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Background: We present comparative data of children with Fanconi anemia undergoing haploidentical hematopoietic stem cell transplantation (HSCT) with or without the addition of rabbit anti-thymocyte globulin (r-ATG) to the conditioning regimen. Patients and methods: This retrospective study included children with Fanconi anemia aged up to 18 years who underwent haploidentical HSCT between January 2015 and December 2022. The children were included in two cohorts in this study. Cohort 1 included children who received conditioning with fludarabine/cyclophosphamide/single fraction of 2 Gy TBI. The children in cohort 2 received the same conditioning along with r-ATG. Post-transplant cyclophosphamide was administered at a dose of 25 mg/kg on day3 and day4 in both cohorts. Results: A total of 35 children were included in the study, 25 in cohort 1 and 10 in cohort 2. Neutrophil engraftment was documented around day 14-16 post infusion in 21 children (84%) in cohort 1 and in 8 children (80%) in cohort 2. There was a significant difference in the incidence of the severity of graft versus host disease (GVHD) between the two cohorts (p = 0.003). In cohort 1, acute GVHD was documented in 17 children (68%), with grade 1/2 skin GVHD in 10 children, and grade 3/4 skin and gut GVHD in 7 children. Grade 4 gut GVHD was the cause of death in three children in cohort 1. In cohort 2, acute GVHD was documented in one child (10%) who had grade 4 skin and gut GVHD and succumbed to the above. Chronic GVHD was noted in nine (36%) children in cohort 1, and in one child (10%) in cohort 2. Cytomegalovirus reactivation was documented in 11 children (44%) in cohort 1 and three children (30%) in cohort 2. Overall survival was found to be 16/25 (64%) in cohort 1, with a median follow-up of 49 months, and 7/10 (70%) in cohort 2, with a median follow-up of 12 months. Conclusion: Serotherapy with r-ATG significantly reduced the incidence of GVHD from 68% to 10% in children with Fanconi anemia, with an increase in overall survival from 64% to 70%, although it did not affect graft failure. Further studies should focus on decreasing graft failure rates with early HSCT before multiple transfusions.

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OBJECTIVE: The present study aims to provide outcome data in children with relapsed acute lymphoblastic leukemia (ALL)over two decades and variables that impact survival. METHODS: The retrospective study included children who were diagnosed with ALL and treated at our center and relapsed between March 2002 and March 2021. RESULTS: A total of 100 children (64 boys, 36 girls) were included; 80 had B-ALL, 20 had T-ALL. 50 children had a very early relapse, while 25 each had an early and late relapse. The site of relapse was bone marrow in 57, isolated central nervous system (CNS) in 10, isolated testicular in 1, and combined bone marrow and CNS relapse in 32 children. Thirty-six families opted for the best supportive care; 23 of these had very early relapse. Among the 35 who were in remission following induction chemotherapy, 32 (91%) underwent hematopoietic stem cell transplantation (HSCT); 17/32 (53%) were alive and disease-free. Overall survival (OS) was 19 (19%) with a median follow-up of 23.5 months with a significantly improved survival post-measurable risk of disease (MRD) based risk stratification (4% vs 35%, P = 0.02). The OS with very early, early, and late relapses were 8%, 28%, and 32% (P = 0.018), and 15%, 12.5%, and 50% with bone marrow, combined and isolated CNS relapses (P = 0.008). CONCLUSION: Relapsed ALL remains a challenge, with OS of 19% and 53% among those who underwent HSCT. Abandonment after relapse continues to be prevalent, and we need to integrate social support for providing care and optimal treatment.

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BACKGROUND: Knowledge about refractive stabilization and the accuracy of postoperative refractive error measurements are crucial for improved patient outcomes after phacoemulsification. Existing guidelines typically recommend waiting 4-6 wk before prescribing corrective lenses. Our research focused on identifying factors that influence refractive errors in the early stages of post-cataract surgery, thus contributing to the existing literature on this topic. AIM: To investigate the time required for refraction stability after uneventful phacoemulsification surgery. METHODS: We compared the variation and statistical significance of the difference in spherical, cylindrical components, and the spherical equivalent between the 1- and 6-wk follow-up period in a group of 257 eyes that underwent uneventful phacoemulsification with foldable intraocular lens implantation, all performed by a single experienced surgeon. The Wilcoxon-Signed Rank Test was utilized to assess the magnitude of the change and determine its statistical significance. The refractive stability was defined as the point at which the change in spherical equivalent was within ± 0.5 dioptres for two consecutive visits. RESULTS: The average age of the patients was 64.9 ± 8.9 yr. The differences observed in both the visits in spherical power (0.1 ± 0.2), cylinder power (0.3 ± 0.4), and spherical equivalent (0.2 ± 0.2) were minimal and not statistically significant. The majority of eyes (93.4%) achieved refractive stability within 6 wk after the surgery. The cylindrical power differed between age groups at the 6th wk post-operative and the difference was statistically significant (P value 0.013). There were no significant differences in refractive stability when considering sex and axial length. CONCLUSION: Phacoemulsification with foldable intraocular lens implantation results in no significant changes in refraction for the majority of cases during the 6-wk follow-up period. Therefore, a spectacle prescription can be given at the completion of 1 wk.

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PURPOSE: To examine the difference in post-operative visual outcomes of cataract surgeries between a tertiary and secondary centre at Aravind Eye Hospitals in Tamil Nadu, India. METHODS: Our retrospective cross-sectional study analysed cataract surgeries at a secondary centre and its associated tertiary centre in 2021. Our main outcome measures were postoperative best corrected visual acuity (BCVA), spherical equivalent, and intraoperative and postoperative complications. Two-sample proportion tests and logistic regression analyses were performed. RESULTS: The analysis of 32,302 cataract surgeries in 2021 of which 4357 were performed at the secondary centre and 27,945 were performed at the tertiary centre showed that the tertiary centre operated on more advanced cataract condition (p < 0.001). Intraoperative (p < 0.001) and post-operative complication rates (p < 0.001) were higher in the tertiary centre. The odds of effective outcomes (BCVA > = 6/12) controlling for all covariates are poorer (p < 0.0001) in the tertiary centre for both phacoemulsification (phaco) and manual small incision cataract surgeries (MSICS). CONCLUSION: World Health Organization recommendations for the effective outcome of cataract surgery are met by both the tertiary and secondary centres, but the odds of effective outcomes in the tertiary centre was lower after adjusting for all known factors. Further investigations of the causes of poor vision in both phaco and MSICS in the tertiary centre are needed to improve the situation.

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PURPOSE: To examine the visual outcomes and risk factors of uveitis cataract eyes after phacoemulsification and manual small-incision cataract surgery (MSICS). SETTING: Tertiary-care eye hospital in southern India. DESIGN: Retrospective interventional case series. METHODS: Of the uveitis eyes operated for cataract surgery between 2017 and 2020, eyes with a minimum of 6 months postoperative follow-up were included. Eyes with ocular trauma, lens subluxation, lens-induced uveitis, or retinal detachment-induced uveitis were excluded. Demography, visual acuity, intraoperative and postoperative records, and surgical outcomes were analyzed. RESULTS: 191 eyes of 191 patients with a mean age of 51.7 ± 14.4 years were included. Phacoemulsification was performed in 134 eyes, and 57 eyes underwent MSICS. Synechiolysis and pupil-expanding maneuvers were required in 74 eyes (38.7%). No differences were noted in the rates of complications between phacoemulsification and MSICS eyes, except at 1 year, where higher rates of posterior capsular opacification and vitritis were noted in MSICS eyes ( P = .018). The visual outcomes of eyes that underwent MSICS and phacoemulsification were comparable ( P = .463). In 12 eyes (13.5%), improvement in vision was not significant. CONCLUSIONS: This study shows phacoemulsification may be a preferred technique in uveitis cataracts, given the lesser incidence of postoperative complications. Patients should be counseled for realistic expectations.

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Purpose: To compare the slit-lamp method and wavefront aberrometry method based on outcomes of toric realignment surgeries. Settings: Tertiary care ophthalmic hospital. Design: Retrospective study. Methods: This study included all eyes undergoing toric intraocular lens (TIOL) realignment surgery between January 2019 and December 2021 for which TIOL axis assessment by slit-lamp method and wavefront aberrometry method was available. Data were retrieved from electronic medical records, and we documented demographics, uncorrected visual acuity (UCVA), subjective refraction, and TIOL axis by slit-lamp and wavefront aberrometry methods on postoperative day 1 and day 14. In patients with misalignment, TIOL was realigned to the original position in group 1 (27 patients) and to an axis based on calculations provided by wavefront aberrometer in group 2 (25 patients). Post-realignment surgery, UCVA, subjective refraction, and TIOL axis by slit-lamp and wavefront aberrometry methods were assessed and analyzed. Results: We analyzed 52 eyes and found that the mean preoperative misalignment with the slit-lamp method (44.9° ±20.0°) and wavefront aberrometry (47.1° ±19.5°) was similar. The corresponding degrees of misalignment post-TIOL repositioning surgeries were 5.2° ±5.2° (slit-lamp method) and 4.7° ±5.1° (wavefront aberrometry) (P = 0.615). Both groups showed significant improvement in median log of minimum angle of resolution (logMAR) UCVA and reduction in median refractive cylinder. Conclusions: Slit-lamp method is as good as wavefront aberrometer method to assess TIOL axis. Toric realignment surgery is found to be safe, and realigning TIOL based on either slit-lamp method or wavefront aberrometer method equally improved UCVA and decreased residual refractive cylinder.

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Purpose: To assess the accuracy of e-Paarvai, an artificial intelligence-based smartphone application (app) that detects and grades cataracts using images taken with a smartphone by comparing with slit lamp-based diagnoses by trained ophthalmologists. Methods: In this prospective diagnostic study conducted between January and April 2022 at a large tertiary-care eye hospital in South India, two screeners were trained to use the app. Patients aged >40 years and with a best-corrected visual acuity <20/40 were recruited for the study. The app is intended to determine whether the eye has immature cataract, mature cataract, posterior chamber intra-ocular lens, or no cataract. The diagnosis of the app was compared with that of trained ophthalmologists based on slit-lamp examinations, the gold standard, and a receiver operating characteristic (ROC) curve was estimated. The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were computed. Results: The two screeners used the app to screen 2,619 eyes of 1,407 patients. In detecting cataracts, the app showed high sensitivity (96%) but low specificity (25%), an overall accuracy of 88%, a PPV of 92.3%, and an NPV of 57.8%. In terms of cataract grading, the accuracy of the app was high in detecting immature cataracts (1,875 eyes, 94.2%), but its accuracy was poor in detecting mature cataracts (73 eyes, 22%), posterior chamber intra-ocular lenses (55 eyes, 29.3%), and clear lenses (2 eyes, 2%). We found that the area under the curve in predicting ophthalmologists' cataract diagnosis could potentially be improved beyond the app's diagnosis based on using images only by incorporating information about patient sex and age (P < 0.0001) and best-corrected visual acuity (P < 0.0001). Conclusions: Although there is room for improvement, e-Paarvai app is a promising approach for diagnosing cataracts in difficult-to-reach populations. Integrating this with existing outreach programs can enhance the case detection rate.

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PURPOSE: To report the outcomes of cataract surgery in patients with Human Immunodeficiency Virus (HIV) infection. Setting Tertiary care ophthalmic hospital DESIGN: Retrospective study METHODS: This study included all eyes of patients with known HIV infection undergoing cataract surgery with a minimum follow-up of 6 months between January 2017 and December 2020. Patients who underwent combined surgeries and pediatric patients were excluded from analysis. Data were retrieved from electronic medical records and we documented demographics, history, detailed anterior and posterior segment examination, pre-operative grade and type of cataract, type of surgery done, its complication and post-operative course. All these parameters were recorded at the baseline visit and at 1 month and 6 months postoperatively. RESULTS: One hundred and twenty nine eyes of 107 HIV infected patients that underwent cataract surgery were evaluated. Mature cataract was seen in 31% of the eyes. Features of HIV related uveitis/retinitis were seen in 21 (16.2%) eyes. Phacoemulsification was performed in 44 (34.1%) eyes while manual small incision cataract surgery (MSICS) was done in 85 (65.9%) eyes. Intra-operative complications were encountered in 4 (3.1%) eyes. At the final follow-up, there was a significant improvement in median corrected distance visual acuity (CDVA) from LogMAR 1.08 (5/60) at baseline to LogMAR 0 (6/6) at 6 months follow-up. CONCLUSION: Patients with HIV infection usually present early and with advanced cataracts. Visual outcomes after cataract surgery are generally good but affected by presence of prior HIV related uveitis or retinitis.

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Purpose: The purpose of this study was to compare and analyze the endothelial cell loss during manual small-incision cataract surgery (MSICS) using the viscoelastic-assisted nucleus removal versus basal salt solution plus technique. Methods: This was a prospective randomized trial of 204 patients who underwent MSICS using viscoelastic-assisted nucleus removal (Group 1- OVD) versus basal salt solution plus technique (Group 2- BSS) at a tertiary eye care hospital in North India from January 2018 to 2021. Of these 204 patients, 103 (50.5%) and 101 (49.5%) were allocated to Group 1 and 2, respectively. The parameters assessed were detailed history, demographics, and anterior and posterior segment details. Visual acuity, intraocular pressure (IOP), keratometry, pachymetry, and endothelial cell density were evaluated preoperatively and postoperatively on day 1 and 30. Results: The mean age of the patients was 64.5 ± 8.2 years (range 48-82 years). There were 129 (63.2%) males and 75 (36.8%) females. The mean LogMAR visual acuity for both groups on day 1 (Group 1- 0.3 ± 0.1, Group 2- 0.5 ± 0.2) and day 30 (Group 1- 0.1 ± 0.2, Group 2- 0.1 ± 0.1) was statistically significant (P < 0.001), and the mean IOP value showed a statistically significant value (P < 0.009) on day 1 in Group 2 (15.0 ± 2.4 mmHg) and on day 30 (P < 0.001) in both the groups (Group 1- 13.6 ± 1.8 mmHg, Group 2- 13.5 ± 2 mmHg). The horizontal and vertical k values also showed a statistically significant difference on day 1 and day 30 (P < 0.001). The mean percentage change of central corneal thickness (CCT) in Group 1 was 17.7% and in Group 2 was 17.4% on day 1, and it was 1.1% on day 30 in both the groups, which was statistically significant (P < 0.001) compared to preoperative values. The percentage change in endothelial cell density on day 1 was 9% in Group 1 and 4.6% in Group 2, which was statistically significant (P < 0.001). On day 30, it was 9.7% and 4.8%, respectively, which was statistically significant (P < 0.001). Conclusion: Our study highlights statistically significant endothelial cell loss with viscoelastic-assisted nuclear delivery compared to BSS-assisted nuclear delivery during MSICS in a short follow-up of 1 month. The CCT values showed a slight increase, and the keratometry and IOP were unaffected compared to the preoperative parameters in both the groups.

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PURPOSE: To study the risk factors, clinical features, management, and outcomes of intraoperative expulsive choroidal hemorrhage (ECH) during cataract surgery. SETTING: Aravind Eye Hospital, Madurai, Tamil Nadu, India. DESIGN: Retrospective hospital-based study. METHODS: Of the 1 167 250 patients who underwent cataract surgery between 2008 and 2020, patients diagnosed with intraoperative ECH were included. Demography, ocular and systemic risk factors, visual acuity, type of ocular anesthesia, intraoperative and postoperative records, management, and surgical outcomes were analyzed. RESULTS: 52 eyes (0.004%) of 1 167 250 patients had ECH. Of the 52 cases, 43 cases (incidence rate 0.006%) were reported in the years 2008 to 2015 and 9 cases (incidence rate 0.002%) in the years 2016 to 2020. The change in the ocular anesthesia from peribulbar and retrobulbar anesthesia (2008 to 2015) to sub-Tenon anesthesia (2016 to 2020) was associated with a reduced rate of ECH ( P = .002). 28 eyes (53.8%) experienced limited ECH and 24 eyes (46.2%) full-blown ECH. The visual outcome was better in eyes with limited ECH compared with full-blown suprachoroidal hemorrhage in all follow-up visits. The median vision (interquartile range) before the cataract surgery and at postoperative day 1 were 1.30 (0.78 to 2.60) and 2.45 (1 to 2.75), respectively. The median final vision (interquartile range) after the secondary surgical intervention was 2.2 (0.60 to 2.60). CONCLUSIONS: This series included 52 eyes with ECH, recognized associations of ECH with different types of anesthesia and with different cataract surgical procedures, and described management of ECH. Postoperative visual outcome was poor.

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PURPOSE: To assess the prevalence of digital eye strain among children and extrapolate the association between knowledge, attitude, and practice patterns related to device use during the coronavirus disease 2019 (COVID-19) lockdowns. METHODS: A cross-sectional, descriptive, questionnaire-based analysis was done to assess the knowledge, attitude, and practice patterns related to digital device use among parents of children attending online classes. RESULTS: A total of 305 responses were obtained. The most common reason for device use was online classes (288 children; 94.4%) and the most common mode was smartphone (263 children; 86.3%). The prevalence of digital eye strain was 64.6%. The mean knowledge score was 48.5 ± 5.1, the mean attitude score was 26.7 ± 4.9, and the mean practice score was 17.8 ± 3.5. The difference between knowledge, attitude, and practice scores among parents of children with and without glasses was not statistically significant (P = .580, .521, and .503, respectively). A direct correlation was found between the knowledge and practice scores (P = .002), but attitude scores did not show a significant correlation (P = .712). CONCLUSIONS: Digital devices have been a boon to continue education during the ongoing COVID-19 pandemic. This study reveals a large knowledge gap among parents related to safe digital device use. Further, there is a need to adopt methods that would help spread awareness to the masses about the effects of excessive screen time in children in the form of digital eye strain and myopia and the corrective measures to avoid the same. [J Pediatr Ophthalmol Strabismus. 2022;59(4):224-235.].

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PURPOSE: To compare the safety and efficacy of Active Sentry handpiece with Ozil handpiece for Centurion phacoemulsification system. SETTING: Tertiary eye center, South India. DESIGN: Prospective observational study. METHODS: 204 eyes of 204 patients with uncomplicated cataract who underwent phacoemulsification cataract surgery with Centurion Vision System were randomized into 2 groups: Ozil handpiece (n = 101) and Active Sentry handpiece (n = 103). Intraoperative factors such as patient pain perception, surgeon comfort level, amount of phacoemulsification energy and aspiration fluid used, and frequency of activation of active surge mitigation (ASM) were analyzed, and postoperatively, corrected distance visual acuity (CDVA) and corneal edema on day 1 were compared. RESULTS: A total of 204 eyes of 204 patients were included among which 101 underwent surgery with ozil hand piece and 103 with Active Sentry handpiece. Patient pain perception was comparable between the groups with no statistically significant differences in patients who had pain-free surgery (66% vs 61.3%) and those who experienced moderate pain (24.3% vs 28.7%). Surgeons were more comfortable using Ozil handpiece during entry into anterior chamber and emulsification of hard nuclei (48.5% vs 28.6%). The mean cumulative dissipated energy for soft and hard cataracts was 5.6 and 4.8 and 9.3 and 9.4 for Ozil and Active Sentry groups, respectively. ASM was activated for 53 eyes (51.5%), of which 42 eyes (79.2%) had soft cataract and 11 eyes (20.7%) hard cataract. Postoperative CDVA and incidence of corneal edema were comparable between the groups. CONCLUSIONS: For Centurion Vision System, Active Sentry handpiece was as safe and efficacious as the Ozil handpiece with added benefit of operating at lower intraocular pressure levels.

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