Scott Lambert, MD

All Publications

• Adrenal Suppression from Topical and Subconjunctival Steroids after Pediatric Cataract Surgery. Ophthalmology Lambert, S. R., Shah, S. 2018; 125 (10): 1644–45

  View details for DOI 10.1016/j.ophtha.2018.06.012

  View details for PubMedID 30243335

• Unilateral retinitis pigmentosa in children. Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus Mercado, C. L., Pham, B. H., Beres, S., Marmor, M. F., Lambert, S. R. 2018

  Abstract

  BACKGROUND: Retinitis pigmentosa (RP) is a group of rare inherited retinal disorders characterized by diffuse progressive degeneration of the retina that typically presents bilaterally. Unilateral RP has not often been reported in children. We present a series of cases that illustrate discrimination between unilateral and asymmetric disease and between dystrophy and acquired degeneration.METHODS: Four patients (9-15 years of age; 3 females) were referred to our institution for possible unilateral RP based on fundus appearance and unilateral symptoms. All underwent full-field electroretinography (ERG), spectral domain optical coherence tomography (SD-OCT), widefield and color fundus photography, and fundus autofluorescence (FAF) imaging. Genetic testing and a vitamin and essential fatty acids panel were also conducted in 1 patient.RESULTS: Unilateral retinal degeneration was confirmed in 2 patients, whose fellow eyes showed no abnormalities on ERG or imaging. The other 2 patients were found to have highly asymmetric retinal degeneration based on ERG, wide-angle images, and repeated examinations (range, 0.3-9.8 years). Genetic testing and blood testing in 1 unilateral case were negative.CONCLUSIONS: Childhood-onset "unilateral RP" remains a difficult and uncertain diagnosis. ERG testing and longitudinal and widefield fundus examination are necessary to exclude asymmetrical disease. Although unilateral degeneration may exist in some children, its inherited or acquired etiology remains poorly understood.

  View details for DOI 10.1016/j.jaapos.2018.08.003

  View details for PubMedID 30243749

• Ophthalmic Technology Assessment: The Use of Beta-Blockers for the Treatment of Periocular Hemangiomas in Infants. Ophthalmology Hutchinson, A. K., Kraker, R. T., Pineles, S. L., VanderVeen, D. K., Wilson, L. B., Galvin, J. A., Lambert, S. R. 2018

  Abstract

  OBJECTIVE: To review the published literature assessing the efficacy of beta-blockers for the treatment of periocular hemangioma in infants.METHODS: Literature searches were conducted in May 2018 in PubMed with no date restrictions and limited to studies published in English and in the Cochrane Library database without any restrictions. The combined searches yielded 437 citations. Of these,16 articles were deemed appropriate for inclusion in this assessment and assigned a level of evidence rating by the panel methodologist.RESULTS: None of the 16 studies included in this assessment were rated level I, 3 were rated level II, and 13 were rated level III. The most common treatment regimen was 2 mg/kg daily oral propranolol, but intralesional and topical beta-blockers were also used. Treatment effect was most often measured in terms of reduction in the size of the lesions, which occurred in the majority of patients. Beta-blockers were consistently shown to reduce astigmatism, but this reduction was shown to be statistically significant in only 2 series. The effect of beta-blockers on amblyopia was not adequately documented. Beta-blockers were generally well tolerated and had mild side effects (fatigue, gastrointestinal upset/diarrhea, restlessness/sleep disturbances, minor wheezing, and cold extremities). Complications severe enough to require cessation of treatment occurred in only 2 patients out of a total of 229 who received beta-blockers.CONCLUSIONS: There is limited evidence to support the safety and efficacy of both topical and systemic beta-blockers to promote regression of periocular hemangiomas. Additional research may confirm the best dosage and route of administration to maximize efficacy in reducing induced astigmatism and amblyopia associated with periocular hemangiomas while minimizing side effects.

  View details for DOI 10.1016/j.ophtha.2018.07.023

  View details for PubMedID 30075202

• Correlation of monocular grating acuity at age 12 months with recognition acuity at age 4.5 years: findings from the Infant Aphakia Treatment Study. Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus Hartmann, E. E., Drews-Botsch, C., DuBois, L. G., Cotsonis, G., Lambert, S. R., Infant Aphakia Treatment Study Group 2018

  Abstract

  PURPOSE: To determine whether grating acuity at age 12 months can be used to predict recognition acuity at age 4.5 years in children treated for unilateral congenital cataract enrolled in the Infant Aphakia Treatment Study (IATS).METHODS: Traveling testers assessed monocular grating acuity at 12 months of age (Teller Acuity Card Test [TACT]) and recognition acuity at 4.5 years of age (Amblyopia Treatment Study Electronic Visual Acuity Testing, HOTV) in children treated for visually significant monocular cataract in the IATS. Spearman rank correlation was used to evaluate the relationship between visual acuities at the two ages in the treated eyes.RESULTS: Visual acuity data at both ages were available for 109 of 114 children (96%). Grating acuity at 12 months of age and recognition acuity at 4.5 years of age were significantly correlated for the treated eyes (rspearman = 0.45; P = 0.001). At age 4.5 years, 67% of the subjects who had grating acuity at 12 months of age within the 95% predictive limits in their treated eye demonstrated recognition acuity better than 20/200. Similarly, at age 4.5 years 67% of the subjects who had grating acuity at age 12 months below the 95% predictive limits in their treated eye demonstrated recognition acuity of 20/200 or worse.CONCLUSIONS: A single grating acuity assessment at age 12 months predicts recognition acuity in a child treated for unilateral congenital cataract in only two-thirds of cases. Clinicians should consider other factors, such as patching compliance and age at surgery, when using an early grating acuity assessment to modify treatment.

  View details for DOI 10.1016/j.jaapos.2018.03.011

  View details for PubMedID 30031874

• Management of Restrictive Esotropia Following Pterygium Surgery Muminovic, I., Toffoli, D., Lambert, S. R. ASSOC RESEARCH VISION OPHTHALMOLOGY INC. 2018

  View details for Web of Science ID 000442912508236

• Assessing Whether the Cataracts Associated With Atopic Dermatitis are Associated With Steroids or Inflammatory Factors. JAMA ophthalmology Lambert, S. R., Teng, J. M. 2018

  View details for DOI 10.1001/jamaophthalmol.2018.2153

  View details for PubMedID 29879288

• Global Practice Patterns in the Management of Infantile Cataracts. Eye & contact lens Koo, E. B., VanderVeen, D. K., Lambert, S. R. 2018

  Abstract

  OBJECTIVES: Surveys are an important tool to assess the impact of research on physicians' approach to patient care. This survey was conducted to assess current practice patterns in the management of infantile cataracts in light of the findings of the Infant Aphakia Treatment Study.METHODS: Pediatric ophthalmologists were emailed a link to the survey using newsletters from American Association of Pediatric Ophthalmology and Strabismus, World Society of Pediatric Ophthalmology and Strabismus, and the Pediatric Listserv. The 17-question survey was anonymous and active during July to August 2016.RESULTS: One hundred twenty-five respondents (North America, 65%; Asia, 12%; Europe, 9%; and other, 14%) reported operating on pediatric cataracts. Most practice in a university setting (55%). There was a strong consensus that unilateral cataract surgery should be performed between ages 4 to 6 weeks and aphakic contact lenses should be used to optically correct their eyes, particularly in children ≤6 months of age. For bilateral cataracts, there was a trend for surgeons to perform cataract surgery at an older age than unilateral cataract surgery. Surgeons who performed less than 5 versus greater than 20 pediatric cataract surgeries/year were more likely to use aphakic contact lenses in children undergoing cataract surgery more than 6 months of age (62% vs. 35%, P=0.04). Most respondents (73%) indicated that the Infant Aphakia Treatment Study had changed how they manage unilateral congenital cataracts.CONCLUSION: Most pediatric cataract surgeons perform congenital cataract surgery between ages 4 to 6 weeks and use aphakic contact lenses for initial optical correction in infants less than 6 months. Surgeons have equal preference for intraocular lenses and contact lenses in infants more than 6 months of age.

  View details for DOI 10.1097/ICL.0000000000000461

  View details for PubMedID 29369235

• Long-term follow-up after vertical extraocular muscle surgery to correct abnormal vertical head posture STRABISMUS Kumar, P., Lambert, S. R. 2018; 26 (3): 150–54

  Abstract

  To report outcomes of vertical extraocular muscle surgery to correct abnormal vertical head posture in children with horizontal infantile nystagmus.Retrospective case series of seven patients evaluated at one institution with abnormal vertical head position (chin-up or chin-down) in the setting of horizontal infantile nystagmus. All patients underwent bilateral vertical extraocular muscle surgery with the same surgeon. Pre- and postoperative vertical head position under binocular viewing conditions was the primary outcome measure. Secondary outcomes included visual acuity, stereopsis, degree of cyclotorsion, and residual ocular alignment.Seven children with infantile nystagmus syndrome, ranging in age from 4 months to 5 years at presentation, underwent extraocular muscle surgery to correct chin-down or chin-up head position associated with a null position in the setting of horizontal nystagmus. Five children had other ocular pathology (albinism, n = 4; cone-rod dystrophy, n = 1). Five of the seven patients had combined vertical recti and oblique muscle surgery. Three of the patients had additional extraocular muscle surgery to correct horizontal misalignment (exotropia, n = 2; esotropia, n = 1). One patient underwent a separate Kestenbaum procedure (bilateral horizontal resection/recession) to correct concomitant horizontal face turn. Median postoperative follow-up was 8.0 years (range 1-9.5 years). Postoperatively, three patients had complete resolution of their abnormal head position, and three had an improvement in their head posture, with a mean reduction in original vertical head position of 25 degrees. One of these patient had a reversal of their head position from a chin-up to a small chin-down position. Only one patient had no clinically significant improvement in their head position. No patients had signs or symptoms of cyclotorsion postoperatively.Combined vertical recti and oblique muscle surgery can successfully improve and even resolve vertical head posture, and prevents cyclorotary disorders postoperatively.

  View details for DOI 10.1080/09273972.2018.1497667

  View details for Web of Science ID 000442237800008

  View details for PubMedID 30060686

• Anisometropia at Age 5 Years after Unilateral Intraocular Lens Implantation During Infancy in the Infant Aphakia Treatment Study (IATS). American journal of ophthalmology Weakley, D., Cotsonis, G., Wilson, M. E., Plager, D. A., Buckley, E. G., Lambert, S. R. 2017

  Abstract

  To report the prevalence of anisometropia at age 5 years after unilateral intraocular lens (IOL) implantation in infants.Prospective randomized clinical trial METHODS: Fifty-seven infants in the Infant Aphakia Treatment Study (IATS) with a unilateral cataract were randomized to IOL implantation with an initial targeted postoperative refractive error of either +8D (infants 28 to <48 days of age) or +6D (infants 48-210 days of age). Anisometropia was calculated at age 5 years. Six patients were excluded from the analyses.Median age at cataract surgery was 2.2 months (IQR, 1.2, 3.5 months). The mean age at the age 5 year follow-up visit was 5.0 ± 0.1 years (range, 4.9 - 5.4 years). The median refractive error at the age 5 year visit of the treated eyes was -2.25 D (IQR -5.13, +0.88 D) and of the fellow eyes +1.50 D (IQR +0.88, +2.25). Median anisometropia was -3.50 D (IQR -8.25, -0.88 D); range (-19.63 to +2.75D). Patients with glaucoma in the treated eye (n=9) had greater anisometropia (glaucoma, median -8.25 D; IQR -11.38, -5.25 D vs. no glaucoma median -2.75; IQR -6.38, -0.75 D; p=0.005) CONCLUSIONS: The majority of pseudophakic eyes had significant anisometropia at age 5 years. Anisometropia was greater in patients that developed glaucoma. Variability in eye growth and myopic shift continue to make refractive outcomes challenging for IOL implantation during infancy.

  View details for DOI 10.1016/j.ajo.2017.05.008

  View details for PubMedID 28526552

• Anti-Vascular Endothelial Growth Factor Therapy for Primary Treatment of Type 1 Retinopathy of Prematurity: A Report by the American Academy of Ophthalmology. Ophthalmology VanderVeen, D. K., Melia, M., Yang, M. B., Hutchinson, A. K., Wilson, L. B., Lambert, S. R. 2017; 124 (5): 619-633

  Abstract

  To review the available evidence on the ocular safety and efficacy of anti-vascular endothelial growth factor (VEGF) agents for the treatment of retinopathy of prematurity (ROP) compared with laser photocoagulation therapy.A literature search of the PubMed and Cochrane Library databases was conducted last on September 6, 2016, with no date restrictions and limited to articles published in English. This search yielded 311 citations, of which 37 were deemed clinically relevant for full-text review. Thirteen of these were selected for inclusion in this assessment. The panel methodologist assigned ratings to the selected articles according to the level of evidence.Of the 13 citations, 6 articles on 5 randomized clinical trials provided level II evidence supporting the use of anti-VEGF agents, either as monotherapy or in combination with laser therapy. The primary outcome for these articles included recurrence of ROP and the need for retreatment (3 articles), retinal structure (2 articles), and refractive outcome (1 article). Seven articles were comparative case series that provided level III evidence. The primary outcomes included the effects of anti-VEGF treatment on development of peripheral retinal vessels (1 article), refractive outcomes (1 article), or both structural and refractive or visual outcomes (5 articles).Current level II and III evidence indicates that intravitreal anti-VEGF therapy is as effective as laser photocoagulation for achieving regression of acute ROP. Although there are distinct ocular advantages to anti-VEGF pharmacotherapy for some cases (such as eyes with zone I disease or aggressive posterior ROP), the disadvantages are that the ROP recurrence rate is higher, and vigilant and extended follow-up is needed because retinal vascularization is usually incomplete. After intravitreal injection, bevacizumab can be detected in serum within 1 day, and serum VEGF levels are suppressed for at least 8 to 12 weeks. The effects of lowering systemic VEGF levels on the developing organ systems of premature infants are unknown, and there are limited long-term data on potential systemic and neurodevelopmental effects after anti-VEGF use for ROP treatment. Anti-VEGF agents should be used judiciously and with awareness of the known and unknown or potential side effects.

  View details for DOI 10.1016/j.ophtha.2016.12.025

  View details for PubMedID 28341474

• Outcomes after superior rectus transposition and medial rectus recession versus vertical recti transposition for sixth nerve palsy. American journal of ophthalmology Lee, Y., Lambert, S. R. 2017

  Abstract

  To compare the effectiveness of superior rectus transposition and medial rectus recession (SRT/MRc) vs inferior and superior rectus transposition (VRT) for acquired sixth nerve palsy.Consecutive, interventional case series.The medical records of a consecutive series of patients with acquired sixth nerve palsy who underwent VRT or SRT/MRc by a single surgeon were reviewed. The preoperative and postoperative findings were compared between the 2 groups.Eight patients (mean age, 46.8 years) underwent SRT/MRc and 8 patients underwent VRT (mean age, 51.1 years). Lateral fixation was performed on all but 4 patients in the VRT group. Preoperative esotropia in primary position and abduction deficit were similar in both groups (SRT/MRc, 41.9 prism diopter [PD], -4.6; VRT, 55.6 PD, -4.5; P = .195, 1.0). The SRT/MRc group underwent a mean MR recession of 6 (range, 5-7) mm. Four patients in the VRT later underwent MR recession (mean 5.3 mm, range 5-6 mm). In addition, 5 patients in the VRT group had 1 or more botulinum toxin injections in the medial rectus muscle. No additional procedures were performed in the SRT/MR group. Fewer additional procedures were performed with SRT/MR (SRT/MR, 0; VRT, 1.8 ± 1.2; P < .010). At last follow-up, residual esotropia (SRT/MRc, 7.1 PD; VRT, 10.3 PD; P = .442) was similar in both groups, but abduction was better in the SRT/MRc group (SRT/MR, -3.0 ± 0.7; VRT, -3.8 ± 0.4; P = .038). There were no new persistent vertical deviations or torsional diplopia.Final outcomes were similar with SRT/MRc vs VRT. However, fewer additional surgical procedures were needed with SRT/MR.

  View details for DOI 10.1016/j.ajo.2017.02.019

  View details for PubMedID 28249714

• Myopic Shift 5 Years after Intraocular Lens Implantation in the Infant Aphakia Treatment Study. Ophthalmology Weakley, D. R., Lynn, M. J., DuBois, L., Cotsonis, G., Wilson, M. E., Buckley, E. G., Plager, D. A., Lambert, S. R. 2017

  Abstract

  To report the myopic shift at 5 years of age after cataract surgery with intraocular lens (IOL) implantation for infants enrolled in the Infant Aphakia Treatment Study (IATS).Refractions were performed at 1 month and every 3 months postoperatively until age 4 years and then at ages 4.25, 4.5, and 5 years. The change in refraction over time was estimated by linear mixed model analysis.Intraocular lens implantation was completed in 56 eyes; 43 were analyzed (median age, 2.4 months; range, 1.0-6.8 months). Exclusions included 11 patients with glaucoma, 1 patient with Stickler syndrome, and 1 patient with an IOL exchange at 8 months postoperatively. The mean rate of change in a myopic direction from 1 month after cataract surgery to age 1.5 years was 0.35 diopters (D)/month (95% confidence interval [CI], 0.29-0.40 D/month); after age 1.5 years, the mean rate of change in a myopic direction was 0.97 D/year (95% CI, 0.66-1.28 D/year). The mean refractive change was 8.97 D (95% CI, 7.25-10.68 D) at age 5 years for children 1 month of age at surgery and 7.22 D (95% CI, 5.54-8.91 D) for children 6 months of age at surgery. The mean refractive error at age 5 years was -2.53 D (95% CI, -4.05 to -1.02).After IOL implantation during infancy, the rate of myopic shift occurs most rapidly during the first 1.5 years of life. Myopic shift varies substantially among patients. If the goal is emmetropia at age 5 years, then the immediate postoperative hypermetropic targets should be +10.5 D at 4 to 6 weeks and +8.50 D from 7 weeks to 6 months. However, even using these targets, it is likely that many children will require additional refractive correction given the high variability of refractive outcomes.

  View details for DOI 10.1016/j.ophtha.2016.12.040

  View details for PubMedID 28215452

• Globe Axial Length Growth at Age 5 Years in the Infant Aphakia Treatment Study. Ophthalmology Wilson, M. E., Trivedi, R. H., Weakley, D. R., Cotsonis, G. A., Lambert, S. R. 2017

  Abstract

  To report the longitudinal change in axial length (AL) from the time of unilateral cataract surgery at age 1 to 7 months to age 5 years, and to compare AL growth of operated eyes with that of fellow unoperated eyes.Comparative case series.Infants enrolled in the Infant Aphakia Treatment Study (IATS).The AL at baseline and age 5 years and change in AL were analyzed relative to treated versus fellow eye, visual outcome, and treatment modality (contact lens [CL] vs. intraocular lens [IOL]). Eyes with glaucoma or glaucoma suspect were excluded from primary analysis but reported separately.The AL growth from preoperative to age 5 years.Seventy patients were eligible; however, AL data for both eyes were available for 64 patients at baseline and 69 patients at age 5 years. The AL was significantly different between treated and fellow eyes preoperatively (18.1 vs. 18.7 mm, P < 0.0001) and at the final follow-up (21.4 vs. 22.1 mm, P = 0.0004). The difference in AL growth between treated and fellow eyes was not significant (3.3 vs. 3.5 mm, P = 0.31). The change in AL in eyes was similar with both treatments (CL 3.2 mm and IOL 3.4 mm, P = 0.53) and did not correlate with visual outcomes (P = 0.85). Eyes receiving additional surgery to clear the visual axis opacification grew significantly more compared with eyes not receiving surgery to clear the visual axis (3.8 vs. 2.7 mm, P = 0.013). Patients with glaucoma showed significantly more eye growth (5.7 mm) than those without glaucoma (3.3 mm) and glaucoma suspects (4.3 mm).Eyes treated for monocular cataract in infancy have axial growth similar to that of fellow eyes, despite having a shorter AL at the time of surgery. The change in AL in eyes was similar with both treatments (CL and IOL), did not correlate with visual outcomes, and was higher in eyes receiving additional surgery to clear the visual axis or eyes diagnosed with glaucoma.

  View details for DOI 10.1016/j.ophtha.2017.01.010

  View details for PubMedID 28196730

• Comparison of the rate of refractive growth in aphakic eyes versus pseudophakic eyes in the Infant Aphakia Treatment Study JOURNAL OF CATARACT AND REFRACTIVE SURGERY Lambert, S. R., Cotsonis, G., DuBois, L., Wilson, M. E., Plager, D. A., Buckley, E. G., McClatchey, S. K. 2016; 42 (12): 1768-1773

  Abstract

  To compare the rate of refractive growth (RRG) between aphakic eyes and pseudophakic eyes in the Infant Aphakia Treatment Study (IATS).Twelve clinical sites across the United States.Randomized clinical trial.Patients randomized to unilateral cataract extraction with contact lens correction versus intraocular lens (IOL) implantation in the IATS had their rate of refractive growth (RRG3) calculated based on the change in refraction from the 1-month postoperative examination to age 5 years. The RRG3 is a logarithmic formula designed to calculate the RRG in children. Two-group t tests were used to compare the mean refractive growth between the contact lens group and IOL group and outcomes based on age at surgery and visual acuity.Longitudinal refractive data were studied for 108 of 114 patients enrolled in the IATS (contact lens group, n = 54; IOL group, n = 54). The mean RRG3 was similar in the contact lens group (-18.0 diopter [D] ± 11.0 [SD]) and the IOL group (-19.0 ± 9.0 D) (P = .49). The RRG3 value was not correlated with age at cataract surgery, glaucoma status, or visual outcome in the IOL group. In the aphakia group, only visual outcome was correlated with refractive growth (P = .01).Infants' eyes had a similar rate of refractive growth after unilateral cataract surgery whether or not an IOL was implanted. A worse visual outcome was associated with a higher RRG in aphakic, but not pseudophakic, eyes.None of the authors has a financial or proprietary interest in any material or method mentioned.

  View details for DOI 10.1016/j.jcrs.2016.09.021

  View details for Web of Science ID 000391518100011

  View details for PubMedID 28007108

• Difluprednate versus prednisolone acetate for inflammation following cataract surgery in pediatric patients: a randomized safety and efficacy study. Eye (London, England) Wilson, M. E., Lambert, S. R., Plager, D. A., Vanderveen, D., Roarty, J., O'Halloran, H. 2016

  View details for DOI 10.1038/eye.2016.244

  View details for PubMedID 27813524

• Strabismus surgery outcomes in the Infant Aphakia Treatment Study (IATS). Journal of AAPOS Bothun, E. D., Lynn, M. J., Christiansen, S. P., Kruger, S. J., VanderVeen, D. K., Neely, D. E., Lambert, S. R. 2016

  Abstract

  To report strabismus surgery frequency and outcomes after monocular infantile cataract surgery with or without IOL implantation.The Infant Aphakia Treatment Study (IATS) is a randomized, multicenter clinical trial comparing treatment of aphakia with a primary IOL or contact lens in 114 infants with a unilateral congenital cataract. This report is a secondary outcome analysis of ocular motor data from IATS patients who underwent strabismus surgery prior to age 5 years.Strabismus surgery was performed in 45 (39%) patients (contact lens group [CL], 37%; IOL group, 42% [P = 0.70]). The indications for strabismus surgery were esotropia (62%), exotropia (33%), and hypertropia (4%). Infants who underwent cataract surgery at a younger age were less likely to undergo strabismus surgery (28-48 days, 12/50 [24%]; 49-210 days, 33/64 [52%]; P = 0.0037). Of the 42 patients who underwent strabismus surgery, 14 (33%) had a postoperative distance alignment within 8(Δ) of orthotropia at age 5 years. The 5-year visual acuity of children with strabismus was the same whether or not strabismus surgery had been performed (1.10 logMAR with surgery vs 1.00 without [P = 0.71]).In this study cohort, cataract surgery performed in the first 6 weeks of life was associated with a reduced frequency of strabismus surgery. Strabismus surgery outcomes in this population are guarded. Surgical improvement of strabismus does not appear to influence long-term visual acuity.

  View details for DOI 10.1016/j.jaapos.2016.09.014

  View details for PubMedID 27815186

• Factors associated with stereopsis and a good visual acuity outcome among children in the Infant Aphakia Treatment Study. Eye (London, England) Lambert, S. R., Dubois, L., Cotsonis, G., Hartmann, E. E., Drews-Botsch, C. 2016; 30 (9): 1221-1228

  Abstract

  PurposeTo identify factors associated with stereopsis in children with good visual acuity after unilateral congenital cataract surgery in the Infant Aphakia Treatment Study.Patients and methodsInfants with a unilateral congenital cataract (n=114) were randomized to IOL implantation or contact lens correction after cataract surgery. At age 4.5 years, a masked examiner assessed HOTV acuity and stereopsis. Adherence to patching was assessed using 48-h recall telephone interviews and 7-day diaries throughout the first 5 years of life. Ocular motility was evaluated at age 5 years. Baseline, postoperative, and adherence findings were compared between patients with 20/40 or better acuity in their treated eyes with or without stereopsis.ResultsThirty (27%) of 112 patients who were evaluated at age 4.5 years had 20/40 or better acuity in their treated eye. Stereopsis was present on one or more tests in 15 of these 30 (50%) children. Baseline characteristics and postoperative findings did not differ between patients with or without stereopsis. Children with stereopsis were more likely to be orthotropic at distance (P=0.003) and were patched for fewer hours per day throughout the first 5 years of life and the difference increased over time (P<0.001). On average children with stereopsis were patched for 3.4 h/day during the first year of life and patching steadily decreased to 1.8 h/day by age 4 years.ConclusionAmong children with good vision following unilateral congenital cataract surgery, orthophoria and fewer hours of patching, particularly during years 2, 3, and 4, are associated with some evidence of stereopsis.

  View details for DOI 10.1038/eye.2016.164

  View details for PubMedID 27472216

• Difluprednate versus prednisolone acetate for inflammation following cataract surgery in pediatric patients: a randomized safety and efficacy study. Eye (London, England) Wilson, M. E., O'Halloran, H., Vanderveen, D., Roarty, J., Plager, D. A., Markwardt, K., Gedif, K., Lambert, S. R. 2016; 30 (9): 1187-1194

  Abstract

  PurposeTo evaluate safety and efficacy of difluprednate 0.05% ophthalmic emulsion for treatment of postoperative inflammation after cataract surgery in pediatric patients.MethodsThis was a phase 3B, multicentre, randomized, double-masked, active-controlled study of patients aged 0-3 years who underwent uncomplicated cataract surgery in one eye, with/without intraocular lens implantation. Patients were randomized to receive difluprednate 0.05% four times daily or prednisolone acetate 1% for 14 days post surgery, followed by tapering for 14 days. Safety included evaluation of adverse events. Primary efficacy was the proportion of patients with an anterior cell grade of 0 (no cells) at day 14; secondary efficacy was a global inflammation score.ResultsForty patients were randomized to each treatment group. Adverse drug reactions included corneal oedema (difluprednate 0.5%, n=1; prednisolone acetate 1%, n=0) and increased intraocular pressure or ocular hypertension (n=2/group). Mean intraocular pressure values during treatment were 2-3 mm Hg higher with difluprednate 0.05% compared with prednisolone acetate 1%; mean values were similar between groups by the first week after treatment cessation. At 2 weeks post surgery, the incidence of complete clearing of anterior chamber cells was similar between groups (difluprednate 0.05%, n=30 (78.9%); prednisolone acetate 1%, n=31 (77.5%). Compared with prednisolone acetate 1%, approximately twice as many difluprednate 0.05%-treated patients had a global inflammation assessment score indicating no inflammation on day 1 (n=12 (30.8%) vs n=7 (17.5%) and day 8 (n=18 (48.7%) vs n=10 (25.0%).ConclusionsDifluprednate 0.05% four times daily showed safety and efficacy profiles similar to prednisolone acetate 1% four times daily in children 0-3 years undergoing cataract surgery.

  View details for DOI 10.1038/eye.2016.132

  View details for PubMedID 27367745

• Associated systemic and ocular disorders in patients with congenital unilateral cataracts: the Infant Aphakia Treatment Study experience. Eye (London, England) Traboulsi, E. I., Vanderveen, D., Morrison, D., Drews-Botsch, C. D., Lambert, S. R. 2016; 30 (9): 1170-1174

  Abstract

  PurposeFive-year prospective data on children enrolled in the Infant Aphakia Treatment Study (IATS) provided an opportunity to explore ocular and systemic associations in patients with a unilateral congenital cataract.MethodsInfants <7 months of age with a unilateral cataract were eligible for IATS screening. We reviewed data pertaining to the exclusion of patients as well as data collected on standardized study forms used at any time for documentation of ocular or systemic disorders.ResultsOverall, 227 infants were referred for possible enrollment. Of these, 10 had insignificant cataracts and 32 refused to participate. Of those excluded, 3 were premature, 27 had significant ocular disease (usually persistent fetal vasculature (PFV) or corneal diameter <9 mm), and 4 had systemic disorders. An additional 26 were excluded at the time of the first EUA, most often because of PFV or variants thereof. On follow-up, in the 114 enrolled patients, the following disorders were diagnosed: Stickler syndrome (1), mitochondrial disease (1), autism (1), and presumed congenital rubella syndrome (1). No patient developed a cataract in the fellow eye.DiscussionSome conditions that can feature unilateral cataracts are diagnosed at birth or very early in life, but others may be diagnosed at varying periods thereafter. PFV and its variants are the most common associated ocular findings in about a quarter of cases of unilateral congenital cataracts.ConclusionAlthough patients with a unilateral cataract may have significant associated abnormalities in the affected eye, most commonly PFV and its variants, the prevalence of associated significant systemic disease is quite low.

  View details for DOI 10.1038/eye.2016.124

  View details for PubMedID 27315350

• Assessment of Adherence to Visual Correction and Occlusion Therapy in the Infant Aphakia Treatment Study. Contemporary clinical trials communications Drews-Botsch, C., Cotsonis, G., Celano, M., Lambert, S. R. 2016; 3: 158-166

  Abstract

  Occlusion therapy throughout early childhood is believed to be efficacious in treating deprivation amblyopia but has not been rigorously assessed in clinical trials. Further, tools to assess adherence to such therapy over an extended period of time are lacking. Using data from the Infant Aphakia Treatment Study, a randomized clinical trial of treatment for unilateral congenital cataract, we examined the use of quarterly 48-hour recall interviews and annual 7-day prospective diaries to assess reported hours of patching in 114 children throughout the first 5 years of life. Consistency of data reported was assessed using correlation coefficients and intraclass correlation coefficients. Both interview and diary data showed excellent consistency with Cronbach's Alpha's ranging from 0.69 to 0.88 for hours of patching and 0.60 to 0.73 for hours of sleep. However, caregivers reported somewhat more adherence in prospective diaries than retrospective interviews. Completion rates, on the other hand, were substantially higher for telephone interviews than prospective diaries. For example, after four years of surgery response rates to telephone interviews exceeded 75% versus completion rates of only 54% for diaries. In situations where occlusion dose monitors cannot be used for assessing adherence to occlusion therapy, such as in infants or over an extended period of time, quantitative assessments of occlusion therapy can be obtained by parental report, either as a series of prospective diaries or a series of recall interviews.

  View details for PubMedID 27981259

• Association Between Occlusion Therapy and Optotype Visual Acuity in Children Using Data From the Infant Aphakia Treatment Study: A Secondary Analysis of a Randomized Clinical Trial. JAMA ophthalmology Drews-Botsch, C., Celano, M., Cotsonis, G., Hartmann, E. E., Lambert, S. R. 2016; 134 (8): 863-869

  Abstract

  Patching has been a mainstay in treating unilateral congenital cataract. However, its efficacy has not been rigorously assessed.To examine the association between patching and visual acuity in a cohort of children treated for unilateral congenital cataract.This study was a secondary analysis of a randomized clinical trial (Infant Aphakia Treatment Study) of infants born from August 1, 2004, through December 31, 2008, who were treated with 1 of 2 treatments for unilateral congenital cataract and followed up to 5 years of age. Data analysis was performed from March 1, 2013, to March 1, 2016.Cataract extraction and randomization to receipt of an intraocular lens vs being left aphakic for the first 5 years of life.Caregivers reported patching in the previous 48 hours in quarterly semistructured telephone interviews. The mean number of hours of patching per day was calculated from surgery to the first birthday (n = 92) and between 12 and 48 months of age (n = 102). Monocular optotype acuity was assessed at 4½ years of age by a traveling examiner using the Aphakia Treatment Study HOTV protocol.The Infant Aphakia Treatment Study enrolled 114 children; 57 were randomized to each treatment group. At 4½ years of age, optotype visual acuity was assessed in 112 children. The current analyses exclude an additional 3 children (2 who had adverse events that limited visual potential and 1 who had Stickler syndrome), leaving 109 total children analyzed (59 female [54.1%] and 92 white [84.4%]). Caregivers reported patching their children a mean (SD) of 3.73 (1.47) hours per day in the first year of life and 3.43 (2.04) hours per day thereafter. An association between reported patching and treatment was not identified (mean difference in first year, -0.29 hours per day; 95% CI, -0.90 to 0.33 hours per day; mean difference between 12 and 48 months of age, -0.40 hours per day; 95% CI, -1.20 to 0.40 hours per day). Visual acuity was associated with reported hours of patching in the first year of life (r = -0.32; 95% CI, -0.49 to -0.13) and between 12 and 48 months of age (r = -0.36; 95% CI, -0.52 to -0.18). However, patching accounted for less than 15% of the variance in logMAR acuity at 4½ years of age.These results support the association of occlusion throughout the preschool years with improved visual acuity in infants treated for unilateral congenital cataract. However, similar visual outcomes were achieved with varying amounts of patching. These conclusions should be interpreted in the context of limitations related to generalizability from incomplete data collected in a clinical trial.clinicaltrials.gov Identifier: NCT00212134.

  View details for DOI 10.1001/jamaophthalmol.2016.1365

  View details for PubMedID 27228110

• Cataract secondary to self-inflicted blunt trauma in children with autism spectrum disorder. Journal of AAPOS Lee, Y., Lenhart, P. D., Lambert, S. R. 2016; 20 (4): 361-362

  Abstract

  We report 3 cases of bilateral cataract secondary to self-inflicted blunt eye trauma in children with autism spectrum disorder (ASD). All 3 children hit their foreheads, orbits, or globes repeatedly for long periods of time and developed cataracts. Clinicians must be aware of this phenomenon to diagnose ocular pathology early and to provide adequate education, counseling, and services to affected patients and their families and to put appropriate postoperative care mechanisms in place to prevent permanent ocular damage.

  View details for DOI 10.1016/j.jaapos.2016.02.013

  View details for PubMedID 27223635

• The Infant Aphakia Treatment Study Contact Lens Experience to Age of 5 Years. Eye & contact lens Russell, B., DuBois, L., Lynn, M., Ward, M. A., Lambert, S. R. 2016: -?

  Abstract

  To describe our experience treating a cohort of unilateral aphakic infants with contact lenses in the Infant Aphakia Treatment Study (IATS).Fifty-seven of the 114 infants in the IATS were randomized to contact lens wear; all were followed until age 5 years, although a few had lapses in care. An examination under anesthesia, including keratometry, was performed at the time of enrollment and at approximately 1 year of age; keratometry was performed again at 5 years of age. A traveling examiner assessed visual acuity at approximately 1 year of age and again at 4.5 years of age.Twenty-four treated eyes (46%) wore silicone elastomer (SE) contact lenses, 11 eyes (19%) rigid gas permeable (GP) contact lenses and 17 eyes (29%) wore both lens types at various points of time. Median logMAR visual acuity was +0.70 (interquartile range (IQR), +0.30 to 1.20) in the SE group and 2.03 (IQR, +0.20 to 2.28) in the GP group at age 4.5 years. The mean (±SD) keratometric power of the treated eyes was 46.3±2.8 diopter (D) at baseline, 44.6±2.3 D at 1 year of age, and 44.3±1.7 D at 5 years of age. Keratometric astigmatism of treated eyes was 1.98±1.37 D at baseline, 1.62±0.98 D at 1 year of age, and 2.00±1.00 D at 5 years of age. Thirteen contact lens-related adverse events occurred among 7 patients after age 1 year.A cohort of infants with unilateral aphakia successfully wore contact lenses with relatively few adverse events.

  View details for PubMedID 27466719

• MULTIMODAL IMAGING OF A FAMILY WITH SPINOCEREBELLAR ATAXIA TYPE 7 DEMONSTRATING PHENOTYPIC VARIATION AND PROGRESSION OF RETINAL DEGENERATION. Retinal cases & brief reports Levinson, J. D., Yan, J., Lambert, S. R., Shankar, S. P. 2016; 10 (3): 267-272

  Abstract

  To report the variability and progression of clinical presentation in three family members with spinocerebellar ataxia Type 7 including early recognizable features on retinal imaging and magnetic resonance imaging.Retrospective case series.The proband, Patient 1 (mother) presented at age 26 with light perception vision. Initial examination was significant for optic disc pallor, vascular attenuation, and central macular atrophy. Two years later, her vision declined to no light perception, and fundus examination demonstrated marked progression of macular atrophy and peripheral bone spicule formation. Seven years after the onset of vision loss, neurologic examination demonstrated ataxia, dysarthria, and slowed saccades. Genetic testing of ATXN7 identified heterozygous 61-CAG trinucleotide repeat expansion confirming the diagnosis of spinocerebellar ataxia Type 7. Patient 2 (son) presented at age 11 with visual acuity of 20/300 bilaterally and decreased color vision. Funduscopic examination was notable for disc pallor, vascular attenuation, and peripheral pigmentary changes. Electroretinography demonstrated diminished rod and cone function, and Goldmann visual field testing revealed paracentral scotoma. Patient 3 (daughter) presented at age 14 with visual acuity of 20/50 bilaterally and minimal funduscopic changes. The only significant ophthalmic finding was retinal thinning with atrophy of the outer nuclear layer and subfoveal ellipsoid zone on optical coherence tomography. Early cerebellar volume loss was also noted on magnetic resonance imaging.The clinical presentation of spinocerebellar ataxia Type 7 can vary widely even within the same family. In individuals with vision loss and normal fundus examination, careful evaluation of optical coherence tomography and brain magnetic resonance imaging facilitates early diagnosis and genetic testing.

  View details for DOI 10.1097/ICB.0000000000000248

  View details for PubMedID 26584329

• The timing of surgery for congenital cataracts Minimizing the risk of glaucoma following cataract surgery while optimizing the visual outcome JOURNAL OF AAPOS Lambert, S. R. 2016; 20 (3): 191-192

  View details for DOI 10.1016/j.jaapos.2016.04.003

  View details for Web of Science ID 000378186200001

  View details for PubMedID 27180288

• Use of the Delphi process in paediatric cataract management BRITISH JOURNAL OF OPHTHALMOLOGY Serafino, M., Trivedi, R. H., Levin, A. V., Wilson, M. E., Nucci, P., Lambert, S. R., Nischal, K. K., Plager, D. A., Bremond-Gignac, D., Kekunnaya, R., Nishina, S., Tehrani, N. N., Ventura, M. C. 2016; 100 (5): 611-615

  Abstract

  To identify areas of consensus and disagreement in the management of paediatric cataract using a modified Delphi approach among individuals recognised for publishing in this field.A modified Delphi method.International paediatric cataract experts with a publishing record in paediatric cataract management.The process consisted of three rounds of anonymous electronic questionnaires followed by a face-to-face meeting, followed by a fourth anonymous electronic questionnaire. The executive committee created questions to be used for the electronic questionnaires. Questions were designed to have unit-based, multiple choice or true-false answers. The questionnaire included issues related to the preoperative, intraoperative and postoperative management of paediatric cataract.Consensus based on 85% of panellists being in agreement for electronic questionnaires or 80% for the face-to-face meeting, and near consensus based on 70%.Sixteen of 22 invited paediatric cataract surgeons agreed to participate. We arrived at consensus or near consensus for 85/108 (78.7%) questions and non-consensus for the remaining 23 (21.3%) questions.Those questions where consensus was not reached highlight areas of either poor evidence or contradicting evidence, and may help investigators identify possible research questions.

  View details for DOI 10.1136/bjophthalmol-2015-307287

  View details for Web of Science ID 000375333000007

  View details for PubMedID 26420824

• Evaluating the evidence for and against the use of IOLs in infants and young children EXPERT REVIEW OF MEDICAL DEVICES Kumar, P., Lambert, S. R. 2016; 13 (4): 381-389

  Abstract

  Congenital cataracts account for 5-20% of childhood blindness worldwide. In the US, the prevalence of visually significant infantile cataracts is anywhere from 3-4 per 10,000 live births. Infantile cataracts need to be removed early in life in order to prevent the onset of deprivation amblyopia. As a result, cataract surgery is usually performed between age 4-8 weeks depending on the laterality and severity of the cataract. Given advances in the field, pediatric cataract surgery is now a safe and effective intervention for infants, but good visual outcomes require occlusion therapy and optical correction. This review will address current perspectives on the use of intraocular lenses to optically correct infants and young children after cataract surgery, as well as novel designs for intraocular lenses and directions for future research.

  View details for DOI 10.1586/17434440.2016.1153967

  View details for Web of Science ID 000372805000002

  View details for PubMedID 26878234

• Diffuse bilateral retinal hemorrhages in an infant with a coagulopathy and prolonged cardiopulmonary resuscitation JOURNAL OF AAPOS Levinson, J. D., Pasquale, M. A., Lambert, S. R. 2016; 20 (2): 166-168

  Abstract

  We report a case of diffuse bilateral retinal and optic nerve sheath hemorrhages in an 8-week-old boy who was found unresponsive. The child underwent prolonged cardiopulmonary resuscitation and was noted on admission to have a coagulopathy. An autopsy determined the cause of death to be a myocardial infarct in the distribution of an anomalous coronary artery. This case demonstrates the difficulty that may occur in establishing whether child abuse caused death in the setting of another potential cause of mortality.

  View details for DOI 10.1016/j.jaapos.201.5.11.003

  View details for Web of Science ID 000374620500016

  View details for PubMedID 26988775

• Clinical Models and Algorithms for the Prediction of Retinopathy of Prematurity A Report by the American Academy of Ophthalmology OPHTHALMOLOGY Hutchinson, A. K., Melia, M., Yang, M. B., VanderVeen, D. K., Wilson, L. B., Lambert, S. R. 2016; 123 (4): 804-816

  Abstract

  To assess the accuracy with which available retinopathy of prematurity (ROP) predictive models detect clinically significant ROP and to what extent and at what risk these models allow for the reduction of screening examinations for ROP.A literature search of the PubMed and Cochrane Library databases was conducted last on May 1, 2015, and yielded 305 citations. After screening the abstracts of all 305 citations and reviewing the full text of 30 potentially eligible articles, the panel members determined that 22 met the inclusion criteria. One article included 2 studies, for a total of 23 studies reviewed. The panel extracted information about study design, study population, the screening algorithm tested, interventions, outcomes, and study quality. The methodologist divided the studies into 2 categories-model development and model validation-and assigned a level of evidence rating to each study. One study was rated level I evidence, 3 studies were rated level II evidence, and 19 studies were rated level III evidence.In some cohorts, some models would have allowed reductions in the number of infants screened for ROP without failing to identify infants requiring treatment. However, the small sample size and limited generalizability of the ROP predictive models included in this review preclude their widespread use to make all-or-none decisions about whether to screen individual infants for ROP. As an alternative, some studies proposed approaches to apply the models to reduce the number of examinations performed in low-risk infants.Additional research is needed to optimize ROP predictive model development, validation, and application before such models can be used widely to reduce the burdensome number of ROP screening examinations.

  View details for DOI 10.1016/j.ophtha.2015.11.003

  View details for Web of Science ID 000372718300025

  View details for PubMedID 26832657

• Should Glasses Be Prescribed for All Children with Moderate Hyperopia? OPHTHALMOLOGY Lambert, S. R. 2016; 123 (4): 676-678

  View details for DOI 10.1016/j.ophtha.2015.12.035

  View details for Web of Science ID 000372718300009

  View details for PubMedID 27012695

• Sensorimotor outcomes by age 5 years after monocular cataract surgery in the Infant Aphakia Treatment Study (IATS) JOURNAL OF AAPOS Bothun, E. D., Lynn, M. J., Christiansen, S. P., Neely, D. E., VanderVeen, D. K., Kruger, S. J., Lambert, S. R. 2016; 20 (1): 49-53

  Abstract

  To evaluate sensorimotor outcomes among children in the Infant Aphakia Treatment Study (IATS).Secondary outcome analysis was performed in this randomized, multicenter, clinical trial comparing treatment of unilateral aphakia with a primary intraocular lens (IOL) or contact lens (CL) correction. The alignment characteristics and sensory status of children through age 5 years were evaluated.In the IATS study, 91 of 112 children (81%) developed strabismus through age 5 years. Of 34 infants who were orthotropic at near 12 months after cataract surgery, at age 5 years 14 (41%) were orthotropic at distance, and 15 (44%) were orthotropic at near at age 5 years without strabismus surgery. Eight of 56 children (14%) in the CL group and 13 of 56 (23%) in the IOL group were orthotropic at distance (P = 0.33) at 5 years of age and had no history of strabismus surgery. Thirteen of 48 (27%) who underwent cataract surgery prior to 49 days of age compared to 8 of 64 (13%) who had surgery after 49 days were orthotropic (P = 0.085). Median visual acuity in the operative eye was 0.4 logMAR (20/50) for children with orthotropia or microtropia (<10(Δ)) versus 1.10 logMAR (20/252) for strabismus ≥10(Δ) (P = 0.0001). Stereopsis was detected in 12 of 21 children (57%) with orthotropia versus 16 of 89 (18%) children with strabismus (P = 0.0006).IOL placement does not reduce the development of strabismus after monocular congenital cataract surgery. Improved ocular alignment by age 5 years correlated strongly with improved visual acuity and stereopsis.

  View details for DOI 10.1016/j.jaapos.2015.11.002

  View details for Web of Science ID 000371187200011

  View details for PubMedID 26917072

• Improved Vision and Contact Lens Wear Time With Piggy-Back Contact Lens Systems in Children After Penetrating Corneal Trauma. Eye & contact lens Cromelin, C., Russell, B., Lambert, S. R. 2016: -?

  Abstract

  Consecutive case series of children treated successfully with "piggy-back" (PB) contact lens systems after corneal trauma.We reviewed the medical record of all children ages 4 to 14 years treated at the Emory Eye Center between January 11, 2003 and January 11, 2013 with PB contact lens systems.Four children with a history of corneal penetrating trauma were treated with a PB lens system, with a mean age of 7±0.08 (range: 6-8) years. Best-corrected spectacle vision was count fingers in two children and logMAR +0.70 (Snellen equivalent 20/100) and logMAR +0.6 (Snellen equivalent 20/80) in the remaining two. The PB lens system was introduced with a mean of 15.7±6.5 (range: 9-22) months after the injury. All patients were initially fitted with gas-permeable (GP) lenses. Each child achieved 11 or more hours of daily contact lens wear time in PB systems. The mean best-corrected logMAR visual acuity using the PB system was 0.26±0.21 (Snellen equivalent 20/36). The mean improvement in best-corrected logMAR between GP and PB lens systems was +0.21±0.11, which corresponds to an improvement of greater than two lines on the Snellen chart.Piggy-back contact lens systems can be helpful to improve vision and contact lens wearing time in children with irregular astigmatism after corneal trauma, who are intolerant of GP contact lenses.

  View details for PubMedID 26808698

  View details for PubMedCentralID PMC4958039

• Changes in Ocular Growth after Pediatric Cataract Surgery. Developments in ophthalmology Lambert, S. R. 2016; 57: 29-39

  Abstract

  The human eye undergoes extensive changes during early childhood, including axial elongation, corneal flattening and reduced lens power. Animal studies have shown that removing the crystalline lens during infancy retards axial elongation. Axial elongation has been studied in children after cataract extraction both directly and indirectly. Children with a unilateral congenital cataract generally have a shorter axial length in their cataractous eye than in their fellow eye. This difference usually persists after cataract surgery. While some studies have reported a modest reduction in axial elongation after cataract extraction, the magnitude of this effect is much less than what has been reported in animal models. Choosing an intraocular lens (IOL) power for implantation into a child's eye is complicated by continued ocular growth, the inaccuracy of IOL power calculation formulas for small eyes, and the difficulty of accurately measuring the biometrics of a child's eye. In addition, given the fixed position of an IOL in the eye, increasing elongation of the posterior segment of the eye relative to the anterior segment magnifies the myopic shift that occurs with ocular growth. The targeted refractive error in young children undergoing IOL implantation should be an undercorrection in anticipation of a future myopic shift.

  View details for DOI 10.1159/000442498

  View details for PubMedID 27043390

• Corneal Changes in Children after Unilateral Cataract Surgery in the Infant Aphakia Treatment Study OPHTHALMOLOGY Morrison, D. G., Lynn, M. J., Freedman, S. F., Orge, F. H., Lambert, S. R. 2015; 122 (11): 2186-2192

  Abstract

  We report endothelial cell (EC) characteristics and central corneal thickness (CCT) from the Infant Aphakia Treatment Study (IATS) patients at the 5-year examination.Randomized, controlled trial of the treatment of unilateral cataract with aphakic contact lens (CL) versus primary intraocular lens (IOL) implant.A total of 114 infants with unilateral cataract.The EC density, coefficient of variation (CV), and percent hexagonal cells were measured by noncontact specular microscopy. The CCT was measured using contact pachymetry. Fellow eyes served as controls.Mean differences between treated and fellow eyes of CL and IOL groups were compared with a paired t test. A 1-way analysis of variance model and the Tukey-Kramer multiple comparison procedure were used to assess the effect of a diagnosis of glaucoma or glaucoma suspect.A total of 105 subjects (52 with CLs, 53 with IOLs) had specular microscopy or corneal thickness data recorded. Mean EC densities were higher in aphakic eyes compared with fellow eyes (3921 vs. 3495 cells/mm2, P<0.0001). Mean CV was higher (27 vs. 24, P=0.0002) and mean percent hexagonal cells was lower (72% vs. 76%, P=0.002) in aphakic eyes compared with fellow eyes. Mean CCT of aphakic eyes was higher than in controls (637 vs. 563 μm, P<0.0001). There was no difference in EC density in eyes treated with IOLs compared with fellow eyes (3445 and 3487 cells/mm2, P=0.68). Means for CV (25 vs. 24, P=0.07) and percent hexagonal cells (74 vs. 76%, P=0.27) were also not significantly different. Mean CCT was higher in eyes with IOLs (605 vs. 571 μm, P<0.0001) compared with fellow eyes. Compared with treated eyes without glaucoma or glaucoma suspect, treated eyes with glaucoma had lower EC density (3289 vs. 3783 cells/mm2, P=0.03) and treated eyes with glaucoma suspect had greater mean corneal thickness (660 vs. 612 μm, P=0.0036).Cataract extraction during infancy with IOL implantation was not associated with a reduced EC count in treated compared with fellow eyes, although CCT was increased. Extended-wear aphakic CLs may cause corneal polymegathism with increased EC density and CCT. Glaucoma diagnosis was associated with reduced EC counts and increased CCT.

  View details for DOI 10.1016/j.ophtha.2015.07.011

  View details for Web of Science ID 000363491800019

  View details for PubMedID 26271843

• Neonatal aphakia is associated with altered levels of dopamine metabolites in the non-human primate retina. Experimental eye research Iuvone, P. M., Haque, R., Fernandes, A., Lambert, S. R. 2015; 140: 187-189

  Abstract

  Neonatal aphakia is associated with retardation of the axial elongation of the neonatal eye. In contrast, form deprivation increases axial elongation, an effect that has been associated with decreased retinal dopamine metabolism. The present investigation was conducted to test the hypothesis that neonatal aphakia induces an effect on the levels of retinal dopamine opposite to form deprivation. Lensectomy and vitrectomy were performed on the right eyes of rhesus monkeys at approximately 1 week of age; their left eyes were unmanipulated. Axial length was measured by A-scan ultrasonography. Prior to surgery, mean axial length of the right and left eyes was identical. Following lens removal, both eyes continued to elongate, however the aphakic eyes elongated at a slower rate resulting in a significant shorter axial length compared to that of the unmanipulated eye. Removal of the crystalline lens had no effect on steady-state dopamine levels in either central or peripheral retina. However, levels of the dopamine metabolites, 3,4-dihydroxyphenylacetic acid and homovanillic acid were significantly elevated in central retina, but not in the peripheral retina of aphakic eyes. Our results support the hypothesis that dopamine is a component of the retinal signaling pathways that are involved in the regulation of eye growth and emmetropization.

  View details for DOI 10.1016/j.exer.2015.09.002

  View details for PubMedID 26368851

• The Association of Race With Childhood Uveitis AMERICAN JOURNAL OF OPHTHALMOLOGY Angeles-Han, S. T., McCracken, C., Yeh, S., Jenkins, K., Stryker, D., Travers, C., Rouster-Stevens, K., Vogler, L. B., Lambert, S. R., Drews-Botsch, C., Prahalad, S. 2015; 160 (5): 919-928

  Abstract

  To identify risk factors for a severe uveitis course among children with noninfectious uveitis.Retrospective cohort study.This was a retrospective analysis of a prospectively collected database. Records of 94 children with uveitis were reviewed at enrollment and every 3-6 months (2011-2015). Severe uveitis was defined as a history of ocular complications or a visual acuity (VA) of ≤20/200. Children were compared by disease, VA, complications, and race. Regression models were used to model risk factors for severe disease. When examining race, we focused on non-Hispanic African-American and non-Hispanic white children only.Of 85 children with uveitis and complete ocular examinations, 27 (32%) had a history of a VA of ≤20/200. A subanalysis of non-Hispanic African-American and white children showed an increased prevalence of VA ≤20/200 in non-Hispanic African-Americans (18/25; 72% vs 4/43; 9%). Non-Hispanic African-Americans were more likely to be diagnosed at an older age (P = .030) and to have intermediate uveitis (P = .026), bilateral disease (P = .032), a history of VA ≤20/50 (P = .002), VA ≤20/200 (P < .001), and a higher rate of complications (P < .001). On multivariable analysis, non-Hispanic African-American race was a significant predictor of blindness (OR = 31.6, 95% CI 5.9-168.5, P < .001), after controlling for uveitis duration. Non-Hispanic African-Americans also developed 2.2 times more unique complications per year of disease than non-Hispanic whites when controlling for uveitis type and duration.There appear to be racial differences in the outcomes of children with uveitis. Non-Hispanic African-American children with non-juvenile idiopathic arthritis-associated uveitis may have worse visual outcomes with increased vision loss and ocular complications. These findings highlight the need for future studies in minority populations.

  View details for DOI 10.1016/j.ajo.2015.08.002

  View details for Web of Science ID 000363914800011

  View details for PubMedID 26255577

• Neonatal aphakia is associated with altered levels of dopamine metabolites in the non-human primate retina EXPERIMENTAL EYE RESEARCH Iuvone, P. M., Hague, R., Fernandes, A., Lambert, S. R. 2015; 140: 187-189

  View details for DOI 10.1016/j.exer.2015.09.002

  View details for Web of Science ID 000364259700021

• Using the Effects of Youngsters' Eyesight on Quality of Life Questionnaire to Measure Visual Outcomes in Children With Uveitis ARTHRITIS CARE & RESEARCH Angeles-Han, S. T., Yeh, S., McCracken, C., Jenkins, K., Stryker, D., Myoung, E., Vogler, L. B., Rouster-Stevens, K., Lambert, S. R., Harrison, M. J., Prahalad, S., Drews-Botsch, C. 2015; 67 (11): 1513-1520

  Abstract

  The Effects of Youngsters' Eyesight on Quality of Life (EYE-Q) is a novel measure of vision-related quality of life (QOL) and function in children. We aim to determine the validity of the EYE-Q in childhood uveitis.We abstracted medical record data on arthritis and uveitis in a convenience sample of children with juvenile idiopathic arthritis (JIA) and/or uveitis. In addition to the EYE-Q, parents and patients completed questionnaires on overall QOL (Pediatric Quality of Life Inventory [PedsQL]), and physical functioning (Childhood Health Assessment Questionnaire [C-HAQ]).Among 57 children (8 JIA, 24 JIA and uveitis, 25 uveitis alone), 102 ocular examinations were performed within 1 month of completing questionnaires. Uveitis patients had bilateral disease (69%), anterior involvement (78%), synechiae (51%), and cataracts (49%). Children with vision loss in their better eye (visual acuity [VA] 20/50 or worse) had worse EYE-Q (P = 0.006) and PedsQL (P = 0.028) scores, but not C-HAQ scores. The EYE-Q moderately correlated with logMAR VA (rs  = -0.43), PedsQL (rs  = 0.43), and C-HAQ (rs  = -0.45), but was not correlated with anterior chamber cells or intraocular pressure. The PedsQL and C-HAQ did not correlate with VA or cells. There were strong correlations between the parent and child EYE-Q (rs  = 0.62). Cronbach's α for the child report was 0.91. The EYE-Q had strong test-retest reliability (rs  = 0.75).The EYE-Q may be an important tool in the assessment of visual outcomes in childhood uveitis and an improvement over general measures in detecting changes in vision-related function.

  View details for DOI 10.1002/acr.22627

  View details for Web of Science ID 000363688600004

  View details for PubMedID 26037544

• Glaucoma-Related Adverse Events in the First 5 Years After Unilateral Cataract Removal in the Infant Aphakia Treatment Study JAMA OPHTHALMOLOGY Freedman, S. F., Lynn, M. J., Beck, A. D., Bothun, E. D., Oerge, F. H., Lambert, S. R. 2015; 133 (8): 907-914

  View details for DOI 10.1001/jamaophthalmol.2015.1329

  View details for Web of Science ID 000361374800012

• Structural changes of the anterior chamber following cataract surgery during infancy JOURNAL OF CATARACT AND REFRACTIVE SURGERY Nguyen, M., Shainberg, M., Beck, A. D., Lambert, S. R. 2015; 41 (8): 1784-1786

  View details for Web of Science ID 000362998800033

  View details for PubMedID 26432141

• Reply: To PMID 25266841. Journal of AAPOS Lambert, S. R., Lenhart, P. D., Leon, M., Hutchinson, A. K. 2015; 19 (4): 393-?

  View details for DOI 10.1016/j.jaapos.2015.05.008

  View details for PubMedID 26296787

• Glaucoma-Related Adverse Events in the First 5 Years After Unilateral Cataract Removal in the Infant Aphakia Treatment Study. JAMA ophthalmology Freedman, S. F., Lynn, M. J., Beck, A. D., Bothun, E. D., Örge, F. H., Lambert, S. R. 2015; 133 (8): 907-914

  Abstract

  Glaucoma-related adverse events constitute major sight-threatening complications of cataract removal in infancy, yet their relationship to aphakia vs primary intraocular lens (IOL) implantation remains unsettled.To identify and characterize cases of glaucoma and glaucoma-related adverse events (glaucoma + glaucoma suspect) among children in the Infant Aphakia Treatment Study by the age of 5 years.A multicenter randomized clinical trial of 114 infants with unilateral congenital cataract in referral centers who were between ages 1 and 6 months at surgery. Mean follow-up was 4.8 years. This secondary analysis was conducted from December 23, 2004, to November 13, 2013.Participants were randomized at cataract surgery to either primary IOL or no IOL implantation (contact lens). Standardized definitions of glaucoma and glaucoma suspect were created for the Infant Aphakia Treatment Study and applied for surveillance and diagnosis.Development of glaucoma and glaucoma + glaucoma suspect in operated on eyes for children up to age 5 years, plus intraocular pressure, visual acuity, and axial length at age 5 years.Product limit estimates of the risk for glaucoma and glaucoma + glaucoma suspect at 4.8 years after surgery were 17% (95% CI, 11%-25%) and 31% (95% CI, 24%-41%), respectively. The contact lens and IOL groups were not significantly different for either outcome: glaucoma (hazard ratio [HR], 0.8; 95% CI, 0.3-2.0; P = .62) and glaucoma + glaucoma suspect (HR, 1.3; 95% CI, 0.6-2.5; P = .58). Younger (vs older) age at surgery conferred an increased risk for glaucoma (26% vs 9%, respectively) at 4.8 years after surgery (HR, 3.2; 95% CI, 1.2-8.3), and smaller (vs larger) corneal diameter showed an increased risk for glaucoma + glaucoma suspect (HR, 2.5; 95% CI, 1.3-5.0). Age and corneal diameter were significantly positively correlated. Glaucoma was predominantly open angle (19 of 20 cases, 95%), most eyes received medication (19 of 20, 95%), and 8 of 20 eyes (40%) underwent surgery.These results suggest that glaucoma-related adverse events are common and increase between ages 1 and 5 years in infants after unilateral cataract removal at 1 to 6 months of age; primary IOL placement does not mitigate their risk but surgery at a younger age increases the risk. Longer follow-up of these children may further characterize risk factors, long-term outcomes, potential differences between eyes having primary IOL vs aphakia, and optimal timing of unilateral congenital cataract removal.clinicaltrials.gov Identifier: NCT00212134.

  View details for DOI 10.1001/jamaophthalmol.2015.1329

  View details for PubMedID 25996491

• Characteristics of a cohort of children with Juvenile Idiopathic Arthritis and JIA-associated Uveitis PEDIATRIC RHEUMATOLOGY Angeles-Han, S. T., McCracken, C., Yeh, S., Jenkins, K., Stryker, D., Rouster-Stevens, K., Vogler, L. B., Lambert, S. R., Drews-Botsch, C., Prahalad, S. 2015; 13

  Abstract

  Juvenile idiopathic arthritis-associated uveitis (JIA-U) can lead to poor visual outcomes and impact a child's quality of life (QOL) and function. Our aim is to identify risk markers of JIA-U and examine differences in the QOL of children with JIA and JIA-U.Rheumatology and ophthalmology record reviews and questionnaires were completed every 4-6 months on 287 children with JIA. We collected arthritis, uveitis, and QOL data. We examined data through last study visit.There were 52/287 (18%) children with JIA-U who were younger at arthritis diagnosis, had oligoarticular persistent JIA, and ANA positive. Confirmed uveitis predictors were age at JIA diagnosis (OR = 0.86) and oligoarticular subtype (OR = 5.92). They had worse vision specific QOL and function, but similar general QOL. Blindness occurred in 17.5% of children but was more common in African American children compared to non-Hispanic Caucasian children ((5/7 (71%) vs. 2/29 (7%), p <0.001) despite a similar uveitis prevalence (22% vs. 16%). Both races had similar complications, although band keratopathy was more frequent in African Americans (75% vs. 15.6%, p = 0.003).We confirm young age at JIA diagnosis and the oligoarticular JIA subtype as predictors of uveitis development. Although we were unable to identify predictors of ocular complications or blindness, AA children appeared to have a more severe disease course manifested by increased ocular complications, vision loss and blindness. Potential causes that warrant additional study include underlying disease severity, access to medical care and referral bias. Further investigation of the risk factors for vision-compromising uveitis and its' long-term effects should be conducted in a large racially diverse population.

  View details for DOI 10.1186/s12969-015-0018-8

  View details for Web of Science ID 000355398900001

  View details for PubMedID 26031738

• Outcomes after Intravitreal Bevacizumab versus Laser Photocoagulation for Retinopathy of Prematurity A 5-Year Retrospective Analysis OPHTHALMOLOGY Hwang, C. K., Hubbard, G. B., Hutchinson, A. K., Lambert, S. R. 2015; 122 (5): 1008-1015

  Abstract

  To determine the relative effectiveness, major complications, and refractive errors associated with intravitreal bevacizumab (IVB) versus panretinal photocoagulation (PRP) to treat type 1 retinopathy of prematurity (ROP).Retrospective case series.Consecutive infants with type 1 ROP who received either IVB or PRP and had at least 6 months of follow-up.The data from infants treated with either IVB or PRP for type 1 ROP between 2008 and 2012 were recorded from 2 medical centers in Atlanta, Georgia.Recurrence rate, complication rate, and refractive error.A total of 54 eyes (28 patients) with type 1 ROP were evaluated: 22 eyes (11 patients) received IVB, and 32 eyes (17 patients) received PRP. Among the 22 eyes treated with IVB, 16 eyes had zone I ROP and 6 eyes had posterior zone II ROP. The number of zone I and II ROP eyes treated with PRP were 5 and 27, respectively. Mean gestational age, birth weight, postmenstrual age at initial treatment, and follow-up period for the infants receiving IVB were 24.2 weeks, 668.1 g, 35.1 weeks, and 21.7 weeks, respectively, and for the infants receiving PRP, these were 24.8 weeks, 701.4 g, 36.1 weeks, and 34.5 weeks, respectively. Retinopathy of prematurity recurred in 3 (14%) of 22 IVB-treated eyes and in 1 (3%) of 32 PRP-treated eyes. Neither retinal detachment nor macular ectopia developed in any of the IVB-treated eyes. In PRP-treated eyes, retinal detachment developed in only 1 eye and macular ectopia developed in 5 eyes. Mean spherical equivalent and postgestational age at the last refraction for IVB-treated eyes were -2.4 diopters (D) and 22.4 months, respectively, and for PRP-treated eyes, these were -5.3 D and 37.1 months, respectively. Mean spherical equivalent for zone I ROP eyes treated with IVB and PRP were -3.7 D and -10.1 D, respectively, and for zone II ROP eyes, these were 0.6 D and -4.7 D, respectively.Both IVB and PRP are effective treatment options for type 1 ROP with low complication rates. IVB was associated with less myopia than PRP, although longer follow-up was available for PRP.

  View details for DOI 10.1016/j.ophtha.2014.12.017

  View details for Web of Science ID 000353337600028

  View details for PubMedID 25687024

• Global challenges in the management of congenital cataract: proceedings of the 4th International Congenital Cataract Symposium held on March 7, 2014, New York, New York. Journal of AAPOS Lenhart, P. D., Courtright, P., Wilson, M. E., Lewallen, S., Taylor, D. S., Ventura, M. C., Bowman, R., Woodward, L., Ditta, L. C., Kruger, S., Haddad, D., El Shakankiri, N., Rai, S. K., Bailey, T., Lambert, S. R. 2015; 19 (2): e1-8

  Abstract

  Childhood cataracts have become a leading cause of preventable childhood blindness in many areas of the world. Here we summarize regional focus group discussions from the 4th Annual International Congenital Cataract Symposium on the current situation, challenges, and recommendations for the management of congenital cataracts in sub-Saharan Africa, the Middle East and North Africa, South Asia, Central America, South America, and developed nations. Strategies for managing congenital cataracts must be adapted and developed according to regional conditions. A basic framework for acceptable outcomes must focus on developing systems to address the critical components of education, access, quality care, and good follow-up.

  View details for DOI 10.1016/j.jaapos.2015.01.013

  View details for PubMedID 25892047

• The Infant Aphakia Treatment Study: Further on intra- and postoperative complications in the intraocular lens group JOURNAL OF AAPOS Lambert, S. R., Plager, D. A., Buckley, E. G., Wilson, M. E., DuBois, L., Drews-Botsch, C. D., Hartmann, E. E., Lynn, M. J. 2015; 19 (2): 101-103

  View details for DOI 10.1016/j.jaapos.2015.01.012

  View details for Web of Science ID 000353664400001

  View details for PubMedID 25892038

• Cost of Intraocular Lens versus Contact Lens Treatment after Unilateral Congenital Cataract Surgery in the Infant Aphakia Treatment Study at Age 5 Years OPHTHALMOLOGY Kruger, S. J., DuBois, L., Becker, E. R., Morrison, D., Wilson, L., Wilson, M. E., Lambert, S. R. 2015; 122 (2): 288-292

  Abstract

  To analyze differences in the cost of treatment for infants randomized to primary intraocular lens (IOL) implantation versus optical correction with a contact lens (CL) after unilateral cataract surgery in the Infant Aphakia Treatment Study (IATS).Retrospective cost analysis of a prospective, randomized clinical trial based on Georgia Medicaid reimbursement data as well as actual costs of supplies used during the study, adjusted for inflation.The IATS is a multicenter (n = 12), randomized clinical trial comparing the optical treatment of aphakia with either primary IOL implantation (n = 57) or CL correction (n = 57) in 114 infants with unilateral congenital cataract.One hundred fourteen infants underwent unilateral cataract surgery and were either corrected optically by primary IOL implantation at the time of surgery or were corrected with a CL after surgery.The mean cost of cataract surgery and all additional surgeries, examinations, and supplies used up to 5 years of age.The 5-year treatment cost of an infant with a unilateral congenital cataract corrected optically with an IOL was $27 090 versus $25 331 for a patient treated with a CL after initial cataract surgery. The total cost of supplies was $3204 in the IOL group versus $7728 in the CL group.Unilateral cataract surgery in infancy coupled with primary IOL implantation is approximately 7% more expensive than aphakia and CL correction. Patient costs are more than double with CL versus IOL treatment.

  View details for DOI 10.1016/j.ophtha.2014.08.037

  View details for Web of Science ID 000348290000020

  View details for PubMedID 25439604

• Predictors of Good Motor and Sensory Outcomes Following Strabismus Surgery for Patients with Third Nerve Palsies. Neuro-ophthalmology Peragallo, J. H., Bruce, B. B., Hutchinson, A. K., Lenhart, P. D., Biousse, V., Newman, N. J., Lambert, S. R. 2015; 39 (1): 12-16

  Abstract

  Strabismus from third nerve palsy (3NP) is difficult to treat. Our goal was to explore factors associated with successful surgical outcomes in 3NP. Institutional records of all adult patients (>18 years) from 1988 to 2012 with 3NP who underwent strabismus surgery or botulinum toxin injections were retrospectively reviewed. Success was defined as absence of diplopia, vertical deviation ≤2 prism dioptres (PD), and horizontal deviation ≤10 PD. Fifty-six patients from four surgeons were included. Thirty (54%) were female; mean age was 48 (range: 20-80). Forty-four (79%) had unilateral 3NP; 9 (16%) had other ocular motor nerve palsies. 3NP were complete in 24 (43%). Underlying aetiology was idiopathic/microvascular in 5 (9%), traumatic in 13 (23%), neoplastic in 12 (21%), aneurysmal in 10 (18%), and other central nervous system related in 16 (29%). Trauma was associated more frequently with aberrant regeneration than other aetiologies: 9/13 (69%) versus 4/43 (9%) (p < 0.01). Ten patients (18%) had >1 surgery. Surgical success was achieved in 28/56 (50%). Success rate was unaffected by aetiology, degree of palsy, pupillary involvement, presence of aberrant regeneration, or number of other cranial nerves involved. However, adjustable sutures were used in 27 patients (48%), and there was a trend toward higher success rates when adjustable versus nonadjustable sutures were used (63% versus 38%; p = 0.06). Unlike with sixth nerve palsies, the aetiology and degree of 3NP does not appear to affect the success rates or number of procedures performed. Strabismus surgeries for 3NP with adjustable sutures may be associated with better outcomes.

  View details for PubMedID 27928324

• Baseline Characteristics of the Infant Aphakia Treatment Study Population: Predicting Recognition Acuity at 4.5 Years of Age INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE Hartmann, E. E., Lynn, M. J., Lambert, S. R. 2015; 56 (1): 388-395

  View details for DOI 10.1167/iovs.14-15464

  View details for Web of Science ID 000351519800042

  View details for PubMedID 25503455

• Stereopsis Results at 4.5 Years of Age in the Infant Aphakia Treatment Study AMERICAN JOURNAL OF OPHTHALMOLOGY Hartmann, E. E., Stout, A. U., Lynn, M. J., Yen, K. G., Kruger, S. J., Lambert, S. R. 2015; 159 (1): 64-70

  Abstract

  To determine whether stereopsis of infants treated for monocular cataracts varies with the type of optical correction used.Randomized prospective clinical trial.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.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).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.

  View details for DOI 10.1016/j.ajo.2014.09.028

  View details for Web of Science ID 000347747500009

  View details for PubMedID 25261241

• Baseline characteristics of the infant aphakia treatment study population: predicting recognition acuity at 4.5 years of age. Investigative ophthalmology & visual science Hartmann, E. E., Lynn, M. J., Lambert, S. R. 2014; 56 (1): 388-395

  Abstract

  To identify patient baseline characteristics that predict recognition acuity at 4.5 years of age in the Infant Aphakia Treatment Study, a study of patients with monocular infantile cataracts.We analyzed baseline characteristics of the 114 infants enrolled in the Infant Aphakia Treatment Study to determine which were most predictive of visual outcome at 4.5 years of age. All infants underwent cataract surgery between 1 and 7 months of age. Monocular acuity was assessed at 4.5 years of age by a traveling examiner using the Amblyopia Treatment Study HOTV protocol.Age at cataract surgery was weakly associated with visual acuity (Spearman rank correlation coefficient = 0.19, P = 0.041) with median visual acuity better among the younger patients (28-48 days: 0.50 logMAR, 49-210 days: 1.10 logMAR, P = 0.046). Patients from families with private insurance had significantly better median visual acuity (0.60 vs. 1.40 logMAR, P = 0.0004). No other baseline characteristic revealed a significant bivariate relationship with visual acuity. A multiple linear regression relating visual acuity to all baseline characteristics demonstrated that only the availability of private insurance was statistically significant, accounting for 12% of the variance.This analysis concurs with previous studies that early surgery is important for good visual outcomes in patients with unilateral infantile cataracts. The fact that only one baseline variable (private insurance) contributed to the multivariate analysis, accounting for 12% of the variance, suggests that predicting visual outcome for these patients is complicated at best, and cannot be estimated from baseline characteristics alone. (ClinicalTrials.gov number, NCT00212134.).

  View details for DOI 10.1167/iovs.14-15464

  View details for PubMedID 25503455

• The Role of Magnetic Resonance Imaging in Diagnosing Optic Nerve Hypoplasia AMERICAN JOURNAL OF OPHTHALMOLOGY Lenhart, P. D., Desai, N. K., Bruce, B. B., Hutchinson, A. K., Lambert, S. R. 2014; 158 (6): 1164-1171

  Abstract

  To establish objective lower limits of normal optic nerve (ON) size in children based on high-resolution orbital magnetic resonance imaging (MRI).Case-control study of patients with ON hypoplasia vs normal controls.A neuroradiologist made 4 measurements of each ON at 2 locations (5 mm posterior to the optic disc and just posterior to the optic canal) in patients with ON hypoplasia and controls aged 0-17 years from an academic eye center and children's hospital. Primary analyses were performed using mixed linear models.Measurements were made in 26 cases of clinically confirmed ON hypoplasia and 31 controls (median age: ON hypoplasia, 1 year; controls, 5.5 years). Nine of 26 cases (35%) and 19 of 31 controls (61%) underwent high-resolution T2-weighted imaging of the orbits. Mean ON diameter was 1.36 mm (95% CI: 1.19-1.54; P < .001) smaller for clinically hypoplastic ONs than for controls. Optic nerve diameter increased by 0.05 mm per year of age (95% CI: 0.03-0.07; P < .001). A lower bound to the 95% prediction interval for normal optic nerves was (2.24 mm + 0.052 × [age in years]) mm and excluded all but 1 case.Age was independently associated with normal ON size by MRI and should be taken into consideration when evaluating ON hypoplasia, based on MRI criteria. We have provided a formula to assist clinicians in objectively determining if ON hypoplasia is present.

  View details for DOI 10.1016/j.ajo.2014.08.013

  View details for Web of Science ID 000345820400010

  View details for PubMedID 25128595

• In reply. JAMA ophthalmology Lambert, S. R., Lynn, M. J., Hartmann, E. E. 2014; 132 (12): 1492-1493

  View details for DOI 10.1001/jamaophthalmol.2014.3542

  View details for PubMedID 25256439

• Reply: To PMID 25077835. American journal of ophthalmology Plager, D. A., Lynn, M. J., Lambert, S. R., Buckley, E. G., Wilson, M. E. 2014; 158 (6): 1361-1362

  View details for DOI 10.1016/j.ajo.2014.09.019

  View details for PubMedID 25457713

• Complications in the First 5 Years Following Cataract Surgery in Infants With and Without Intraocular Lens Implantation in the Infant Aphakia Treatment Study AMERICAN JOURNAL OF OPHTHALMOLOGY Plager, D. A., Lynn, M. J., Buckley, E. G., Wilson, M. E., Lambert, S. R. 2014; 158 (5): 892-898

  Abstract

  To compare rates and severity of complications between infants undergoing cataract surgery with and without intraocular lens (IOL) implantation.Prospective randomized clinical trial.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.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 < .0001) in the IOL group than in the contact lens group. However, the number of patients with adverse events in the contact lens group increased (15 to 24) in postoperative years 2-5 compared to the first postoperative year, while it decreased (44 to 14) in years 2-5 compared to the first postoperative year in the IOL group. If only one half of the patients in the contact lens (aphakic) group eventually undergo secondary IOL implantation, the number of additional intraocular surgeries in the 2 groups will be approximately equal.The increased rate of complications, adverse events, and additional intraocular surgeries associated with IOL implantation in infants <7 months of age militates toward leaving babies aphakic if it is considered likely that the family will be successful with contact lens correction.

  View details for DOI 10.1016/j.ajo.2014.07.031

  View details for Web of Science ID 000343783400006

  View details for PubMedID 25077835

• The cost-effectiveness of different strategies to evaluate optic disk drusen in children JOURNAL OF AAPOS Leon, M., Hutchinson, A. K., Lenhart, P. D., Lambert, S. R. 2014; 18 (5): 449-452

  Abstract

  To compare the costs of diagnostic work-up for optic disk drusen where ophthalmic ultrasound was performed prior to imaging and invasive studies with those where ophthalmic ultrasound was performed after such studies.The medical records of patients <18 years of age evaluated at a tertiary referral center between 2007 and 2012 for "swollen" optic nerves were retrospectively reviewed. The main outcome measure was cost of diagnostic work-up according to Georgia Medicaid global reimbursement rates.A total of 46 children with a B-scan ultrasound-confirmed diagnosis of calcified optic disk drusen were included. Neuroimaging was performed in 23 patients, of whom 20 had the study prior to ophthalmic ultrasound. The mean cost of evaluations for patients undergoing ancillary testing prior to ophthalmic ultrasound was $1,173; for those undergoing ancillary testing after, $305.Because optic disk drusen can mimic the appearance of papilledema, it is more cost-effective to perform ophthalmic ultrasonography prior to neuroimaging, especially when the patient is asymptomatic. If ophthalmic ultrasonography confirms the presence of drusen, it is more cost-effective to reassess the clinical picture before proceeding with further tests.

  View details for DOI 10.1016/j.jaapos.2014.06.006

  View details for Web of Science ID 000343854200009

  View details for PubMedID 25266841

• Author reply: To PMID 23419803. Ophthalmology Bothun, E. D., Lynn, M. J., Lambert, S. R. 2014; 121 (10)

  View details for DOI 10.1016/j.ophtha.2014.05.011

  View details for PubMedID 24909820

• The effects of surgical factors on postoperative astigmatism in patients enrolled in the Infant Aphakia Treatment Study (IATS) JOURNAL OF AAPOS Wall, P. B., Lee, J. A., Lynn, M. J., Lambert, S. R., Traboulsi, E. I. 2014; 18 (5): 441-445

  Abstract

  To evaluate the effect of surgical factors on postoperative astigmatism in infants undergoing cataract extraction with or without intraocular lens (IOL) implantation.The Infant Aphakia Treatment Study is a multicenter clinical trial in which 114 infants with unilateral congenital cataracts were randomized to undergo cataract extraction with IOL placement or contact lens aphakic correction. Surgical videos were reviewed with regard to incision type and location, whether the incision was extended, the number of sutures placed, and technique of closure. Corneal astigmatism was measured using a handheld keratometer prior to surgery and at 1 year of age.Corneal astigmatism decreased from a mean of 1.92 D at baseline to 1.62 D at age 1 year in the contact lens group but remained almost unchanged from 2.00 D to 2.09 D in the IOL group (P = 0.023). There was no statistical difference between the amount of corneal astigmatism with regard to incision type (P = 0.214) and no increase in astigmatism with extension of the incision to facilitate IOL placement (P = 0.849) at 1 year. The number of sutures and technique of closure did not influence the amount of astigmatism at 1 year.At the age of 1 year following cataract extraction in infants, contact lens correction and the lack of IOL placement are associated with a significant decrease in postoperative corneal astigmatism compared to IOL placement. No other surgical factors considered in this study had a statistically significant effect on corneal astigmatism.

  View details for DOI 10.1016/j.jaapos.2014.06.016

  View details for Web of Science ID 000343854200007

  View details for PubMedID 25266831

• Postoperative Glaucoma Following Infantile Cataract Surgery An Individual Patient Data Meta-analysis JAMA OPHTHALMOLOGY Mataftsi, A., Haidich, A., Kokkali, S., Rabiah, P. K., Birch, E., Stager, D. R., Cheong-Leen, R., Singh, V., Egbert, J. E., Astle, W. F., Lambert, S. R., Amitabh, P., Khan, A. O., Grigg, J., Arvanitidou, M., Dimitrakos, S. A., Nischal, K. K. 2014; 132 (9): 1059-1067

  Abstract

  Infantile cataract surgery bears a significant risk for postoperative glaucoma, and no consensus exists on factors that may reduce this risk.To assess the effect of primary intraocular lens implantation and timing of surgery on the incidence of postoperative glaucoma.We searched multiple databases to July 14, 2013, to identify studies with eligible patients, including PubMed, MEDLINE, EMBASE, ISI Web of Science, Scopus, Central, Google Scholar, Intute, and Tripdata. We also searched abstracts of ophthalmology society meetings.We included studies reporting on postoperative glaucoma in infants undergoing cataract surgery with regular follow-up for at least 1 year. Infants with concurrent ocular anomalies were excluded.Authors of eligible studies were invited to contribute individual patient data on infants who met the inclusion criteria. We also performed an aggregate data meta-analysis of published studies that did not contribute to the individual patient data. Data were pooled using a random-effects model.Time to glaucoma with the effect of primary implantation, additional postoperative intraocular procedures, and age at surgery.Seven centers contributed individual patient data on 470 infants with a median age at surgery of 3.0 months and median follow-up of 6.0 years. Eighty patients (17.0%) developed glaucoma at a median follow-up of 4.3 years. Only 2 of these patients had a pseudophakic eye. The risk for postoperative glaucoma appeared to be lower after primary implantation (hazard ratio [HR], 0.10 [95% CI, 0.01-0.70]; P = .02; I(2) = 34%), higher after surgery at 4 weeks or younger (HR, 2.10 [95% CI, 1.14-3.84]; P = .02; I(2) = 0%), and higher after additional procedures (HR, 2.52 [95% CI, 1.11-5.72]; P = .03; I(2) = 32%). In multivariable analysis, additional procedures independently increased the risk for glaucoma (HR, 2.25 [95% CI, 1.20-4.21]; P = .01), and primary implantation independently reduced it (HR, 0.10 [95% CI, 0.01-0.76]; P = .03). Results were similar in the aggregate data meta-analysis that included data from 10 published articles.Although confounding factors such as size of the eye and surgeon experience are not accounted for in this meta-analysis, the risk for postoperative glaucoma after infantile cataract surgery appears to be influenced by the timing of surgery, primary implantation, and additional intraocular surgery.

  View details for DOI 10.1001/jamaophthalmol.2014.1042

  View details for Web of Science ID 000343059600006

  View details for PubMedID 24921712

• Nystagmus and Related Fixation Instabilities Following Extraction of Unilateral Infantile Cataract in the Infant Aphakia Treatment Study (IATS) INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE Felius, J., Busettini, C., Lynn, M. J., Hartmann, E. E., Lambert, S. R. 2014; 55 (8): 5332-5337

  Abstract

  To study eye movements in a large group of children after the removal of unilateral infantile cataract, and to compare fixation instabilities between treatment groups with or without IOL implantation.The Infant Aphakia Treatment Study (IATS) is a randomized, multicenter clinical trial comparing IOL to contact lens (CL) treatment with a unilateral infantile cataract in participants who underwent cataract surgery at 1 to 6 months of age. At age 4.5 years, eye movements were recorded in 103 participants, using a high-speed video camera while the child performed a fixation task. The recordings were inspected by masked readers for the presence of fixation instabilities (nystagmus and saccadic oscillations).Overall, fixation instabilities were observed in 50 (60%) of 83 children who had evaluable recordings, with no differences between treatment groups (27 [64%] of 42 in the IOL group, 23 [56%] of 41 in the CL group; P = 0.51). Nystagmus was seen in 38% and saccadic oscillations in 31%, with no differences between treatment groups (P > 0.33). Children without a fixation instability had better visual acuity (P = 0.04).Nystagmus and saccadic oscillations are well-known consequences of infantile cataracts, presumably the result of visual deprivation during the critical period of visual development. After early cataract extraction, successful optical correction may reduce further form deprivation and minimize the incidence of these fixation instabilities. In this study, no differences in the presence of fixation instabilities were found between the two treatment strategies (CL or IOL) for optical correction after cataract removal. (ClinicalTrials.gov number, NCT00212134.).

  View details for DOI 10.1167/iovs.14-14710

  View details for Web of Science ID 000343145500087

  View details for PubMedID 25097243

• Comparison of Contact Lens and Intraocular Lens Correction of Monocular Aphakia During Infancy A Randomized Clinical Trial of HOTV Optotype Acuity at Age 4.5 Years and Clinical Findings at Age 5 Years JAMA OPHTHALMOLOGY Lambert, S. R., Lynn, M. J., Hartmann, E. E., DuBois, L., Drews-Botsch, C., Freedman, S. F., Plager, D. A., Buckley, E. G., Wilson, E. 2014; 132 (6): 676-682

  Abstract

  The efficacy and safety of primary intraocular lens (IOL) implantation during early infancy is unknown.To compare the visual outcomes of patients optically corrected with contact lenses vs IOLs following unilateral cataract surgery during early infancy.The Infant Aphakia Treatment Study is a randomized clinical trial with 5 years of follow-up that involved 114 infants with unilateral congenital cataracts at 12 sites. A traveling examiner assessed visual acuity at age 4.5 years.Cataract surgery with or without primary IOL implantation. Contact lenses were used to correct aphakia in patients who did not receive IOLs. Treatment was determined through random assignment.HOTV optotype visual acuity at 4.5 years of age.The median logMAR visual acuity was not significantly different between the treated eyes in the 2 treatment groups (both, 0.90 [20/159]; P = .54). About 50% of treated eyes in both groups had visual acuity less than or equal to 20/200. Significantly more patients in the IOL group had at least 1 adverse event after cataract surgery (contact lens, 56%; IOL, 81%; P = .02). The most common adverse events in the IOL group were lens reproliferation into the visual axis, pupillary membranes, and corectopia. Glaucoma/glaucoma suspect occurred in 35% of treated eyes in the contact lens group vs 28% of eyes in the IOL group (P = .55). Since the initial cataract surgery, significantly more patients in the IOL group have had at least 1 additional intraocular surgery (contact lens, 21%; IOL, 72%; P < .001).There was no significant difference between the median visual acuity of operated eyes in children who underwent primary IOL implantation and those left aphakic. However, there were significantly more adverse events and additional intraoperative procedures in the IOL group. When operating on an infant younger than 7 months of age with a unilateral cataract, we recommend leaving the eye aphakic and focusing the eye with a contact lens. Primary IOL implantation should be reserved for those infants where, in the opinion of the surgeon, the cost and handling of a contact lens would be so burdensome as to result in significant periods of uncorrected aphakia.clinicaltrials.gov Identifier: NCT00212134

  View details for DOI 10.1001/jamaophthalmol.2014.531

  View details for Web of Science ID 000337889000004

  View details for PubMedID 24604348

• Reply: To PMID 24011524. American journal of ophthalmology VanderVeen, D. K., Trivedi, R. H., Nizam, A., Lynn, M. J., Lambert, S. R. 2014; 157 (6): 1332-1333

  View details for DOI 10.1016/j.ajo.2014.02.053

  View details for PubMedID 24881851

• The role of preoperative biometry in selecting initial contact lens power in the Infant Aphakia Treatment Study JOURNAL OF AAPOS Trivedi, R. H., Lambert, S. R., Lynn, M. J., Wilson, M. E. 2014; 18 (3): 251-254

  Abstract

  To investigate the role of preoperative biometry for selecting initial contact lens power.Patients randomized to receive contact lenses in the Infant Aphakia Treatment Study (IATS) were retrospectively analyzed. Inclusion criteria were availability of both a preoperative immersion axial length measurement and a 1-month postoperative refractive value. The target contact lens power for distance was determined using 1-month postoperative spherical equivalent refraction (after adjusting for a vertex distance) over the known contact lens power. We compared targeted contact lens power for distance with three other treatment techniques: (1) 30 D contact lens (32 D minus 2 D overcorrection for near vision based on IATS protocol); (2) regression-estimated contact lens power of 84.4 - 3.2 × axial length; and (3) IOL power calculated using the Sanders-Retzlaff-Kraff (SRK/T) regression formula with a modified A-constant (112.176). Prediction error (targeted minus estimated contact lens power) and its absolute values were calculated.A total of 34 eyes of 34 patients met inclusion criteria. Age at the time of cataract surgery was 2.4 ± 1.7 months. Follow-up refraction was performed at 31 ± 3 days after surgery. Target contact lens power for distance was 26.0 ± 4.5 D for the IATS cohort (which excluded infants with corneal diameter <9 mm). The mean prediction error was -4.0, -1.0, and -2.0 D and mean absolute prediction error was 4.4, 2.2, and 2.9 D, respectively, for 30 D contact lens, regression, and SRK/T-estimated power.Preoperative biometry can be used to estimate contact lens power for distance if an accurate refraction cannot be obtained initially.

  View details for DOI 10.1016/j.jaapos.2014.01.012

  View details for Web of Science ID 000337715900009

  View details for PubMedID 24924278

• Functional and Motor Outcomes of Strabismus Surgery for Chronic Isolated Adult Sixth Nerve Palsy. Neuro-ophthalmology Peragallo, J. H., Bruce, B. B., Hutchinson, A. K., Lenhart, P. D., Biousse, V., Newman, N. J., Lambert, S. R. 2014; 38 (6): 320-325

  Abstract

  Abducens nerve palsy is the most common acquired ocular motor nerve palsy in adults. Chronic cases of abducens palsy often require surgical intervention to relieve disabling diplopia. The goal of this study was to identify factors associated with surgical outcomes in isolated abducens palsy. Medical records of all adult patients from 1988 to 2012 with abducens palsies who underwent strabismus procedures were retrospectively reviewed. Motor alignment, extraocular motility, and sensory outcomes were recorded. Success was defined as absence of diplopia without prisms or face turn, vertical deviation ≤2 prism dioptres (PD), and horizontal deviation ≤10 PD. Eighty-one patients (age range: 20-86 years) met inclusion criteria. Success was achieved in 58% of patients. Final success rates for abducens palsy were 50% for neoplastic, 59% traumatic, 57% for microvascular/unknown, and 67% for other central nervous system causes (p > 0.05). Patients with an underlying neoplastic or traumatic aetiology required more than one strabismus procedure more often than those with microvascular/idiopathic or other central nervous system causes (48% vs. 24%; p = 0.03). For complete abducens palsies, patients who underwent Hummelsheim-type procedures had a higher success rate than those who underwent a full-tendon vertical rectus muscle transposition. (78% versus 35%; p = 0.049). Success rates for strabismus procedures in patients with abducens palsies are similar across all aetiologies. Frequency of re-operation is higher among those patients with neoplastic or traumatic aetiologies for their abducens palsies. Hummelsheim-type procedures have a higher success rate for complete abducens palsies than full-tendon vertical rectus transpositions.

  View details for PubMedID 27928319

• Strabismus surgery outcomes after scleral buckling procedures for retinal reattachment. Strabismus Chang, J. H., Hutchinson, A. K., Zhang, M., Lambert, S. R. 2013; 21 (4): 235-241

  Abstract

  To investigate the outcomes of strabismus surgery in patients with a prior history of a scleral buckling procedure for retinal reattachment.We reviewed the medical records of 18 patients who underwent strabismus surgery following a scleral buckling procedure and investigated the effect of multiple variables on postoperative alignment after strabismus surgery including gender, age, surgeon, number of strabismus surgeries, adjustable suture use, previous pars plana vitrectomy, preoperative best-corrected visual acuity, and scleral buckle removal. Outcomes were considered successful if there was ≤ 10 prism diopters (PD) residual horizontal and/or ≤ 4 PD residual vertical deviations. Statistical analyses were performed using Fisher's exact test, Mann-Whitney test, and nominal logistic regression.Success using our criteria of motor alignment was achieved in 6 of 18 eyes (33%). A higher rate of success was found in the scleral buckle removal group (success with buckle removal, 62.5%; success without buckle removal, 10.0%; p = 0.04). Nominal logistic regression analysis showed scleral buckle removal was the most significant factor associated with successful surgical alignment (p = 0.03; odds ratio = 16.67). Although the success rate was higher in the adjustable suture group (50% in adjustable group vs 14.3% in non-adjustable group: Fisher's exact test, p = 0.30), this difference was not statistically significant. No retinal redetachments occurred after scleral buckle removal.These results suggest that scleral buckles can be safely removed in selected patients with strabismus following retinal reattachment surgery and scleral buckle removal may improve ocular alignment following strabismus surgery.

  View details for DOI 10.3109/09273972.2013.851260

  View details for PubMedID 24299332

• Predictability of Intraocular Lens Power Calculation Formulae in Infantile Eyes With Unilateral Congenital Cataract: Results from the Infant Aphakia Treatment Study AMERICAN JOURNAL OF OPHTHALMOLOGY VanderVeen, D. K., Trivedi, R. H., Nizam, A., Lynn, M. J., Lambert, S. R. 2013; 156 (6): 1252-1260

  Abstract

  To compare accuracy of intraocular lens (IOL) power calculation formulae in infantile eyes with primary IOL implantation.Comparative case series.The Hoffer Q, Holladay 1, Holladay 2, Sanders-Retzlaff-Kraff (SRK) II, and Sanders-Retzlaff-Kraff theoretic (SRK/T) formulae were used to calculate predicted postoperative refraction for eyes that received primary IOL implantation in the Infant Aphakia Treatment Study. The protocol targeted postoperative hyperopia of +6.0 or +8.0 diopters (D). Eyes were excluded for invalid biometry, lack of refractive data at the specified postoperative visit, diagnosis of glaucoma or suspected glaucoma, or sulcus IOL placement. Actual refraction 1 month after surgery was converted to spherical equivalent and prediction error (predicted refraction - actual refraction) was calculated. Baseline characteristics were analyzed for effect on prediction error for each formula. The main outcome measure was absolute prediction error.Forty-three eyes were studied; mean axial length was 18.1 ± 1.1 mm (in 23 eyes, it was <18.0 mm). Average age at surgery was 2.5 ± 1.5 months. Holladay 1 showed the lowest median absolute prediction error (1.2 D); a paired comparison of medians showed clinically similar results using the Holladay 1 and SRK/T formulae (median difference, 0.3 D). Comparison of the mean absolute prediction error showed the lowest values using the SRK/T formula (1.4 ± 1.1 D), followed by the Holladay 1 formula (1.7 ± 1.3 D). Calculations with an optimized constant showed the lowest values and no significant difference between the Holladay 1 and SRK/T formulae (median difference, 0.3 D). Eyes with globe AL of less than 18 mm had the largest mean and median prediction error and absolute prediction error, regardless of the formula used.The Holladay 1 and SRK/T formulae gave equally good results and had the best predictive value for infant eyes.

  View details for DOI 10.1016/j.ajo.2013.07.014

  View details for Web of Science ID 000327674900024

  View details for PubMedID 24011524

• Fibrin Glue for Closure of Conjunctival Incision in Strabismus Surgery A Report by the American Academy of Ophthalmology OPHTHALMOLOGY Yang, M. B., Melia, M., Lambert, S. R., Chiang, M. F., Simpson, J. L., Buffenn, A. N. 2013; 120 (9): 1935-1941

  Abstract

  To evaluate the severity of postoperative inflammation, degree of patient discomfort, adequacy of wound closure, and length of operating time when using fibrin glue compared with sutures to close limbal conjunctival incisions after strabismus surgery.Literature searches of the PubMed and Cochrane Library databases were last conducted on January 24, 2013, and resulted in 24 citations, including 2 not in the English language. All citations were reviewed in full text. Five studies compared fibrin glue (68 eyes) with sutures (74 eyes) for closure of limbal conjunctival incisions in patients undergoing strabismus surgery and were included in this assessment; no studies were found that evaluated fornix incisions. A quality rating was assigned to each study using criteria specifically developed for this assessment.No level I studies were found, and 5 level II studies were identified. There was significantly less postoperative inflammation and patient discomfort for 1 to 3 weeks after strabismus surgery for eyes treated with fibrin glue compared with sutures. In 3 studies that evaluated wound apposition, 2 of 50 eyes (4%) with conjunctival incisions that were initially closed using fibrin glue subsequently developed a wound gap that required suture repair. In the 2 studies that compared surgical time, fibrin glue required 1 to 5 minutes less time than suturing in 1 study and 55% less time (3.8 vs. 8.4 minutes) in a second study. These 5 studies did not evaluate the cost-effectiveness or risk of viral transmission from fibrin glue.Studies in the literature suggest that the off-label use of fibrin glue to close limbal conjunctival incisions in strabismus surgery resulted in less postoperative inflammation and required shorter operating time compared with sutures, but it increased the percentage of wounds requiring subsequent repair with sutures.The author(s) have no proprietary or commercial interest in any materials discussed in this article.

  View details for DOI 10.1016/j.ophtha.2013.05.008

  View details for Web of Science ID 000324045800051

  View details for PubMedID 23820078

• Reply: To PMID 23639132. American journal of ophthalmology Lambert, S. R., Superak, H. M., Lynn, M. J., Beck, A. D. 2013; 156 (3): 629-?

  View details for DOI 10.1016/j.ajo.2013.06.002

  View details for PubMedID 23953163

• Long-term Risk of Glaucoma After Congenital Cataract Surgery AMERICAN JOURNAL OF OPHTHALMOLOGY Lambert, S. R., Purohit, A., Superak, H. M., Lynn, M. J., Beck, A. D. 2013; 156 (2): 355-361

  Abstract

  To report the long-term risk of glaucoma development in children following congenital cataract surgery.Retrospective interventional consecutive case series.We retrospectively reviewed the records of 62 eyes of 37 children who underwent congenital cataract surgery when <7 months of age by the same surgeon using a limbal approach. The Kaplan-Meier method was used to calculate the probability of an eye's developing glaucoma and/or becoming a glaucoma suspect over time.The median age of surgery was 2.0 months and the median follow-up after cataract surgery was 7.9 years (range, 3.2-23.5 years). Nine eyes (14.5%) developed glaucoma a median of 4.3 months after cataract surgery and an additional 16 eyes (25.8%) were diagnosed as glaucoma suspects a median of 8.0 years after cataract surgery. The probability of an eye's developing glaucoma was estimated to be 19.5% (95% CI: 10.0%-36.1%) by 10 years after congenital cataract surgery. When the probability of glaucoma and glaucoma suspect were combined, the risk increased to 63.0% (95% CI: 43.6%-82.3%).Long-term monitoring of eyes after congenital cataract surgery is important because we estimate that nearly two thirds of these eyes will develop glaucoma or become glaucoma suspects by 10 years after cataract surgery.

  View details for DOI 10.1016/j.ajo.2013.03.013

  View details for Web of Science ID 000322748200021

  View details for PubMedID 23639132

• One-Year Strabismus Outcomes in the Infant Aphakia Treatment Study OPHTHALMOLOGY Bothun, E. D., Cleveland, J., Lynn, M. J., Christiansen, S. P., VanderVeen, D. K., Neely, D. E., Kruger, S. J., Lambert, S. R. 2013; 120 (6): 1227-1231

  Abstract

  To evaluate the characteristics of strabismus in infants who underwent cataract surgery with and without intraocular lens (IOL) implantation.Secondary outcome analysis in a prospective, randomized clinical trial.The Infant Aphakia Treatment Study is a randomized, multicenter (n = 12), clinical trial comparing treatment of aphakia with a primary IOL or contact lens in 114 infants with a unilateral congenital cataract.Infants underwent cataract surgery with or without placement of an IOL.The proportion of patients in whom strabismus developed during the first 12 months of follow-up was calculated using the life-table method and was compared across treatment groups and age strata using a log-rank test.Strabismus developed within the first 12 months of follow-up in 38 pseudophakic infants (life-table estimate, 66.7%) and 42 infants (life-table estimate, 74.5%) treated with contact lenses (P = 0.59). The younger cohort (<49 days) at the time of surgery demonstrated less strabismus (29 of 50; life-table estimate, 58.0%) than the older cohort (≥ 49 days; 51 of 64; life-table estimate, 80.0%; P<0.01).Intraocular lens placement does not prevent the early development of strabismus after congenital cataract surgery. However, strabismus was less likely to develop in infants whose cataract was removed at an earlier age.The author(s) have no proprietary or commercial interest in any materials discussed in this article.

  View details for DOI 10.1016/j.ophtha.2012.11.039

  View details for Web of Science ID 000320650700028

  View details for PubMedID 23419803

• Rebound Tonometry in Children A Report by the American Academy of Ophthalmology OPHTHALMOLOGY Lambert, S. R., Melia, M., Buffenn, A. N., Chiang, M. F., Simpson, J. L., Yang, M. B. 2013; 120 (4): E21-E27

  Abstract

  To compare intraocular pressure (IOP) measurements in children 18 years of age and younger using rebound tonometry and applanation tonometry and the feasibility of using these techniques in children.Literature searches of the PubMed and the Cochrane Library databases were last conducted in June 2012 and resulted in 43 citations, including citations not in English. Of these 43 citations, 4 studies met the inclusion criterion following full text review. A level of evidence rating was assigned to each study using criteria specifically developed for this assessment.No level I study was found in the literature search, and 2 level II and 2 level III studies were identified. Intraocular pressure was 2 to 3 mmHg higher using rebound tonometry compared with Goldmann applanation tonometry in the 2 level II studies performed in a clinic setting and in 1 level III study performed on children under general anesthesia. However, IOP was lower in 1 level III study in which noncontact applanation tonometry was compared with rebound tonometry. Bland-Altman plots showed that the difference in IOP for rebound versus Goldmann applanation tonometry increased as the IOP increased. The success rate for measuring IOP was markedly higher in children 3 years of age and younger using rebound tonometry compared with noncontact tonometry in 1 level III study.Rebound tonometry seems to be a reasonably accurate instrument that allows the IOP to be measured in many children without using general anesthesia. More data are required to assess better how the differences between instruments vary with IOP measurement.

  View details for DOI 10.1016/j.ophtha.2012.09.058

  View details for Web of Science ID 000317343500001

  View details for PubMedID 23395545

• Candida species lens abscesses in infants with a history of neonatal Candida sepsis JOURNAL OF AAPOS Couser, N. L., Hubbard, G. B., Lee, L. B., Hutchinson, A. K., Lambert, S. R. 2013; 17 (2): 208-210

  Abstract

  We report 3 patients who were delivered prematurely and who developed a lens opacity with signs of ocular inflammation at a postgestational age of 10-52 weeks. All patients had been treated for Candida sepsis as neonates. Each patient underwent lensectomy and anterior vitrectomy. In 2 infants Candida albicans was cultured from the lens/anterior chamber membrane; in 1 Candida parapsilosis was cultured from a lens aspirate. Despite eradication of the fungal infection, outcomes were poor: 2 eyes developed glaucoma, and 1 developed a retinal detachment with subsequent phthisis.

  View details for DOI 10.1016/j.jaapos.2012.11.006

  View details for Web of Science ID 000319027700020

  View details for PubMedID 23622453

• Cost of Intraocular Lens versus Contact Lens Treatment after Unilateral Congenital Cataract Surgery: Retrospective Analysis at Age 1 Year OPHTHALMOLOGY Carrigan, A. K., DuBois, L. G., Becker, E. R., Lambert, S. R. 2013; 120 (1): 14-19

  Abstract

  To describe the differences in treatment costs for infants randomized to contact lens correction versus primary intraocular lens (IOL) implantation after unilateral cataract surgery in the Infant Aphakia Treatment Study (IATS).Retrospective cost analysis of a prospective, randomized clinical trial based on Georgia Medicaid data and the actual costs of supplies used.The IATS is a randomized, multicenter (n=12) clinical trial comparing treatment of aphakia with a primary IOL or contact lens in 114 infants with unilateral congenital cataract.Infants underwent cataract surgery with or without placement of an IOL.The mean cost of cataract surgery and all additional surgeries, examinations, and supplies used up to 12 months of age.The mean cost of treatment for a unilateral congenital cataract with primary IOL implantation was $14 752 versus $10 726 with contact lens correction. The initial cataract surgery accounted for approximately 50% of the treatment costs for both groups. Contact lens costs accounted for 15% ($1600/patient) in the aphakic group, whereas glasses costs represented only 4% ($535/patient) in the IOL group. The increased costs in the IOL group were primarily due to the higher cost of cataract surgery in this group ($7302 vs. $5357) and the cost of additional operations.For IATS patients up to 12 months of age, cataract surgery coupled with IOL implantation and spectacle correction was 37.5% (∼$4000) more expensive than cataract surgery coupled with contact lens correction.The author(s) have no proprietary or commercial interest in any materials discussed in this article.

  View details for DOI 10.1016/j.ophtha.2012.07.049

  View details for Web of Science ID 000313011700005

  View details for PubMedID 23047003

• The efficacy of botulinum toxin treatment for children with a persistent esotropia following bilateral medial rectus recessions and lateral rectus resections. The American orthoptic journal Lambert, S. R., Shainberg, M. J. 2013; 63: 24-28

  Abstract

  To report on the outcomes of treating children with a persistent esotropia with an injection of botulinum toxin in a medial rectus muscle.The medical records were reviewed of all children at one institution with a persistent esotropia after bilateral medial rectus recessions and bilateral lateral rectus resections then treated with a botulinum toxin injection.Five patients with a mean preoperative esotropia of 37Δ (range 25–50Δ) underwent bilateral medial rectus recessions and then bilateral lateral rectus resections. Their residual esotropia (mean of 25Δ; range 18–35Δ) was then treated with a single injection of 3–5 units of botulinum toxin into one medial rectus muscle. The patients were then followed for a mean of 34 months (range 14–79 months). At last follow-up, two patients had an esotropia <10Δ. The other three patients had no long-term improvement in their ocular alignment. Two of these patients then underwent additional strabismus surgery. In both cases, they then developed a consecutive exotropia.Treatment with a single injection of botulinum toxin was beneficial in 2 of 5 children. Botulinum toxin treatment alone did not result in a consecutive exotropia in any patients treated.

  View details for DOI 10.3368/aoj.63.1.24

  View details for PubMedID 24260804

• A Randomized Trial Comparing the Cost-effectiveness of 2 Approaches for Treating Unilateral Nasolacrimal Duct Obstruction ARCHIVES OF OPHTHALMOLOGY Lee, K. A., Chandler, D. L., Repka, M. X., Beck, R. W., Foster, N. C., Frick, K. D., Golden, R. P., Lambert, S. R., Melia, M., Tien, D. R., Weakley, D. R. 2012; 130 (12): 1525-1533

  Abstract

  To compare the cost-effectiveness of 2 approaches for treating unilateral nasolacrimal duct obstruction (NLDO).One hundred sixty-three infants aged 6 to less than 10 months with unilateral NLDO were randomly assigned to receive immediate office-based nasolacrimal duct probing (n = 82) or 6 months of observation/nonsurgical management (n = 81) followed by probing in a facility for persistent symptoms.Treatment success was defined as the absence of clinical signs of NLDO (epiphora, increased tear lake, mucous discharge) on masked examination at age 18 months. Cost of treatment between randomization and age 18 months included costs for all surgical procedures and medications.In the observation/deferred facility-probing group, NLDO resolved within 6 months without surgery in 44 of the 67 patients (66%; 95% CI, 54% to 76%) who completed the 6-month visit. Twenty-two (27%) of the 81 patients in the observation/deferred facility-probing group underwent surgery, 4 of whom were operated on within the initial 6 months. At age 18 months, 69 of 75 patients (92%) in the immediate office-probing group were treatment successes, compared with 58 of 71 observation/deferred facility-probing group patients (82%) (10% difference in success; 95% CI, -1% to 21%). The mean cost of treatment was $562 in the immediate office-probing group compared with $701 in the observation/deferred facility-probing group (difference, -$139; 95% CI, -$377 to $94). The immediate office-probing group experienced 3.0 fewer months of symptoms (95% CI, -1.8 to -4.0).The immediate office-probing approach is likely more cost-effective than observation followed by deferred facility probing if needed. Adoption of the immediate office-probing approach would result in probing in approximately two-thirds of infants whose obstruction would have resolved within 6 months of nonsurgical management, but would largely avoid the need for probing under general anesthesia.Although unilateral NLDO often resolves without surgery, immediate office probing is an effective and potentially cost-saving treatment option.clinicaltrials.gov Identifier: NCT00780741.

  View details for Web of Science ID 000312195300003

  View details for PubMedID 23229693

• Botulinum toxin a treatment of consecutive esotropia in children. Strabismus Couser, N. L., Lambert, S. R. 2012; 20 (4): 158-161

  Abstract

  To assess the outcome of botulinum A toxin (BTXA) to treat surgically overcorrected intermittent exotropia in children.A retrospective analysis was performed on a series of children with consecutive esotropia treated with BTXA.Six children with a mean consecutive esotropia of 21 prism diopters (PD) were treated with BTXA at a mean of 19.8 months following strabismus surgery. Two patients underwent a single injection, three patients 2 injections, and one patient 3 injections. Complications included transient ptosis and a vertical deviation. Mean follow-up from last BTXA injection was 16 months. At last follow-up, 4 of the 6 patients were orthotropic and stereopsis was present in 4 of 5 patients old enough to cooperate with testing. One patient was treated with strabismus surgery following a single BTXA injection.BTXA is an efficacious treatment for consecutive esotropia in children. However, in our series, two-thirds of patients required multiple injections to achieve the desired outcome and one ultimately had an additional strabismus surgery.

  View details for DOI 10.3109/09273972.2012.702324

  View details for PubMedID 23211140

• Axial Elongation following Cataract Surgery during the First Year of Life in the Infant Aphakia Treatment Study INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE Lambert, S. R., Lynn, M. J., DuBois, L. G., Cotsonis, G. A., Hartmann, E. E., Wilson, M. E. 2012; 53 (12): 7539-7545

  Abstract

  To compare ocular axial elongation in infants after unilateral cataract surgery corrected with a contact lens (CL) or primary intraocular lens (IOL) implantation.Baseline axial length (AL) was measured at the time of cataract surgery (1-6 months) and at age 1 year. AL at baseline and age 1 year and the change in length/mo were analyzed in relation to treatment modality, cataractous versus fellow eye, and age at surgery using linear mixed models.Mean baseline AL did not differ between the CL and IOL groups for either cataractous or fellow eyes. Eyes with cataracts were shorter than fellow eyes by an average of 0.6 mm (95% confidence interval [CI], 0.4-0.8 mm; P < 0.0001). For the operated eyes, the mean change in AL/mo was smaller in the CL group (0.17 mm/mo) than in the IOL group (0.24 mm/mo) (P = 0.0006) and was independent of age at surgery (P = 0.19). In contrast, the change in AL/mo for fellow eyes decreased with older age at surgery (P < 0.0001). At age 1 year, operated eyes treated with a CL were 0.6 mm shorter on average than operated eyes treated with an IOL (P = 0.009).At baseline, eyes with cataracts were shorter than fellow eyes. The change in AL/mo was smaller in operated eyes treated with a CL than in operated eyes treated with an IOL, but was not significantly related to age at surgery. (ClinicalTrials.gov number, NCT00212134.).

  View details for DOI 10.1167/iovs.12-10285

  View details for Web of Science ID 000313053500015

  View details for PubMedID 23074203

• The Infant Aphakia Treatment Study Contact Lens Experience: One-Year Outcomes EYE & CONTACT LENS-SCIENCE AND CLINICAL PRACTICE Russell, B., Ward, M. A., Lynn, M., DuBois, L., Lambert, S. R. 2012; 38 (4): 234-239

  Abstract

  We describe our experience correcting a cohort of infants with contact lenses in the Infant Aphakia Treatment Study.Fifty-seven infants 1-6 months of age were randomized to contact lens wear. An examination under anesthesia was performed at the time of enrollment and at approximately 1 year of age. A traveling examiner assessed visual acuity at approximately 1 year of age.Forty-two treated eyes (74 %) were fitted with silicone elastomer (SE) contact lenses; 12 eyes (21 %) with rigid gas permeable (RGP) contact lenses, and 3 eyes (5%) wore both lens types. Median visual acuity was +0.80 logMAR in both lens type-wearing groups. The mean (± SD) keratometric power of the treated eyes was 46.3±2.8 D at baseline and 44.6±2.3 D at 1 year of age for a mean decrease of 0.2±0.2 D/mo. Keratometric astigmatism of treated eyes was 1.98±1.37 D at baseline and 1.62±0.98 D at 1 year of age for a mean decrease of 0.05±0.2 D/mo. The mean RGP lens base curve at baseline was 47.62 D±2.62 D versus 47.00 D±3.50 D at 12 months after surgery. Children wearing SE lenses required a mean of 10.9 replacements (range 2-24) compared to 16.8 replacements (range 8-32) for children wearing RGP lenses. Three adverse events occurred.Contact lenses were worn successfully with relatively few adverse events by a cohort of infants with unilateral aphakia. The visual acuity results were identical independent of the contact lens material or modality. RGP lenses needed replacement more often than SE lenses.

  View details for DOI 10.1097/ICL.0b013e3182562dc0

  View details for Web of Science ID 000306083800007

  View details for PubMedID 22669008

• Detection of Clinically Significant Retinopathy of Prematurity Using Wide-angle Digital Retinal Photography A Report by the American Academy of Ophthalmology OPHTHALMOLOGY Chiang, M. F., Melia, M., Buffenn, A. N., Lambert, S. R., Recchia, F. M., Simpson, J. L., Yang, M. B. 2012; 119 (6): 1272-1280

  Abstract

  To evaluate the accuracy of detecting clinically significant retinopathy of prematurity (ROP) using wide-angle digital retinal photography.Literature searches of PubMed and the Cochrane Library databases were conducted last on December 7, 2010, and yielded 414 unique citations. The authors assessed these 414 citations and marked 82 that potentially met the inclusion criteria. These 82 studies were reviewed in full text; 28 studies met inclusion criteria. The authors extracted from these studies information about study design, interventions, outcomes, and study quality. After data abstraction, 18 were excluded for study deficiencies or because they were superseded by a more recent publication. The methodologist reviewed the remaining 10 studies and assigned ratings of evidence quality; 7 studies were rated level I evidence and 3 studies were rated level III evidence.There is level I evidence from ≥5 studies demonstrating that digital retinal photography has high accuracy for detection of clinically significant ROP. Level III studies have reported high accuracy, without any detectable complications, from real-world operational programs intended to detect clinically significant ROP through remote site interpretation of wide-angle retinal photographs.Wide-angle digital retinal photography has the potential to complement standard ROP care. It may provide advantages through objective documentation of clinical examination findings, improved recognition of disease progression by comparing previous photographs, and the creation of image libraries for education and research.Proprietary or commercial disclosure may be found after the references.

  View details for DOI 10.1016/j.ophtha.2012.01.002

  View details for Web of Science ID 000304717100026

  View details for PubMedID 22541632

• Resolution of Congenital Nasolacrimal Duct Obstruction With Nonsurgical Management ARCHIVES OF OPHTHALMOLOGY Petersen, D. B., Chandler, D. L., Repka, M. X., Beck, R. W., Crouch, E. R., Lee, K. A., Melia, M., Morrison, D. G., Orge, F. H., Ticho, B. H., Sala, N. A., Hodde, R. M., Zeto, V. L., Schweinler, B. R., Plum, L. W., McMurtrey, J. R., Tien, R., Yoon-Huang, G. H., McGuinness, M. B., De La Rosa, J. M., Bailey, C. M., Marinaro, J. A., Khammar, A. J., Clausius, D. A., Coletta, J. B., Imler, B. C., Bremer, D. L., Cassady, C. M., Golden, R. P., McGregor, M. L., Rogers, D. L., Rogers, G. L., Fellows, R. R., McMillin, M. C., Rinehart, T. M., Wagner, A. J., Brendez, J. A., Cox, R. E., Weakley, D. R., Dias, C. L., Zhang, X. W., Crouch, E. R., Crouch, E. R., Ventura, G. G., Morrison, D. G., Fraine, L. A., Biernacki, R. J., Franklin, C. C., Haskins, K. J., Hoover, D. L., Huston, P. A., Lambert, S. R., Hutchinson, A. K., Lenhart, P. D., Shainberg, M. J., Robb, R. A., Couser, N. L., Morse, C. L., Fang, C. C., Christian, M. L., Karlsson, V. X., Traboulsi, E., Rychwalski, P. J., Crowe, S. W., Silbert, D. I., Singman, E. L., Matta, N. S., Leckemby, G. L., Pavlica, M. R., Summers, C. G., Bothun, E. D., De Becker, I., Holleschau, A. M., de Melo, A. I., Hogue, K. M., Merrill, K. S., Miller, A. M., Jackson, J. L., LaRiviere, S. S., Goldblum, T. A., Alfaro, A., Atkinson, S., Trythall, C. L., Hendrickson, A. B., Avallone, J. M., Bryant, C. R., Peyton, W. E., Orge, F. H., Schoeck, S. E., Colon, B. J., Mar, N. X., Vaswani, R. S. 2012; 130 (6): 730-734

  View details for Web of Science ID 000305145600012

• Long-term Outcomes of Undercorrection Versus Full Correction After Unilateral Intraocular Lens Implantation in Children AMERICAN JOURNAL OF OPHTHALMOLOGY Lambert, S. R., Archer, S. M., Wilson, M. E., Trivedi, R. H., Del Monte, M. A., Lynn, M. 2012; 153 (4): 602-608

  Abstract

  To evaluate the impact of full correction vs undercorrection on the magnitude of the myopic shift and postoperative visual acuity after unilateral intraocular lens (IOL) implantation in children.Retrospective case control study.The medical records of 24 children who underwent unilateral cataract surgery and IOL implantation at 2 to <6 years of age were reviewed. The patients were divided into 2 groups based on their 1-month-postoperative refraction: Group 1 (full correction) -1.0 to +1.0 diopter (D) and Group 2 (undercorrection) ≥+2.0 D. The main outcome measures included the change in refractive error per year and visual acuity for the pseudophakic eyes at last follow-up visit. The groups were compared using the independent groups t test and Wilcoxon rank sum test.The mean age at surgery (Group 1, 4.2±0.9 years, n=12; Group 2, 4.5±1.0 years, n=12; P=.45) and mean follow-up (Group 1, 5.8±3.7 years; Group 2, 6.1±3.5 years; P=.69) were similar for the 2 groups. The change in refractive error (Group 1, -0.4±0.5 D/y; Group 2, -0.3±0.2 D/y; P=.70) and last median logMAR acuity (Group 1, 0.4; Group 2, 0.4; P=.54) were not significantly different between the 2 groups.We did not find a significant difference in the myopic shift or the postoperative visual acuity in children aged 2 to <6 years of age following unilateral cataract surgery and IOL implantation if the initial postoperative refractive error was near emmetropia or undercorrected by 2 diopters or more.

  View details for DOI 10.1016/j.ajo.2011.08.046

  View details for Web of Science ID 000302387100003

  View details for PubMedID 22206805

• Current Role of Cryotherapy in Retinopathy of Prematurity A Report by the American Academy of Ophthalmology OPHTHALMOLOGY Simpson, J. L., Melia, M., Yang, M. B., Buffenn, A. N., Chiang, M. F., Lambert, S. R. 2012; 119 (4): 873-877

  Abstract

  To evaluate the role of cryotherapy in the current treatment of retinopathy of prematurity (ROP).Literature searches of PubMed and the Cochrane Library were conducted on December 2, 2009, for articles published after 1984. The searches included all languages and retrieved 187 relevant citations. Thirteen articles were deemed relevant to the assessment question and were rated according to the strength of evidence. Four articles reported results from 2 large multicenter randomized clinical trials, and the remaining 9 articles reported results of 3 small randomized trials that directly compared cryotherapy and laser.Neither of the multicenter randomized clinical trials was a direct comparison of cryotherapy with laser. These studies were used to evaluate the comparative trials based on treatment criteria, study populations, and clinical results. Higher percentages of poor structural and functional outcomes generally were seen in eyes treated with cryotherapy compared with eyes undergoing laser treatment. Higher rates of systemic complications and myopia also were identified after treatment with cryotherapy.Despite a relative paucity of level I evidence directly comparing cryotherapy and laser treatment for threshold ROP, the literature suggests that neonatal facilities should gain access to laser technology and laser-trained ophthalmic staff to achieve better outcomes for treatment of the disease.

  View details for DOI 10.1016/j.ophtha.2012.01.003

  View details for Web of Science ID 000302355400029

  View details for PubMedID 22381808

• Congenital Fibrovascular Pupillary Membranes: Clinical and Histopathologic Findings OPHTHALMOLOGY Lambert, S. R., Buckley, E. G., Lenhart, P. D., Zhang, Q., Grossniklaus, H. E. 2012; 119 (3): 634-641

  Abstract

  To report the clinical and histopathologic findings associated with congenital fibrovascular pupillary membranes.Case series.Seven infants were included, 6 with a unilateral congenital pupillary membrane and 1 with classic persistent fetal vasculature (PFV).Patients underwent a membranectomy, pupilloplasty, or lensectomy. Histopathologic examination was performed on the excised membranes.Visual acuity and pupil size.Four of the 6 patients with a unilateral congenital pupillary membrane had 1 or more recurrences after a membranectomy and pupilloplasty. The most recent pupil size ranged from 2 to 5 mm in the affected eye. When last tested, the vision in the affected eye was excellent in 4 of the 6 patients. The 2 patients without recurrences of the pupillary membranes underwent multiple iris sphincterotomies at the time of the initial surgery. Histopathologic examination of 2 primary pupillary membranes showed fibrovascular tissue that did not stain for neuron-specific enolase. Smooth muscle actin was only present in vascular walls. In contrast, histopathology of a recurrent pupillary membrane revealed collagenized fibrovascular tissue that was immunoreactive for smooth muscle actin. Finally, histopathology of the retrolenticular membrane excised from an infant with classic PFV was similar to the latter aside from hypercellularity.Congenital fibrovascular pupillary membranes in infants are likely a variant of PFV that may recur if incompletely excised. The risk of these membranes recurring may be reduced by excising as much as the membrane as possible and enlarging the pupil with iris sphincterotomies. A lensectomy should be avoided if possible.

  View details for DOI 10.1016/j.ophtha.2011.08.043

  View details for Web of Science ID 000300957200030

  View details for PubMedID 22197437

• Glaucoma-Related Adverse Events in the Infant Aphakia Treatment Study 1-Year Results ARCHIVES OF OPHTHALMOLOGY Beck, A. D., Freedman, S. F., Lynn, M. J., Bothun, E., Neely, D. E., Lambert, S. R. 2012; 130 (3): 300-305

  Abstract

  To report the incidence of glaucoma and glaucoma suspects in the IATS, and to evaluate risk factors for the development of a glaucoma-related adverse event in patients in the IATS in the first year of follow-up.A total of 114 infants between 1 and 6 months of age with a unilateral congenital cataract were assigned to undergo cataract surgery either with or without an intraocular lens implant. Standardized definitions of glaucoma and glaucoma suspect were created and used in the IATS.Of these 114 patients, 10 (9%) developed glaucoma and 4 (4%) had glaucoma suspect, for a total of 14 patients (12%) with a glaucoma-related adverse event in the treated eye through the first year of follow-up. Of the 57 patients who underwent lensectomy and anterior vitrectomy, 5 (9%) developed a glaucoma-related adverse event; of the 57 patients who underwent an intraocular lens implant, 9 (16%) developed a glaucoma-related adverse event. The odds of developing a glaucoma-related adverse event were 3.1 times higher for a child with persistent fetal vasculature and 1.6 times higher for each month of age younger at cataract surgery.Modern surgical techniques do not eliminate the early development of glaucoma following congenital cataract surgery with or without an intraocular lens implant. Younger patients with or without persistent fetal vasculature seem more likely to develop a glaucoma-related adverse event in the first year of follow-up. Vigilance for the early development of glaucoma is needed following congenital cataract surgery, especially when surgery is performed during early infancy or for a child with persistent fetal vasculature. Five-year follow-up data for the IATS will likely reveal more glaucoma-related adverse events.clinicaltrials.gov Identifier: NCT00212134.

  View details for DOI 10.1001/archophthalmol.2011.347

  View details for Web of Science ID 000301353400003

  View details for PubMedID 22084157

• Predictability of Intraocular Lens Calculation and Early Refractive Status The Infant Aphakia Treatment Study ARCHIVES OF OPHTHALMOLOGY VanderVeen, D. K., Nizam, A., Lynn, M. J., Bothun, E. D., McClatchey, S. K., Weakley, D. R., DuBois, L. G., Lambert, S. R. 2012; 130 (3): 293-299

  Abstract

  To report the accuracy of intraocular lens (IOL) power calculations and the early refractive status in pseudophakic eyes of infants in the Infant Aphakia Treatment Study.Eyes randomized to receive primary IOL implantation were targeted for a postoperative refraction of +8.0 diopters (D) for infants 28 to 48 days old at surgery and +6.0 D for those 49 days or older to younger than 7 months at surgery using the Holladay 1 formula. Refraction 1 month after surgery was converted to spherical equivalent, and prediction error (PE; defined as the calculated refraction minus the actual refraction) and absolute PE were calculated. Baseline eye and surgery characteristics and A-scan quality were analyzed to compare their effect on PE.Prediction error.Fifty-six eyes underwent primary IOL implantation; 7 were excluded for lack of postoperative refraction (n = 5) or incorrect technique in refraction (n = 1) or biometry (n = 1). Overall mean (SD) absolute PE was 1.8 (1.3) D and mean (SD) PE was +1.0 (2.0) D. Absolute PE was less than 1 D in 41% of eyes but greater than 2 D in 41% of eyes. Mean IOL power implanted was 29.9 D (range, 11.5-40.0 D); most eyes (88%) implanted with an IOL of 30.0 D or greater had less postoperative hyperopia than planned. Multivariate analysis revealed that only short axial length (<18 mm) was significant for higher PE.Short axial length correlates with higher PE after IOL placement in infants. Less hyperopia than anticipated occurs with axial lengths of less than 18 mm or high-power IOLs. Application to Clinical Practice Quality A-scans are essential and higher PE is common, with a tendency for less hyperopia than expected.clinicaltrials.gov Identifier: NCT00212134.

  View details for Web of Science ID 000301353400002

  View details for PubMedID 22411658

• Improved Ocular Alignment with Adjustable Sutures in Adults Undergoing Strabismus Surgery OPHTHALMOLOGY Zhang, M. S., Hutchinson, A. K., Drack, A. V., Cleveland, J., Lambert, S. R. 2012; 119 (2): 396-402

  Abstract

  To assess whether outcomes of strabismus surgery are improved by using the adjustable suture technique and to determine which subgroups of strabismus patients benefit most from the adjustable suture technique.A retrospective chart review.A total of 535 adults who underwent strabismus surgery between 1989 and 2010.Success was defined as ≤10 prism diopters (PD) for horizontal deviations and ≤2 PD for vertical deviations. Differences in the proportion of successful strabismus surgery were analyzed using a chi-square test with an alpha of 0.05.Ocular alignment in primary position at a 7-day to 12-week follow-up examination.A total of 491 patients met the inclusion criteria (nonadjustable suture, n = 186; adjustable suture, n = 305). The success rates for the nonadjustable and adjustable groups were 61.3% and 74.8%, respectively (χ(2)=9.91, P=0.0016). Adjustable suture use was particularly beneficial for patients undergoing a reoperation for childhood strabismus (success rate: nonadjustable, 42.4%; adjustable, 65.7%; P=0.0268; n = 100). The differences in outcomes were not statistically significant for patients with childhood strabismus undergoing a primary surgery (nonadjustable, 65.0%; adjustable, 81.4%; P=0.1354; n = 90) or with thyroid orbitopathy (nonadjustable, 76.7%; adjustable, 74.1%; P=0.8204; n = 57).Strabismus surgery using adjustable sutures was associated with improved short-term ocular alignment compared with strabismus surgery without the use of adjustable sutures. Adjustable sutures were most beneficial for patients undergoing reoperations for childhood strabismus.

  View details for DOI 10.1016/j.ophtha.2011.07.044

  View details for Web of Science ID 000299950300031

  View details for PubMedID 22036633

• Posterior capsular plaque in bilateral congenital cataracts JOURNAL OF AAPOS Morrison, D. G., Lambert, S. R., Wilson, M. E. 2012; 16 (1): 17-20

  Abstract

  Many cases of unilateral congenital cataract have an associated posterior capsular plaque. Observations such as this have led to the hypothesis that posterior capsular plaque is caused by persistent fetal vasculature, generally a unilateral condition, even if no obvious hyaloid remnant is visible. We investigated the incidence of posterior capsular plaque associated with bilateral congenital cataracts.Children with bilateral congenital cataracts were identified from three surgical practices. Children were included if lensectomies were performed prior to age 7 months. Surgical videos were evaluated for cataract morphology.Fourteen children with bilateral cataracts were identified. Surgical videos were available for 24 eyes. Five eyes (21%) had evidence of persistent hyaloid remnants. Of the remaining 19 eyes, cataracts were categorized as nuclear in 10 cases, nuclear with cortical extension in 6 cases, and posterior cortical in 3 cases. Evidence of posterior capsular plaque was seen in seven cases (37%) on video review.Video review revealed a much lower incidence of posterior capsular plaque in eyes with bilateral congenital cataracts compared to unilateral congenital cataracts, lending presumptive evidence to the hypothesis that most unilateral lens opacities arise from persistent fetal vasculature.

  View details for DOI 10.1010/j.jaapos.2011.08.010

  View details for Web of Science ID 000301304700006

  View details for PubMedID 22244507

• Complications, Adverse Events, and Additional Intraocular Surgery 1 Year After Cataract Surgery in the Infant Aphakia Treatment Study OPHTHALMOLOGY Plager, D. A., Lynn, M. J., Buckley, E. G., Wilson, M. E., Lambert, S. R. 2011; 118 (12): 2330-2334

  Abstract

  To compare rates and severity of complications between infants undergoing cataract surgery with and without intraocular lens (IOL) implantation.Prospective, randomized clinical trial.The Infant Aphakia Treatment Study (IATS) is a randomized, multicenter (n = 12) clinical trial comparing treatment of aphakia with a primary IOL or contact lens in 114 infants with unilateral congenital cataract.Infants underwent cataract surgery with or without placement of an IOL.The rate, character, and severity of intraoperative complications (ICs), adverse events (AEs), and additional intraocular surgeries (AISs) during the first postoperative year in the 2 groups were analyzed.There were more patients with ICs (28% vs. 11%; P = 0.031), AEs (77% vs. 25%; P<0.0001), and AISs (63% vs. 12%; P<0.0001) in the IOL group than the contact lens group. Iris prolapse was the most common IC. The most common AE was visual axis opacification, and the most common additional intraocular reoperation was a clearing of visual axis opacification.The rates of ICs, AEs, and AISs 1 year after surgery were numerically higher in the IOL group, but their functional impact does not clearly favor either treatment group.The author(s) have no proprietary or commercial interest in any materials discussed in this article.

  View details for DOI 10.1016/j.ophtha.2011.06.017

  View details for Web of Science ID 000298138000005

  View details for PubMedID 21925737

• The Infant Aphakia Treatment Study: Evaluation of cataract morphology in eyes with monocular cataracts JOURNAL OF AAPOS Wilson, M. E., Trivedi, R. H., Morrison, D. G., Lambert, S. R., Buckley, E. G., Plager, D. A., Lynn, M. J. 2011; 15 (5): 421-426

  Abstract

  To describe a video-documented assessment of cataract type in the eyes of patients with monocular infantile cataract who were enrolled in the Infant Aphakia Treatment Study.The Infant Aphakia Treatment Study is a randomized clinical trial in which the investigators compared intraocular lens (IOL) versus contact lens correction in 114 infants, aged 28 days to <7 months. A total of 83 videos were available for morphological analysis of cataract. Three examiners reviewed all surgical recordings and agreed on the cataract characteristics by using a score sheet to record the lens layer or configuration of the opacity.Nuclear cataract was present in 45 of 83 eyes (54%). Posterior capsule plaque was observed in 73 eyes (88%). All eyes with fetal nuclear cataract had associated posterior capsule plaque. Cortical cataract without nuclear involvement was seen in 21 eyes (25%). Posterior bowing of the posterior capsule was noted in 4 eyes (5%). Evidence of persistent fetal vasculature (PFV) was present in 18 eyes (22%). PFV was the only finding in 5 eyes but was also seen in combination with nuclear (7 eyes) and cortical cataracts (6 eyes). The entire lens was white in 3 eyes (4%), whereas the lens was partially resorbed in 7 (8%) eyes. Anterior capsule fibrosis was noted in 5 eyes with advanced cataract (1 with total cataract, 4 with partially resorbed lens).Nuclear opacities were common, but many different cataract types presented in infancy. PFV occurred in isolation or in association with cataract. Posterior capsule plaque was frequently noted, especially when a nuclear cataract was present.

  View details for DOI 10.1016/j.jaapos.2011.05.016

  View details for Web of Science ID 000297657200006

  View details for PubMedID 22108352

• Infant Aphakia Treatment Study: Effects of persistent fetal vasculature on outcome at 1 year of age JOURNAL OF AAPOS Morrison, D. G., Wilson, M. E., Trivedi, R. H., Lambert, S. R., Lynn, M. J. 2011; 15 (5): 427-431

  Abstract

  The Infant Aphakia Treatment Study is a randomized trial that compares the treatment of unilateral congenital cataract with primary intraocular lens (IOL) implantation versus aphakic contact lens (CLs). The purpose of this study was to compare the outcomes for infants with lens opacity associated with persistent fetal vasculature (PFV) to those without.Retrospective subgroup analysis of grating visual acuity at 1 year of age and adverse events up to 1 year after surgery in eyes identified intraoperatively as having evidence of mild PFV from the IATS.Of 83 infants, 18 (22%: 11 CL, 7 IOL) had PFV. Median logMAR visual acuity was 0.88 for patients with PFV and 0.80 for patients without PFV (P = 0.46). One or more adverse events up to 1 year after surgery occurred in 12 infants (67%) with PFV and 30 infants (46%) without PFV (P = 0.18). The incidence of adverse events was significantly greater in patients with PFV compared with patients without PFV in the CL group (55% vs 20%, P = 0.049) but not in the IOL group (86% vs 71%, P = 0.65), possibly because all children receiving IOLs had greater rates of adverse events when compared with aphakic children (73% vs 29%, P < 0.001).Aphakic infants with mild PFV treated with CL had a greater incidence of adverse events after lensectomy compared with children with other forms of unilateral congenital cataract; nevertheless, similar visual outcomes at 1 year after surgery were obtained.

  View details for DOI 10.1016/j.jaapos.2011.06.004

  View details for Web of Science ID 000297657200007

  View details for PubMedID 22108353

• Development of a Vision-Related Quality of Life Instrument for Children Ages 8-18 Years for Use in Juvenile Idiopathic Arthritis-Associated Uveitis ARTHRITIS CARE & RESEARCH Angeles-Han, S. T., Griffin, K. W., Harrison, M. J., Lehman, T. J., Leong, T., Robb, R. R., Shainberg, M., Ponder, L., Lenhart, P., Hutchinson, A., Srivastava, S. K., Prahalad, S., Lambert, S. R., Drews-Botsch, C. 2011; 63 (9): 1254-1261

  Abstract

  To determine the validity and reliability of a novel questionnaire to measure vision-related quality of life (VRQOL) in children ages 8-18 years for use in juvenile idiopathic arthritis (JIA)-associated uveitis: the Effects of Youngsters' Eyesight on Quality of Life (EYE-Q).Several steps validated the EYE-Q. We interviewed experts and children on how vision affects a child's activities. We developed new items and selected relevant items from existing instruments. We administered initial versions of the EYE-Q to normal-sighted children and those with JIA-associated uveitis. For this study, children with various (or no) ocular conditions were recruited from a clinical population. Visual acuity and contrast sensitivity were performed, and the EYE-Q and Pediatric Quality of Life Inventory (PedsQL) were administered. The EYE-Q was repeated 10 days later. Patients, parents, and physicians rated vision severity.Of 120 patients, 48% were female, 46.7% had no visual impairment, and 53.3% had bilateral eye involvement. The mean age was 11.3 years. There were significant differences in the measures based on visual acuity (P < 0.001). Children with more severe visual acuity and bilateral eye involvement had worse EYE-Q scores (P < 0.001). There were significant associations between the EYE-Q and PedsQL (r = 0.375), repeat EYE-Q (r = 0.864), and clinical measures of ocular disease (r = -0.620).Our study provides evidence of the validity and reliability of the EYE-Q in the measurement of VRQOL. The EYE-Q may complement clinical measures of visual impairment and overall QOL and become an important tool in the assessment of QOL in JIA-associated uveitis.

  View details for DOI 10.1002/acr.20524

  View details for Web of Science ID 000295254900006

  View details for PubMedID 21678564

• Partial coherence interferometry versus immersion ultrasonography for axial length measurement in children JOURNAL OF CATARACT AND REFRACTIVE SURGERY Lenhart, P. D., Hutchinson, A. K., Lynn, M. J., Lambert, S. R. 2010; 36 (12): 2100-2104

  Abstract

  To determine whether measurements obtained by partial coherence interferometry (PCI) correlate well with measurements obtained using immersion ultrasound (US) in children.Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia, USA.Evaluation of a diagnostic test or technology.The charts of pediatric patients who had cataract surgery from August 2008 to September 2009 were reviewed. Axial length (AL) measurements in the operative eye were obtained using PCI at the preoperative clinic visit and then using immersion US in the operating room before surgery. The data were compared to determine the degree of agreement.The charts of 18 patients (27 eyes) were reviewed. Preoperative AL measurements by PCI were obtained in 21 eyes (78%). On average, the PCI-measured ALs were 0.1 mm less than the immersion US values (95% confidence interval, -0.2 to -0.1; P = .002). All eyes with an AL of 23.5 mm or less had lower PCI values than immersion US values. There was no systematic pattern of 1 measurement being greater or less than the other in eyes with an AL longer than 23.5 mm.There was a systematic difference in AL measurement between PCI and immersion US, with PCI tending to give lower values, particularly in eyes with an AL of 23.5 mm or less. Depending on the length of the eye, a 0.1 mm error in AL measurement could result in a 0.25 to 0.75 diopter difference in intraocular lens calculation that could be clinically significant in some patients.

  View details for DOI 10.1016/j.jcrs.2010.07.013

  View details for Web of Science ID 000285429600014

  View details for PubMedID 21111313

• Toxic anterior segment syndrome after pediatric cataract surgery JOURNAL OF AAPOS Lambert, S. R. 2010; 14 (5): 381-382

  View details for DOI 10.1016/j.jaapos.2010.08.007

  View details for Web of Science ID 000283897900001

  View details for PubMedID 20863727

• Simultaneous vs Sequential Bilateral Cataract Surgery for Infants With Congenital Cataracts Visual Outcomes, Adverse Events, and Economic Costs ARCHIVES OF OPHTHALMOLOGY Dave, H., Phoenix, V., Becker, E. R., Lambert, S. R. 2010; 128 (8): 1050-1054

  Abstract

  To compare the incidence of adverse events and visual outcomes and to compare the economic costs of sequential vs simultaneous bilateral cataract surgery for infants with congenital cataracts.Retrospective review of simultaneous vs sequential bilateral cataract surgery for infants with congenital cataracts who underwent cataract surgery when 6 months or younger at our institution.Records were available for 10 children who underwent sequential surgery at a mean age of 49 days for the first eye and 17 children who underwent simultaneous surgery at a mean age of 68 days (P = .25). We found a similar incidence of adverse events between the 2 treatment groups. Intraoperative or postoperative complications occurred in 14 eyes. The most common postoperative complication was glaucoma. No eyes developed endophthalmitis. The mean (SD) absolute interocular difference in logMAR visual acuities between the 2 treatment groups was 0.47 (0.76) for the sequential group and 0.44 (0.40) for the simultaneous group (P = .92). Payments for the hospital, drugs, supplies, and professional services were on average 21.9% lower per patient in the simultaneous group.Simultaneous bilateral cataract surgery for infants with congenital cataracts is associated with a 21.9% reduction in medical payments and no discernible difference in the incidence of adverse events or visual outcomes. However, our small sample size limits our ability to make meaningful comparisons of the relative risks and visual benefits of the 2 procedures.

  View details for Web of Science ID 000280909700014

  View details for PubMedID 20697007

• Late spontaneous resolution of congenital Brown syndrome JOURNAL OF AAPOS Lambert, S. R. 2010; 14 (4): 373-375

  Abstract

  Brown syndrome is characterized by restricted elevation in adduction. Congenital Brown syndrome usually is diagnosed during early childhood. It is believed to be caused by an abnormality of the superior oblique tendon as it passes through the trochlea. The natural history of Brown syndrome is poorly characterized. Many patients with congenital Brown syndrome undergo strabismus surgery during childhood in an attempt to correct the problem surgically. This report describes spontaneous regression of congenital Brown syndrome in an adult.

  View details for DOI 10.1016/j.jaapos.2010.04.011

  View details for Web of Science ID 000281524600020

  View details for PubMedID 20637665

• Assessment of Risk Factors for Infantile Cataracts Using a Case-Control Study National Birth Defects Prevention Study, 2000-2004 OPHTHALMOLOGY Prakalapakorn, S. G., Rasmussen, S. A., Lambert, S. R., Honein, M. A. 2010; 117 (8): 1500-1505

  Abstract

  To identify risk factors for infantile cataracts of unknown etiology.Case-control study.Case infants (n = 152) and control infants (n = 4205) enrolled in the National Birth Defects Prevention Study for birth years 2000-2004.Multivariate analysis was performed exploring associations for risk factors for bilateral and unilateral infantile cataracts of unknown etiology.Infantile cataracts of unknown etiology.Maternal interviews were completed for 43 case infants with bilateral and 109 with unilateral infantile cataracts of unknown etiology. Very low birth weight (<1500 g) was associated with both unilateral (adjusted odds ratio [OR], 6.0; 95% confidence interval [CI], 2.2-16.3) and bilateral (OR, 13.2; 95% CI, 4.2-41.1) cataracts, whereas low birth weight (1500-2499 g) was only associated with bilateral cataracts (OR, 3.3; 95% CI, 1.3-8.1). Infants with unilateral cataracts were more likely to be born to primigravid women (OR, 1.6; 95% CI, 1.0-2.7) than women with > or =2 previous pregnancies, although this was of borderline significance. Although not significant, effect estimates were elevated suggesting a possible association between unilateral cataracts and maternal substance abuse during pregnancy, and between bilateral cataracts and urinary tract infection during pregnancy and aspirin use during pregnancy.Very low birth weight is associated with both bilateral and unilateral cataracts, whereas low birth weight is associated with bilateral cataracts and primigravidity with unilateral cataracts. Other associations, although not statistically significant, suggest risk factors that merit further research.

  View details for DOI 10.1016/j.ophtha.2009.12.026

  View details for Web of Science ID 000280598900006

  View details for PubMedID 20363508

• A Randomized Clinical Trial Comparing Contact Lens With Intraocular Lens Correction of Monocular Aphakia During Infancy Grating Acuity and Adverse Events at Age 1 Year ARCHIVES OF OPHTHALMOLOGY Lambert, S. R., Buckley, E. G., Drews-Botsch, C., DuBois, L., Hartmann, E. E., Lynn, M. J., Plager, D. A., Wilson, M. E., Bridgman, B., Celano, M., Cleveland, J., Cotsonis, G., Drews-Botsch, C., Freret, N., Lu, L., Nizam, A., Swanson, S., Tutu-Gxashe, T., Edwards, C., Busettini, C., Hayley, S., Everett, D. F., Russell, B., Ward, M. 2010; 128 (7): 810-818

  View details for Web of Science ID 000279871900001

• A randomized clinical trial comparing contact lens with intraocular lens correction of monocular aphakia during infancy: grating acuity and adverse events at age 1 year. Archives of ophthalmology Lambert, S. R., Buckley, E. G., Drews-Botsch, C., DuBois, L., Hartmann, E. E., Lynn, M. J., Plager, D. A., Wilson, M. E. 2010; 128 (7): 810-818

  Abstract

  To compare the visual outcomes and adverse events of contact lens with primary intraocular lens (IOL) correction of monocular aphakia during infancy.In a randomized, multicenter (12 sites) clinical trial, 114 infants with a unilateral congenital cataract were assigned to undergo cataract surgery between 1 to 6 months of age either with or without primary IOL implantation. Contact lenses were used to correct aphakia in patients who did not receive IOLs. Grating visual acuity was tested at 1 year of age by a masked traveling examiner.Grating visual acuity at 1 year of age.The median logMAR visual acuity was not significantly different between the treated eyes in the 2 groups (contact lens group, 0.80; IOL group, 0.97; P = .19). More patients in the IOL group underwent 1 or more additional intraocular operations than patients in the contact lens group (63% vs 12%; P < .001). Most of these additional operations were performed to clear lens reproliferation and pupillary membranes from the visual axis.There was no statistically significant difference in grating visual acuity at age 1 year between the IOL and contact lens groups; however, additional intraocular operations were performed more frequently in the IOL group.Until longer-term follow-up data are available, caution should be exercised when performing IOL implantation in children aged 6 months or younger given the higher incidence of adverse events and the absence of an improved short-term visual outcome compared with contact lens use.

  View details for DOI 10.1001/archophthalmol.2010.101

  View details for PubMedID 20457949

• From the Laboratory to the Clinic: Molecular Genetic Testing in Pediatric Ophthalmology AMERICAN JOURNAL OF OPHTHALMOLOGY Drack, A. V., Lambert, S. R., Stone, E. M. 2010; 149 (1): 10-17

  Abstract

  To review the current state of molecular genetic testing as it relates to pediatric ophthalmology and to discuss its uses.Review and evaluation of available molecular genetic testing.Literature review and discussion of testing in practice based on the authors' clinical and laboratory experience.Fee-for-service testing for many genetic eye diseases now is available. A report is always generated for fee-for-service testing. Detection of DNA variants in genes known to cause eye disease must be interpreted taking into account the variability of the human genome, the presence of benign variants (polymorphisms), and the carrier frequency of recessive alleles. Negative results in genetic testing are helpful in some disorders for which most of the causative genes are known and many disease-causing variants have already been reported, but are less helpful in those that currently have many undiscovered causative genes or novel mutations. Research-based testing also is available, but does not always yield a result. Patients with RPE65-associated Leber congenital amaurosis may be eligible for the current gene therapy trial. Patients with a variety of disorders may benefit from improved surveillance if their genetic diagnosis is known.Entry into the genetic testing system often is via the patient's ophthalmologist. Collaboration with geneticists and genetic counselors, use of web sites to keep up with the ever-changing availability and detection rates, and knowledge of clinical trials, when combined with excellent clinical diagnosis, can improve diagnosis and allow eligible patients to participate in treatment trials.

  View details for DOI 10.1016/j.ajo.2009.08.038

  View details for Web of Science ID 000273270300004

  View details for PubMedID 20103038

• The infant aphakia treatment study: design and clinical measures at enrollment. Archives of ophthalmology Lambert, S. R., Buckley, E. G., Drews-Botsch, C., DuBois, L., Hartmann, E., Lynn, M. J., Plager, D. A., Wilson, M. E. 2010; 128 (1): 21-27

  Abstract

  To compare the use of contact lenses and intraocular lenses (IOLs) for the optical correction of unilateral aphakia during infancy.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.Grating acuity at 12 months of age and HOTV visual acuity at 4 1/2 years of age.This study should determine whether either treatment for an infant with a visually significant unilateral congenital cataract results in a better visual outcome.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.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.

  View details for DOI 10.1001/archophthalmol.2009.350

  View details for PubMedID 20065212

• The value of serial personal photographs in timing the onset of unilateral cataracts in children JOURNAL OF AAPOS Sawhney, G. K., Hutchinson, A. K., Lambert, S. R. 2009; 13 (5): 459-462

  Abstract

  To determine the value of serial personal photographs in timing the onset of unilateral cataracts in children over 6 months of age.Personal photographs from children with unilateral cataracts who underwent cataract extraction and intraocular lens implantation when > or =6 months of age were reviewed. Photographs were evaluated for changes in the red reflex, which might indicate the presence of a cataract.Twelve children underwent cataract surgery at a mean age of 37 months. They were followed for a mean of 32 months. Ten children were diagnosed as having an acquired cataract by photographic review documenting a previously normal red reflex. The visual acuity in the affected eye of 4 of these children improved to > or =20/60. Cataracts were visible on photographs prior to clinical diagnosis in 6 patients, from 0.5 to 22 months prior to clinical diagnosis. Visual outcomes did not relate closely to the photographically documented duration of the cataract prior to treatment. Photographs were not helpful in timing the onset of cataract in 2 children due to the poor quality of the images.Serial personal photographs are sometimes helpful in determining whether cataracts are acquired. However, the usefulness of personal photographs alone in predicting the visual outcome after cataract surgery was limited in this small, retrospective study.

  View details for DOI 10.1016/j.jaapos.2009.08.007

  View details for Web of Science ID 000271051700007

  View details for PubMedID 19840724

• Outcomes of cataract surgery following radiation treatment for retinoblastoma JOURNAL OF AAPOS Payne, J. F., Hutchinson, A. K., Hubbard, B., Lambert, S. R. 2009; 13 (5): 454-458

  Abstract

  To report the outcomes after cataract extraction in eyes previously treated for retinoblastoma.We reviewed the medical records of consecutive patients treated with cataract extraction at our institution for radiation-induced cataract following retinoblastoma treatment between 1992 and 2007.Twelve eyes of 11 patients were included. The median age at diagnosis of retinoblastoma was 7.4 months. The median quiescent interval before cataract surgery was 34.6 months (range, 17-240 months). All patients underwent limbal-based extracapsular cataract extraction with scleral-tunnel wound construction. Anterior vitrectomy and posterior capsulotomy were performed in 7 eyes; an intraocular lens was placed in 10 eyes. A laser capsulotomy was subsequently performed in 5 eyes. No postoperative complications occurred. One patient underwent a pars plana vitrectomy for vitreous hemorrhage and proliferative retinopathy 5 years after cataract extraction. The median follow-up after surgery was 6.0 years (range, 1.1-12.3 years). Final visual acuity was between 20/20 and 20/60 in 6 eyes, between 20/70 and 20/200 in 2 eyes, and worse than 20/200 in 4 eyes. No intraocular tumor recurrences or metastases occurred. Two patients developed secondary rhabdomyosarcomas; 1 patient succumbed to the disease.Limbal-based cataract extraction in our series was not associated with tumor recurrence or metastasis. The optimal quiescent interval is not known; however, no tumor recurrences occurred in our series when a minimum 17-month quiescent period was achieved. Visual improvement was noted in every patient, although several patients only experienced modest gains.

  View details for DOI 10.1016/j.jaapos.2009.06.002

  View details for Web of Science ID 000271051700006

  View details for PubMedID 19840723

• Long-term visual outcome of penetrating keratoplasty in infants and children with Peters anomaly JOURNAL OF AAPOS Yang, L. L., Lambert, S. R., Drews-Botsch, C., Stulting, R. D. 2009; 13 (2): 175-180

  Abstract

  To determine the long-term visual outcome of penetrating keratoplasty for Peters anomaly and to identify prognostic factors affecting final vision.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.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 < 0.001) and larger donor corneas (p < 0.001) were independent predictors of poor outcome. Postoperative complications included graft failure (n = 44), cataract (n = 15), glaucoma (n = 14), retinal detachment (n = 16), and phthisis (n = 22). More than half of the eyes (n = 18) without graft failure, retinal detachment and/or phthisis saw 20/400 or better.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 (>or=8 mm) were the only independent predictors of a poor visual outcome.

  View details for DOI 10.1016/j.jaapos.2008.10.007

  View details for Web of Science ID 000265445100015

  View details for PubMedID 19393517

• Visual outcome following the reduction or cessation of patching therapy after early unilateral cataract surgery ARCHIVES OF OPHTHALMOLOGY Lambert, S. R., Plager, D. A., Lynn, M. J., Wilson, M. E. 2008; 126 (8): 1071-1074

  Abstract

  To evaluate the impact on visual acuity of reducing or abandoning patching therapy during the first 6 years of life following early unilateral cataract surgery.We reviewed the medical records of 9 children with unilateral congenital cataracts who underwent cataract surgery when 6 weeks or younger. All had good compliance with optical correction until 6 years of age and with patching therapy until at least 12 months of age.The children underwent cataract surgery at a mean (SD) age of 21.7 (9.5) days. At 12 months of age, the children were patched a mean (SD) of 6.7 (2.4) hours/d. Patching compliance declined steadily thereafter. By 6 years of age, they were only being patched a mean (SD) of 1.7 (2.0) hours/d. Four of the 9 children abandoned patching prior to the 6-year examination. Acuities improved or remained the same for 3 of these children but worsened for 1 child by 2 lines.Our study suggests that some children who undergo early unilateral cataract surgery and are compliant with their optical correction and patching during early childhood can maintain a good visual outcome even if patching therapy is reduced or discontinued prior to their sixth birthday.

  View details for Web of Science ID 000258377500007

  View details for PubMedID 18695101

• ASCRS white paper: Hydrophobic acrylic intraocular lenses in children JOURNAL OF CATARACT AND REFRACTIVE SURGERY Wilson, M. E., Trivedi, R. H., Buckley, E. G., Granet, D. B., Lambert, S. R., Plager, D. A., Sinskey, R. M., Vasavada, A. R. 2007; 33 (11): 1966-1973

  View details for DOI 10.1016/j.jcrs.2007.06.047

  View details for Web of Science ID 000250792900034

  View details for PubMedID 17964406

• Congenital rubella syndrome: the end is in sight BRITISH JOURNAL OF OPHTHALMOLOGY Lambert, S. R. 2007; 91 (11): 1418-1419

  View details for DOI 10.1136/bjo.2007.117960

  View details for Web of Science ID 000250229300003

  View details for PubMedID 17947261

• Longitudinal changes in the cylinder power of children with accommodative esotropia JOURNAL OF AAPOS Lambert, S. R., Lynn, M. 2007; 11 (1): 55-59

  Abstract

  To assess the longitudinal changes in the cylindrical refractive errors of children with accommodative esotropia according to the age when glasses were prescribed.Refractive errors were followed longitudinally for 120 children with accommodative esotropia for a mean of 4.4 +/- 2.5 years. Cycloplegic refractions were performed using an autorefractor. The refractive data were analyzed for three groups of children based on their age at the time spectacles were prescribed.In all three age groups, the mean cylinder power was initially about 1.0 D. Over time, the mean cylinder power in the two younger age groups gradually increased by about 0.5 D. In the oldest age group, the mean cylinder power remained relatively unchanged with some suggestion of a decrease in the later years. A regression model demonstrated the statistical significance of both the age when spectacles were prescribed (p < 0.0001) and the interaction of age and time after spectacles were prescribed (p = 0.0034).Longitudinal changes in cylinder power for children with accommodative esotropia vary according to their age when spectacle wear is initiated. The greatest increase in cylinder developed in the children who were youngest when initially treated.

  View details for DOI 10.1016/j.jaapos.2006.09.024

  View details for Web of Science ID 000244587300013

  View details for PubMedID 17307685

• Amblyopia in the phakic eye after unilateral congenital cataract extraction JOURNAL OF AAPOS Ruth, A. L., Lambert, S. R. 2006; 10 (6): 587-588

  Abstract

  The treatment of unilateral congenital cataract remains a challenge because form deprivation early in life leads to amblyopia. Visual outcomes after congenital cataract extraction have improved dramatically with earlier surgery, greater attention to optical correction of the aphakia, and part-time occlusion therapy of the phakic eye. A published review of unilateral congenital cataract studies between 1988 and 2004 found that 88% of patients with primary intraocular lens implantation achieved 20/200 or better visual acuity and a mean of 20% achieved 20/40 or better visual acuity. Improved outcomes are attributable, in part, to surgical intervention at an earlier age. It has been shown that surgical intervention during the first 6 weeks of age is associated with a better visual prognosis than surgery at a later age. In this report, we describe a patient with unilateral congenital cataract treated with cataract extraction and intraocular lens implantation at 8 weeks of age who had a better visual outcome in his operative eye than in his phakic eye.

  View details for DOI 10.1016/j.jaapos.2006.08.003

  View details for Web of Science ID 000243239000018

  View details for PubMedID 17189159

• Intracranial vascular anomalies in patients with morning glory disk anomaly AMERICAN JOURNAL OF OPHTHALMOLOGY Lenhart, P. D., Lambert, S. R., Newman, N. J., Biousse, V., Atkinson, D. S., Traboulsi, E. I., Hutchinson, A. K. 2006; 142 (4): 644-650

  Abstract

  An association between morning glory disk anomaly (MGDA) and intracranial vascular anomalies including Moyamoya disease has been recognized. We evaluated a series of patients with MGDA to ascertain the frequency of cerebrovascular anomalies.Retrospective observational case series.We reviewed the neurologic histories and neuroimaging studies of twenty patients with MGDA at two institutions between 1982 and 2004. Magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) of the brain was performed on all patients who had not undergone neuroimaging. MRI/MRA studies done for 40 pediatric patients without MGDA were also evaluated for cerebrovascular anomalies. The prevalence of anomalies in the two groups was compared by Fisher exact test.Nine of 20 patients (45%) with MGDA had cerebrovascular anomalies. Anomalies ranged from agenesis of the A1 segment of the anterior cerebral artery to bilateral stenosis of the internal carotid arteries with moyamoya disease. Three patients underwent revascularization procedures. Ten of 40 patients (25%) in the control group had any intracranial vascular anomaly, whereas only two of 40 (5%) had an abnormality of the anterior circulation, the most common finding in the MGDA group.We recommend that all patients with MGDA undergo MRI/MRA or computerized tomographic angiography to detect vascular and structural brain anomalies. It may be unclear whether cerebrovascular anomalies represent isolated congenital anomalies or findings of progressive occlusive cerebrovascular disease. Follow-up imaging should be considered in patients with cerebrovascular anomalies and is clearly indicated if neurologic signs or symptoms are present.

  View details for DOI 10.1016/j.ajo.2006.05.040

  View details for Web of Science ID 000241098900017

  View details for PubMedID 17011858

• Cryptic cerebral visual impairment in children BRITISH JOURNAL OF OPHTHALMOLOGY Lowery, R. S., Atkinson, D., Lambert, S. R. 2006; 90 (8): 960-963

  Abstract

  To review the medical records of seven children with a delayed diagnosis of cerebral visual impairment.The charts of a consecutive series of children examined in a university based ophthalmology clinic with a delayed diagnosis of cerebral visual impairment were reviewed. Their neuroimaging studies were evaluated by a paediatric neuroradiologist.The seven patients ranged in age from 2 years to 10 years at the time of presentation and had acuities ranging from 20/40 to 20/400. Their visual fields ranged from full visual fields to homonymous hemianopias. Neuroimaging findings ranged from subtle occipital lobe abnormalities to obvious cerebral volume loss.Cerebral visual impairment can be cryptic in children with mild visual impairment. Neuroimaging studies and visual field testing can help to differentiate this condition from other causes of visual impairment.

  View details for DOI 10.1136/bjo.094250

  View details for Web of Science ID 000239111000013

  View details for PubMedID 16687450

• Emmetropization in accommodative esotropia: an update and review. Comprehensive ophthalmology update Lowery, R. S., Hutchinson, A., Lambert, S. R. 2006; 7 (3): 145-149

  Abstract

  Many children with accommodative esotropia are able to successfully discontinue spectacle wear, while others require spectacle correction into adulthood. Parents often ask about the likelihood of glasses being required on a long-term basis and whether use of spectacles will cause dependency. Most infants are hyperopic and gradually become emmetropic. The extent to which accommodation and spectacle use affect this process is still debated. However, certain characteristics, such as degree of hyperopia, can help predict long-term spectacle requirement.

  View details for PubMedID 16882403

• Longitudinal changes in the spherical equivalent refractive error of children with accommodative esotropia BRITISH JOURNAL OF OPHTHALMOLOGY Lambert, S. R., Lynn, M. J. 2006; 90 (3): 357-361

  Abstract

  To assess the longitudinal changes in the spherical equivalent (SE) refractive errors of children with accommodative esotropia as a function of the age when glasses were prescribed.Refractive errors were followed longitudinally for 126 children with accommodative esotropia for a mean of 4.4 (SD 2.5) years. Cycloplegic refractions were performed using an autorefractor for older children and retinoscopy for younger children. The refractive data were analysed for three groups of children based on their age at the time spectacles were prescribed.The initial SE refractive error was age dependent (<2 years, 5.1 (1.9) D; 2-<4 years, 4.2 (1.9) D; 4-8 years, 3.8 (1.7) D). Children in all age groups had an initial increase in their SE refractive error, followed by a later decrease; however, the greatest decrease occurred in the patients in the oldest age group. The SE refractive error peaked 1 year after spectacles were prescribed for the children 4-8 years of age versus 6 years after spectacles were prescribed for the children less than 2 years of age.Longitudinal changes in SE refractive error for children with accommodative esotropia vary as a function of their age when spectacle wear is initiated.

  View details for DOI 10.1136/bjo.2005.078105

  View details for Web of Science ID 000235424800030

  View details for PubMedID 16488962

• Is there a latent period for the surgical treatment of children with dense bilateral congenital cataracts? JOURNAL OF AAPOS Lambert, S. R., Lynn, M. J., Reeves, R., Plager, D. A., Buckley, E. G., Wilson, M. E. 2006; 10 (1): 30-36

  Abstract

  It generally is accepted that cataract surgery during the first 6 weeks of life is associated with the best visual outcomes for children with dense unilateral congenital cataracts. The latent period for children with dense bilateral congenital cataracts has not been clearly defined.The best-corrected visual acuity (BCVA) at 4 to 6 years of age was collected retrospectively on a series of 43 children with dense bilateral congenital cataracts from 4 institutions. All of the children underwent a lensectomy, posterior capsulotomy, and anterior vitrectomy at 36 weeks of age or younger.Cataract surgery was performed at a mean age of 11.5 weeks for the better-seeing eye. BCVA was assessed when the children were a mean of 5.3 years of age. The visual acuities of 26 (60%) were 20/40 or better, 12 (28%) were 20/50 to 20/80, and 5 (12%) were 20/100 or worse. There was a trend for worse BCVA with increasing age at the time of surgery (r = 0.28, P = 0.07). We noted that a BCVA of 20/100 or worse occurred only among eyes undergoing surgery when infants were older than 10 weeks (10 weeks: 5/22 = 23%, P = 0.049). Children with preoperative nystagmus had worse visual outcomes; only 38% of children with preoperative nystagmus achieved a BCVA of 20/40 or better compared with 74% of children without preoperative nystagmus (P = 0.03).Previous reports have proposed that cataract surgery during the first 5 to 8 weeks of life is associated with better visual outcomes in children with dense bilateral congenital cataracts. Our results would suggest that good visual outcomes can be achieved beyond this age, but the incidence of poor visual outcomes increases if cataract surgery is delayed beyond 10 weeks of age. The absence of preoperative nystagmus is a better predictor of a good visual outcome than the age at surgery.

  View details for DOI 10.1016/j.jaapops.2005.10.002

  View details for Web of Science ID 000236083400007

  View details for PubMedID 16527677

• A prospective study of alternating occlusion before surgical alignment for infantile esotropia: One-year postoperative motor results JOURNAL OF AAPOS Ing, M. R., Norcia, A., Stager, D., Black, B., Hoffman, R., Mazow, M., Troia, S., Scott, W., Lambert, S. 2006; 10 (1): 49-53

  Abstract

  Alternating occlusion before surgical alignment has been suggested by some strabismologists as a possible method to enhance the treatment of infantile esotropia.This report presents 1-year outcome results of a prospective trial of patients with infantile esotropia who were assigned randomly to alternating occlusion or control groups.Forty-four patients with infantile esotropia were measured at entry into the study, at the time of surgery, at 6 weeks, and 1 year postoperatively. Alternating occlusion was full time and symmetric for those with no amblyopia but asymmetric for those with amblyopia. The control group that did not receive alternating occlusion had occlusion for amblyopia only. Initial surgeries were performed when the patients were between the ages of 6 and 13 months.Overall, the patients showed an increase of 9.14 prism diopters (PD) during a mean preoperative follow-up of 4.2 months. Seventy-five percent of all patients were aligned by the initial surgery. Ninety-one percent of those patients aligned within 10 PD at 6 weeks also were aligned 1 year postoperatively. The results were similar for the alternate occlusion group and the control group.In our sample of patients, alternating occlusion does not detectably alter the increase in angle of deviation between the dates of entry and the date of the initial surgical alignment procedure, nor does it influence the postoperative alignment at 6 weeks or at 1 year.

  View details for DOI 10.1016/j.jaapos.2005.09.006

  View details for Web of Science ID 000236083400010

  View details for PubMedID 16527680

• Conjunctival granulomas caused by synthetic fibers: Report of two cases and review of literature JOURNAL OF AAPOS Schmack, I., Kang, S. J., Grossniklaus, H. E., Lambert, S. R. 2005; 9 (6): 567-571

  Abstract

  We sought to demonstrate the histopathologic and ultrastructural features of conjunctival foreign body granulomas because of synthetic fibers and to compare them to other cases published in the literature.A 2- and a 7-year-old girl were referred for the surgical removal of slow-growing unilateral inferior conjunctival masses with a lack of primary trauma or surgery. In this report, we describe the light and electron microscopic findings of the 2 cases and review the literature of similar cases using the Medline database.Histopathologic and ultrastructural examination of both specimens revealed a granulomatous inflammatory cell response, including histiocytes and multinucleated foreign body giant cells around acellular, uniform sized, oval to round birefringent fibers with manufacturing artifacts. Thirteen other patients with conjunctival synthetic fiber granulomas were identified from the literature.On the basis of the findings in our cases and the review of literature, it appears that conjunctival synthetic fiber granulomas are not a rare entity but are not recognized frequently by ophthalmologists. The most reliable clinical sign to suggest this diagnosis is the presence of a unilateral inferior conjunctival mass in a child or adolescent. Histopathologic and ultrastructural evaluation appears to be the only way to specifically diagnose this condition with certainty.

  View details for DOI 10.1016/j.jaapos.2005.07.006

  View details for Web of Science ID 000234776400014

  View details for PubMedID 16414525

• Iris hypoplasia and aorticopulmonary septal defect: A neurocristopathy JOURNAL OF AAPOS Bergstrom, C. S., Saunders, R. A., Hutchinson, A. K., Lambert, S. R. 2005; 9 (3): 264-267

  Abstract

  Errors in neural crest development are responsible for a number of ocular disorders. In some cases, these ocular abnormalities may be associated with systemic disorders, some of which may be life threatening.We reviewed the medical record of 3 children from 2 institutions with a similar constellation of ocular and cardiac abnormalities.All 3 children had iris hypoplasia with pupils 6-7 mm in diameter that had minimal or no response to light or pharmacological agents. The ocular examinations were otherwise normal. All 3 children also had large aorticopulmonary septal defects which were surgically repaired.We report an association between iris hypoplasia and aorticopulmonary septal defects. Like the iris stroma, the aorticopulmonary septum forms from neural crest cells during embryogenesis which divide the truncus arteriosus into the ascending aorta and pulmonary trunk. Children with iris hypoplasia should undergo an immediate cardiac evaluation. It is important that opthalmologists be aware of the association between these two disorders since an aorticopulmonary septal defect is a life-threatening disorder.

  View details for Web of Science ID 000230049700012

  View details for PubMedID 15956947

• Trigeminal nerve hypoplasia and aplasia in children with Goldenhar syndrome and corneal hypoesthesia JOURNAL OF AAPOS VIllanueva, O., Atkinson, D. S., Lambert, S. R. 2005; 9 (2): 202-204

  View details for DOI 10.1016/j.jappos.2004.12.016

  View details for Web of Science ID 000228808400022

  View details for PubMedID 15838455

• Neonatal aphakia retards ocular growth and alters scleral gene expression in rhesus monkeys MOLECULAR VISION Tarnuzzer, R. W., Fernandes, A., Iuvone, P. M., Lambert, S. R. 2005; 11 (2-4): 36-49

  Abstract

  We hypothesize that remodeling of the scleral extracellular matrix, involving collagen and proteoglycan synthesis and turnover, is a key process involved in ocular growth. Decreased axial elongation is observed following neonatal removal of the crystalline lens in a rhesus monkey model of congenital cataract. We wanted to determine changes in gene expression in the operated and companion eye following lensectomy, especially for extracellular matrix in the sclera.Between 4 and 7 days of age, infant monkeys underwent surgical removal of the lens from the right eye. Axial lengths of the operated and unmanipulated fellow eyes were measured and when interocular differences of >0.4 mm were achieved, monkeys were sacrificed and RNA was isolated from sclera. In order to determine changes in scleral gene expression in aphakic versus control eyes, we used Clontech's Atlas Gene Array (Human Cancer Array version 1.2) hybridized with total RNA from three monkeys.Atlas Gene Array analysis demonstrated differential expression of several genes in the operated versus the unmanipulated eye. Most notably, there was a statistically significant increase in expression of several extracellular matrix (ECM) genes including: aggrecan, decorin, biglycan, several collagens, and tenascin in the RNA from the sclera of the aphakic eyes when compared to the unmanipulated eyes. Genes for several matrix metalloproteinases (MMPs) showed no significant change following lens removal although there was a trend towards decreased expression. There were also statistically significant changes in the pattern of gene expression in the operated eye relative to the unmanipulated eye for cell adhesion, cell cycle, apoptosis, and cytoskeleton transcripts.Our results suggest that removal of the crystalline lens alters gene expression in the sclera with a prominent upregulation of ECM transcripts. These data support recent evidence that remodeling of the ECM composition of the sclera may be an important regulator of ocular growth.

  View details for Web of Science ID 000226405100003

  View details for PubMedID 15660023

• A prospective study of alternating occlusion prior to surgical alignment for infantile esotropia: one-year postoperative motor results. Transactions of the American Ophthalmological Society Ing, M. R., Norcia, A., Stager, D., Black, B., Hoffman, R., Mazow, M., Troia, S., Scott, W., Lambert, S. 2005; 103: 31-35

  Abstract

  Alternating occlusion prior to surgical alignment has been suggested by some strabismologists to possibly enhance the treatment of infantile esotropia. This report presents the data for 44 patients prospectively enrolled by random assignment to an alternating occlusion or no occlusion subgroup followed for 1 year postoperatively.All patients were measured at entry into the study, at the time of surgery, and at 6 weeks and 1 year postoperatively. Alternating occlusion was full-time and symmetric for those with no amblyopia but asymmetric for those with amblyopia. The subgroup that did not receive alternating occlusion had occlusion for amblyopia only. Initial surgeries were performed between the ages of 6 and 13 months.The patients, as a whole, showed a significant increase of 9.14 prism diopters when followed for a mean of 4.2 months prior to initial surgery (P < .00027). Seventy-five percent of all patients were aligned by the initial surgery. Ninety-one percent of those patients aligned at 6 weeks were also aligned at the 1-year postoperative date. The results were similar for both the control group and the patients treated with alternating occlusion.In our sample of patients, alternating occlusion does not detectably alter the increase in angle of deviation between the dates of entry and the date of the initial surgical alignment procedure, nor does it influence the postoperative alignment at 6 weeks or at 1 year.

  View details for PubMedID 17057785

• Optotype acuity and re-operation rate after unilateral cataract surgery during the first 6 months of life with or without IOL implantation BRITISH JOURNAL OF OPHTHALMOLOGY Lambert, S. R., Lynn, M., Drews-Botsch, C., Dubois, L., Plager, D. A., Medow, N. B., Wilson, M. E., Buckley, E. G. 2004; 88 (11): 1387-1390

  Abstract

  To compare optotype acuities and re-operation rates in children corrected with a contact lens (CL) compared with an intraocular lens (IOL) following unilateral cataract extraction during infancy in a non-randomised, retrospective case series.25 infants with a unilateral congenital cataract underwent cataract surgery with (IOL group, n = 12) or without (CL group, n = 13) IOL implantation when <7 months of age. Optotype acuities were assessed in 19 of these children at a mean age of 4.3 years (range 3.3-5.5 years). The number of re-operations were assessed in 21 children.The visual acuity results were similar in the two treatment groups (p = 0.99); however, two of the four (50%) children in the IOL group compared with two of the seven (28%) children in the CL group undergoing surgery during the first 6 weeks of life had 20/40 or better visual acuity. The children in the IOL group had more re-operations than the children in the CL group (mean 1.1 v 0.36). Most of the re-operations in the IOL group were membranectomies performed during the first year of life (median 8.0 months) whereas all of the re-operations in the CL group were the implantation of a secondary IOL later in childhood (mean 2.2 years).Optotype acuities were similar for the children corrected with a CL compared with IOL, while the children in the IOL group underwent more re-operations .

  View details for DOI 10.1136/bjo.2004.045609

  View details for Web of Science ID 000224510500008

  View details for PubMedID 15489478

• Longitudinal changes in the refractive errors of children with tears in Descemet's membrane following forceps injuries JOURNAL OF AAPOS Lambert, S. R., Drack, A. V., Hutchinson, A. K. 2004; 8 (4): 368-370

  Abstract

  Eyes with tears in Descemet's membrane secondary to forceps injuries frequently develop myopic astigmatism. Little is known regarding the longitudinal changes in the refractive errors of these eyes.We performed a retrospective review of two children with tears in Descemet's membrane following forceps injuries to their left eyes. The refractive errors were followed longitudinally.Both patients developed myopic astigmatism in their affected eye and received optical correction and occlusion therapy. In both cases there was reduction in the myopic refractive error during early childhood. While the astigmatic refractive error was stable in one patient it decreased in the second patient.Myopia decreased in two children with myopic astigmatism secondary to tears in Descemet's membrane. Myopia in children with tears in Desemet's membrane is likely due to deformation of the cornea rather than form-deprivation.

  View details for DOI 10.1016/j.jaapos.2004.04.008

  View details for Web of Science ID 000223355600013

  View details for PubMedID 15314599

• Treatment of congenital cataract BRITISH JOURNAL OF OPHTHALMOLOGY Lambert, S. R. 2004; 88 (7): 854-855

  View details for DOI 10.1136/bjo.2004.045401

  View details for Web of Science ID 000222093100002

  View details for PubMedID 15205222

• Surgical management of glaucoma in infants and children with Peters' anomaly - Long-term structural and functional outcome OPHTHALMOLOGY Yang, L. L., Lambert, S. R., Lynn, M. J., Stulting, R. D. 2004; 111 (1): 112-117

  Abstract

  To determine the long-term outcome of surgery for congenital glaucoma in infants and children with Peters' anomaly.Retrospective review of a consecutive interventional case series.An urban academic tertiary referral institution.Thirty-four eyes of 19 children are subjects of this report. Included are all children 12 years of age or younger with Peters' anomaly who underwent surgery for primary congenital glaucoma between January 1971 and December 1992 and completed a minimum of 3 years of follow-up from the date of the first glaucoma surgery.The surgical procedures performed were trabeculectomy, trabeculotomy, goniotomy, Molteno shunt implantation, cyclodialysis, and cyclocryotherapy.Primary outcome measures were intraocular pressure (IOP) control and final postoperative visual acuity. Intraocular pressure control was defined as complete success (IOP21 mmHg with or without antiglaucoma medication, inoperable retinal detachment, phthisis, or chronic hypotony, defined as an IOP of

  View details for DOI 10.1016/j.ophtha.2003.02.002

  View details for Web of Science ID 000187806800018

  View details for PubMedID 14711722

• Intraocular lens implantation during infancy: Perceptions of parents and the American Association for Pediatric Ophthalmology and Strabismus members JOURNAL OF AAPOS Lambert, S. R., Lynn, N., Drews-Botsch, C., Dubois, L., Wilson, M. E., Plager, D. A., Wheeler, D. T., Christiansen, S. P., Crouch, E. R., Buckley, E. G., Stager, D., Donahue, S. P. 2003; 7 (6): 400-405

  Abstract

  To determine whether a randomized clinical trial, the Infant Aphakia Treatment Study, comparing intraocular lens (IOL) implantation with contact lens (CL) correction for infants with a unilateral congenital cataract (UCC), is feasible by (1) ascertaining whether American Association for Pediatric Ophthalmology and Strabismus (AAPOS) members have equipoise regarding these two treatments and (2) evaluating the willingness of parents to agree to randomization.All AAPOS members were surveyed in August 1997 and again in June 2001 regarding their use of CLs and IOL implants to correct infants vision after unilateral cataract surgery. In addition, a pilot study was begun in March 2002 to evaluate the safety of IOL implantation during infancy and the willingness of parents to randomize their children with a UCC to either IOL implantation or CL correction.In 1997, 89% of the 260 respondents reported that in the previous year they had treated at least one infant with a UCC, but only 4% had implanted an IOL in an infant <7 months old. Silsoft (Bausch & Lomb, Rochester, NY) CL correction was the preferred treatment choice for 84% of the respondents. In 2001, 21% of the 279 respondents had implanted an IOL in an infant. On a scale from 1 to 10 with 1 strongly favoring an IOL implant and 10 strongly favoring a CL, the median score was 7.5. Sixty-one percent of the respondents indicated that they would be willing to randomize children with a UCC to one of these two treatments. The main concerns about IOL implantation were poor predictability of power changes, postoperative complications, inflammation, and technical difficulty of surgery. The main concerns about CL correction were poor compliance, high lens loss rate, high cost, and keratitis. In our pilot study, 30 infants <7 months of age were evaluated at nine clinical centers for a visually significant UCC. Of 24 infants eligible for randomization, the parents of 17 (71%) agreed to randomization.Although most AAPOS members still favor CL correction after cataract surgery for a UCC, five times as many had implanted an IOL in an infant in 2001 compared with the number in 1997. Parents were almost equally divided in their preference for IOL implant versus CL correction. Given the relative equipoise of AAPOS members regarding these treatments and the willingness of more than two thirds of parents to agree to randomization, it seems likely that a randomized clinical trial comparing these two treatments could indeed be conducted.

  View details for DOI 10.1016/j.jaapos.2003.08.004

  View details for Web of Science ID 000187624700007

  View details for PubMedID 14730292

• Parenting stress among caregivers of children with congenital cataracts JOURNAL OF AAPOS Drews, C., Celano, M., Plager, D. A., Lambert, S. R. 2003; 7 (4): 244-250

  Abstract

  To examine parenting stress among caregivers of young children with congenital cataracts and to assess whether diagnostic and/or treatment differences are associated with differences in perceived parenting stress.Parents of 41 preschool-age children with congenital cataracts (13 with bilateral cataracts [BCCs] and 28 with unilateral cataracts [UCCs], of whom 14 were aphakic and 14 were pseudophakic) completed the Parenting Stress Index (PSI) and/or a disease-specific parental stress measure, ie, the Ocular Treatment Index (OTI).The 28-item OTI had excellent internal consistency (alpha = 0.94) and supported three of four a priori validity hypotheses. Parents of children with congenital cataracts reported normal parenting stress levels on the PSI. Parents of children with UCCs tended to report higher levels of stress, but not significantly so, than did parents of children with BCCs. Among parents of children with UCCs, those whose children were aphakic reported higher levels of stress on the OTI and all of the PSI subscales than did parents of pseudophakic children. These differences were statistically significant for two subscales (Adaptability [P =.03] and Mood [P =.01]).Although parents of children with congenital cataracts generally did not report increased parenting stress levels, clinicians should be aware that parenting stress can adversely impact patients' families. We did observe higher stress levels in parents with children who had UCCs and did not receive an intraocular lens-particularly stress related to their child's reaction to sensory stimulation and mood-compared with parents of pseudophakic children. Thus, clinicians may want to consider parenting stress levels when choosing a treatment for children with UCCs, especially because such stress has been associated with poor treatment compliance for children with other chronic conditions.

  View details for DOI 10.1016/S1091-8531(03)00118-6

  View details for Web of Science ID 000184856500005

  View details for PubMedID 12917610

• Congenital iris coloboma repair using a modified McCannel suture technique AMERICAN JOURNAL OF OPHTHALMOLOGY Blackmon, D. M., Lambert, S. R. 2003; 135 (5): 730-732

  Abstract

  To report a repair of congenital iris coloboma using a modified McCannel suture technique.Interventional case report.Retrospective intervention procedure.Postoperative repair resulted in restoration of pupil, anterior support of posterior chamber intraocular lens, and improved appearance.The modified McCannel suture technique provides acceptable functional and cosmetic repair of congenital iris colobomas.

  View details for Web of Science ID 000182477800034

  View details for PubMedID 12719095

• Clinical features predictive of successfully weaning from spectacles those children with accommodative esotropia JOURNAL OF AAPOS Lambert, S. R., Lynn, M., Sramek, J., Hutcheson, K. A. 2003; 7 (1): 7-13

  Abstract

  It has been reported that most children with accommodative esotropia are not able to discontinue spectacle wear as they become older. We conducted a prospective study to determine which factors are predictive of successfully weaning children from spectacles.Beginning in 1995, children with fully accommodative esotropia with a baseline refractive error of + 1.50 to + 5.00 diopters (D) were gradually weaned from their hyperopic correction. Patients with amblyopia or who had previously undergone strabismus surgery were excluded. Children were weaned in 0.50 D increments until spectacles were discontinued or they developed esotropia, asthenopia, or decreased vision. A multivariate analysis was performed to assess the association between successful weaning and various clinical characteristics.Twelve of 20 children (60%) were successfully weaned from spectacles. Spectacles were prescribed at a mean age of 4.2 +/- 1.5 years, and weaning was initiated at a mean age of 8.0 +/- 1.1 years. The spherical equivalent of the least hyperopic eye when spectacles were prescribed was 2.99 +/- 1.06 D. The clinical characteristic most clearly associated with successful weaning was the refractive error at the time glasses were prescribed. Whereas 10 of 11 (91%) patients with < 3 D of hyperopia were weaned from spectacles, only 2 of 9 (22%) patients with 3 to 5 D of hyperopia were successfully weaned from their spectacles (P =.005).Many children with fully accommodative esotropia can be weaned out of spectacles during the grade school years. The degree of baseline hyperopia appears to be one of the best predictors of success.

  View details for DOI 10.1016/S1091-8531(02)42010-1

  View details for Web of Science ID 000181952900002

  View details for PubMedID 12690363

• Weaning children with accommodative esotropia out of spectacles: a pilot study BRITISH JOURNAL OF OPHTHALMOLOGY Hutcheson, K. A., Ellish, N. J., Lambert, S. R. 2003; 87 (1): 4-7

  Abstract

  Many children with accommodative esotropia must continue spectacle use throughout life. This study was undertaken to determine which factors are predictive of successfully weaning children with accommodative esotropia out of spectacles.A retrospective review of 10 children with accommodative esotropia, who were gradually weaned from their hyperopic correction, and three age matched controls was performed. The main outcome measure was resolution or non-resolution of esotropia following weaning and eventual discontinuation of spectacles. Secondary outcome measures were final refractive error and the final esotropic or esophoric angle without correction.Six patients were successfully weaned from spectacles. At the completion of the weaning period one child was orthophoric and the other five children had well controlled esophorias. The other four patients remained spectacle dependent because of persistent esotropia or decreased vision without spectacles. The baseline and final refractive errors were significantly lower in the children successfully weaned from spectacles (p = 0.014). While the children who were successfully weaned from spectacles were older when initially diagnosed with accommodative esotropia (4.6 v 2.5 years), this difference was not statistically significant (p = 0.09).Some children with accommodative esotropia may be weaned out of spectacles during the grade school years with resolution of their esotropia. It is likely that gradual reduction of the hyperopic correction increases divergence amplitudes, but it is unclear whether this facilitates emmetropisation.

  View details for Web of Science ID 000180180700003

  View details for PubMedID 12488252

• The use of an image guidance system in retrieving lost medial rectus muscles JOURNAL OF AAPOS Srivastava, S. K., Reichman, O. S., Lambert, S. R. 2002; 6 (5): 309-314

  Abstract

  Finding a lost medial rectus muscle can be difficult. We present 2 patients in whom image guidance technology assisted in the recovery of a lost medial rectus muscle.Two patients (ages 19 and 33), both with lost medial rectus muscles, underwent computerized tomography of their heads, faces, and orbits. The muscles were both lost after strabismus surgery 7 and 19 years ago, respectively. These images were then loaded into an image guidance system (LandmarX, Xomed Co, Jacksonville, Fla) and served as a "map" to locate the lost medial rectus muscle intraoperatively. Using a transnasal endoscopic approach, the lamina papyracea was exposed. The area of the lamina closest to the lost medial rectus was identified using a guidance probe. After the periorbita was exposed, the guidance probe was used to confirm the location of the medial rectus muscle. The muscle was then isolated transnasally and passed into the orbit using forceps. It was then sutured onto the globe.The medial rectus was identified transnasally and reattached to the globe in both cases. Adduction improved from complete deficits in both patients to 80% and 50% adduction deficits, respectively.The transnasal endoscopic approach to retrieve the medial rectus has been described before. For these patients, image guidance technology successfully identified the appropriate areas for the surgeons to locate the lost muscle. The use of this technique decreased operative times and reduced the extent of the dissection required, which should also reduce long-term scarring.Image guidance technology, with its capability of 3-dimensional images, can be used to assist surgeons in finding lost medial rectus muscles.

  View details for DOI 10.1067/mpa.2002.127117

  View details for Web of Science ID 000178745800007

  View details for PubMedID 12381990

• Strabisms - Are there more exotropes than esotropes in Hong Kong? BRITISH JOURNAL OF OPHTHALMOLOGY Lambert, S. R. 2002; 86 (8): 835-836

  View details for Web of Science ID 000177249200001

  View details for PubMedID 12140194

• Clinical outcome of confluent laser photoablation for retinopathy of prematurity JOURNAL OF AAPOS Fallaha, N., Lynn, M. J., AABERG, T. M., Lambert, S. R. 2002; 6 (2): 81-85

  Abstract

  During the past decade, laser photoablation has supplanted cryotherapy as the standard treatment for threshold retinopathy of prematurity (ROP). We evaluated the progression and the complication rate in a cohort of infants with threshold ROP treated with a confluent technique.We retrospectively studied 47 patients with ROP (91 eyes), treated with confluent diode laser photoablation. Four main outcomes were evaluated: (1) the rate of progression, (2) the frequency of laser retreatment, (3) postoperative complications, and (4) postoperative refractive error.A mean of 1943 +/- 912 laser burns were administered in a confluent pattern to 91 eyes with threshold ROP. Progression to stage 4 or 5 disease occurred in 13 of 29 eyes (44.8%) with zone I and posterior zone II and in 2 of 51 eyes (3.9%) with anterior zone II ROP (P =.01). Eyes with anterior zone II ROP that received more than or equal to 2000 burns progressed more than those that received fewer than 2000 burns. Only 1 eye (1%) needed a supplemental laser treatment. Postoperative complications included corneal edema (2.3%), anterior segment ischemia (2.3%), vitreous hemorrhage (7.9%), posterior synechiae (2.3%), cataract (4.9%), and macular ectopia (12%). The mean spherical equivalent at the last follow-up was -4.52 +/- 5.63 D.In our series, infants received more laser burns than infants reported in the literature receiving scatter or near-confluent treatment. While confluent treatment almost eliminated supplemental treatment, it was associated with a similar rate of progression and complications as has been reported with other patterns of laser treatment.

  View details for DOI 10.1067/mpa.2002.121452

  View details for Web of Science ID 000175625800005

  View details for PubMedID 11997803

• A Randomized trial of atropine vs patching for treatment of moderate amblyopia in children ARCHIVES OF OPHTHALMOLOGY GLASER, S. R., Matazinski, A. M., Sclar, D. M., Sala, N. A., Vroman, C. M., Tanner, C. E., Stager, D. R., Berry, P. M., Stager, D. R., Felius, J., Wilkerson, J. A., Pesheva, M. P., Birch, E. E., Jeffrey, B. G., O'Connor, A. R., Tien, D. R., Bulan, G. E., Christ, H. C., DeWaele, L. B., Young, D. A., Astle, W. F., Ells, A. L., Hayduk, C. R., Kerr, C. I., McAlester, M. S., Peddie, H. J., Vibert, H. M., Hertle, R. W., Mellow, S. D., FitzGibbon, E. J., Foster, G. E., Arnold, R. W., Armitage, M. D., Brusseau, N. H., Ruttum, M. S., Kivlin, J. D., Picard, V. R., Chesner, M. J., Cotter, S. A., Barnhardt, C. N., Shin, S. M., Chu, R. H., Asiain, L., Flores, Y. F., Lee, G., Lee, J. H., Fort, S. C., Slutsky, J. L., Paysse, E. A., Coats, D. K., Brady-McCreery, K. M., Sanchez, A. D., Correodor, V., Donahue, S., Foss, C., Ozier, J. A., Biernacki, R. J., Tomlinson, E., Wheeler, D. T., Beaudet, K. A., Bateman, C. L., Hartwell, M. A., Ruben, J. B., Desai, D., Parrish, S. A., Louie, T. D., Rutstein, R. P., Marsh-Tootle, W. L., Baldwin, C. H., Becker, K. T., Repka, M. X., Hunter, D. G., Mattheu, J. S., Broome, S. O., Goodman, C. R., Neely, D. E., Plager, D. A., Sprunger, D. T., Bates, D. J., Galli, J., Whitaker, M. E., Tamkins, S. M., Gonzalez, M., Jacobs, S., Collins, M. L., McCarus, C. L., Brown, J. N., Conlan, D. B., Lambert, S. R., Yang, L., Elliott, A. T., Fallaha, N., Cruz, O. A., Davitt, B. V., Havertape, S. A., Miyazaki, E. A., Bosch, M. B., Levada, A. J., Matchett, T. L., Moya, A. Z., Mulligan, C. C., Weiss, S. K., Pelletier, H. J., Droste, P. J., Peters, R. J., Hilbrands, J., Sheeran, K. A., Smith, D. K., Vanraze-nswaay, C. L., Weakley, D. R., Dias, C. L., Johnson, D. A., James, R. D., Claes, P. G., Drake, K. K., Holmes, J. M., Nielsen, B. A., Garcia, M., Kroening, R. M., Leske, D. A., Levisen, M. L., Miller, D. K., Priebe, D. M., Spitzer, J. A., Scheiman, M. M., Bailey, J. A., Zinzer, K. T., Kulp, M. T., Kitts, T. L., Earley, M. J., Awner, S., Olitsky, S. E., Silbert, D. I., Wagner, A. E., Castillo, K. M., Matta, N. S., Meshey, T. L., Myers-Ely, P., Piper, W. L., Scaringi, D. M., Snavely, P. M., Walker, L. J., Burns, C. L., Barsoum-Homsy, M., Lawrence, L. I., Wallace, D. K., Sullivan, M. J., MILLER, J. M., Aparisi, T. A., Funk-Weyant, J., Taylor, M., Bulau, S., Scott, W. E., Ottar-Pfeifer, W. I., Kutschke, P. J., Wilken, K. M., Summers, C. G., Christiansen, S. P., Holleschau, A. M., Cook, S. M., LaVoie, J. D., Merrill, K. S., Elsas, F. J., Metz, T. H., Mizell, M. L., Bennett, S. O., Crouch, E. R., Ruark, K. D., Ventura, G. G., Paciuc, M., Schnadower, M. M., Velasco, C., Dries, D. C., Vengco, V. J., Olson, R. J., Hoffman, R. O., Bracken, S. F., Remington, P. L., Yen, K. G., Wiggins, R. E., Baumgartner, S. A., Knecht, M., Constance, C. E., Benson, S. L., Hahn-Parrott, L. A., Motley, W. E., Poole, R. M., Forbes, B. J., Quinn, G. E., Ehnbom, M. L., Maturo, M. C., Phillips, D. R., Miller, M. M., Maybodi, M., Greger, C., Magoon, E. H., Kannam, P. A., McAtee, L. A., Andrews, M., Hoge, C. M., Saunders, R. A., Hoxie, J. P., Langdale, L. M., Lenhart, K. D., Moore, B., Weissberg, E. M., Duckman, R. H., Fitzgerald, D. E., Vricella, M., Beck, R. W., Moke, P. S., Blair, R. C., Cole, S. R., Kraker, R. T., Gillespie, H. A., Boyle, N. M., Lawson, A. N., Gillett, J. A., Mares, S. T., Dale, B. B., Everett, D. F., Birch, E. E., Hertle, R. W., Holmes, J. M., Repka, M. X., Quinn, G. E., Saunders, R. A., SIMONS, K., Cotter, S. A., Scheiman, M. M., MILLER, J. M., Pesheva, M. P., Picard, V. R., Sala, N. A., Tien, R., Barlow, W., Buckley, E. G., Davis, B., Dobson, V., Keltner, J. L., Osman, H., Palmer, E. A., Phelps, D. L. 2002; 120 (3): 268-278

  Abstract

  To compare patching and atropine as treatments for moderate amblyopia in children younger than 7 years.In a randomized clinical trial, 419 children younger than 7 years with amblyopia and visual acuity in the range of 20/40 to 20/100 were assigned to receive either patching or atropine at 47 clinical sites.Visual acuity in the amblyopic eye and sound eye after 6 months.Visual acuity in the amblyopic eye improved in both groups (improvement from baseline to 6 months was 3.16 lines in the patching group and 2.84 lines in the atropine group). Improvement was initially faster in the patching group, but after 6 months, the difference in visual acuity between treatment groups was small and clinically inconsequential (mean difference at 6 months, 0.034 logMAR units; 95% confidence interval, 0.005-0.064 logMAR units). The 6-month acuity was 20/30 or better in the amblyopic eye and/or improved from baseline by 3 or more lines in 79% of the patching group and 74% of the atropine group. Both treatments were well tolerated, although atropine had a slightly higher degree of acceptability on a parental questionnaire. More patients in the atropine group than in the patching group had reduced acuity in the sound eye at 6 months, but this did not persist with further follow-up.Atropine and patching produce improvement of similar magnitude, and both are appropriate modalities for the initial treatment of moderate amblyopia in children aged 3 to less than 7 years.

  View details for Web of Science ID 000174300900001

  View details for PubMedID 11879129

• Diffusion-weighted magnetic resonance imaging in shaken baby syndrome AMERICAN JOURNAL OF OPHTHALMOLOGY Biousse, V., Suh, D. Y., Newman, N. J., Davis, P. C., Mapstone, T., Lambert, S. R. 2002; 133 (2): 249-255

  Abstract

  To evaluate the role of diffusion-weighted magnetic resonance imaging (DWIMRI) in the diagnosis and management of children with suspected or confirmed Shaken Baby Syndrome (SBS).This was a retrospective interventional case series of all infants and children younger than 2 years of age admitted to a children's hospital. We retrospectively reviewed medical records and neuroimaging findings of all children younger than 2 years of age with confirmed or suspected SBS admitted to a children's hospital. Inclusion criteria were documented ocular examination by an ophthalmologist and a brain MRI with DWI. Twenty-six infants and children were included. Other children were excluded. Children with proven SBS were diagnosed with "confirmed SBS," while children in whom the diagnosis of SBS remained uncertain were diagnosed with "suspected SBS."Twenty-six infants and children with mean age of 7.1 months (range, 6 weeks-24 months) were included, 18 with confirmed SBS. All 26 patients had a subdural hematoma, 10 had associated occult bone fractures, and 18 had retinal hemorrhages. Seven of the eight cases without retinal hemorrhages had isolated subdural hematoma without parenchymal brain lesions on both conventional MRI and DWIMRI. SBS was confirmed in only one case with a normal fundus. Among the 18 patients with retinal hemorrhages, SBS was confirmed in all but one case. All 18 patients with confirmed SBS had an abnormal DWIMRI. In 13 patients, DWI showed lesions that were larger than on conventional MRI. In patients with brain parenchymal lesions, the DWIMRI characteristics suggested cerebral ischemia, which appears to play a major role in SBS.In all patients with confirmed SBS, DWIMRI was abnormal and suggested diffuse or posterior cerebral ischemia, in addition to subdural hematomas in the pathogenesis of this disorder.

  View details for Web of Science ID 000173669900011

  View details for PubMedID 11812430

• Pediatric cataracts. Ophthalmology clinics of North America Fallaha, N., Lambert, S. R. 2001; 14 (3): 479-492

  Abstract

  The management of infantile cataracts continues to be a challenging task, however with new surgical techniques, IOL implantation, and new patching regimens, the visual outcomes are improving.

  View details for PubMedID 11705148

• Accommodative esotropia. Ophthalmology clinics of North America Lambert, S. R. 2001; 14 (3): 425-432

  Abstract

  Accommodative esotropia is the most common type of strabismus with a very favorable prognosis if the appropriate treatment is initiated promptly. Spectacles remain the preferred treatment, but both contact lenses and refractive surgery may be helpful in some situations to correct the associated hyperopia. Strabismus surgery should only be used if optical correction or refractive surgery are ineffective in restoring normal ocular alignment.

  View details for PubMedID 11705142

• Peters' anomaly. A synopsis of surgical management and visual outcome. Ophthalmology clinics of North America Yang, L. L., Lambert, S. R. 2001; 14 (3): 467-477

  Abstract

  Peters' anomaly is not an isolated anterior segment abnormality, but occurs as a diverse, phenotypically heterogeneous condition associated with multiple underlying ocular and systemic defects. Surgical treatment of Peters' anomaly is a complex and challenging undertaking. The broad spectrum of disease severity, the lack of uniformity in clinical features, the differences and overlap of preoperative risk factors, and the variability in the numbers and types of intraocular procedures that are performed on individual eyes, as well as postoperative complications all contribute to the difficulties involved in surgical management. This article discloses useful information pertaining to keratoplasty for Peters' anomaly. The study by Yang et al showed that long-term graft clarity could be achieved in 36% of eyes. However, 93% of all clear grafts were first grafts, whereas only 7% were second grafts. All third or subsequent grafts failed. Other important findings were: (1) the significantly greater chance of maintaining a clear graft with initial grafts, compared with subsequent grafts; (2) the disclosure of periods of differential risk for graft failure; and (3) the identification of risk factors for graft failure. Surgical intervention involving one or more procedures is effective in controlling IOP in 32% of eyes with associated congenital glaucoma. Still, multiple procedures and adjunctive medical therapy are often required to achieve and maintain adequate IOP control. The visual outcome is guarded in children with Peters' anomaly. Achieving a satisfactory visual outcome and preventing further visual loss is impeded by the presence of congenital anterior and posterior segment anomalies, structural defects of the CNS, cognitive dysfunction and amblyopia, as well as postoperative complications such as graft failure, cataract, inoperable retinal detachment, and phthisis.

  View details for PubMedID 11705147

• Slipped and lost extraocular muscles. Ophthalmology clinics of North America Lenart, T. D., Lambert, S. R. 2001; 14 (3): 433-442

  Abstract

  A slipped or lost muscle should be considered in the differential diagnosis of a patient presenting with a marked limitation of duction and inability to rotate the eye beyond the midline. Loss of a rectus muscle can occur after strabismus surgery, trauma, paranasal sinus surgery, orbital surgery, or retinal detachment surgery. The extraocular rectus muscle most frequently slipped or lost is the medial rectus muscle. Forced ductions, active force generation, saccadic velocity studies, differential intraocular pressure measurements, and orbital imaging studies may aid in identifying a slipped or lost muscle. However, no single diagnostic test provides absolute reliability for determining a lost muscle. Slipped muscles develop when the muscular capsule is imbricated without including the muscle or muscle tendon during strabismus surgery. When the capsule is reattached to the sclera, the tendon and muscle are then free to slip posteriorally from the site of attachment. Slipped muscles are retrieved by following the thin avascular muscle capsule posteriorally until the muscle is identified. A lost muscle can be found using a traditional conjunctival approach, by an external orbitotomy, or by an endoscopic transnasal approach. Although many diagnostic maneuvers are useful in identifying a lost rectus muscle, the oculocardiac reflex is the most important. Once the lost muscle is identified, the muscle should be imbricated with a nonabsorbable synthetic suture and securely reattached to the globe.

  View details for PubMedID 11705143

• A comparison of grating visual acuity, strabismus, and reoperation outcomes among children with aphakia and pseudophakia after unilateral cataract surgery during the first six months of life JOURNAL OF AAPOS Lambert, S. R., Lynn, M., Drews-Botsch, C., Loupe, D., Plager, D. A., Medow, N. B., Wilson, M. E., Buckley, E. G., Drack, A. V., Fawcett, S. L. 2001; 5 (2): 70-75

  Abstract

  The method of correcting aphakia after unilateral cataract extraction during infancy is controversial. Some authorities advocate correction with an intraocular lens (IOL) whereas others advocate correction with a contact lens (CL). We compared grating visual acuity, alignment, and reoperative outcomes in age-matched children treated with these 2 modalities at 5 clinical centers.Twenty-five infants born in 1997 or 1998 with a dense unilateral congenital cataract who had cataract surgery coupled with (IOL group, n = 12) or without (CL group, n = 13) primary IOL implantation were enrolled in this study. All patients were prescribed half-time occlusion therapy. In July 1999, their grating visual acuities, ocular alignments, and reoperation rates were assessed.The mean grating visual acuity (LogMAR) for the affected eye was 0.70 +/- 0.32 for the IOL group and 0.87 +/- 0.31 for the CL group (P =.19). The mean interocular difference in grating visual acuity was 0.26 +/- 0.30 for the IOL group and 0.50 +/- 0.28 for the CL group (P =.048). The incidence of strabismus (>10 PD) was 75% in the IOL group compared with 92% in the CL group (P =.24). The incidence of reoperations was 83% in the IOL group compared with 23% in the CL group (P =.003).Our preliminary data suggest that correcting aphakia after unilateral congenital cataract surgery with primary IOL implantation results in an improved visual outcome but a higher rate of complications requiring reoperation. A randomized clinical trial, the Infant Aphakia Treatment Study, is planned to further study the optimal treatment for aphakia following unilateral cataract extraction during infancy.

  View details for Web of Science ID 000168151900002

  View details for PubMedID 11304812

• Retrieval of lost medial rectus muscles with a combined ophthalmologic and otolaryngologic surgical approach AMERICAN JOURNAL OF OPHTHALMOLOGY Lenart, T. D., Reichman, O. S., McMahon, S. J., Lambert, S. R. 2000; 130 (5): 645-652

  Abstract

  To report retrieval of a medial rectus muscle completely detached from the globe and lost in the orbital tissue in four eyes.A lost medial rectus muscle was retrieved in four eyes of four patients with either a transcutaneous medial orbitotomy approach or a transnasal endoscopic ethmoid sinus approach.The lost medial rectus muscle was successfully retrieved in all four patients. One patient lost the medial rectus muscle secondary to trauma, and the other three cases resulted from complications of strabismus surgery. The mean preoperative angle of exotropia was 44 prism diopters. The endoscopic approach was attempted in three patients, and the medial rectus muscle was successfully found in two of these patients. In one case in which the endoscopic approach was used, an image guidance system was used to aid in finding the lost medial rectus muscle. The endoscopic approach was abandoned in one case in which the medial rectus muscle could not be identified after extensive searching, but the muscle was subsequently found by means of the transcutaneous medial orbitotomy approach. A transcutaneous medial orbitotomy alone was used to find the lost medial rectus muscle in one of the cases. The postoperative ocular deviation for primary position at distance fixation was a mean of 24 prism diopters of exotropia. With one additional operation in two patients, the mean ocular deviation was less than 12 prism diopters.We successfully retrieved a lost medial rectus muscle in four patients with the use of nontraditional strabismus surgical techniques. We effectively combined techniques taken from both ophthalmology and otorhinolaryngology to help solve this difficult problem.

  View details for Web of Science ID 000165385300011

  View details for PubMedID 11078843

• Tonic pupil associated with congenital neuroblastoma, Hirschsprung disease, and central hypoventilation syndrome AMERICAN JOURNAL OF OPHTHALMOLOGY Lambert, S. R., Yang, L. L., Stone, C. 2000; 130 (2): 238-240

  Abstract

  To report the association of tonic pupil, congenital neuroblastoma, Hirschsprung disease, and central hypoventilation syndrome.Case reports.Two infants with central hypoventilation syndrome and Hirschsprung disease were evaluated for dilated, nonreactive pupils present since birth. In both cases, pharmacologic testing with dilute pilocarpine confirmed denervation supersensitivity consistent with tonic pupil. The diagnosis of congenital neuroblastoma was subsequently established in both infants.The close association of these conditions in these two young children supports the concept of a common neural crest abnormality being present. A careful systemic evaluation to rule out congenital neuroblastoma should be performed in any young child who presents with tonic pupil in the setting of central hypoventilation syndrome and Hirschsprung disease.

  View details for Web of Science ID 000089601800018

  View details for PubMedID 11004304

• Isolated comitant esotropia and chiari I malformation AMERICAN JOURNAL OF OPHTHALMOLOGY Biousse, V., Newman, N. J., Petermann, S. H., Lambert, S. R. 2000; 130 (2): 216-220

  Abstract

  To report four patients with isolated comitant esotropia and Chiari I malformation and discuss the most appropriate management.Case reports and literature review.All four patients (5, 14, 16, and 37 years of age) presented with an isolated comitant esotropia that led to the diagnosis of Chiari I malformation. The first two patients underwent uncomplicated neurosurgical decompression of their malformation, followed by complete resolution of their esotropia. The third patient underwent strabismus surgery and experienced initial resolution of the esotropia, but eventual recurrence resulted in the strabismus surgery being repeated 5 years later. The fourth patient had strabismus surgery with resolution of the esotropia but only 2 months of follow-up.Although management of patients with Chiari I malformation and severe neurologic findings typically includes surgical decompression, management is less straightforward in cases with subtle findings or in which ocular findings are isolated. The decision to perform neurosurgical decompression or strabismus surgery should still be made on a case-by-case basis, with the understanding that strabismus surgery may provide only temporary ocular alignment.

  View details for Web of Science ID 000089601800011

  View details for PubMedID 11004297

• Cataract and phthisis bulbi after laser photoablation for threshold retinopathy of prematurity AMERICAN JOURNAL OF OPHTHALMOLOGY Lambert, S. R., Capone, A., Cingle, K. A., Drack, A. V. 2000; 129 (5): 585-591

  Abstract

  To describe the visual and structural outcome of eyes that developed a dense cataract after laser photoablation for threshold retinopathy of prematurity.A retrospective review of eight consecutive infants who developed dense cataract(s) after bilateral laser photoablation for threshold retinopathy of prematurity. Of the 10 eyes with cataract, five eyes were treated with a diode laser and five with an argon laser. The stage and zone of the retinopathy of prematurity, number of burns applied, time of onset of the cataract, clinical findings at the time of cataract surgery, and the course after cataract surgery were reviewed.Six eyes had zone 1 disease and four had zone 2 disease. The mean number of burns applied per eye was 2532 +/- 856 (range, 1400 to 4500). A cataract was diagnosed a median of 3 [corrected] weeks (range, 1 to 28 weeks) after laser photoablation. Nine of the 10 cataracts were sufficiently dense to preclude a view of the fundus. All 10 eyes had clinical signs suggestive of an inflammatory or ischemic process that included one or more of the following findings: corneal edema, pupillary membrane, iris atrophy, depigmentation of ciliary processes, pigment on the anterior lens surface, posterior synechiae, hyphema, and shallow anterior chamber. Nine eyes underwent cataract surgery. Five of the 10 eyes had retinal detachment ranging in severity from stage 4A to stage 5 at the time of cataract surgery. Nine of the 10 eyes progressed to phthisis bulbi and no light perception.A dense cataract developing in the eye of an infant after laser photoablation for threshold retinopathy of prematurity is associated with a poor visual prognosis. The constellation of associated clinical findings appears to be most consistent with anterior segment ischemia.

  View details for Web of Science ID 000087281200003

  View details for PubMedID 10844048

• Heterochromia after pediatric cataract surgery JOURNAL OF AAPOS Lenart, T. D., Drack, A. V., Tarnuzzer, R. W., Fernandes, A., Lambert, S. R. 2000; 4 (1): 40-45

  Abstract

  Changes in iris color have been noted anecdotally after cataract surgery in infants, but they have not been studied systematically. The mechanism for these iris color changes has not previously been reported in the biomedical literature.Photographs were taken of both eyes of 15 children and 11 rhesus monkeys who had undergone unilateral cataract surgery. Masked examiners reviewed the photographs and compared the iris color of the eyes that were operated on with the eyes that were not operated on. Between 4 and 6 weeks postoperatively, the level of prostaglandin F(2alpha) in the aqueous humor (n = 4) and vitreous humor (n = 2) was measured in both the operated and nonoperated eyes of 4 monkeys that had undergone a neonatal lensectomy during the first 5 days of life.Thirteen of 15 children had a darker iris color in the operated eye in relation to the nonoperated (control) eye. Four of 11 monkeys had a uniformly darker iris in the operated eye; the other 7 monkeys had regional darkening or patches of darker iris in the eye that was operated on. The prostaglandin F(2alpha) levels in neonatal monkeys were higher in the aqueous humor and in the vitreous humor of the operated eye in relation to the nonoperated eye.In some children, cataract surgery is associated with a darkening of the iris color in the operated eye. We speculate that this darkening results from an exuberant prostaglandin release stimulated by the cataract surgery and may occur through the same or a similar mechanism by which latanoprost causes the darkening of iris color.

  View details for Web of Science ID 000085455200007

  View details for PubMedID 10675870

• A comparison of the rate of refractive growth in pediatric aphakic and pseudophakic eyes OPHTHALMOLOGY MCCLATCHEY, S. K., Dahan, E., Maselli, E., Gimbel, H. V., Wilson, M. E., Lambert, S. R., Buckley, E. G., Freedman, S. F., Plager, D. A., PARKS, M. M. 2000; 107 (1): 118-122

  Abstract

  To compare the rate of refractive growth in pseudophakic children's eyes to that of aphakic eyes.Multicenter, retrospective observational case series.83 patients with pseudophakic eyes (100 eyes) and 74 patients with aphakic eyes (106 eyes), with an age of surgery between 3 months and 10 years and a minimum follow-up time of 3 years or more, depending on the age at surgery.A logarithmic model was used to analyze the rate of refractive growth for each eye.Age at surgery, intraocular lens power, intraocular lens A-constant, initial postoperative refraction, final refraction, and final age.Overall, pseudophakic eyes showed a lesser rate of refractive growth than aphakic eyes (-4.6 diopter vs. -5.7 diopter, P = 0.03). This trend was also present but less significant when the eyes were grouped into those less than 6 months of age at surgery (-3.3 diopter vs. -4.6 diopter, P = 0.09) and older patients (-5.0 diopter vs. -6.1 diopter, P = 0.07). However, the mean quantity of myopic shift was greater in pseudophakic eyes than in aphakic eyes (-5.26 diopter vs. -4.54 diopter), despite shorter follow-up times in the pseudophakic eyes. This is due to the optical effects of a constant intraocular lens power in a growing eye.Pediatric pseudophakic eyes have a slightly lesser rate of refractive growth than aphakic eyes. The new rate values should be used for predicting future refractions in these eyes.

  View details for Web of Science ID 000084724900038

  View details for PubMedID 10647729

• Visual outcome after contact lens and intraocular lens correction of neonatal monocular aphakia in monkeys INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE Boothe, R. G., Louden, T., Aiyer, A., Izquierdo, A., Drews, C., Lambert, S. R. 2000; 41 (1): 110-119

  Abstract

  A monkey model was used to evaluate intraocular lenses (IOLs) and extended-wear contact lenses (EWCLs) for the optical treatment of infantile aphakia in humans. Specifically, the relative effectiveness of EWCLs used alone and IOLs used in combination with EWCLs in preventing amblyopia was assessed.A total of 33 rhesus monkeys was studied in this project, 24 assigned to experimental treatment groups and 9 to normal controls. Contact lenses made from a diffusing material or dyed opaque were placed on one eye at birth to simulate an infantile cataract. A unilateral lensectomy was then performed on the same eye within 2.5 weeks after birth. In 15 monkeys this was combined with implantation of an IOL. The eyes were left aphakic in the remaining 9 animals. EWCLs were used to adjust the optical correction of both aphakic and pseudophakic eyes to a near point (3-5 D). Opaque lenses were used to maintain daily part-time (approximately 70%) occlusion of the fellow eye. The primary outcome measure was grating acuity assessed with behavioral methods. Some animals were also assessed for acuity with sweep visually evoked potentials (VEPs) and for optotype acuity (Landolt C) with behavioral methods.Two of the animals with IOLs developed complications in the eye that precluded completion of the behavioral assessment protocol. Only behavioral outcomes obtained before or in the absence of surgical complications are presented. There was a developmental delay in the maturation of grating acuity in both eyes of both treatment groups. Normal adult levels of grating acuity were eventually achieved in the group treated with IOLs combined with EWCLs. Grating acuity was significantly poorer than normal in aphakic eyes treated only with EWCLs. Comparison of the two treatment groups revealed that pseudophakic eyes treated with multifocal IOLs had significantly better gating acuity than aphakic eyes. Assessments of optotype acuity and sweep VEP acuity revealed amblyopic deficits in both pseudophakic and aphakic eyes.Given an absence of serious postoperative complications, neonatal correction of aphakia with IOLs combined with EWCLs can lead to normal grating acuity in a primate model. Correction with EWCLs alone was not sufficient to produce normal grating acuity. Multifocal IOL treatments combined with EWCL provided a significantly better outcome than EWCL methods alone. However, neither IOL nor EWCL methods were able to prevent amblyopia as evaluated using behavioral testing with optotypes or with sweep VEPs.

  View details for Web of Science ID 000084634200019

  View details for PubMedID 10634609

• Unilateral intraocular lens implantation during the first six months of life JOURNAL OF AAPOS Lambert, S. R., Buckley, E. G., Plager, D. A., Medow, N. B., Wilson, M. E. 1999; 3 (6): 344-349

  Abstract

  The purpose of this study was to determine the incidence of postoperative complications and the occurrence of myopic shift in infantile eyes after cataract surgery and implantation of an intraocular lens (IOL).Cataract surgery and IOL implantation was performed on 11 infants with unilateral congenital cataracts who had a mean age of 10+/-6 weeks at 5 clinical centers. IOLs with a mean power of 26.2+/-2.3 D were implanted using a standardized protocol. The infants were then followed up for a mean of 13+/-6 months for postoperative complications and longitudinal changes in their refractive error.Eight of the 11 eyes had postoperative complications and were treated with a reoperation. Complications included open-angle glaucoma (n = 2), lens reproliferation into the visual axis (n = 2), pupillary membranes (n = 2), and corectopia (n = 2). A younger age at the time of surgery was positively correlated with the development of a postoperative complication requiring a reoperation (P = .03). A mean myopic shift of 5.49 D occurred in these eyes a year after surgery. No preoperative factors could be identified that correlated with the magnitude of the myopic shift.IOL implantation during infancy is associated with a high complication rate frequently requiring reoperation. A large myopic shift typically occurs that necessitates an initial or a late overcorrection with spectacles or a contact lens, depending on the power of the IOL implanted. Until ongoing randomized trials are completed, we recommend that surgeons exercise caution before implanting IOLs in the eyes of infants.

  View details for Web of Science ID 000084320800006

  View details for PubMedID 10613578

• Anterior hyaloid face opacification after pediatric Nd : YAG laser capsulotomy JOURNAL OF AAPOS Hutcheson, K. A., Drack, A. V., Ellish, N. J., Lambert, S. R. 1999; 3 (5): 303-307

  Abstract

  The purpose of this study was to examine the clarity of the visual axis after Nd:YAG laser capsulotomy following cataract extraction and primary intraocular lens implantation in a pediatric population.A retrospective review was performed of all cases of cataract extraction and primary intraocular lens implantation over a period of 5 years. A group of children who had been treated by primary surgical posterior capsulotomy and anterior vitrectomy (Group 1) was used as the "gold standard," with whom the children treated with Nd:YAG laser capsulotomy (Group 2) were compared. The groups were studied for the incidence of opacification of the visual axis after the primary procedure.Data on 78 eyes were reviewed, and 56 eyes met inclusion criteria. Of these, 33 eyes were treated with primary posterior capsulotomy and anterior vitrectomy (Group 1) and 23 eyes were treated with Nd:YAG laser capsulotomy (Group 2). One eye (3%) of Group 1 experienced postoperative visual axis reopacification. Thirteen (57%) of 23 eyes in Group 2 experienced reopacification, requiring retreatment. Four eyes (17%) treated with Nd:YAG laser required a third treatment.In our series, 57% of patients treated with Nd:YAG laser capsulotomy experienced reopacification across the anterior hyaloid face. With the removal of the anterior vitreous at the time of cataract extraction, the scaffolding for cell migration is removed and reopacification of the visual axis is rarely seen. For patients in whom slit-lamp capsulotomy is not possible, especially if there is no Nd:YAG laser available for use in the operating room or when loss to follow-up may be an issue, primary posterior capsulotomy and anterior vitrectomy should be strongly considered.

  View details for Web of Science ID 000083252200009

  View details for PubMedID 10532576

• Effect of age at time of cataract surgery on subsequent axial length growth in infant eyes JOURNAL OF CATARACT AND REFRACTIVE SURGERY Griener, E. D., Dahan, E., Lambert, S. R. 1999; 25 (9): 1209-1213

  Abstract

  To determine whether removal of the crystalline lens and placement of an intraocular lens (IOL) in human infant eyes retard the growth of the pseudophakic eye.A unilateral lensectomy with placement of a posterior chamber IOL in the sulcus was performed in 11 infants between 2 and 4 months of age. Axial length measurements of both eyes were obtained preoperatively and postoperatively.Patients were followed for a mean of 5.6 years. In 7 patients, the mean axial growth was 0.46 mm less in the pseudophakic eye than in the fellow eye (range 0.15 to 0.70 mm). In 1 patient, there was no interocular axial length difference and in 3, the pseudophakic eye was longer. When measurements from the only patient with microphthalmia were excluded, the interocular difference in axial growth was highly significant (signed rank test, P = .006). Median visual acuity of the pseudophakic eyes at the last follow-up was 20/60 (range 20/30 to 20/200). The final visual acuity in the pseudophakic eyes did not correlate with the degree of interocular axial length difference (P > .05).Our study suggests there may be a reduction in axial growth in infantile eyes following cataract extraction and IOL implantation. This effect probably reduces the magnitude of the myopic shift in these eyes.

  View details for Web of Science ID 000082249300010

  View details for PubMedID 10476503

• IOLs in the first year of life JOURNAL OF PEDIATRIC OPHTHALMOLOGY & STRABISMUS Buckley, E., Lambert, S. R., Wilson, M. E. 1999; 36 (5): 281-286

  View details for Web of Science ID 000082788900007

  View details for PubMedID 10505833

• Successful treatment of tractional corectopia using 2 mJ of energy with an Nd : YAG laser JOURNAL OF AAPOS Griener, E., Lambert, S. R. 1999; 3 (4): 250-251

  Abstract

  Although the Nd:YAG laser is most commonly used to perform posterior capsulotomies after cataract surgery, it has also been used to treat a variety of other anterior segment abnormalities including tractional corectopia, iridocorneal adhesions, persistent pupillary membranes, and posterior synechiae. Numerous reports on the use of the Nd:YAG laser to treat structures in the anterior segment have emphasized the need to use higher pulse energy for pupillary membranes, compared with the lower settings required for posterior capsulotomy. Steinert and Puliafito noted that single pulses of 4 to 12 mJ may be required to treat pupillary membranes "in a manner similar to that of a stonemason chipping at marble" in their description treating a membrane considerably thicker than what we describe. We report the successful treatment of tractional corectopia due to an anterior membrane strand in a child with only 2 mJ of total energy.

  View details for Web of Science ID 000082042700011

  View details for PubMedID 10477229

• Management of monocular congenital cataracts EYE Lambert, S. R. 1999; 13: 474-479

  Abstract

  Monocular congenital cataracts have been treated for the past two decades with early surgery, contact lens correction and patching of the unaffected eye. While an occasional patient has had a good visual outcome with this treatment regiment, the majority end up being legally blind in the aphakic eye. Intraocular lenses (IOLs) are increasingly being used as an alternative means of optically correcting aphakia during infancy. A growing body of literature suggests that an IOL correction is associated with an improved visual outcome and a relatively low incidence of post-operative complications. A monkey model has also been used to study the safety and feasibility of correcting aphakia in neonates with IOLs. These studies have revealed that the visual outcome is as good if not better with an IOL correction. A multicentre clinical trial, the Infant Aphakia Treatment Study (IATS), is being organised in the United States to critically compare an IOL and contact lens correction for infantile aphakia.

  View details for Web of Science ID 000081374500013

  View details for PubMedID 10627827

• Long-term results of corneal graft survival in infants and children with Peters anomaly OPHTHALMOLOGY Yang, L. L., Lambert, S. R., Lynn, M. J., Stulting, R. D. 1999; 106 (4): 833-848

  Abstract

  To determine the long-term results of corneal graft survival after penetrating keratoplasty for Peters anomaly and to identify risk factors for graft failure.Noncontrolled interventional case series: a single-center retrospective review of a consecutive surgical series.The records of all children 12 years of age or younger who underwent penetrating keratoplasty for Peters anomaly between January 1971 and December 1992 were reviewed. All study eyes had completed a minimum of 3 years of follow-up from the date of first keratoplasty and had undergone most of their corneal surgery at Emory University.Characteristics of the recipient, the eye, the donor, and the surgical procedure were analyzed for their influence on survival of the first graft. Survival probabilities were estimated using the Kaplan-Meier method. Multivariate regression analysis was performed to estimate relative risks and adjusted survival probabilities.Graft clarity.One hundred forty-four penetrating keratoplasties were performed in 72 eyes of 47 patients. The median age at first keratoplasty was 4.4 months. The median follow-up was 11.1 years. Fifty-four percent of eyes received one graft, 18% received two grafts, and 28% received three or more grafts. The overall probability of maintaining a clear first graft was 56% at 6 months, 49% at 12 months, 44% at 3 years, and 35% at 10 years. The probability of second or subsequent grafts surviving for 3 years was less than 10%. Thirty-nine percent of eyes had a clear graft at the time of review; 36% of eyes had a clear first graft. Multivariate analysis identified disease severity, donor cornea size, coexisting central nervous system abnormalities, and quadrants of anterior synechiae as the strongest risk factors for graft failure. Supplemental multivariate analysis, restricted to observable preoperative variables, identified stromal vessels, total limbal opacification, and preoperative glaucoma as independent preoperative predictors of graft failure. Allograft rejection was the most frequently identified cause of graft failure. Major complications after keratoplasty were phthisis, retinal detachment, cataract, and glaucoma.The overall long-term probability of maintaining a clear graft after initial penetrating keratoplasty for Peters anomaly is 35% +/- 0.06%, with subsequent grafts rarely surviving. Eyes with severe disease, larger donor corneas, coexisting central nervous system abnormalities, and anterior synechiae have significantly poorer outcomes than eyes without these factors. These data should be carefully considered before recommending corneal transplantation for Peters anomaly, particularly after previous graft failure.

  View details for Web of Science ID 000079410200044

  View details for PubMedID 10201611

• Cyclic esotropia after a traumatic sixth nerve palsy in a child. Journal of AAPOS Hutcheson, K. A., Lambert, S. R. 1998; 2 (6): 376-377

  Abstract

  Cyclic esotropia is a rare phenomenon in which esotropia and orthophoria alternate over a period of 48 to 96 hours. The mechanism that underlies the phenomenon is unknown. Cyclic esotropia often occurs after a fusion-disrupting event. We report an unusual case of cycling esotropia with onset after a traumatic sixth nerve palsy. The cyclic phase persisted for 2 years, following a 48-hour alternate-day pattern. After strabismus surgery for the esotropic angle, the deviation disappeared and the patient remained orthotropic, with 1 year of follow-up to date.

  View details for PubMedID 10532729

• Pseudotumor cerebri in children. Journal of AAPOS Phillips, P. H., Repka, M. X., Lambert, S. R. 1998; 2 (1): 33-38

  Abstract

  Demographic and outcome data in the era of modern neuroimaging are needed to describe pseudotumor cerebri in children.We reviewed the medical records of children less than 18 years old who were diagnosed with pseudotumor cerebri between 1977 and 1997. We defined pseudotumor cerebri as (1) increased intracranial pressure, (2) normal or small ventricles, and (3) normal cerebrospinal fluid composition. The condition might be idiopathic or the result of a nontumor etiology.Thirty-seven patients had an initial diagnosis of pseudotumor cerebri. Two patients were subsequently diagnosed with a central nervous system malignancy and were excluded from further analysis. The remaining 35 patients included 10 patients with idiopathic pseudotumor cerebri and 25 patients with disorders reported to be associated with pseudotumor cerebri. The mean age was 10.6 years with a range of 3 to 17 years. Twenty patients (57%) were female and 13 patients (37%) were obese. At presentation 4 patients had a visual acuity less than 20/40 in the best eye and 10 patients had visual field deficits. Seventeen patients (49%) had cranial nerve deficits, all of which resolved with normalization of the intracranial pressure. Follow-up data were obtained on 30 patients. Only one patient had a final visual acuity less than 20/40 in the best eye, whereas six patients had residual visual field deficits. Ten patients (33%) had optic nerve atrophy.There was no gender predominance, and associated etiologic factors were common in these children with pseudotumor cerebri. Permanent visual loss occurs in some children with pseudotumor cerebri. Quantitative perimetry and optic nerve examination were more sensitive than visual acuity determination in detecting damage to the visual sensory system. In rare instances the patient diagnosed with pseudotumor cerebri will be found after extended follow-up to harbor an intracranial neoplasm.

  View details for PubMedID 10532364

• Electroretinographic findings in infants with the shaken baby syndrome JOURNAL OF PEDIATRIC OPHTHALMOLOGY & STRABISMUS Fishman, C. D., Dasher, W. B., Lambert, S. R. 1998; 35 (1): 22-26

  Abstract

  To determine if electroretinography is helpful in the work-up of children with the shaken baby syndrome.Six children with retinal hemorrhages and the shaken baby syndrome underwent electroretinography (ERG). The ERGs of these six children were compared with six age-matched controls using the sign-rank test.Neither the implicit time nor the amplitude of the white scotopic ERG response was significantly different between these patients and age-matched controls. Although the amplitude of the blue scotopic and 30 Hz flicker responses were attenuated (p < 0.05), the implicit times were not significantly different from controls. Three of the patients had serial ERGs recorded. The b-wave implicit time and amplitude improved in two of these patients. The ERG was helpful in distinguishing between a CNS and a retinal cause of visual loss in one child.The ERG can be helpful in assessing retinal function in children with the shaken baby syndrome who have persistent visual impairment. In most cases, the ERG is not helpful in the initial assessment of children with the shaken baby syndrome.

  View details for Web of Science ID 000072042100009

  View details for PubMedID 9503311

• Balloon dilatation for treatment of resistant nasolacrimal duct obstruction. Journal of AAPOS Hutcheson, K. A., Drack, A. V., Lambert, S. R. 1997; 1 (4): 241-244

  Abstract

  Our purpose was to report our experience with balloon catheter dilatation for resistant nasolacrimal duct obstruction.Patients enrolled had symptoms of nasolacrimal duct obstruction and (1) had failed previous probing or (2) were more than 2 years old. Balloon dilatation was performed with a LacriCATH lacrimal catheter (Atrion Medical Products, Birmingham, Ala.). A subset of patients had Silastic silicone rubber (Dow Corning, Midland, Mich.) intubation after balloon dilatation. Success was determined by clinical examination a minimum of 6 weeks later.Twenty-one lacrimal systems of 12 patients were treated (age range 4 months to 7 years). Of the patient subset treated with a LacriCATH lacrimal catheter alone, 9 of 18 systems demonstrated complete resolution of symptoms. Three of the 12 patients underwent balloon dilatation intraoperatively after attempts at Silastic silicone rubber intubation were unsuccessful. In two of these patients, who were younger, Silastic silicone rubber tubes passed easily after balloon treatment; however, in an older patient, age 5 years, intubation still could not be accomplished.Common clinical strategy for treatment of resistant nasolacrimal obstruction includes repeat probing, intubation of the nasolacrimal system with Silastic silicone rubber tubes, or dacryocystorhinostomy. Balloon catheter dilatation is an alternative or adjunct to consider. Factors that may affect the success of treatment include the age of the patient, the complexity of the nasolacrimal anatomy, and use of adjunctive systemic antibiotics and steroids.

  View details for PubMedID 10532771

• Myopic shift after intraocular lens implantation during childhood OPHTHALMOLOGY Hutchinson, A. K., DREWSBOTSCH, C., Lambert, S. R. 1997; 104 (11): 1752-1757

  Abstract

  The purpose of the study is to evaluate the myopic shift that occurs in children 3 to 9 years of age who undergo cataract extraction with primary intraocular lens (IOL) implantation.A review of 18 children (mean, 6.3 +/- 0.5 year; range, 3-9 years) who had undergone primary IOL implantation was undertaken. Patients were observed for an average of 3.2 years. The initial and last postoperative refractive errors were compared.The mean myopic shift was -0.99 +/- 0.22 diopter (D) (median, 1.0 D) with a range of -3.25 to +0.38 D. The difference in the myopic shift of the children 3 to 5 years of age (-0.94 +/- 0.30 D) was not significantly different from the myopic shift occurring in the children 6 to 9 years of age (-1.07 +/- 0.35 D). The myopic shift was less than 1.5 D in 70% of the eyes and only 3 eyes had a myopic shift greater than 2 D. Ninety percent of the children achieved a visual acuity of 20/40 or better in their pseudophakic eye or eyes.Although each patient should be evaluated on an individual basis, the authors recommend undercorrecting most children 3 to 9 years of age by 1 D from the IOL power predicted to achieve emmetropia.

  View details for Web of Science ID A1997YF41700014

  View details for PubMedID 9373102

• Strabismus and mitochondrial defects in chronic progressive external ophthalmoplegia AMERICAN JOURNAL OF OPHTHALMOLOGY Sorkin, J. A., Shoffner, J. M., Grossniklaus, H. E., Drack, A. V., Lambert, S. R. 1997; 123 (2): 235-242

  Abstract

  To describe the results of strabismus surgery on three patients with chronic progressive external ophthalmoplegia, a group of rare disorders characterized by ptosis and slowly progressive ophthalmoparesis that has been shown to result from defects in mitochondrial DNA.Strabismus surgery using the adjustable suture technique was performed in three patients with strabismus and chronic progressive external ophthalmoplegia confirmed by clinical, biochemical, histopathologic, and genetic criteria. All three patients had mitochondrial DNA deletions. Two patients were exotropic; one patient was esotropic.Rectus muscle recessions were initially unsuccessful in correcting strabismus in one patient, although a subsequent procedure employing rectus muscle resections was successful in alleviating a significant head turn and improved ocular alignment. In the two other patients, a single procedure consisting of rectus muscle recessions combined with large rectus muscle resections successfully achieved good postoperative alignment. The amount of surgery performed in these three patients exceeded that predicted in standard strabismus tables.The myopathic process that results in chronic progressive external ophthalmoplegia renders rectus muscle recessions less effective compared with resections for correcting the associated strabismus seen in these patients. Rectus muscle resections therefore should be an integral procedure in the surgical management of the strabismus associated with chronic progressive external ophthalmoplegia.

  View details for Web of Science ID A1997WG30500011

  View details for PubMedID 9186130

• Longitudinal changes in axial length in pseudophakic children JOURNAL OF CATARACT AND REFRACTIVE SURGERY Sorkin, J. A., Lambert, S. R. 1997; 23: 624-628

  Abstract

  To evaluate the axial length changes that occur in children 3 to 9 years of age who had cataract extraction with primary intraocular lens (IOL) implantation.Emory Eye Center, Emory University, and Egleston Children's Hospital, Atlanta, Georgia, USA.A review of 17 eyes in 15 children (mean age 6.4 years; range 3 to 9 years) who had primary IOL implantation was undertaken. Patients were followed for an average of 3.1 years. The initial and final axial length measurements and refractive errors were compared.The overall change in mean axial length in the 17 eyes was 0.64 mm. The overall mean myopic shift was -1.01 diopters (D) (range -3.00 +0.50 D). Eyes with traumatic cataracts experienced more axial elongation than eyes with developmental/congenital cataracts (0.97 mm versus -0.01 mm; P = .03). Ninety-four percent of patients obtained a final visual acuity of 20/40 or better.Eyes with traumatic cataracts experienced more axial elongation than eyes with developmental/congenital cataracts after cataract extraction and IOL implantation.

  View details for Web of Science ID A1997XT32700006

  View details for PubMedID 9278815

• Optic nerve hypoplasia: Absence of posterior pituitary bright signal on magnetic resonance imaging correlates with diabetes insipidus AMERICAN JOURNAL OF OPHTHALMOLOGY Sorkin, J. A., Davis, P. C., Meacham, L. R., Parks, J. S., Drack, A. V., Lambert, S. R. 1996; 122 (5): 717-723

  Abstract

  Magnetic resonance imaging has been used to examine children with optic nerve hypoplasia for pituitary abnormalities who may be at risk for anterior pituitary hormonal deficiencies. We correlated the sellar and optic pathway anatomic findings on magnetic resonance imaging in children with optic nerve hypoplasia with findings from their endocrinologic and ophthalmologic examinations to determine whether magnetic resonance imaging findings predict anterior and posterior pituitary dysfunction.A retrospective review identified five children with optic nerve hypoplasia and endocrinopathy who also underwent high resolution volumetric magnetic resonance imaging.All children had severe bilateral optic nerve hypoplasia and anterior pituitary hormone deficiencies. Three children had no recognizable intrasellar or ectopic posterior pituitary bright spot on magnetic resonance imaging; all had clinical evidence of diabetes insipidus. Two patients with a recognizable but ectopic posterior pituitary did not have diabetes insipidus.Children with optic nerve hypoplasia and no recognizable posterior lobe of the pituitary gland on magnetic resonance imaging are at risk for both anterior and posterior pituitary dysfunction, whereas those with a posterior lobe on magnetic resonance imaging appear to have intact posterior pituitary function.

  View details for Web of Science ID A1996VQ70100014

  View details for PubMedID 8909213

• Infantile cataracts (vol 40, pg 451, 1996) SURVEY OF OPHTHALMOLOGY Lambert, S. R., Drack, A. V. 1996; 41 (1): A8-A8

  View details for Web of Science ID A1996VA66100001

• Infantile cataracts SURVEY OF OPHTHALMOLOGY Lambert, S. R., Drack, A. V. 1996; 40 (6): 427-458

  Abstract

  Cataracts are one of the most treatable causes of visual impairment during infancy. Recent epidemiological studies have shown that they have a prevalence of 1.2 to 6.0 cases per 10,000 infants. The morphology of infantile cataracts can be helpful in establishing their etiology and prognosis. Early surgery and optical correction have resulted in an improved outcome for infants with either unilateral or bilateral cataracts. While contact lenses continue to be the standard means of optically correcting an infant's eyes after cataract surgery, intraocular lenses are gaining in popularity as an alternative means of optically correcting these eyes. Post-operative complications occur more commonly after infantile than adult cataract surgery and many of these complications do not develop until years later. As a result, it is critical that children be followed closely on a long term basis after infantile cataract surgery.

  View details for Web of Science ID A1996UM14000001

  View details for PubMedID 8724637

• Congenital entropion and congenital corneal ulcer AMERICAN JOURNAL OF OPHTHALMOLOGY Yang, L. L., Lambert, S. R., Chapman, J., Stulting, R. D. 1996; 121 (3): 329-331

  Abstract

  We studied a case of corneal ulceration in utero from lower eyelid entropion.A 3-week-old male infant was referred for examination of a left corneal ulcer that was present at birth and unresponsive to antibiotics.Examination disclosed a lower eyelid entropion that was treated surgically by a nonincisional method, leaving a central leukoma after re-epithelialization.Congenital lower eyelid entropion should be included in the differential diagnosis of congenital corneal opacities.

  View details for Web of Science ID A1996TY95200018

  View details for PubMedID 8597283

• Retinoblastoma recurrence after iodine 125 plaque application RETINA-THE JOURNAL OF RETINAL AND VITREOUS DISEASES Spraul, C. W., Lim, J. I., Lambert, S. R., Grossniklaus, H. E. 1996; 16 (2): 135-138

  Abstract

  The authors report the clinicopathologic features of a recurrent retinoblastoma after radioactive iodine 125 (125l) plaque therapy.The clinical and histopathologic findings of a 1-year-old boy with a recurrent retinoblastoma after 125l radioactive plaque application were reviewed. The patient's enucleated right eye was processed for routine histologic evaluation. Immunostains for glial fibrillary acid protein and S-100 protein were performed.Examination of the enucleated eye revealed a retinal tumor composed of two populations of cells. One population consisted of poorly differentiated cells, and the second population at the base of the tumor contained well-differentiated cells with numerous fleurettes. Immunohistochemical stains were positive for glial fibrillary acid protein and S-100 protein in the well-differentiated portion of the tumor.This case illustrates a recurrent retinoblastoma that may have arisen from radioresistant well-differentiated cells after 125l plaque therapy.

  View details for Web of Science ID A1996UF08100009

  View details for PubMedID 8724958

• ELECTRORETINOGRAPHIC FINDINGS IN STAGE-5 RETINOPATHY OF PREMATURITY AFTER RETINAL REATTACHMENT RETINA-THE JOURNAL OF RETINAL AND VITREOUS DISEASES Cherry, T. A., Lambert, S. R., Capone, A. 1995; 15 (1): 21-24

  Abstract

  Infants with stage 5 retinopathy of prematurity (ROP) usually experience severe visual loss, even when the retina is reattached surgically. Such visual loss has been attributed to persistent retinal dysfunction in most cases, but this has not been established electroretinographically.Electroretinograms (ERGs) were performed on 5 eyes of 4 children with partial or complete retinal attachment after vitreoretinal surgery for stage 5 ROP. Duration of detachment before surgery was 2 weeks in 1 eye, 4-5.5 weeks in 3 eyes, and 15 weeks in 1 eye. The ERG findings for these eyes were then compared with ERG findings in a child with regressed ROP after laser photoablation of the peripheral avascular retina.All of the eyes with surgically repaired stage 5 ROP had nonrecordable ERGs. The child with regressed ROP after laser photoablation had an attenuated but recordable ERG.Severe retinal dysfunction persists in eyes with stage 5 ROP even after the retina is surgically reattached, which is probably one of the primary reasons that these eyes continue to have a poor visual outcome.

  View details for Web of Science ID A1995QH33400003

  View details for PubMedID 7754242

• REAPPRAISAL OF OCCLUSION THERAPY FOR SEVERE STRUCTURAL ABNORMALITIES OF THE OPTIC DISC AND MACULA JOURNAL OF PEDIATRIC OPHTHALMOLOGY & STRABISMUS Yang, L. L., Lambert, S. R. 1995; 32 (1): 37-41

  Abstract

  The visual function of some children with structural defects of the macula or optic nerve has previously been shown to improve with occlusion therapy. The charts of five children, ages 4 to 8 years, who had various types of severe structural abnormalities were reviewed. Two patients had optic nerve hypoplasia; there was one case each of foveal hypoplasia, posterior persistent hyperplastic primary vitreous, and retinopathy of prematurity. All the patients had undergone extensive amblyopia therapy prior to referral. In three children, occlusion therapy had been initiated and continued intensively for several months or longer without recognizing the presence of a severe, underlying structural abnormality. In none of the five children was there any clear-cut evidence of visual improvement. In some cases, the long period of enforced iatrogenic vision impairment resulted in significant psychosocial harm and developmental delay. In all five cases, it was structural changes rather than amblyopia that ultimately accounted for visual loss. The authors stress the importance of a meticulous fundus examination directed at finding organic defects prior to patching. Close, periodic monitoring of visual function should then follow. To spare the child unnecessary psychosocial impairment, it is advisable to follow established recommended thresholds for terminating occlusion therapy.

  View details for Web of Science ID A1995QK36500008

  View details for PubMedID 7752032

• CLINICAL AND SUBCLINICAL OPHTHALMIC FINDINGS WITH RETINOL DEFICIENCY OPHTHALMOLOGY Newman, N. J., Capone, A., LEEPER, H. F., ODAY, D. G., Mandell, B., Lambert, S. R., THOFT, R. A. 1994; 101 (6): 1077-1083

  Abstract

  Patients at risk for retinol deficiency in developed countries include those with hepatic dysfunction and malabsorption states. Symptoms of retinol deficiency may go unrecognized or unreported.The authors describe 15 patients with hepatic dysfunction, two of whom had procedures that would predispose to malabsorption and were ophthalmologically symptomatic of retinol depletion. The other 13 patients were ophthalmologically asymptomatic liver transplant candidates examined prospectively for subclinical evidence of retinol deficiency. Combined laboratory analysis, Schirmer's testing, conjunctival impression cytology, and electroretinography were performed.Twelve of 15 patients had serum retinol levels below the lower limit of normal. Aqueous tear production was reduced in 7 of 14 patients. Abnormal conjunctival morphology was noted in 6 of 12 patients. Electroretinograms were abnormal in the two patients who were visually symptomatic and in seven of nine patients who were ophthalmologically asymptomatic.Subclinical, physiologically significant retinol deficiency may be a frequent and unrecognized problem among patients with hepatic dysfunction.

  View details for Web of Science ID A1994NR47400027

  View details for PubMedID 8008350

• MULTIFOCAL VERSUS MONOFOCAL CORRECTION OF NEONATAL MONOCULAR APHAKIA JOURNAL OF PEDIATRIC OPHTHALMOLOGY & STRABISMUS Lambert, S. R., Fernandes, A., DREWSBOTSCH, C., Boothe, R. G. 1994; 31 (3): 195-201

  Abstract

  We compared the efficacy of monofocal and multifocal intraocular lenses (IOLs) as a means of optically rehabilitating eyes with monocular congenital cataracts using a primate model. A congenital cataract was simulated in the right eye of 20 monkeys from birth to 2 weeks of age with an opalescent contact lens. A lensectomy was then performed and a monofocal IOL was implanted in 11 eyes and a multifocal IOL in 9 eyes. Any residual refractive error was then corrected with an extended-wear contact lens. The fellow eyes were then randomized to no treatment or 70% occlusion therapy. Grating acuity was assessed using forced-choice preferential looking with Teller acuity cards at regular intervals. At 32 weeks of age, the pseudophakic eyes were mildly amblyopic, but the grating acuities were not significantly different between the pseudophakic eyes with multifocal versus monofocal IOLs or between subjects who had received occlusion therapy versus no treatment of the fellow eye. We conclude that at least through 32 weeks of age, multifocal and monofocal IOLs are of similar efficacy and occlusion therapy may be less important to prevent amblyopia from developing in pseudophakic eyes compared to contact-lens corrected aphakic eyes.

  View details for Web of Science ID A1994NW52900012

  View details for PubMedID 7931955

• CONCORDANCE AND RECESSIVE INHERITANCE OF LEBER CONGENITAL AMAUROSIS AMERICAN JOURNAL OF MEDICAL GENETICS Lambert, S. R., Sherman, S., Taylor, D., Kriss, A., Coffey, R., Pembrey, M. 1993; 46 (3): 275-277

  Abstract

  Pedigrees were obtained on 43 patients with confirmed Leber congenital amaurosis, a rare form of blindness caused by congenital dysfunction of the retina. All of the pedigrees were consistent with autosomal recessive inheritance and the segregation frequency using classical segregation analysis was 0.24 +/- 0.07. Six of the 7 affected sib pairs were concordant in regards to systemic abnormalities. Despite previous reports that Leber congenital amaurosis can be inherited either as an autosomal dominant or recessive condition, our findings support only an autosomal recessive inheritance of this disorder.

  View details for Web of Science ID A1993KZ61000006

  View details for PubMedID 8488871

• SUPERIOR RECTUS MUSCLE OVERACTION AFTER CATARACT-EXTRACTION AMERICAN JOURNAL OF OPHTHALMOLOGY Grimmett, M. R., Lambert, S. R. 1992; 114 (1): 72-80

  Abstract

  Four patients with an ipsilateral hypertropia after cataract extraction consistent with superior rectus muscle overaction were identified between March 1990 and April 1992. Operative trauma was the most likely causative factor, as other likely conditions were excluded. The proposed pathogenesis for all cases is similar to that of botulinum type-A toxin therapy: a transient postoperative weakness of the ipsilateral inferior rectur muscle leads to a contracture or strengthening of the ipsilateral antagonist (the superior rectus muscle). Possible mechanisms of injury that would result in a transient inferior rectus muscle palsy would include anesthetic myotoxicity or direct trauma to the muscle and related structures from the retrobulbar injection (or subconjunctival injection). Surgical intervention consisting of an ipsilateral superior rectus muscle recession and posterior fixation sutures (when the vertical incomitance was large) yielded excellent results in restoring single binocular vision. Possible preventive measures would include using a minimal volume of anesthetic along with careful needle placement.

  View details for Web of Science ID A1992JC19500012

  View details for PubMedID 1621788

• THE OPTIC DISK IN LEBER CONGENITAL AMAUROSIS JOURNAL OF PEDIATRIC OPHTHALMOLOGY & STRABISMUS Sullivan, T. J., Lambert, S. R., Buncic, J. R., Musarella, M. A. 1992; 29 (4): 246-249

  Abstract

  The typical fundus appearance in Leber congenital amaurosis (LCA) in infancy is normal. Later in childhood, clinical heterogeneity develops and a variety of fundal abnormalities may be seen. These commonly include optic atrophy, retinal arteriolar attenuation, and a variety of pigmentary changes. We retrospectively reviewed the optic disc findings of 77 patients with LCA whom we had examined to confirm our clinical impression that the optic discs are frequently normal. Age at examination ranged from infancy to the fourth decade. The optic discs were normal in 53 (69%) of the 77 patients examined; 18 (23%) had varying degrees of optic atrophy; 2 (3%), pseudopapilledema; and 1 (1%), grey discs. The optic discs could not be seen in 3 (4%) patients. We conclude that the optic discs are frequently normal in appearance, even in older patients with LCA.

  View details for Web of Science ID A1992JK32800012

  View details for PubMedID 1512668

• RIGID GAS PERMEABLE CONTACT-LENS CORRECTION OF APHAKIA FOLLOWING CONGENITAL CATARACT REMOVAL DURING INFANCY JOURNAL OF PEDIATRIC OPHTHALMOLOGY & STRABISMUS AMOS, C. F., Lambert, S. R., Ward, M. A. 1992; 29 (4): 243-245

  Abstract

  Rigid gas permeable (RGP) contact lenses were prescribed and worn with little difficulty by 10 consecutive aphakic infants. Fluoroperm 92 lenses were used instead of silicone lenses (Bausch & Lomb) because of their lower cost, ease in handling, flexibility in design, and better neutralization of astigmatism. Lenses were worn on a daily wear basis (with occasional overnight wear) to reduce the risk of keratitis. Keratometry readings were taken at the time of surgery with the patient in a supine position. Trial lens fitting was performed 1 to 7 days later using a Burton Lamp. None of the patients developed keratitis, corneal neovascularization, or the sucked-on-lens syndrome. The lenses were well tolerated and the parents soon became adept at handling them. We now use RGP lenses as our lens of choice when treating aphakic infants.

  View details for Web of Science ID A1992JK32800011

  View details for PubMedID 1512667

• ISOLATED CONGENITAL HEMIANOPIA CAUSED BY PRENATAL INJURY TO THE OPTIC RADIATION ARCHIVES OF NEUROLOGY Ragge, N. K., Barkovich, A. J., Hoyt, W. F., Lambert, S. R. 1991; 48 (10): 1088-1091

  Abstract

  Isolated congenital hemianopias are typically caused by developmental abnormalities of the occipital lobe cortex. We describe two patients with an incidental partial hemianopia associated with unilateral periventricular leukomalacia that was acquired prenatally. Magnetic resonance imaging scans suggest a late second or early third trimester unilateral cerebral ischemic event.

  View details for Web of Science ID A1991GJ52600022

  View details for PubMedID 1929905

• EFFECTS OF APOMORPHINE, A DOPAMINE RECEPTOR AGONIST, ON OCULAR REFRACTION AND AXIAL ELONGATION IN A PRIMATE MODEL OF MYOPIA INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE Iuvone, P. M., TIGGES, M., Stone, R. A., Lambert, S., Laties, A. M. 1991; 32 (5): 1674-1677

  Abstract

  The authors examined the effect of local administration of a dopamine receptor agonist on visual deprivation-induced excessive ocular growth and myopia. Eight rhesus monkeys were monocularly deprived of vision from birth with opaque contact lenses. Four of the monkeys received drops of 1% apomorphine HCl 2-3 times/day in the occluded eye; the four control monkeys received vehicle only. Axial lengths were determined by A-scan ultrasonography at birth and at 5-7 months of age. The authors assessed the axial elongation by comparing the postnatal growth in the axial dimension of the occluded eyes with the postnatal growth in nonoccluded eyes. In three of the four control monkeys, occlusion increased axial growth by an average of 1.3 mm. In contrast, they found that growth of the occluded and nonoccluded eyes of the apomorphine-treated monkeys was equivalent, except in one monkey whose nonoccluded eye did not develop normally and was anomalously small. At 6.5-9.5 months of age, three of four controls had myopic refractive errors (-3 to -7 diopters) in the occluded eyes; three of four of the apomorphine-treated monkeys had hyperopic refractive errors (+1-(+)3 diopters) in their occluded eyes. The occluded eye of the fourth monkey was only -0.5 diopters myopic. The findings suggest that apomorphine administration retards excessive axial elongation and the concomitant development of myopia associated with visual deprivation in primates.

  View details for Web of Science ID A1991FH19700033

  View details for PubMedID 2016144

• DETECTION OF ISOLATED OCCIPITAL LOBE ANOMALIES DURING EARLY-CHILDHOOD DEVELOPMENTAL MEDICINE AND CHILD NEUROLOGY Lambert, S. R., Kriss, A., Taylor, D. 1990; 32 (5): 451-455

  Abstract

  The authors report two children with isolated occipital lobe anomalies detected by visual evoked potentials (VEPs) and confirmed by MRI and CT scanning. Both had a markedly asymmetrical occipital distribution of flash and pattern VEPs. One child had acuity reduced to 6/36, and testing on confrontation suggested an homonymous hemianopia. The second child was visually inattentive to one side during infancy. Neither child had band atrophy of the optic disc or an afferent pupillary defect. Isolated abnormalities of the occipital lobes are difficult to detect by clinical examination during infancy and early childhood. Recording VEPs from a horizontal array of occipital electrodes can be helpful in detecting subtle occipital lobe abnormalities.

  View details for Web of Science ID A1990DC43400010

  View details for PubMedID 2354756

• JOUBERT SYNDROME - A CLINICO-RADIOLOGICAL STUDY NEURORADIOLOGY Kendall, B., Kingsley, D., Lambert, S. R., Taylor, D., Finn, P. 1990; 31 (6): 502-506

  Abstract

  A characteristic malformation of the cerebellum, including dysgenesis of the vermis and enlargement of the fourth ventricle was observed on computed tomography (CT) in 16 children on review of our consecutive material. Seven of these children underwent magnetic resonance imaging (MRI) which showed hypoplasia of the brainstem in addition to cerebellar vermian dysgenesis. One child had, in addition, dysgenesis of the corpus callosum. All these children were developmentally delayed, and many had neonatal breathing abnormalities, congenital retinal dystrophy and supranuclear ocular motor abnormalities. Joubert's syndrome should be suspected in children in whom dysgenesis of the cerebellar vermis and hypoplasia of the brainstem is shown on CT or MRI.

  View details for Web of Science ID A1990CL09000008

  View details for PubMedID 2352633

• THE INFANT WITH NYSTAGMUS, NORMAL APPEARING FUNDI, BUT AN ABNORMAL ERG SURVEY OF OPHTHALMOLOGY Lambert, S. R., Taylor, D., Kriss, A. 1989; 34 (3): 173-186

  Abstract

  Many retinal disorders present during infancy with nystagmus, decreased vision, and normal-appearing fundi, but an abnormal ERG. The most common of these disorders are Leber's congenital amaurosis, achromatopsia, and congenital stationary night-blindness. Other disorders with similar ocular manifestations may be associated with a variety of life-threatening systemic abnormalities. This review describes the clinical, electrophysiological, and laboratory findings that can be used to distinguish among these conditions.

  View details for Web of Science ID A1989CD60800002

  View details for PubMedID 2694415

• AUTOSOMAL RECESSIVE MICROCEPHALY, MENTAL-RETARDATION WITH NONPIGMENTARY RETINOPATHY AND A DISTINCTIVE ELECTRORETINOGRAM NEUROPEDIATRICS Harbord, M. G., Lambert, S. R., Kriss, A., Brett, E. M., Baraitser, M., Supramaniam, G. 1989; 20 (3): 139-141

  Abstract

  The association of microcephaly and mental retardation with a non-pigmentary retinopathy is described in three siblings of consanguineous parents. The electroretinogram showed the distinctive appearance of markedly attenuated "b" wave but normal "a" wave suggestive of a retinal dystrophy primarily affecting post-receptoral elements in the inner retina. This appears to be an autosomal recessive condition which has not been previously reported.

  View details for Web of Science ID A1989AN16500003

  View details for PubMedID 2779741

• FOLLOW-UP AND DIAGNOSTIC REAPPRAISAL OF 75 PATIENTS WITH LEBERS CONGENITAL AMAUROSIS AMERICAN JOURNAL OF OPHTHALMOLOGY Lambert, S. R., Kriss, A., Taylor, D., Coffey, R., Pembrey, M. 1989; 107 (6): 624-631

  Abstract

  We reexamined 75 children in whom Leber's congenital amaurosis had been previously diagnosed. On review, 30 of these patients had an ocular or systemic disorder other than Leber's congenital amaurosis. The most common of these revised diagnoses were congenital stationary night blindness, achromatopsia, infantile-onset retinitis pigmentosa, Joubert's syndrome, Zellweger syndrome, and infantile Refsum's disease. Of the 45 patients with Leber's congenital amaurosis, mental retardation occurred in six patients, and visual deterioration in six patients. Leber's congenital amaurosis should only be diagnosed if other known ocular and systemic disorders have been carefully excluded.

  View details for Web of Science ID A1989U824900007

  View details for PubMedID 2658617

• JOUBERT SYNDROME ARCHIVES OF OPHTHALMOLOGY Lambert, S. R., Kriss, A., Gresty, M., Benton, S., Taylor, D. 1989; 107 (5): 709-713

  Abstract

  Joubert syndrome is an autosomal recessive condition in which there is a variable combination of central nervous system defects with a distinctive congenital retinal dystrophy, ocular motor abnormalities, and respiratory abnormalities in early infancy. The retinal dystrophy has been previously classified as a variant of Leber's congenital amaurosis. We report electrophysiologic and eye movement findings in a series of seven consecutive children with Joubert syndrome. Unlike patients with Leber's congenital amaurosis, all but one of these children had preserved flash and pattern-reversal visual evoked potentials. Six of the seven children had abnormalities of smooth pursuit, optokinetic nystagmus, and saccades. Six of the children had nystagmus: three had a pendular torsional nystagmus and three had a form of see-saw nystagmus. An alternating hyperdeviation was present in five of the patients, two of whom also had a tonic deviation of their eyes laterally. All seven patients had cerebellar vermis hypoplasia on a magnetic resonance imaging scan. Developmentally delayed children with an absent or highly attenuated electroretinogram should be investigated for Joubert syndrome.

  View details for Web of Science ID A1989U513600026

  View details for PubMedID 2470340

• DELAYED VISUAL MATURATION - A LONGITUDINAL CLINICAL AND ELECTROPHYSIOLOGICAL ASSESSMENT OPHTHALMOLOGY Lambert, S. R., Kriss, A., Taylor, D. 1989; 96 (4): 524-529

  Abstract

  Delayed visual maturation is an idiopathic condition characterized by visual inattention during infancy. The authors followed longitudinally nine children with an initial diagnosis of delayed visual maturation and compared their electroretinograms (ERGs) and visual evoked potentials with those of age-matched controls. Eight of the nine patients consistently had normal visual evoked potentials to flash and to pattern stimulation. All of the children had normal ERGs. Visually mediated behavior gradually developed in all of these children when they were between 3 and 8 months of age (mean, 5.5 months). Five patients also were delayed in other spheres of development. Visual evoked potentials are helpful in formulating a visual prognosis for children with delayed visual maturation.

  View details for Web of Science ID A1989U004000017

  View details for PubMedID 2726183

• DETECTION AND TREATMENT OF INFANTILE CATARACTS INTERNATIONAL OPHTHALMOLOGY CLINICS Lambert, S. R., Amaya, L., Taylor, D. 1989; 29 (1): 51-56

  View details for Web of Science ID A1989T328200012

  View details for PubMedID 2917818

• OCULAR MANIFESTATIONS OF THE CONGENITAL VARICELLA SYNDROME ARCHIVES OF OPHTHALMOLOGY Lambert, S. R., Taylor, D., Kriss, A., Holzel, H., Heard, S. 1989; 107 (1): 52-56

  Abstract

  Three children developed the congenital varicella syndrome following a maternal varicella infection during the second trimester of pregnancy. Systemic findings included a bulbar palsy, mild hemiparesis, cicatricial skin lesions, developmental delay, and learning difficulties. Ocular findings included chorioretinitis, atrophy and hypoplasia of the optic discs, a congenital cataract, and Horner's syndrome. Electroretinograms and pattern-reversal visual-evoked potentials recorded from the four eyes with chorioretinitis were attenuated and degraded. Two of the children had been misdiagnosed as having congenital toxoplasmosis. Chorioretinal scars probably occur more frequently with the congenital varicella syndrome than has been previously recognized and should be included in the differential diagnosis of congenital chorioretinal scars. Hypoplasia of the optic disc may develop after an intrauterine varicella infection during the 16th week of gestation. Children with the congenital varicella syndrome should be examined by an ophthalmologist to exclude ocular abnormalities. These abnormalities are often subtle and difficult to diagnose correctly.

  View details for Web of Science ID A1989R752500020

  View details for PubMedID 2910286

• CONGENITAL IDIOPATHIC MICROCORIA AMERICAN JOURNAL OF OPHTHALMOLOGY Lambert, S. R., Amaya, L., Taylor, D. 1988; 106 (5): 590-594

  Abstract

  Five infants had congenital unilateral microcoria. The red reflex was absent and vision was severely reduced in all five eyes preoperatively. A pupil was created surgically in three of the eyes, resulting in a variable improvement in vision. The underlying lens was clear in all three children. The origin of the pupillary anomaly is unknown, but probably is related to remnants of the fetal pupillary membrane. Early surgical treatment and vigorous occlusion therapy can result in useful vision.

  View details for Web of Science ID A1988Q822800013

  View details for PubMedID 3189475

• VISUAL RECOVERY FROM HYPOXIC CORTICAL BLINDNESS DURING CHILDHOOD ARCHIVES OF OPHTHALMOLOGY Lambert, S. R., Hoyt, C. S., Jan, J. E., Barkovich, J., Flodmark, O. 1987; 105 (10): 1371-1377

  Abstract

  We reviewed the clinical courses and computed tomographic (CT) and magnetic resonance imaging (MRI) scans of 30 infants and children with cortical blindness following hypoxic insults. The degree of injury to the striate and parastriate cortices and the area of the optic radiations were graded from 0 to 4 by a neuroradiologist. Only two children had normal scans of the posterior visual pathway and both had favorable visual outcomes. The visual recovery differed significantly with respect to the age at which the hypoxic insult occurred and CT and MRI abnormalities in the area of the optic radiations, but not with abnormalities in the striate or parastriate cortices. Our results suggest that CT and MRI scanning are helpful in prognosticating the visual potential of children with hypoxic cortical blindness.

  View details for Web of Science ID A1987K258700024

  View details for PubMedID 3662911

• OPTIC-NERVE HYPOPLASIA SURVEY OF OPHTHALMOLOGY Lambert, S. R., Hoyt, C. S., NARAHARA, M. H. 1987; 32 (1): 1-9

  Abstract

  Optic nerve hypoplasia is an easily overlooked, nonprogressive developmental anomaly which results in a wide range of visual deficits. It is frequently associated with clinically significant central nervous system and endocrine abnormalities. Maternal substance abuse is increasingly recognized in many cases. A supranormal regression of optic nerve axons in utero, rather than a primary failure of differentiation, is proposed as the pathogenesis.

  View details for Web of Science ID A1987K222000001

  View details for PubMedID 3310293

• OPTIC-NERVE SHEATH AND RETINAL HEMORRHAGES ASSOCIATED WITH THE SHAKEN BABY SYNDROME ARCHIVES OF OPHTHALMOLOGY Lambert, S. R., Johnson, T. E., Hoyt, C. S. 1986; 104 (10): 1509-1512

  Abstract

  A 13-month-old child with the pathognomonic findings of the shaken baby syndrome died secondary to cerebellar herniation. Pathologic examination disclosed extensive intraocular, optic nerve sheath, and intracranial hemorrhages, despite a paucity of external signs of trauma. Many of the hemorrhages were old, suggesting that the child had experienced multiple episodes of trauma. Hemorrhages of the optic nerve sheaths have not been previously reported with the shaken baby syndrome but probably accompany this condition frequently. Our patient's ocular hemorrhages may have resulted from a sudden rise in intracranial pressure.

  View details for Web of Science ID A1986E235500024

  View details for PubMedID 3767684

• SPONTANEOUS REGRESSION OF A CHOROIDAL MELANOMA ARCHIVES OF OPHTHALMOLOGY Lambert, S. R., Char, D. H., Howes, E., Crawford, J. B., Wells, J. 1986; 104 (5): 732-734

  Abstract

  Although spontaneous regression of uveal malignant melanoma is rare, its occurrence is not uncommon in cutaneous melanomas. We report a 15-year follow-up of an enlarging posterior pigmented tumor. It initially appeared to spontaneously regress into a flat chorioretinal scar, but, ten years later, it grew markedly and the eye was enucleated. Histopathologic changes similar to those found in regressed cutaneous melanomas were located in the region of the chorioretinal scar.

  View details for Web of Science ID A1986C174900029

  View details for PubMedID 3707414

• SEROUS RETINAL DETACHMENTS IN THROMBOTIC THROMBOCYTOPENIC PURPURA ARCHIVES OF OPHTHALMOLOGY Lambert, S. R., High, K. A., Cotlier, E., Benz, E. J. 1985; 103 (8): 1172-1174

  Abstract

  Serous retinal detachments are a rare ocular complication of thrombotic thrombocytopenic purpura (TTP), with only six previous case reports in the literature. We have recently observed two patients with relapsing TTP who developed bilateral serous retinal detachments during acute exacerbations of their disease. In contrast to all but one of the previously described patients, both of our patients survived the episode during which retinal detachments occurred. Hypertension appears to contribute to the development of retinal detachments in TTP, and vigorous efforts at blood pressure control are indicated. Serous retinal detachments may be a more frequent component of chronic relapsing TTP than has been suspected.

  View details for Web of Science ID A1985ANL3100019

  View details for PubMedID 4040743

• DOWN-REGULATION OCCURS NORMALLY IN CULTURED EPSTEIN-BARR VIRUS-TRANSFORMED LYMPHOCYTES FROM PATIENTS WITH EXTREME INSULIN RESISTANCE - DISCREPANCY BETWEEN DOWN-REGULATION INVIVO AND INVITRO DIABETES Wagman, L. D., Lambert, S. R., McElduff, A., Roth, J., GORDEN, P., Taylor, S. I. 1984; 33 (5): 421-427

  Abstract

  Levels of fasting plasma insulin are generally inversely correlated with 125I-insulin binding to circulating blood cells. In disease states associated with hyperinsulinemia (e.g., obesity and non-insulin-dependent diabetes mellitus), 125I-insulin binding is usually low. In contrast, 125I-insulin binding to circulating cells may be normal in patients with certain forms of extreme insulin resistance despite marked hyperinsulinemia. To explain this paradox, it has been proposed that postbinding defects in insulin action may give rise to defects in downregulation. We have employed cultured Epstein-Barr virus (EBV)-transformed lymphocytes from eight patients with extreme insulin resistance to address the question of whether there is a defect in the downregulation process in vitro. In this cell type, insulin leads to a decrease in the number of insulin receptors on the cell surface by accelerating the rate of degradation of insulin receptors. We could not detect any abnormality in in vitro down-regulation with cultured EBV-transformed lymphocytes from insulin-resistant patients. The apparent discrepancy between the in vivo and in vitro studies raises the possibility that some factor in the patient's internal milieu may prevent insulin-induced downregulation. An alternative possible explanation might be that the mechanism of downregulation in vitro differs from the mechanism whereby receptor number is regulated in vivo in insulin's target cells.

  View details for Web of Science ID A1984SR39400003

  View details for PubMedID 6327434

• THE ORIGINS OF SATTLERS VEIL AMERICAN JOURNAL OF OPHTHALMOLOGY Lambert, S. R., Klyce, S. D. 1981; 91 (1): 51-56

  Abstract

  The symptoms of Sattler's veil accompany prolonged wear of thick contact lenses and are thought to be the result of epithelial hypoxia. We studied light scattering in the isolated rabbit cornea after hypoxia with optical techniques. We found that hypoxia produced a polygonal mesh of light scattering, outlining cells in the intermediate and basal epithelium. An optical transform of this mesh produced a halo of the same dimensions observed in Sattler's veil. Additionally, prolonged hypoxia produced an increase in stromal light scattering that may add to increased glare sensitivity. Consistent changes in epithelial thickness were not observed, raising the probability of radial swelling or compensatory epithelial volume regulation.

  View details for Web of Science ID A1981KZ33500007

  View details for PubMedID 7234929