Pneumatic trabecular bypass versus trabeculotomy in the management of primary congenital glaucoma.

BACKGROUND: The optimal surgical management in primary congenital glaucoma (PCG) remains a subject of debate. The aim of this study was to assess efficacy of pneumatic trabecular bypass (PTB) in comparison to conventional trabeculotomy (T) in the treatment of PCG. METHODS: In a prospective comparative experimental study, one eye per child suffering from PCG underwent either PTB or T. Complete examinations were performed before surgery, postoperatively at one and seven days, then monthly for a minimum of six months. The main outcome measures were the IOP, number of IOP-lowering medications, change in cup/disc ratio, and corneal clarity. RESULTS: Seventeen eyes (patients) were operated on for PTB compared to 25 eyes (patients) in the T group. The mean (±SD, range) preoperative IOP in the PTB and T groups was 34.7 (6.4, 26-48) mmHg and 26.4 (6.6, 18-44) mmHg, respectively, and these dropped at six months of follow-up to 14.9 (3.6, 11-24) mmHg and 18.8 (8.0, 6-34) mmHg, respectively. The mean reductions of IOP were 55.87 %(±11) and 28.4 %(±28.8), (p = 0.001), where those for cup/disc ratio were 39.0 %(±29) and 17.5 %(±39.7) (p = 0.088) in the PTB and T groups, respectively. The number of IOP-lowering medications had dropped from 1.7 ± 0.5 to 0.7 ± 1.0 in PTB patients, compared to an insignificant change in the T group (0.64 ± 0.9 & 0.62 ± 0.9 pre- and post-operatively respectively). Corneal clarity improved in 13 eyes (76.5 %) in the PTB group, while three eyes (12 %) had worsened in the T group. Total cumulative chances for success were 88.2 % (15 out of 17 eyes), compared to 56 % (14 out of 25 eyes), in the PTB and T groups, respectively (p = 0.027). CONCLUSIONS: PTB is a promising surgical technique for the control of primary congenital glaucoma. A randomized controlled trial with a longer follow-up is recommended.

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.

Comparison of dynamic contour tonometry and Goldmann applanation tonometry in relation to central corneal thickness in primary congenital glaucoma.

BACKGROUND: To compare intraocular pressure (IOP) measurements obtained with dynamic contour tonometer (DCT) and Goldmann applanation tonometer (GAT), and to investigate their relationship to central corneal thickness (CCT) in primary congenital glaucoma (PCG) eyes. METHODS: Thirty-one eyes of 31 PCG patients (25.7 ± 7.2 years old) were examined. PCG was defined as elevated IOP, enlarged corneal diameter (buphthalmos), Haab's striae and abnormal findings at gonioscopy. The mean of three measurements of GAT, DCT (quality scores 1 and 2), and CCT were obtained and assessed for agreement by means of Bland-Altman plot and for Spearman correlation test. RESULTS: Mean CCT was 534 ± 72.3 µm (range: 430 to 610 µm). Mean IOP measurements were 15.1 ± 4.2 mmHg (range: 5.5 to 22.7 mmHg) for DCT and 14.5 ± 5.6 mmHg (range: 7.0 to 34.0 mmHg) for GAT (P = 0.244). Spearman correlation tests showed that IOP difference (DCT - GAT) was not correlated with CCT (r (2) = 0.023, P = 0.417). IOP measurements by DCT were weakly but statistically correlated with those obtained with GAT (r(2) = 0.213, P = 0.0089). Bland-Altman analysis revealed poor agreement between DCT and GAT readings, considering the 95 % confidence intervals of ± 10.45 mmHg. CONCLUSIONS: The differences between DCT and GAT readings were not influenced by CCT in this series of patients. Considering the weak correlation and the poor agreement observed between GAT and DCT measurements and that they both may be affected by corneal biomechanical changes, these methods should not be used interchangeably, and may possibly give no meaningful IOP values in PCG patients.

Bilateral buphthalmia in a 4-month-old Texas longhorn steer.

Congenital ocular disease occurs uncommonly in cattle, with multiple abnormalities reported only sporadically in the literature. This report describes a case of anterior segment dysgenesis resulting in glaucoma in a 4-month-old Texas Longhorn steer. On clinical exam, bilateral buphthalmia was present and intraocular pressures exceeded 47 mm Hg in both eyes. On histopathologic examination, the iridocorneal angle and filtration apparatus were distorted due to collapse of the ciliary cleft and anterior displacement of the anterior portion of the ciliary body. No evidence of inflammation or other causes of glaucoma were recognized.

Shrinkage of the scleral canal during cupping reversal in children.

PURPOSE: The mechanism of cupping reversal seen after lowering intraocular pressure (IOP) in pediatric glaucoma is unknown. Theories include forward movement of the lamina cribrosa or shrinkage of a stretched scleral canal. Our study aimed to quantify changes in optic disc size occurring in children who had undergone glaucoma surgery. DESIGN: Retrospective, single-center, observational case series. PARTICIPANTS: Children undergoing incisional surgery for pediatric glaucoma at the University of California, Davis. METHODS: The electronic charts of all patients with pediatric glaucoma were reviewed for the presence of RetCam digital optic nerve photographs (Clarity Medical Systems, Pleasanton, CA). Cases in which the photographs (baseline and follow-up after surgical intervention) were of sufficient quality were analyzed. The optic disc margin was outlined manually using ImageJ software. Inter-session changes in magnification were accounted for by drawing a control polygon joining 4 or 5 fixed landmarks (e.g., vessel crossings) to include a second larger area containing the optic nerve. The optic disc area (in pixels adjusted with the control polygon) was compared between baseline and follow-up images. MAIN OUTCOME MEASURES: Change in disc area between baseline and follow-up after surgery. RESULTS: We identified 29 eyes for which baseline and follow-up images were available for analysis. Fifteen eyes of 9 children showed clinically obvious cupping reversal. Fourteen eyes of 12 children showed no cupping reversal. Disc area decreased by 6.8% (95% confidence interval [CI], -10.0 to -3.3) in the obvious reversal group and increased by 4.3% (95% CI, +1.0 to +7.6) in the no reversal group after surgery (P < 0.0001; Student t test). Percent change in disc area is correlated to percent change in IOP (r=0.540; P=0.0025) and axial length (r=0.534; P=0.0028). CONCLUSIONS: When cupping reversal is clinically apparent after successful IOP-lowering surgery for congenital glaucoma, the scleral canal shrinks in area. In contrast, when cupping reversal is not observed, the scleral ring continues to enlarge, indicating ongoing stress on the optic nerve. Clinically obvious cupping reversal is less frequently observed in adults after surgery, which may reflect a lower elasticity of the scleral ring in adults compared with children. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Surgical results in the management of advanced primary congenital glaucoma in a rural pediatric population.

OBJECTIVE: To present the anatomic and functional results of surgical treatment for advanced primary congenital glaucoma (PCG) in a rural setting. DESIGN: Retrospective, observational case series. PARTICIPANTS: Forty eyes of 22 consecutive patients diagnosed with PCG and who underwent surgical treatment. METHODS: All eyes underwent surgical treatment for PCG. Type of surgery and postoperative complications were noted. We examined anatomic and functional indices before and after the operation. MAIN OUTCOME MEASURES: Visual acuity (VA), intraocular pressure (IOP), refractive spherical error, cup-to-disc ratio (CDR), and horizontal corneal diameter (HCD). RESULTS: Average age at the time of surgery was 3.3 years (range, 0.4-10) and the mean follow-up was 6 months (range, 1-11). 15% showed marked lens dislocation owing to the severe buphthalmos at presentation. Preoperative IOP was 54 ± 2 mmHg, HCD was 15.1 ± 0.3 mm, and CDR (when visible) was 0.8 ± 0.02. Surgical intervention included 31 trabeculotomies, 6 Ahmed Glaucoma Valve implants, 1 goniotomy, and 2 eviscerations. Serious complications were noted in 4 eyes. Final postoperative IOP was 23 ± 2 mmHg (P < 0.0001). Patients were significantly more likely to have ambulatory VA (following objects or better) after operation (18% vs 64%; P < 0.0001). CONCLUSIONS: Our surgical outcomes in children with advanced PCG demonstrated moderate overall improvement in IOP and modest improvement of VA. Deep deprivation amblyopia, severe disease manifestation at presentation, opaque corneas, and frequent lens dislocation limited the possible success. These data further signify the need for effective, timely screening of children and prompt recognition by health care workers to reduce the rate of avoidable blindness in developing countries.

Outcomes of goniotomy for primary congenital glaucoma in East Africa.

OBJECTIVE: To evaluate the effectiveness of goniotomy to treat primary congenital glaucoma in an east African population. DESIGN: A retrospective series using case records. For patients who underwent goniotomies in both eyes, only the first was analyzed. PARTICIPANTS: Forty-seven children undergoing goniotomy (47/80 operated eyes analyzed). Thirty-six children (77%) were male. Mean age was 19 months (range 1-204 months, standard deviation [SD] = 35). INTERVENTION: First goniotomy was performed from the temporal side. Repeat surgery, if necessary, was performed via nasal side approach and 100% ethanol-soaked cotton-tipped swabs were used to clear the corneal epithelium when necessary for angle visualization. MAIN OUTCOME MEASURES: Postoperative intraocular pressure (IOP) <21 mmHg was considered success. Potential risk factors for failure were analyzed. Quantitative visual acuities were measured with Cardiff acuity cards and Lea tests in older children. RESULTS: A total of 36 of 47 children (77%) returned for follow-up. Mean follow-up interval was 12 months (range, 1-62 months; SD = 15). A total of 28 of 47 children (60%) had >3 months of follow-up, and 11 children had >1 year of follow-up. A total of 12 of 47 eyes (26%) required repeat goniotomy. Three of these required further trabeculectomy with mitomycin C, 1 of which required eventual enucleation after failure to control IOP, enlargement, and pain. A total of 11 of 28 children (39%) with >3 months follow-up required repeat surgery. A total of 24 of 28 children (86%) with follow-up intervals of at least 3 months achieved postoperative IOPs of <21 mmHg. Mean IOPs decreased (t = 6.1, P < 0.0005) from 23.4 mmHg (SD = 5.3 mmHg) to 14.4 mmHg (SD = 7.6 mmHg). Only 1 predictor of failure to achieve successful control of IOP was identified: female gender (relative risk = 5.3, 95% confidence interval, 1.1-26.3). Corneas were clear in 4 of 47 eyes (8.5%) preoperatively and 32 of 41 eyes (78%) postoperatively. Of 25 children with preoperative and postoperative acuities measured, 22 (88%) recorded improvement. CONCLUSIONS: Preliminary results of goniotomy in an African population with advanced disease and late presentation are reasonably encouraging. Overall results are comparable with historical drainage procedures, with the advantage of preserving conjunctiva. Further studies are justified.

Comparison of latanoprost and timolol in pediatric glaucoma: a phase 3, 12-week, randomized, double-masked multicenter study.

OBJECTIVE: To compare the efficacy and safety of latanoprost versus timolol in pediatric patients with glaucoma. DESIGN: Prospective, randomized, double-masked, 12-week, multicenter study. PARTICIPANTS: Individuals aged ≤18 years with glaucoma. METHODS: Stratified by age, diagnosis, and intraocular pressure (IOP) level, subjects were randomized (1:1) to latanoprost vehicle at 8 am and latanoprost 0.005% at 8 pm or timolol 0.5% (0.25% for those aged <3 years) twice daily (8 am, 8 pm). At baseline and weeks 1, 4, and 12, IOP and ocular safety were assessed and adverse events were recorded. Therapy was switched to open-label latanoprost pm and timolol am and pm for uncontrolled IOP. MAIN OUTCOME MEASURES: Mean IOP reduction from baseline to week 12. Latanoprost was considered noninferior to timolol if the lower limit of the 95% confidence interval (CI) of the difference was >-3 mmHg. A proportion of responders (subjects with ≥15% IOP reduction at weeks 4 and 12) were evaluated. Analyses were performed in diagnosis subgroups: primary congenital glaucoma (PCG) and non-PCG. RESULTS: In total, 137 subjects were treated (safety population; 12-18 years, n=48; 3-<12 years, n=55; 0-<3 years, n=34). Mean age was 8.8±5.5 years, and mean baseline IOP was 27.7±6.17 mmHg; 125 subjects completed the study, and 107 subjects were in the per protocol population. Mean IOP reductions for latanoprost and timolol at week 12 were 7.2 and 5.7 mmHg, respectively, with a difference of 1.5 mmHg (95% CI, -0.8 to 3.7; P=0.21). Responder rates were 60% for latanoprost and 52% for timolol (P=0.33). Between-treatment differences in mean IOP reduction for PCG and non-PCG subgroups were 0.6 mmHg (95% CI, -2.3 to 3.4) and 2.6 mmHg (95% CI, -0.8 to 6.1), respectively. Responder rates for latanoprost versus timolol were 50% versus 46% for the PCG group and 72% versus 57% for the non-PCG group. Both therapies were well tolerated. CONCLUSIONS: Latanoprost 0.005% is not inferior (i.e., is either more or similarly effective) to timolol and produces clinically relevant IOP reductions across pediatric patients with and without PCG. Both latanoprost and timolol had favorable safety profiles over the duration of this 3-month trial. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

Combined trabeculotomy-trabeculectomy augmented with 5-fluorouracil in paediatric glaucoma.

BACKGROUND: To describe our experience of combined trabeculotomy-trabeculectomy in paediatric glaucomas with a special emphasis on the use of 5-fluorouracil and releasable sutures. DESIGN: Retrospective review carried out at Manchester Royal Eye Hospital, UK, a tertiary referral centre. PARTICIPANTS: Twenty-nine eyes of 21 consecutive patients with congenital glaucoma undergoing combined trabeculotomy-trabeculectomy augmented with 5-fluorouracil. METHODS: 5-Fluorouracil augmented combined trabeculotomy-trabeculectomy was carried out with intense postoperative management and suture adjustment of releasable sutures within the first 3 weeks after surgery. Peribleb 5-fluorouracil injections were given repeatedly if there were signs of aggressive bleb scarring. MAIN OUTCOME MEASURES: Absolute success was defined as intraocular pressure of 21 mmHg or less, clear cornea and absence of progressive glaucomatous optic disc changes at last follow up, whereas qualified success was defined as these endpoints with anti-glaucoma medication. RESULTS: Absolute success was achieved in 19 out of 29 eyes (65.5%), and a further 4 (13.8%) had qualified success. There was no difference in the surgical outcomes of primary infantile glaucoma and secondary causes of paediatric glaucoma such as anterior segment dysgenesis. Combined trabeculotomy-trabeculectomy had a significantly greater success rate as a secondary procedure rather than as a primary procedure. CONCLUSION: 5-Fluorouracil-enhanced combined trabeculotomy-trabeculectomy with releasable sutures appears to be an effective procedure for congenital glaucoma refractory to goniotomy. It is less effective as a primary procedure when severe corneal haze prevents goniotomy in newborn congenital glaucoma. Intense postoperative monitoring including active bleb manipulation with needling and 5-fluorouracil injections may increase the success of the procedure.

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.

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.

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.

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.].

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.

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.

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.