Assessment of intraocular foreign body using high resolution 3D ultrasound imaging.

Ocular trauma often involves intraocular foreign bodies (IOFBs) that pose challenges in accurate diagnosis due to their size, shape, and material composition. In this study, we proposed a novel whole-eye 3D ophthalmic ultrasound B-scan (3D-UBS) system for automating image acquisition and improved 3D visualization, thereby improving sensitivity for detecting IOFBs. 3D-UBS utilizes 14 MHz Clarius L20 probe, a motorized translation stage, and a surgical microscope for precise placement and movement. The system's 3D point spread function (PSF) is 0.377 × 0.550 × 0.894 mm3 characterized by the full-width at half-maximum intensity values in the axial, lateral and elevation directions. Digital phantom and ex vivo ocular models were prepared using four types of IOFBs (i.e., plastic, wood, metal, and glass). Ex vivo models were imaged with both 3D-UBS and clinical computed tomography (CT). Image preprocessing was performed on 3D-UBS images to remove uneven illumination and speckle noise. Multiplanar reformatting in 3D-UBS provides optimal plane selection after acquisition, reducing the need for a trained ultrasonographer. 3D-UBS outperforms CT in contrast for wood and plastic, with mean contrast improvement of 2.43 and 1.84 times, respectively. 3D-UBS was able to identify wood and plastic IOFBs larger than 250 µm and 300 in diameter, respectively. CT, with its wider PSF, was only able to detect wood and plastic IOFBs larger than 600 and 550 µm, respectively. Although contrast was higher in CT for metal and glass IOFBs, 3D-UBS provided sufficient contrast to identify those. 3D-UBS provides an easy-to-use, non-expert imaging approach for identifying small IOFBs of different materials and related ocular injuries at the point of care.

Nonperfusion of the Far Peripheral Retina in Highly Myopic Stickler Syndrome.

Purpose: To describe the clinical course of 3 patients with Stickler syndrome found on fluorescein angiography (FA) to have nonperfusion of the peripheral retina. Methods: Three patients with confirmed Stickler syndrome were examined under anesthesia. Genetic testing and FA were performed. Results: Each patient had characteristic ocular findings of Stickler syndrome, including high myopia with vitreoretinal degeneration. FA was performed on each patient and showed 360 degrees of nonperfusion of the retinal periphery in all eyes, with mild leakage in Case 3. Conclusions: The current series presents evidence of peripheral retinal nonperfusion in 3 consecutive patients with Stickler syndrome. Based on these findings, the authors recommend adopting FA as a standard imaging modality and using laser photocoagulation to treat the areas of retinal nonperfusion for all patients with Stickler syndrome.

Lack of racial and ethnic diversity in pediatric ophthalmology clinical trials from 2000 to 2022.

PURPOSE: To examine the prevalence of and factors associated with racial and ethnic reporting and trends in such reporting and to assess whether categories of race and ethnicity have been under- or over-represented in pediatric ophthalmology randomized control trials (RCTs) in the United States. METHODS: We systematically searched the literature on pediatric ophthalmology RCTs in high-impact factor ophthalmology journals published between 2000 and 2022. Logistic regression was used to assess parameters linked to race/ethnicity reporting; linear regression, to gauge the relationship between publication year and race/ethnicity reporting. The racial and ethnic composition of RCTs was contrasted with 2010 US census data by calculating percentage difference. RESULTS: Of 170 eligible articles, 89 (52.4%) included race/ethnicity data. Multivariable analysis showed that academic (OR = 12.19; 95% CI, 3.34-44.44) and government (OR = 3.91; 95% CI, 1.20-12.72) funding was linked to data reporting. During the study period, publication year and race/ethnicity reporting had a nonstatistically significant 1.0% annual increase (r = 0.29, P = 0.18). White participants were over-represented, with a percentage difference of 16.7% (95% CI, 11.8%-21.7%), whereas Hispanic individuals were under-represented, with a percentage difference of -7.6% (95% CI, -11.2% to -4.1%) compared to the 2010 US census data. CONCLUSIONS: Our results indicate a gradual rise in reported race and/or ethnicity in published pediatric ophthalmology RCTs, though not statistically significant, both in the United States and globally. Notably, under-representation of Hispanic, over-representation of White, and proportional representation of Black and Asian individuals were observed in US-based studies.

Critical Role of CD55 in Controlling Wound Healing.

How reparative processes are coordinated following injury is incompletely understood. In recent studies, we showed that autocrine C3a and C5a receptor (C3ar1 and C5ar1) G protein-coupled receptor signaling plays an obligate role in vascular endothelial growth factor receptor 2 growth signaling in vascular endothelial cells. We documented the same interconnection for platelet-derived growth factor receptor growth signaling in smooth muscle cells, epidermal growth factor receptor growth signaling in epidermal cells, and fibroblast growth factor receptor signaling in fibroblasts, indicative of a generalized cell growth regulatory mechanism. In this study, we examined one physiological consequence of this signaling circuit. We found that disabling CD55 (also known as decay accelerating factor), which lifts restraint on autocrine C3ar1/C5ar1 signaling, concomitantly augments the growth of each cell type. The mechanism is heightened C3ar1/C5ar1 signaling resulting from the loss of CD55's restraint jointly potentiating growth factor production by each cell type. Examination of the effect of lifted CD55 restraint in four types of injury (burn, corneal denudation, ear lobe puncture, and reengraftment of autologous skin) showed that disabled CD55 function robustly accelerated healing in all cases, whereas disabled C3ar1/C5ar1 signaling universally retarded it. In wild-type mice with burns or injured corneas, applying a mouse anti-mouse CD55 blocking Ab (against CD55's active site) to wounds accelerated the healing rate by 40-70%. To our knowledge, these results provide new insights into mechanisms that underlie wound repair and open up a new tool for accelerating healing.

Outcomes for maximal bilateral lateral rectus recession in large-angle exotropia.

BACKGROUND: Surgical treatment for large-angle exotropia can be challenging. The aim of this study was to evaluate short-term surgical outcomes of patients with large-angle exotropia (≥50Δ) undergoing maximal bilateral lateral rectus muscle recession of 10 mm. METHODS: This was a retrospective study of consecutive patients at our institution who underwent maximal bilateral lateral rectus muscle recession for exodeviation ≥50Δ from January 1, 2008, to July 22, 2022. We subdivided the cohort into large-angle exotropia (largest amount of exodeviation at near and/or distance ≥50Δ and <65Δ) and very large-angle exotropia (largest exodeviation ≥65Δ). Patients with a history of prior eye muscle surgery, neurologic deficits, and three- or four-muscle surgery were excluded. RESULTS: A total of 22 patients were included. Mean preoperative exodeviation at distance was 51.9Δ in the large-angle group and 67.5Δ in the very-large-angle group (P = 0.001). Outcomes for the large-angle and very-large angle groups were, respectively, as follows: mean follow-up, 31.1 weeks and 11.8 weeks (P = 0.97); success, 75.0% and 16.7% (P = 0.02); undercorrection rates, 18.7% and 83.3% (P = 0.01); and mean postoperative exodeviation at distance, 3.7Δ ± 6.3Δ and 28.0Δ ± 13.5Δ (P = 0.001). CONCLUSIONS: Our study identified good surgical outcomes (75%) with maximal bilateral lateral rectus muscle recession of 10 mm in treating patients with large-angle exotropia between 50Δ and <65Δ. Other surgical techniques such as recession-resection and three- or four-muscle surgery may result in better outcomes when treating patients with exotropia ≥65Δ.

The impact of the first peak of the COVID-19 pandemic on childhood myopia control practice patterns among ophthalmologists-an international pediatric ophthalmology and strabismus council global perspective.

BACKGROUND: The prevalence of myopia keeps increasing during the COVID-19 pandemic. We aimed to map the worldwide treatment preferences of ophthalmologists managing myopia control during the first wave of the pandemic. METHODS: An online questionnaire inquiring about pharmacological and optical treatment patterns during the first half of 2020 was sent to pediatric ophthalmology as well as general ophthalmology memberships worldwide. The results among pediatric ophthalmologists were compared to a previous study we performed before the pandemic. RESULTS: A total of 2269 respondents from 94 countries were included. Most respondents were pediatric ophthalmologists (64.6%), followed by ophthalmologists from other subspecialties (32.3%). The preferred modality for all geographical regions was a combination therapy of pharmacological and optical treatments. When evaluated independently, the pharmacological treatment was more popular than the optical treatment in most regions other than East Asia (P < 0.001). Compared to a pre-pandemic questionnaire, the participation of pediatric ophthalmologists affiliated with non-university hospitals increased. Additionally, the prevalence of respondents utilizing either any type of pharmacological treatment and those that using only evidence-based treatments increased globally. Although a decline in the use of optical treatment was evident worldwide, the use of evidence-based optical treatments increased. CONCLUSION: Ophthalmologists around the world preferred a combination therapy of pharmacological and optical treatments. More pediatric ophthalmologists treated myopia progression and preferred a better evidence-based approach to control myopia. These trends reflect a positive response and more awareness of the rising prevalence of myopia due to the increased burden of myopia imposed by the COVID-19 pandemic.

Deep Learning Segmentation, Visualization, and Automated 3D Assessment of Ciliary Body in 3D Ultrasound Biomicroscopy Images.

Purpose: This study aimed to develop a fully automated deep learning ciliary body segmentation and assessment approach in three-dimensional ultrasound biomicroscopy (3D-UBM) images. Methods: Each 3D-UBM eye volume was aligned to the optic axis via multiplanar reformatting. Ciliary muscle and processes were manually annotated, and Deeplab-v3+ models with different loss functions were trained to segment the ciliary body (ciliary muscle and processes) in both en face and radial images. Results: We trained and tested the models on 4320 radial and 3864 en face images from 12 cadaver eye volumes. Deep learning models trained on radial images with Dice loss achieved the highest mean F1-score (0.89) for ciliary body segmentation. For three-class segmentation (ciliary muscle, processes, and background), radial images with Dice loss achieved the highest mean F1-score (0.75 for the ciliary process and 0.82 for the ciliary muscle). Part of the ciliary muscle (10.9%) was misclassified as the ciliary process and vice versa, which occurred owing to the difficulty in differentiating the ciliary muscle-processes border, even by experts. Deep learning segmentation made further editing by experts at least seven times faster than a fully manual approach. In eight cadaver eyes, the average ciliary muscle, process, and body volumes were 56 ± 9, 43 ± 13, and 99 ± 18 mm3, respectively. The average surface area of the ciliary muscle, process, and body were 346 ± 45, 363 ± 83, and 709 ± 80 mm2, respectively. We performed transscleral cyclophotocoagulation in cadaver eyes to shrink the ciliary processes. Both manual and automated measurements from deep learning segmentation show a decrease in volume, surface area, and 360° cross-sectional area measurements. Conclusions: The proposed deep learning segmentation of the ciliary body and 3D measurements showed transscleral cyclophotocoagulation-related changes in the ciliary body. Translational Relevance: Automated ciliary body assessment using 3D-UBM has the translational potential for ophthalmic treatment planning and monitoring.

Clinical 3D Imaging of the Anterior Segment With Ultrasound Biomicroscopy.

Purpose: Ultrasound biomicroscopy (UBM) is an important ophthalmic imaging modality due to its ability to see behind pigmented iris and to visualize anterior chamber when the eye's transparency is compromised. We created a three-dimensional UBM (3D-UBM) system and acquired example images to illustrate its potential. Methods: A commercial 50-MHz two-dimensional UBM (2D-UBM) system was attached to a precision translation stage and translated across the eye to acquire an image volume. The stage was mounted on a surgical microscope, which enabled safe, stable positioning. Image processing steps included image alignment, noise reduction, and calibration. 3D visualization included alignment of the optic axis, multiplanar reformatting at arbitrary orientations, and volume rendering with optimized transfer functions. Scans were performed on cadaver and rabbit eyes. Results: 3D-UBM allowed visualization of the anterior segment tissues within a 3D anatomical context, unlike 2D-UBM. En face views and interactive slicer operations suggested an ability to plan and assess treatments, including lens placement and microcatheter cannulation of Schlemm's canal. Interactive software allowed us to make accurate measurements of tissue structures (e.g., iridocorneal angles, cyst volumes). In addition, unique measurements of ciliary tissues included single ciliary process volumes of 0.234 ± 0.093 mm3 with surface areas of 3.02 ± 1.07 mm2 and ciliary muscle volume of 67.87 mm3. Conclusions: 3D-UBM imaging of the anterior segment can be used to enable unique visualization and quantification of anterior segment structures. Translational Relevance: 3D-UBM provides informative 3D imaging of tissues in the eye that are invisible to light to potentially provide physicians with improved diagnosis, treatment planning, and treatment assessment as compared to conventional 2D-UBM.

Neurodevelopmental outcome of preterm infants enrolled in myo-inositol randomized controlled trial.

OBJECTIVE: This study evaluates the 24-month follow-up for the NICHD Neonatal Research Network (NRN) Inositol for Retinopathy Trial. STUDY DESIGN: Bayley Scales of Infants Development-III and a standardized neurosensory examination were performed in infants enrolled in the main trial. Moderate/severe NDI was defined as BSID-III Cognitive or Motor composite score <85, moderate or severe cerebral palsy, blindness, or hearing loss that prevents communication despite amplification were assessed. RESULTS: Primary outcome was determined for 605/638 (95%). The mean gestational age was 25.8 ± 1.3 weeks and mean birthweight was 805 ± 192 g. Treatment group did not affect the risk for the composite outcome of death or survival with moderate/severe NDI (60% vs 56%, p = 0.40). CONCLUSIONS: Treatment group did not affect the risk of death or survival with moderate/severe NDI. Despite early termination, this study represents the largest RCT of extremely preterm infants treated with myo-inositol with neurodevelopmental outcome data.

CASE REPORT OF THE ROLE OF OPTICAL COHERENCE TOMOGRAPHY IN RECOMBINANT GROWTH HORMONE THERAPY.

PURPOSE: To report the correlation between recombinant growth hormone (rhGH) dosage and retinal nerve fiber layer (RNFL) thickness values measured by optical coherence tomography in a case of pseudotumor cerebri syndrome (PTCS) after rhGH. METHODS: An 11-year-old girl was receiving rhGH for panhypopituitarism. The patient developed PTCS, and her rhGH dose was adjusted using optical coherence tomography RNFL thickness measurements. The linear correlation coefficient (r) and coefficient of determination (r2) were calculated to assess the relationship between RNFL thickness and rhGH dose. RESULTS: As the rhGH dosage was increased, the RNFL thickness values also increased, especially when acetazolamide was excluded because of its confounding effect. (r = 0.64) In separate subgroup analysis, a higher acetazolamide dosage strongly correlated with reduced RNFL thickness (r = 0.77). CONCLUSION: Although PTCS is a rare complication after rhGH therapy, its detrimental effects cannot be ignored. In our case report, we used optical coherence tomography RNFL values in addition to clinical findings to carefully titrate the rhGH dosage to prevent a flare-up of PTCS. Despite the obvious need for larger studies, our case report shows the value of RNFL thickness measured by optical coherence tomography and the valuable additional data it provides to refine rhGH therapy as an adjunct noninvasive method in PTCS.

Atypical ophthalmic manifestations of disseminated intravascular coagulation in a newborn.

Ophthalmic manifestations of disseminated intravascular coagulation (DIC) in a newborn are rare. Ocular involvement typically involves the posterior segment bilaterally and manifests as choroidal and/or retinal hemorrhages associated with fibrin thrombi in the choriocapillaris. We present the case of a newborn girl with DIC secondary to sepsis who subsequently developed nonclearing hyphema, related secondary cataract, 360° posterior synechiae, and nonclearing vitreous hemorrhage in the right eye and diffuse retinal hemorrhage in the left eye. The right fundus was not visible because of intraocular bleeding. The patient underwent cataract surgery at 39 days of life and was left aphakic. Two weeks later, she required subsequent vitrectomy because of a nonclearing vitreous hemorrhage. She developed amblyopia of the right eye and a right esotropia that required strabismus surgery. At 7 years of age, the patient's visual acuity was 20/50 in the right eye and 20/20 in the left eye.

Contractile morning glory disk anomaly: analysis of the cyclic contractions and literature review.

PURPOSE: To investigate the contraction mechanism of morning glory disk anomaly using computer-assisted analysis of the cyclic contractions frame by frame and to review the literature on contractile morning glory disk anomaly cases. METHODS: The fundus video of the patient under anesthesia was recorded using RetCam C300 lens. Frames at 1 second intervals were extracted to quality-preserving TIFF images and manually selected areas of cup, disk, and pigmented ring were measured with image analysis software. Measurements were repeated twice, and estimated uncertainties were calculated. RESULTS: Two full-contraction phases and 1 half- and 1 full-dilation phase were recorded and analyzed. At the end of the second dilation phase, cup, disk, and pigmented ring were dilated to 93.7%, 97.6%, and 98.3% of their areas after first dilation. At the end of the second contraction cup, disk and pigmented ring areas remained 1.24, 1.01, and 1.01 times larger, respectively, than the areas at the end of the first contraction. CONCLUSIONS: Ectopic cholinergic muscle contraction alone is unlikely to explain the cyclic contraction dilation movements under constant stimulus. Potentially present smooth muscle cells can be provoked by the stretch caused by the influx of fluid into the subretinal space. Change in spatial configuration and oscillations with decreasing magnitudes can be explained by decreasing contractions due to decreasing stretch with decreasing fluid influx in each cycle after the initial triggering of outside pressure, causing the greatest pressure gradient and the greatest fluid influx.

Incidence and Management of Glaucoma or Glaucoma Suspect in the First Year After Pediatric Lensectomy.

Importance: Glaucoma can occur following cataract removal in children, and determining the risk for and factors associated with glaucoma and glaucoma suspect in a large cohort of children after lensectomy can guide clinical practice. Objective: To estimate the incidence of glaucoma and glaucoma suspect and describe its management in the first year following lensectomy in children before 13 years of age. Design, Setting, and Participants: A multicenter clinical research registry containing data for 1361 eyes of 994 children who underwent unilateral or bilateral lensectomy between June 2012 and July 2015 at 1 of 61 sites in the United States (n = 57), Canada (n = 3), and the United Kingdom (n = 1). Patients were eligible for inclusion in the study if they were enrolled in the registry within 45 days after lensectomy and had at least 1 office visit between 6 and 18 months after lensectomy. Patient data were reviewed, and glaucoma and glaucoma suspect were diagnosed by investigators using standardized criteria. Statistical analysis was performed between June 2017 and August 2019. Exposures: Clinical care 6 to 18 months after lensectomy. Main Outcomes and Measures: Incidence risk using standardized definitions of glaucoma and glaucoma suspect after lensectomy. Results: Among 702 patients included in this cohort study, 353 (50.3%) were male and 427 (60.8%) were white; mean age at lensectomy was 3.4 years (range, 0.04-12.9 years). After lensectomy, glaucoma or glaucoma suspect was diagnosed in 66 of 970 eyes (adjusted overall incidence risk, 6.3%; 95% CI, 4.8%-8.3%). Glaucoma was diagnosed in 52 of the 66 eyes, and glaucoma suspect was diagnosed in the other 14 eyes. Mean age at lensectomy in these 66 eyes was 1.9 years (range, 0.07-11.2 years), and 40 of the 66 (60.6%) were eyes of female patients. Glaucoma surgery was performed in 23 of the 66 eyes (34.8%) at a median of 3.3 months (range, 0.9-14.8 months) after lensectomy. The incidence risk of glaucoma or glaucoma suspect was 15.7% (99% CI, 10.1%-24.5%) for 256 eyes of infants 3 months or younger at lensectomy vs 3.4% (99% CI, 1.9%-6.2%) for 714 eyes of infants older than 3 months (relative risk, 4.57; 99% CI, 2.19-9.57; P < .001) and 11.2% (99% CI, 7.6%-16.7%) for 438 aphakic eyes vs 2.6% (99% CI, 1.2%-5.6%) for 532 pseudophakic eyes (relative risk, 4.29; 99% CI, 1.84-10.01; P < .001). No association was observed between risk of developing glaucoma or glaucoma suspect and any of the following variables: sex, race/ethnicity, laterality of lensectomy, performance of anterior vitrectomy, prelensectomy presence of anterior segment abnormality, or intraoperative complications. Conclusions and Relevance: This study found that glaucoma or glaucoma suspect developed in a small number of eyes in the first year after lensectomy and may be associated with aphakia and younger age at lensectomy. Frequent monitoring for signs of glaucoma following lensectomy is warranted, especially in infants 3 months or younger at lensectomy and in children with aphakia after lensectomy.

Effects of Myo-inositol on Type 1 Retinopathy of Prematurity Among Preterm Infants <28 Weeks' Gestational Age: A Randomized Clinical Trial.

Importance: Previous studies of myo-inositol in preterm infants with respiratory distress found reduced severity of retinopathy of prematurity (ROP) and less frequent ROP, death, and intraventricular hemorrhage. However, no large trials have tested its efficacy or safety. Objective: To test the adverse events and efficacy of myo-inositol to reduce type 1 ROP among infants younger than 28 weeks' gestational age. Design, Setting, and Participants: Randomized clinical trial included 638 infants younger than 28 weeks' gestational age enrolled from 18 neonatal intensive care centers throughout the United States from April 17, 2014, to September 4, 2015; final date of follow-up was February 12, 2016. The planned enrollment of 1760 participants would permit detection of an absolute reduction in death or type 1 ROP of 7% with 90% power. The trial was terminated early due to a statistically significantly higher mortality rate in the myo-inositol group. Interventions: A 40-mg/kg dose of myo-inositol was given every 12 hours (initially intravenously, then enterally when feeding; n = 317) or placebo (n = 321) for up to 10 weeks. Main Outcomes and Measures: Type 1 ROP or death before determination of ROP outcome was designated as unfavorable. The designated favorable outcome was survival without type 1 ROP. Results: Among 638 infants (mean, 26 weeks' gestational age; 50% male), 632 (99%) received the trial drug or placebo and 589 (92%) had a study outcome. Death or type 1 ROP occurred more often in the myo-inositol group vs the placebo group (29% vs 21%, respectively; adjusted risk difference, 7% [95% CI, 0%-13%]; adjusted relative risk, 1.41 [95% CI, 1.08-1.83], P = .01). All-cause death before 55 weeks' postmenstrual age occurred in 18% of the myo-inositol group and in 11% of the placebo group (adjusted risk difference, 6% [95% CI, 0%-11%]; adjusted relative risk, 1.66 [95% CI, 1.14-2.43], P = .007). The most common serious adverse events up to 7 days of receiving the ending dose were necrotizing enterocolitis (6% for myo-inositol vs 4% for placebo), poor perfusion or hypotension (7% vs 4%, respectively), intraventricular hemorrhage (10% vs 9%), systemic infection (16% vs 11%), and respiratory distress (15% vs 13%). Conclusions and Relevance: Among premature infants younger than 28 weeks' gestational age, treatment with myo-inositol for up to 10 weeks did not reduce the risk of type 1 ROP or death vs placebo. These findings do not support the use of myo-inositol among premature infants; however, the early termination of the trial limits definitive conclusions.

Longitudinal Studies and Eye-Movement-Based Treatments of Infantile Nystagmus Syndrome: Estimated and Measured Therapeutic Improvements in Three Complex Cases.

INTRODUCTION AND PURPOSE: To demonstrate the utility of using eye-movement data to reveal the diagnostic characteristics of infantile nystagmus syndrome (INS), determine treatment, and both estimate and document therapeutic improvements in three patients with well-developed foveation periods, fairly broad, lateral gaze "nulls," head turns, strabismus, and complex, multiplanar nystagmus. PATIENTS AND METHODS: Infrared reflection, magnetic search coil, and high-speed digital video systems were used to record the eye movements of INS patients, pre- and post-Kestenbaum null-point correction surgery (horizontal or vertical). Data were analyzed and estimations made, using the eXpanded Nystagmus Acuity Function (NAFX) that is part of the OMtools toolbox for MATLAB. RESULTS: In all three subjects (S1-S3), both peak NAFX and longest foveation domain (LFD) improved from their pre-Kestenbaum values. S1: 0.700-0.745 (6.4%) and 25-34° (36%), respectively. S2: 0.445-0.633 (42.4%) and >40° to >50° (10%), respectively. S3: 0.250-0.300 (20%) and 13° to ≫18° (see text), respectively. CONCLUSIONS: S1: Even at the high ends of the pre-therapy NAFX and LFD spectra, INS foveation (and therefore, visual-function) improvements may be adequate to justify nystagmus surgery and provide clinical improvements beneficial to the patient. S2: INS foveation improvements in the vertical plane are equal to those originally estimated using the horizontal data in prior patients. S3: Two apparent NAFX peaks can be converted into a very broad peak by surgery based on the preferred lower peak.

Effects of augmented tenotomy and reattachment in the infantile nystagmus syndrome.

PURPOSE: To test the hypothesis that augmented tenotomy and reattachment surgery (AT-R), which involves placing an additional suture in each distal tendon during the 4-muscle tenotomy and reattachment (T-R) or other infantile nystagmus syndrome (INS) procedures, could increase the beneficial effects of many types of extraocular muscle (EOM) surgery to treat INS. METHODS: Both infrared reflection and high-speed digital video systems were used to record the eye movements in 4 patients with INS before and after AT-R surgery. Data were analyzed using the eXpanded Nystagmus Acuity Function (NAFX) that is part of the OMtools software. RESULTS: Placement of the augmentation suture did not interfere with Kestenbaum, Anderson, bilateral medial rectus muscle recession, or T-R surgeries. The therapeutic effects of AT-R were similar to but not equal to those from the traditional single-suture surgeries (ie, broadening longest foveation domain [LFD] but no improvement of NAFX peak). The average of the NAFX percent improvements after AT-R was within 31% of those estimated from NAFX values before T-R; the average of the percent broadenings of the LFD values after AT-R was within 16%. CONCLUSIONS: The AT-R does not improve the foveation quality in INS above the traditional T-R surgery. It is not improved by an additional suture; indeed, some improvements may be diminished by the added suture. The hypothesized augmented-tendon suture technique (sans tenotomy) has been modified and remains to be tested.

Ocular manifestations of X-linked dominant FAM58A mutation in toe syndactyly, telecanthus, anogenital, and renal malformations ('STAR') syndrome.

PURPOSE: To report the newest ophthalmic manifestations of a mother-daughter pair diagnosed with toe syndactyly, telecanthus, anogenital and renal malformations (STAR) syndrome, a rare X-linked developmental disorder. METHODS: The medical and ophthalmic records were reviewed for a mother-daughter pair diagnosed with FAM58A confirmed STAR syndrome on chromosome Xq28. RESULTS: The mother at birth had left foot syndactyly, telecanthus, anal stenosis, and clitoromegaly and was told at 19 she had a hypoplastic left kidney. The daughter, born at 38 weeks after a complication of oligohydramnios, had a more severe presentation, demonstrating toe syndactyly, telecanthus, anal stenosis, clitoromegaly, bilateral renal hypoplasia, ureteral reflux, urogenital sinus, and congenital heart disease amongst others. The pair shared similar ophthalmic findings, though those of the daughter were more pronounced. They included bilateral, medial upper eyelid prominences with madarosis, mild peripapillary atrophy, and soft macular drusen with the daughter also displaying optic nerve hypoplasia and peripheral anterior synechia in the iridocorneal angle. CONCLUSION: These ophthalmic findings are the first reported to our knowledge in association with STAR syndrome. The literature frequently demonstrates that patients with developmental anomalies often have ocular manifestations, warranting a full ophthalmic examination when the diagnosis of STAR syndrome has been made or is being considered.

Visual Acuity Improvement of Amblyopia in an Adult With Levodopa/Carbidopa Treatment.

Amblyopia is the leading cause of visual loss in children, affecting 2% to 3% of the population. Occlusion of the dominant eye is the primary and best treatment, although efficacy decreases after 6 years of age. As a result, levodopa/carbidopa has been explored as an adjunct to conventional therapy and has been shown to have an immediate impact on visual acuity. Several studies to date have shown mixed results on the benefit of supplementing occlusion therapy with levodopa/carbidopa, although they have primarily studied children. The authors describe the oldest patient (46 years old) documented in the literature to have shown improvement in visual acuity using levodopa/carbidopa. He was given a 16-week course at a dose in line with previous studies while being effectively occluded full time due to a glaucomatous right eye with no light perception. On 3-month follow-up, his left eye improved two lines and stabilized at 6 months.

Corneal Changes in Children after Unilateral Cataract Surgery in the Infant Aphakia Treatment Study.

PURPOSE: We report endothelial cell (EC) characteristics and central corneal thickness (CCT) from the Infant Aphakia Treatment Study (IATS) patients at the 5-year examination. DESIGN: Randomized, controlled trial of the treatment of unilateral cataract with aphakic contact lens (CL) versus primary intraocular lens (IOL) implant. PARTICIPANTS: A total of 114 infants with unilateral cataract. METHODS: 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. MAIN OUTCOME MEASURES: 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. RESULTS: 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). CONCLUSIONS: 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.

Canalicular laceration repair using a viscoelastic injection to locate and dilate the proximal torn edge.

BACKGROUND: Canalicular lacerations are common complications of eyelid trauma in the pediatric population. Irrigating air, water, and colored or viscous agents through the intact canaliculus have been suggested to identify the torn proximal edge. We report our experience in repairing canalicular lacerations using a novel viscoelastic injection technique with a Monoka monocanalicular stent. METHODS: The medical records of patients <18 years of age who underwent repair of a canalicular laceration with a monocanalicular stent using superficial viscoelastic deployment to locate the torn canaliculus were retrospectively reviewed. Demographics, cause of eyelid injury, surgical management using our novel viscoelastic injection technique, and outcome were analyzed. RESULTS: A total of 38 children with lid lacerations were identified, of whom the 17 with canalicular involvement were included (mean age, 6.27 years). Canalicular injury in these 17 was due to dog bite (9 patients) and shearing trauma (8 patients). In 11 patients, the injury was located in the lower lid; in 4, the upper lid; and in 2, combined upper and lower lids. All patients had good anatomic repair and on follow-up had negative dye disappearance tests and were free of tearing. CONCLUSIONS: Deploying viscoelastic superficially near, and injecting into the injured canaliculus can improve visualization of the operative field by retracting the surrounding tissue and tamponading any bleeding, which aids in location and dilation of the torn canaliculus initially and in subsequent steps, eases intubation into the lubricated torn canaliculus and nasolacrimal duct, and avoids iatrogenic injury to an uninjured canaliculus.