Macular and peripapillary vascular parameters in the fellow eyes of unilateral primary congenital glaucoma: a comparative study.

PURPOSE: To study the fellow eyes of patients with unilateral primary congenital glaucoma (PCG) using optical coherence tomography angiography (OCTA) and compare them to normal age- and refractive error-matched healthy controls. METHODS: Using OCTA, the foveal avascular zone (FAZ) area, cup:disk ratio, vessel density (VD) of the optic nerve head (ONH) and peripapillary area and the macular VD in superficial (SCP) and deep vascular complexes in both 3 mm and 6 mm scans of both groups were compared. Clinical data included best-corrected visual acuity (BCVA), cycloplegic refraction, intraocular pressure (IOP), anterior and posterior segments examination findings, including ONH cup:disk ratio. RESULTS: A total of 48 eyes of 48 children (24 eyes in each group) were included. There was no difference in the mean retinal nerve fiber layer thickness, cup:disk ratio, baseline visual acuity, or spherical equivalent between groups (P > 0.05). In the 3 mm macular scan, the VD of the SCP at the fovea was significantly higher in the PCG group compared to controls (P = 0.04). In the ONH scans, there was a significantly reduced inside the disk VD in the PCG group compared to controls (P = 0.03). There was no significant difference in other macular and ONH vascular parameters between groups (P > 0.05). CONCLUSIONS: In our study cohort, there was no difference in most of the macular and ONH vascular parameters between groups. However, the fellow eyes of PCG patients exhibited higher VD of the SCP at the fovea and reduced inside the disk VD compared with control eyes.

Risk Factors for Blindness in Children With Primary Congenital Glaucoma-Follow-up of a Registry Cohort.

PURPOSE: To evaluate the baseline features associated with blindness in a cohort of children with primary congenital glaucoma (PCG) from a hospital registry. DESIGN: Retrospective clinical cohort study. METHODS: Setting: Observational cohort study. StudyPopulation: The registry included all children who presented to our tertiary care institute between 1995 and 2014 with a diagnosis of childhood glaucoma. ObservationProcedure: Baseline characteristics at initial presentation of children with PCG in the registry who were blind at the last follow-up were compared with those who were not blind, using bivariate and then multivariate regressions to account for potential confounders. MainOutcomeMeasures: Blindness was defined as a best-corrected visual acuity of 3/60 (20/400) or worse in the better eye at the final follow-up. RESULTS: The eligible sample consisted of 196 children with a mean age of 9.54 ± 22.44 months at presentation. After a mean ± standard deviation follow-up of 8.49 ± 3.85 years, 20 (10.2%) children were blind. The baseline demographic factors, intraocular pressure, horizontal corneal diameter, spherical equivalent, axial length, and corneal thickness, were similar for the "blind" and "not blind" groups (P > .05). In the multivariate regression, only the severity of corneal opacification remained significantly (P < .001) associated with the risk of blindness (odds ratio = 4.05; 95% confidence interval: 1.89-8.85). CONCLUSION: Corneal clouding is a predictor of future blindness in children with PCG. Children with severe corneal clouding may need more aggressive intraocular pressure control, closer follow-up, and earlier counseling.

Neonatal-Onset Congenital Ectropion Uveae: A Distinct Phenotype of Newborn Glaucoma.

PURPOSE: To describe neonatal-onset congenital ectropion uveae (N-CEU) as a distinct clinical entity of newborn glaucoma (NG) and to study its significance toward the severity and outcome of NG. DESIGN: Prospective clinical cohort study. METHODS: The study took place at a tertiary care postgraduate teaching institute. It included consecutive patients with NG who presented between July 1, 2016 and September 30, 2017, with a minimum postoperative follow-up of 1 year. Infants with any ocular anomaly apart from CEU were excluded. Patients with N-CEU were compared with those with neonatal-onset primary congenital glaucoma (N-PCG). All infants underwent goniotomy or trabeculotomy, with trabeculectomy depending on corneal clarity. Clinical features at presentation and outcome 1 year after surgery were defined as good or satisfactory if intraocular pressure was ≤16.0 mm Hg under anesthesia without or with topical medications, respectively, and poor if the infant required additional surgery. RESULTS: Twenty eyes of 10 patients with N-CEU were compared with 16 eyes of 9 patients with N-PCG. Infants with N-CEU had significantly worse corneal clarity (mean grade 2.0 ± 0.7 vs 1.4 ± 0.8; P = .026) and poorer outcomes compared with those with N-PCG. Seven of 16 (43.7%) eyes with N-PCG had a cornea clear enough at presentation for a goniotomy compared with only 2 of the 20 (10%) eyes with N-CEU (P = .026). Thirteen of 16 (81.2%) eyes with N-PCG had a good or satisfactory outcome compared with 6 of 20 (30%) eyes with N-CEU (P = .001). CONCLUSIONS: N-CEU appears to be distinct from the unilateral CEU in older patients described in the literature and may be considered a poorer prognosis phenotype of neonatal-onset glaucoma.

SVEP1 as a Genetic Modifier of TEK-Related Primary Congenital Glaucoma.

Purpose: Affecting children by age 3, primary congenital glaucoma (PCG) can cause debilitating vision loss by the developmental impairment of aqueous drainage resulting in high intraocular pressure (IOP), globe enlargement, and optic neuropathy. TEK haploinsufficiency accounts for 5% of PCG in diverse populations, with low penetrance explained by variable dysgenesis of Schlemm's canal (SC) in mice. We report eight families with TEK-related PCG, and provide evidence for SVEP1 as a disease modifier in family 8 with a higher penetrance and severity. Methods: Exome sequencing identified coding/splice site variants with an allele frequency less than 0.0001 (gnomAD). TEK variant effects were assayed in construct-transfected HEK293 cells via detection of autophosphorylated (active) TEK protein. An enucleated eye from an affected member of family 8 was examined via histology. SVEP1 expression in developing outflow tissues was detected by immunofluorescent staining of 7-day mouse anterior segments. SVEP1 stimulation of TEK expression in human umbilical vascular endothelial cells (HUVECs) was measured by TaqMan quantitative PCR. Results: Heterozygous TEK loss-of-function alleles were identified in eight PCG families, with parent-child disease transmission observed in two pedigrees. Family 8 exhibited greater disease penetrance and severity, histology revealed absence of SC in one eye, and SVEP1:p.R997C was identified in four of the five affected individuals. During SC development, SVEP1 is secreted by surrounding tissues. SVEP1:p.R997C abrogates stimulation of TEK expression by HUVECs. Conclusions: We provide further evidence for PCG caused by TEK haploinsufficiency, affirm autosomal dominant inheritance in two pedigrees, and propose SVEP1 as a modifier of TEK expression during SC development, affecting disease penetrance and severity.

Primary Congenital Glaucoma: Trends in Presentation Over 3 Decades at a Tertiary Eye Care Center in India.

PRECIS: In this study, huge time delay was observed between first primary congenital glaucoma (PCG) symptom recognition by parents to ophthalmological diagnosis and this delay was unchanged over time. Bilateral affliction and mean presenting intraocular pressure (IOP) remained unaltered. PURPOSE: To compare the trends in presentation of patients with PCG over 3 different time periods at 10-year intervals (1998, 2008, and 2018) managed by a single surgeon at one of the largest tertiary eye care centers in South India. METHODS: Retrospective analysis of 313 eyes of 172 patients diagnosed to have PCG and treated at the L V Prasad Eye Institute (LVPEI) between January and December of the 3 specified years. Data collected included age at symptom recognition and at presentation, sex, affected eye, signs and symptoms, time between onset of symptoms and diagnosis (diagnostic delay), corneal clarity, and IOP. RESULTS: Of the 172 patients, 48 (27.9%), 76 (44.2%), and 48 (27.9%) belonged to 1998, 2008, and 2018, respectively. Bilateral affliction (79% to 84%) was common and there was no sex predilection. The median age at recognition of first symptoms by caregivers/parents was 1 day (interquartile range, 1 to 30), and at diagnosis was 120 days (interquartile range, 30 to 378). Overall, presentation to LVPEI was delayed by >3 months in 80 (46.5%) children, and there was no statistically significant difference in the duration of delay over time (P=0.13). There was no significant difference in the mean presenting IOP over time (P>0.05). CONCLUSIONS: We provide unique single center-based information of trends in the presentation of patients with PCG over 3 decades. Diagnostic delay was common, and a large number of characteristics of patients with PCG in South India have remained unchanged, particularly, age at onset and at diagnosis and laterality.

From Conventional Angle Surgery to 360-Degree Trabeculotomy in Pediatric Glaucoma.

PURPOSE: To describe the transition from conventional angle surgery (CAS), trabeculotomy with rigid probe or goniotomy, to 360-degree trabeculotomy assisted with microcatheter (MCT). DESIGN: Retrospective comparative interventional case series. METHODS: Review of consecutive children with glaucoma undergoing angle surgery, including cases with previous surgery, from January 2012 until March 2018 at Moorfields Eye Hospital. Main outcome measure was success rate, defined as intraocular pressure (IOP) ≤21 mm Hg with a minimum of 20% of IOP reduction and no further glaucoma surgery (complete success: without the need of glaucoma drops; qualified success: drops were needed to keep the IOP under control). RESULTS: Among the 106 eyes (77 patients) included were 54 MCT and 52 CAS eyes. At last visit, after a single surgery, qualified success was 85% (46 eyes) in MCT and 37% (19 eyes) in CAS. Complete success was 69% (37 cases) in MCT and 23% (12 cases) in CAS. The mean (95% confidence interval) change in axial length after surgery was -0.03 mm (-0.34 to 0.40) for MCT and +1.35 mm (-0.64 to 1.62) for CAS (P < .001). The percentage of IOP reduction was 52.1% in MCT and 45.5% in CAS (P = .1616). Further glaucoma surgery was required in 5.5% (3) in MCT and 63.4% (33) in CAS. At 1 year, 94.3% of MCT cases achieved qualified success compared to 34.6% of CAS (P < .0001). No significant complications were found on either group. CONCLUSION: MCT achieved better results with significantly lower reoperation rates. The transition from CAS to MCT can be easily achieved, even in difficult cases or those previously operated.

Outcomes of Circumferential Trabeculotomy and Converted 180-Degree Traditional Trabeculotomy in Patients With Neonatal-onset Primary Congenital Glaucoma.

PRéCIS:: The present study demonstrates that the surgical success rate of circumferential trabeculotomy is 64.7% according to target intraocular pressure (IOP) ≤21 mm Hg and ≥30% reduction from baseline in patients with neonatal-onset primary congenital glaucoma (PCG), with an average follow-up of 35.1 months. PURPOSE: The aim of the study was to evaluate the surgical success rate and applicability of circumferential trabeculotomy compared with converted traditional trabeculotomy in patients with neonatal-onset PCG in Turkey. PATIENTS AND METHODS: A retrospective study enrolled 42 eyes of 42 patients with an average age of 34.0±6.7 (14 to 59) days at the time of the surgery, who underwent 5/0 prolene suture-assisted circumferential trabeculotomy or rigid probe-assisted traditional trabeculotomy for the treatment of neonatal-onset PCG. IOP at baseline, 1st, 2nd, 3rd, 6th, 9th, 12th, 24th, and 36th months of follow-up, need of glaucoma eye drop, need of further glaucoma surgery, cup to disc ratio, axial length, corneal diameters, and presence of corneal clouding were recorded. Surgical success was defined as IOP <21 mm Hg and ≥30% reduction from baseline without any further glaucoma surgery. RESULTS: Seventeen eyes underwent circumferential trabeculotomy and 25 eyes with a failure in completing circumferential trabeculotomy underwent traditional trabeculotomy. The applicability rate of circumferential trabeculotomy was 40.4%. The mean preoperative IOP value was 33.2±7.6 mm Hg. The average follow-up time was 35.1±21.7 (12 to 95) months. Surgical success rates of circumferential trabeculotomy at the first, second, and third year of follow-up were 70.6%, 64.7%, and 64.7%, respectively. Surgical success rates of traditional trabeculotomy at the first, second, and third year of follow-up were 60%, 48%, and 44%, respectively. CONCLUSIONS: Circumferential trabeculotomy seems to be a safer and more successful procedure than traditional trabeculotomy for the treatment of neonatal-onset PCG. Unlike the traditional procedure, the IOP-lowering effect of circumferential trabeculotomy could be maintained up to ~36 months.

XEN Gel Stent in Pediatric Glaucoma.

PURPOSE: To report a series of 3 eyes of 3 children with congenital glaucoma receiving the XEN gel stent implant. PATIENTS AND METHODS: An interventional case series is reported. RESULTS: Three eyes underwent implantation of the gel stent. In 2 cases, the gel implant procedure was performed after failed trabeculotomy, while in the third eye, primary gel stent implantation was performed. One eye received 2 implants. Three procedures were performed via an ab interno approach while the fourth was performed ab externo. No device-related complications were observed. In all 3 eyes, intraocular pressure was controlled without the use of topical medications through 6 to 24 months of follow-up. CONCLUSIONS: The gel stent implant can be safely used in children with congenital glaucoma and effectively lowers intraocular pressure in these eyes. This procedure is a viable follow-up to, or replacement for, traditional angle surgery in eyes with congenital glaucoma.

Ahmed Valve Tube Extension in Pediatric Glaucoma.

PRéCIS:: Ahmed valve tube extender showed good long-term outcomes for retracted tubes in pediatric glaucoma. The procedure is safe with limited complications. PURPOSE: To analyze the long-term outcomes of Ahmed valve tube extension in pediatric glaucoma patients. PATIENTS AND METHODS: This study analyzed the records of all children who underwent surgery involving extension of a pediatric Ahmed valve tube, treated at a tertiary medical center between 2007 and 2018. Surgical success was defined as intraocular pressure between 6 and 22 mm Hg and reduced by at least 20% from its preoperative value, with or without intraocular pressure lowering medications, without additional surgical procedures and without vision loss. RESULTS: Fourteen eyes of 11 children were treated with the Ahmed tube extender during the study period. Mean age of all children was 5.7±4.7 years (range, 3 mo to 16 y). The most common type of glaucoma was congenital glaucoma (n=6, 42.9%). The mean age at the time of initial valve implant was 2.39±3.89 years and the mean duration from initial insertion to the extension was 3.39±2.95 years. The mean follow-up was 72.8±43.3 months (range, 12 to 140 mo), with 85.7% followed for >2 years. The mean survival was 34.1±37.2 months after the extension. Three surgeries (21.4%) were primary failures, 8 patients (57.1%) failed during follow-up (mean, 40.6±44.4 mo after the extension; range, 1 to 125 mo), whereas 3 patients (21.4%) maintained the successful outcome until their last follow-up visit (mean, 37.7±24.1 mo; range, 12 to 70 mo). CONCLUSIONS: The Ahmed valve extender can often save a retracted or obstructed tube. The procedure has limited complications and long-term efficacy in one third of the patients.

Influence of Axial Length on Intraocular Pressure Measurement With Three Tonometers in Childhood Glaucoma.

PURPOSE: To determine the agreement between intraocular pressure (IOP) measurements obtained using the handheld version of the Goldmann applanation (Perkins; Clement-Clarke, Haag-Streit, Harlow, United Kingdom), rebound Icare-Pro (Icare, Tiolat Oy, Helsinki, Finland), and Tonopen XL (Reichert Inc., Depew, NY) tonometers in children with childhood glaucoma and to identify factors that may affect those measurements. METHODS: Ninety-one eyes of 46 children with early-onset childhood glaucoma were included in this cross-sectional study in which IOP, ocular axial length, anterior chamber depth, lens thickness, vitreous length, and central corneal thickness measurements were obtained under general anesthesia. Agreement between tonometers was evaluated using intraclass correlation coefficients (ICCs) and the Bland-Altman method. The influence of ocular biometric parameters and central corneal thickness on IOP measurements was analyzed using multiple linear regression analysis. RESULTS: The mean age of the children in the current study was 29.1 months (range: 13 to 31 months). The Icare-Pro and Tonopen XL overestimated IOP measurements compared to the Perkins tonometer (Icare-Pro-Perkins mean IOP difference: 2.2 ± 3.4 mm Hg, P < .0001, 95% confidence interval [CI]: 1.5 to 2.9 vs Tonopen XL-Perkins mean IOP difference: 6.7 ± 7.1 mm Hg, P < .0001, 95% CI: 5.2 to 8.2). The Icare-Pro showed greater agreement with the Perkins tonometer than the Tonopen XL (ICC: 0.789, 95% CI: 0.697 to 0.856, P < .0001 vs 0.453, 95% CI: 0.272 to 0.603, P < .0001). Ocular axial length affected IOP measurements the most, finding increased impact on Tonopen XL (slope: 0.086, 95% CI: 0.013 to 0.16, P = .022 vs 0.997, 95% CI: 0.369 to 1.625, P = .002 vs 1.571, 95% CI: 0.541 to 2.602, P < .0001 for Perkins, Icare-Pro, and Tonopen XL IOP measurements, respectively). CONCLUSIONS: Ocular axial length affects IOP measured by the Perkins, Icare-Pro, and Tonopen XL devices in patients with childhood glaucoma. The Icare-Pro shows more agreement with the Perkins tonometer than the Tonopen XL; therefore, it seems to be a more suitable option for these patients. [J Pediatr Ophthalmol Strabismus. 2020;57(1):27-32.].

Surgical Outcomes of Microcatheter-assisted Trabeculotomy as a Secondary Procedure in Patients with Primary Congenital Glaucoma.

PURPOSE: To investigate the efficacy and safety of illuminated microcatheter-assisted trabeculotomy as a secondary procedure in patients with primary congenital glaucoma (PCG). METHODS: This retrospective case series included patients with PCG who underwent trabeculotomy using an illuminated microcatheter with the intent of catheterizing the full circumference of Schlemm's canal in a single procedure. Success was defined as intraocular pressure (IOP) ≤21 mmHg, with or without the use of glaucoma medications. Clinical examination data were collected for up to 36 months postoperatively. RESULTS: Surgery was performed on 16 eyes of 16 patients. The mean patient age was 75.1 ± 69.4 months (range: 4.0-216.0 months). Complete catheterization was achieved in 11 of the 16 eyes (69%), whereas partial catheterization was achieved in five of the 16 eyes (31%). All eyes had previously undergone surgery for PCG. The mean follow-up duration was 20.3 ± 9.0 months (range, 12.0-36.0 months). IOP was reduced from a mean of 31.8 ± 6.6 mmHg preoperatively to 15.6 ± 3.7 mmHg at the final follow-up (P < 0.001). The mean preoperative number of glaucoma medications was 3.9 ± 0.5, which was reduced to 1.1 ± 1.6 at the final follow-up (P = 0.001). Ten (62.5%) of the 16 eyes did not require glaucoma medication by the final follow-up. Early transient postoperative hyphema occurred in six eyes (37.5%). No other complications were noted. All corneas were clear at the final follow-up. CONCLUSION: Ab externo circumferential trabeculotomy using an illuminated microcatheter may be safe and effective as a secondary surgical option for children with PCG after unsuccessful glaucoma surgery.

Beyond Intraocular Pressure: Visual Functioning and Quality of Life in Primary Congenital Glaucoma and Secondary Childhood Glaucoma.

PURPOSE: To compare the visual functioning (VF) and vision-related QoL (VRQoL) of children 8-18 years old treated for primary congenital glaucoma (PCG) and secondary childhood glaucoma. DESIGN: Cross-sectional study. METHODS: A total of 309 children 8-18 years old treated for PCG and secondary childhood glaucoma between 2000 and 2010 by a single pediatric glaucoma specialist were prospectively enrolled at LV Prasad Eye Institute, Hyderabad, India. Children completed 2 questionnaires, the LV Prasad Functional Vision Questionnaire-II (LVP-FVQ-II), and the Impact of Vision Impairment-Children (IVI-C) questionnaire. Rasch-calibrated scores from both these questionnaires were used to compare the VF and VFQoL between the 2 groups. RESULTS: Mean ages of the children were 12.2 and 12.6 years in the PCG (53%, median age at diagnosis = 5 months) and secondary glaucoma groups (47%, median age at diagnosis = 3 years), respectively. A majority (80%) of children had bilateral glaucoma and underwent filtering surgery (83%). Mean better eye logMAR visual acuity (VA) was comparable between PCG and secondary childhood glaucoma groups (0.49 vs 0.52, respectively; P = 0.59). Children with PCG reported significantly better VF and VRQoL than secondary childhood glaucoma patients. Unadjusted and adjusted childhood glaucoma group comparisons revealed secondary childhood glaucoma to be associated with worse VF and VRQoL compared to PCG (difference for VF, -0.83; 95% confidence interval [CI], -1.34 to 0.31; P = 0.002; 0.39; 95% CI, 0.16-0.62; P = 0.001 for VRQoL). CONCLUSIONS: Results show that children with treated PCG experience significantly better VF and VRQoL than those with secondary childhood glaucoma, despite comparable VA and IOP.

Micropulsed diode laser cyclophotocoagulation in recurrent pediatric glaucoma.

PURPOSE: To evaluate the intermediate-term efficacy and safety of micropulsed diode laser cyclophotocoagulation in recurrent pediatric glaucoma. PATIENTS AND METHODS: A prospective interventional study included children <16 years old diagnosed with recurrent glaucoma, attending Mansoura University, during the period from July 2017 to November 2017. Micropulsed diode laser sessions were performed in all the cases. The main outcome was the intraocular pressure reduction with monitoring of complications as secondary outcome. The mean follow-up period was 15.08 ± 1.1 (mean: 12-16) months. RESULTS: A total of 36 eyes of 29 patients were included (62% males) with median age of 24 months. Primary congenital glaucoma represented 47.2% of the initial diagnoses. At the 15th month, the mean intraocular pressure dropped significantly from 37.5 ± 11.3 mmHg at baseline to 20.03 ± 2.7 mmHg (p < 0.001) with 37.15% reduction. The mean number of glaucoma medications decreased significantly from 2.6 ± 0.5 pretreatment to 1.7 ± 0.6 at the 15th month (p < 0.001). A total of 24 eyes (66.7%) required second session of treatment with mean number of 1.7 ± 0.5 sessions per eye. The cumulative probability of qualified success was 69.4%, 58.3%, 52.8%, 47.2%, and 41.7% at 1, 3, 6, 12, and 15 months after treatment. Qualified success was achieved in 61% at 15 months without statistically significant difference between the initial diagnoses (p = 0.61). None of the eyes developed any major ocular complications throughout the follow-up period. CONCLUSION: Micropulsed diode laser was proved to be a safe approach with relative effectiveness in controlling intraocular pressure in children with recurrent glaucoma.

Use of ab interno Kahook Dual Blade trabeculectomy for treatment of primary congenital glaucoma.

INTRODUCTION: Primary congenital glaucoma is a rare ocular disorder that is responsible for 0.01%-0.04% of total blindness worldwide.1 The goal of congenital glaucoma management is to allow for proper development of the immature visual system by controlling intraocular pressure. Medical therapy usually provides a supportive role to temporarily reduce intraocular pressure, but patients typically require iridocorneal angle surgery to facilitate aqueous humor outflow. In this report, we describe the use of minimally invasive ab interno Kahook Dual Blade trabeculectomy for treatment of primary congenital glaucoma. CASE DESCRIPTION: A 13-month-old male with bilateral primary congenital glaucoma due to a loss of function TEK mutation. He had bilateral findings of elevated intraocular pressures, buphthalmos, Haab's striae, photophobia, and myopia. Over the course of 6 weeks, three ab interno trabeculectomies with a Kahook Dual Blade were performed in the patient's left eye and one in the patient's right eye. After 3 months, intraocular pressures while receiving pressure reducing ophthalmic drops bilaterally reduced from 43 to 21 mmHg in the right eye after a single surgery and from 44 to 34 mmHg in the left eye after three surgeries, eventually requiring glaucoma drainage implant placement. There were no complications. CONCLUSION: Ab interno Kahook Dual Blade Trabeculectomy is a minimally invasive and potentially successful procedure for the treatment of congenital glaucoma. The safety profile of minimally invasive glaucoma surgery warrants consideration for congenital glaucoma patients, as they usually require iridocorneal angle surgery because pharmacologic therapy is typically inadequate.

Trabeculotomy Ab Interno With the Trab360 Device for Childhood Glaucomas.

PURPOSE: To report outcomes and complications of trabeculotomy ab interno using the Trab360 device (Trab360; Sight Sciences, Menlo Park, California, USA) in eyes with childhood glaucomas. DESIGN: Multicenter retrospective interventional case series. METHODS: Eyes with childhood glaucomas that underwent Trab360 with at least 3 months follow-up were evaluated. Postoperative intraocular pressure (IOP) less than or equal to 24 mm Hg with or without medications and no additional surgery defined success. RESULTS: Forty-six eyes of 41 patients were included. Median age at surgery was 12 months (range 1-325 months, mean 71 months); 54% prior to 20 months. A total of 48% were right eyes; 48% were male. Mean treatment was 290°. Median follow-up was 14.5 months (range 6-34 months, mean 16.2 months). Median preoperative IOP was 30 mm Hg (range 18-49 mm Hg, mean 30.9 mm Hg); median postoperative IOP was 18 mm Hg (range 5-40 mm Hg, mean 20.3 mm Hg]. Median number of preoperative glaucoma medications was 2.5 (range 0-5, mean 2.6); median number postoperatively was 1 (range 0-4, mean 1.6). Success was achieved in 67.4% (95% CI: 51.9%-80.0%) of eyes. Among 40 eyes for which Trab360 was the first glaucoma surgery, success rate was 70% (95% CI 53.3%-82.9%). Success was achieved in 81% (95% CI 57.4%-93.7%) of primary congenital glaucoma (PCG) eyes. Among 18 PCG eyes for which Trab360 was the first glaucoma surgery, success rate was 83.3% (95% CI 57.7%-95.6%). Two eyes (4.3%) suffered cyclodialysis. There were no other significant complications. CONCLUSIONS: Trab360 success resembles literature on other angle surgeries for childhood glaucomas. Good surgical technique and caution in high-risk angles is imperative to avoid cyclodialysis. Our study is limited by the imperfections inherent in any retrospective analysis. Single-incision ab interno trabeculotomy with the Trab360 device is effective and safe for treating childhood glaucomas, especially PCG.

Long-term functional outcomes of different subtypes of primary congenital glaucoma.

AIM: To analyse long-term visual outcomes across different subtypes of primary congenital glaucoma (PCG). METHODS: Patients with PCG with a minimum of 5-year follow-up post surgery were included in the study. Snellen visual acuity recordings taken at their last follow-up were analysed. We evaluated the results using Kaplan-Meier curves to predict the probability of maintaining good vision (as defined by a visual acuity of 6/18 or better) in our patients after 30-year follow-up. The results were also analysed to determine whether there were any differences in the long-term visual acuities with time between the neonatal and infantile PCG. We also analysed the reasons for poor visual outcomes. RESULTS: We assessed a cohort of 140 patients with PCG (235 eyes) with an average follow-up of 127±62.8 months (range 60-400 months). Overall, the proportion of eyes with good visual acuity was 89 (37.9%), those with fair visual acuity between 6/60 and 6/18 was 41 (17.4%), and those with poor visual acuity (≤6/60) was 105 (44.7%). We found a significant difference (p=0.047) between neonatal and infantile patients with PCG whereby the neonatal cohort fared worse off in terms of visual morbidity. On Kaplan-Meier analysis, the cumulative probability of survival of a visual acuity of 6/18 or better was more among the infantile PCG in comparison to the neonatal PCG (p=0.039) eyes, and more among the bilateral than the unilateral affected eyes (p=0.029). Amblyopia was the most important cause for poor visual acuity as shown on a Cox proportional-hazards regression model. CONCLUSIONS: Long-term visual outcomes of infantile are better than neonatal PCG. Eyes with unilateral have worse visual outcomes compared with those with bilateral PCG because of the development of dense amblyopia.

Longitudinal reproducibility of spectral domain optical coherence tomography in children with physiologic cupping and stable glaucoma.

PURPOSE: To determine whether Spectralis (Heidelberg, Germany) spectral domain optical coherence tomography (SD-OCT) measurements are reproducible over time in children with physiologic cupping and stable glaucoma. METHODS: Subjects were identified from a subset of participants in an earlier retrospective study conducted by our group and included children (<18 years of age) with physiologic cupping and stable primary congenital glaucoma (PCG) having had at least 2 SD-OCTs over a period of more than 1 between April 2010 and September 2015. Thicknesses of average peripapillary retinal nerve fiber layer (pRNFL) and six individual sectors and volumes of three segmented retinal layers and total retina were measured. Spectralis review software was used for segmentation. Intraclass correlation coefficients (ICC) and coefficient of variation (COV) were calculated. RESULTS: A total of 35 eyes of 35 children were included: 15 eyes had physiologic cupping; 20 eyes, PCG. Mean ages at initial SD-OCT were 11.2 ± 3.3 years and 9.7 ± 3.3, respectively; mean intervals between first and last imaging were 2.2 ± 1.1 and 3.0 ± 1.4 years, respectively. ICCs across three visits for both groups for average and sectoral pRNFL thicknesses were 0.887-0.997 and for segmented retinal volumes were 0.806-0.993. ICCs for total retinal volume for physiologic cupping and PCG were 0.993 and 0.954, respectively. COVs for average pRNFL thickness were 0.9% and 1.7%, respectively. For all other measurements, COVs ranged from 0.3% to 5.4%. CONCLUSIONS: Reproducibility of longitudinal SD-OCT measurements for average pRNFL thickness in children with stable glaucoma over about 2 years is comparable to short-term reproducibility (COV) in normal children (1.16%) and normal and glaucoma adults (1.62%-3.4%).

XEN-augmented Baerveldt Implantation for Refractory Childhood Glaucoma: A Retrospective Case Series.

BACKGROUND: Tube implants can lead to long-term decompensation of the cornea after decades, in particular in complicated childhood glaucoma, because of a variety of causes including contact between the tube tip and the corneal endothelium. The augmentation of a XEN implant with a Baerveldt (250) tube implant, introduced 2016 by Mermoud et al for refractory glaucoma in adult patients, may reduce this risk in children. In our retrospective study, we report on the XEN-augmented Baerveldt (250) tube implant in children. PATIENTS AND METHODS: Ten consecutive patients with refractory childhood glaucoma who underwent XEN-augmented Baerveldt implantation by a single surgeon (F.G.) between January 2016 and August 2018 were retrospectively analyzed. In this technique, a XEN tube was inserted under a standard scleral flap from outside into the anterior chamber and its outer end was connected to the Baerveldt (250) tube. The follow-up consisted of regular anterior and posterior segment examination with intraocular pressure (IOP) measurements using a hand-held slit lamp and a Perkins applanation tonometer and/or rebound tonometer (Icare). RESULTS: Ten cases (6 female individuals, 4 male individuals, median age 6 years) were operated using a XEN-augmented Baerveldt implant [aphakic glaucoma, primary congenital glaucoma, secondary congenital glaucoma (Peters anomaly), ectropion uveae, and Stickler syndrome were 4, 3, 1, 1, and 1]. Before surgery (XEN-augmented Baerveldt implantation), median IOP was 31 mm Hg. At the last postoperative follow-up, the mean IOP was 18.5 mm Hg with a median follow-up period of 13 months (range, 3 to 21 mo). No shallow or flat anterior chamber was observed. In all cases, this technique could be performed without intraoperative complications. In 3 cases, however, a surgical revision was necessary. There was no prolonged postoperative hypotony or short-term corneal alteration. By using an S-shaped loop the original Baerveldt tube length could be preserved for potential future use. In a total of 6 out of 10 cases, the procedure was a complete success (IOP, ≤20 mm Hg without additional medication). CONCLUSIONS: XEN-augmented Baerveldt implantation was used for refractory pediatric glaucoma in which conventional surgery had already failed. A new additional modification using an S-shaped loop was introduced. Short-term IOP control seems promising, but long-term results are still pending and the long-term efficacy and safety must still be proven before this technique can be generally recommended.

Childhood Glaucoma: Long-Term Outcomes of Glaucoma Drainage Device Implantation Within the First 2 Years of Life.

PRéCIS:: Glaucoma drainage device (GDD) implantation within the first 2 years of life yields an overall success rate of 59.0% at 5 years. It is safe and requires a relatively low number of postoperative interventions. PURPOSE: The purpose of this study was to evaluate the long-term outcomes of the treatment of childhood glaucoma with GDDs within the first 2 years of life. METHODS: A total of 43 children (60 eyes) having undergone GDD implantation within the first 2 years of life at Moorfields Eye Hospital between July 2005 and November 2014 were included in this retrospective case series. Kaplan-Meier survival curves were created for the evaluation of surgical success. Log-rank analysis was performed for the detection of risk factors for failure. MAIN OUTCOME MEASURES: Overall success rates at 1, 5, and 7 years after surgery. Surgical success: intraocular pressure ≥5/≤21 mm Hg, no further glaucoma surgery required, nonoccurrence of loss of perception of light or devastating complications. RESULTS: The results are listed as follows: Median duration of follow-up was 48.0 months. Median age at surgery was 11.5 months. Seventy-three percentage of children were white. Most common types of glaucoma were primary congenital glaucoma [(PCG); 67%], glaucoma following cataract surgery (18%), and anterior segment dysgenesis (10%). Overall success rates were 93%, 59%, and 59%, respectively. There were no differences in the survival rates as a function of PCG/non-PCG, sex, and removal of intraluminal stent suture. Mean number of general anesthesia administrations during the first postoperative year was 1.8. CONCLUSIONS: GDD implantation within the first 2 years of life is effective and safe. The low number of general anesthesia administrations required during the first postoperative year could help to reduce the burden placed on children and carers. Further research is required to directly compare the efficiency of GDD implantation with other surgical options.