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Purpose: To report the corneal tomographic characteristics of eyes that had surgery for controlled primary congenital glaucoma (PCG) and to compare it with an age-matched group of normal children. Methods: The study was conducted on 44 (24 right) eyes of 27 children (18 boys) who had surgery for PCG and 60 (31 right) eyes of 32 age-matched control children (9 boys). Children with PCG had a thorough ophthalmic examination and the demographic and medical and surgical history was retrieved from the patients' clinical chart records. After clinical examination, the study participants underwent Scheimpflug imaging using the Pentacam (Oculus Optikgeräte GmbH). Results: Both study and control children were approximately 9 years of age at the time of the study ( P = .39). The eyes with PCG had a spherical equivalent mean ± standard deviation of −4.70 ± 3.50 diopters (D). Eyes with PCG demonstrated a cylinder of −2.80 ± 2.10 D, flatter corneas than control eyes ( P = .0002 and .0012 for flat and steep keratometry, respectively), and statistically significantly higher astigmatism ( P = .011) and thinner corneas, both at pupil center ( P < .001) and thinnest location ( P < .001). There was a statistically significant negative correlation between the postoperative axial length (at the time of the scan) and each of the Scheimpflug parameters flat keratometry ( P = .002), steep keratometry ( P = .003), pachymetry at pupil center ( P = .01), and pachymetry at thinnest location ( P = .009). Conclusions: The eyes of patients with PCG have thinner, flatter, and more astigmatic corneas than the eyes of age-matched control children. [ J Pediatr Ophthalmol Strabismus . 2021;58(4):218–223.]

Purpose A central diagnostic tool in adult glaucoma is the peripapillary retinal nerve fibre layer (pRNFL) thickness. It can be assessed by scanning laser polarimetry (SLP) or optical coherence tomography (OCT). However, studies investigating the relevance of pRNFL measurements in children are rare. This study aims to compare the glaucoma diagnosing ability of SLP and OCT pRNFL thickness measurements in a paediatric population. Methods This retrospective study included 105 children (glaucoma: 22 (21.0%); healthy glaucoma suspects: 83 (79.0%)) aged 4–18 years, examined with SLP (GDxPro/ECC, Carl Zeiss Meditec) and spectral-domain OCT (SPECTRALIS®, Heidelberg Engineering). The thickness of pRNFL sectors was compared between diseased and healthy participants. Areas under the receiver-operating characteristic curves (AUC) and logistic regression results were used to compare the glaucoma discriminative capacity between SLP and OCT measurements. Results Using OCT, pRNFL thickness was decreased in the superior, nasal, and inferior quadrants of glaucoma patients compared to healthy controls (P P = 0.011). A correlation between glaucoma diagnosis and OCT-measured pRNFL thickness was found in all quadrants (P P = 0.037) and inferior quadrant (P = 0.0019). Finally, the AUCs of OCT measurements were markedly higher than those of SLP (e.g., inferior quadrant: OCT 0.83, SLP 0.68). Conclusion pRNFL thickness measurements using both OCT and SLP, correlate notably with the presence of glaucoma. In general, the diagnostic performance of pRNFL thickness measurements seems higher for OCT than for SLP. Thus, pRNFL thickness measurements could provide important information, complementing conventional clinical and functional parameters in the diagnostic process of paediatric glaucoma.

We evaluated the long-term visual outcomes in children with primary congenital glaucoma and determined the factors associated with the final visual outcomes. Medical records of children with primary congenital glaucoma between 2005 and 2016, seen at Seoul National University Children’s Hospital in South Korea, were reviewed. The minimum follow-up period after surgery for primary congenital glaucoma was 3 years. Visual acuity (VA) was categorized into good (≧20/70) and poor (

A 13-year-old female patient with refractory primary congenital glaucoma (PCG) in the right eye who had a history of multiple glaucoma operations underwent ab interno 180-degree trabeculectomy with the Kahook Dual Blade (KDB) targeting the nasal and inferior angles. On postoperative day 1, the intraocular pressure (IOP) of the right eye reduced from 43 to 15 mmHg while on medical therapy. The patient maintained this IOP level throughout the 6-month follow-up. Ab interno KDB trabeculectomy targeting both nasal and inferior angles may be an effective and safe procedure for the treatment of PCG even in eyes with a history of previously failed glaucoma procedures.

Purpose To assess the number of infants at risk of delayed primary congenital glaucoma (PCG) evaluation due to long travel times to specialists. Design Cross-sectional geospatial service coverage analysis. Methods All American Glaucoma Society (AGS) and American Association for Pediatric Ophthalmology and Strabismus (AAPOS) provider locations were geocoded using each organization’s member directory. Sixty-minute drive time regions to providers were generated using ArcGIS Pro (Esri, Redlands, CA). The geographic intersection of AGS and AAPOS service areas was computed because patients typically require visits to both types of specialists. American Community Survey data was then overlaid to estimate the number of infants within and beyond the AGS/AAPOS service areas. Results One thousand twenty-nine AGS and 1,040 AAPOS provider locations were geocoded. The analysis yielded 944,047 infants age 0-1 year (23.6%) who live beyond the AGS/AAPOS service areas. Therefore, approximately 14 to 94 new PCG cases/year may be at risk of delayed diagnosis due to living in a potential service desert. Compared to children living within the AGS/AAPOS service areas, children under age 6 years in these potential service deserts were more likely to live in households earning below the US federal poverty level, lack health insurance, and live in a single parent home. These communities are disproportionately likely to experience other rural health disparities, and are more prevalent across the Great Plains. Conclusion Service coverage analysis is a useful tool for identifying underserved regions for PCG referrals and evaluation. These data may assist in targeting screening programs in low access areas for pediatric glaucoma care.

Objective To compare intraocular pressure (IOP) measurements obtained using the Icare 200™ (IC200) rebound tonometer and the hand-held version of the Goldmann Applanation Tonometer (Perkins™ tonometer, GAT) in patients with primary congenital glaucoma (PCG) and in healthy subjects. Material and methods A total of 42 eyes of healthy subjects (G1) and 40 patients with PCG (G2) were analysed. The following clinical data were collected: gender, age, Cup/Disc ratio, central corneal thickness (CCT). IOP was determined in the examination room using the IC200 and GAT tonometers, in the same order. Agreement between both tonometers was determined using the intraclass correlation coefficient (ICC) and Bland-Altman plot. A linear regression analysis was used to establish the IOP was affected by the studied variables. Results Mean IOP between both tonometers (IC200 minus GAT) was: G1 = 15.91 (2.57) mmHg vs. 15.06 (2.12) mmHg (mean difference, MD = 0.84 (0.50) mmHg; P Excellent agreement was found between IC200 and GAT in both groups (ICC = G1: 0.875 (95% CI; 0.768−0.933; P Conclusions There was excellent agreement between the IC200 and GAT tonometers, both in healthy subjects and PCG, with a trend to overestimate IOP when measured with IC200. There was no influence by CCT on IOP measurements in patients with PGC.

Purpose: To report a case of neurotrophic keratopathy (NK) in a patient affected by primary congenital glaucoma (PCG) who undergone glaucoma drainage implant surgery. NK was successfully treated with human recombinant Nerve Growth Factor (Cenegermin) eye drops. Case report description: A 46-years-old patient affected by primary congenital glaucoma underwent Ahmed glaucoma valve implantation in the right eye, after several unsuccessful surgeries to control intraocular pressure (IOP) since the age of three. Surgical intervention was performed without complications, and IOP was well-controlled post-operatively. However, 1 month after surgery, he developed NK with stromal ulceration, initially treated with a topical combination of preservative-free artificial tears and antibiotic ointment. As NK did not resolve, a new treatment with Cenegermin 20 µg/ml eye drops, 6 times daily, was started. Outcome: NK completely resolved after 8 weeks of Cenegermin treatment, with complete restoration of corneal integrity and improvement of corneal sensitivity and transparency. Visual acuity in the operated eye reverted to the pre-operative value. Conclusion: Cenegermin was extremely effective in restoring corneal integrity in this PCG patient with NK.

Mohamed F Farid, Mohamed Anany, Mohamed A Awwad Ophthalmology Department, Benha University, Benha, EgyptCorrespondence: Mohamed A AwwadOphthalmology Department, Benha University, PO Box: 13511, 1 Fareed Nada Street, Benha, EgyptEmail mohdzahir82.ma@gmail.comPurpose: To present outcomes of surgical management of primary congenital glaucoma (PCG) in children less than one year of age in a population based at the Nile Delta.Methods: A retrospective review of medical records of patients with PCG less than one year of age at presentation who underwent surgical intervention in a tertiary care facility based at the Nile Delta. All patients underwent measurement of intraocular pressure (IOP), horizontal corneal diameter (HCD), cup-to-disc ratio (CDR) before and after surgery and a minimum of 6 months follow up was required. Surgical success was defined as IOP less than 22mmHg without medications and without progression of main disease parameters.Results: The review revealed 44 eyes of 26 consecutive patients who underwent surgical treatment for PCG. Average age at surgery was 5.2 months and mean follow up was 18.5 months. Preoperative IOP was 28.5± 4 mmHg, HCD was 13.7± 0.7mm, and CDR (when visible) was 0.65± 0.18. A total of 69 surgical procedures were performed with an average of 1.56 procedures per eye. Postoperative IOP was 13.3± 4.8 mmHg, HCD was 12.8± 0.9mm, and CDR was 0.3± 0.2 (P< 0.0001). Surgical success was achieved in 32 eyes (72.7%) while sight-threatening postoperative complications were reported in 3 eyes.Conclusion: Surgical management of PCG younger than one year of age achieved good success rate in the region of the Nile Delta with low rate of visually significant postoperative complications. However, larger studies with longer follow up are needed to fully reveal the overall characteristics of PCG in the region.Keywords: primary congenital glaucoma, intraocular pressure, trabeculotomy, trabeculectomy, Ahmed glaucoma valve, the Nile Delta

Primary congenital glaucoma (PCG) is a rare glaucoma type that develops in early infantile period and contributes to an elevated pressure on ocular cavity. Variants in gene are the most encountered in PCG cases. The prevalence of PCG is relatively high among Arabs, however its genetic epidemiology remains understudied. This study aims to systematically identify all reported PCG disease-causing variants in the Arab population and investigate their potential genotype-phenotype correlations. We searched four different databases (PubMed, ScienceDirect, Google Scholar, and Scopus) from the time of inception until July 2020. Broad search terms were used to capture all possible information about the genetic epidemiology of PCG among Arabs. We identified a total of 77 disease-causing variants in 361 patients and 88 families; of these, 33 were unique to Arabs. Sixty-nine variants were identified in the gene, five variants were in the gene and single variants were reported in , and genes. The most common reported variant was the c.182 G > A in the gene. All identified variants were from ten Arab Countries (Saudi Arabia, Kuwait, Oman, Egypt, Morocco, Lebanon, Tunisia, Iraq, Algeria, and Mauritania). We identified 44 shared variants with other ethnicities demonstrated a distinctive genotype-phenotype correlation. Consanguinity was observed in the majority of Arab PCG patients, ranging from 45% to 100%. PCG causing variants were identified in 10 Arab countries, which were mostly detected in the gene. Arab patients with PCG seem to have distinctive genotype-phenotype correlations.

Purpose: The aim of this study was to evaluate long-term intraocular pressures that provide stabilization/reversal of glaucomatous neuropathy after surgery in primary congenital glaucoma (PCG). Methods: Prospective evaluation of consecutive PCG patients who underwent trabeculectomy-trabeculotomy and followed up for ≥2 years. Records of regularly performed examination under anesthesia were maintained to ascertain intraocular pressure, (IOP), fundus, refraction and corneal diameter. Outcomes – Primary: cup: disc ratio and intraocular pressure, Secondary: corneal changes and refractive error Results: A total of 174 eyes of 108 children with PCG had a preoperative IOP of 22.44 ± 9.5 mm Hg. Postoperative review IOP was 11.8 ± 4.5 mm Hg, cup-disc-ratio was 0.52 ± 0.23 and corneal diameter was 12.75 ± 0.9 mm. Primary outcomes: Linear regression analysis showed a significant positive correlation of review IOP with cup disc ratio, P = 0.004. 67.9%, of eyes at a review IOP range of 6-12 mm Hg showed reversal, 14.1%, were stable, at 6-15 mm Hg, while 3.84% showed an increase in cup: disc ratio 16-22 mm Hg. Patients operated before 6 months of age had a significantly smaller final cup-disc ratio, P = 0.0013. Patients with a final cup: disc ratio of >0.9 were significantly older at surgery, P < 0.001. Secondary outcomes: There was a positive correlation of final myopia with review IOP on linear regression analysis, P = 0.012. The final spherical error in eyes having cup disc ratio of ≤ 0.5 was –0.96 ± 4.5 diopters, as against -3.45 ± 7.7 diopters in eyes having cup-disc ratio of 0.6-0.8 and -3.8 ± 6.9 diopters in eyes with cup disc ratio of ≥0.9, P = 0.015. There was no significant change in corneal diameter. Conclusion: Patients operated after 6 months of age had a larger final cup: disc ratio, while postoperative review intraocular pressures over 2 years of at least ≤15 mm Hg in primary congenital glaucoma eyes, commonly lead to reversal/stability of the neuropathy, and a lower incidence and degree of myopia.

Background: Primary prevention of primary congenital glaucoma (PCG) includes improving families of children with PGC. We evaluated the level of knowledge and attitudes of parents of children on PCG in Saudi Arabia. Methodology: This was a personal interview-based survey of parents of children with PCG at a tertiary eye hospital in Saudi Arabia. The study was conducted in 2018. A close-ended questionnaire in Arabic was used. Demographic data were collected on the children and parents. Clinical data on PCG were collected from electronic case records. Five questions each on knowledge and attitudes toward genetic counseling were queried. A Likert-type scale was used to collect the responses. Rasch analysis was carried out for knowledge and attitudes. The score was correlated with demographics and clinical determinants. p < 0.05 was considered statistically significant. Results: The study sample comprised 60 participants. The median Rasch score for knowledge on genetic counseling for PCG was -4.57 [interquartile range (IQR) -7.28; -1.0]. The median Rasch score for attitudes toward genetic counseling for PCG was -8.9 (IQR -11.6: -5.9). Parents with more than one family member with PCG had a significantly higher knowledge than those with one family member with PCG (p = 0.007). Knowledge of etiology and genetic counseling was significantly better if the child had residual vision amenable to low vision care (p < 0.001). The Rasch scores for knowledge and attitude were positively correlated (p < 0.001). Conclusion: Knowledge of the cause of PCG and genetic counseling was high among parents. The positive attitude toward genetic counseling could be useful for the primary prevention of CG in Saudi Arabia.

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.

To evaluate the surgical success after trabeculotomy for primary congenital glaucoma (PCG) in Denmark in the last 40 years.Retrospective review of medical files on children who all underwent trabeculotomy as the first surgical intervention between January 1rst, 1977 and December 31, 2016. Information on diagnosis and surgical procedures were extracted. Primary outcome was surgical success was defined as intraocular pressure (IOP) 21 mmHg without medications one year after surgery. Secondary outcome was the number of reoperations needed.Out of 118 children with PCG, 96 children (144 eyes) had an ab externo trabeculotomy as primary IOP lowering procedure opening Schlemm's channel 4 clock hours into the anterior chamber. Complete surgical success at one year was achieved in 73% (106 of 144 eyes). On the long term, IOP could be controlled by one surgical procedure in 2/3 of children, eight eyes required5 surgical procedures to control IOP. In 12.1% of children the second glaucoma procedure was performed5 years after the initial trabeculotomy. There was a tendency towards greater risk of needing a second procedure in patients 3 months of age at primary trabeculotomy (hazard ratio, HR, 2.01, 95% CI 0.96-4.22) and in boys (HR 2.02, 95% CI 0.97-4.18) and a lower risk of requiring a third surgery if the second surgery was trabeculectomy with MMC.Dedicated follow-up of patients with PCG is essential as the disease may continue to progress even after years of quiescence and some children need multiple surgeries to control IOP. Additional glaucoma procedures are required in 1/3 of children and boys and younger patients seem to be at greater risk.

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.

This prospective study aimed to compare the surgical outcomes of ab externo 240-degree trabeculotomy and trabeculotomy–trabeculectomy in managing primary congenital glaucoma (PCG). The study included 30 eyes of 30 patients aged less than 3 years who were diagnosed with PCG and was conducted in Menofia University Hospital between November 2018 and January 2020. The studied eyes were divided into two equal groups. Ab externo 240-degree trabeculotomy and combined trabeculotomy with trabeculectomy with MMC were performed for groups I and II, respectively. Post-operative measurements included intraocular pressure (IOP), optic disc changes, alteration in corneal clarity and transverse corneal diameter, as they are needed for antiglaucoma therapy. Post-operative complications were followed up for 1-year. There was significant reduction in IOP in both groups during the follow-up period. The reduction was higher in group I, where mean preoperative IOP was 28.20 ± 1.74 mmHg. The IOP in the group II was 28.07 ± 3.51 mmHg. The mean IOP decreased to 13.93 ± 1.8 mmHg and 14.60 ± 1.24 mmHg in groups I and II, respectively, after 1 year (P

Purpose To assess the long-term results of viscotrabeculotomy in infants with primary congenital glaucoma and to compare its outcome with conventional trabeculotomy. Patients and methods A prospective randomized comparative study included infants with primary congenital glaucoma younger than 2 years. Patients were divided into two groups: viscotrabeculotomy group and conventional trabeculotomy group. Preoperative and postoperative intraocular pressures, corneal diameter, intraoperative and postoperative complications, and success rates were compared between two groups. All the patients were followed up for 5 years. Results The study included 154 eyes of 92 infants distributed randomly among the two groups; 78 and 76 eyes in viscotrabeculotomy and conventional trabeculotomy groups, respectively. In both groups, there was a statistically significant intraocular pressure reduction at all time points of the follow-up periods compared to the preoperative values (p < 0.0001). At 5 years, viscotrabeculotomy group showed significant reduction of the mean postoperative intraocular pressure (49.47%) than conventional trabeculotomy group (48.64%) (p < 0.0001). Intraocular pressure was statistically lower in viscotrabeculotomy starting from 12th month and till the end of the follow-up. At 5 years, the total success rate of viscotrabeculotomy group was 89.74% compared to 85.53% in conventional trabeculotomy group without significant difference (p = 0.487). The postoperative mean values of the cup/disk ratio in viscotrabeculotomy group showed statistically lower values compared to conventional trabeculotomy group (p = 0.019). Postoperative hyphema was statistically higher in conventional trabeculotomy group (p Conclusion Viscotrabeculotomy and conventional trabeculotomy proved to be effective in cases of primary congenital glaucoma. Viscotrabeculotomy appeared to have prolonged stability in controlling the intraocular pressure with higher success rates and lower complications.

Abdelhamid Elhofi, Hany Ahmed Helaly Ophthalmology Department, Faculty of Medicine, Alexandria University, Alexandria, EgyptCorrespondence: Hany Ahmed Helaly 30 Roshdy Street, Roshdy, Alexandria, EgyptTel +20 1225466733Email hany209209@yahoo.comPurpose: To evaluate the safety and efficacy of non-penetrating deep sclerectomy with a collagen implant versus trabeculectomy in primary congenital glaucoma.Subjects and Methods: This was a retrospective interventional comparative non-randomized clinical study that included 80 eyes of 80 children presenting with primary congenital glaucoma under the age of 3 years. Forty eyes have undergone non-penetrating deep sclerectomy (NPDS) and the other 40 eyes have undergone penetrating trabeculectomy. The main outcome measure was the reduction in intraocular pressure (IOP). Secondary outcomes included percentage of patients with complete and qualified success, need for topical antiglaucoma medications, rate of complications, and need for further interventions. Complete success of the surgical outcome was considered an IOP ≤ 16 mmHg with no antiglaucoma medications. Qualified success was considered an IOP ≤ 16 mmHg using antiglaucoma medications.Results: The mean preoperative IOP was 27.4 ± 6.9 and 28.5 ± 6.6 mmHg in NPDS and trabeculectomy groups, respectively (p = 0.175). At the end of the follow-up period, the mean IOP was 11.2 ± 4.5 and 11.1 ± 3.4 mmHg with a mean reduction of 16.2 and 17.4 mmHg in NPDS and trabeculectomy groups, respectively. At the end of the follow-up period, ie at 36 months postoperative, the complete success rate was 60% (24 eyes) versus 57.5% (23 eyes), the qualified success rate was 25% (10 eyes) versus 25% (10 eyes), the overall success rate was 85% (34 eyes) versus 82.5% (33 eyes), and the rate of failure was 15% (6 eyes) versus 17.5% (7 eyes) in NPDS and trabeculectomy groups, respectively (p = 0.952). Eight cases (20%) of the trabeculectomy group had shallow anterior chamber. None of the NPDS group cases suffered from shallow anterior chamber.Conclusion: Non-penetrating deep sclerectomy appears to be an efficient and safe surgical alternative to penetrating trabeculectomy in treatment of primary congenital glaucoma. Non-penetrating deep sclerectomy has fewer postoperative complications in comparison to penetrating trabeculectomy with a comparative postoperative reduction in the intraocular pressure and overall success rates.Trial Registration: The trial was registered on 11/01/2020 with number PACTR202002874953456 (https://pactr.samrc.ac.za).Keywords: deep sclerectomy, primary congenital glaucoma, non-penetrating, childhood glaucoma, non-penetrating deep sclerectomy, trabeculectomy

Purpose To evaluate the safety and efficacy of non-penetrating deep sclerectomy with a collagen implant versus trabeculectomy in primary congenital glaucoma. Subjects and methods This was a retrospective interventional comparative non-randomized clinical study that included 80 eyes of 80 children presenting with primary congenital glaucoma under the age of 3 years. Forty eyes have undergone non-penetrating deep sclerectomy (NPDS) and the other 40 eyes have undergone penetrating trabeculectomy. The main outcome measure was the reduction in intraocular pressure (IOP). Secondary outcomes included percentage of patients with complete and qualified success, need for topical antiglaucoma medications, rate of complications, and need for further interventions. Complete success of the surgical outcome was considered an IOP ≤16 mmHg with no antiglaucoma medications. Qualified success was considered an IOP ≤16 mmHg using antiglaucoma medications. Results The mean preoperative IOP was 27.4 ± 6.9 and 28.5 ± 6.6 mmHg in NPDS and trabeculectomy groups, respectively (p = 0.175). At the end of the follow -up period, the mean IOP was 11.2 ± 4.5 and 11.1 ± 3.4 mmHg with a mean reduction of 16.2 and 17.4 mmHg in NPDS and trabeculectomy groups, respectively. At the end of the follow -up period, ie at 36 months postoperative, the complete success rate was 60% (24 eyes) versus 57.5% (23 eyes), the qualified success rate was 25% (10 eyes) versus 25% (10 eyes), the overall success rate was 85% (34 eyes) versus 82.5% (33 eyes), and the rate of failure was 15% (6 eyes) versus 17.5% (7 eyes) in NPDS and trabeculectomy groups, respectively (p = 0.952). Eight cases (20%) of the trabeculectomy group had shallow anterior chamber. None of the NPDS group cases suffered from shallow anterior chamber. Conclusion Non-penetrating deep sclerectomy appears to be an efficient and safe surgical alternative to penetrating trabeculectomy in treatment of primary congenital glaucoma. Non-penetrating deep sclerectomy has fewer postoperative complications in comparison to penetrating trabeculectomy with a comparative postoperative reduction in the intraocular pressure and overall success rates. Trial registration The trial was registered on 11/01/2020 with number PACTR202002874953456 (https://pactr.samrc.ac.za).

Precis Central corneal thickness (CCT) may increase over time in children affected by primary congenital glaucoma and treated with latanoprost for at least 30 months. Purpose The purpose of this study was to investigate CCT modification over time in a population of primary pediatric glaucoma (PPG) patients prescribed a monotherapy of latanoprost. Materials and methods The present paper reports the results of a post hoc analysis on patients enrolled in the Glaucoma Italian Pediatric Study (GIPSy). Children affected by PPG, with a postsurgical intraocular pressure between 22 and 26 mm Hg and treated with latanoprost monotherapy for at least 30 months were eligible for the analysis. CCT variation from baseline was investigated over the follow-up using univariable and multivariable longitudinal linear mixed models. The impact of age, sex, and intraocular pressure on CCT variation were evaluated taking into account the interaction of each variable with time. Results Twenty-seven eyes (20 patients) were included in the analysis. Mean duration of latanoprost treatment was 36.6 months (SD 2.5) and mean CCT at baseline was 551 μm (SD 37.7). A significant increase of CCT over time was revealed by multivariable analysis, taking into account the impact of age at baseline and its interaction with time (P=0.03). The interaction between age and time was significant (P=0.04), indicating that older age at baseline was associated with lower increase of CCT over time. No variation of CCT was found in univariable analysis (P=0.28). Conclusion In this population of PPG patients treated with latanoprost for at least 30 months, CCT significantly increased over time, when the impact of age and its interaction with time were considered.

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.

Aim To describe the clinical and epidemiological characteristics of children with pediatric glaucoma (PG) treated in an ophthalmologic national reference center. Materials and methods A retrospective study of patients diagnosed with PG in a national ophthalmologic reference center was made, between 2005 and 2015. Clinical findings, type of treatment, and the follow-up were evaluated. Results A total of 89 patients (145 eyes) were included. The median age of diagnosis was 2.0 years. The most frequent type of glaucoma was primary PG with 67.4% of affected patients, primary congenital glaucoma (PCG) being more frequent (69 eyes) than juvenile open-angle glaucoma (JOAG 36 eyes). Secondary PG accounted for 32.6% of the cases (40 eyes). At least one surgical procedure was needed in 56.6% of all studied eyes, and 10.7% of eyes had more than two surgical procedures. Even more, eyes with PCG had surgery in 88.4% of cases. On the contrary, eyes with JOAG did not require surgery. In the last assessment, the distribution of cases according visual acuity did not show differences. However, it is important to note that patients with secondary PG maintained a good vision only in 17.9% of cases. Conclusion Pediatric glaucoma is a heterogeneous group of diseases, and due to its low incidence, descriptive reports of large cohorts are not available. This study has a well-detailed report of PG characteristics in a national reference center. The frequency of JOAG in the present study was significantly higher than that reported in other studies. Also, clinical characteristics of all glaucoma described have some differences from data published. Clinical significance There are few studies that describe characteristics of PG. This study is an important tool to analyze the characteristics of PG in an effort to better know the disease. How to cite this article Saavedra C, Rios HA, Belalcazar S, et al. Characteristics of Pediatric Glaucoma in a Latin American Reference Center. J Curr Glaucoma Pract 2020;14(1):10-15.

Purpose Primary congenital glaucoma (PCG) is a severe type of glaucoma that occurs early in life. PCG is usually inherited in an autosomal recessive pattern. Cytochrome P450, family 1, subfamily B, polypeptide 1 (CYP1B1) gene is reported to be PCG-related gene. It codes for the CYP1B1 enzyme which is considered as phase I xenobiotic-metabolizing enzyme and its function is related to the eye oxidative homeostasis and correspondingly to the normal development of the eye. This is the first genetic study in Iraq that investigates the CYP1B1 polymorphisms behind the PCG disease. Methods Genomic DNA was extracted from the whole blood of 100 unrelated Iraqi PCG patients and 100 healthy children, all of them were aged between 1 month and 3 years. All the coding sequence of CYP1B1 gene was amplified using polymerase chain reaction; restriction fragment length polymorphism was used to follow G61E and E229K mutations. Direct sequencing was performed to screen for other mutations. Results CYP1B1 mutations were identified in 78 (78%) of the patients. We detected a total of eight mutations: Four missense mutations (c.182G>A, c.685G>A, g.6813G>A, and g.6705G>A), one silence mutation (D449D) and three insertions (g.10068ins10069, g.10138ins10139, and g.10191ins10192). Five mutations (g.6813G>A, g.6705G>A, g.10068ins10069, g.10138ins10139, and g.10191ins10192) are novel. G61E is the only mutation that was detected in patients merely. Conclusions CYP1B1 mutation (G61E) is considered as PCG-related allele in the Iraqi population.

Background: Primary congenital glaucoma (PCG) is the most common childhood glaucoma. It is caused by a defective development of the trabecular meshwork and the anterior chamber angle, which is believed to be inherited as an autosomal recessive disorder. The disease is characterised by high intraocular pressure (IOP), buphthalmos with corneal enlargement, and tears in Descemet’s membrane. Although rare, it is a significant cause of blindness in children worldwide. Case presentation: A 12-year-old Romani girl came to the department of ophthalmology complaining of epiphora and right eye enlargement. In her medical history, recurrent eye redness, lacrimation, and photosensitivity were noted, with symptoms lasting 7-9 years, which had been treated as conjunctivitis. Upon examination, enlargement of the right eye (axial length 25.63 mm) and divergent strabismus could be seen. Visual acuity in the enlarged eye was reduced to hand movement and slit lamp showed corneal clouding and Haab’s striae. Applanation tonometry measured IOP of 34 mmHg in the right eye. Fundus examination of the affected eye revealed a pale optic disc with a cup-to-disc ratio of 0.9 and visual field test showed residual temporal island of vision. The left eye had a complete normal finding. Based on clinical examination and imaging, PCG was diagnosed and trabeculectomy with mitomycin-C was performed on the right eye. Reassessment after four weeks showed a formed filtration bubble with IOP of 10 mmHg in the operated eye, but severe amblyopia persisted. After 12 months, visual acuity, IOP values, and visual field remained unchanged. Conclusion: Atrophy of the optic nerve caused by congenital glaucoma leads to a severe visual impairment in early years of life. This case illustrates the importance of early recognition and appropriate management of PCG to improve prognosis and to avoid irreversible damage to the visual function.

Autosomal recessive congenital hereditary endothelial dystrophy (CHED2) may be misdiagnosed as primary congenital glaucoma (PCG) due to similar clinical phenotypes during early infancy. In this study, we identified a family with CHED2, which was previously misdiagnosed as having PCG, and followed up for 9 years. Linkage analysis was first completed in eight PCG-affected families, followed by whole-exome sequencing (WES) in family PKGM3. The following in silico tools were used to predict the pathogenic effects of identified variants: I-Mutant 2.0, SIFT, Polyphen-2, PROVEAN, mutation taster and PhD-SNP. After detecting an SLC4A11 variant in one family, detailed ophthalmic examinations were performed again to confirm the diagnosis. Six out of eight families had CYP1B1 gene variants responsible for PCG. However, in family PKGM3, no variants in the known PCG genes were identified. WES identified a homozygous missense variant c.2024A>C, p.(Glu675Ala) in SLC4A11. Based on the WES findings, the affected individuals underwent detailed ophthalmic examinations and were re-diagnosed with CHED2 leading to secondary glaucoma. Our results expand the genetic spectrum of CHED2. This is the first report from Pakistan of a Glu675Ala variant with CHED2 leading to secondary glaucoma. The p.Glu675Ala variant is likely a founder mutation in the Pakistani population. Our findings suggest that genome-wide neonatal screening is worthwhile to avoid the misdiagnosis of phenotypically similar diseases such as CHED2 and PCG.

Purposes: Recent studies have suggested that loss-of-function mutations of the tunica intima endothelial receptor tyrosine kinase (TEK) are responsible for approximately 5% of primary congenital glaucoma (PCG) cases in diverse populations. However, the causative role of TEK mutations has not been studied in Chinese PCG patients. Here, we report the mutation spectrum of TEK after screening a large cohort of PCG patients of Chinese Han origin and analyze the identified variants in functional assays.Methods: TEK-targeted next-generation sequencing (NGS) was performed in 200 PCG patients. Candidate variants were prioritized by mutation type and allele frequency in public datasets. Plasmids containing wild type and identified variants of TEK were constructed and used to assess protein expression, solubility, receptor auto-phosphorylation, and response to ligand stimulation in cell-based assays.Results: Ten missense and one nonsense heterozygous variants were detected by NGS in 11 families. The clinical features of TEK variants carriers were comparable to that of TEK-mutated patients identified in other populations and CYP1B1-mutated individuals from in-house database. Functional analysis confirmed four variants involving evolutionarily conserved residues to be loss-of-function, while one variant (p.R1003H) located in tyrosine kinase domain seemed to be an activating mutation. However, our results did not support the pathogenicity of the other five variants (p.H52R, p.M131I, p.M228V, p.H494Y, and p.L888P).Conclusion: We provide evidence for TEK variants to be causative in Chinese PCG patients for the first time. Attention needs to be paid to TEK mutations in future genetic testing.