Efficacy of Ab Interno Revision for Trabeculectomy Bleb Failure using a Minimally Invasive Approach: A Prospective Study.

PRCIS: Ab interno bleb revision using the Grover and Fellman spatula offers potential benefits in improved intraocular pressure control and spare the conjunctiva for enhanced bleb management. PURPOSE: This study aimed to evaluate the safety and effectiveness of Ab-Interno Bleb Revision (AIBR) for treating failed trabeculectomy blebs. METHODS: This non-comparative case series enrolled glaucoma patients with failed trabeculectomy blebs, who underwent AIBR and were followed up for one year. Eligible patients received subconjunctival Mitomycin C 0.1 mL of 0.2 mg/mL (20 µg) five days before the AIBR, performed using the Grover and Fellman spatula. The primary outcome measures were intraocular pressure (IOP) and the usage of IOP-lowering medications one year after the procedure. Secondary outcomes included the procedure's failure rate and complications. Complete success was defined as achieving an IOP between 5-21 mmHg without medication, while qualified success required additional medical treatment. RESULTS: The study comprised 23 eyes from 23 patients, having a mean age of 59.66±14.93 years. Preoperatively, the mean IOP was 23.54±10.7 mmHg, and the mean anti-glaucoma medication requirement was 3.99±0.65, both significantly reduced to 15.7±6.9 mm Hg (P=0.009) and 1.26±1.2 (P <0.001) at one year, respectively. Overall, 19 eyes (82.6%) achieved success (Complete success: 39.2%, Qualified success: 43.4%), while in 4 (17.4%) patients the procedure failed (IOP >21 mmHg). 5 patients had transient hyphema, but there were no sight-threatening complications such as hypotony or choroidal detachment. CONCLUSION: Ab-Interno Bleb Revision (AIBR) is a safe, effective, and minimally invasive intervention for managing failed trabeculectomy blebs. By eliminating the need to reopen the conjunctiva, this technique offers a promising alternative for the treatment of this challenging condition.

CYP1B1 and MYOC variants in neonatal-onset versus infantile-onset primary congenital glaucoma.

OBJECTIVE: To compare CYP1B1 and MYOC variants in a cohort of neonatal-onset (NO) and infantile-onset (IO) primary congenital glaucoma (PCG). METHODS: This prospective observational study included 43 infants with PCG (14 NO and 29 IO) presenting between January 2017 and January 2019 with a minimum 1-year follow-up. CYP1B1 and MYOC genes were screened using Sanger sequencing with in-silico analysis of the variants using Polymorphism Phenotyping v.2 and Protein Variation Effect Analyser platforms. Allelic frequency was estimated using Genome Aggregation Database (gnomAd). Disease presentation and outcome were correlated to the genetic variants in both groups. RESULTS: Babies with CYP1B1 mutations had more severe disease at presentation and worse outcomes. Six of 14 (42.8%) NO glaucoma and 5 of 29 (17.2%) IO harboured CYP1B1 mutations. Five of six babies in the NO group and three of five in the IO group harboured the variant c.1169G>A, [p.R390H]. They required more surgeries and had a poorer outcome. On in-silico analysis c.1169G>A, [p.R390H] scored very likely pathogenic. Two patients in the IO group who had the c.1294C>G, [p.L432V] variant had a good outcome. Five of 14 NO-PCG and 8 of 29 IO-PCG harboured the variant c.227G>A, [p.R76K] in the MYOC gene, which was scored benign by in-silico analysis, and was also found in 2 of 15 normal controls. CONCLUSIONS: Patients with CYP1B1 pathogenic variants had a poorer outcome than those without. We found more NO PCG babies with CYP1B1 mutations compared with IO PCG. This may be one of the reasons for NO PCG having a poorer prognosis compared with IO PCG.

Primary aphakia: clinical recognition is the key to diagnosis.

PURPOSE: To describe the presentation and treatment outcomes of a cohort of children with primary aphakia (PA). METHODS: Clinical photographs and ultrasound biomicroscopy (UBM) images of children presenting with sclerocornea and undetermined anterior segment dysgenesis between July 2017 and December 2020 were reviewed retrospectively. Children who had no crystalline lens visible on UBM were included. RESULTS: A total of 124 UBM images were captured for 124 children with cloudy corneas. Twelve children were identified with congenital primary aphakia: 5 had bilateral buphthalmos, 2 had buphthalmos in one eye and microphthalmos in the other, and 5 had bilateral sclerocornea-microphthalmia complex. All patients had a peculiar silvery-blue corneal appearance, with fine vascularization on the corneal surface. The overall corneal thickness was 409.1 ± 8.7 µm. The intraocular pressure (IOP) in eyes with glaucoma was 24.5 ± 7.3 mm Hg; in microphthalmic eyes, 11.4 ± 3.4 mm Hg (P <0.001). The raised IOP was treated with limited trans-scleral cyclophotocoagulation under transillumination and topical antiglaucoma medications. Children with glaucoma gained ambulatory vision with spectacles. CONCLUSIONS: Congenital primary aphakia has a characteristic clinical appearance and may present as buphthalmos or microphthalmos, depending on the extent of dysgenesis. Incisional surgery may result in phthisis because of ciliary body dysgenesis and unpredictable aqueous production.

Unilateral advanced glaucoma in isolated congenital ectropion uveae with ipsilateral ptosis: A pictorial description of five children.

Aim: To report the cases of five children with unilateral advanced glaucoma in isolated congenital ectropion uveae (CEU) with ipsilateral ptosis and myopia. Methods: This is an ambispective observational case series. After diagnosing one patient with CEU and glaucoma, consecutive patients presenting with unilateral ptosis, congenital iris anomaly, and glaucoma between 2014 to 2020, and had completed a minimum one-year postoperative follow-up, were analyzed. Results: Of the 1421 newly registered pediatric glaucoma patients in the period under review, five children were diagnosed with CEU. All patients presented with gradual painless diminution of vision in the left eye in early adolescence. The left eye of all patients had peculiar clinical features: mild congenital ptosis, high iris insertion, crypt-less smooth iris surface, congenital ectropion uveae, pigments over anterior lens capsule, high myopia, advanced glaucomatous optic disc cupping, and very high intraocular pressure (IOP), which was > 45 mmHg in all cases. The right eye showed signs of angle dysgenesis with mild anterior iris insertion and numerous fine iris processes. Antiglaucoma medications and angle surgery failed to control the IOP, and all children required glaucoma filtration surgery, resulting in reasonable IOP control. Despite the older age, postoperative strict amblyopia treatment resulted in significant improvement in vision. Conclusions: Although ectropion uveae and ptosis have been present since birth, unilaterality, and the asymptomatic nature of the disease led to the late presentation with irreversible damage. Early surgical management and amblyopia therapy are the cornerstones of management. Abbreviations: CEU = Congenital ectropion uvea, CIES = Congenital Iris Ectropion Syndrome, ASD = Anterior segment dysgenesis syndrome, BCVA = Best-corrected visual acuity, IOP = Intraocular pressure.

Clinical profile and outcome of early surgery in neonatal-onset glaucoma presenting over a 5-year period.

BACKGROUND: Neonatal-onset glaucoma (NOG) is a severe form of childhood glaucoma and is not always due to primary congenital glaucoma (PCG). Due to advances in neonatal care, the incidence of NOG is rising, but it remains an under-reported entity. The objective of the paper was to study the clinical profiles, surgical and visual outcomes of NOG at least 1 year following early surgery. METHODS: Prospective interventional cohort study at a tertiary care referral centre. Babies with NOG, who presented between January 2013 and December 2017, had a history suggestive of disease onset within 1 month of birth, and underwent surgery by 3 months of age, were prospectively enrolled. Those who completed a 1-year follow-up after surgery were analysed. RESULTS: 94 eyes of 53 babies were analysed. 35 (66%) had PCG. Neonatal congenital ectropion uveae, congenital rubella syndrome, Peter's anomaly and Sturge-Weber syndrome comprised the non-PCG group. The mean age at presentation and surgery was 24.8±21.9, and 36.7±29.9 days. Additional glaucoma surgery was required in 43 of the 94 eyes (45.7%). PCG had significantly better outcomes than other glaucomas at all time points. 28.3% of eyes had good vision (LogMar (0-0.5)), 34.7% had moderate visual impairment (LogMar 0.7-1.0) and 16% were blind (LogMar <1.62) . CONCLUSION: Our study shows that NOG does not always have a dismal prognosis. A small but significant proportion could have other underlying conditions than PCG. Timely surgery and rigorous amblyopia therapy resulted in good outcomes in terms of intraocular pressure control and vision in this cohort.

Goniotomy for initial and re-surgery for childhood glaucoma in Northern India.

Purpose: The aim of this study was to describe the effectiveness of goniotomy for childhood glaucoma in Indian eyes. Methods: Consecutive patients with pediatric glaucoma who underwent goniotomy between July 2017 and June 2020, in a single center in Northwest India were prospectively analyzed. Goniotomy was done as a primary procedure or a re-do surgery of the untreated angle in failed filtering surgery. Success was defined as intraocular pressure (IOP) ≦18 mm Hg with or without 2 topical medications. Results: A total of 172 eyes of 126 children underwent goniotomy during this period (37.9% of all pediatric glaucoma surgeries). Goniotomy comprised 132 of 211 (62.5%) primary pediatric glaucoma surgeries and 40 of 243 (16.5%) re-do surgeries. 145, 112, and 54 eyes had a six months, 1-year and 2-year follow-up, respectively. At 1 year, success rates in Primary Congenital Glaucoma (PCG) were 79.7% for primary surgery and 68.4% for re-do surgery. For non-PCG eyes, the success rate was 62% at 1 year. Among PCG subgroups, infantile and newborn glaucoma had 87.5% and 57.1% success rates, respectively. On logistic regression analysis, lower baseline IOP and lesser axial length at presentation were significantly predictive of successful outcomes (P = 0.03 and P = 0.02, respectively). At 1 year, in the primary surgery group, 50% had good vision (better than logMAR 0.5), 28.9% had moderate (better than LOGMAR 1.0) and 20% had severe visual impairment. There were no significant intraoperative or post-operative complications. Conclusion: Goniotomy appears to be an effective surgery for childhood glaucoma in Indian eyes. Being minimally invasive, it obviates the need for conjunctival and scleral dissection and antifibrotic agents.

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.

Evaluation of Intranasal Dexmedetomidine as a Procedural Sedative for Ophthalmic Examination of Children With Glaucoma.

PRECIS: This study evaluated 2 doses of intranasal dexmedetomidine (IND) (3.0 and 3.5 µg/kg) as a procedural sedative for postoperative examination of children with glaucoma. A dose of 3.5 µg/kg was more efficacious and obviated the need for repeated general anesthesia. PURPOSE: This study was carried out to determine the safety and effective dose of IND as a procedural sedative for postoperative follow-up examinations after glaucoma surgery in children in place of repeated examination under anesthesia. MATERIALS AND METHODS: In this prospective randomized double-blinded interventional study, consecutive children aged 6 months to 6 years were randomized to receive 3.0 and 3.5 µg/kg IND using a mucosal atomizer device in the preoperative area of the operating room, under continuous monitoring of vital signs. Intranasal midazolam 0.25 mg/kg was used as a rescue agent in case of inadequate sedation, and general anesthesia was administered in case of persistent failure. All infants underwent a complete anterior and posterior segment evaluation including intraocular pressure and corneal diameter measurements. RESULTS: A total of 30 and 31 children aged 23.9±15.0 and 19.2±10.1 months, respectively, received 3.0 and 3.5 µg/kg IND. Adequate sedation was possible in 18 of 30 (60%) children receiving 3.0 µg/kg and 24 of 31 (77.4%) receiving 3.5 µg/kg IND alone (P=0.17). In combination with midazolam, successful sedations were 86.6% versus 100%, respectively (P=0.052). One patient in the 3.5 µg/kg group had ventricular arrhythmia, reversed with dextrose-saline infusion and injection glycopyrrolate. CONCLUSIONS: IND appears to be a safe and effective procedural sedative for postoperative follow-up examinations of pediatric glaucoma patients at doses of 3 and 3.5 µg/kg. The dose of 3.5 µg/kg was successful in more children.