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INTRODUCTION: Mutations in BEST1 gene have been linked to the development of refractory angle closure glaucoma (ACG). This study aims to delineate the clinical characteristics, genetic mutations, and disease progression in patients with autosomal recessive bestrophinopathy (ARB) and autosomal dominant Best vitelliform macular dystrophy (BVMD) who are presented with treatment-resistant ACG. METHODS: This retrospective analysis encompasses a comprehensive ophthalmic assessment, retinal imaging, and mutational profiling of six patients diagnosed with bestrophinopathy and concurrent ACG, with a particular emphasis on the risk of post-glaucoma filtration surgery malignant glaucoma (MG). Exome sequencing was conducted utilizing a next-generation sequencing (NGS) based gene panel. RESULTS: The cohort included five patients with ARB and one with BVMD, with a mean (±SD) age at ACG diagnosis of 35.1 ± 6.9 years. NGS analysis revealed homozygous BEST1 variants in four patients (ARB; cases 1-4) and a heterozygous BEST1 variant in one patient (BVMD; case 5). One patient (ARB; case 6), despite a recessive pedigree, showed a single heterozygous variant, suggesting the presence of an undetected heterozygous variant indicative of compound heterozygous autosomal recessive inheritance. A novel non-frameshift deletion (c.841_843delTTC; p.Phe281del) was identified in case 2. Surgical intervention was required due to uncontrolled glaucoma in all cases except case 4. All five cases that underwent glaucoma filtration surgery developed MG, which was effectively managed with combined iridozonulo-hyaloido-vitrectomy (IZHV) and pars plana vitrectomy (PPV). Cases 5 and 6, harboring a heterozygous pathogenic variant (c.241 G>A; p.Val81Met), experienced refractory MG and corneal decompensation necessitating multiple interventions. CONCLUSION: Genomic analysis plays a pivotal role in the management of bestrophinopathies with ACG. Characterization of mutational types facilitates prognostication and enables timely interventions. IZHV with PPV emerges as a promising standalone or adjunctive procedure for the management of glaucoma among patients with BEST1 mutations and ACG.

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AIM: To report a one-year clinical outcomes of low-dose laser cycloplasty (LCP) among malignant glaucoma patients. METHODS: In this prospective, multicenter, non-comparative clinical study, participants with malignant glaucoma were recruited and underwent LCP at eight ophthalmic centers in China. Patients were followed up at 1wk, 1, 3, 6, and 12mo. Intraocular pressure (IOP), number of glaucoma medications, anterior chamber depth (ACD), and complications were recorded. Anatomical success was defined as the reformation of the anterior chamber based on slit-lamp biomicroscopy. Recurrence was defined by the presence of a shallow or flat anterior chamber after initial recovery from treatment. RESULTS: A total of 34 eyes received LCP. Mean IOP and medications decreased from 36.1±11.5 mm Hg with 3.3±1.5 glaucoma medications pre-treatment to 20.9±9.8 mm Hg (P<0.001) with 2.9±1.6 medications (P=0.046) at 1d, and 17.4±6.7 mm Hg (P<0.001) with 1.3±1.7 medications (P<0.001) at 12mo. The ACD increased from 1.1±0.8 mm at baseline to 1.7±1.0 mm and to 2.0±0.5 mm at 1d and 12mo, respectively. A total of 32 (94.1%) eyes achieved initial anatomical success. During follow-up, 2 (5.9%) eyes failed and 8 (23.5%) eyes relapsed, yielding a 12-month anatomical success rate of 64.3%. Complications including anterior synechia (8.82%), choroidal/ciliary detachment (5.88%) and hypopyon (2.94%) were observed within 1wk. CONCLUSION: LCP is simple, safe, and effective in reforming the anterior chamber in malignant glaucoma.

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PURPOSE: To evaluate the outcomes of postoperative aqueous misdirection and factors predicting failure of interventions. METHODS: This retrospective study included 49 eyes from 47 patients with aqueous misdirection following glaucoma or cataract surgery. Resolution of aqueous misdirection (AM) was deepening of the central anterior chamber (AC) and intraocular pressure (IOP) ≤ 21 mmHg. The Cox proportional hazards regression model was used to evaluate risk factors for failure of various treatments. RESULTS: 10/49 eyes (20%) resolved with conservative management, and 39/49 eyes (80%) needed multiple intervention, of which 95% (37/39) eyes achieved resolution of aqueous misdirection. Pseudophakia predicted the need for multiple interventions with a hazard ratio of 2.391 (1.158-4.935), p = 0.02). Among the risk factors assessed for resolution of AM, longer axial length (HR: 0.61 (0.414-0.891), p < 0.01) and eyes with prior glaucoma surgery predicted resolution (HR: 0.142 (0.027-0.741), p < 0.01) and delayed presentation predicted failure (HR: 1.002 (1.0002-1.0031), p < 0.02). CONCLUSION: Pseudophakic eyes were more refractory and predicted the need for multiple interventions. Eyes with prior glaucoma surgery and those with longer axial length had achieved resolution faster, and delayed presentation was a risk factor for failure to resolve.

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BACKGROUND: The importance of communicating the anterior chamber and vitreous cavity for managing malignant glaucoma (MG) is widely recognized. This study investigated the impact of improved minimally invasive anterior vitrectomy (IAV) on the prognosis of MG. METHODS: This retrospective interventional study included patients with MG who underwent conventional surgery or improved minimally IAV in Nanchang Aier Eye Hospital between January 2011 and April 2021. For the improved step, a small amount of triamcinolone acetonide was injected into the vicinity of the iris. Then, the residual vitreous body adhering to triamcinolone acetonide was excised. Comparisons were made using repeated measures ANOVA, t-test, and chi-squared test. RESULTS: Thirty-one eyes from 26 patients were included: 15 eyes from 13 patients in the conventional group and 16 eyes from 13 patients in the IAV group. The 1-week, 1-month, and 3-month intraocular pressure (IOP) and the 3-month mean central anterior chamber depth were comparable between the two groups (all P > 0.05). The conventional group showed one eye with intraoperative vitreous hemorrhage and two eyes with postoperative re-shallowing of the anterior chamber; such events did not occur in the IAV group, and none developed corneal endothelial decompensation, IOL deviation, suprachoroidal hemorrhage, or retinal detachment during treatment and follow-up. CONCLUSION: Patients with MG who undergo improved minimally IAV might have similar postoperative IOP and central anterior chamber depth compared with conventional surgery but with reduced complications such as intraoperative vitreous hemorrhage and postoperative re-shallowing of the anterior chamber. Improved minimally IAV might be an alternative surgery for MG.

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PUROPSE: Aqueous misdirection syndrome (AMS) is an aggressive post-operative glaucoma unresponsive to conventional measures with grave outcomes. In this report, we describe a rare case of AMS following silicon oil removal in a vitrectomized eye. METHODS: A diabetic patient with tractional retinal detachment underwent pars plana vitrectomy with silicon oil injection. Following retinal reattachment, silicon oil removal was performed at three months. Post oil removal she developed increased intraocular pressure with shallowing of both the peripheral and central anterior chamber suggestive of AMS. RESULTS: Initial medical management with anti-glaucoma medications and cycloplegics was not beneficial. A pars plana lensectomy with complete anterior hyaloidectomy along with a surgical peripheral iridectomy helped relieve the aqueous misdirection. CONCLUSION: AMS can rarely occur following vitrectomy and is likely secondary to intact anterior hyaloid. Lensectomy along with zonulo-hyaloido-iridectomy is essential. This report highlights the occurrence of this rare complication and its effective management.

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PURPOSE: To assess the efficacy and safety of pars plana vitrectomy with irido-zonulo-hyaloidotomy (IZH) for fluid misdirection syndrome (FMS) in pseudophakic eyes. METHODS: This was a retrospective case series study of patients treated with pars plana vitrectomy with IZH for FMS between February 2017 and March 2020. Complete success was defined as central anterior chamber (AC) deepening with an intraocular pressure (IOP) of 21mmHg or less (on 2 consecutive visits at least 1 week apart) without topical or systemic glaucoma medications. Qualified success was defined as central AC deepening with an IOP of 21mmHg or less (on 2 consecutive visits at least 1 week apart) with topical or systemic glaucoma medications. RESULTS: Twelve eyes of 12 patients with a diagnosis of FMS were included. The mean age of the population was 73.6±15.4 years [39-90] with a majority of women (58.3%). Prior surgeries at the time of FMS diagnosis were trabeculectomy (4 eyes) and non-perforating deep sclerectomy (2 eyes). At presentation, mean IOP was 38.2±9.8mmHg, which decreased to 17.9±7.7mmHg (P<0.0001) at final follow-up (mean follow-up of 4.9±4.3 months). Complete success was achieved in 6 eyes (50%) and qualified success in 10 eyes (83%), with two eyes failing treatment. There was no statistical significant relationship between demographic data and clinical success (P > 0.05). CONCLUSION: Pars plana vitrectomy combined with IZH appears to be a safe and effective technique for the treatment of FMS in pseudophakic patients.

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INTRODUCTION: The use of the neodymium:yttrium-aluminum-garnet (Nd:YAG) laser to treat malignant glaucoma (MG) has been described in the literature since the 1980s. However, the technique has been reported to have a short-term effect, with a notable relapse rate. In the present study, we report the efficacy and durability of a modified Nd:YAG laser treatment methodology for treatment of pseudophakic or aphakic MG. METHODS: Patients with chronic angle-closure glaucoma and deemed at high risk of developing post-operative MG received prophylactic peripheral iridectomy during their conventional operation beginning in 2017. When the diagnosis of pseudophakic or aphakic MG was confirmed, a thorough Nd:YAG laser capsulo/zonulo-hyaloido-vitreolysis (CZHV) was performed through iridectomy, along with standardized pre- and post-laser medications. This retrospective case series includes 14 eyes of 11 patients with MG who had surgical preset iridectomy and modified Nd:YAG laser CZHV between 2017 and 2022. Outcome measures included resolution and recurrence of MG and incidence of treatment complications. RESULTS: The mean follow-up was 27.1 ± 15.0 months (range, 12-48). Long-term resolution of MG was obtained in all included eyes at the end of the follow-up. Six eyes (42.9%) achieved long-term resolution with a single Nd:YAG laser intervention. Eight eyes (57.1%) achieved long-term resolution following two to three laser interventions, with two eyes (14.3%) experiencing recurrence. There was no complication during the follow-up. At the final visit, a significant reduction (P = 0.0001) in the mean intraocular pressure (IOP) was observed (13.1 ± 2.8 mmHg) compared to presentation (21.4 ± 6.3 mmHg). CONCLUSION: The modified Nd:YAG laser treatment methodology is a minimally invasive option to manage pseudophakic or aphakic MG with sustained effectiveness. Reduced inflammatory reactions due to prophylactic peripheral iridectomy, rapid diagnosis, and timely treatment initiation have all contributed to the favorable outcomes associated with this modified treatment methodology.

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PURPOSE: To report the contributing factors to the successful long-term treatment outcomes of a large series of patients with malignant glaucoma (MG). DESIGN: Retrospective, interventional, consecutive case series. PARTICIPANTS: This study used data collected from 1997 to 2022 from the Glaucoma Division of the Stein Eye Institute, University of California, Los Angeles (UCLA). All patients with MG who underwent treatment at UCLA were enrolled. METHODS: The following demographic and clinical data were collected and analyzed for their relevance to successful treatment: age, gender, ethnicity, glaucoma family history, visual acuity (VA), intraocular pressure (IOP), lens status, prior glaucoma diagnosis, prior ocular surgery, prior use of antiglaucoma agents, ultrasonic axial length, qualitative anterior chamber (AC) depth, and treatment methods and outcomes. MAIN OUTCOME MEASURES: Anatomical success was defined as restoration of normal AC depth, indicating relief of the MG episode. Complete success was defined as anatomical success and the reduction of IOP to < 21 mmHg without further surgery, with or without medications. RESULTS: A total of 74 eyes of 73 patients were identified with a diagnosis of MG. The median (interquartile range) age of the patients at the time of MG presentation was 70 years (19.5) and 49 (75.4%) patients were female. The most common prior diagnosis before MG was primary angle closure glaucoma (PACG) (34 eyes, 51.5%). The initiating event for 30 eyes (45.5%) was glaucoma surgery and for 21 eyes (31.8%) was cataract surgery. Most eyes were pseudophakic (57, 86.4%). Fifty-six eyes underwent medical treatment; MG resolved in 2 eyes with medical treatment alone. Nine eyes (7 eyes = treatment naïve; 2 eyes = failed medical treatment) underwent laser treatment and MG resolved in 5 eyes. Among the 55 eyes which had surgical treatment, 52 eyes failed medical treatment and 3 eyes were treatment naïve. The anatomical success rate with surgical treatment was 96.4% and the most commonly performed surgical procedure was combined pars plana antero-central vitrectomy, hyaloido-zonulectomy, and iridectomy. CONCLUSIONS: Female gender, PACG, and glaucoma surgery were predisposing factors for the development of MG. Medical treatment alone for MG was inadequate in the vast majority of cases. A surgical technique consisting of combined pars plana antero-central vitrectomy, hyaloido-zonulectomy and iridectomy consistently produced high long-term success. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

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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.

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OBJECTIVE: To evaluate the surgical outcomes of anterior chamber restoration in patients with malignant glaucoma and a prolonged absence of the anterior chamber. METHODS: Five patients with malignant glaucoma and a long-term absence of the anterior chamber underwent a combination of anterior pars plana vitrectomy (aPPV), phacoemulsification cataract excision, intraocular lens implantation, peripheral iridotomy (PI), goniosynechialysis (GSL) (referred to aPPV + P + I + PI + GSL) at Beijing Tongren Hospital from October 2018 to June 2021. The study compared the changes in their visual acuity, intraocular pressure (IOP) and medication requirements between the pre-surgery period and their most recent follow-up visit. RESULTS: The five patients did not report any discomfort, such as pain, tearing, swelling, etc., in their affected eyes, and maintained a stable restoration of the anterior chamber. Among the affected eyes, only one eye demonstrated improved vision during the follow-up visit, while the remaining four eyes did not show any significant improvement. One eye underwent transscleral cyclophotocoagulation as an additional procedure, while the other four eyes did not require any further surgical intervention. In all cases, the intraocular pressure (IOP) was successfully controlled below 30 mmHg. Post-surgery, four eyes still required cycloplegia treatment, and three eyes continued to rely on eye drops to manage their IOP. CONCLUSION: Despite minimal improvement in vision, surgical intervention successfully restored the anterior chamber in malignant glaucoma patients with a prolonged absence of anterior chamber. This restoration contributed to alleviating subjective complaints of discomfort and delaying eyeball atrophy.

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Malignant glaucoma is characterized by ciliary block or aqueous misdirection, shallowing of the anterior chamber with elevated intraocular pressure (IOP), resistance to treatment, and rapid progression to blindness. However, the exact pathogenic mechanism is yet to be established. Here, we report a case of malignant glaucoma caused by immediate primary phacoemulsification for acute primary angle closure (APAC). A 90-year-old woman, who had experienced right eye pain and blurred vision one day prior, had a cataract in the same eye without phacodonesis. The right eye IOP was 39 mmHg, preoperative anterior chamber depth was 1.00 mm, and the axial length was 22.31 mm. We diagnosed APAC in the right eye and performed phacoemulsification. On postoperative day one, the IOP decreased to the normal range (15 mmHg), the anterior chamber deepened, and the angle became open. However, one week after phacoemulsification, the anterior chamber and angle became shallower and closer again. We diagnosed the patient with malignant glaucoma, performed hyaloid-zonulo-iridectomy, and administered 1% atropine eye drops postoperatively. As a result, the IOP was limited to a 10 mmHg range with an open angle and deep anterior chamber. Malignant glaucoma can be caused by immediate primary phacoemulsification for APAC.

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Aim: We report on an 88-year-old female patient who presented with a relapse in aqueous misdirection 15 years after being treated with pars plana vitrectomy (PPV) with hyaloidotomy-zonulectomy-iridotomy (HZI) for the same cause. Case description: A 73-year-old pseudophakic woman with a history of pseudoexfoliation underwent a trabeculectomy in the left eye in our institution because of uncontrolled intraocular pressure. Days after trabeculectomy, the patient developed aqueous misdirection and was treated with PPV combined with HZI. In the following years, the patient had regular follow-up appointments in our instruction, and she had normal intraocular pressures in the left eye without any treatment. A total of 15 years after the vitrectomy, the patient developed gradual swallowing in the anterior chamber and increased intraocular pressure. Neodymium (Nd): yttrium aluminum garnet (YAG) laser hyaloidotomy through the iridectomy opening was performed, the anterior chamber was immediately deepened, and the intraocular pressure was reduced to normal limits. The anterior chamber remained deep, and the intraocular pressure remained normal through the 36 months of follow-up. Conclusion: Relapse of aqueous misdirection may occur many years after vitrectomy, even when HZI is performed. An Nd: YAG- laser hyaloidotomy should be attempted before a second HZI is considered. Clinical relevance: A close and long-lasting follow-up of patients with aqueous misdirection is warranted, especially if the patients are treated not with complete PPV but by a limited disruption of the anterior hyaloid face along with HIZ. How to cite this article: Halkiadakis I, Tzimis V, Markopoulos I, et al. Late-onset Relapse of Aqueous Misdirection after Pars Plana Vitrectomy: Case Report and Literature Review. J Curr Glaucoma Pract 2023;17(1):49-51.

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PURPOSE: To report a case of Epstein-Barr virus (EBV) keratouveitis-induced malignant glaucoma after repeat penetrating keratoplasty (PK). METHODS: Retrospective review of the patient's medical records and review of literature on EBV corneal endotheliitis and/or anterior uveitis. RESULTS: A 78-year-old Thai female patient presented with a markedly edematous corneal graft, dense pigmented keratic precipitates, fibrinous anterior chamber reaction, uniformly flat anterior chamber, and ocular hypertension of 55 mmHg in the left eye on the first day after the third PK. An aqueous tap for polymerase chain reaction analysis was positive for EBV DNA but negative for other herpesviruses. The patient was diagnosed with EBV endotheliitis and anterior uveitis-induced malignant glaucoma; and successfully treated with oral valacyclovir and topical 2% ganciclovir eye drops. CONCLUSIONS: EBV endotheliitis and anterior uveitis can induce malignant glaucoma following PK. A high index of suspicion is required when a patient has a history of unexplained multiple graft rejections.

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BACKGROUND: Malignant glaucoma, caused by aqueous misdirection, is a challenging post-surgical complication presented with normal/high intraocular pressure and shallowing of the central and peripheral anterior chambers. Its incidence is about 0.6%-4.0%. It can be secondary to filtering surgeries, laser iridotomy, and cataract surgery. Short axial length and a history of angle closure glaucoma are its main risk factors. Here, we report a bilateral malignant glaucoma with bullous keratopathy in the patient's left eye. CASE SUMMARY: We present a case of bilateral malignant glaucoma. The cause of malignant glaucoma for each eye of this patient was different. Hence, the management strategy and selection of surgical methods were also different. However, the normal anterior chamber was ultimately maintained, and maximum visual function was preserved. Even though the left eye received multiple surgeries and corneal endothelial decompensation occurred, the formation of a retroendothelial fibrous membrane partially compensated for the function of the corneal endothelium. CONCLUSION: The formation of a retroendothelial fibrous membrane partially compensated for the function of the corneal endothelium.

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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.

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Purpose: To report the outcomes of medical and surgical management in patients diagnosed with aqueous misdirection syndrome (AMS). Patients and Methods: A retrospective chart review of all cases diagnosed with AMS at a single tertiary care eye center during the period from 2014 to 2021. Outcome measures were anatomical success (deepening of the anterior chamber (AC)), functional success (improvement in visual acuity), and treatment success (control of intraocular pressure (IOP)). Results: A total of 26 eyes with AMS from 24 patients were included. The patients were followed for a mean duration of 24 ± 18 months. Although some patients initially responded to medical and laser therapy, all but one (3.8%) eventually required surgery during the first 3 months after presentation. The mean duration from presentation until surgery was 45.9 ± 45.8 days (range: 2-119 days). The majority of cases (69.2%) were managed by pars plana vitrectomy. At the last follow-up visit, anatomical success was achieved in 20 (76%) eyes, 15 (57%) eyes had a final visual acuity that was either similar to or better than baseline, and successful control of IOP was achieved in 17 (65%) eyes. Univariate analysis revealed that a history of trabeculectomy as a cause of AMS was a risk factor for treatment failure (OR, 7.8; 95% CI, 1.16-52.35; P, 0.02). Conclusion: Our findings indicate that medical and laser management of AMS provide temporary control, and almost all patients eventually require surgery within the first 3 months. A history of trabeculectomy was found to be a risk factor for treatment failure.

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BACKGROUND: X-linked retinoschisis (XLRS) is a rare inherited bilateral retinal degeneration caused by mutations in RS1 gene, occurring exclusively in men. Various ocular complications associated with XLRS are reported, and angle closure glaucoma in these eyes is one such complication that is refractory and needs surgery for intraocular pressure control. Glaucoma surgery in these eyes often results in refractory malignant glaucoma with its serious sequelae. Several surgical modifications to prevent this complication have been tried with no or limited success. METHODOLOGY: In this report, we present a case of XLRS in a young male with a 22-year follow-up. We have described the natural history and progression of retinal disease and glaucoma. RESULTS: Refractory angle closure glaucoma in our patient was treated with core vitrectomy, phacoemulsification with intraocular lens implantation, and irido-zonulo-hyaloido-vitrectomy. This helped in successful deepening of anterior chamber, good IOP control, and preventing malignant glaucoma. CONCLUSION: Our case highlights the role of vitrectomy in managing the secondary angle closure glaucoma in eyes with X-LRS.

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INTRODUCTION: This study aimed to illustrate the efficacy of the combination of lens extraction, trabeculectomy, and anterior vitrectomy in patients with secondary angle-closure glaucoma (ACG) with autosomal recessive bestrophinopathy or Best vitelliform macular dystrophy. METHODS: This is a retrospective self-controlled case series study. Five patients undergoing a single trabeculectomy in one eye and triple surgery in the other eye were enrolled. All patients underwent a complete ophthalmic examination that included best-corrected visual acuity (BCVA), intraocular pressure (IOP), ultrasound biomicroscopy, and static gonioscopy. Multimodal fundus imaging was performed, including color fundus photography, fundus autofluorescence, and optical coherence tomography. Genetic testing was also analyzed. RESULTS:  Among the 10 eyes, the mean IOP was 31.4 ± 4.7 mmHg before surgery. The mean axial length (AL) was 21.53 mm and the anterior chamber depth (ACD) was 2.31 mm. There were no statistically significant differences in preoperative IOP, BCVA, ACD, and AL between the two groups (all P > 0.05). The mean follow-up time was 64.0 months. All five eyes with a single trabeculectomy developed malignant glaucoma (MG). No complications were found in the other five eyes with triple surgery, and the anterior chamber was deepened and stable after surgery until the last visit. The mean IOP at the last visit was normalized to 16 mmHg without using any medications. CONCLUSIONS:  Triple surgery is superior to single trabeculectomy for patients with ACG and BEST1 mutation, effectively bypassing MG complications. The vitreous may play a vital role in the mechanism of ACG in those patients and the high incidence of MG after filtering surgery.

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Purpose: To describe a case of bilateral pseudophakic malignant glaucoma treated with a new variant of irido-zonulo-hyaloid-vitrectomy. Observations: A patient who developed refractory bilateral pseudophakic malignant glaucoma underwent an irido-zonulo-hyaloid-vitrectomy via the anterior chamber in both eyes, one 2-months apart from the other. The procedure involved the use of an 18 Gauge needle to perform a straight vertical irido-zonulo-hyaloidotomy and a 25 Gauge vitreous cutter to complete the vitrectomy. The intraocular pressure was recorded on day 1, day 7, day 14, then monthly until 6 months postoperatively, whereas an ultrasound biomicroscopy of the anterior chamber was performed on day 1 and 6 months after the operation. The malignant glaucoma resolved in both eyes with no intra- nor post-operative complications. At 6 months from the surgeries none of the eyes developed recurrences. Conclusions: The new variant of irido-zonulo-hyaloid-vitrectomy we propose for the management of refractory pseudophakic malignant glaucoma resulted to be a safe and effective technique.

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Purpose: To report a case of a bilateral complex uveitic glaucoma (UG) with pupillary block, rupture of the anterior lens capsule, and malignant glaucoma in a young high-myopic patient and to report anterior segment optical coherence tomography (AS-OCT) findings initially and following surgery. Methods: A 21-year-old high-myopic woman who had a history of anterior uveitis with extensive posterior synechiae, presented with acute bilateral ocular pain, redness, and blurred vision following bilateral Nd: YAG laser peripheral iridotomy (LPI). Results: Visual acuity was limited to light perception in both eyes (OU), with a flat anterior chamber (AC) and anterior luxation of lens fragments. Intraocular pressure (IOP) was over 60 mmHg OU. AS-OCT showed closed angles and hyperreflective heterogeneous material within the flat AC. The iris and lens fragments were plated against the corneal endothelium OU. We performed an urgent pars plana vitrectomy associated with lensectomy. It was uneventful in OU. Repeated AS-OCT revealed a deep AC, widely open angles, and aphakia. IOP was lowered to 9 mmHg and visual acuity improved to 5/10 in OU. Conclusion: Performing LPI might be harmful in the presence of UG with extensive posterior synechia, resulting in complex mechanism glaucoma with aqueous misdirection syndrome associated with a pupillary block due to anterior lens luxation, even in high-myopic eyes. Nd: YAG LPI should not be performed simultaneously in OU, especially in pathologic eyes, to prevent bilateral vision-threatening complications. AS-OCT was of great help, allowing easy and detailed ultrastructural assessment of the ACs, and iridocorneal angles before and after surgery.