Surgical Outcomes of Early Versus Late Onset Glaucoma Associated with Sturge-Weber Syndrome.

PURPOSE: To compare long-term surgical outcomes of glaucoma associated with Sturge-Weber syndrome (SWS) in eyes with early and late onset disease. METHODS: Medical records of children with glaucoma associated with SWS who underwent surgical treatment between January 1990 and December 2018 were reviewed. Those diagnosed ≤2 years of age were categorized as early onset while those who were diagnosed >2 years of age were late onset. Failure was defined as intraocular pressure (IOP) >21 mm Hg or reduced <20% below baseline on two consecutive follow-up visits after 3 months, IOP ≤5 mm Hg on two consecutive follow-up visits, reoperation for glaucoma or a complication, or loss of light perception. RESULTS: Forty three eyes of 36 children were studied, including 26 eyes in early onset group and 17 eyes in late onset group. The early onset group more frequently presented with buphthalmos, corneal edema and Haab's striae while late onset group had higher baseline IOP, larger cup-to-disc ratio and longer axial length. The most commonly performed primary surgery was trabeculotomy (50%) in early onset group and tube shunt implantation (71%) in late onset group. The cumulative probability of failure after 5 years follow-up was 50.6% in early onset group and 50.9% in late onset group (P=0.56). Postoperative complications occurred in 3 eyes (12%) in early onset group and 11 eyes (65%) in late onset group (P<0.001). CONCLUSION: Early and late onset SWS glaucoma may represent two entities with different pathogenetic mechanisms, clinical presentations, primary surgical choices and outcomes, though this needs corroboration in future studies.

Glaucoma drainage device implantation in a pregnant woman with axenfeld-rieger syndrome.

Axenfeld-Rieger syndrome (ARS) is a rare disease characterized by anterior segment anomalies with or without glaucoma. A 28-year-old antenatal female diagnosed with ARS presented with uncontrolled intraocular pressures (IOPs) and advanced glaucoma despite maximal medical therapy and progressive vision loss during her third trimester. The refractory and progressive nature of the disease, with useful vision in one eye, necessitated immediate surgical intervention, following which there was successful control of IOP, lasting till the final follow-up at 18 months. We discuss the role of glaucoma surgery, for an ARS patient with refractory glaucoma during the third trimester with a comprehensive review of literature.

A comparative study on surgical outcomes of trabeculectomy with and without anti-metabolites in juvenile open-angle glaucoma.

Purpose: To compare the surgical outcomes of trabeculectomy with and without anti-metabolites in patients with juvenile open-angle glaucoma (JOAG). Methods: This retrospective comparative case series included 98 eyes of 66 patients with JOAG who underwent either trabeculectomy without anti-metabolites (group A, n = 53 eyes) or with anti-metabolites (group B, n = 45 eyes) with a minimum of 2 years follow-up. The main outcome measures were intra-ocular pressure (IOP), number of glaucoma medications, visual acuity, additional surgical interventions, surgical complications, and risk factors for failure. Surgical failure was defined as IOP >18 mmHg or failure to reduce IOP by <30% from the baseline value or IOP ≤5 mmHg or re-operation for refractory glaucoma or a complication or loss of light perception vision. Results: The mean post-operative IOP reduced significantly from baseline at all post-operative visits until 6 months and thereafter. The cumulative probability of failure at 2 years was 28.7% in group A [95% confidence interval (CI) = 17.6-44.8%] and 29.1% in group B (95% CI = 17.1-46.7%) (P = 0.78). Surgical complications occurred in 18 eyes (34%) in group A and 19 eyes (42%) in group B. Re-operations for glaucoma or complications were performed in two eyes (3.8%) in group A and two eyes (4.4%) in group B. Cox-hazard regression model revealed male gender (HR = 0.29; P = 0.008), baseline high IOP (HR = 0.95; P = 0.002), and an increased number of pre-operative glaucoma medications (HR = 2.08; P = 0.010) as significant factors associated with failure. Conclusion: : Our study results on trabeculectomy in JOAG revealed a success of 71% in both groups at 2 years follow-up. There was no significant difference in success or failure rates between the two groups. The risk factors for poor surgical outcome in JOAG were male gender, baseline high IOP, and an increased number of glaucoma medications.

Evaluation of the agreement and reliability of Transpalpebral Tonometers compared with Goldmann Applanation Tonometer - A systematic Review and Meta-Analysis Protocol.

In 2020, the global prevalence of glaucoma was estimated to be 76 million and it was projected to increase to 111.8 million by 2040. Accurate intraocular pressure (IOP) measurement is imperative in glaucoma management since it is the only modifiable risk factor. Numerous studies have compared the reliability of IOP measured using transpalpebral tonometers and Goldmann applanation tonometry (GAT). This systematic review and meta-analysis aims to update the existing literature with a reliability and agreement comparison of transpalpebral tonometers against the gold standard GAT for IOP measurement among individuals presenting for ophthalmic examinations. The data collection will be performed using a predefined search strategy through electronic databases. Prospective methods-comparison studies published between January 2000 and September 2022 will be included. Studies will be deemed eligible if they report empirical findings on the agreement between transpalpebral tonometry and Goldmann applanation tonometry. The standard deviation and limits of agreement between each study and their pooled estimate along with weights and percentage of error will be reported using a forest plot. Cochrane's Q test and the I2 statistic will be used to assess heterogeneity, and the publication bias will be investigated using a funnel plot, Begg's and Egger's tests. The review results will provide additional evidence on the reliability of transpalpebral tonometers that, in turn, could possibly assist practitioners to make informed decision about using it as a screening or diagnostic device for clinical practice, outreach camps, or home-based screening. Institutional Ethics Committee registration number: RET202200390. PROSPERO Registration Number: CRD42022321693.

Short-term outcomes of micropulse transscleral cyclophotocoagulation as a primary versus additional therapy in eyes with uncontrolled glaucoma.

Purpose: To study the safety and efficacy outcomes of Micropulse Transscleral Cyclophotocoagulation (MP-TSCPC) as a primary versus additional therapy in eyes with uncontrolled glaucoma. Methods: This was a prospective, interventional, comparative study. All patients with advanced and refractory glaucoma treated with MP-TSCPC from April 2020 to December 2020 were recruited in this study. Results: A total of 77 eyes of 77 patients were analyzed. Group A (n = 33), included patients with advanced glaucoma at high risk for invasive surgery, who underwent MP-TSCPC as the primary intervention, and group B (n = 44) included patients who had undergone previous surgical intervention and MP-TSCPC was used additionally to control the intraocular pressure (IOP). Mean IOP and mean number of antiglaucoma medications were 34.06 (13.9) mmHg and 3.64 (0.7), respectively, in group A and 35.61 (11.5) mmHg and 3.73 (0.9), respectively, in Group B. Postoperatively, the mean IOP and percentage of IOP reduction were significantly lower at 1, 3, and 6 months, that is, 20.78 (32%), 22.07 (30%), and 19.09 (37%), respectively, in group A and 23.68 (35%), 19.50 (44%), and 19.61 (42%), respectively, in group B, but there was no difference between the groups at all visits. Postoperative need for ocular hypotensive drugs did not differ in group A (P = 0.231); however, it was significantly lower in group B (P = 0.027). Group A had 87%, 77%, and 74% success rates at 1, 3, and 6 months, respectively, whereas group B had 91%, 86%, and 77% success rates at 1, 3, and 6 months, respectively. Postoperative complications and intervention did not reveal any statistical difference between the two groups. Conclusion: MP-TSCPC may be considered as a temporizing measure both as a primary or as an additional intervention to control the IOP in eyes with refractory and advanced glaucoma that have a high risk of vision-threatening complications with invasive surgery.

Outcomes of the Aurolab aqueous drainage implant and trabeculectomy with mitomycin C in iridocorneal endothelial syndrome.

PURPOSE: To compare the surgical outcomes of the Aurolab aqueous drainage implant (AADI) and trabeculectomy with mitomycin C (MMC) in patients with glaucoma secondary to iridocorneal endothelial (ICE) syndrome. MATERIALS AND METHODS: This retrospective comparative case series included 41 eyes of 41 patients with ICE syndrome and glaucoma who underwent either a trabeculectomy with MMC (n = 20) or AADI surgery (n = 21) with a minimum of 2 years follow-up. Outcome measures included intraocular pressure (IOP), the use of glaucoma medications, visual acuity, additional surgical interventions, and surgical complications. Surgical failure was defined as IOP > 21 mmHg or reduced < 20% from baseline, IOP ≤ 5 mmHg, reoperation for glaucoma or a complication, or loss of light perception vision. RESULTS: The cumulative probability of failure at 2 years was 50% in the trabeculectomy group (95%CI = 31-83%) and 24% in the AADI group (95%CI = 11-48%) (p = 0.09). The IOP was consistently lower in the AADI group compared with the trabeculectomy group at 6 months and thereafter. Surgical complications occurred in 13 eyes (65%) in the trabeculectomy group and 12 eyes (57%) in the AADI group (p = 0.71). Reoperations for glaucoma or complications were performed in 12 eyes (60%) in the trabeculectomy group and 5 patients (24%) in the tube group (p = 0.06). Cox proportional hazards showed that AADI had a 53% lower risk of failure at 2 years (p = 0.18; HR = 0.47; 95%CI = 0.16-1.40). CONCLUSION: AADI surgery achieved lower mean IOPs than trabeculectomy with MMC in managing glaucoma secondary to ICE syndrome. A trend toward lower rates of surgical failure and reoperations for glaucoma and complications was observed following AADI placement compared with trabeculectomy with MMC in eyes with ICE syndrome.

Ocular features in a patient presenting with a rare combination of multiple phakomatoses.

Phakomatoses are a group of congenital disorders characterised by hamartomatous lesions of the skin as well as the central and peripheral nervous systems. The presence of naevus flammeus or port-wine stain is a characteristic feature of many such disorders including Sturge-Weber syndrome (SWS), Klippel-Trenaunay syndrome (KTS) and Phakomatosis pigmentovascularis (PPV).We describe the ocular findings in a patient with coexisting PPV, SWS and KTS.

Short-term surgical outcomes of twin-site combined phaco-emulsification and mitomycin-C augmented trabeculectomy in pseudo-exfoliation glaucoma versus primary open-angle glaucoma.

Purpose: To compare the short-term outcomes of combined phaco-emulsification with posterior chamber intra-ocular lens and mitomycin augmented trabeculectomy in patients with pseudo-exfoliation glaucoma (PXFG) versus primary open-angle glaucoma (POAG). Methods: A total of 144 eyes of 144 patients were enrolled in this prospective interventional comparative study, 72 each of which had PXFG and POAG, respectively. All patients underwent twin-site combined phaco-trabeculectomy at a tertiary eye center in India between December 2017 and December 2018 and were followed up for a period of 12 months. The main outcome measures were intra-ocular pressure (IOP), best corrected visual acuity (BCVA), total surgical time, rate of intra-operative and post-operative complications, and the number of ocular hypotensive medications needed. Success rates were determined via Kaplan-Meier survival analysis. Results: The mean age was 63.9 ± 7.9 years in the POAG group and 66.4 ± 6.8 years in the PXFG group (P - 0.04). The baseline BCVA, IOP, and cup-disc ratio were comparable between two groups. Intra-operative complications and post-operative outcomes were comparable between the two groups. There was a significant drop in anti-glaucoma medications in both groups. Six patients, three (4.2%) in each group, were lost to follow-up after 6 months. Three patients (4.2%) in PXFG needed additional glaucoma intervention for controlling IOP, one patient needed a non-valved glaucoma drainage device, and two patients required diode cyclo-photocoagulation within the follow-up period. Conclusion: Patients with PXFG had a longer surgical time than POAG. Similar success and complication rates were observed following combined twin-site phaco-trabeculectomy in both POAG and PXFG at 1 year. Combined glaucoma surgery resulted in good IOP control, improvement in BCVA, and lower requirement of ocular hypotensives in both the groups.

Correlation of pattern of visual field loss by perimetry and anterior chamber angle parameters by anterior segment optical coherence tomography in primary angle-closure glaucoma.

Purpose: To correlate and analyze the pattern of the visual field (VF) defects by perimetry and anterior chamber angle parameters by AS-OCT in primary angle-closure glaucoma (PACG) across varied severity levels on presentation to a tertiary eye care center. Methods: This was a cross-sectional study, which included 323 eyes of clinically diagnosed cases of PACG. Glaucoma severity was categorized according to mean deviation (MD) as mild (-6.00 dB or more), moderate (-6.01 to -12.00 dB), and severe (-12.01 to -30.00 dB). AS-OCT measured the nasal (N) and temporal (T) angle opening distance at 500 µm (AOD 500) and 750 µm (AOD 750), anterior chamber angle (ACA), lens vault (LV), and anterior chamber width (ACW). The VF severity was then correlated with the AS-OCT parameters using statistical analysis. Results: The mean age ± standard deviation (SD) of the patients included in the study was 56.03 ± 8.6 years, with a 1:1.2 gender ratio. The number of eyes with mild, moderate, and severe VFs were 140 (43.3%), 88 (27.24%), and 95 (29.41%), respectively. There was no statistically significant correlation in the mean anterior chamber angle parameters (AOD 500, AOD 750, ACA 500, ACA 750, LV, ACW, and axial length (AL)) among the groups. However, the correlation between AOD 500 and LV thickness was found to be significant (P = 0.0000) with a negative Spearman's rank correlation coefficient (r = -0.3329). Conclusion: The ACA parameters obtained by AS-OCT along the horizontal axis after elimination of pupillary block by laser peripheral iridotomy do not correlate and cannot be used to assess the disease severity of PACG.

Comparison of clinical and biometric characteristics between nanophthalmic children and age-matched controls.

Purpose: To compare the clinical and biometric characteristics of children presenting with nanophthalmos (NO group) with that of age-matched controls (CO group). Methods: Electronic medical records of 40 children (<18 years of age) with diagnosis of nanophthalmos (NO), presented to a tertiary center in Tamil Nadu between January 2010 and December 2019, were reviewed and compared with 30 age-matched controls (CO) presenting for routine eye examination between October 2019 and December 2019. Clinical parameters compared were best-corrected visual acuity (BCVA), axial length (AxL), keratometry (K), anterior chamber depth (ACD), lens thickness (LT), retinochoroidal scleral thickness (RCS), corneal diameter, central corneal thickness (CCT), intraocular pressure (IOP), lens axial length factor (LAF), and lens thickness/anterior chamber depth ratio (LT/ACD). Results: Mean age of the NO group was 8.95 ± 4.0 years. Mean spherical equivalent (SE) in NO group was 10.87 ± 3.1 D and was inversely correlated to AxL (r = -0.46, P value = 0.003). All biometric parameters (AxL, ACD, LT, RCS, LAF, and LT/ACD), except CCT were significantly different between NO and CO groups. NO group children had 52.5% visual impairment with BCVA ≤ 6/24 and 17.5% had esotropia. Common ocular associations in NO group were amblyopia (64.3%), primary angle-closure glaucoma (PACG) (17.8%), pigmentary retinopathy (14.3%), and retinal detachment (3.6%). Angle-closure disease was seen in 50% of NO group and 30% underwent laser peripheral iridotomy (LPI). There was a significant difference in SE, ACD, and LAF among NO children with AxL <17 mm or >17 mm. Multivariable regression analysis revealed a significant correlation of SE and ACD with AxL. Conclusion: Nanophthalmos in children often present as amblyopia with visual impairment and strabismus. NO group with AxL <17 mm, had angle-closure disease as a common association with significantly lower ACD, higher SE, and LAF. All morphometric characteristics, except CCT, were significantly different between NO and CO groups. Close monitoring with serial biometry in NO group is needed for the timely diagnosis and prompt intervention to avoid visual impairment, due to glaucoma.

A report on a series of nanophthalmos with histopathology and immunohistochemistry analyses using light microscopy.

We aimed to study the histopathological and immunohistochemistry features in clinically diagnosed cases of nanophthalmos using light microscopy. This was an observational comparative study. We enrolled four eyes of four consecutive patients with nanophthalmos and visually significant cataract, who underwent cataract surgery with prophylactic posterior sclerostomy. Histological analysis of the excised scleral tissue was done and compared with age-matched cadaver controls between January 2021 and October 2021. Hematoxylin and Eosin (H&E) stains were used for histological analysis, and was further supplemented with immunohistochemistry (IHC) and immunofluorescence (IF) analyses using a simple light microscope. The immunostained sections were analyzed using confocal microscope for the fibronectin expression level. The main outcome measure was demonstration of histological changes of sclera in nanophthalmic eyes undergoing cataract surgery. Light microscopic features of nanophthalmos revealed thick fibers with fraying and lightly stained cores, irregular serrated edges, and randomly interspersed fibroblasts compared to regular arrangement of collagen fibers seen in cadaver controls. Immunohistochemistry analysis with anti-fibronectin antibody showed strong positivity in clustered fibers in nanophthalmos, and less intense diffuse staining in cadaver tissue. Histoclinical correlation was observed in one nanophthalmic scleral tissue with axial length less than 17 mm showing severe disorganization with diffuse collagenization, loss of fibrillary architecture compared to another specimen with axial length more than 17 mm. Simple, cost-effective light microscopy using basic stains was effective in identifying the characteristic histopathological features in nanophthalmic eyes, and this was further highlighted by immunohistochemistry and immunofluorescence analyses.

Comparison of uveal parameters between acute primary angle-closure eyes and fellow eyes in South Indian population.

Purpose: : To analyze the ocular biometric parameters of eyes with acute primary angle closure (APAC) as compared to fellow eyes. Methods: : A cross-sectional study was conducted on 27 patients presenting with recent onset APAC to a tertiary eye institute in India. Anterior and posterior ocular biometric parameters were measured simultaneously by anterior segment optical coherence tomography (AS-OCT), A-scan, ultrasound biomicroscopy (UBM), and B-mode ultrasonogram (USG). The parameters measured were anterior chamber depth (ACD), anterior chamber angle (ACA), angle opening distance (AOD500, AOD750), lens vault (LV), axial length (AL), ciliary body thickness maximum (CBTmax) and at the point of scleral spur (CBT0), anterior placement of the ciliary body (APCB), and retinochoroidal thickness (RCS). Results: Mean age ± SD of patients with APAC was 55.66 ± 7.2 years with female preponderance (21:6 patients). Mean presenting IOP ± SD of the affected eye and fellow eye were 54.74 ± 11.67 mm Hg and 18.7 ± 11.67 mm Hg, respectively. Eyes with APAC had statistically significant narrower anterior ocular biometric parameters, higher LV, decreased ciliary body thickness, more APCB, and longer AL than the fellow eyes. CBTmax is the only variable that had significance (ß = -0.421,95% CI: -0.806 to - 0.035, P = 0.034) in the univariate analysis with RCS thickness in APAC eyes. Further, there was a correlation between CBT0 and APCB with CBTmax both in univariate (ß = 0.894, P < 0.0001 and ß = -0.351, P = 0.039) and multivariable analysis (ß = 0.911, P < 0.0001 and ß = -0.416, P = 0.016). Conclusion: Compared to the fellow eyes, APAC eyes had different ocular biometric parameters. In addition to known biometric parameters associated with pupillary block (narrower anterior biometric parameters-ACA, ACD, and AOD), our study found multiple nonpupillary block factors such as higher lens vault and thinner and more anteriorly placed ciliary body to be associated with APAC.

Peripapillary and macular retinoschisis - A vision-threatening sequelae of advanced glaucomatous cupping.

PURPOSE: To present a selected case series of advanced glaucoma-associated peripapillary and macular retinoschisis and response to various treatment strategies with a comprehensive literature review. METHODS: Retrospective observational case series. Retrospective review of five selected cases of advanced glaucoma with peripapillary and macular retinoschisis. RESULTS: All five patients had advanced glaucomatous damage with macular and peripapillary retinoschisis, three (patients 2, 3, and 5) had a neurosensory detachment of the macula. Increased intraocular pressure was managed with maximal antiglaucoma medications and G6 micropulse diode laser treatment in the first patient, transscleral diode laser in the second patient, mitomycin-C augmented trabeculectomy in the third patient, maximal antiglaucoma medications alone in the fourth patient, pars plana vitrectomy followed by trabeculectomy in the fifth patient. CONCLUSION: We speculate that peripapillary and macular retinoschisis may indicate a vision-threatening sequelae of advanced glaucoma. The probable inciting factor for this vision-threatening pathology being elevated intraocular pressure, fluctuations in intraocular pressure, and chronic glaucoma with advanced cupping. We emphasize that meticulous examination of the macula in patients with advanced glaucoma is mandatory. It is imperative to do OCT macula in patients with advanced glaucoma to diagnose this distinct entity at an earlier stage and preserve the existing visual potential.

Incidence and risk factors for postoperative intraocular pressure response to topical prednisolone eye drops in patients undergoing phacoemulsification.

AIM/PURPOSE: To report the incidence, risk factors, and magnitude of steroid response in individuals receiving topical 1% prednisolone acetate eye drops following phacoemulsification surgery MATERIALS AND METHODS: Postoperative IOP of 1118 consecutive patients who had uneventful cataract surgery and used 1% topical prednisolone acetate were studied. Baseline ocular parameters like best-corrected visual acuity, IOP, and slit-lamp examination findings were noted preoperatively and at postoperative day 30. Incidence of postoperative intraocular pressure response to steroid was analyzed and graded as mild, moderate, or severe and risk factors studied. RESULTS: The mean age of our study cohort was 59.49 ± 7.25 years. The overall incidence of steroid response was 3.2%, (2.8% being moderate responders, and 0.4% high responders). Mean preoperative IOP was 14.67 ± 2.2 mm Hg in the study cohort (n = 1118). Mean postoperative IOP was 21.33 ± 7.97 mm Hg in the steroid responder (SR) and 14.66 ± 2.8 mm Hg in the non-responder (NR), with a statistically significant difference from the baseline IOP in the SR group (p < 0.001) and no difference in the NR. Univariate analysis revealed younger age and high axial length as risk factors but on multiple regression analysis, only younger age < 50 years was found to be a significant risk factor for steroid response. CONCLUSION: The overall steroid response in this population post-cataract surgery was low with most being moderate responders. Younger age and higher axial length were identified as risk factors for steroid response, and hence this warrants the judicious use of steroids in such individuals.