final follow-up, the mean vertical tropic deviation was 0. Six patients were operated on within 4 days of VRT. Surgical strategies included recession of one vertical rectus muscle and repositioning of a posterior fixation suture. Conclusions In children undergoing VRT for esotropic Duane syndrome, the complication of a vertical deviation occurred in 8.5% of cases. The vertical deviation was completely ameliorated in each case by one surgical procedure involving recession of one of the transposed muscles.

A comparison of Lea Symbol vs ETDRS letter distance visual acuity in a population of young children with a high prevalence of astigmatism.

Abstract: Purpose To compare visual acuity results obtained by use of the Lea Symbols chart with results obtained with Early Treatment Diabetic Retinopathy Study (ETDRS) charts in young children who are members of a population with a high prevalence of astigmatism. Methods Subjects were 438 children ages 5 through 7 years who were enrolled in kindergarten or first grade on the Tohono O'odham Reservation: 241 (55%) had astigmatism ≥1.00 D in one or both eyes (range, 0.00-6.75 D). While wearing best correction, each child had right eye visual acuity tested with the 62 cm by 65 cm Lea Symbols chart at 3 m and with the 62 cm by 65 cm ETDRS chart at 4 m. Visual acuity was scored as the smallest optotype size at which the child correctly identified 3 of a maximum of 5 optotypes. ETDRS visual acuity also was scored based on the total number of letters that the child correctly identified. Results Correlation between Lea Symbols visual acuity and ETDRS visual acuity was 0.78 (p Conclusions In this population of young children, in whom the primary source of reduced visual acuity is astigmatism-related amblyopia, the Lea Symbols chart produced visual acuity scores that were about 0.5 line better than visual acuity scores obtained with ETDRS charts.

Image analysis for retinopathy of prematurity diagnosis.

Abstract: Purpose To review findings from the authors' published studies involving telemedicine and image analysis for retinopathy of prematurity (ROP) diagnosis. Methods Twenty-two ROP experts interpreted a set of 34 wide-angle retinal images for presence of plus disease. For each image, a reference standard diagnosis was defined from expert consensus. A computer-based system was used to measure individual and linear combinations of image parameters for arteries and veins: integrated curvature (IC), diameter, and tortuosity index (TI). Sensitivity, specificity, and receiver operating characteristic areas under the curve (AUC) for plus disease diagnosis were determined for each expert. Sensitivity and specificity curves were calculated for the computer-based system by varying the diagnostic cutoffs for arterial IC and venous diameter. Individual vessels from the original 34 images were identified with particular diagnostic cutoffs, and combined into composite wide-angle images using graphics editing software. Results For plus disease diagnosis, expert sensitivity ranged from 0.308-1.000, specificity from 0.571-1.000, and AUC from 0.784 to 1.000. Among computer system parameters, one linear combination had AUC 0.967, which was greater than that of 18 of 22 (81.8%) experts. Composite computer-generated images were produced using the arterial IC and venous diameter values associated with 75% under-diagnosis of plus disease (ie, 25% sensitivity cutoff), 50% under-diagnosis of plus disease (ie, 50% sensitivity cutoff), and 25% under-diagnosis of plus disease (ie, 75% sensitivity cutoff). Conclusions Computer-based image analysis has the potential to diagnose severe ROP with comparable or better accuracy than experts, and could provide added value to telemedicine systems. Future quantitative definitions of plus disease might improve diagnostic objectivity.

The use of amniotic membrane in reducing adhesions after strabismus surgery.

Abstract: Postoperative adhesions after strabismus surgery may influence surgical outcome. Different techniques have been used with varying success to reduce these adhesions. We describe a novel surgical technique in which amniotic membrane is used to wrap the extraocular muscles.