Appropriateness of Ophthalmology Recommendations From an Online Chat-Based Artificial Intelligence Model.

Objective: To determine the appropriateness of ophthalmology recommendations from an online chat-based artificial intelligence model to ophthalmology questions. Patients and Methods: Cross-sectional qualitative study from April 1, 2023, to April 30, 2023. A total of 192 questions were generated spanning all ophthalmic subspecialties. Each question was posed to a large language model (LLM) 3 times. The responses were graded by appropriate subspecialists as appropriate, inappropriate, or unreliable in 2 grading contexts. The first grading context was if the information was presented on a patient information site. The second was an LLM-generated draft response to patient queries sent by the electronic medical record (EMR). Appropriate was defined as accurate and specific enough to serve as a surrogate for physician-approved information. Main outcome measure was percentage of appropriate responses per subspecialty. Results: For patient information site-related questions, the LLM provided an overall average of 79% appropriate responses. Variable rates of average appropriateness were observed across ophthalmic subspecialties for patient information site information ranging from 56% to 100%: cataract or refractive (92%), cornea (56%), glaucoma (72%), neuro-ophthalmology (67%), oculoplastic or orbital surgery (80%), ocular oncology (100%), pediatrics (89%), vitreoretinal diseases (86%), and uveitis (65%). For draft responses to patient questions via EMR, the LLM provided an overall average of 74% appropriate responses and varied by subspecialty: cataract or refractive (85%), cornea (54%), glaucoma (77%), neuro-ophthalmology (63%), oculoplastic or orbital surgery (62%), ocular oncology (90%), pediatrics (94%), vitreoretinal diseases (88%), and uveitis (55%). Stratifying grades across health information categories (disease and condition, risk and prevention, surgery-related, and treatment and management) showed notable but insignificant variations, with disease and condition often rated highest (72% and 69%) for appropriateness and surgery-related (55% and 51%) lowest, in both contexts. Conclusion: This LLM reported mostly appropriate responses across multiple ophthalmology subspecialties in the context of both patient information sites and EMR-related responses to patient questions. Current LLM offerings require optimization and improvement before widespread clinical use.

Incidence of Glaucoma-Related Adverse Events in the First 5 Years After Pediatric Lensectomy.

Importance: Glaucoma can develop following cataract removal in children. Objective: To assess the cumulative incidence of glaucoma-related adverse events (defined as glaucoma or glaucoma suspect) and factors associated with risk of these adverse events in the first 5 years after lensectomy prior to 13 years of age. Design, Setting, and Participants: This cohort study used longitudinal registry data collected at enrollment and annually for 5 years from 45 institutional and 16 community sites. Participants were children aged 12 years or younger with at least 1 office visit after lensectomy from June 2012 to July 2015. Data were analyzed from February through December 2022. Exposures: Usual clinical care after lensectomy. Main Outcomes and Measures: The main outcomes were cumulative incidence of glaucoma-related adverse events and baseline factors associated with risk of these adverse events. Results: The study included 810 children (1049 eyes); 443 eyes of 321 children (55% female; mean [SD] age, 0.89 [1.97] years) were aphakic after lensectomy, and 606 eyes of 489 children (53% male; mean [SD] age, 5.65 [3.32] years) were pseudophakic. The 5-year cumulative incidence of glaucoma-related adverse events was 29% (95% CI, 25%-34%) in 443 eyes with aphakia and 7% (95% CI, 5%-9%) in 606 eyes with pseudophakia; 7% (95% CI, 5%-10%) of aphakic eyes and 3% (95% CI, 2%-5%) of pseudophakic eyes were diagnosed as glaucoma suspect. Among aphakic eyes, a higher risk for glaucoma-related adverse events was associated with 4 of 8 factors, including age less than 3 months (vs ≥3 months: adjusted hazard ratio [aHR], 2.88; 99% CI, 1.57-5.23), abnormal anterior segment (vs normal: aHR, 2.88; 99% CI, 1.56-5.30), intraoperative complications at time of lensectomy (vs none; aHR, 2.25; 99% CI, 1.04-4.87), and bilaterality (vs unilaterality: aHR, 1.88; 99% CI, 1.02-3.48). Neither of the 2 factors evaluated for pseudophakic eyes, laterality and anterior vitrectomy, were associated with risk of glaucoma-related adverse events. Conclusions and Relevance: In this cohort study, glaucoma-related adverse events were common after cataract surgery in children; age less than 3 months at surgery was associated with elevated risk of the adverse events in aphakic eyes. Children with pseudophakia, who were older at surgery, less frequently developed a glaucoma-related adverse event within 5 years of lensectomy. The findings suggest that ongoing monitoring for the development of glaucoma is needed after lensectomy at any age.

Long-term strabismus outcomes after unilateral infantile cataract surgery in the Infant Aphakia Treatment Study.

PURPOSE: To characterize long-term strabismus outcomes in children in the Infant Aphakia Treatment Study (IATS). METHODS: This study was a secondary data analysis of long-term ocular alignment characteristics of children aged 10.5 years who had previously been enrolled in a randomized clinical trial evaluating aphakic management after unilateral cataract surgery between 1 and 6 months of age. RESULTS: In the IATS study, 96 of 109 children (88%) developed strabismus through age 10.5 years. Half of the 20 children who were orthophoric at distance through age 5 years maintained orthophoria at distance fixation at 10.5 years. Esotropia was the most common type of strabismus prior to age 5 years (56/109 [51%]), whereas exotropia (49/109 [45%]) was the most common type of strabismus at 10.5 years (esotropia, 21%; isolated hypertropia, 17%). Strabismus surgery had been performed on 52 children (48%), with 18 of these (35%) achieving microtropia <10Δ. Strabismus was equally prevalent in children randomized to contact lens care compared with those randomized to primary intraocular lens implantation (45/54 [83%] vs 45/55 [82%]; P = 0.8). Median visual acuity in the study eye was 0.56 logMAR (20/72) for children with orthotropia or microtropia <10Δ versus 1.30 logMAR (20/400) for strabismus ≥10Δ (P = 0.0003). CONCLUSIONS: Strabismus-in particular, exotropia-is common irrespective of aphakia management 10 years following infant monocular cataract surgery. The delayed emergence of exotropia with longer follow-up indicates a need for caution in managing early esotropia in these children. Children with better visual acuity at 10 years of age are more likely to have better ocular alignment.

De novo PBX1 variant in a patient with glaucoma, kidney anomalies, and developmental delay: An expansion of the CAKUTHED phenotype.

An infant was referred for evaluation of congenital glaucoma and corneal clouding. In addition, he had a pelvic kidney, hypotonia, patent ductus arteriosus, abnormal pinnae, and developmental delay. Exome sequencing identified a previously unpublished de novo single nucleotide insertion in PBX1 c.400dupG (NM_002585.3), predicted to cause a frameshift resulting in a truncated protein with loss of function (p.Ala134Glyfs*65). Identification of this loss of function variant supports the diagnosis of congenital anomalies of the kidney and urinary tract syndrome with or without hearing loss, abnormal ears, or developmental delay (CAKUTHED). Here, we propose glaucoma as an extra-renal manifestation associated with PBX1-related disease due to the relationship of PBX1 with MEIS1, MEIS2, and FOXC1 transcription factors associated with eye development.

Ultra-low-dose (boom-boom) radiotherapy for management of recurrent ocular post-transplant lymphoproliferative disorder.

PURPOSE: To report a case of recurrent iris post-transplant lymphoproliferative disorder (PTLD) treated with ultra-low-dose (boom-boom) radiotherapy (RT). OBSERVATIONS: A 12-year-old Caucasian male with a history of bilateral, recurrent iris PTLD of the extranodal marginal zone lymphoma (MALT) type presented with persistent bilateral anterior chamber cellular infiltration, which was incompletely controlled on topical corticosteroids, and with elevated intraocular pressure (IOP) in the right eye secondary to steroid response. The patient was diagnosed with PTLD recurrence and was successfully treated with ultra-low-dose RT to both eyes in 2 fractions of 2 Gy. At 15 month follow-up the patient maintained complete disease control with normal IOP off all topical ophthalmic medications. CONCLUSIONS AND IMPORTANCE: Ultra-low-dose RT for ocular PTLD of the MALT subtype represents a novel therapeutic approach that may provide a durable treatment response and could be considered as either primary or adjuvant therapy for this rare condition.

Visual Outcomes and Complications After Lensectomy for Traumatic Cataract in Children.

IMPORTANCE: The Pediatric Eye Disease Investigator Group Cataract Registry provides a multicenter assessment of visual outcomes and complications after lensectomy for traumatic pediatric cataract. OBJECTIVE: To report visual acuity (VA) and the cumulative proportion with strabismus, glaucoma, and other ocular complications by 15 months after lensectomy for traumatic cataract among children younger than 13 years at the time of surgery. DESIGN, SETTING, AND PARTICIPANTS: From June 18, 2012, to July 8, 2015, 1266 eyes of 994 children from 33 pediatric eye care practices seen within 45 days after lensectomy were enrolled in a multicenter, prospective observational registry. Of these, 74 eyes of 72 participants undergoing lensectomy for traumatic cataract were included in a cohort study. Follow-up was completed by November 2, 2015, and data were analyzed from March 20, 2018, to July 7, 2020. EXPOSURES: Lensectomy after ocular trauma. MAIN OUTCOMES AND MEASURES: Best-corrected VA from 9 to 15 months after lensectomy for traumatic cataract (for those 3 years or older) and the cumulative proportion with strabismus, glaucoma, and other ocular complications by 15 months. RESULTS: Of 994 participants in the registry, 84 (8%) had traumatic cataract. The median age at lensectomy for 72 participants examined within 15 months after surgery was 7.3 (range, 0.1-12.6) years; 46 (64%) were boys. An intraocular lens was placed in 57 of 74 eyes (77%). In children 3 years or older at outcome, the median best-corrected VA was 20/250 (range, 20/20 to worse than 20/800) in 6 eyes with aphakia and 20/63 (range, 20/20 to 20/200) in 26 eyes with pseudophakia. Postoperative visual axis opacification was reported in 18 of 27 eyes with pseudophakia without primary posterior capsulotomy (15-month cumulative proportion, 77%; 95% CI, 58%-92%). The cumulative proportion with strabismus was 43% (95% CI, 31%-58%) in 64 participants with ocular alignment data; exotropia was present in 14 of 23 participants (61%). The cumulative proportion with glaucoma was 6% (95% CI, 2%-16%). CONCLUSIONS AND RELEVANCE: Trauma was not a common cause of pediatric cataract requiring surgery. For children with traumatic cataract, substantial ocular morbidity including permanent vision loss was found, and long-term eye and vision monitoring are needed for glaucoma, strabismus, and capsular opacification.

Anterior segment ischemia after laser for retinopathy of prematurity previously treated with antivascular endothelial growth factor.

PURPOSE: To assess the risk of vision-threatening anterior segment ischemia (ASI) among retinopathy of prematurity (ROP) patients treated with anti-VEGF followed by laser photocoagulation. METHODS: The medical records of all infants treated for threshold ROP with laser photocoagulation with and without prior anti-VEGF injections from January 1, 2002, through December 2018 at Mayo Clinic were retrospectively reviewed for the prevalence of vision-threatening ASI. RESULTS: A total of 241 eyes of 122 infants were included. Mean gestational age was 25.1 weeks (range, 22.9-28.7); mean birth weight was 687.6 g (range, 360-1310 g). Of the 54 eyes (27 patients) treated with anti-VEGF prior to laser, 4 developed ASI (including corneal edema, cataracts, and choroidal effusion) compared with 2 of the 187 eyes (95 patients) treated with laser therapy alone (P = 0.008). Infants receiving both anti-VEGF and laser had a younger gestational age at birth (24.5 vs 25.3 weeks; P < 0.001) and lower birth weight (591.4 g vs 715.0 g; P < 0.001) than those who received laser alone. In multivariate analysis, early gestational age at birth was associated with development of ASI (P = 0.03); the association with anti-VEGF treatment (P = 0.07) fell short of statistical significance. CONCLUSIONS: The prevalence of vision-threatening ASI was higher among infants treated with intravitreal anti-VEGF followed by laser compared to those treated with laser alone. Further investigation is warranted to confirm this finding and identify potential factors for decreasing the risk of ASI.

Outcomes of Bilateral Cataract Surgery in Infants 7 to 24 Months of Age Using the Toddler Aphakia and Pseudophakia Treatment Study Registry.

PURPOSE: To evaluate outcomes of bilateral cataract surgery in children aged 7 to 24 months and compare rates of adverse events (AEs) with other Toddler Aphakia and Pseudophakia Study (TAPS) registry outcomes. DESIGN: Retrospective clinical study at 10 Infant Aphakia Treatment Study (IATS) sites. Statistical analyses comparing this cohort with previously reported TAPS registry cohorts. PARTICIPANTS: Children enrolled in the TAPS registry between 2004 and 2010. METHODS: Children underwent bilateral cataract surgery with or without intraocular lens (IOL) placement at age 7 to 24 months with 5 years of postsurgical follow-up. MAIN OUTCOME MEASURES: Visual acuity (VA), occurrence of strabismus, AEs, and reoperations. RESULTS: A total of 40 children (76 eyes) who underwent bilateral cataract surgery with primary posterior capsulectomy were identified with a median age at cataract surgery of 11 months (7-23); 68% received a primary IOL. Recurrent visual axis opacification (VAO) occurred in 7.5% and was associated only with the use of an IOL (odds ratio, 6.10; P = 0.005). Glaucoma suspect (GS) was diagnosed in 2.5%, but no child developed glaucoma. In this bilateral cohort, AEs (8/40, 20%), including glaucoma or GS and VAO, and reoperations occurred in a similar proportion to that of the published unilateral TAPS cohort. When analyzed with children aged 1 to 7 months at bilateral surgery, the incidence of AEs and glaucoma or GS correlated strongly with age at surgery (P = 0.011/0.004) and glaucoma correlated with microcornea (P = 0.040) but not with IOL insertion (P = 0.15). CONCLUSIONS: Follow-up to age 5 years after bilateral cataract surgery in children aged 7 to 24 months reveals a low rate of VAO and very rare glaucoma or GS diagnosis compared with infants with cataracts operated at < 7 months of age despite primary IOL implantation in most children in the group aged 7 to 24 months. The use of an IOL increases the risk of VAO irrespective of age at surgery.

Outcomes of secondary intraocular lens implantation in the Infant Aphakia Treatment Study.

PURPOSE: To report outcomes of secondary intraocular lens (IOL) implantation in the Infant Aphakia Treatment Study (IATS). SETTING: Multicenter clinical practice. DESIGN: Secondary analysis of patients enrolled in a randomized clinical trial. METHODS: Details regarding all secondary IOL surgeries conducted in children enrolled in the IATS were compiled. Visual outcomes, refractive outcomes, and adverse events at the age of 10½ years were evaluated. Comparisons were made with eyes that remained aphakic and with eyes randomized to primary IOL placement. RESULTS: The study included 114 infants, 57 in the aphakic group and 57 in the primary IOL group; 55 of 57 patients randomized to aphakia with contact lens correction were seen for the 10½-year study visit; 24 (44%) of 55 eyes had secondary IOL surgery. Median age at IOL surgery was 5.4 years (range 1.7 to 10.3 years). Mean absolute prediction error was 1.00 ± 0.70 diopters (D). At age 10½ years, the median logarithm of the minimum angle of resolution visual acuity (VA) was 0.9 (range 0.2 to 1.7), similar to VA in the 31 eyes still aphakic (0.8, range 0.1 to 2.9); the number of eyes with stable or improved VA scores between the 4½-year and 10½-year study visits was also similar (78% secondary IOL eyes; 84% aphakic eyes). For eyes undergoing IOL implantation after the 4½-year study visit (n = 22), the mean refraction at age 10½ years was -3.20 ± 2.70 D (range -9.90 to 1.10 D), compared with -5.50 ± 6.60 D (n = 53, range -26.50 to 3.00 D) in eyes with primary IOL (P = .03). CONCLUSIONS: Delayed IOL implantation allows a more predictable refractive outcome at age 10½ years, although the range of refractive error is still large.

Impact of Standardized Prescribing Guidelines on Postoperative Opioid Prescriptions after Ophthalmic Surgery.

PURPOSE: To determine the effect of implementing standardized opioid prescribing guidelines on prescription patterns for acute pain after ophthalmic surgery in opioid-naïve patients. DESIGN: Quality improvement study. PARTICIPANTS: Ophthalmic surgeons in an academic department of ophthalmology. METHODS: Postoperative opioid prescribing patterns were compared before and after the implementation of standardized opioid prescribing guidelines for ophthalmic surgery at an academic institution. Only prescriptions provided to opioid-naïve patients 18 years of age or older were included. Surgeons reached a consensus for standardized prescribing guidelines appropriate for the type of surgery within their subspecialty. Guidelines were disseminated in conjunction with postsurgical pain management education to all ophthalmologists in the department, including trainees. The frequency of opioid prescriptions, the quantity of opioid prescribed (converted to oral morphine equivalent [OME]), and opioid prescription refill rates were compared before and after intervention. MAIN OUTCOME MEASURES: Prescriptions with more than 80 OME, frequency of opioid prescriptions, mean OME, and refill rates. RESULTS: Of 5349 ophthalmic surgeries performed during the 2 assessment periods, 196 (3.7%) were associated with opioid prescriptions for acute postoperative pain. The frequency of opioid prescriptions decreased to 3.0% (81/2736) after intervention compared with 4.4% (115/2613) before intervention (P = 0.005). When opioids were prescribed, the mean OME decreased from 93 (range, 27-500) before intervention to 42 (range, 14-100) after intervention (P = 0.003). The number of prescriptions for more than 80 OME decreased from 56 (2.1%) before intervention to 4 (0.1%) after intervention (P < 0.001). Based on the standardized guidelines, 103 of the 115 (89.6%) preintervention opioid prescriptions would not have adhered to the guidelines, whereas 39 of the 81 (48.1%) postintervention prescriptions did not adhere to the guidelines (P < 0.001). The proportion of refill prescriptions did not differ before and after intervention (P = 0.44). CONCLUSIONS: The process of discussing postsurgical pain management and developing standardized opioid prescribing guidelines reduces overprescribing of opioids after ophthalmic surgery without increasing refill rates. Continued education is required to improve adherence to the prescribing guidelines further.

Outcomes of Bilateral Cataracts Removed in Infants 1 to 7 Months of Age Using the Toddler Aphakia and Pseudophakia Treatment Study Registry.

PURPOSE: To evaluate outcomes of bilateral cataract surgery in infants 1 to 7 months of age performed by Infant Aphakia Treatment Study (IATS) investigators during IATS recruitment and to compare them with IATS unilateral outcomes. DESIGN: Retrospective case series review at 10 IATS sites. PARTICIPANTS: The Toddler Aphakia and Pseudophakia Study (TAPS) is a registry of children treated by surgeons who participated in the IATS. METHODS: Children underwent bilateral cataract surgery with or without intraocular lens (IOL) placement during IATS enrollment years 2004 through 2010. MAIN OUTCOME MEASURES: Visual acuity (VA), strabismus, adverse events (AEs), and reoperations. RESULTS: One hundred seventy-eight eyes (96 children) were identified with a median age of 2.5 months (range, 1-7 months) at the time of cataract surgery. Forty-two eyes (24%) received primary IOL implantation. Median VA of the better-seeing eye at final study visit closest to 5 years of age with optotype VA testing was 0.35 logarithm of the minimum angle of resolution (logMAR; optotype equivalent, 20/45; range, 0.00-1.18 logMAR) in both aphakic and pseudophakic children. Corrected VA was excellent (<20/40) in 29% of better-seeing eyes, 15% of worse-seeing eyes. One percent showed poor acuity (≥20/200) in the better-seeing eye, 12% in the worse-seeing eye. Younger age at surgery and smaller (<9.5 mm) corneal diameter at surgery conferred an increased risk for glaucoma or glaucoma suspect designation (younger age: odds ratio [OR], 1.44; P = 0.037; and smaller cornea: OR, 3.95; P = 0.045). Adverse events also were associated with these 2 variables on multivariate analysis (younger age: OR, 1.36; P = 0.023; and smaller cornea: OR, 4.78; P = 0.057). Visual axis opacification was more common in pseudophakic (32%) than aphakic (8%) eyes (P = 0.009). Unplanned intraocular reoperation occurred in 28% of first enrolled eyes (including glaucoma surgery in 10%). CONCLUSIONS: Visual acuity after bilateral cataract surgery in infants younger than 7 months is good, despite frequent systemic and ocular comorbidities. Although aphakia management did not affect VA outcome or AE incidence, IOL placement increased the risk of visual axis opacification. Adverse events and glaucoma correlated with a younger age at surgery and glaucoma correlated with the presence of microcornea.

Postoperative Nausea and Vomiting and Phase I Post-anesthesia Recovery After Strabismus Operations.

PURPOSE: To ascertain postoperative nausea and vomiting (PONV) rates in adult patients after strabismus operations and assess causes for prolonged post-anesthesia recovery. METHODS: This was a retrospective observational study of consecutive adult patients who underwent strabismus operations at one institution from January 1, 2010, to May 31, 2017. The anesthetic records were abstracted and PONV rates were ascertained. On the basis of the cohort's 75th percentile of anesthesia recovery duration, patients were categorized into goal recovery (lower three quartiles) and prolonged recovery (upper quartile). Multivariable logistic regression analyses were performed to assess associations between clinical characteristics and prolonged recovery. RESULTS: A total of 794 adult patients who underwent strabismus surgery were identified. PONV was present in 31 (3.9%) patients. The median (interquartile range) post-anesthesia recovery was 45 minutes (range: 33 to 63 minutes). Prolonged recovery was associated with long-term benzodiazepine use (odds ratio [OR]: 3.07; 95% CI [confidence interval]: 1.23 to 7.80; P = .02). Patients with prolonged recovery had higher rates of PONV (15 [7.2%] vs 16 [2.7%], P = .007), oversedation (107 [51.4%] vs 226 [38.6%], P = .001), and postoperative analgesic administration (138 [66.4%] vs 222 [37.9%], P < .001). Inverse associations were found between desflurane and oversedation (OR: 0.63; 95% CI: 0.45 to 0.86; P = .004) and between acetaminophen administration and postoperative analgesic administration (OR: 0.57; 95% CI: 0.38 to 0.86; P = .007). CONCLUSIONS: Adult patients undergoing general anesthesia for strabismus surgery had a low PONV rate. However, the presence of PONV was associated with delayed recovery room discharge. Other factors associated with prolonged Phase I recovery were long-term benzodiazepine use and longer operations, which likely resulted in an increased need for anesthetic agents and therefore more intense postoperative sedation. [J Pediatr Ophthalmol Strabismus. 2019;56(3):151-156.].

Visual Acuity and Ophthalmic Outcomes in the Year After Cataract Surgery Among Children Younger Than 13 Years.

Importance: Cataract is an important cause of visual impairment in children. Outcomes reported from a large clinical disease-specific registry can provide real-world estimates of visual outcomes and rates of adverse events in clinical practice. Objective: To describe visual acuity and refractive error outcomes, as well as rates of amblyopia, glaucoma, and additional eye surgery, during the first year after lensectomy in children. Design, Setting, and Participants: A prospective observational study was conducted from June 18, 2012, to July 8, 2015, at 61 pediatric eye care practices among 880 children younger than 13 years at the time of lensectomy in at least 1 eye with follow-up within 15 months after surgery. Statistical analysis was performed from December 12, 2016, to December 14, 2018. Exposures: Lensectomy with or without implantation of an intraocular lens. Main Outcomes and Measures: Visual acuity as well as rates of amblyopia, glaucoma, suspected glaucoma, and other intraocular surgery. Results: Among the 880 children (432 girls and 448 boys; mean [SD] age at annual follow-up, 4.9 [3.8] years) in the study, lens surgery was bilateral in 362 (41.1%; 95% CI, 37.9%-44.4%) children and unilateral in 518 (58.9%; 95% CI, 55.6%-62.1%). An intraocular lens was implanted in 654 of 1132 eyes (60.2%; 95% CI, 57.0%-63.4% [proportions reported for eye-level outcomes account for the potential correlation induced by enrolling both eyes of some individuals; for participants who received bilateral surgery, these numbers will differ from the quotient of the number of cases divided by the total sample size]). Amblyopia was identified in 449 children (51.0%; 95% CI, 47.7%-54.3%). In children age 3 years or older, mean visual acuity was 0.30 logMAR (about 20/40) in 153 bilateral pseudophakic eyes, 0.49 logMAR (about 20/63) in 141 unilateral pseudophakic eyes, 0.47 logMAR (about 20/63) in 21 bilateral aphakic eyes, and 0.61 logMAR (about 20/80) in 17 unilateral aphakic eyes. Mean visual acuity improved with older age at surgery in eyes with bilateral pseudophakia by 0.2 logMAR line (99% CI, 0.02-0.4; P = .005) and by 0.3 logMAR line (99% CI, 0.04-0.60; P = .004) in eyes with unilateral pseudophakia. A new diagnosis of glaucoma or suspected glaucoma was made in 67 of 1064 eyes that did not have glaucoma prior to lensectomy (5.9%; 95% CI, 4.6%-7.7%); 36 of 273 eyes with bilateral aphakia (13.2%; 95% CI, 9.0%-19.0%), 5 of 308 eyes with bilateral pseudophakia (1.5%; 95% CI, 0.6%-4.2%), 14 of 178 eyes with unilateral aphakia (7.9%; 95% CI, 4.7%-12.8%), and 12 of 305 eyes with unilateral pseudophakia (3.9%; 95% CI, 2.2%-6.8%). Additional intraocular surgery, most commonly vitrectomy or membranectomy to clear the visual axis, was performed in 189 of 1132 eyes (17.0%; 95% CI, 14.8%-19.6%). Conclusions and Relevance: Amblyopia was frequently observed during the first year after lensectomy in this cohort of children younger than 13 years. For children age 2 years or older at surgery visual acuity was typically less than normal for age and was worse with unilateral cataract. Management of visual axis obscuration was the most common complication requiring surgical intervention during the first year after surgery.

Outcomes of Unilateral Cataracts in Infants and Toddlers 7 to 24 Months of Age: Toddler Aphakia and Pseudophakia Study (TAPS).

PURPOSE: To evaluate outcomes of unilateral cataract surgery in children 7 to 24 months of age. DESIGN: Retrospective case series at 10 Infant Aphakia Treatment Study (IATS) sites. PARTICIPANTS: The Toddler Aphakia and Pseudophakia Study is a registry of children treated by surgeons who participated in the IATS. METHODS: Children underwent unilateral cataract surgery with or without intraocular lens (IOL) placement during the IATS enrollment years of 2004 and 2010. MAIN OUTCOME MEASURES: Intraoperative complications, adverse events (AEs), visual acuity, and strabismus. RESULTS: Fifty-six children were included with a mean postoperative follow-up of 47.6 months. Median age at cataract surgery was 13.9 months (range, 7.2-22.9). Ninety-two percent received a primary IOL. Intraoperative complications occurred in 4 patients (7%). At 5 years of age, visual acuity of treated eyes was very good (≥20/40) in 11% and poor (≤20/200) in 44%. Adverse events were identified in 24%, with a 4% incidence of glaucoma suspect. An additional unplanned intraocular surgery occurred in 14% of children. Neither AEs nor intraocular reoperations were more common for children with surgery at 7 to 12 months of age than for those who underwent surgery at 13 to 24 months of age (AE rate, 21% vs. 25% [P = 0.60]; reoperation rate, 13% vs. 16% [P = 1.00]). CONCLUSIONS: Although most children underwent IOL implantation concurrent with unilateral cataract removal, the incidence of complications, reoperations, and glaucoma was low when surgery was performed between 7 and 24 months of age and compared favorably with same-site IATS data for infants undergoing surgery before 7 months of age. Our study showed that IOL implantation is relatively safe in children older than 6 months and younger than 2 years.

Incidence and Ocular Features of Pediatric Myasthenias.

PURPOSE: To report the incidence, demographics, and ocular findings of children with myasthenia. DESIGN: Retrospective cohort study. METHODS: The medical records of all children (<19 years) examined at Mayo Clinic with any form of myasthenia from January 1 1966, through December 31, 2015, were retrospectively reviewed. RESULTS: A total of 364 children were evaluated during the study period, of which 6 children were residents of the Olmsted County at the time of their diagnosis, yielding an annual age- and sex-adjusted incidence of 0.35 per 100 000 <19 years, or 1 in 285 714 <19 years. The incidence of juvenile myasthenia gravis (JMG) and congenital myasthenic syndrome (CMS) was 0.12 and 0.23 per 100 000, respectively. Of the 364 study children, 217 (59.6%) had JMG, 141 (38.7%) had CMS, and 6 (1.7%) had Lambert-Eaton syndrome, diagnosed at a median age of 13.5, 5.1, and 12.6 years, respectively. A majority of the JMG and CMS patients had ocular involvement (90.3% and 85.1%, respectively), including ptosis and ocular movement deficits. Among children with at least 1 year of follow-up (JMG; median, 7.1 years, CMS; median, 7.0 years), improvement was seen in 88.8% of JMG patients (complete remission in 31.3%) and in 58.3% of CMS patients. CONCLUSION: Although relatively rare, myasthenia gravis in children has 2 predominant forms, CMS and JMG, both of which commonly have ocular involvement. Improvement is more likely in children with the juvenile form.

Combined intraocular and systemic rituximab for ocular lymphoproliferative disorder with extranodal marginal zone lymphoma-type morphology after heart transplant.

Post-transplant lymphoproliferative disorder (PTLD) has rarely been associated with ocular manifestations. We report a case of bilateral ocular involvement by PTLD with histopathologic features of extranodal marginal zone (MALT) lymphoma in an 8-year-old boy following orthotopic heart transplantation. The anterior segment disease was treated successfully using a combination of intraocular and systemic injections of humanized anti-CD20 antibody (rituximab).

Population-based Incidence of Intraocular Lens Exchange in Olmsted County, Minnesota.

PURPOSE: To determine the population-based incidence of pseudophakic intraocular lens exchange in Olmsted County, Minnesota. DESIGN: Retrospective review of a population-based cohort. METHODS: Patients undergoing pseudophakic intraocular lens exchange in Olmsted County, Minnesota, between January 1, 1986 and December 31, 2016 were identified from the Rochester Epidemiology Project medical record linkage system. Indications and outcomes were determined, and the incidence rate was calculated as cases per 1 000 000 person-years. Poisson regression analysis was used to assess changes in incidence over time, and the cumulative probability of needing a lens exchange was estimated by Kaplan-Meier analysis. RESULTS: Eighty cases of intraocular lens exchange were identified, yielding an overall age- and sex-adjusted incidence rate of 28.4 per million (confidence interval [CI], 22.1-34.7), which increased over the study period (P = .04). The 30-year cumulative probability of intraocular lens exchange among patients undergoing cataract surgery was 1.5% (CI, 0.6%-2.4%), increasing at a relatively constant rate. Dislocated lenses accounted for 72.5% of lens exchanges. Unplanned refractive outcome of primary cataract surgery and uveitis-glaucoma-hyphema syndrome from squared-edged haptics emerged as newer indications for intraocular lens exchange. CONCLUSIONS: The population-based incidence of pseudophakic intraocular lens exchange has increased over the last 30 years, and can be explained by the increase in incidence rate of cataract surgery over the same period. Surgeons should be aware of emerging indications of intraocular lens exchange, which reflect changes in lens design and increasing expectations of refractive outcomes.

One- Versus Two-Muscle Surgery for Presumed Unilateral Fourth Nerve Palsy Associated With Moderate Angle Hyperdeviations.

PURPOSE: To compare 1-muscle vs 2-muscle surgery for moderate-angle hyperdeviations owing to presumed unilateral fourth nerve palsy. DESIGN: Retrospective chart review. METHODS: Seventy-three patients (aged 5-86 years) underwent either 1- or 2-muscle surgery at our institution for moderate hyperdeviation owing to presumed unilateral fourth nerve palsy, measuring 14-25 prism diopters (PD) in straight-ahead gaze at distance fixation. Six-week and 1-year motor success was defined as zero vertical deviation or 1-4 PD undercorrection at distance, overcorrection as any reversal of hypertropia, and undercorrection as >4 PD. Diplopia success was defined as no diplopia, or only rarely for distance straight ahead and reading. RESULTS: Twenty-eight patients underwent 1-muscle surgery, and 45 patients underwent 2-muscle surgery. Motor success was similar (64% vs 67%, P > .99 at 6 weeks; 47% vs 55%, P = .8 at 1 year, n = 46), but there were more undercorrections at 6 weeks with 1-muscle surgery (36% vs 16%, P = .09) and more overcorrections at 6 weeks with 2-muscle surgery (0% vs 18%, P = .02). Diplopia success was also somewhat similar between 1- and 2-muscle surgery at 6 weeks (73% vs 60%, P = .5) and 1 year (45% vs 59%, P = .5). CONCLUSION: For moderate-angle hyperdeviations owing to presumed unilateral fourth nerve palsy, there appears no clear advantage of 2-muscle surgery for motor outcomes. Diplopia success was similar between 1- and 2-muscle surgery, owing to a greater number of less symptomatic undercorrections with 1-muscle surgery and a smaller number of more symptomatic overcorrections with 2-muscle surgery.

Strabismus surgery outcomes in the Infant Aphakia Treatment Study (IATS) at age 5 years.

PURPOSE: To report strabismus surgery frequency and outcomes after monocular infantile cataract surgery with or without IOL implantation. METHODS: 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. RESULTS: 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 8Δ 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]). CONCLUSIONS: 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.

A novel explanation of corneal clouding in a bone marrow transplant-treated patient with Hurler syndrome.

One common complication of mucopolysaccharidosis I-Hurler (MPS1-H) is corneal clouding, which occurs despite current treatments, including bone marrow transplantation. Human corneas were obtained from a 14 year old subject with MPS1-H and visual disability from progressive corneal clouding despite a prior bone marrow transplant at age 2. This was compared to a cornea from a 17 year old donated to our eye bank after his accidental death. The corneas were analyzed microscopically after staining with Alcian blue, antibodies to collagen I, IV, VI, and α-smooth muscle actin. Differences in levels of expression of the indicated molecules were assessed. Corneas from Hurler and control mice were examined similarly to determine potential mechanistic overlap. The MPS1-H subject cornea showed elevations in mucopolysaccharide deposition. The MPS1-H and Hurler mice corneas showed increased and disorganized expression of collagen I and IV relative to the control corneas. The MPS1-H corneas also showed increased and disordered expression of collagen VI. Positive expression of α-smooth muscle actin indicated myofibroblast conversion within the MPS1-H cornea in both the patient and mutant mouse material compared to normal human and control mouse cornea. Increased deposition of collagens and smooth muscle actin correlate with corneal clouding, providing a potential mechanism for corneal clouding despite bone marrow transplantation in MPS1-H patients. It might be possible to prevent or slow the onset of corneal clouding by treating the cornea with drugs known to prevent myofibroblast conversion.