Efficacy of Evidence-based Nursing for Senile Cataract Complicated with Primary Angle-closure Glaucoma.

Objective: To explore the efficacy and ocular indicator changes of evidence-based nursing in elderly patients with cataracts complicated with primary angle-closure glaucoma (PACG) after surgery. Methods: 100 elderly cataract patients combined with PACG treated in the People's Hospital of Xinjiang Uygur Autonomous Region between February 2019 and October 2020 were included in the study and equally assigned to a control group and an experimental group by random draw. The control group adopted conventional nursing, and the experimental group intervened with evidence-based nursing. A thorough analysis was conducted based on the comparison of nursing effectiveness, nursing satisfaction, complication rate at 1, 2, and 3 weeks after surgery, QLI (Quality of Life Index), PSQI (Pittsburgh Sleep Quality Index), SPEED (Standard Patient Evaluation of Eye Dryness), SEEQ (Salisbury Eye Evaluation Questionnaire), eyesight and intraocular pressure between two groups of patients. Results: The evidence-based nursing was more effective in treating patients with senile cataracts and PACG after surgery compared with routine care (P = .002). The patients in the experimental group were more satisfied with evidence-based nursing than those in the other group (P < .001). The experimental group yielded a more desirable outcome than the control group in terms of the complication rate of patients of the two groups at 1 week, 2 weeks, and 3 weeks after surgery (P = .02, .003, < .001). In contrast to the group with routine care, the evidence-based nursing group obtained significantly higher scores of QLI (P < .001), but intensely lower results of PSQI (P < .001). Lower SPEED and SEEQ results of the experimental group were observed, as compared to the control group (P = .001, < .001). The patients in the experimental group enjoyed better eyesight and intraocular pressure after the evidence-based nursing in comparison with the control group (both P < .001). The group with evidence-based intervention garnered a more desirable result than the group with routine care about the complication rate at 1, 2, and 3 weeks after surgery, PSQI, SPEED, SEEQ and intraocular pressure. Conclusion: Evidence-based nursing intervention reaped huge fruits in the improvement of the efficiency and quality of nursing work and also in the optimization of the ocular indicators of patients, which is highly applicable in clinical practice.

Truncation mutations in MYRF underlie primary angle closure glaucoma.

Mutations in myelin regulatory factor (MYRF), a gene mapped to 11q12-q13.3, are responsible for autosomal dominant high hyperopia and seem to be associated with angle closure glaucoma, which is one of the leading causes of irreversible blindness worldwide. Whether there is a causal link from the MYRF mutations to the pathogenesis of primary angle-closure glaucoma (PACG) remains unclear at this time. Six truncation mutations, including five novel and one previously reported, in MYRF are identified in seven new probands with hyperopia, of whom all six adults have glaucoma, further confirming the association of MYRF mutations with PACG. Immunofluorescence microscopy demonstrates enriched expression of MYRF in the ciliary body and ganglion cell layer in humans and mice. Myrfmut/+ mice have elevated IOP and fewer ganglion cells along with thinner retinal nerve fiber layer with ganglion cell layer than wild-type. Transcriptome sequencing of Myrfmut/+ retinas shows downregulation of Dnmt3a, a gene previously associated with PACG. Co-immunoprecipitation demonstrates a physical association of DNMT3A with MYRF. DNA methylation sequencing identifies several glaucoma-related cell events in Myrfmut/+ retinas. The interaction between MYRF and DNMT3A underlies MYRF-associated PACG and provides clues for pursuing further investigation into the pathogenesis of PACG and therapeutic target.

Factors associated with severe corneal endothelial damage following acute primary angle closure in Chinese subjects.

PURPOSE: To investigate the corneal endothelial damage caused by acute primary angle closure (APAC) and related risk factors for severe corneal endothelial cell damage in Chinese subjects. METHODS: In this multicentre retrospective study, 160 Chinese patients (171 eyes) diagnosed with APAC were recruited. Endothelial cell density (ECD) and morphological changes short after APAC were studied. Univariate regression and multivariate regression were used to identify risk factors associated with the extent of ECD reduction, including age, gender, education level, patients' location, systemic diseases, APAC duration (hours), highest recorded intraocular pressure (IOP), and presenting IOP. Factors associated with the probability of severe corneal damage (ECD lower than 1000/mm2) were analysed based on a linear function. RESULTS: After one APAC episode, 12.28% eyes had ECD lower than 1000/mm2, 30.41% had ECD between 1000 and 2000/mm2, and 57.31% had ECD more than 2000/mm2. Attack duration was the only factor associated with severe endothelial damage (p < 0.0001). If the attack were to be subsided within 15.0 h, possibility of ECD lower than 1000/mm2 could be controlled under 1%. CONCLUSION: Shortly after the abortion of APAC, 12.28% patients experienced severe endothelial cell damage with ECD less than 1000/mm2. The only factor associated with severe ECD decrease was attack duration. Immediate and effective treatment is pivotal for preserving corneal endothelial function in APAC patients.

Risk factors of surgical failure in combined phacoemulsification and excisional goniotomy for angle-closure glaucoma.

PURPOSE: To evaluate the therapeutic success, and risk factors for combined phacoemulsification and Kahook Dual Blade excisional goniotomy (Phaco/KDB) in primary angle-closure glaucoma (PACG). METHODS: A retrospective review was conducted on glaucoma patients who underwent Phaco/KDB between September 2019 and August 2021 at 2 ophthalmology centers. Complete success was defined as unmedicated intraocular pressure (IOP) ≤ 18 mmHg with no further glaucoma surgery, while the medicated IOP ≤ 18 mmHg was defined as qualified success with lesser medications than at baseline. RESULTS: Seventy-two eyes (64 patients) with a mean age of 67.1 ± 8.1 years were included in this study, and 70.2% were female. The mean medicated baseline IOP decreased from 23.4 ± 8.1 to 16.6 ± 3.9 mmHg at an average of 11.6 ± 3.7 months of follow-up (- 29.1%; P < 0.00). Medications decreased from 2.6 ± 1.3 to 0.4 ± 0.9 (- 82.6%; P < 0.001). Complete success and qualified success were achieved in 65.3% and 79.2% of eyes, respectively. Male gender [hazard ratio (HR): 6.00 (1.57-22.9); P = 0.009] was observed a risk factor for surgical failure, whereas higher axial length (HR:0.37 (0.16-0.86); P = 0.021), and a combined circumferential goniosynechialysis procedure (HR: 0.13 (0.02-0.74); P = 0.022) lowered the risk of surgical failure. Hyphema, postoperative shallowing anterior chamber, and IOP spike were the most common complications. The cumulative survival proportion for qualified success at 12 months was 82.5% ± 0.05 (95% CI, 0.70-0.90). CONCLUSION: Phaco/KDB provided acceptable therapeutic success rate and may, therefore, be recommended in patients with coexisting cataract and PACG. Identifying patients with risk factors preoperatively may help clinicians predict surgical success.

Phaco-goniosynechialysis versus phaco-trabeculectomy in patients with refractory primary angle-closure glaucoma: a comparative study.

PURPOSE: To compare the effects of phacoemulsification with intraocular lens implantation (phaco) combined with goniosynechialysis (phaco + GSL) versus phaco with trabeculectomy (phaco + trab) for the management of primary angle-closure glaucoma (PACG) refractory to peripheral anterior synechiae (PAS) of over 180°. METHODS: This retrospective study followed 77 eyes of 77 patients for at least 6 months. Intraocular pressure (IOP), best-corrected visual acuity (BCVA), number of glaucoma drugs, and PAS were recorded at the preoperative baseline and evaluated at each postoperative follow-up visit. The National Eye Institute Visual Functioning Questionnaire-25 (NEI VFQ-25) was administered to patients enrolled in this study. Pearson's correlation analysis and multivariate linear analysis were performed to identify factors influencing changes in NEI VFQ-25 scores and to identify factors associated with increases in NEI VFQ-25 scores after the operation. RESULTS: In total, seventy-seven eyes were included (43 with phaco + GSL and 34 with phaco + trab). Comparing preoperative baseline and month 6 after surgery measurements revealed that both groups found significant improvements in IOP, PAS, BCVA and the number of glaucoma drugs (P < 0.05). Baseline NEI VFQ-25 scores were similar in the two groups, but there was a significant difference in postoperative NEI VFQ-25 scores (74.47 ± 10.39 in phaco + GSL vs. 69.57 ± 8.54 in phaco + trab, P = 0.048 < 0.05), and the phaco + GSL group had better scores at the time of the last follow-up. The change in preoperative scores and the number of glaucoma drugs was significantly correlated with postoperative scores in the phaco + GSL group. CONCLUSION: Phaco + GSL treatment is as safe and effective as phaco + trab for refractory PACG patients, and patients' subjective experience improved significantly after phaco + GSL surgery.

Bilateral angle closure glaucoma with retinitis pigmentosa in young patients: case series.

BACKGROUND: To report the ocular characteristics and management of three cases of retinitis pigmentosa (RP) concurrent primary angle closure glaucoma (PACG). CASE PRESENTATION: Three middle-aged patients presenting with diminished vision, high intraocular pressure (IOP), and typical fundus manifestations of RP were clinically evaluated. The individualized treatment was based on the ocular conditions of each case. A novel genetic alteration in ZNF408 was identified in one patient. Two patients with short-axial eyes received unilateral combined trabeculectomy, cataract surgery, and Irido-zonulo-hyaloid-vitrectomy. One of them had a subluxated lens, managed with a capsular tension ring implantation. Their contralateral eyes, respectively, underwent laser peripheral iridotomy (LPI) and transscleral cyclophotocoagulation. The third patient underwent bilaterally combined laser peripheral iridoplasty, LPI, and medication. Ultimately, all patients achieved the target IOP during a two-year follow-up. CONCLUSION: Young patients with RP may have a risk of developing angle closure glaucoma, and conversely, patients with angle closure glaucoma at younger age should be aware of the presence of RP. Therefore, routine gonioscopy and IOP monitoring are required for RP patients, and detailed fundus examinations are warranted for young PACG patients.

A unique case of bilateral nanophthalmos and pigmentary retinal abnormality with unilateral angle closure glaucoma and optic disc pit.

BACKGROUND: Microophthalmos or 'dwarf eye' is characterized by an axial length 2 standard deviation less than age-matched controls. It is classified into nanophthalmos, relative anterior microphthalmos, and posterior microphthalmos based on the anterior segment: posterior segment ratio. Nanophthalmos can occur in association with optic disc drusen, foveoschisis, and retinitis pigmentosa, as an autosomal recessive syndrome linked to mutations in the MFRP gene. We report a case of bilateral nanophthalmos and pigmentary retinopathy with angle closure glaucoma and optic disc pit in one eye. We believe this to be the first case presenting with optic disc pit in association with nanophthalmos. CASE PRESENTATION: A 56-year-old female presented with bilateral small eyes, high hypermetropia, shallow anterior chamber depth, increased lens thickness, mid-peripheral retinal flecks, and macular edema. She also had high intraocular pressure in the right eye, with a disc cupping of 0.9 with an Optic disc pit. The macular edema in the right eye was found to occur in association with the Optic disc pit, whereas, in the left eye, it was associated with intra-retinal hemorrhages and diagnosed as macular branch retinal vein occlusion secondary to hypertension. She was started on anti-glaucoma medications in both eyes and planned for Anti-VEGF injection in the left eye. CONCLUSION: This case report is unique as it reports an association of Nanophthalmos with Optic Disc pit, with an associated angle closure glaucoma in the same eye, an association which has never been previously reported in the literature.

Management of angle-closure glaucoma with X-linked retinoschisis: a case report.

BACKGROUND: X-linked retinoschisis (XLRS), due to mutations in the RS1 gene, is a common genetically determined form of macular degeneration. This report describes an unusual case of angle-closure glaucoma (ACG) with XLRS and discusses the treatment. CASE PRESENTATION: A 39-year-old Chinese man with an X chromosome-recessive inherited c.489G > A variant in the RS1 gene was diagnosed as XLRS and ACG, presenting with cystic macular lesions, shallow anterior chamber depth (ACD), and angle-closure with uncontrolled intraocular pressure (IOP). Malignant glaucoma occurred following trabeculectomy combining phacoemulsification with intraocular lens (IOL) implantation and goniosynechialysis. Subsequent anterior vitrectomy and irido-zonulo-hyaloid-vitrectomy (IZHV) effectively lowered IOP and deepened ACD, but the cystic cavity became larger. CONCLUSIONS: There is a potential risk of malignant glaucoma in ACG patients with XLRS after filtering surgery. Although anterior vitrectomy can effectively resolve aqueous misdirection, the macular retinoschisis may get worse. Awareness of this risk may aid in surgical planning and postoperative management in these patients.

Visual Acuity and Visual Field Changes in Patients with End-Stage PACG with Tubular Visual Field after Cataract Surgery.

INTRODUCTION: To investigate the impact of cataract surgery on visual acuity and visual field (VF) in patients with end-stage glaucoma with tubular VF, and assess the risk of severe visual impairment. METHODS: Retrospective analysis of the case data of patients with end-stage glaucoma with tubular VF who underwent cataract surgery in our hospital in the past 7 years. RESULTS: A total of 59 patients with 63 eyes were enrolled, 62 eyes were primary angle-closure glaucoma (PACG) and 1 eye was primary open-angle glaucoma. The last follow-up time was an average of 9 months, and no cases of severe vision loss occurred. Best corrected visual acuity (BCVA) improved significantly after surgery (0.57 ± 0.46 vs. 0.45 ± 0.43 logarithm of the minimum angle of resolution, p < 0.01), and there was a significant drop in intraocular pressure (IOP; 22.85 ± 9.7 vs. 16.07 ± 3.38, p < 0.01), a reduced number of glaucoma medications (2 ± 1.32 vs. 0.5 ± 1, p < 0.01), statistical improvement in VF index (VFI) and mean defect (MD) (12.3% ± 7.65% vs. 16.1% ± 9.84%, p < 0.01; -29.09 ± 2.16 vs. -28.31 ± 3.01, p < 0.01) after surgery. The higher the preoperative VFI and MD were, the better the postoperative BCVA (r = -0.387, r = -0.347, respectively). The degree of postoperative VFI improvement was significantly correlated with preoperative MD (r = 0.372, p < 0.01). During the follow-up period, 5 eyes (8%) underwent anti-glaucoma surgery due to elevated IOP. CONCLUSION: Cataract surgery can significantly improve visual acuity and VF in patients with end-stage PACG with tubular VF, and no patients have severe visual impairment. The less preoperative VF damage there is, the greater the postoperative visual acuity and VF improvement. Poor IOP control is the main cause of further damage to postoperative visual acuity and VF.

Ocular Characteristics of Pseudophakic Eyes with Malignant Glaucoma.

INTRODUCTION: The aim of the study was to investigate the clinical characteristics and treatment outcomes of the pseudophakic eyes with malignant glaucoma (MG). METHODS: This retrospective case-control study enrolled 53 eyes of 47 patients with primary angle-closure glaucoma having cataract surgery history, including 19 patients (25 eyes) diagnosed with MG and 28 patients (28 eyes) without MG as the match. Among patients diagnosed with MG, 14 patients (18 eyes) underwent zonulo-hyaloido-vitrectomy (ZHV) and the other 5 patients (7 eyes) received conservative treatments. The visual acuity, refraction status, intraocular pressure (IOP), extent of peripheral anterior synechia, classes of anti-glaucoma medications, and ultrasound biomicroscopy (UBM) examination were recorded before cataract surgery, at the diagnosis of MG, and 3 months after ZHV or atropine application, respectively. RESULTS: In the pseudophakic eyes with MG, the IOP cannot be well controlled compared to the matched eyes (27.24 ± 8.72 mm Hg vs. 14.30 ± 2.63 mm Hg, p < 0.001). In addition, there was a difference in the average spherical equivalent refractive error between 2 groups of patients (-2.23 ± 0.84 D in MG vs. -0.12 ± 0.64 D in the matched eyes, p < 0.001). By UBM analysis, the anterior chamber depth (ACD) was shallower in MG than that in the matched eyes (2.34 ± 0.20 mm vs. 3.47 ± 0.29 mm, p < 0.001). The difference between the anterior vault distance of the pseudophakic eyes with MG and that of the matched eyes was also significant (p < 0.001). After treated with ZHV, the IOP was greatly decreased from 27.84 ± 10.14 mm Hg to 15.85 ± 4.41 mm Hg (p < 0.001). The refractive error also changed from -2.11 ± 0.91 D to +0.42 ± 0.99 D (p < 0.001). At the same time, the central ACD was significantly deepened from 2.30 ± 0.39 mm to 3.30 ± 0.31 mm (p < 0.001). CONCLUSION: Uncontrolled IOP and shallow anterior chamber both centrally and peripherally are the primary clinical characteristics for the pseudophakic eyes with MG. An unexpected refractive error or myopic shift for the eyes with PACG after cataract surgery can be an important hint for diagnosis of MG. The typical UBM image is an anterior displacement of the lens-iris diaphragm and a bow-shaped change of the intraocular lens. It is an effective way to treat pseudophakic MG with the ZHV through a peripheral iridectomy.

Efficacy and safety of primary customized phacoemulsification combined with goniosynechialysis for refractory acute primary angle closure.

PURPOSE: To assess the safety, efficacy, and long-term clinical outcomes of primary customized phacoemulsification (phaco) combined with goniosynechialysis (GSL; phaco-GSL) in refractory acute primary angle closure (APAC) eyes with uncontrolled high intraocular pressure (IOP). METHODS: This retrospective case series comprised 51 eyes of 42 consecutive patients with refractory APAC and high IOP who were treated using primary customized phaco-GSL at 3 hospitals in China, from 2014 to 2021. Preoperative and postoperative IOP, corrected distant visual acuity (CDVA), corneal endothelial cell density (CECD), intraoperative and postoperative complications were recorded. The safety, efficacy and subsequent long-term clinical outcomes were analyzed. RESULTS: The mean CDVA (LogMAR) was improved from 1.67 ± 0.94 preoperatively to 0.23 ± 0.26 postoperatively (P < 0.001). Preoperative CECD was 2309.39 ± 541.03 cells/mm2 in 33 eyes and inaccessible in 18 eyes due to severe corneal edema; at the final follow-up, the mean CECD of all patients was 1823.50 ± 533.40 cells/mm2 (P < 0.001). The mean IOP decreased from 48.51 ± 6.25 mmHg preoperatively to 15.66 ± 2.27 mmHg at the final follow-up (P < 0.001). Among 51 eyes, additional customized procedures performed were corneal indentation in 42 eyes, epithelial debridement in 9 eyes, giant epithelial bullae view in 4 eyes, pars-plana fluid aspiration in 3 eyes, and secondary intraocular lens implantation in 7 eyes. The IOP of all eyes was well controlled eventually and 47 eyes (92.16%) were successfully treated by phaco-GSL alone. No significant intraoperative or postoperative complications were observed. CONCLUSIONS: Primary customized phaco-GSL is a safe and effective surgical management strategy for patients with refractory APAC and high IOP.

Branch retina vein occlusion combined with angle-closure glaucoma is associated with a mutation in BEST1: a case report.

BACKGROUND: It is rare for a patient to be diagnosed with branch retina vein occlusion (BRVO), angle-closure glaucoma (ACG) and autosomal recessive bestrophinopathy (ARB). ARB is strongly associated with ACG. Although glaucoma is a significant risk factor for RVO, there is a plausible relationship between ACG and BRVO. To discuss correlation of these diseases is necessary. CASE PRESENTATION: The genetic testing and medical treatment of a patient with ocular fundus diseases and ACG were recorded. We present a 47-year-old male patient with BRVO who was diagnosed with angle-closure glaucoma and a homozygous mutation of c.140G > A (p.R47H) in BEST1. Intravitreal ranibizumab was administered in combination with three antiglaucomatous eyedrops to lower intraocular pressure (IOP) in the right eye. One month later, BCVA improved to 0.3. IOP was controlled at 13 mmHg. CONCLUSIONS: ACG was likely combined to ARB, while there's a plausible relationship between ACG and BRVO.

Phacoemulsification, Intraocular Lens Implantation, Goniosynechialysis, and Pseudo-Pupilloplasty for Refractory Acute Primary Angle Closure with Atonic Dilated Pupil.

Objective To evaluate the efficacy and safety of prompt phacoemulsification, intraocular lens implantation, visco-goniosynechialysis, combined with pseudo-pupilloplasty for refractory acute primary angle closure (APAC) with atonic dilated pupil and to describe a feasible method of pupilloplasty. Methods A consecutive series of refractory APAC patients who had atonic dilated pupil and undergone prompt phacoemulsification combined with pseudo-pupilloplasty at our center were retrospectively analyzed. Pseudo-pupilloplasty referred to a method of pupilloplasty which included 4.5-mm capsulorhexis, postoperative opacification of anterior capsule residue, and ultimate pseudo-pupil formation. Preoperative and postoperative measurements included intraocular pressure (IOP), best corrected visual acuity (BCVA), and anterior chamber depth (ACD). Intraoperative and postoperative complications were documented. The process of pseudo-pupil formation was also observed. Results A total of 20 eyes of 19 APAC patients were followed up for 19.7 ± 9.8 months. IOP was lowered from preoperative 44.0 ± 9.8 mmHg to 15.5 ± 2.6 mmHg at final visit (t=11.945, P< 0.001). ACD was deepened from preoperative 1.77 ± 0.21 mm to 3.40 ± 0.20 mm at final visit (t=-27.711, P< 0.001). Twelve of 20 eyes had residual angle synechiae, whereas only 3 eyes needed anti-glaucoma medications. No severe complication was observed. All eyes had pseudo-pupil gradually formed within 3 months, accompanied with the gradual improvement of BCVA from preoperative 1.18 ± 0.55 to 0.58 ± 0.22, 0.26 ± 0.09, 0.11 ± 0.09, and 0.11 ± 0.09 at postoperative day 1, month 1, month 3, and last visit. Conclusions Prompt phacoemulsification-goniosynechialysis is effective and safe for refractory APAC with atonic dilated pupil. Pseudo-pupilloplasty is a feasible procedure for pupil reconstruction.

[A family with nanophthalmos caused by a TMEM98 gene variant].

A family with nanophthalmos was genetically investigated. The proband of this family was a 34-year-old male, who was diagnosed as binocular glaucoma two years ago and was admitted to Beijing Tongren Eye Center due to impaired vision in his right eye for 2 months. The intraocular pressure was controlled with two anti-glaucoma medications, the range of the angle closure was>300 degrees, and the cup-disk ratio was significantly increased in both eyes. The axial length of the right and left eyes was 17.45 mm and 17.36 mm, respectively. A heterozygous missense variant of the TMEM98 gene was detected by gene sequencing (C. 602G>C, P.R201p). Both eyes were diagnosed with nanophthalmos and secondary angle-closure glaucoma. The father of the proband was found to carry a homozygous variant in the same gene, while the daughter of the proband carried the same heterozygous variant as the proband. Both of them were diagnosed as binocular nanophthalmos in combination with clinical manifestations.

Evaluation of MYRF as a candidate gene for primary angle closure glaucoma.

PURPOSE: Primary angle-closure glaucoma (PACG) is a leading cause of blindness. Despite tremendous human effort and financial input, no definitive causative gene has been identified either through genome-wide association or Mendelian family studies. In the current study, novel candidate genes for PACG were investigated by studying the variants of nanophthalmos-associated genes. METHODS: A case-control study was conducted that included 45 PACG patients and 12 normal controls with short axial length (AL, less than 23.5 mm but more than 20.5 mm). Whole-exome sequencing (WES) was performed to screen the variants in previously identified nanophthalmos-associated genes, as well as other risk genes. RESULTS: The age range of the 45 PACG patients was 24 to 80 years, with an average AL of 21.87±0.65 mm (range: 20.54-23.45 mm) in the right eye and 21.89±0.64 mm (range 20.60-23.23 mm) in the left eye. Four novel myelin regulatory factor (MYRF) gene missense variants (p.G117S, p.H1057R, p.H230R, and p.R316C) were identified in four out of the 45 enrolled PACG patients, respectively. No MYRF or other nanophthalmos-associated gene variants were detected in the 12 normal controls. CONCLUSIONS: An appropriate approach was adopted to investigate the genetics of PACG through nanophthalmos-associated genes. A genetic variant, MYRF, was identified in four out of 45 PACG patients, which might be a novel candidate gene for PACG.

Impact of laser iridotomy on headache symptoms in angle-closure subjects.

BACKGROUND: Migraine symptoms are frequently referred by glaucoma patients. Although most studies analyze headache in the acute setting of angle closure, many patients with chronic occludable angles also complain of headaches. The aim of this study was to determine the impact of laser peripheral iridotomy (LPI) on the magnitude and frequency of headache symptoms in patients with occludable angles. METHODS: Prospective cohort study. Patients with indication for prophylactic LPI due to occludable iridocorneal angle were included. Headache symptoms were assessed before and at least 4 weeks after LPI using the Headache Impact Test-6 (HIT-6) questionnaire. A HIT-6 score of ≥ 50 points was labeled as a clinically significant headache. RESULTS: Thirty-one subjects were included. Prophylactic LPI was performed in 60 eyes, as 2 patients were pseudophakic in the fellow eye. Baseline HIT-6 score was 59.9 ± 11.8, with over three quarters of these patients scoring higher than 50 points (n = 24). A statistically significant reduction in HIT-6 score was found after LPI treatment (45.4 ± 7.7, p < 0.01). Sub-analysis within the clinically symptomatic subjects disclosed a significant improvement after treatment in this group (baseline, 65.3 ± 6.2 vs post-LPI 46.2 ± 8.3, p < 0.01), with a high baseline HIT-6 score being predictive of a symptomatic improvement after LPI (χ2(8) = 15.3, p = 0.001). This is mimicked from the patient's perspective, as the two subjective questions after LPI, concerning pain intensity and frequency, report that 79.2% had a statistically significant improvement of the headaches. CONCLUSIONS: Within our sample, the majority of patients with occludable angles had clinically relevant headaches. LPI provided symptomatic relief in the majority of those patients with high HIT-6 scores. Further studies are needed to explore the relationships between headache and angle anatomy.

Outcomes of effective goniosynechialysis under an endoscopic view combined with phacoemulsification in residual angle-closure glaucoma with lens opacity: 6-month results.

PURPOSE: We aimed to evaluate the efficacy and safety of effective goniosynechialysis (GSL) under an endoscopic view combined with phacoemulsification in residual angle-closure glaucoma with lens opacity. METHODS: This was a retrospective study. Patients with residual angle-closure glaucoma, lens opacity, and uncontrolled intraocular pressure (IOP) who were receiving anti-glaucoma medications were selected to undergo effective GSL under an endoscopic view combined with phacoemulsification. Follow-up examinations were conducted until 6 months postoperatively. RESULTS: Twenty-five eyes of 24 patients diagnosed with residual angle-closure glaucoma and lens opacity and peripheral anterior synechiae (PAS) at least ≥ 270° were included. Their mean age was 61.32 ± 6.11 years. Preoperatively, the mean (standard error) IOP was 29.69 (11.22) mmHg, and the median number of IOP-lowering medications used was 3.0. The decreases in the rates of IOP of the patients were 44.29%, 52.17%, 46.95%, 48.37%, and 47.29% at 1 day, 1 week, 1 month, 2.5 months, and 6 months after the surgery, respectively. At 6 months, the median number of IOP-lowering medications used decreased from 3 to 0 and the range of PAS compared to the baseline decreased from 312° to 107° (P < 0.001). We also found that 21/25 eyes achieved improved or stable visual acuity after surgery. Postoperative complications included transiently elevated IOP (12.0%), exudation (8.0%), and hyphema (4.0%). CONCLUSIONS: Phacoemulsification combined with effective GSL under an endoscopic view may reopen residual angle-closure glaucoma and reduce the number of IOP-lowering medications for up to 6 months. It is an effective and safe method for patients with residual angle-closure glaucoma and lens opacity.

Does TRABECULECTOMY meet the 10-10-10 challenge in PACG, POAG, JOAG and Secondary glaucomas?

PURPOSE: Evaluation of the intraocular pressure (IOP) lowering efficacy of trabeculectomies over > 10 years and their ability to stabilize glaucomatous optic neuropathy. METHODS: In total, 181 eyes (136 patients), which underwent trabeculectomy (10 min surgery) at least 10 years prior and were on regular follow-up, were evaluated. Qualified/complete success was taken as criteria A: IOP ≤ 12 mmHg, B: IOP ≤ 15 mmHg and C: IOP ≤ 18 mmHg, with all > 5 mmHg, with/without medications. Target IOP in advanced glaucoma is about 10 mmHg, therefore trabeculectomy 10-10-10 challenge! RESULTS: The mean age of patients was 46.32 ± 11.50 years. Absolute success was 50.27%, 54.14% and 59.66% according to criteria A, B and C at last follow-up, while qualified success was 70.11%, 81.77% and 96.13%. An IOP of ≤ 12 mmHg was noted in 34, 64.15%, PACG eyes, 14, 73.68%, POAG, 15, 65.22%, JOAG and 64, 74.42%, secondary glaucoma eyes. The reduction in IOP overall was 64.83 ± 16.80% at last review and was 59.47 ± 16.07% in PACG, 62.40 ± 17.72% in POAG, 71.89 ± 8.50% in JOAG and 67.74 ± 18.10% in secondary glaucoma eyes. "Target" IOP was achieved in 97.29% of early glaucoma, 85.71% moderate glaucoma and 70% severe glaucoma eyes. 97.24% of patients were perimetrically stable. 2.21% of eyes post-trabeculectomy had a shallow anterior chamber needing surgical intervention. Visual acuity was maintained or better in 93.92% of patients, with a cataract surgery performed in 6.63% eyes. A repeat trabeculectomy was performed in 3.31% of eyes. CONCLUSION: Trabeculectomy 10-10-10 is achievable in the long term, with few complications or repeat surgical interventions in the majority of POAG, PACG, JOAG and secondary glaucomas. Therefore, trabeculectomy should not be relegated to a last resort, but should be undertaken as soon as possible, if medical therapy is inadequate, unaffordable or compliance is an issue.

A new rhodopsin R135W mutation induces endoplasmic reticulum stress and apoptosis in retinal pigment epithelial cells.

Rhodopsin mutations are associated with the autosomal-dominant form of retinitis pigmentosa (RP). Here we report simultaneous occurrence of RP associated with bilateral nanophthalmos and acute angle-closure glaucoma in patient with a new mutation in rhodopsin (R135W). ARPE-19 cells were transfected with myc-tagged wild-type (WT) and R135W rhodopsin constructs. The half-life of WT and R135W rhodopsin was analyzed via cycloheximide chase analysis. We found that R135W rhodopsin was accumulated in the endoplasmic reticulum (ER) and induced unfolded protein response (UPR) and apoptosis. Moreover, chaperone HSP70 alleviated ER stress and prevented apoptosis induced by R135W rhodopsin by attenuating UPR signaling. These findings reveal the novel pathogenic mechanism of RP and suggest that chaperone HSP70 has potential therapeutic significance for RP.

Autosomal dominant nanophthalmos and high hyperopia associated with a C-terminal frameshift variant in MYRF.

Purpose: Nanophthalmos is a rare subtype of microphthalmia associated with high hyperopia and an increased risk of angle-closure glaucoma. We investigated the genetic cause of nanophthalmos and high hyperopia in an autosomal dominant kindred. Methods: A proband with short axial length, high hyperopia, and dextrocardia was subjected to exome sequencing. Human and rodent gene expression data sets were used to investigate the expression of relevant genes. Results: We identified a segregating heterozygous frameshift variant at the 3' end of the penultimate exon of MYRF. Using Myc-MYRF chromatin immunoprecipitation data from rat oligodendrocytes, MYRF was found to bind immediately upstream of the transcriptional start site of Tmem98, a gene that itself has been implicated in autosomal dominant nanophthalmos. MYRF and TMEM98 were found to be expressed in the human retina, with a similar pattern of expression across several dissected human eye tissues. Conclusions: C-terminal variants in MYRF, which are expected to escape nonsense-mediated decay, represent a rare cause of autosomal dominant nanophthalmos with or without dextrocardia or congenital diaphragmatic hernia.