UNASSIGNED: Primary congenital glaucoma (PCG) is a challenging condition to diagnose, treat and effectively monitor. Serial assessment of intraocular pressure (IOP), optic disc cupping, refraction, and axial length (AxL) after surgery are useful to assess disease control. This study aimed to evaluate AxL changes in relation to IOP changes following glaucoma surgery in children with PCG.
UNASSIGNED: We retrospectively studied AxL changes in children with PCG undergoing surgery. Eyes of children aged ≤ 4 years that did not have prior ocular surgery and that underwent at least one glaucoma surgery during the course of follow-up between June 2014 and July 2018, were included. The effect of change in IOP on change in AxL was estimated using linear mixed effects models.
UNASSIGNED: A total of 105 eyes (of 72 children) with PCG underwent glaucoma surgery representing 26.4% (105/397) eyes. The mean ± SD age of children at baseline was 3.53 ± 4.04 months. At baseline, the mean IOP and AxL were 26.63 ± 9.57 mmHg and 21.67 ± 1.82 mm, respectively. During the course of follow-up post-surgery, the IOP decreased by a mean of 7.25 ± 12.08 mmHg while the AxL increased by a mean of 0.70 ± 1.40 mm. A multivariable mixed effects linear regression revealed that change in AxL was significantly associated with change in IOP (p=0.030) and time since first surgery (p<0.001). A substantial reduction in IOP (≥35 mmHg) was needed at 3 months post-surgery, for AxL to regress.
UNASSIGNED: In children with PCG who undergo glaucoma surgery, change in IOP significantly influences change in AxL. For AxL to regress, a substantial reduction in IOP is needed post-surgery.

Childhood glaucoma (CG) encompasses a heterogeneous group of genetic eye disorders that is responsible for approximately 5% of childhood blindness worldwide. Understanding the molecular aetiology is key to improving diagnosis, prognosis and unlocking the potential for optimising clinical management. In this study, we investigated 86 CG cases from 78 unrelated families of diverse ethnic backgrounds, recruited into the Genomics England 100,000 Genomes Project (GE100KGP) rare disease cohort, to improve the genetic diagnostic yield. Using the Genomics England/Genomic Medicine Centres (GE/GMC) diagnostic pipeline, 13 unrelated families were solved (13/78, 17%). Further interrogation using an expanded gene panel yielded a molecular diagnosis in 7 more unrelated families (7/78, 9%). This analysis effectively raises the total number of solved CG families in the GE100KGP to 26% (20/78 families). Twenty-five percent (5/20) of the solved families had primary congenital glaucoma (PCG), while 75% (15/20) had secondary CG; 53% of this group had non-acquired ocular anomalies (including iris hypoplasia, megalocornea, ectopia pupillae, retinal dystrophy, and refractive errors) and 47% had non-acquired systemic diseases such as cardiac abnormalities, hearing impairment, and developmental delay. CYP1B1 was the most frequently implicated gene, accounting for 55% (11/20) of the solved families. We identified two novel likely pathogenic variants in the TEK gene, in addition to one novel pathogenic copy number variant (CNV) in FOXC1. Variants that passed undetected in the GE100KGP diagnostic pipeline were likely due to limitations of the tiering process, the use of smaller gene panels during analysis, and the prioritisation of coding SNVs and indels over larger structural variants, CNVs, and non-coding variants.

Childhood glaucoma, a significant cause of global blindness, represents a heterogeneous group of disorders categorized into primary or secondary forms. Primary childhood glaucoma stands as the most prevalent subtype, comprising primary congenital glaucoma (PCG) and juvenile open-angle glaucoma (JOAG). Presently, multiple genes are implicated in inherited forms of primary childhood glaucoma. This comprehensive review delves into genetic investigations into primary childhood glaucoma, with a focus on identifying causative genes, understanding their inheritance patterns, exploring essential biological pathways in disease pathogenesis, and utilizing animal models to study these mechanisms. Specifically, attention is directed towards genes such as CYP1B1 (cytochrome P450 family 1 subfamily B member 1), LTBP2 (latent transforming growth factor beta binding protein 2), TEK (TEK receptor tyrosine kinase), ANGPT1 (angiopoietin 1), and FOXC1 (forkhead box C1), all associated with PCG; and MYOC (myocilin), associated with JOAG. Through exploring these genetic factors, this review aims to deepen our understanding of the intricate pathogenesis of primary childhood glaucoma, thereby facilitating the development of enhanced diagnostic and therapeutic strategies.

UNASSIGNED: This study aims to present two different types of giant bleb formation following Ahmed Glaucoma Valve (AGV) implantation: an anterior enlarged giant bleb and a posterior enlarged giant bleb.
UNASSIGNED: In Case 1, a 70-year-old Japanese male underwent AGV implantation for neovascular glaucoma in his right eye (OD). Preoperatively, the patient\'s intraocular pressure (IOP) and best corrected visual acuity (BCVA) were 23 mmHg and 0.6, respectively, OD, while using 3 antiglaucoma topical medications. Two months post-surgery, the patient began experiencing double vision. Slit lamp evaluation revealed no abnormalities, IOP and BCVA were 24.0 mmHg and 0.8, respectively, OD. A posteriorly enlarged bleb in the superotemporal quadrant OD was found to be causing displacement on T2-weighted orbital MRI. The patient underwent surgical excision of the anterior bleb wall. By three weeks post-surgery, the double vision resolved; IOP and BCVA were 17 mmHg and 0.7, respectively, and a normal bleb in the slit lamp evaluation was identified OD. In Case 2, a 10-year-old Japanese female underwent AGV implantation for childhood glaucoma associated with congenital cataract OD. Preoperatively, IOP and BCVA were 30 mmHg and 0.5, respectively, OD, while using 3 antiglaucoma topical medications. She underwent pars plana vitrectomy (PPV) in addition to AGV implantation. Seven months post-surgery, slip lamp evaluation revealed an anteriorly enlarged giant bleb that only cause her a cosmetic concern.
UNASSIGNED: There are two types of giant bleb formation following AGV implantation based on the direction of the enlargement: an anterior enlarged giant bleb and a posterior enlarged giant bleb. The introduction of this classification contribute to better understanding and management of this unusual surgical complication.

BACKGROUND: Childhood glaucoma is a major cause of childhood blindness worldwide. The profile of childhood glaucoma has not been well characterized in sub-Saharan Africa. Thus, this study was designed to describe demographics, clinical features, managements of childhood glaucoma, and improvements in visual acuity (VA) and intraocular pressure (IOP) from baseline to final visit.
METHODS: This retrospective study included glaucoma patients below 18 years old who were diagnosed between September 2019 to August 2022. Childhood glaucoma diagnosis and classification was made as per the Childhood Glaucoma Research Network Classification (CGRN).
RESULTS: A total of 105 children (181 eyes) were diagnosed with glaucoma. The most common type of childhood glaucoma was primary congenital glaucoma (PCG) constituting (42%, n = 76 eyes, 95% confidence interval (CI), 34.7-49.5%; P = 0.037), followed by glaucoma suspect (22.1%, n = 40 eyes, 95% CI, 16.3-28.9%; P < 0.001) and juvenile open-angle glaucoma (JOAG) (15.5%, n = 28 eyes, 95% CI, 10.5-21.6%; P < 0.001). While the most common type of secondary glaucoma was steroid-induced glaucoma, followed by glaucoma following cataract surgery. Bilateral glaucoma was found in 72.4% (n = 76 children, 95% CI, 62.8-80.7%; P < 0.001) of children. In both primary and secondary glaucoma, boys were affected more than girls, in ratio of 2:1 and 2.7:1, respectively. The mean age at presentation for patients with PCG was 2.7 years. Close to 93.4% (71) of PCG eyes were managed surgically, of which majority underwent combined trabeculotomy and trabeculectomy (CTT). Most of secondary glaucoma cases were treated medically. Overall, 85.3% (111) of eyes had successful control of IOP ≤ 21 mmHg.
CONCLUSIONS: PCG was the most common type of childhood glaucoma. One of a well-recognized challenge in developing countries, late presentation of patients with PCG, was also observed in our study. Which highlights, the need of increasing access to eye-care service and awareness of childhood glaucoma as a major public health issue. Steroid-induced glaucoma was the most common type of secondary glaucoma; appropriate measures should be taken to prevent this preventable glaucoma.

OBJECTIVE: The aim of this study concerns the evaluation of peripapillary vessel indices in childhood glaucoma (CG) and healthy subjects.
METHODS: In this prospective, unicenter, observational cross-sectional study, patients with CG and age and sex-matched healthy subjects were included. We compared retinal nerve fiber layer (RNFL) measurements in optical coherence tomography (OCT), peripapillary vessel density (PVD), and the flux index (FI) of the superficial vascular plexus from OCT angiography (OCT-A) between CG patients and control groups.
RESULTS: We included 39 patients (68 eyes) with CG and 50 (95 eyes) healthy subjects. The peripapillary RNFL thickness, vessel density, and flux index were significantly lower in the CG group than in the control group. The mean PVD of CG patients was 0.52 ± 0.043%, compared with 0.55 ± 0.014%, p < 0.0001 in healthy subjects. The mean FI was 0.32 ± 0.054 versus 0.37 ± 0.028, p < 0.0001, in CG patients and healthy subjects, respectively. PVD and FI in the superior, inferior, and temporal sectors were significantly lower in CG. The peripapillary RNFL thickness showed a higher area under the ROC curve (AUROC) for discriminating healthy and CG eyes and was significantly different than the PVD (0.797, 95%CI 0.726-0.869; p < 0.0001 vs. 0.664, 95%CI 0.574-0.752; p 0.00037), p 0.012.
CONCLUSIONS: PVD and FI show lower values in CG and correlate with RNFL thickness measurement but have lower diagnostic ability than RNFL thickness measurement. Our results reveal possible differences in the pathogenesis of microvascular compromise in childhood glaucoma patients.

UNASSIGNED: To ascertain the prevalence and clinical features of the various types of childhood glaucoma at a tertiary eye care hospital in Northern India.
UNASSIGNED: Retrospective chart review of all children less than 16 years of age with childhood glaucoma who presented from 1st April 2014 to 31st March 2019, who was diagnosed to have any subtype of childhood glaucoma as per Childhood Glaucoma Research Network (CGRN) classification and advised appropriate management.
UNASSIGNED: Out of 405 children with childhood glaucoma, 36% had primary glaucoma, whereas the rest had secondary glaucoma. Primary congenital glaucoma (PCG) was the most common form of primary glaucoma. Glaucoma associated with acquired conditions was the most common cause of secondary glaucoma. Primary glaucoma was mostly bilateral in contrast to secondary glaucoma. The most common age of presentation with primary glaucoma was <1 year of age, and in children with secondary glaucoma was 11-16 years. On presentation, 80% of eyes had intraocular pressure (IOP) of >20 mm Hg and 70% had cupping of >0.7. Eyes with PCG were primarily managed surgically.
UNASSIGNED: In our cohort, PCG was the most common primary childhood glaucoma. Traumatic glaucoma was the most common secondary glaucoma. Since childhood glaucoma is an important cause of visual morbidity in children, its timely diagnosis and prompt management are essential to prevent irreversible visual loss.
UNASSIGNED: Understanding the disease pattern, their presenting features, and the proportion of different types of childhood glaucoma can help in planning appropriate eye care services, create awareness and better allocate resources to plan appropriate management strategies. Screening programs and counseling of parents should also be strengthened.
UNASSIGNED: Dubey S, Jain K, Pegu J, et al. Profile of Childhood Glaucoma Attending a Tertiary Eye Care Center in Northern India. J Curr Glaucoma Pract 2023;17(2):68-74.

BACKGROUND: There is no consensus and few reports as to the surgical management of encapsulated Ahmed glaucoma drainage devices (GDD) which no longer control intraocular pressure (IOP), especially within the pediatric population. The purpose of this study was to report outcomes of exchanging the Ahmed GDD for a Baerveldt GDD in children with refractory glaucoma.
METHODS: Retrospective review of children (< 18yrs) who underwent removal of Ahmed FP7 and placement of Baerveldt 350 (2016-2021) with ≥ 3-month follow-up. Surgical success was defined as IOP 5-20 mmHg without additional IOP-lowering surgeries or visually devastating complications. Outcomes included change in best-corrected visual acuity (BCVA), intraocular pressure (IOP), and number of glaucoma medications.
RESULTS: Twelve eyes of 10 patients underwent superotemporal Ahmed FP7 to Baerveldt 350 GDD exchange at 8.8 ± 3.6 years. Time to Ahmed failure was 2.7 ± 1.9 years with 1-, 3-, and 5-year survival rates of 83% with a 95% CI[48,95], 33% with a 95% CI[10, 59], and 8% with a 95% CI[0, 30]. At final follow-up (2.5 ± 1.8 years), success rate for Baerveldt 350 GDDs was 75% (9 of 12 eyes) with 1 and 3-yr survival rates of 100% and 71% with 95% CI[25,92], respectively. IOP (24.1 ± 2.9 vs. 14.9 ± 3.1 mmHg) and number of glaucoma medications (3.7 ± 0.7 vs. 2.7 ± 1.1) were significantly decreased (p < 0.004). BCVA remained stable. Two eyes required cycloablation and 1 eye developed a retinal detachment.
CONCLUSIONS: Ahmed removal with Baerveldt placement can improve IOP control with fewer medications in cases of refractory pediatric glaucoma. However, more eyes with greater follow-up are required to determine long-term outcomes.

OBJECTIVE: To analyze and calculate the relative cost of various childhood glaucoma surgical interventions per mm Hg intraocular pressure (IOP) reduction ($/mm Hg).
METHODS: Representative index studies were reviewed to quantitate the reduction of mean IOP and glaucoma medications for each surgical intervention in childhood glaucoma. A US perspective was adopted, using Medicare allowable costs to calculate cost/mm Hg IOP reduction ($/mm Hg) at 1y postoperatively.
RESULTS: At 1y postoperatively, the cost/mm Hg IOP reduction was $226/mm Hg for microcatheter-assisted circumferential trabeculotomy, $284/mm Hg for cyclophotocoagulation, $288/mm Hg for conventional ab-externo trabeculotomy, $338/mm Hg for Ahmed glaucoma valve, $350/mm Hg for Baerveldt glaucoma implant, $351/mm Hg for goniotomy, and $400/mm Hg for trabeculectomy.
CONCLUSIONS: Microcatheter-assisted circumferential trabeculotomy is the most cost-efficient surgical method to lower IOP in childhood glaucoma, while trabeculectomy is the least cost-efficient surgical method.

The research activity in pediatric glaucoma (PG) was qualitatively and quantitatively evaluated using a scientometric approach.
The \"Web of Science\" database was accessed for primary bibliometric data regarding PG using search terms \"pediatric glaucoma,\" \"paediatric glaucoma,\" \"congenital glaucoma,\" and \"childhood glaucoma.\" The data was analyzed for total research productivity, citations, and scientific output in terms of journals, countries, institutions, and authors. The results were further characterized for coauthorship links and visualized by VOS viewer software. Also, the top 25 cited articles were reviewed with the above bibliometric characteristics.
One thousand two hundred and sixty-nine items were obtained from our search query from 1955 to 2022; these received 15,485 citations, originated from 78 countries. The top-3 contributing countries were the United States of America (n = 369), India (n = 134), and China (n = 127). LV Prasad Eye Institute (n = 58), Duke University (n = 44), and King Khalid Eye Specialist Hospital (n = 42) were the top-3 productive institutes. The top-3 prolific authors were Mandal AK (n = 53), Freedman, SF (n = 36), and Sarfarazi, M (n = 33). Journal wise, \"Investigative Ophthalmology\" (n = 187), \"Journal of Glaucoma\" (n = 92), and \"Journal of AAPOS\" (n = 68) were the journals in which the most articles were published. The top-25 cited documents received 3564 citations and were published between 1977 and 2016. The key areas of interest were basic sciences (genetics of childhood glaucoma) and surgical management.
United States of America, LVPEI, Mandal AK, and \"Investigative Ophthalmology\" were the top rankers as far as the productivity and publications related to PG are concerned. Articles on molecular genetics in PG have received interest among the ophthalmology community.