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

PRCIS: Long-term intraocular pressure control can be difficult to achieve in eyes with Sturge-Weber syndrome glaucoma. The most commonly performed primary surgery was trabeculotomyin early onset disease and tube shunt implantation in late onset disease. 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 2 consecutive follow-up visits after 3 months, IOP ≤5 mm Hg on 2 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 the early-onset group and 17 eyes in the late-onset group. The early-onset group more frequently presented with buphthalmos, corneal edema, and Haab 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 the 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). CONCLUSIONS: Early and late-onset SWS glaucoma may represent 2 entities with different pathogenetic mechanisms, clinical presentations, primary surgical choices, and outcomes, though this needs corroboration in future studies.

Incidence, outcomes, and risk of surgical failure after development of early hypotony following Aurolab aqueous drainage implant surgery.

Purpose: To report the incidence, outcomes, and risk of surgical failure after early postoperative hypotony following Aurolab Aqueous Drainage Implant (AADI) surgery for adult and pediatric refractory glaucoma. Methods: Medical records of patients who underwent AADI between January 2013 and March 2017 with a minimum of 2-years follow-up were retrospectively reviewed. Early postoperative hypotony was defined as IOP ≤5 mmHg within the first 3 months after AADI. Surgical failure of AADI was defined as IOP >21 mmHg or reduced <20% below baseline on two consecutive follow-up visits after 3 months, IOP ≤5 mmHg on two consecutive follow-up visits after 3 months, reoperation for glaucoma or a complication, or loss of light perception vision. Results: Early postoperative hypotony was seen in 15/213 eyes (7%) in the adult group and in 6/101 eyes (6%) in the pediatric group. The onset of hypotony was significantly earlier in the pediatric group (median = 39 days post AADI, IQR = 20-58 days) compared with adult eyes (median = 51 days post AADI, IQR = 30-72 days) (P = 0.02). Eyes with early postoperative hypotony did not have an increased risk of cumulative surgical failure as compared with eyes without hypotony in both adult (33.3% vs. 23.7%; P = 0.48) and pediatric (33.3% vs. 13.7%; P = 0.16) refractory glaucoma. All eyes recovered from hypotony, though one adult eye developed retinal detachment and one pediatric eye developed corneal decompensation and lost vision. Conclusion: Early postoperative hypotony was an infrequent complication post AADI and occurred earlier in pediatric eyes. Early postoperative hypotony did not increase risk of surgical failure up to 2 years.

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.

Long-term surgical outcomes of Aurolab aqueous drainage implant in pediatric eyes with primary congenital glaucoma versus aphakic glaucoma.

PURPOSE: To compare the outcomes of the Aurolab aqueous drainage implant (AADI) placed in eyes with refractory primary congenital glaucoma (PCG) versus aphakic glaucoma (APG). DESIGN: Retrospective comparative interventional case series. METHODS: Case files of consecutive eyes with PCG or APG that underwent AADI surgery between January 2013 and December 2016 and had a minimum 4 years follow-up were extracted from a computerised database. 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 after 3 months, reoperation for glaucoma or a complication, or loss of light perception. RESULTS: Eighty-nine eyes underwent AADI placement, including 42 eyes (47%) with PCG and 47 eyes (53%) with APG. Both groups were comparable at baseline. At 1 year, the APG group had lower mean IOP (13.6±8.1 mm Hg vs 17.6±7.5 mm Hg, p=0.02) with use of fewer IOP-lowering medications (0.8±1.0 vs 1.5±1.0, p=0.01) than the PCG group. The cumulative failure rate at 4 years was 57% (95% CI 43% to 72%) in PCG versus 40% (95% CI 28% to 56%) in the APG eyes (p=0.11). Eyes with PCG had greater tube-related complications (48% vs 38%, p=0.07) and number of reoperations (40% vs 32%, p=0.02) compared with eyes with APG. CONCLUSIONS: Eyes with APG had relatively better outcomes after AADI placement compared with PCG during 4 years of follow-up. Reoperations accounted for more than 70% of the failures.

Intermediate-term outcomes of the Aurolab aqueous drainage implant in neovascular glaucoma.

PURPOSE: To assess the outcomes of the non-valved Aurolab aqueous drainage implant (AADI) in neovascular glaucoma (NVG). METHODS: Data of consecutive patients with NVG who underwent AADI and had a minimum follow-up of 2 years were included. The primary outcome measure was the cumulative rate of surgical failure defined as intraocular pressure (IOP) >21 mm Hg or reduced <20% below baseline, IOP ≤5 mm Hg, reoperation for glaucoma or a complication, or loss of light perception vision. RESULTS: We included 85 eyes of 85 patients with NVG, with a mean age of 61.2±9.3 years. The most common aetiologies were proliferative diabetic retinopathy (n=43) and central retinal vein occlusion (n=24). The mean IOP decreased from 36.8±12.5 mm Hg at baseline to 15.8±7.5 mm Hg at 2-year follow-up (p<0.001) and the number of IOP-lowering medications reduced from 3.4±0.8 to 1.5±1.1 (p<0.001). The cumulative rate of failure increased from 3.1% (95% CI 1.1% to 11.8%) at 1 year to 33.8% (95% CI 20.4% to 52.5%) at 2 years. Multivariable analysis showed that eyes with open angles had a lower risk of failure (HR 0.17, 95% CI 0.10 to 1.03, p=0.09). The logarithm of minimum angle of resolution visual acuity declined from 0.98±0.7 to 1.8±1.0 at 2 years (p<0.001). CONCLUSION: Approximately one-third of NVG eyes that received the AADI failed after 2 years of follow-up similar to other series. Early AADI implantation at the open angle stage of NVG may yield better results.

Outcomes of Glaucoma Drainage Device Implantation and Trabeculectomy With Mitomycin C in Glaucoma Secondary to Aniridia.

PURPOSE: To compare the outcomes of Aurolab aqueous drainage implant (AADI; Aurolab) placement and trabeculectomy with mitomycin C (MMC) in patients with glaucoma secondary to aniridia. DESIGN: Retrospective comparative interventional case series. METHODS: This study included patients with congenital aniridia who underwent AADI implantation or trabeculectomy with MMC. Surgical failure was defined as IOP > 21 mm Hg or reduced <20% from baseline, IOP ≤ 5 mm Hg, reoperation for glaucoma or a complication, or loss of light perception vision. RESULTS: A total of 30 eyes of 30 patients underwent surgical treatment, including 18 eyes that received an AADI and 12 eyes that had a trabeculectomy with MMC. The cumulative probability of failure at 2 years was 11.1% (95% CI = 2.9%-37.6%) in the AADI group and 58.3% (95% CI = 33.5%-84.8%) in the trabeculectomy group (P = .05, log-rank). At 2 years, IOP (mean ± SD) was 14.1 ± 2.8 mm Hg in the AADI group and 19.6 ± 6.6 mm Hg in the trabeculectomy group (P = .02), and the number of glaucoma medications was 1.7 ± 0.9 in the AADI group and 2.2 ± 0.8 in the trabeculectomy group (P = .25). Surgical complications developed in 1 patient in each treatment group (P = .65). Cataract surgery was performed in 5 (42%) patients in the trabeculectomy group and no patients in the AADI group (P = .01). CONCLUSIONS: Placement of an AADI resulted in lower IOP and a higher rate of surgical success compared to trabeculectomy with MMC in eyes with glaucoma associated with aniridia. Cataract extraction was more frequently required after trabeculectomy with MMC than AADI implantation.

Incidence and Outcomes of Hypertensive Phase Following Aurolab Aqueous Drainage Implant Surgery in Adults with Refractory Glaucoma.

PURPOSE: To report the incidence and outcomes of hypertensive phase (HP) following Aurolab Aqueous Drainage Implant (AADI) (Aurolab) surgery in adults with refractory glaucoma. DESIGN: Retrospective, noncomparative, interventional case series. METHODS: All eyes that received the AADI and had a minimum of 2-year follow-up were identified, and data of patients who had intraocular pressure (IOP) ≤21 mm Hg at 6 weeks (ie, the time at which the tube-ligature suture dissolves) were used for statistical analysis. HP was defined as IOP >21 mm Hg during the first 3 months after the release of the tube ligating suture (with or without medications) in the absence of tube obstruction. RESULTS: A total of 200 eyes were included in the study, and HP was seen in 64 eyes (32%) with a peak IOP (mean ± SD) of 29.6 ± 7.8 mm Hg and peak incidence at 2-3 months after surgery. HP resolved within 3 months of its onset in 60 of the 64 eyes (94%) with additional IOP-lowering medications. The cumulative success rates were 71.8% (95% CI = 59.3%-81.2%) in HP eyes and 76.4% (95% CI = 68.7%-82.7%) in non-HP eyes (P = .23). Unadjusted Cox proportional hazards analysis showed that eyes experiencing HP had a marginally higher risk of failure (HR = 1.16, 95% CI = 0.6-2.1), but this relationship was not statistically significant (P = .61). CONCLUSIONS: A third of eyes that underwent AADI placement experienced HP. HP was successfully managed with additional IOP-lowering medications in a majority of cases and did not have a significant influence on long-term success rate.

Surgical Outcomes of Superotemporal Versus Inferonasal Placement of Aurolab Aqueous Drainage Implant in Refractory Pediatric Glaucoma.

PURPOSE: We sought to describe the outcomes of the Aurolab aqueous drainage implant (AADI) placed in the superotemporal (ST) versus the inferonasal (IN) quadrant in pediatric eyes with refractory glaucoma. DESIGN: Retrospective comparative interventional case series. METHODS: This was a retrospective study of patients ≤18 years of age who underwent AADI implantation and completed a minimum of 2-year follow-up. The choice of the quadrant depended upon the amount of scarring and conjunctival mobility. Cumulative success at 2 years was defined as intraocular pressure (IOP) ≤21 mm Hg or reduced by ≥20% below baseline on 2 consecutive follow-up visits after 3 months, IOP ≤5 mm Hg on 2 consecutive follow-up visits after 3 months, reoperation for glaucoma or a complication, or loss of light perception vision. RESULTS: A total of 144 patients (144 eyes) underwent AADI placement, including 48 eyes (33%) in the IN and 96 eyes (67%) in the ST quadrants. The IOP was significantly higher in the IN group (17.5 ± 7.4 mm Hg vs 13.7 ± 6.2 mm Hg, P = .005) with a greater number of medications (1.5 ± 1.0 vs 0.8 ± 0.9, P = .001) after 2 years of follow-up. Cumulative success rates at 2 years were 50.7% (95% confidence interval 35.4%-63.9%) in the IN group and 65.6% (95% confidence interval 56.5%-75.7%) in the ST group (P = .15). Complications occurred more frequently in the IN group, with significantly more tube exposure (12% vs 0%, P = .05). CONCLUSIONS: Placement of the AADI in the ST quadrant has better IOP-related outcomes and is a safer surgical option in pediatric eyes compared with the IN quadrant. It may be prudent to avoid AADI in the IN quadrant in children unless the ST location is not a viable option.

Aurolab Aqueous Drainage Implant With and Without Scleral Patch Graft in Refractory Adult and Pediatric Glaucomas: A Comparative Study.

PURPOSE: To compare the 2-year outcomes of eyes that received the Aurolab aqueous drainage implant (AADI) with and without a scleral patch graft. DESIGN: Retrospective comparative interventional case series. METHODS: Eyes with AADI and a minimum of a 2-year follow-up were included. Eyes that underwent implantation before January 2016 had surgery with a scleral patch graft covering the distal end of the tube, whereas those that were implanted after this period underwent surgery using a needle-generated scleral tunnel without the patch graft. The cumulative failure of the AADI was defined as intraocular pressure (IOP) >18 mm Hg or not reduced by 30% below baseline on 2 consecutive follow-up visits after 3 months. RESULTS: We included 215 adult eyes (n = 147 with patch graft, n = 68 without patch graft) and 111 pediatric eyes (n = 73 with patch graft, n = 38 without a patch graft). The mean IOP in eyes without the patch graft was higher at 1 month in adult eyes (before, 27.5 ± 14.1 vs after, 22.3 ± 11.1; P = .01) but not in pediatric eyes (14.3 ± before, 5.8 vs after, 17.8 ± 11.0; P = .39); there were no differences in IOP, vision, number of antiglaucoma medications, and complications between groups at all other time points. None of the eyes without the patch graft experienced tube exposure. Cumulative success rates at 2 years in adults (66.2% vs 63.9%, respectively; P = .85) were similar to those in children (77.2% vs 71.9%, respectively; P = .83) with both techniques. CONCLUSIONS: AADI placed without a scleral patch graft is as safe and effective as AADI placed with a patch graft in pediatric and adult refractory glaucomas.

Mutant RAMP2 causes primary open-angle glaucoma via the CRLR-cAMP axis.

PURPOSE: Primary open-angle glaucoma (POAG) is the leading cause of irreversible blindness worldwide and mutations in known genes can only explain 5-6% of POAG. This study was conducted to identify novel POAG-causing genes and explore the pathogenesis of this disease. METHODS: Exome sequencing was performed in a Han Chinese cohort comprising 398 sporadic cases with POAG and 2010 controls, followed by replication studies by Sanger sequencing. A heterozygous Ramp2 knockout mouse model was generated for in vivo functional study. RESULTS: Using exome sequencing analysis and replication studies, we identified pathogenic variants in receptor activity-modifying protein 2 (RAMP2) within three genetically diverse populations (Han Chinese, German, and Indian). Six heterozygous RAMP2 pathogenic variants (Glu39Asp, Glu54Lys, Phe103Ser, Asn113Lysfs*10, Glu143Lys, and Ser171Arg) were identified among 16 of 4763 POAG patients, whereas no variants were detected in any exon of RAMP2 in 10,953 control individuals. Mutant RAMP2s aggregated in transfected cells and resulted in damage to the AM-RAMP2/CRLR-cAMP signaling pathway. Ablation of one Ramp2 allele led to cAMP reduction and retinal ganglion cell death in mice. CONCLUSION: This study demonstrated that disruption of RAMP2/CRLR-cAMP axis could cause POAG and identified a potential therapeutic intervention for POAG.