Anterior Chamber Intraocular Lens Implantation Versus Four-Flanged Scleral Fixation Technique for Patients With Loss of Capsular Support During Routine Cataract Surgery.

PURPOSE: To compare the outcomes of anterior chamber intraocular lens (AC-IOL) implantation versus the four-flanged scleral fixation technique in eyes with loss of capsular support during routine cataract surgery. METHODS: This was a retrospective cohort study of all patients in whom an IOL was implanted either during or after short-term aphakia due to loss of capsular and sulcus support in a routine cataract surgery with at least 6 months of follow-up time between 2015 and 2023 in a tertiary medical center in Israel. Two different IOL implantation techniques were compared: the implantation of an angle-supported ACIOL and four-flanged scleral fixation. Main outcome measures included postoperative complications such as pseudophakic bullous keratopathy, loss of intraocular pressure control and glaucoma, IOL subluxation, and retinal detachment. RESULTS: Sixty-five eyes of 65 patients were included in the study, 33 eyes in the AC-IOL group and 32 eyes in the flange group. Follow-up time was 29.92 ± 20.02 months in the AC-IOL group and 20.17 ± 15.56 months in the flange group (P = .087). Pseudophakic bullous keratopathy was observed in 10 (30.3%) patients in the AC-IOL group and in 1 (3.1%) patient in the flange group (P = .04). This association remained significant using survival analysis (P = .006). In 4 (12.1%) patients in the AC-IOL group, a glaucoma filtering procedure was performed to control the intraocular pressure compared with none in the flange group (P = .042). CONCLUSIONS: In the setting of loss of capsular support during routine cataract surgery, four-flanged scleral fixation showed an overall lower rate of complications and significantly reduced rate of subsequent surgical interventions. [J Refract Surg. 2024;40(8):e520-e526.].

Best Disease: Global Mutations Review, Genotype-Phenotype Correlation, and Prevalence Analysis in the Israeli Population.

Purpose: To review all reported disease-causing mutations in BEST1, perform genotype-phenotype correlation, and estimate disease prevalence in the Israeli population. Methods: Medical records of patients diagnosed with Best disease and allied diseases from nine Israeli medical centers over the past 20 years were collected, as were clinical data including ocular findings, electrophysiology results, and retina imaging. Mutation detection involved mainly whole exome sequencing and candidate gene analysis. Demographic data were obtained from the Israeli Bureau of Statistics (January 2023). A bibliometric study was also conducted to gather mutation data from online sources. Results: A total of 134 patients were clinically diagnosed with Best disease and related conditions. The estimated prevalence of Best disease was calculated to be 1 in 127,000, with higher rates among Arab Muslims (1 in 76,000) than Jews (1 in 145,000). Genetic causes were identified in 76 individuals (57%), primarily showing autosomal-dominant inheritance due to BEST1 mutations (58 patients). Critical conserved domains were identified consisting of a high percentage of dominant missense mutations, primarily in transmembrane domains and the intracellular region (Ca2+ binding domain) of the BEST1 protein. Conclusions: This study represents the largest cohort of patients with Best disease reported in Israel and globally. The prevalence in Israel is akin to that in Denmark but is lower than that in the United States. Critical conserved domains within the BEST1 protein are pivotal for normal functioning, and even minor missense alterations in these areas lead to a dominant disease manifestation. Genetic testing is indispensable as the gold standard for Best disease diagnosis due to the variable clinical presentation of the disease.