Phacogoniotomy versus phacotrabeculectomy for advanced primary angle-closure glaucoma with cataract: A randomized non-inferiority trial.

PURPOSE: To investigate the effectiveness and safety of phacogoniotomy versus phacotrabeculectomy (PVP) among patients with advanced primary angle-closure glaucoma (PACG) and cataracts. DESIGN: Multicenter, randomized controlled, non-inferiority trial. METHODS: A total of 124 patients (124 eyes) with advanced PACG and cataracts were enrolled, with 65 in the phacogoniotomy group and 59 in the phacotrabeculectomy group. Patients were followed up for 12 months with standardized evaluations. The primary outcome was the reduction in intraocular pressure (IOP) from baseline to 12 months postoperatively, of which a non-inferiority margin of 4 mmHg was evaluated. Secondary outcomes included the cumulative surgical success rate, postoperative complications, and changes in the number of glaucoma medications. RESULTS: After 12 months, phacogoniotomy demonstrated non-inferiority to phacotrabeculectomy in terms of IOP reduction, with mean IOP reductions of - 26.1 mmHg and - 25.7 mmHg (P = 0.383), respectively, from baseline values of around 40 mmHg. Both groups experienced a significant reduction in the mean number of medications used postoperatively (P < 0.001). The cumulative success rate was comparable between the groups (P = 0.890). However, phacogoniotomy had a lower rate of postoperative complications and interventions (12.3% and 4.6%) compared to phacotrabeculectomy (23.7% and 20.3% respectively). The phacogoniotomy group reported shorter surgery time (22.1 ± 6.5 vs. 38.8 ± 11.1 min; P = 0.030) and higher quality of life (EQ-5D-5 L) improvement at 12 months (7.0 ± 11.5 vs. 3.0 ± 12.9, P = 0.010) than the phacotrabeculectomy group. CONCLUSIONS: Phacogoniotomy was non-inferior to phacotrabeculectomy in terms of IOP reduction for advanced PACG and cataracts. Additionally, phacogoniotomy provided a shorter surgical time, lower postoperative complication rate, fewer postoperative interventions, and better postoperative quality of life.

Phacoemulsification combined with goniosynechialysis versus phacoemulsification alone for patients with primary angle­closure disease: A meta-analysis.

This meta-analysis aims to systematically compare the efficacy between phacoemulsification (PE) combined with goniosynechialysis (GSL) and PE alone for primary angle-closure disease (PACD) patients. All the data were searched from the PubMed, EMBASE and the Cochrane Library. The Cochrane Handbook was used to evaluate the quality of the included studies. Additionally, this meta-analysis was performed by using the Revman 5.4 software. Nine randomized controlled trials (RCTs) were included in this study. Compared with PE alone group, PE+GSL could result significant reduction in the IOP (MD, 1.81; p = 0.002). In the instrumental subgroup, also more reduction of IOP was shown in the PE+GSL group (MD, 2.11; p = 0.02). In the viscogonioplasty (VGP) subgroup, there was not no statistical difference between PE alone group and PE+GSL group (MD, 1.53; p = 0.11). Also, more reduction of peripheral anterior synechiae (PAS) was shown in the PE+GSL group (MD,59.15; p<0.00001). For the change in angle open distance (AOD)500, AOD 750, trabecular-iris space (TISA)500, number of glaucoma medications and best corrected visual acuity (BCVA), there was no difference between two groups (p = 0.25, 0.35, 0.17, 0.56, 0.08). For TISA 750, more improvement was shown in the PE+GSL group (p<0.00001). Instrumental separation had better effect on lowering IOP when it combined with PE. Both instrumental separation and VGP could reduce postoperative PAS. The operation of GSL has no obvious effect on postoperative vision.

Prevalence and Risk Factors of Blindness Among Primary Angle Closure Glaucoma Patients in the United States: An IRIS Registry Analysis.

PURPOSE: To assess the prevalence and risk factors of blindness among patients newly diagnosed with primary angle closure glaucoma (PACG) in the United States. DESIGN: Retrospective cross-sectional study. METHODS: Eligible patients from the American Academy of Ophthalmology (AAO) Intelligent Research in Sight (IRIS) Registry had newly diagnosed PACG, defined as: 1) observable during a 24-month lookback period from index date of PACG diagnosis; 2) no history of eye drops, laser, or cataract surgery unless preceded by a diagnosis of anatomical narrow angle (ANA); and 3) no history of glaucoma surgery. Logistic regression models were developed to identify risk factors for any (one or both eyes) or bilateral (both eyes) blindness (visual acuity ≤20/200) at first diagnosis of PACG. RESULTS: Among 43,901 eligible patients, overall prevalence of any and bilateral blindness were 11.5% and 1.8%, respectively. Black and Hispanic patients were at higher risk of any (odds ratios [ORs] 1.42 and 1.21, respectively; P < .001) and bilateral (ORs 2.04 and 1.53, respectively; P < .001) blindness compared with non-Hispanic White patients adjusted for ocular comorbidities. Age <50 or >80 years, male sex, Medicaid or Medicare insurance product, and Southern or Western practice region also conferred a higher risk of blindness (OR > 1.28; P ≤ .01). CONCLUSIONS: Blindness affects 1 of 9 patients with newly diagnosed PACG in the IRIS Registry. Black and Hispanic patients and Medicaid and Medicare recipients are at significantly higher risk. These findings highlight the severe ocular morbidity among patients with PACG and the need for improved disease awareness and detection methods.

Outcomes of Filtering Surgery Versus Clear Lens Extraction in Young Patients With Angle-Closure Glaucoma.

PURPOSE: To compare the effect of filtering surgery versus clear lens extraction in young patients with medically uncontrolled angle-closure glaucoma (ACG). DESIGN: Retrospective, nonrandomized, comparative, interventional study. METHODS: We reviewed the medical charts of patients with the following scenarios: (1) age ≤40 years; (2) diagnosis of ACG without cataract, including primary angle-closure glaucoma (PACG), nanophthalmic ACG, and ACG combined with retinal dystrophies; and (3) ACG undergoing filtering surgery or clear lens extraction. The main outcomes including intraocular pressure (IOP), number of medications, best-corrected visual acuity, and severe complications were extracted at the postoperative early (within 1 week) and late stage (>3 months) follow-up. RESULTS: Data from 160 eyes of 130 young patients with ACG were available. Eyes with 76 PACG, 12 nanophthalmic ACG, and 26 ACG with retinal diseases underwent filtering surgery, whereas eyes with 22 PACG, 12 nanophthalmic ACG, and 12 ACG with retinal diseases received clear lens extraction. Overall, filtering surgery and clear lens extraction resulted in significant but comparable IOP and drug reductions at the postoperative late stage in each ACG subgroup, with similar complete success rates between 2 treatments (all P > .05). Regarding the safety, filtering surgery and patients with retinal diseases were independent factors associated with postoperative malignant glaucoma (P < .05 in both multivariable logistic regression models). CONCLUSIONS: This study highlights that the efficacy of clear lens extraction is comparable to that of filtering surgery in medically uncontrolled ACG in young patients, but clear lens extraction is safer, especially for young patients with ACG comorbid with retinal diseases.

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.

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.

Comparative study of surgical outcomes of twin-site phacotrabeculectomy with mitomycin C in primary open-angle glaucoma versus primary angle-closure glaucoma.

Purpose: To compare the surgical outcomes of twin-site phacotrabeculectomy with Mitomycin C (MMC) in primary open-angle glaucoma (POAG) versus primary angle-closure glaucoma (PACG). Methods: Prospective, comparative, observational study. Patients with visually significant cataract and primary glaucoma were divided into two groups: POAG and PACG. They underwent twin-site phacotrabeculectomy with MMC and followed up on days 1, 15, 1 month, 6 months, and 12 months. Baseline and follow-up visits were compared to find the differences in intraocular pressure (IOP), antiglaucoma medications (AGM), success rates, anterior chamber depth (ACD), and axial length (AXL). Results: There were 50 eyes each in POAG and PACG groups. Mean IOP reduction from baseline to 12 months (21.22 ± 6.0 to 11.40 ± 2.8-POAG group vs 24.16 ± 7.6 to 12.42 ± 3.2-PACG group) was statistically significant in both groups (P < 0.001), but no significant difference between groups (P = 0.095). There was a statistically significant decline in the number of AGM in POAG [1.66 (0.7) to 0.38 (0.7), P < 0.001] and PACG [2.10 (0.7) to 0.70 (0.8), P < 0.001]; the decline was more in POAG (P = 0.012) at last visit. Probability of overall (complete and qualified) success at 12 months postop was 72.0% [95% confidence interval (CI): 57.4-82.4] in PACG and 84.0% (95% CI: 70.5-91.7) in POAG group. There was a significant increase in ACD and a decrease in AXL in both groups (P < 0.001). More interventions were required in the PACG group (38, P = 0.012). Conclusion: Phacotrabeculectomy with MMC causes a significant reduction in IOP and improvement in biometric parameters in both POAG and PACG. Patients with PACG required more postoperative interventions, while a lesser number of antiglaucoma medications were needed in POAG patients.

Clinical update in nanophthalmos: Features, diseases and complications associated.

Nanophthalmos is a rare congenital condition of the eyeball that is characterised by a smaller size of the anterior and posterior segments without associated ocular malformations. Typical features that have traditionally been described in these eyes are short axial length, thickened sclera, cornea with a smaller diameter, narrow anterior chamber, and an increased lens to globe volume ratio. However, at present, there is still a lack of recognised diagnostic criteria for nanophthalmos and a classification of its severity. Its clinical relevance stems from the increased risk of multiple ocular conditions, such as high hyperopia, amblyopia, angle-closure glaucoma, retinal detachment, and cataracts. Likewise, in relation to surgery in these eyes, there are particularities in cataract and glaucoma surgery and with a greater risk of associated intra- and postoperative complications. In this way, the treatment of nanophthalmos focuses on controlling the associated eye conditions and reducing and controlling surgical complications. This review aims to update what has been published in recent years regarding nanophthalmos.

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.

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.

Glauco-Claw: A novice implant for chronic angle-closure glaucoma.

This article describes 1-year outcomes of a new intra-ocular implant, "Glauco-Claw," in refractory chronic angle-closure glaucoma (ACG). Glauco-Claw is a novice polymethylmethacrylate implant with a central ring and five claws placed circumferentially. It was placed in the anterior chamber and the peripheral iris was tucked into the claws, thus causing goniosynechialysis and preventing the reformation of goniosynechiae. It was implanted in five eyes of five patients, and they were followed up for 1 year. Target intra-ocular pressure was achieved and maintained in all the patients till the last follow-up. Two patients did not require any anti-glaucoma medication. No significant complications were observed in any patient. Glauco-Claw could be another armamentarium in the management of refractory chronic ACG.

Zonulopathy Identified During Cataract Extraction in Patients With Primary Angle Closure Disease.

PRCIS: The proportion, clinical characteristics, and risk factors of zonulopathy in primary angle closure disease (PACD) were analyzed. Zonulopathy is an underrecognized common finding in PACD, especially in patients with acute angle closure (AAC). PURPOSE: To examine the proportion and risk factors associated with intraoperative zonulopathy in PACD. PATIENTS AND METHODS: This is a retrospective analysis of 88 consecutive patients with PACD who underwent bilateral cataract extraction at Beijing Tongren Hospital from August 1, 2020 to August 1, 2022. Zonulopathy was diagnosed based on intraoperative findings including the presence of a lens equator, radial folds of the anterior capsule while making capsulorhexis, and other signs of the unstable capsular bag. The subjects were grouped based on their PACD subtype diagnoses: AAC, primary angle closure glaucoma (PACG), primary angle closure (PAC), or primary angle closure suspect (PACS). Multivariate logistic regression was performed to identify risk factors associated with zonulopathy. The proportion and the risk factors of zonulopathy were estimated in patients with PACD and in PACD subtypes. RESULTS: Of 88 patients with PACD (67.3 ± 6.9 y old, 19 males and 69 females), the overall proportion of zonulopathy was 45.5% of patients (40/88) and 30.1% of eyes (53/176). Among the PACD subtypes, the proportion of zonulopathy was highest (69.0%) in AAC, followed by 39.1% in PACG, and 15.3% in PAC and PACS combined. AAC was an independent risk factor associated with zonulopathy ( P = 0.015, AAC vs PACG, PAC, and PACS combined; odds ratio: 0.340, CI: 0.142-0.814). Shallower anterior chamber depth ( P = 0.031) and greater lens thickness ( P = 0.036), but not laser iridotomy, were associated with an increased proportion of zonulopathy. CONCLUSIONS: Zonulopathy is common in PACD, especially in patients with AAC. Shallow anterior chamber depth and thick lens thickness were associated with an increased proportion of zonulopathy.

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.

Unilateral chronic angle-closure glaucoma in a pediatric patient with neurofibromatosis: a case report.

Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disease that causes multi-system damage. It is rarely associated with angle-closure glaucoma, especially in pediatric patients. We herein report a case of unilateral chronic angle-closure glaucoma in a patient with NF1. A 5-year-old girl with a large subcutaneous soft mass and multiple scattered coffee-milk spots presented with low vision, increased intraocular pressure, and angle closure in her right eye. Lisch nodules were seen in both eyes. In her right eye, ectropion uveae was observed at the top and bottom margins of the pupil. Magnetic resonance imaging of the skull and orbit revealed no abnormalities. Finally, trabeculectomy was performed on the right eye, after which the right eye showed a stable intraocular pressure. NF1 combined with angle-closure glaucoma is rare and easily missed in the clinical setting. Early diagnosis and treatment may achieve good results.

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.

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.

Zonule-Associated Gene Variants in Isolated Ectopia Lentis and Glaucoma.

PRCIS: We report 3 novel variants in fibrillin-1 (FBN1) and latent transforming growth factor-ß-binding protein 2 (LTBP2) in 3 families with isolated ectopia lentis (EL), which shed new light on the diagnosis and genetic counseling of EL and secondary glaucoma in clinical settings. PURPOSE: To explore the genetic mechanism in 3 families with isolated EL and secondary angle closure glaucoma. METHODS: Three Han Chinese families with EL and glaucoma were recruited. All of the participants underwent complete ocular and general physical examinations and DNA samples were extracted from peripheral venous blood and screened for disease-causing variants using whole exome and Sanger sequencing. In silico analyses were performed to predict the structural and functional changes in gene variants and abnormal proteins. RESULTS: All 3 probands presented with EL and pupillary-blocking glaucoma. Genetic testing showed that all the patients have zonule-related gene mutations, with the proband (II:1), as well as his mother (I:2) and daughters (III:1 and III:2) from family 1 carrying a heterozygous mutation in FBN1 gene (c.6493G>T:p.(V2165L)); the proband (II:1) from family 2 carrying a heterozygous mutation in FBN1 gene (c.2543C>A:p.(T848N)), and the proband (II:1) from family 3 carrying a pair of compound heterozygous mutations in LTBP2 gene (c.4825T>A:p.(C1609S) / c.529T>C:p.(W177R)). No other genetic variants were found to be associated with the phenotypes of patients and other family members in this study. All variants are predicted to affect the structure and function of proteins as risk factors for EL based on bioinformatics analysis. CONCLUSION: Four novel mutations were identified in 3 families with EL, suggesting an intimate link between specific mutations in FBN1 and LTBP2 and isolated EL and angle closure glaucoma. Our results expanded the variant spectrum of zonule-related genes and helped explore the underlying molecular pathology of these disorders.

Morphometric Assessment of the Ciliary Body in Patients With Marfan Syndrome and Ectopia Lentis: A Quantitative Study Using Ultrasound Biomicroscopy.

PURPOSE: To explore the biometric characteristics of the ciliary body in patients with Marfan syndrome (MFS) and ectopia lentis (EL). DESIGN: Cross-sectional study. METHODS: Seventy-two consecutive patients with MFS and EL and 72 nondiseased control subjects were recruited. Ciliary body biometric parameters such as ciliary muscle cross-sectional area at 2000 µm from the scleral spur (CMA2000), ciliary muscle thickness at 1000 µm from the scleral spur (CMT1000), and maximum ciliary body thickness (CBTmax) were measured from multiple directions with ultrasound biomicroscopy (UBM). The relationship between ciliary body parameters and other ocular characteristics was also evaluated. RESULTS: Average CMA2000, CMT1000, and CBTmax were 0.692 ± 0.015 mm2, 0.405 ± 0.010 mm, and 0.855 ± 0.023 mm in eyes of patients with MFS, respectively, and were significantly smaller than these values in control subjects (all P < .001). The prevalence of ciliary body thinning was 22.2% in the MFS group vs 0 in the control group (P < .001); eyes with more severe EL had smaller CMA2000 (P = .050), thinner CMT1000 (P = .022), and shorter CBTmax (P = .015). Patients with microspherophakia (MSP) had even smaller CMA2000 (P = .033) and CMT1000 (P = .044) than those without MSP. The most common subluxation direction was in the superonasal quadrant (n = 25; 39.7%), which probably correlates with the thinnest CMT1000 in the inferotemporal quadrant (P = .005). CONCLUSIONS: Patients with MFS and EL had thinner ciliary muscles, shorter ciliary processes, and a higher prevalence of ciliary body thinning, especially those with MSP. Both the extent and direction of subluxation were associated with ciliary body biometry..

Penetrating canaloplasty in angle-closure glaucoma secondary to iridocorneal endothelial syndrome following multiple failed filtering surgeries: A case report.

RATIONALE: Angle-closure glaucoma secondary to iridocorneal endothelial syndrome (ICE) is challenging to treat, especially in patients who have already undergone multiple surgical procedures. Long-term success is difficult to achieve with traditional filtration surgery again. This case report describes a novel nonbleb-dependent surgery for managing such a young patient. PATIENT CONCERNS: A 30-year-old male with glaucoma secondary to ICE was referred to West China Hospital, Sichuan University for uncontrolled intraocular pressure following multiple failed filtering surgeries under maximum topical antiglaucoma medications in his right eye. DIAGNOSES: The patient was diagnosed with angle-closure glaucoma secondary to ICE in the right eye based on a series of ophthalmic examinations. INTERVENTIONS: Penetrating canaloplasty was performed to manage glaucoma secondary to ICE in the right eye. OUTCOMES: The patient's visual acuity improved, the intraocular pressure was reduced to 11 to 15 mm Hg through 30 months of follow-up, and no antiglaucoma medication or additional surgical procedures were needed. LESSONS: Penetrating canaloplasty could be considered as an option for the treatment of refractory angle-closure glaucoma secondary to ICE with extensive angle adhesion.

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.