PURPOSE To report on the biometric findings of adults and children with Marfan syndrome (MFS) recruited from 2 annual National Marfan Foundation conferences (2012 and 2015). DESIGN Cross-sectional study. METHODS Subjects diagnosed with MFS by Ghent 2 nosology were included for analysis. Subjects were divided into “adults” (≥16 years of age) and “children” (5–15 years of age). Biometric data included values for refractive error, axial length (AL), corneal curvature, anterior chamber depth, lens thickness, and central corneal thickness. RESULTS Of the 117 subjects evaluated, 74 (35 adults, 32 children, and 7 children <5 years of age) had a definite diagnosis of MFS and were included in the study. The AL was longer (25.25 ± 0.32 mm vs 24.24 ± 0.33 mm, P [ .03) and the lens was thicker (3.94 ± 0.09 mm vs 3.62 ± 0.10 mm, P [ .03) in adults. Both groups had flat corneas (average keratometry [Kmed] of 41.59 ± 0.35 diopters [D] in adults vs 40.89 ± 0.36 D in children, P [ .17). A negative correlation was found between AL and Kmed (L0.33, P < .001). The corneas of patients with MFS with ectopia lentis (EL) were significantly flatter and with higher degree of corneal astigmatism compared to patients without EL (Kmed of 40.68 ± 0.31 D vs 41.75 ± 0.28 D, P < .01 and corneal astigmatism of 1.68 ± 0.16 D vs 1.13 ± 0.14 D, P =.01). CONCLUSIONS Children with established MFS have flat corneas at least to the same degree as adults. Corneas of patients with MFS with EL are flatter and have a higher degree of corneal astigmatism. We strongly suggest that corneal parameters should be measured if MFS is suspected, especially in children that may not yet have developed EL.
Orbital dermoid cysts are benign congenital choristomas. They are common in pediatric population, developing adjacent to suture lines, most commonly located in antero-lateral fronto-zygomatic suture, and are slowly progressive. Complete surgical excision without rupture of cyst is the standard of care. Deep orbital cysts cause proptosis, require imaging, and may present a surgical challenge with a difficult approach. Rupture of the cyst leads to severe inflammatory reaction in surrounding tissues. Overall prognosis remains good with isolated reports of malignancy masquerading as dermoid cysts.
<h4>PURPOSE</h4> <p>To evaluate the clinical characteristics and surgical outcomes of medial rectus (MR) recessions in patients with Graves’ ophthalmopathy.</p> <h4>PATIENTS AND METHODS</h4> <p>The clinical records of 32 patients with Graves’ ophthalmopathy who underwent MR recessions with adjustable sutures for restrictive esotropia were reviewed. The clinical characteristics of patients, the size of the esodeviations, the limitations of ductions, the surgical doses, and observed responses to surgery were recorded and analyzed. Main outcome measures included the ratio of predicted to observed correction for MR recessions, improvement in ductions, and restoration of binocular status.</p> <h4>RESULTS</h4> <p>The mean age of the 32 patients (20 women, 12 men) at surgery was 54.1 ± 11.4 years. The mean duration of thyroid eye disease was 4.3 ± 5.4 years (range, 1 to 24 years). The ratios of predicted to observed correction for esodeviations at distance and near, respectively, were 2.21 ± 1.24 and 2.16 ± 1.81 at the time of adjustment and 1.61 ± 0.37 and 1.84 ± 0.90 at final follow-up. The limitation of abduction improved from -2.3 ± 1.3 to -0.75 ± 0.98. Binocular single vision was achieved in 73% of patients, and a further 10% of patients were able to fuse with prisms. A history of decompression was present in 75% of cases. Patients with a history of decompression had more restriction in abduction (-2.49 vs -1.78, <i>P</i> = .061), more frequently required bilateral surgery (75% vs 62.5%), and had a higher ratio of predicted to observed correction (1.71 ± 0.37 vs 1.37 ± 0.28, <i>P</i> = .043).</p> <h4>CONCLUSIONS</h4> <p>Patients with Graves’ ophthalmopathy who undergo MR recession for restrictive esotropia are prone to undercorrection. A history of decompression is associated with a less favorable clinical outcome. Augmented surgery, adjustable sutures, or both are recommended for improved surgical outcomes.</p> <p><cite>J Pediatr Ophthalmol Strabismus</cite> 2007;44:93-100.</p> <h4>AUTHORS</h4> <p>The authors were from the Massachusetts Eye & Ear Infirmary, Boston, Massachusetts. Dr. Mocan is currently from Hacettepe University, Ankara, Turkey. Dr. Ament is currently from Boston University School of Medicine, Boston, Massachusetts. Dr. Azar is currently from the University of Illinois at Chicago, Chicago, Illinois.</p> <p>Originally submitted October 8, 2005.</p> <p>Accepted for publication December 22, 2005.</p> <p>Address correspondence to Nathalie Azar, MD, Massachusetts Eye & Ear Infirmary, Pediatric Ophthalmology & Strabismus Service, Floor 1, 243 Charles Street, Boston, MA 02114.</p> <p>The authors have no industry relationships to disclose.</p> <p>The audience is advised that this continuing medical education activity may contain references to unlabeled uses of FDA-approved products or to products not approved by the FDA for use in the United States. The faculty members have been made aware of their obligation to disclose such usage.</p> <p>The material presented at or in any Vindico Medical Education continuing medical education activity does not necessarily reflect the views and opinions of Vindico Medical Education or SLACK Incorporated. Neither Vindico Medical Education or SLACK Incorporated, nor the faculty endorse or recommend any techniques, commercial products, or manufacturers. The faculty/author may discuss the use of materials and/or products that have not yet been approved by the U.S. Food and Drug Administration. All readers and continuing education participants should verify all information before treating patients or utilizing any product.</p>
IMPORTANCECataract is an important cause of visual impairment in children. Data from a large pediatric cataract surgery registry can provide real-world estimates of visual outcomes and the 5-year cumulative incidence of adverse events. OBJECTIVE To assess visual acuity (VA), incidence of complications and additional eye operations, and refractive error outcomes 5 years after pediatric lensectomy among children younger than 13 years. DESIGN, SETTING, AND PARTICIPANTSThis prospective cohort study used data from the Pediatric Eye Disease Investigator Group clinical research registry. From June 2012 to July 2015, 61 eye care practices in the US, Canada, and the UK enrolled children from birth to less than 13 years of age who had undergone lensectomy for any reason during the preceding 45 days. Data were collected from medical record reviews annually thereafter for 5 years until September 28, 2020. EXPOSURES Lensectomy with or without implantation of an intraocular lens (IOL).MAIN OUTCOMES AND MEASURES Best-corrected VA and refractive error were measured from 4 to 6 years after the initial lensectomy. Cox proportional hazards regression was used to assess the 5-year incidence of glaucoma or glaucoma suspect and additional eye operations. Factors were evaluated separately for unilateral and bilateral aphakia and pseudophakia.RESULTS A total of 994 children (1268 eyes) undergoing bilateral or unilateral lensectomy were included (504 [51%] male; median age, 3.6 years; range, 2 weeks to 12.9 years). Five years after the initial lensectomy, the median VA among 701 eyes with available VA data (55%) was 20/63 (range, 20/40 to 20/100) in 182 of 316 bilateral aphakic eyes (58%), 20/32 (range, 20/25 to 20/50) in 209 of 386 bilateral pseudophakic eyes (54%), 20/200 (range, 20/50 to 20/618) in 124 of 202 unilateral aphakic eyes (61%), and 20/65 (range, 20/32 to 20/230) in 186 of 364 unilateral pseudophakic eyes (51%). The 5-year cumulative incidence of glaucoma or glaucoma suspect was 46% (95% CI, 28%-59%) in participants with bilateral aphakia, 7% (95% CI, 1%-12%) in those with bilateral pseudophakia, 25% (95% CI, 15%-34%) in those with unilateral aphakia, and 17% (95% CI, 5%-28%) in those with unilateral pseudophakia. The most common additional eye surgery was clearing the visual axis, with a 5-year cumulative incidence of 13% (95% CI, 8%-17%) in participants with bilateral aphakia, 33% (95% CI, 26%-39%) in those with bilateral pseudophakia, 11% (95% CI, 6%-15%) in those with unilateral aphakia, and 34% (95% CI, 28%-39%) in those with unilateral pseudophakia. The median 5-year change in spherical equivalent refractive error was −8.38 D (IQR, −11.38 D to −2.75 D) among 89 bilateral aphakic eyes, −1.63 D (IQR, −3.13 D to −0.25 D) among 130 bilateral pseudophakic eyes, −10.75 D (IQR, −20.50 D to −4.50 D) among 43 unilateral aphakic eyes, and −1.94 D (IQR, −3.25 D to −0.69 D) among 112 unilateral pseudophakic eyes. CONCLUSIONS AND RELEVANCEIn this cohort study, development of glaucoma or glaucoma suspect was common in children 5 ye...
To develop a reproducible ex vivo model of corneal endothelial cell injury using phacoemulsification in porcine eyes and evaluate the effects of mesenchymal stromal cell secretome in this injury model.
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