Microcatheter–Assisted Circumferential Trabeculotomy in Primary Congenital Glaucoma: Long-Term Clinical Outcomes

Purpose: The purpose of this study was to report the long-term efficacy and clinical outcomes of microcatheter-assisted circumferential trabeculotomy (MCT) in children with primary congenital glaucoma (PCG). Methods: This is a single-center retrospective study including consecutive children with PCG who underwent MCT with > two years follow up. The primary outcome was surgical success, defined as intraocular pressure (IOP) ≤ 21 mmHg with (qualified) or without (complete) medications, measured at six months, one year, and then annually. Secondary outcomes were visual acuity (VA), refraction, axial length (AXL), complications, reinterventions, and number of medications. Results: Twelve eyes of ten patients were included. In eight children only one eye was affected. The mean ± standard deviation (SD) age at surgery was 6.3 ± 4.1 months. The mean postoperative follow-up was 66 ± 35 months. The mean IOP was 34.3 ± 9.6 mmHg preoperatively and 14.6 ± 2.3 mmHg postoperatively at the last visit (p < 0.001). Complete success was achieved at all time points in 10 out of 12 eyes, while 2 eyes had a qualified success. At three years of age, the mean VA of the operated eyes was 0.25 ± 0.12 logMAR, the mean spherical equivalent was −0.78 ± 1.43 diopters, and the mean AXL was 23.78 mm. Transient hyphema was the only complication observed. None of the children required additional glaucoma surgery. Conclusions: Circumferential trabeculotomy for PCG effectively lowers the IOP at more than two years after surgery. Following this procedure, the prognosis for the visual function is good, and the refractive error is low. Postoperative complications were not significant.

Co-existing lacrimal drainage anomalies in eyes with congenital Glaucoma

Aim To evaluate the occurrence of co-existing congenital nasolacrimal duct obstruction (CNLDO) and other lacrimal anomalies in eyes with congenital glaucoma (CG). Methods Retrospective analysis of children aged ≤1-year with co-existing CG and CNLDO seen between 1998 and 2019, treatment interventions and outcomes. Results During the study period, 1993 children had CG and 6203 children had CNLDO, among the children aged 1-year or less. Of these, 51 children (73 eyes) had co-existing CG and CNLDO. The prevalence of CNLDO in CG was 2.5% (51/1993) and the prevalence of CG among CNLDO was 0.8% (51/6203). Median age (Interquartile range, IQR) was 53 days (IQR: 8, 155), when they were diagnosed with CG. Among the children with CNLDO, 68 eyes (93.1%) had simple CNLDO, and 5 eyes (6.9%) had complex CNLDO. Associated lacrimal anomalies were present in 7/73 eyes, including congenital lacrimal fistula in two eyes, upper punctal agenesis and upper mid-canalicular obstruction in two eyes each, and upper single canalicular-wall hypoplasia in one eye. Lacrimal syringing and probing were successful in 69/72 eyes (95.8%), and failed in 3 eyes (4.2%). These 3 eyes had complex CNLDO and underwent Dacryocystorhinostomy (DCR) with Mitomycin-C and intubation. Sixty-two eyes underwent combined trabeculotomy and trabeculectomy, and two eyes underwent trabeculectomy. Median age at glaucoma surgery was 87 days (IQR: 54, 193). Median time delay for glaucoma surgery after probing was 18 days (IQR: 12, 35). Conclusion Among children aged ≤1-year presenting with congenital glaucoma coexisting lacrimal drainage anomalies was noted in 2.5% and simple CNLDO was the commonest. Early intervention is of paramount importance to prevent sight-threatening intraocular infection with CNLDO, and to minimize the delay of glaucoma surgery. The outcomes of lacrimal probing were excellent, however complex CNLDO required DCR.

Long-term outcomes in Primary congenital glaucoma, aniridia and anterior segment dysgenesis

Aim To determine the long-term outcomes of a cohort of complex patients with primary congenital glaucoma, aniridia and anterior segment dysgenesis. Methods Retrospective consecutive series between 1990–2021 in two UK tertiary centres: Guy's and St Thomas’ NHS Foundation Trust and King's College Hospital NHS Foundation Trust. We recorded the number and types of surgical and laser treatments along with preoperative and postoperative data, including intraocular pressures (IOP) and anti-glaucoma medications. Results A total of 41 eyes of 21 patients were included. Primary diagnoses were primary congenital glaucoma in 16 eyes (39.0%), aniridia in 14 eyes (34.2%), and anterior segment dysgenesis in 8 eyes (19.5%). Sixteen eyes (39.0%) had one or more glaucoma surgery or laser procedures for advanced glaucoma, and the long-term follow-up was 12.8 ± 3.6 years. There was a significant decrease in postoperative IOP (mmHg) at 3 months (16.5 ± 1.6; p = 0.0067), 6 months (18.7 ± 2.1; p = 0.0386), 12 months (18.6 ± 1.7; p = 0.0229), 3 years (14.7 ± 1.2; p = 0.0126), 5 years (15.5 ± 1.8; p = 0.0330) and 10 years (15.4 ± 2.3; p = 0.7780), compared to preoperatively (24.1 ± 2.6). Surgical success (complete and qualified) was 62.5%, 50.0%, 43.8%, 46.2%, 45.5% and 28.6% at 3 months, 6 months, 12 months, 3 years, 5 years and 10 years, respectively. There was no significant change in the number of anti-glaucoma drugs postoperatively ( p > 0.05). Four eyes (25.0%) had postoperative complications (hyphaema, hypotony) that resolved after conservative management. Conclusions Surgical management of these complex eyes with advanced glaucoma is challenging. Overall, the cohort had good surgical outcomes with a significant decrease in IOP by 36.1% after long-term follow-up.

Characteristics and long-term outcomes of childhood glaucoma: a retrospective-cohort study

Purpose: To evaluate the clinical characteristics and treatment outcomes of patients with childhood glaucoma. Methods: We retrospectively reviewed the data of patients with childhood glaucoma who visited the glaucoma clinics at the Queen Sirikit National Institute of Child Health and the King Chulalongkorn Memorial Hospital between January 2008 and January 2018. The diagnosis was based on the Childhood Glaucoma Research Network classification. We recorded their clinical characteristics and requirement of any glaucoma interventions. Results: A total of 691 eyes from 423 patients were included in this study. The patients predominantly comprised boys. The average follow-up duration was 71.3±63.8 months. The mean age at presentation was 3.9±4.4 years. Most patients presented with a high initial intraocular pressure (IOP). The average intial IOP of all patients was 28.5±11.2 mmHg. Glaucoma associated with non-acquired ocular anomalies (22.9%) was the most common subtype, followed by primary congenital glaucoma (20.8%). We recorded a family history of glaucoma in 6.4% of patients of the 234 patients with an available family history. Most patients had bilateral glaucoma (63.4%) and required at least one intervention (51.5%). The average IOP at the latest follow-up visit was 19.1±10.8 mmHg. All glaucoma types had significantly lower IOP, compared to that at their baselines (all p<0.001). Moreover, most patients had an unfavourable visual acuity (49.5%) at their latest visit. Conclusions: Secondary glaucoma associated with non-acquired ocular anomalies is the most common subtype of glaucoma. The majority of patients had unfavourable visual outcomes. These real-world findings are fundamental to acquire a better understanding of childhood glaucoma.

Pseudoretinoblastoma: Distribution Based on Gender, Age, and Laterality

Purpose: To investigate the distribution of pseudoretinoblastoma (PSRB) cases based on gender, age, and lateralityMethods: The clinical records of 607 patients (851 eyes) who were referred for suspicion of retinoblastoma between October 1998 and May 2021 were retrospectively evaluated. Patients were stratified by age as follows: ≤1 year, >1-3 years, >3-5 years, and >5 years.Results: PSRB conditions were detected in 190/607 (31.3%) patients. Of 190 patients, 129 (67.9%) were males and 61 (32.1%) were females (p=0.001). The 3 most common diagnoses were persistent fetal vasculature (PFV; 16.3%), Coats disease (15.3%), and optic nerve head drusen (ONHD; 5.3%). In males, the 3 most common diagnoses were Coats disease (20.2%), PFV (14.0%), and chorioretinal coloboma (6.2%). The 3 most common diagnoses in females included PFV (21.3%), retinal dysplasia, congenital glaucoma, and combined hamartoma (each accounting for 6.6%). PFV was the most common diagnosis in ≤1 year old patient group (26.6%). Coats disease and PFV were the most common diagnoses in >1-3 years old patient group (16.7%, for each diagnosis). Coats disease was the most common diagnosis in >3-5 years old (30.8%) and >5 years old patient groups (13.1%). PSRBs were unilateral in 121/190 (63.7%) patients and bilateral in 69/190 (36.3%). The most common unilateral and bilateral diagnoses were Coats disease (24.0%) and PFV (24.6%) respectively.Conclusion: In our study, 31.3% of patients referred for suspicion of retinoblastoma received the diagnosis of PSRB. PSRB spectrum encompasses different diseases, the distribution of which differ depending on gender, age, and laterality.

A case of congenital glaucoma in type I neurofibromatosis

BACKGROUND: Recklinghausens generalized neurofibromatosis (NF) is a hereditary disease characterized by the formation of benign tumors from the nervous tissue that provokes skin and bone changes of various types. Often, involvement in the pathological process and the organ of vision. The variability of eye lesions in NF-I type is known according to the clinical course, severity, and clinical forms. Gliomas and atrophy of the optic nerves, neurofibromas in the iris, sclera, conjunctiva, eyelids, less often glaucoma, buphthalmos, orbital osteodystrophy are described. RESULTS: The article describes a rare case of type I neurofibromatosis, the first clinical manifestation of congenital glaucoma. Modern complex ophthalmological examination made it possible to identify the cause and clarify the nature of the development of glaucoma. CONCLUSION: The need for an interdisciplinary approach to the diagnosis, dispensary observation, and treatment of NF-I is emphasized.