Primary Congenital Glaucoma Patients Research Articles

Primary Congenital Glaucoma presenting with Acute Corneal Hydrops: a challenging approach to amblyopia prevention

Introduction: Acute hydrops is a rare initial manifestation of Primary Congenital Glaucoma (PCG). It can result in significant corneal opacity, posing an additional risk for amblyopia development in PCG patients. We report a case of a newborn with PCG presenting with acute corneal hydrops and significant corneal scarring, discussing the management and challenges of amblyopia prevention. Case Presentation: A 3-day-old female newborn was referred to our department due to bilateral corneal clouding. Anterior segment examination revealed bilateral corneal edema, with severe corneal prolapse in the right eye consistent with acute hydrops. The anterior chamber structures of the right eye were indiscernible due to significant corneal edema. The left eye presented a deep, quiet anterior chamber with no dysgenesis of anterior segment structures. Intraocular pressure (IOP) measurements were >40 mmHg in both eyes. Corneal diameters were 10 mm OD and 12 mm OS. Fundoscopic posterior segment assessment was not possible due to unclear media. B-scan ultrasonography revealed no significant changes. The patient underwent urgent bilateral trabeculotomy. Postoperatively, intense mechanical stretching induced by severely increased IOP was visible, with the right cornea exhibiting a central opacity managed with topical corticosteroids and showing improvement. Right corneal opacity spared the superior cornea, and atropine drops combined with left eye occlusion were employed to mitigate right eye deprivation amblyopia. Eighteen months later, the patient can fixate and follow objects with the right eye. Left eye corneal transparency was restored, and bilateral IOP control was achieved. Conclusion: Despite prompt surgical treatment, acute hydrops presents a postoperative management challenge and may result in irreversible corneal scarring. Timely management of corneal opacities is crucial to prevent amblyopia. A multidisciplinary approach is essential for the best visual outcome.

Progression to bilaterality in unilateral primary congenital glaucoma

PurposeTo evaluate the incidence of rise in intraocular pressure (IOP) in fellow eyes of patients with unilateral primary congenital glaucoma (PCG) and to identify risk factors for IOP increase over long-term follow-up. MethodsThe medical records of unilateral PCG patients who had completed at least 5 years of follow-up were reviewed retrospectively. The incidence of developing ocular hypertension / glaucoma in fellow eyes was analyzed. Fellow eye progressors were those which showed an increase in optic nerve cupping by at least 0.2 since the first presentation or had IOP of >21 mm Hg on two occasions. The risk factors for progression that were analyzed included IOP, visual acuity, axial length, central corneal thickness (CCT), corneal diameters (CD), presence or absence of angle dysgenesis on high-resolution anterior segment optical coherence tomography (AS-OCT), and morphology of aqueous outflow pathways. ResultsAfter a median follow-up of 8.2 years (range, 5-25.5) progression to bilateral disease was found in 17 of 54 patients (32%), of whom 8 (15%) developed ocular hypertension and 9 (17%) developed glaucoma in the fellow eye. Among the unaffected fellow eyes, those with a larger CD (>12 mm), measured after at least 5 years’ follow-up, were ten times more likely to progress (P = 0.01; OR = 9.5 [95% CI, 1.7-54.3]). The presence of a patent supraciliary channel was significantly more frequently associated in fellow eyes compared with affected eyes on AS-OCT (OR = 1.4 [95% CI, 0.46-4.68]). ConclusionsOne-third of unaffected fellow eyes of unilateral PCG eventually progress over time, most often after 5 years. Larger CD at follow-up in the fellow eye is strongly predictive for progression.

Mutation Screening of the Cyp1b1 Gene Reveals Novel and Recurrent Pathogenic Variants in Pakistani Primary Congenital Glaucoma Patients

Mutation Screening of the Cyp1b1 Gene Reveals Novel and Recurrent Pathogenic Variants in Pakistani Primary Congenital Glaucoma Patients

TEK gene-related primary congenital glaucoma: Phenotypic features and mutational spectrum in a Mexican cohort of 10 unrelated families.

Primary congenital glaucoma (PCG) is one of the leading causes of visual damage and blindness, severely affecting the quality of life of affected children. It is characterized by cupping of the optic disc and loss of ganglion cells due to elevated intraocular pressure. While most PCG patients exhibit epiphora, photophobia, and buphthalmos with corneal opacity, variability in phenotypic manifestations is not uncommon. Prompt diagnosis and treatment of PCG affected individuals becomes relevant to preserve visual function throughout their lives. Most PCG cases are sporadic or autosomal recessive; however, an incompletely dominant autosomal dominant form arising from mutations in the TEK gene has recently been demonstrated. Here, we describe the clinical and mutational features of a cohort of Mexican patients with TEK-related PCG. Our results support the involvement of the TEK gene as an important cause of the disease in our ethnic group and expand the mutational spectrum causing PCG by reporting 10 novel disease-causing variants.

A comparison of rebound and applanation tonometry in children with and without primary congenital glaucoma

Background: Intraocular pressure (IOP) measurement should be accurate in a paediatric population with primary congenital glaucoma (PCG).Aim: This study aims to investigate the difference between the change in IOP measurements using rebound tonometry (RBT) and handheld applanation tonometry (AT) (Perkins applanation tonometer [PAT]) in patients with and without PCG.Setting: Johannesburg, South Africa.Methods: Measurements were taken under anaesthesia, using RBT and AT at 0 min, 5 min and 10 mins after induction and prior to intubation. Corneal pachymetry and corneal diameters were measured.Results: Sixty-five children were included, 19 with PCG and 46 without PCG. The mean age (standard deviation [s.d.]) was 3.2 (2.25) years and 4.8 (2.4) years, respectively. The overall mean difference in IOP between RBT and PAT across both PCG and non-PCG groups was found to be 4.92 mmHg (95% confidence interval [CI]: 2.80 – 7.03) p 0.001, with RBT having higher readings. This difference was greater in the PCG group, with the IOP difference of 9.05 mmHg (95% CI: 2.6 – 15.5) p = 0.004. Mean corneal pachymetry (s.d.) was 585.6 (81.48) µm in the PCG group and 518.31 (39.9) µm in the non-PCG group. Univariate analysis showed that IOP was significantly related to corneal pachymetry, with a 11 mmHg increase in IOP for every 100 μm change in corneal thickness for measurements done with RBT (p 0.001), compared to 4 mmHg using PAT (p = 0.008).Conclusion: Intraocular pressure measurements done with RBT in children with and without PCG were overestimated compared to PAT.Contribution: This difference was more pronounced in PCG patients. In addition, IOP was significantly related to corneal thickness.

Macular and peripapillary vascular parameters in the fellow eyes of unilateral primary congenital glaucoma: a comparative study

PurposeTo 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. MethodsUsing 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. ResultsA total of 48 eyes of 48 children (24 eyes in each group) were included. There was no difference in the mean RNFL 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). ConclusionsIn 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.

Prognostic Significance of Early Postoperative Choroidal Detachment in Patients with Congenital Glaucoma Operated with Nonpenetrating Deep Sclerectomy.

To assess the association between early postoperative choroidal detachment and intraocular pressure (IOP) following nonpenetrating deep sclerectomy in pediatric primary congenital glaucoma. Retrospective double-arm cohort study. Setting. Single center in Saudi Arabia. Patients. Seventy-two eyes of 45 patients were evaluated. Primary congenital glaucoma patients aged 0-3 years undergoing nonpenetrating deep sclerectomy as the first procedure from 2014 to 2021 were divided into groups with (n = 20) and without (n = 52) postoperative choroidal detachment. Main Outcome Measures. The primary outcome was complete surgical success, defined as an intraocular pressure below 21 mmHg without medication or additional surgery at 24 months. The intraocular pressure was evaluated in the first 72 hours after surgery and at 1, 3, 6, 12, 18, and 24 months. Kaplan-Meier survival analysis over 24 months was used to evaluate this outcome in both cohorts. The secondary outcome was the time to choroidal detachment resolution. There was no significant difference in surgical success between choroidal detachment and nonchoroidal detachment groups (P = 0.12). Preoperative and 2-year postoperative intraocular pressure was similar between groups, with a significant decrease in intraocular pressure from baseline (P < 0.001) in both the groups. The median time to choroidal detachment resolution was 27 days, and 90% of choroidal detachment cases were resolved with medical therapy. Postoperative choroidal detachment does not appear to significantly impact intraocular pressure or surgical success at 24 months following nonpenetrating deep sclerectomy for primary congenital glaucoma. Choroidal detachment typically resolves within one month of treatment. These findings suggest that transient choroidal detachment has a benign course in patients with primary congenital glaucoma undergoing deep sclerectomies.

Detección y caracterización de defectos del campo visual mediante perimetría Octopus en glaucoma congénito primario

PurposeTo detect and characterise visual field (VF) defects using static Octopus perimetry in patients with primary congenital glaucoma (PCG) and to determine VF quality and time duration. Material and methodsEighty-eight eyes of 70 patients diagnosed with PCG were included. Assessments were performed using an Octopus 900 and each eye was assessed with the tendency-oriented perimetry (G-TOP) algorithm. Quantitative VF data were collected: quality data (false positive and negative response, and time duration) and results of mean deviation (MD) and square root of loss variance (sLV). Qualitative data were collected: the presence of diffuse and localized defects, the affected hemifield and grade of defects using the Aulhorn and Karmeyer classification. Correlations between perimetric results and clinical variables were analysed. ResultsMedian age was 11 (8-17) years. 65.9% (58/88) of PCG eyes showed VF defects. Diffuse defects were observed in 10/58 eyes (16.94%) (mean MD=23.92 [SD: 2.52]) dB) and localized defects in 48/58 eyes (82.75%). The most frequent defect was spot-like/stroke-like/incipient paracentral scotoma (n=15), nasal step (n=8), adding arcuate defect (n=2), half ring-shaped (n=13) and concentric defect with a central island (n=9). And the most frequent affected visual hemifield was inferior hemifield. Mean test duration was 2min 12s (SD: 21.6s). MD and sLV values were correlated with best corrected visual acuity (BCVA), cup to disc ratio and number of antiglaucoma surgeries (all P<.001). ConclusionA high number of diffuse and localized defects were identified using Octopus perimetry in PCG patients. The most frequent defect was paracentral scotoma and inferior hemifield was the most affected.

Detection and characterisation of visual field defects using Octopus perimetry in congenital glaucoma

PurposeTo detect and characterise visual field (VF) defects using static Octopus perimetry in patients with primary congenital glaucoma (PCG) and to determine VF quality and time duration. Material and methodsEighty-eight eyes of 70 patients diagnosed with PCG were included. Assessments were performed using an Octopus 900 and each eye was assessed with the tendency-oriented perimetry (G-TOP) algorithm. Quantitative VF data were collected: quality data (false positive and negative response, and time duration) and results of mean deviation (MD) and square root of loss variance (sLV). Qualitative data were collected: the presence of diffuse and localized defects, the affected hemifield and grade of defects using the Aulhorn and Karmeyer classification. Correlations between perimetric results and clinical variables were analysed. ResultsMedian age was 11 (8–17) years. 65.9% (58/88) of PCG eyes showed VF defects. Diffuse defects were observed in 10/58 eyes (16.94%) (mean MD = 23.92 [SD: 2.52]) dB) and localized defects in 48/58 eyes (82.75%). The most frequent defect was spot-like/stroke-like/incipient paracentral scotoma (n = 15), nasal step (n = 8), adding arcuate defect (n = 2), half ring-shaped (n = 13) and concentric defect with a central island (n = 9). And the most frequent affected visual hemifield was inferior hemifield. Mean test duration was 2 min 12 s (SD: 21.6 s). MD and sLV values were correlated with best corrected visual acuity (BCVA), cup to disc ratio and number of antiglaucoma surgeries (all P < .001). ConclusionA high number of diffuse and localized defects were identified using Octopus perimetry in PCG patients. The most frequent defect was paracentral scotoma and inferior hemifield was the most affected.

The correlation of anterior segment structures in primary congenital glaucoma by ultrasound biomicroscopy with disease severity and surgical outcomes

PurposeTo evaluate the anterior segment structures using ultrasound biomicroscopy (UBM) in primary congenital glaucoma (PCG) and explore their correlation with disease severity and surgical outcomes.MethodsClinical information of PCG patients who underwent UBM prior to their first glaucoma surgeries from September 2014 to March 2021 were reviewed. The study included 214 UBM images of 154 PCG eyes and 60 fellow unaffected eyes. Anterior segment characteristics were analyzed. UBM parameters, including the iris thickness (IT) at variant distances from the pupil edge and iris root, anterior chamber depth (ACD), and pupil diameter (PD), were compared between two groups and their relationship with clinical factors and surgical outcomes were analyzed in PCG eyes.ResultsPCG eyes had unclear scleral spur, thin iris, wide anterior chamber angle, deep anterior chamber, rarefied ciliary body, elongated ciliary processes, and abnormal anterior iris insertion. ITs were thinner, ACD was deeper, and PD was larger in PCG eyes than fellow unaffected eyes (all P < 0.001). In PCG eyes, thinner ITs correlated with bilateral involvement and earlier age at presentation, and larger PD correlated with earlier age at presentation (P = 0.030) and higher intraocular pressure (P < 0.001). Thinner IT2 (P = 0.046) and larger PD (P = 0.049) were identified as risk factors for surgical failure.ConclusionUBM is a powerful technique to exam anterior segment structures in PCG. The anatomical features are associated with disease severity and surgical outcomes, providing essential clinical insights.

Thrombospondin Mutations and Primary Congenital Glaucoma Patients in a United States Population.

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Aqueous Humor Cytokine Profile in Primary Congenital Glaucoma.

Cytokine profile in patients with primary open-angle glaucoma (POAG) differs from that in healthy controls. Due to the different pathophysiological mechanisms involved in the genesis of primary congenital glaucoma (PCG) and POAG, it is possible that the cytokine profile could also differ. The main objective of this study was to compare the concentrations of cytokines in the aqueous humor of patients with PCG with those of POAG patients and a control group. A cross-sectional study was conducted. Aqueous humor samples were taken from PCG and POAG patients eligible for glaucoma or cataract surgery and from patients undergoing cataract surgery. Twenty-seven cytokines were analyzed using the Human Cytokine 27-Plex Immunoassay Kit (Bio-Rad Laboratories, Hercules, CA, USA). A total of 107 subjects were included: patients with PCG (n = 19), patients with POAG (n = 54), and a control group (CG) of patients undergoing cataract surgery (n = 34). Most cytokines measured in aqueous humor in PCG presented decreased values compared with POAG and controls. A statistically significant difference was observed in IL-1ra, IL-2, IL-5, IL-7, IL-8, IL-10, IL-12, IL-15, IL-17A, Eotaxin, FGF basic, G-CSF, GM-CSF, IFN-γ, MIP-1α, PDGF-bb, MIP-1β, RANTES, TNF-α, and VEGF. PCG patients have a cytokine profile in aqueous humor different from POAG patients and patients without glaucoma, characterized by lower concentrations of multiple cytokines.

High prevalence of glaucoma-associated CYP1B1 mutation (p.G61E) in primary congenital and open angle glaucoma patients in Pakistan

The Journal of the National Science Foundation of Sri Lanka publishes the results of research in all aspects of Science and Technology. The journal also has a website at http://www.nsf.gov.lk/. 2021 Impact Factor: 0.682The JNSF provides immediate open access to its content on the principle that making research freely available to the public supports a greater global exchange of knowledge.

Posterior corneal morphological changes in primary congenital glaucoma.

Purpose:To compare posterior corneal morphology between older treated and younger untreated children with primary congenital glaucoma (PCG) using anterior segment optical coherence tomography (ASOCT) and intraoperative OCT (iOCT), respectively.Methods: In this comparative study, ASOCT of older PCG children were compared with iOCT of younger untreated PCG patients. Differences between the two groups with respect to posterior corneal morphology were studied.Results: Observed morphological patterns within posterior cornea in older treated (age: 72–300 months) children (87 eyes) included Descemet’s membrane (DM) excrescences (70%), thickened DM (35%), intracameral twin protuberances (92%), and DM detachment (26%). Changes within pre-Descemet’s layer (PDL) (28%) included thickening, breaks, and detachments. Extent of Haab’s striae was associated with thickness of DM/PDL complex (P = 0.008) when analyzed in the treated group. In contrast, in the untreated group (n = 53 eyes, age 1–63 months), posterior corneal changes were limited to diffuse hyper-reflectivity of the DM/PDL complex, with absence of DM tears.Conclusion: Posterior cornea thickens and Haab’s striae become more circumscribed in eyes of older treated children compared to untreated PCG eyes, probably reflecting a healing response of posterior cornea over time.

Ocular Aberrations in Eyes with Primary Congenital Glaucoma

Purpose To highlight the magnitude of ocular higher order aberrations (HOA) and lower order aberrations (LOA), including component contributions from corneal and internal planes in Primary Congenital Glaucoma (PCG) patients. Methods Consecutive treated PCG patients co-operative for ocular examination and aberrometry, were enrolled over two years for this cross-sectional, comparative, single-center, unmasked study. Best-corrected visual acuity, refraction, IOP, wavefront aberrometry and topography (iTrace) were performed and results were compared with unaffected fellow eyes of unilateral glaucoma patients as well as age and sex-matched controls with no ocular anomalies other than treatable refractive error. Results Both eyes of 32 consecutive PCG patients (17 unilateral, 15 bilateral) and 39 controls were enrolled. The median LogMAR corrected distance visual acuity of PCG eyes was 0.68 (IQR: 0.2–1.8). Total ocular (Root mean square (RMS) 1.7 µm vs 0.3 µm, p = 0.014), corneal (RMS 1.1 µm vs 0.3 µm, p = 0.004) and internal (RMS 1.1 µm vs 0.2 µm, p = 0.013) aberrations, as well as HOAs and LOAs at each plane, were significantly higher in PCG eyes than in controls. Component HOAs from corneal and internal planes were positively correlated with each other (p < 0.001; rs: 0.7). Total aberrations were greater in the affected eyes of PCG compared to the rest. The predominant subtype of HOAs in PCG was coma and trefoil. PCG with corneal opacity/Haab’s striae had significantly higher astigmatism than the affected eyes with clear corneae at the corneal plane (p = 0.02). The aberrations were not statistically associated with the corneal diameter or refractive error in PCG eyes. Conclusions Significantly greater aberrations (Total, HOAs and LOAs, at corneal as well as an internal plane) were seen among eyes affected with PCG. Though the exact impact of these aberrations on the final visual outcome is difficult to determine, these could play a pertinent role in compromising visual function, thus impacting the management of visual rehabilitation in these patients.

Consanguinity and severity of primary congenital glaucoma

To evaluate the severity of primary congenital glaucoma (PCG) among children born of consanguineous marriage. In this case-control study, the medical records of unrelated consanguineous patients and unrelated nonconsanguineous (control) PCG patients seen at a single tertiary eye care facility were retrospectively reviewed. Those with a minimum of 5 years' follow-up were included. Data collected included age at presentation, corneal diameter, axial length, corneal haze at presentation and its persistence after surgery, need for repeat surgery, and final visual acuity. A total of 130 PCG patients were included: 30 patients born of consanguineous marriage and 100 nonconsanguineous control patients. The median age of presentation for consanguineous cases was 3 months (range, 1-36) compared with 10 months (range, 2-24) for nonconsanguineous cases (P < 0.001). Mean corneal diameter for consanguineous cases was 13 ± 0.82 mm and for nonconsanguineous cases was 12.41mm ± 1.18 mm (P = 0.002). Consanguineous cases also had a significantly higher prevalence of corneal haze persisting after surgery (P < 0.001) and need for repeat IOP-lowering surgery (P = 0.039). The consanguineous group had 44 eyes (73%) with severe PCG compared with 69 (34.5%) in the nonconsanguineous group (P < 0.001). In this study cohort, children with PCG born of consanguineous parents were more severely affected at presentation compared with children born of nonconsanguineous parents; they also had poorer outcomes with IOP-lowering surgery independent of severity at presentation. It is however possible that a founder effect with consanguinity over multiple generations could account for our observations.

Exome-based mutation screening in South African children with primary congenital glaucoma.

To identify pathogenic variants in a cohort of 23 black South African children with sporadic primary congenital glaucoma (PCG) using an exome-based approach. Children with PCG were recruited from two Paediatric Ophthalmology Clinics in Johannesburg, South Africa. Whole exome sequencing was performed on genomic DNA. Of the 23 children, 19 were male and 19 had bilateral PCG. A variant prioritization strategy was employed whereby variants in known PCG genes (CYP1B1, LTBP2 and TEK) were evaluated first, followed by the identification of putative disease-causing variants in other genes related to eye diseases and phenotypes. Validated pathogenic variants in the CYP1B1 gene (c.1169 G>A; p.Arg390His) and TEK gene (c.922 G>A; p.Gly308Arg) were identified in one child each. No LTBP2 mutations were identified in this cohort. In silico predictions identified potentially damaging rare variants in genes previously associated with eye development phenotypes or glaucoma in a further 12 children. This study demonstrates the value of whole exome sequencing in identifying disease-causing variants in African children with PCG. It is the first report of a TEK disease-causing variant in an African PCG patient. Potential causative variants detected in PCG candidate genes warrant further investigation.

Outcomes of Surgical Interventions in Primary Congenital Glaucoma Patients.

The aim was to report long-term surgical success of primary congenital glaucoma (PCG) patients in Thailand. PCG patients who underwent one of the following primary operations: trabeculotomy, goniotomy, trabeculectomy, combined trabeculotrabeculectomy (CTT) and diode transscleral cyclophotocoagulation (TSCPC) between January 1992 and January 2018 were reviewed. Surgical success was defined as intraocular pressure (IOP) between 5 and 21 mm Hg with or without antiglaucoma medications. Failure was defined as IOP ≤5 or ≥21 mm Hg for 2 consecutive visits, or when an additional glaucoma surgery was required to control IOP. Survival curves were analyzed using multilevel mixed-effect Weibull model. A total of 81 eyes from 55 PCG patients were included. Surgical procedures involved 20 goniotomies, 15 trabeculotomies, 16 trabeculectomies, 15 CTT, and 15 TSCPC. Median follow-up time was 24 months (interquartile range: 9 to 60 mo). Overall success rates were 68.8% at 1 year, 63.8% at 3 years, and 53.7% at 5 years. All types of surgery except TSCPC had comparable cumulative 1 year success rates ranging from 78.5% to 83.3%. Cumulative success rates of trabeculotomy (80.05%) and CTT (79.4%) were maintained at 3 and 5 years and were the highest among all procedures at 5 years. TSCPC had a significantly lower success rate compared with other types of surgery (hazard ratio: 7.4 to 13.1, all P=0.01). All patients receiving primary TSCPC showed no success at 48 months. Primary trabeculotomy and primary CTT demonstrated the highest long-term success rates in PCG patients.

Trabeculotomy Versus Combined Trabeculotomy-Trabeculectomy for Management of Primary Congenital Glaucoma.

Trabeculotomy (T) alone is as safe and effective as combined trabeculotomy-trabeculectomy (TT) in patients with primary congenital glaucoma (PCG) and requires significantly fewer examinations under anesthesia in the postoperative period. The purpose of this study was to compare the outcomes of T and combined TT as the initial surgical procedure for patients with PCG. In this retrospective study, medical records of patients who had undergone T or TT as the initial procedure for PCG were reviewed. The primary outcome measure was a surgical success, defined as 5≤ intraocular pressure (IOP) ≤21 mm Hg, IOP reduction of at least 20% with no need for further glaucoma surgery. Secondary outcome measures were the number of glaucoma medications and complications. A total of 134 eyes from 134 patients with a mean follow-up of 8±6.6 years were included consisting of 106 and 28 eyes, which had undergone T and TT, respectively. Success rates were comparable with both surgical procedures at 1, 2, 3, 4, and 5 years with no statistically significant difference. IOP was significantly decreased from 27.5±4.2 mm Hg preoperatively to 15.62±3.4 mm Hg at the 5-year follow-up visit in the T group and from 25.3±6.5 to 17.1±3.2 mm Hg in the TT group (P=0.1) there was no significant difference between the 2 groups in the number of glaucoma medications (P=0.2). Patients in the T group required significantly fewer examinations under anesthesia than the TT group (7.3±4.4 vs. 10.1±3.9, P=0.04). Trabeculotomy alone seems as effective as combined trabeculotomy-trabeculectomy and may significantly reduce exposure to anesthesia in primary congenital glaucoma patients.

Peripapillary and macular vascular parameters by optical coherence tomography angiography in primary congenital glaucoma.

To evaluate the differences in peripapillary and macular vascular parameters by optical coherence tomography angiography (OCTA) between patients with primary congenital glaucoma (PCG) and healthy controls; and to determine their diagnostic accuracy. Observational, cross-sectional study including 39 eyes with PCG and 78 healthy eyes. Only one eye per patient was included. All included patients underwent a comprehensive ophthalmic examination and peripapillary and macular analysis were performed by AngioplexTM OCTA (Cirrus HD-OCT 5000) with a 4.5 × 4.5 mm optic nerve head scan and 6 × 6 mm macular scan. Global data and quadrant data from peripapillary vascular parameters and global data and circular sectors data from macular superficial plexus parameters were compared between groups. The glaucoma discrimination capability of these parameters was calculated as areas under the receiver operating characteristics curve (AUC ROC). Mean age was 14.1 ± 8.7 years for the PCG patients and 11.7 ± 6.2 years for controls (p = 0.093). All vascular peripapillary measurements (global and quadrants; all p < 0.001) and all macular measurements (p < 0.042) excepting perfusion density in the inner circle (p = 0.087), were reduced in the PCG group compared to controls. According to AUC ROC, peripapillary (all ≥ 0.706) and macular parameters (all ≥ 0.699) showed good diagnostic capacity. AUC ROC for the most discriminatory measurements corresponding to blood flux index (0.887) and whole macula vascular density (0.855) were similar (p = 0.085). Peripapillary and macular vascular parameters by OCTA are decreased in patients with PCG, showing a good capacity to discriminate between normal and glaucomatous eyes.