Huntington's disease and neurovascular structure of retina.

BACKGROUND: Retinal biomarkers in neurodegenerative disorders have attracted much attention in recent years. Recent studies have reported visual dysfunction in Huntington's disease (HD). However, little is known about retinal structural changes in HD. METHODS: A total of 50 subjects, including 25 motor-manifest HD patients and 25 gender- and age-matched controls, were enrolled. Unified Huntington's Disease Rating Score-Motor part was assessed in HD patients. Spectral-domain Optical Coherence Tomography (SD-OCT) was used to evaluate the macular thickness and peripapillary retinal nerve fiber layer (pRNFL). Superficial and deep capillary plexus densities were measured using OCT angiography (OCTA). To account for inter-eye correlation, generalized estimating equation (GEE) model was used. RESULTS: HD patients had a significant reduction in macular thickness in both inner and outer superior sectors and the inferior outer sector. Inferior pRNFLs were significantly decreased in thickness. There was no significant difference in retinal capillary plexus density between the two groups. Age and disease duration were negatively correlated with macular thickness in HD patients. However, the severity of motor involvement was not correlated with SD-OCT or OCTA parameters. CONCLUSIONS: We observed attenuated pRNFL and macular retinal thickness in patients with HD, independent of macular capillary plexus parameters. It can support the hypothesis that the retina may be a potential biomarker for monitoring the neurodegenerative process in HD.

The effect of the hypertensive phase on the long-term outcomes of Ahmed glaucoma valve (AGV) implantation.

BACKGROUND: To investigate the long-term effect of hypertensive phase (HP) on the clinical outcomes of Ahmed glaucoma valve (AGV) implantation. METHOD: The records of patients with different etiologies of glaucoma who underwent AGV implantation with at least 3 years of follow-up were retrospectively reviewed. HP was defined as the IOP > 21 mm Hg during the first three months after surgery. The main outcome measure was cumulative success defined as 5 < IOP ≤ 21 mmHg and 20% reduction from the baseline with or without IOP lowering medications. Results that do not achieve cumulative success or undergo glaucoma reoperation during the follow-up period are considered failures. The secondary outcome measures were intraocular pressure (IOP) and the number of glaucoma medications. RESULTS: A total of 120 patients (28 patients of HP, 92 patients without HP) with an average age (± SD) of 48.9 ± 19.6 years and a mean follow-up of 4.5 ± 1.4 years were enrolled. The mean duration of survival was 5.3 ± 0.5 years in HP which was significantly shorter than 6.4 ± 0.2 years in non-HP (log rank = 4.2, P = 0.04). Mean IOP and number of IOP lowering agents were higher in postoperative visits at 1,2, 3, and 4 years in HP patients compared with non-HP (all Ps < 0.01). Higher baseline IOP was significantly associated with higher rates of surgical failure. CONCLUSION: In the long-term follow-up, the duration of survival was significantly longer in the non-HP group. In the non-HP group, the failure rate was significantly lower than the HP group.

Effect of trabeculectomy on optic nerve head and macular vessel density: an optical coherence tomography angiography study.

PURPOSE: To investigate the effect of trabeculectomy on peripapillary and macular vessel density (VD) using optical coherence tomography angiography (OCT-A) in patients with primary open-angle glaucoma. METHODS: This is a prospective, interventional study on 32 glaucoma eyes (21 patients) who underwent trabeculectomy. Optic nerve head (ONH) and macular structural and OCT-A scans were performed before surgery and at one and six months postoperatively with the Avanti device (AngioVue System, Optovue Inc., Fremont, CA). The primary outcomes of interest were changes in global and regional peripapillary and macular VD. RESULTS: The average (standard deviation) age and visual field mean deviation of the sample were 58.9(9.9) years and -14.5(9.0) dB, respectively. The average (standard deviation) baseline intraocular pressure significantly decreased from 24.5(10.4) mmHg to 9.6(2.1) mmHg, 6 months after the surgery (P< 0.001). The mean (standard deviation) whole image VD of ONH had increased from 37.6(8.3)% to 40.5(9.1)% at 6 months after the surgery (P = 0.01) while its changes were not significant at month 1 (38.1(9.5)%, P = 0.64). The average of radial peripapillary circle (RPC) VD increased from 36.5(10.3)% to 39.3(10.8)% at 6 months (P = 0.04) compared to 1-month post-trabeculectomy (37.0(11.4)%, P = 0.71). Similar patterns at postoperative months 1 and 6 were also observed in RPC VD at superior and inferior hemifields as well as nasal and temporal quadrants. The observed changes in VD of macular, foveal, peri-, and parafoveal were not significant at superficial or deep slabs at 1 and 6 months after trabeculectomy (all P values > 0.05). CONCLUSION: Our study demonstrated significant improvements in peripapillary vessel density at 6-month following trabeculectomy.

Effects of fasting on peripapillary capillary density, peripapillary nerve fiber layer, intraocular pressure and central corneal thickness.

PURPOSE: To investigate the effects of fasting on intraocular pressure (IOP), central corneal thickness (CCT), radial peripapillary capillary (RPC) density and retinal nerve fiber layer (RNFL) thickness during Islamic fasting month of Ramadan. METHODS: Twenty-seven healthy fasting volunteers were enrolled. All subjects underwent full ophthalmic examination and optical coherence tomography angiography (OCTA) of both eyes. All measurements were recorded first in the morning (8:00-10:00 a.m.) and then in the evening (4:00-6:00 p.m.). The first visit was performed during the second and third week of Ramadan and then two months later in a nonfasting routine day. RESULTS: Mean age of participants was 40.07 ± 9.29 years. A significant decrease was found for evening IOP (11.17 ± 2.29 mmHg) in comparison with morning IOP (12.00 ± 2.28) (p = 0.00) only on fasting days. A decrease was observed for CCT both on fasting (6 µm) and nonfasting days (3 µm) (p = 0.00 and p = 0.02, respectively) in the evening. There was a significant increase in whole and peripapillary RPC density (%) on fasting days (48.79 ± 3.08 morning, 49.72 ± 2.85 evening for whole and 50.57 ± 4.06 morning, 51.64 ± 3.71 evening for peripapillary) (p = 0.00). Average RNFL thickness was decreased from morning to evening both on fasting days (0.80 µm) and nonfasting days (1.25 µm) (p = 0.00). Optic nerve head (ONH) vertical cup/disc (C/D) ratio was greater on fasting days (0.30 ± 0.25 morning, 0.31 ± 0.24 evening) in comparison with nonfasting days (0.27 ± 0.25 morning, 0.28 ± 0.25 evening) (p = 0.02). CONCLUSION: Fasting decreases the IOP and CCT in healthy subjects. OCTA revealed significant difference in RPC vessel density, RNFL thickness and ONH vertical C/D ratio during fasting hours in comparison with nonfasting days.

Effect of subconjunctival Bevacizumab injection on the outcome of Ahmed glaucoma valve implantation: a randomized control trial.

IMPORTANCE: The effect of subconjunctival Bevacizumab injection on the outcome of Ahmed glaucoma valve (AGV) implantation. BACKGROUND: Evaluation of efficacy and safety of subconjunctival Bevacizumab injection adjunctive to AGV implantation. DESIGN: Prospective and randomized clinical trial. PARTICIPANTS: Fifty eyes of 50 patients with diagnosis of glaucoma that were candidate for AGV surgery were included. METHODS: In 25 eyes, conventional AGV surgery (group 1) and in 25 eyes AGV surgery with subconjunctival Bevacizumab (group 2) was performed by block randomization MAIN OUTCOME MEASURES: The primary outcome measure was surgical success. Outcome measures were compared at postoperative month 3, 6 and 12. RESULTS: Mean age of patients was 58.76 ± 12.11 and 51.36 ± 15.44 years in group 1 and 2 respectively (P = 0.06). Mean intraocular pressure (IOP) at baseline was 24.88 ± 7.62 mmHg in group 1 and 27.52 ± 8.57 mmHg in group 2 which decreased to15.4 ± 4.4 mmHg in group 1 and 13.42 ± 2.9 mmHg in group 2 (P < 0.00) at last follow up. Surgical success was defined in two level: postoperative IOP ≤ 21 mmHg with at least 20% reduction in IOP (Criterion A), either with no medication (complete success) or with no more than two medications (qualified success) and criterion B with the same definition but the IOP ≤ 18 mmHg The cumulative success according to criterion A and B was 77.8%, 72.2% in group 1 and 89.5% in group 2, respectively, at the end of follow-up. CONCLUSIONS AND RELEVANCE: Subconjunctival injection of Bevacizumab adjunctive to AGV implantation leads to higher success rate compared with AGV alone in one year follow-up.

Comparison between visual field defect in pigmentary glaucoma and primary open-angle glaucoma.

To compare visual field defect patterns between pigmentary glaucoma and primary open-angle glaucoma. Retrospective, comparative study. Patients with diagnosis of primary open-angle glaucoma (POAG) and pigmentary glaucoma (PG) in mild to moderate stages were enrolled in this study. Each of the 52 point locations in total and pattern deviation plot (excluding 2 points adjacent to blind spot) of 24-2 Humphrey visual field as well as six predetermined sectors were compared using SPSS software version 20. Comparisons between 2 groups were performed with the Student t test for continuous variables and the Chi-square test for categorical variables. Thirty-eight eyes of 24 patients with a mean age of 66.26 ± 11 years (range 48-81 years) in the POAG group and 36 eyes of 22 patients with a mean age of 50.52 ± 11 years (range 36-69 years) in the PG group were studied. (P = 0.00). More deviation was detected in points 1, 3, 4, and 32 in total deviation (P = 0.03, P = 0.015, P = 0.018, P = 0.023) and in points 3, 4, and 32 in pattern deviation (P = 0.015, P = 0.049, P = 0.030) in the POAG group, which are the temporal parts of the field. It seems that the temporal area of the visual field in primary open-angle glaucoma is more susceptible to damage in comparison with pigmentary glaucoma.

Transient Myopia and Ciliochoroidal Effusion following Influenza Vaccination.

Introduction: The aim of the study was to report a case of ocular adverse events following influenza vaccination which involved bilateral myopic shift, ciliochoroidal effusion, and fine retinal folds in a middle-aged woman. Case Presentation: A 42-year-old female presented with sudden-onset painless binocular decreased distance vision. She had received a quadrivalent influenza vaccine (Influvac Tetra) injection 10 days prior, a few days after which she experienced blurred vision. Her initial uncorrected visual acuity and refraction were 20/200 and -3.00/-1.00 × 180 in the right eye, 20/100 and -3.00/-0.50 × 50 in the left eye. Intraocular pressure was within normal limits bilaterally. Anterior segment examination was significant for bilateral shallow anterior chambers (AC) and narrow iridocorneal angles with no cells or flare. Fundus examination was notable for bilateral fine chorioretinal folds radiating from the macula and bilateral low-lying choroidal effusion in the far periphery. Ultrasound biomicroscopy confirmed bilateral ciliochoroidal effusions, shallow AC, and anterior rotation of the ciliary bodies. The patient was started on oral and topical corticosteroids plus atropine and timolol eye drops after which prompt improvement was observed. Restoration of visual acuity and complete resolution of the symptoms without any further complication were observed after 2 weeks. Conclusion: The temporal association between the onset of the patient's symptoms and influenza vaccination, in the absence of any pertinent medical conditions or medications, significantly implicates causality. Future research and case reports can help in corroborating this ocular adverse event attributed to influenza vaccination.

Outcomes of Trabeculectomy and Ahmed Glaucoma Valve Implantation in Patients With Iridocorneal Endothelial Syndrome.

PRCIS: Our study highlights the long-term success of trabeculectomy or Ahmed glaucoma valve (AGV) surgery in patients with glaucoma secondary to iridocorneal endothelial (ICE) syndrome. However, many ICE syndrome cases may need multiple glaucoma surgeries to achieve controlled intraocular pressure (IOP), with/without concomitant corneal graft surgery. OBJECTIVE: To evaluate the long-term outcome of trabeculectomy and AGV implantation in ICE syndrome. METHODS: Patients with glaucoma secondary to ICE syndrome who underwent either trabeculectomy or AGV surgery with intraoperative adjunctive mitomycin-C from 2009 to 2020 were included in this study. All patients were followed for at least 6 months after initial surgery. The main outcome measures were IOP, number of IOP-lowering medications, and surgical success. Surgical success was defined as complete according to the levels of IOP ( < 18) and at least 20% reduction from preoperative IOP without medications and qualified as a complete success but with medications, where the number of medications was less than preoperative numbers. Cumulative success was the sum of the qualified and complete success. RESULTS: Twenty-nine eyes of 29 patients were included. Trabeculectomy was done in 13 patients (group A, 44.8%) and 16 patients underwent AGV surgery (group B, 55.2%). The median age was 50 (42-56.50) and 47 (36.75-52.75) years in groups A and B, respectively ( P = 0.10). All patients completed at least 2 years of follow-up. Mean IOP was not significantly different between groups preoperatively ( P = 0.70) and the effect of the type of surgery on IOP was not statistically significant at multiple follow-up time points (repeated measures analysis of variance, P = 0.44). The mean IOP decreased from 35.76 ± 6.36 mm Hg preoperatively to 16.00 ± 3.10 in group A and from 36.12 ± 8.11 mm Hg to 17.00 ± 3.75 in group B ( P = 0.449) at year 2 of follow-up. The effect of the type of surgery was not significant on the total number of IOP-lowering medications used throughout the study (repeated measures analysis of variance, P = 0.81). Kaplan-Meier analysis shows complete success in 14 patients (48.3%), 11 patients (37.9%), and 7 patients (24.1%) at 6-month, 1-year, and 2-year follow-up, respectively. The cumulative success rate was 95% at 2 years follow-up for all patients. CONCLUSIONS: In 2-year follow-up, trabeculectomy or AGV significantly reduced the IOP in glaucoma patients secondary to ICE syndrome.

Contribution of the latent transforming growth factor-ß binding protein 2 gene to etiology of primary open angle glaucoma and pseudoexfoliation syndrome.

PURPOSE: To assess for the first time the possible contribution of latent transforming growth factor (TGF)-beta binding protein 2 (LTBP2), an extracellular matrix (ECM) protein that associates with fibrillin-1-containing microfibrils, to the etiology of primary open angle glaucoma (POAG) and pseudoexfoliation (PEX) syndrome. Mutations in LTBP2 have previously been shown to be the cause of primary congenital glaucoma (PCG) and other disorders that often manifest as secondary glaucoma. METHODS: All exons of LTBP2 were sequenced in the DNA of 42 unrelated patients with POAG and 48 unrelated patients with PEX syndrome. Contribution of candidate variations to disease was assessed by screening in control individuals and use of biochemical, bioinformatics, and evolutionary criteria, and in one case by segregation analysis within the family of a proband with POAG. Microscopy was performed on the skin of a patient with PEX syndrome whose condition developed into PEX glaucoma during the course of the study and on the skin of her son previously identified with PCG who harbored the same LTBP2 mutation. RESULTS: Among the 30 sequence variations observed in LTBP2, five found in five patients with POAG and two found in two patients with PEX glaucoma syndrome may contribute to their diseases. One of the mutations was observed in a patient with POAG and in a patient with PEX glaucoma syndrome. Light, fluorescent, and electron microscopy showed that a mutation present in one of the individuals affected with PEX glaucoma syndrome and in her son affected with PCG causes disruptions in the ECM. CONCLUSIONS: Some LTBP2 sequence variations can contribute to the etiology of POAG and PEX glaucoma syndrome. It is not expected that in these diseases LTBP2 mutations behave in a strictly Mendelian fashion with complete penetrance. In conjunction with recent findings, the results suggest that anomalies in the ECM are among the factors that can contribute to POAG and PEX glaucoma syndrome. LTBP2 and other related ECM protein coding genes should be screened in larger cohorts with these diseases, which are common disorders and important to the public health.

Intraocular pressure after exposure to moderate altitude.

BACKGROUND: To study intraocular pressure (IOP) alteration in healthy individuals following a rapid effortless increase in altitude from 1900 m above sea level (ASL) to 3740 m ASL. METHODS: Intraocular pressure, blood pressure, pulse rate, and arterial oxygen tension were determined in both eyes of healthy volunteers at the lower altitude. Participants were taken to a higher altitude of 3740 m ASL (1840-m altitude gain) via gondola lift, which took 30 minutes. All measurements were repeated at the higher altitude. Pearson and Spearman correlation analysis was conducted to assess the correlations among the variables. A paired t-test and linear regression were also used to compare IOP before and after ascending. The accepted level of significance for all tests was p <0.05. RESULTS: Fifty-four healthy volunteers participated in the study. Four eyes of three subjects with IOP higher than 21 mmHg were excluded. Intraocular pressure ± SD (range) decreased from 14.9 ± 2.6 mmHg (9-21 mmHg) to 14.3 ± 2.4 mmHg (11-20 mmHg) (p = 0.02) after the ascent. Arterial oxygen saturation decreased from 95.4 % to 91.5 % (p < 0.001). Neither of the participants complained of any ocular or systemic symptoms during or after ascending to the higher altitude. Mean IOP, before and after ascending, was positively correlated with systolic blood pressure before and after the increase in altitude (Pearson correlation coefficient, 0.41, p = 0.002 and Pearson correlation coefficient, 0.37, p = 0.006, respectively). Intraocular pressure changes did not correlate with age, pulse rate, or arterial oxygen saturation. CONCLUSION: A rapid, effortless increase in altitude (over a moderate range in altitude) decreases IOP in healthy individuals. The observed decrease may not be clinically significant; however, it shows the versatility of IOP control mechanisms in response to alteration in altitude and temperature.

Subtenon triamcinolone injection to treat serous choroidal detachment after glaucoma surgery: A case series.

PURPOSE: To report the effect of subtenon triamcinolone acetonide (TA) injection in the treatment of serous choroidal detachment (SCD) after glaucoma surgery. METHODS: In this prospective case series, patients with persistent, non-resolving, or progressive SCD after glaucoma surgery were enrolled. For those with non-resolving or progressive SCD despite of using systemic corticosteroids, topical atropine and topical steroids, one milliliter of TA (40mg/mL) was injected inferotemporally into the posterior subtenon space. RESULTS: Sixteen consecutive patients with a mean ± SD age of 70.12 ± 11.12 years were included in this study. After injection of subtenon TA, SCD was completely resolved after 1 to 4 weeks, with deepening of the anterior chamber in all cases. All cases were followed for at least 6 months after the injection with no signs of recurrence. CONCLUSION: Subtenon injection of TA is a safe and effective modality of treatment for resolving a persistent or progressive SCD after glaucoma surgeries.

LTBP2 mutations cause Weill-Marchesani and Weill-Marchesani-like syndrome and affect disruptions in the extracellular matrix.

Latent transforming growth factor (TGF) beta-binding protein 2 (LTBP2) is an extracellular matrix (ECM) protein that associates with fibrillin-1 containing microfibrils. Various factors prompted considering LTBP2 in the etiology of isolated ectopia lentis and associated conditions such as Weill-Marchesani syndrome (WMS) and Marfan syndrome (MFS). LTBP2 was screened in 30 unrelated Iranian patients. Mutations were found only in one WMS proband and one MFS proband. Homozygous c.3529G>A (p.Val1177Met) was shown to cause autosomal recessive WMS or WM-like syndrome by several approaches, including homozygosity mapping. Light, fluorescent, and electron microscopy evidenced disruptions of the microfibrillar network in the ECM of the proband's skin. In conjunction with recent findings regarding other ECM proteins, the results presented strongly support the contention that anomalies in WMS patients are due to disruptions in the ECM. Heterozygous c.1642C >T (p.Arg548*) possibly contributed to MFS-related phenotypes, including ocular manifestations, mitral valve prolapse, and pectus excavatum, but was not cause of MFS.

Superior ophthalmic vein thrombosis following coil embolization of posterior communicating artery aneurysm: Report of two cases.

INTRODUCTION: The purpose was to present two patients with superior ophthalmic vein thrombosis following coil embolization of posterior communicating artery aneurysm, that have not been reported after this particular procedure yet. METHODS: We present two patients with subarachnoid hemorrhage due to posterior communicating artery aneurysms, who were treated with stent-assisted coil embolization. Shortly after the procedure, both cases demonstrated painful proptosis, ophthalmoplegia, increased intraocular pressure, intraretinal hemorrhage and macular edema on the ipsilateral side, with congestion of intraorbital tissues and thickened and dilated superior ophthalmic vein in neuroimaging investigation. The occlusion was confirmed by digital subtraction angiography. RESULTS: Patients were treated with anticoagulant agents and systemic corticosteroids immediately and then received medical treatment by an ophthalmologist for ocular complications of superior ophthalmic vein thrombosis. CONCLUSION: Isolated superior ophthalmic vein thrombosis (SOVT) is rare, but may be associated with sight-threatening complications. After intracranial interventions on cerebral vessels, clinical features of SOVT including choroidal effusion syndrome should be investigated, and the patients should receive prompt and proper care including ophthalmic medications and systemic anticoagulants to restrict the ocular and systemic complications.

Trans-Scleral Cyclophotocoagulation in Refractory Pigment Dispersion-Like Glaucoma Secondary to Ciliary Body Melanoma.

Purpose: To report a case of ciliary body melanoma with acute high intraocular pressure (IOP) due to pigment dispersion, treated by limited trans-scleral cyclophotocoagulation (TSCPC) and plaque radiotherapy. Methods: A 33-year-old woman was referred to clinic with acute ocular pain and decreased visual acuity from 1 week before presentation. The IOP was 55 mmHg accompanied by red eye, perilimbal injection, mild corneal edema (stromal and epithelial), scattered pigment dust on central corneal endothelium, 4+ anterior chamber pigments, and pigmented cells. Gonioscopy revealed a bulging mass posterior to the iris root, about 2 o'clock width alongside a heavy dark brown pigmentation of all angle structures. Ultrasound biomicroscopy confirmed a ciliary body mass of about 4.5 mm × 4 mm × 3.3 mm in the superior ciliary region, in favor of melanoma. Due to no response to maximal antiglaucoma therapy, a limited TSCPC in the inferior hemisclera was done. After control of the IOP, plaque radiotherapy with Ru-106 was done. Results: Three days after the cyclophotocoagulation, IOP decreased to 18 mmHg. Visual acuity reached to 20/25 and IOP remained 18 mmHg, with timolol/dorzolamide drop twice a day. Anterior chamber pigments gradually decreased, and antiglaucoma and steroid drops were tapered during 1 month, thereafter. The IOP was 14-16 mmHg with timolol/dorzolamide bid at months 3 and 6 of follow-up and 21-22 mmHg without any drop at months 12 and 18, with no sign of glaucomatous optic neuropathy. Conclusion: Limited cyclophotocoagulation may be a good choice for controlling the high refractory IOP in cases of intraocular neoplasms such as ciliary body melanoma, which are planned for salvage therapy such as plaque radiotherapy.

Trabeculectomy in patients with diabetes: subconjunctival Mitomycin C with or without intravitreal bevacizumab.

BACKGROUND/AIMS: To evaluate the success of Mitomycin C (MMC) augmented trabeculectomy with or without intravitreal bevacizumab in patients with diabetes without neovascular glaucoma. METHODS: Fifty-six patients with diabetes who needed trabeculectomy were randomised to either combination of 2.5 mg intravitreal bevacizumab and subconjunctival MMC (group A, 28 eyes) or subconjunctival MMC alone (group B, 28 eyes). The main outcome measures were intraocular pressure (IOP), number of antiglaucoma medications, bleb morphology and success probability. Surgical success was defined as complete according to two levels of IOP (≤18 and <15 mm Hg) and at least 20% reduction from preoperative IOP without antiglaucoma medications and qualified as complete success but with antiglaucoma medications. Total success was the sum of complete and qualified success. RESULTS: The mean age was 65.2±12.9 and 67.4±10.2 years in groups A and B, respectively (p=0.50). All patients completed at least 1 year of follow-up. The mean 1-year IOP decreased from 25.2±12.0 mm Hg to 10.1±2.8 in group A and from 26.4±11.6 mm Hg to 15.2±6.9 in group B (all p<0.001). Overall, postoperative IOP measurement was statistically significantly lower in group A at month 12 (p=0.001). The number of medications in groups A and B at month 12 was 0.3±0.8 and 1.0±1.2, respectively (p=0.02). The cumulative probability of success at month 12 was not statistically significant (89.3% group A, 78.6% group B; p=0.27). CONCLUSION: In patients with diabetes with primary trabeculectomy, combined administration of intravitreal bevacizumab and subconjunctival MMC resulted in lower IOP and number of antiglaucoma medication compared with subconjunctival MMC alone.

Early Macular Thickness Changes after Trabeculectomy and Combined Phaco-Trabeculectomy.

Purpose: To assess postoperative changes in central retinal thickness (RT) following trabeculectomy and combined phaco-trabeculectomy using spectral domain-optical coherence tomography. Methods: In a prospective interventional comparative study, 64 consecutive glaucoma patients who underwent trabeculectomy (32 eyes) or phaco-trabeculectomy (32 eyes) were included. A macular thickness map using the Early Treatment Diabetic Retinopathy Study circles of 1 mm, 3 mm, and 6 mm was the standard to evaluate the 9-subfield thickness preoperatively and again at 1 and 3 months after surgery. Four subfields in each of the 3 mm and 6 mm rings were considered parafoveal and perifoveal regions, respectively. Results: Preoperative measurements were similar in the two groups, except patients in the combined group which were older (P = 0.002). The mean RT in the combined phaco-trabeculectomy group at month 1 was significantly higher than baseline measurements at central subfield retinal thickness (CSRT) (P = 0.01), temporal (P = 0.001), and inferior (P = 0.04) parafoveal and temporal (P = 0.01), superior (P = 0.02), and nasal (P < 0.001) perifoveal quadrants; however, RT changes in the trabeculectomy-only group were not statistically significant at months 1 and 3 (P > 0.05). The increase in the temporal perifoveal RT of the combined phaco-trabeculectomy group persisted at month 3 (P = 0.01), while the RT in other sectors returned to preoperative values. The two treatment groups did not differ in terms of changes in the CSRT over time (P = 0.37). In addition, no difference was observed between the treatment groups regarding the parafoveal RTs at each time points (0.06 ≤ P ≤ 0.29). Conclusions: There was no significant difference in the pattern of changes of CSRT and parafoveal RT between trabeculectomy and combined phaco-trabeculectomy treatment groups up to 3 months after surgery. Some detectable increase in RT in the combined phaco-trabeculectomy will reverse to baseline values 3 months after surgery, except in the temporal perifoveal region.

Loss of function mutations in the gene encoding latent transforming growth factor beta binding protein 2, LTBP2, cause primary congenital glaucoma.

Glaucoma is a heterogeneous group of optic neuropathies that manifests by optic nerve head cupping or degeneration of the optic nerve, resulting in a specific pattern of visual field loss. Glaucoma leads to blindness if left untreated, and is considered the second leading cause of blindness worldwide. The subgroup primary congenital glaucoma (PCG) is characterized by an anatomical defect in the trabecular meshwork, and age at onset in the neonatal or infantile period. It is the most severe form of glaucoma. CYP1B1 was the first gene genetically linked to PCG, and CYP1B1 mutations are the cause of disease in 20-100% of patients in different populations. Here, we report that LTBP2 encoding latent transforming growth factor beta binding protein 2 is a PCG causing gene, confirming results recently reported. A disease-associated locus on chromosome 14 was identified by performing whole genome autozygosity mapping in Iranian PCG families using high density single nucleotide polymorphism chips, and two disease-segregating loss of function mutations in LTBP2, p.Ser472fsX3 and p.Tyr1793fsX55, were observed in two families while sequencing candidate genes in the locus. The p.Tyr1793fsX55 mutation affects an amino acid close to the C-terminal of the encoded protein. Subsequently, LTBP2 expression was shown in human eyes, including the trabecular meshwork and ciliary processes that are thought to be relevant to the etiology of PCG.

Ocular biometric changes after Ahmed glaucoma valve implantation.

PURPOSE: To investigate the ocular biometric changes after uneventful Ahmed glaucoma valve implantation. METHODS: Patients with refractory glaucoma who were candidate for Ahmed valve surgery were prospectively included in this study. Patients with a history of any kinds of corneal surgery were excluded. Refractive status, intraocular pressure, axial length, anterior chamber parameters including anterior and posterior mean keratometry, central corneal thicknesses, and anterior chamber depth, anterior chamber volume, and anterior chamber angle were evaluated at baseline and 1 and 3 months after surgery. RESULTS: A total of 20 eyes from 20 patients were included. Mean intraocular pressure at baseline was 33.4 ± 12.3 mm Hg that significantly decreased to 14.6 ± 6.2 mm Hg at 1 month and 13.5 ± 4.3 mm Hg at 3 months after Ahmed glaucoma valve implantation (p < 0.001). Mean number of medications was 3.6 ± 1.3 at baseline which significantly decreased to 1.0 ± 1.3 at 3 months after Ahmed glaucoma valve implantation (p < 0.001). Axial length decreased significantly from 23.69 ± 1.95 to 23.47 ± 1.91 mm (p < 0.001) at month 3. There were no significant changes in other parameters such as mean spherical equivalent, anterior chamber volume, anterior chamber angle, anterior chamber depth, and keratometry at the end of month 3 after surgery (p > 0.05 in all). CONCLUSION: Ahmed glaucoma valve implantation had a significant effect on axial length at 3 months after surgery but its effect on keratometry and other anterior chamber parameters was not significant.

Vision-related quality of life in patients with a history of congenital glaucoma.

PURPOSE: To assess the vision-related quality of life in adult patients with a history of primary congenital glaucoma. METHODS: In a cross-sectional hospital-based study eligible patients with a history of primary congenital glaucoma aged more than 18 were recruited in the study. Patients with secondary glaucoma and monocular patients were excluded. All patients underwent a complete ophthalmologic examination. The subjects were requested to answer a Persian approved version of the 25-item National Eye Institute Visual Function Questionnaire (NEI VFQ-25) questionnaire. RESULTS: A total of 23 congenital glaucoma patients have enrolled in the study. The mean age was 29.22 (9.3 SD) and mean IOP was 13.82 (5.19 SD) and 15.69 (5.26 SD) in right and left eyes, respectively. The mean number of medications was 1.13 (1.25 SD) in the right and 1.30 (1.18 SD) in the left eyes. Among all scores of NEI-VFQ-25, the lowest score belonged to mental health 53.71 (29.72) and the highest score was color vision score 83.69 (20.79). We found a significant influence of visual field defect on many subscales including general health and general vision (p = 0.007, R = +0.65) and (p = 0.002, R = +0.71) respectively. The Mean Defect (MD) was associated with low social functioning and peripheral vision too (p = 0.035, R = +0.53) and (p = 0.001, R = +0.76) respectively. Age had a negative impact on the general vision subscale (p = 0.003, R = -0.59). CONCLUSION: Our study showed that visual field defect was strongly associated with many subscales scores in (NEI VFQ-25) questionnaire.

Postoperative Blepharoptosis after Trabeculectomy versus Ahmed Glaucoma Valve Implantation.

PURPOSE: To investigate the frequency of persistent postoperative ptosis (PP) following trabeculectomy or Ahmed glaucoma valve (AGV) implantation and to analyze the associated factors. METHODS: It is a prospective observational study on glaucoma patients who underwent trabeculectomy or AGV implantation from October 2015 to June 2017 in a tertiary center. Margin reflex distance 1 and 2 (MRD1 and 2) and levator function were measured before and at least 6 months, postoperatively. Clinically significant ptosis was defined as ≥2 mm drop of MRD1. RESULTS: One hundred and fourteen patients (124 eyelids) including 76 patients (87 eyelids) with trabeculectomy and 35 patients (37 eyelids) with AGV implantation were included. The mean age was 55.50 (standard deviation = 17.54) years. Most of the surgeries were performed under general anesthesia (87.9%, 109/124) between 30 and 60 min (53.2%, 66/124) by residents (39.5%, 49/124). Trabeculectomy and AGV groups did not differ in terms of pre, intra, and postoperative variables (0.1≤ P ≤0.9) except duration of surgery (P = 0.01) and sex (P = 0.04). Clinically significant persistent PP was observed in 12.9% (16/124) in total, 13.7% (12/87) in the trabeculectomy group, and 10.8% (4/37) in the AGV group (P = 0.6). Male gender (ß coefficient = 2.56, 95% confidence interval (CI) = 4.76-0.36, P = 0.02) and a higher preoperative MRD1 (ß coefficient = 1.24, 95% CI = 0.52-1.95, P = 0.001) were the only factors affecting the frequency of clinically significant PP. CONCLUSIONS: Postoperative blepharoptosis occurred in 12.9% of eyes after glaucoma procedures. Male gender and higher preoperative MRD1 were significantly associated with a higher frequency of postglaucoma surgery blepharoptosis.