Minimally Invasive Surgery for Intraocular Lens Removal and Intrascleral Intraocular Lens Fixation with Trabeculectomy in a Patient with Dislocated Intraocular Lens and Elevated Intraocular Pressure

A 88-year-old female who was being treated for end-stage pseudoexfoliation syndrome was referred to our hospital for treatment of dislocated intraocular lens (IOL) and the elevated intraocular pressure (IOP) and in the right eye (RE). At the first visit to our hospital, best-corrected visual acuity (BCVA) was 0.2 in the RE and 0.02 in the left eye (LE). IOP was 47 mm Hg in the RE and 21 mm Hg in the LE. Slit-lamp examination showed no abnormalities in anterior segments and dislocated IOL in the RE. Fundus photograph showed optic disc pallor in both eyes. We performed the combined therapy of flanged intrascleral IOL fixation with the double-needle technique and trabeculectomy. Throughout the follow-up period, BCVA slightly improved from 0.2 to 0.4 in the RE. The angle of tilt of the IOL was 6.6, 7.9, and 8.7° as measured by swept-source optical coherence tomography at 1, 4, and 6 months after the surgery, respectively. The IOP remained less than 10 mm Hg without having to administer any other glaucoma medications. Furthermore, any complications associated with the surgery were not confirmed

ABCC6

Purpose. To report the spectrum of ABCC6 variants in Japanese patients with angioid streaks (AS). Patients and Methods. This was a single-center cohort study. The medical records of 20 patients with AS from 18 unrelated Japanese families were retrospectively reviewed. Screening of the ABCC6 gene (exons 1 to 31) was performed using PCR-based Sanger sequencing. Results. Eight ABCC6 variants were identified as candidate disease-causing variants. These eight variants included five known variants (p.Q378X, p.R419Q, p.V848CfsX83, p.R1114C, and p.R1357W), one previously reported variant (p.N428S) of unknown significance, and two novel variants (c.1939C>T [p.H647Y] and c.3374C>T [p.S1125F]); the three latter variants were determined to be variants of significance. The following four variants were frequently identified: p.V848CfsX83 (14/40 alleles, 35.0%), p.Q378X (7/40 alleles, 17.5%), p.R1357W (6/40 alleles, 15.0%), and p.R419Q (4/40 alleles, 10.0%). The ABCC6 variants were identified in compound heterozygous or homozygous states in 13 of 18 probands. Two families showed a pseudodominant inheritance pattern. Pseudoxanthoma elasticum was seen in 15 of 17 patients (88.2%) who underwent dermatological examination. Conclusions. We identified disease-causing ABCC6 variants that were in homozygous or compound heterozygous states in 13 of 18 families (72.2%). Our results indicated that ABCC6 variants play a significant role in patients with AS in the Japanese population

Surgical outcome comparisons of multifocal IOLs of Lentis Comfort LS-313 MF15 and Tecnis Eyhance DIB00V

AIM: To compare the surgical outcomes of a multifocal intraocular lens (IOL; Lentis Comfort LS-313 MF15) with those of an enhanced monofocal IOL (Tecnis Eyhance DIB00V). METHODS: This retrospective study included patients who underwent cataract surgery with LS-313 MF15 or Eyhance IOL implantation. Data regarding patient demographics, surgical records, and ophthalmic examination before the cataract surgery and one and three months postoperatively were collected. Visual acuities, refractive values, defocus curves, contrast sensitivities and subjective symptoms were evaluated. RESULTS: Among the 71 eyes (47 patients) included in this study, 32 eyes (20 patients) underwent LS-313 MF15 IOL implantation, and 39 eyes (27 patients) underwent Eyhance IOL implantation. No significant differences were observed in age, axial length, or refractive error between the two groups preoperatively. Furthermore, the distance-corrected and uncorrected distance visual acuities one month postoperatively did not differ between the groups, and both groups had sufficient visual acuities at the distances of 5, 1 m, 70, 50, and 30 cm. Other ophthalmic data, including subjective symptoms based on the 14-item Visual Function Index Questionnaire, monocular defocus curves, contrast sensitivities, and halo and glare, did not differ between the groups three months postoperatively. Moreover, both groups had good outcomes. The spherical equivalent one month postoperatively was significantly myopic in the LS-313 MF15 group compared with that in the Eyhance group (P=0.033); however, this difference was not observed three months postoperatively (P=0.471). CONCLUSION: Comparison of the surgical outcomes of LS-313 MF15 with those of Eyhance with different optical properties reveal that both IOLs show good postoperative outcomes, with no significant differences being noted between the two IOLs

Clinical Course of OCTA en face Imaging Findings in a Patient with HELLP Syndrome

A 36-year-old primigravid woman with suspected gestational hypertension was referred to our hospital for further examination and treatment of bilateral serous retinal detachment (SRD). At the previous hospital immediately after the onset of visual symptoms (decreased visual acuity), the best-corrected visual acuity (BCVA) had been 0.1 in the right eye (RE) and 0.08 in the left eye (LE). Funduscopy revealed diffuse SRD and white discoloration at the posterior pole but no retinal tear in both eyes (BE). Optical coherence tomography (OCT) also revealed SRD. One day after the onset, we diagnosed bilateral SRD due to preeclampsia with HELLP syndrome because of elevated blood pressure, hemolysis, elevated liver enzymes, and low platelet and performed an emergency cesarean section. One week after the onset, BCVA improved to 0.3 in BE. Funduscopy showed resolution of SRD and the remaining white discoloration at the posterior pole. OCT showed complete resolution of SRD, blurred external limiting membrane, and an invisible ellipsoid zone. OCT angiography (OCTA) segmentation of the deep retinal layer (using en face imaging) revealed multiple white materials, mainly in the macula. Although funduscopy and OCT showed almost normal appearance by 2 months after the onset, BCVA was not completely improved. OCTA using en face imaging revealed shrinkage of multiple white materials but remnant material at the posterior pole. One year after the onset, BCVA was 0.9 in the RE and 0.8 in the LE. OCTA using en face imaging revealed remnant white materials but marked improvement overall

Machine Learning Prediction for Supplemental Oxygen Requirement in Patients with COVID-19

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Novel homozygous CLN3

Abstract Background Biallelic CLN3 gene variants have been found in either juvenile‐onset neuronal ceroid lipofuscinosis (JNCL) or isolated retinal dystrophy. It has been reported that most JNCL patients carry a common 1.02‐kb deletion variant homozygously. Clinical characteristics of patients with biallelic CLN3 missense variants are not well elucidated. Methods We described a 26‐year‐old Japanese male patient with isolated retinal dystrophy. Whole‐exome sequencing (WES) and transmission electron microscopy (TEM) were performed. Results Whole‐exome sequencing identified a novel homozygous CLN3 missense variant [c.482C>T; p.(Ser161Leu)]. Ophthalmoscopy revealed retinal degeneration and macular atrophy, and later attenuated retinal vessels. Severely reduced responses were observed in both rod and cone electroretinograms. In TEM of the patient's lymphocytes, fingerprint profiles, which are specific findings in CLN3‐associated JNCL, were observed in 16/624 (2.56%) lymphocytes of the patient, who has never exhibited neurological signs during the 13‐year follow‐up period. Conclusion Our results indicated that this novel CLN3 missense variant is associated with teenage‐onset isolated retinal dystrophy. This is the first report of any patient with CLN3‐associated disorder in the Japanese population. Although fingerprint profiles have never been reported in CLN3‐associated isolated retinal dystrophy, these profiles were observed, albeit infrequently, suggesting that frequency of the fingerprint profiles might depend on variant types

Noninvasive regional cerebral oxygen saturation for neurological prognostication of patients with out-of-hospital cardiac arrest: a prospective multicenter observational study.

[Aim]To investigate the association between regional brain oxygen saturation (rSO2) at hospital arrival and neurological outcomes at 90 days in patients with out-of-hospital cardiac arrest (OHCA). [Methods]The Japan-Prediction of neurological Outcomes in patients post cardiac arrest (J-POP) registry is a prospective, multicenter, cohort study to test whether rSO2 predicts neurological outcomes after OHCA. We measured rSO2 in OHCA patients immediately after hospital arrival using a near-infrared spectrometer placed on the forehead with non-blinded fashion. The primary endpoint was “neurological outcomes” at 90 days after OHCA. [Results]EMS providers are not permitted to terminate CPR in the field in Japan, and so most patients with OHCA who are treated by EMS personnel are transported to emergency hospitals. Among 1017 OHCA patients, 672 patients including 52 comatose patients with pulses detectable (8%) and 620 cardiac arrest patients (92%) at hospital arrival were enrolled prospectively and consecutively. Twenty-nine patients with good neurological outcome had a significantly higher value of rSO2 at hospital arrival than 643 patients with poor neurological outcome (mean [±SD] 55.6 ± 20.8% vs. 19.7 ± 11.0%, p 42% for predicting good neurological outcome, with sensitivity 0.79 (95% confidence interval [CI], 0.60?0.92), specificity 0.95 (95% CI, 0.93?0.96), positive predictive value, 0.41 (95% CI, 0.28?0.55), negative predictive value, 0.99 (95% CI, 0.98?1.00), and area under the curve 0.90 (95% CI, 0.88?0.92). [ConclusionThe rSO2 at hospital arrival can predict good neurological outcome at 90 days after OHCA

Characteristics of regional cerebral oxygen saturation levels in patients with out-of-hospital cardiac arrest with or without return of spontaneous circulation: A prospective observational multicentre study.

[Aim] Our study aimed at filling the fundamental knowledge gap on the characteristics of regional brain oxygen saturation (rSO2) levels in out-of-hospital cardiac arrest (OHCA) patients with or without return of spontaneous circulation (ROSC) upon arrival at the hospital for estimating the quality of cardiopulmonary resuscitation and neurological prognostication in these patients. [Methods] We enrolled 1921 OHCA patients from the Japan – Prediction of Neurological Outcomes in Patients Post-cardiac Arrest Registry and measured their rSO2 immediately upon arrival at the hospital by near-infrared spectroscopy using two independent forehead probes (right and left). We also assessed the percentage of patients with a good neurological outcome (defined as cerebral performance categories 1 or 2) 90 days post cardiac arrest. [Results] After 90 days, 79 (4%) patients had good neurological outcomes and a median lower rSO2 level of 15% (15–20%). Compared to patients without ROSC upon arrival at the hospital, those with ROSC had significantly higher rSO2 levels (56% [39–65%] vs. 15% [15–17%], respectively; P < 0.01), and significantly correlated right- and left-sided regional brain oxygen saturation levels (R = 0.94 vs. 0.66, respectively). In both groups, the percentage of patients with a good 90-day neurological outcome increased significantly in proportion to their rSO2 levels upon arrival at the hospital (P < 0.01). [Conclusion] Our data indicate that measuring rSO2 levels might be effective for both monitoring the quality of resuscitation and neurological prognostication in patients with OHCA