Granular corneal dystrophy type 2 (GCD2) is an autosomal dominant corneal stromal dystrophy that is caused by p.Arg124His mutation of transforming growth factor β induced (TGFBI) gene. It is characterized by well demarcated granular shaped opacities in central anterior stroma and as the disease progresses, extrusion of the deposits results in ocular pain due to corneal epithelial erosion. Also, diffuse corneal haze which appears late, causes decrease in visual acuity. The prevalence of GCD2 is high in East Asia including Korea. Homozygous patients show a severe phenotype from an early age, and the heterozygote phenotype varies among patients, depending on several types of compound heterozygous TGFBI mutations. In the initial stage, conservative treatments such as artificial tears, antibiotic eye drops, and bandage contact lenses are used to treat corneal erosion. Different surgical methods are used depending on the depth and extent of the stromal deposits. Phototherapeutic keratectomy removes anterior opacities and is advantageous in terms of its applicability and repeatability. For deeper lesions, deep anterior lamellar keratoplasty can be used as the endothelial layer is not always affected. Recurrence following these treatments are reported within a wide range of rates in different studies due to varying definition of recurrence and follow-up period. In patients who have undergone corneal laser vision-correction surgeries such as photorefractive keratectomy, LASEK, or LASIK including SMILE surgery, corneal opacity exacerbates rapidly with severe deterioration of visual acuity. Further investigations on new treatments of GCD2 are necessary.

Purpose: To evaluate the effect of the Active Sentry handpiece of the Centurion Vision System compared to the Centurion Ozil handpiece for phacoemulsification in cataract surgery. Methods: A retrospective study was conducted on 281 patients (449 eyes) who underwent cataract surgery between August 2020 and June 2021. Preoperative measurements, intraoperative parameters, complication rate, and postoperative outcomes were compared between the Active Sentry handpiece and the Centurion Ozil handpiece groups. Additionally, the parameters were compared in different cataract severity groups and multiple predictive factors for the number of active surge mitigation (ASM) actuations were assessed with the Active Sentry handpiece. Results: There were 198 eyes in the Active Sentry group and 251 eyes in the Centurion Ozil group. There were no statistically significant differences between the two groups, as the cumulative dissipated energy in the Active Sentry and Centurion Ozil groups were 8.32 ± 7.74 and 7.87 ± 9.25 μJ, respectively (p = 0.576). Total surgery time, ultrasound usage time, aspiration time, amount of fluid aspirated, postoperative corrected distant visual acuity, and postoperative decrease in corneal endothelial cell density were comparable between the two groups. The significant contributors to the number of ASM actuations were age, preoperative corrected distant visual acuity, axial length, and total ultrasound time. Conclusions There was no clear advantage of the Active Sentry handpiece compared to the Centurion Ozil handpiece. ASM actuation increases with age, poor visual acuity before surgery, short axial length, and prolonged ultrasound usage time. It is expected that in more severe and high-risk cataract surgery, the Active Sentry handpiece functions more effectively, possibly affecting the safety and prognosis.

Purpose: To evaluate the long-term maintenance rate and associated factors of silicone punctal plugs in patients with Sjögren’s syndrome (SS). Materials and Methods: We retrospectively reviewed the medical records of 163 patients with SS who underwent silicone punctal plug insertion between December 2013 and July 2021 at Severance Hospital. The status of punctal plug insertions was classified into the following three categories by the clinician: maintenance, spontaneous loss, and intended removal. Cox proportional hazards model was used to evaluate the risk factors for spontaneous loss. Results: The mean maintenance period was 12.8±15.3 (median 7.07) months. The rate of spontaneous loss was 58%, and the rate of punctal plug removal by the clinician was 14%. The number of prior plug insertions was a risk factor for spontaneous loss [hazard ratio (HR) 1.055, p=0.035]. The upper eyelid punctum was at a higher risk than the lower one (p=0.042). Small-sized plugs showed a significantly higher risk for spontaneous loss than large-sized ones (HR 1.287, p=0.035). Flow-controller type plugs were more vulnerable to spontaneous loss than complete occluders [Micro FlowTM vs. EagleFlex® (HR 2.707, p=0.008) and Micro FlowTM vs. UltraplugTM (HR 3.402, p=0.005)]. The most common reason for removal was tear overflow (5.6%). Conclusion In repeated insertion, characteristics of the punctal plug, including the type and size, and location of plug insertion, influenced the spontaneous loss of plugs. The management of punctal plugs, including insertion, maintenance, and removal, requires personalized strategies for versatile situations.

Purpose: A patient with heterozygous granular corneal dystrophy type 2 (GCD2) underwent phacoemulsification with multifocal intraocular lens insertion, and complained of visual discomfort. We investigated the cause of the discomfort and visual function in this case.Case summary: A 59-year-old woman with granular opacity had slit lamp photographs taken 5 years earlier. Two years later, she underwent phacoemulsification with multifocal intraocular lens (Trifocal AT Lisa tri toric 839MP®, Carl Zeiss Meditec AG, Inc., Jena, Germany) insertion in both eyes at a local clinic. She felt very uncomfortable after the surgery, but the granular and lattice opacities due to GCD2 of her corneas remained stationary for 5 years. Her visual acuity decreased from preoperatively (preoperative: right 0.5, left 0.6; last visit: right 0.3, left 0.4). Her contrast sensitivity was also decreased and the total higher order aberration was increased (right 1.590 μm, left 1.194 μm), compared to normal range. Conclusions Multifocal intraocular lens insertion in cataract surgery can lead to severe declines in contrast sensitivity and visual acuity and increased higher-order aberration in a GCD2 patient. It may not be advisable to use multifocal intraocular lenses in a GCD2 patient.

Purpose: Granular corneal dystrophy type 2 (GCD2) is a hereditary disease that features granular and lattice stromal deposits in the cornea. There are homozygotes and heterozygotes and the opacities are exacerbated by corneal trauma or surgery, such as laser in situ keratomileusis (LASIK). As there is individual variability in GCD2 phenotypes, we investigated various corneal features of GCD2 patients in their twenties, the main age group for refractive surgery. Methods: From genetically confirmed GCD2 patients who had an R124H mutation of the transforming growth factor β induced (TGFBI) gene at age 20 to 29 years, we chose representative patients: one homozygote; one compound heterozygote; one simple heterozygote with a severe phenotype with many granular deposits; one common heterozygote; and four heterozygotes with normal corneas. The corneas of all patients were subject to slit-lamp examination and photographed. Results: The homozygote had confluent granular deposits covering the cornea. The compound heterozygote had granular and lattice deposits covering the center of the cornea. The patient with a severe phenotype had more than 30 granular deposits in one eye, but was a simple GCD2 heterozygote, verified by full-sequencing of the TGFBI gene. In the four patients with normal corneas, a single small lesion was subsequently detected during follow-up in two, at 3 weeks and 6 months, respectively. Both corneas were judged clear at chance examinations. Conclusions Among Koreans in their twenties, GCD2 patients have various phenotypes, from clear corneas to severe confluent opacities. There are GCD2 heterozygotes with nearly clear corneas, so caution must be taken when choosing patients for refractive surgery.

Purpose: To evaluate the clinical availability of a multifunctional ocular biometric unit, MR-6000, for simultaneous keratometry, tonometry, topography, and pachymetry evaluation, and compare anterior segment measurements with five other devices: autokeratometer (KR-1), Scheimpflug camera (Pentacam HR), swept-source optical coherence tomography (IOLMaster 700), Placido disk scanning-slit topography (Orbscan II), and noncontact tonometry (FT-1000). Methods: Thirty eyes from thirty patients who visited Severance Hospital for cataract surgery were examined using MR6000 and the other devices. The mean keratometry, central corneal thickness (CCT), white-to-white (WTW) distance, and intraocular pressure (IOP) values were compared. Repeated measures analysis of variance, Wilcoxon signed-rank test, intraclass correlation coefficient (ICC), and Bland-Altman plot were used to assess the correlation and agreement between devices. Results: Thirty eyes of thirty patients were evaluated. Statistically significant differences in mean keratometry between MR6000, KR-1, Pentacam HR, and IOLMaster 700 were not observed (p > 0.05). All five devices, including Orbscan II, had almost perfect agreement in measuring keratometry (ICC > 0.80, p < 0.05). CCT measured by MR-6000 was significantly different from that of Pentacam HR and Orbscan II measurements (p < 0.05) but correlated with that of Pentacam HR and Orbscan II measurements (ICC > 0.60, p < 0.05). The WTW distance measured by MR-6000 was not significantly different from that measured by IOLMaster 700 but was different from that measured by Orbscan II. IOP measured by MR-6000 was not correlated with FT-1000. Conclusions Keratometric values obtained through MR-6000 can be used interchangeably with other devices based on good correlation and agreement. However, the CCT, WTW, and IOP values were not interchangeable with a single multifunctional unit for cataract surgery preoperative examination.

Purpose: To compare anterior biometry measurements using placido-scanning-slit topography, rotating Scheimpflug tomography, and swept-source optical coherence tomography. Methods: A retrospective review consisted of 80 eyes of 49 participants who underwent anterior chamber depth (ACD), central corneal thickness (CCT), and keratometry examination on the same day. We used placido-scanning-slit topography (ORBscan II), rotating Scheimpflug tomography (Pentacam HR), and swept-source optical coherence tomography (CASIA SS1000). The intraclass correlation coefficients and Bland-Altman plots were used to evaluate the agreement and differences between measurements. Results: The mean ACD values were 2.88 ± 0.43, 2.82 ± 0.50, and 2.68 ± 0.44 mm; and the mean CCT values were 536.96 ± 31.19, 543.79 ± 31.04, and 561.41 ± 32.60 μm; and the mean keratometry (Km) were 43.81 ± 1.69, 43.81 ± 1.77, and 44.65 ± 1.95 diopters; as measured by CASIA SS-1000, Pentacam HR, and ORBscan II, respectively. Among the three devices, ACD was deepest to shallowest in the order of CASIA SS-1000, Pentacam HR, and ORBscan II (p < 0.05). The CCT was thickest to thinnest in the order of ORBscan II, Pentacam HR, and CASIA SS-1000 (p < 0.05). No significant differences in Km values were examined between CASIA SS-1000 and Pentacam HR, whereas ORBscan II overestimated Km with a statistically significant difference compared to the other two devices. Conclusions High level of agreement was found between CASIA SS-1000 and Pentacam HR for anterior parameters, including ACD, CCT, and Km, suggesting interchangeability. However, ORBscan II measurements differed considerably with the measurements obtained from the other two devices; therefore, it should not be used interchangeably. However, further studies with repeatability test should be considered in order to elucidate the reliability of each device.