Primary Congenital Glaucoma Eyes Research Articles

Secondary intervention after failed initial intervention for primary congenital glaucoma

To report on the outcome of subsequent interventions after failed initial intervention for primary congenital glaucoma (PCG). Retrospective chart review of children presenting with PCG and failed the initial glaucoma surgery in Alexandria Main University Hospital from 2005 to 2017. The data included demographics, preoperative, operative and postoperative clinical characteristics. Success was defined as IOP<16mmHg and <20% from the presenting IOP and C/D ratio like or better than presentation. The charts of 531 (260 right, 49%) eyes of 360 (224 males, 62%) children presenting with PCG and failed the initial glaucoma surgery (58 [31 right, 53%] eyes [10.9%] of 50 [28 males, 56%] children) revealed that at initial presentation, the mean±standard deviation (range, median) of the age of the children subjected to one successful glaucoma procedure and the study children was 8.6±11.7 (0.6-109, 5.2) and 4.9±6.1 (0.5-34.4, 3.0) months, respectively. Each of the study eyes was subjected to 2.6±0.8 (2-5.2) glaucoma procedures. The mean±standard deviation (range, median) duration between the initial and second glaucoma surgery and the duration of follow-up was 6.9±7.8 (0.7-39,3.9) and 61.5±32.1 (12.0-139.1, 60.1) months, respectively. At the final follow-up visit success was reported in 41 (70.1%) eyes by IOP<16mmHg criterion, 39 (67.2%) eyes by IOP>20% reduction criterion and in 28 (out of 35 eyes, 80.0%) by the optic nerve condition (C/D ratio) criterion. Success by all 3 criteria was reported in 25 (out of 35 eyes, 71%) eyes. A repeat glaucoma surgical procedure maybe needed in up to 11% of operated PCG eyes, with the subsequent glaucoma surgical procedures being successful by about 70%.

Comparative posterior corneal profile of keratoconus hydrops versus Haab's striae in congenital glaucoma.

Keratoconus eyes develop corneal decompensation more often compared to eyes with primary congenital glaucoma (PCG) following Descemet's membrane (DM) tear. This study was conducted to compare the posterior corneal morphology in areas with DM breaks with regards to DM and pre-Descemet's layer (PDL) between the two. In this cross-sectional comparative study, anterior segment optical coherence tomography (AS-OCT) scans of the posterior cornea of advanced keratoconus eyes with hydrops ( n = 12), PCG eyes with Haab's striae ( n = 15), and healthy control eyes ( n = 14) were compared for DM-PDL morphology. These were further corroborated by the histopathology of corneal buttons from keratoconus ( n = 14) and PCG ( n = 13) cases obtained following penetrating keratoplasty and compared with controls (enucleated retinoblastoma globes, n = 6) on light microscopy and collagen IV immunostaining. AS-OCT showed a thicker median DM/PDL complex in PCG (80 µm) versus keratoconus eyes (36 µm, P = 0.01; Kruskal-Wallis test). The median height and length of detached DM-PDL were significantly more in keratoconus versus PCG (145 μm, 1766.1 ± 1320.6 μm vs. 26.5 μm, 453.3 ± 303.2 μm, respectively, P = 0.012; Kruskal-Wallis test). Type-1 DM/PDL detachment (seen as a characteristic taut chord) in keratoconus (90%) was the most common morphological pattern versus intracameral twin protuberance (92%) following DM breaks in PCG. Histopathology confirmed thicker DM in PCG (median: 63.4 μm) versus keratoconus eyes (median: 33.2 µm) or controls (27.1 μm) ( P = 0.001; Kruskal-Wallis test). Greater height/length of DM/PDL detachment compounded by poor healing response (lower DM/PDL thickness) probably causes more frequent corneal decompensation in keratoconus eyes when compared to PCG eyes following DM tears.

Childhood glaucoma profile in a Southwestern Ethiopia tertiary care center: a retrospective study

BackgroundChildhood 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.MethodsThis 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).ResultsA 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.ConclusionPCG 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.

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.

Long-term functional and structural outcomes in patients with primary congenital glaucoma-A Danish nationwide study.

Evaluation of long-term functional and structural outcomes in patients with primary congenital glaucoma (PCG) based on visual acuity (VA), visual field (VF) using standard automated perimetry, and peripapillary retinal nerve fibre layer thickness (pRNFL). We retrospectively reviewed medical records of all patients diagnosed with PCG in Denmark from 1977 to 2016. Severe vision loss was defined as VA <6/60 and/or VF >20 decibels (dB). Prognostic factors were evaluated in a correlation matrix. The median age of the 94 patients (153 PCG eyes) was 12 years (IQR 9-16). In PCG eyes 62% had VA ≥6/18 but 22% had <6/60. VA in the better seeing eye was ≥6/18 in 90% and <6/60 in 5%. VF was measured in 59 PCG eyes and the median mean defect was 5.1 dB (IQR 2.1-9.6) with 52% better than 6 dB and 9% worse than 20 dB. Generalized pRNFL was reduced below the age-expected 1st percentile in 29% of the 58 PCG eyes where pRNFL was measured. Poor VA, poor VF and reduced pRNFL were all correlated (p = 0.0001). More surgeries (p < 0.0001) and longer diagnostic delay (p = 0.004) were associated with poorer vision and to a lesser degree with poor VF pRNFL. In Denmark, most patients with bilateral PCG retain VA ≥6/18 in the better seeing eye. Poor VA was associated with poor VF. Longer diagnostic delay and more surgeries were associated with a poorer prognosis.

Comparative analysis of posterior cornea between hydrops in keratoconus and Haab's striae in primary congenital glaucoma

AbstractPurpose: More eyes with keratoconus (KC) require keratoplasty after hydrops when compared to those after haab striae in primary congenital glaucoma (PCG). To compare descemet's membrane (DM) tear morphology in KC and PCG eyes using ASOCT and histopathology.Methods: ASOCT of KC eyes with hydrops (n = 12) and PCG eyes with Haab's striae (n = 15) were evaluated and compared with healthy eyes (n = 14) for descemet's membrane/ pre‐descemet's layer (DM‐PDL) morphology. These features were also assessed on histopathology of corneal button obtained from KC eyes (n = 14) and PCG (n = 13) after keratoplasty and compared with control tissues (retinoblastoma not involving cornea, n = 6) on light microscopy and immunostaining.Results: ASOCT showed thinner DM‐PDL in KC eyes(47.1 ± 25.5 μ) than that of PCG (77.2 ± 26.2 μ) (p = 0.01) inside areas of healed hydrops/ Haab's striae. DM‐PDL detachment was seen in 90% of KC eyes and 26% of PCG eyes. The height and angle of DM‐PDL detachment of KC eyes was more than that of PCG eyes. Similar to ASOCT findings, histopathology showed thinner DM‐PDL in KC eyes (62.6 ± 57.3 μ) compared to PCG (80.3 ± 78.7 μ) inside areas of healed hydrops/ Haab's striae but thicker than that of controls (32.6 ± 7.8 μ) (p‐0.01).Conclusions: Lesser fibroproliferative response and greater height of detachment ensure a poor healing after acute DM/ PDL tears in KC. This differential response of the posterior cornea may be responsible for greater need for keratoplasty in KC versus in PCG.

Comparative evaluation of corneal characteristics in eyes of primary congenital glaucoma and Axenfeld‐Rieger syndrome

AbstractPurpose: To evaluate and compare the primary dysgenesis of cornea in Primary Congenital Glaucoma (PCG) and Axenfeld‐Rieger Syndrome (ARS).Methods: 26 eyes of PCG and 40 eyes of ARS were included. The following parameters were noted: clinico‐demographic profile; ASOCT of cornea/angle; Ocular biomechanical parameters, intraocular pressure (IOP) and central corneal thickness(CCT) (using Corvis ST); corneal aberration (iTrace, Tracey Technologies); morphological and cellular structure of the cornea (Confoscan HRT‐III:RCM) and histopathology of cornea (after keratoplasty).Results: Mean corneal diameter of PCG (13.4 ± 1 mm) was higher (p = 0.01) than ARS (11.7 ± 1.6 mm). Haab striae were observed more in the eyes of PCG (63.6%) than ARS (5%). Posterior corneal hyperreflectivity was present in 100% PCG eyes v/s 85.2% ARS (p = 0.006). Angle dysgenesis was comparable: PCG (85.4%) and ARS (90.7%). The presence of a double membrane sign, indicating thickened PDL and DM, was visible exclusively in eyes of PCG (29.2%) v/s singular posterior corneal hyperreflectivity (85.2%), indicating thickened DM complex, visible in eyes of ARS. Corneas of ARS were 52.3 μm thicker than PCG. In eyes of both PCG and ARS, the most common total ocular aberration was astigmatism. The endothelial cell count in PCG {1703 ± 661 cells/mm2} and ARS {1442 ± 397 cells/mm2} was comparable. The PDL thickness in the eyes of PCG (11.8 μm) was greater than ARS (9.1 μm). In immunohistochemistry, all tissues of PCG (n = 9) were collagen IV positive (indicating presence of PDL), two tissues of ARS (22.2%) were collagen IV negative.Conclusions: The difference in characteristic of PDL may cause difference in corneal enlargement and angle differences between the two groups.

In vivo identification of angle dysgenesis

AbstractAnterior segment Spectral domain–optical coherence tomography (SD‐OCT) is being increasingly used in glaucoma patients, primarily to investigate the anterior chamber angle and visualize the trabecular meshwork (TM) and Schlemm's canal (SC) in vivo. With the advent of angle surgery for glaucoma much interest has been generated in the aqueous outflow pathways. Our studies have shown that in vivo imaging of anterior chamber angle with ASOCT can been used to detect gross features of angle dysgenesis in primary congenital glaucoma (PCG), juvenile onset open angle glaucoma (JOAG) and adult onset primary open angle glaucoma (POAG), which has been described either as an absence of SC and/or the presence of abnormal tissue or a hyper‐reflective membrane within angle recess. This membrane is similar to what Barkan had described in PCG and was later described to be an angle cicatrization that portends a poorer success with goniotomy. Absence of development of SC was also shown by Hollander et al to be associated with a severe form of goniodysgenesis while its presence was seen in less severe goniodysgenesis on histopathological studies involving PCG. In fact, angle dysgenesis on ASOCT (ADoA), can be observed even in eyes with gonioscopically normal appearing angles. It is known that gonioscopy provides only a superficial representation of the histopathological anomalies affecting the angle. The presence of a thick compact tissue in the TM samples of Juvenile glaucoma patients has been observed, irrespective of whether they had an apparent goniodysgenesis or not. Hence, what appears to be morphologically normal angle on gonioscopy may harbour dysgenesis of the TM or even the SC. Primary congenital glaucoma, JOAG and adult onset POAG form a spectrum in terms of severity of angle dysgenesis. While most of the PCG eyes have features of ADoA, the same are present in 40% of JOAG eyes and in up to 35% of adult onset POAG. The size of the SC has also shown to be decreased in patients with adult‐onset open angle glaucoma based on in vivo OCT studies. We also present interesting observations of angle dysgenesis in fellow eyes of children with unilateral PCG.

“Coming up short” with an illuminated microcatheter for circumferential trabeculotomy ab externo in the buphthalmic infant eye: frequency and options

Angle surgery constitutes initial surgical treatment for most primary congenital glaucoma (PCG) cases. An illuminated microcatheter (iTrack microcatheter/Nova Eye Medical, Fremont, CA) facilitates circumferential trabeculotomy (360trab) but may not “reach” 360° in buphthalmic PCG eyes. This study’s purpose is the creation and validation of a model using preoperative horizontal corneal diameter (HCD) to predict whether the iTrack will traverse all 360° of Schlemm canal (SC) to assist surgical planning.

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.

Evaluation of visual field changes with retinal nerve fiber layer thickness in primary congenital glaucoma.

To evaluate visual field changes in primary congenital glaucoma (PCG) with retinal nerve fiber layer thickness on optical coherence tomography. In this cross-sectional, observational study, consecutive PCG children who underwent combined trabeculotomy with trabeculectomy and on regular follow-up were enrolled. All patients were aged over four years and co-operative for RNFL OCT and visual field examination. Perimetry was done on Humphrey visual field (HVF) analyzer using 30-2 and 10-2 SITA standard algorithms as appropriate. If a reliable automated perimetry was not feasible, kinetic perimetry was done. The following were noted at baseline and every follow-up: age, sex, visual acuity, intraocular pressure (IOP), cup-disc ratio (CDR), corneal diameters, refraction, any topical antiglaucoma medications, surgeries underwent, age at surgery and duration between surgery and final examination. Forty-eight eyes of 34 children operated for PCG and 19 eyes of 17 controls were analyzed. A statistically significant thinner average RNFL thickness of 87.2 ± 28 μm was noted in PCG eyes as compared to controls with 100.6 ± 7.2 μm (P = 0.04). The mean cup-disc area ratio on OCT in PCG eyes was 0.43 ± 0.2 (0.02-0.93) and in control eyes was 0.23 ± 0.07 (0.1-0.4) (P < 0.001). On RNFL OCT, there was significant focal RNFL loss in temporal superior (P = 0.003), nasal inferior (P = 0.037) and temporal inferior (P < 0.001) quadrants compared to controls. Among PCG eyes, 20/48 eyes (41.7%), had definitive, reproducible glaucomatous VF defects. Mean baseline IOP in PCG eyes with VF defect was 28.7 ± 5.7 mmHg and in eyes with normal VF was 24.6 ± 5.9 mmHg (P = 0.03). On univariate regression analysis, higher baseline IOP was significantly associated with both RNFL loss (odds ratio (OR): -2.17) and VF defects (OR: 3.35). Fluctuation in follow-up IOP (OR: 3.33) was also significantly associated with the presence of VF defects. On multivariable regression analysis maximum, IOP was significantly associated with RNFL loss and VF defects. Peripapillary RNFL thickness could be used to identify PCG eyes having visual field loss and possibly poor visual function from PCG eyes without visual field defects. Baseline and follow-up IOP, significantly correlated with RNFL thickness in PCG eyes.

Long-Term Visual Outcomes of Primary Congenital Glaucoma in China

Introduction: The aim of this study was to investigate the long-term visual outcomes and factors associated with vision loss in Chinese patients with primary congenital glaucoma (PCG) after successful intraocular pressure (IOP) control (IOP ≤21 mm Hg). Methods: PCG patients with IOP control who were examined in the glaucoma clinic at Zhongshan Ophthalmic Center from 2019 to 2020 were enrolled. The final visual outcome was evaluated by the best corrected visual acuity (VA). Univariate and multivariate analyses were used to investigate the associations of visual impairment with potential risk factors. The causes for decreased VA (<20/50) were also analyzed. Results: Fifty-nine patients (95 eyes) were included in the cohort, with a mean age of 8.7 years. The mean logMAR VA was 0.62 ± 0.64. The VAs of eyes treated for PCG were good (≥20/50) in 56%, fair (20/60–20/200) in 30%, and poor (<20/200) in 14%. The most common cause of decreased VA was amblyopia (64.3%). Multivariate logistic regression analysis showed that undergoing multiple surgeries (OR: 4.86, 95% CI: 1.11–21.16, p = 0.035) was significantly associated with visual impairment. Conclusion: The results showed that good VA was attainable in approximately half of PCG eyes under IOP control. Prompt and effective treatment of PCG, management of amblyopia and ocular comorbidities may be potential steps toward achieving good visual outcomes in PCG patients.

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.

Ultrasound biomicroscopy of the anterior segment in patients with primary congenital glaucoma: a review of the literature.

Primary congenital glaucoma (PCG) is a form of childhood glaucoma caused by maldevelopment of the anterior chamber. Disease severity differs greatly amongst patients. Ultrasound biomicroscopy (UBM) is a non-invasive technique that can visualize the anterior segment in infants invivo. The purpose of this narrative review is to make an overview of the UBM data in PCG and study the applicability of UBM in characterizing the disease. An online search was performed on PubMed in December 2020. After a critical appraisal of the included articles, study and patient characteristics were summarized. The UBM measurements of the anterior segment in PCG of the different studies were analysed. Six studies were included in this review. All were cross-sectional prospective studies. A total of 221 PCG eyes were examined. PCG eyes showed a larger trabecular iris angle, decreased iris thickness, narrower or absent Schlemm's canal and an increased zonular length compared to controls. Abnormal tissue membrane covering the trabecular meshwork and abnormal insertion of the iris and ciliary process were frequently found. The success rate of glaucoma surgery depended on the severity of anterior segment malformations found with UBM. Malformations of the anterior segment in PCG can be demonstrated by UBM invivo. This imaging can help to characterize disease severity and might support surgical treatment decisions.

Pediatric Glaucoma in a University Hospital.

Aims and objectivesThis study aimed at describing the characteristics and outcomes of pediatric glaucoma patients in a university hospital in Lebanon and comparing them between primary congenital glaucoma (PCG) and secondary glaucoma.Materials and methodsSixty-four eyes of 40 pediatric glaucoma patients with a follow-up of 1 year or more were reviewed. Parameters were studied for both PCG and secondary glaucoma groups. Three major outcomes were considered: intraocular pressure (IOP) control, final best-corrected visual acuity (BCVA), and the proportion of failed angle surgeries.ResultsSixty-four eyes were included divided into 29 PCG and 35 secondary glaucoma eyes, followed up for a mean of 6.6 ± 4.7 years. Eyes with PCG presented earlier, with a higher initial IOP and underwent earlier surgery than their secondary glaucoma counterparts. Although 85.9% of all eyes achieved good IOP control, 44.4% of them had a BCVA ≥20/50 with PCG having a better visual prognosis. Secondary glaucoma eyes needed more medications at the end of their follow-up. In this group, it was shown that a later age at first surgery, unilateral disease, and multiple surgeries were associated with a BCVA < 20/50.ConclusionPatients with PCG presented earlier and with a higher IOP than secondary glaucoma; they were also operated on earlier. Both groups demonstrated good IOP control at the end of the follow-up period with PCG eyes having a superior visual outcome. Secondary glaucoma eyes required a higher number of medications at their final visit. In this population, multiple surgeries, unilateral disease, and a delayed first surgery conferred a poorer visual prognosis.Clinical significanceData regarding the characteristics of pediatric glaucoma, especially in the Middle East, are scarce. Our study sheds the light on the presentation of different types of pediatric glaucoma and their outcomes namely IOP control and visual acuity.How to cite this articleDakroub M, Khair D, Noureddine B, et al. Pediatric Glaucoma in a University Hospital. J Curr Glaucoma Pract 2021;15(1):8–13.

Visual Evoked Potentials in Operated Primary Congenital Glaucoma: A Cross-Sectional Comparative Study.

BackgroundTo evaluate the VEP parameters in operated controlled primary congenital glaucoma (PCG) eyes and compare them to normal age similar children eyes.MethodsA cross-sectional comparative study conducted on 34 (19 right) eyes of 26 (19 males) children operated for PCG and 30 (17 right) eyes of 22 (12 males) age similar control children in a university-based practice. All study participants were subjected to a standard protocol of examination and electrophysiological testing (single flash VEP response, light adapted, pupils not dilated), reporting on the P2 implicit time, N1-P1 and N2-P2 (amplitude).ResultsThe mean±SD of the age of the study children and controls was 43.22±33.2 and 55.68±35.2 months respectively (p=0.217). The mean±SD IOP and cup/disc ratio of the study children were 18.4±5.2 and 5.2±3.3mmHg and 0.7±0.2 and 0.3±0.3 at presentation and at testing, respectively. The VEP testing was conducted after 21.5±21.3 months of surgery for PCG. There was no statistically significant differences in P2, N1-P1 and N2-P2 between patients and controls (p=0.941, 0.916,0.945, respectively). There was no statistically significant correlation between most of the clinical characteristics of the study eyes and any of the studied VEP parameters.ConclusionOperated controlled PCG eyes have VEP parameters that match their normal fellow children.

Lens in Primary Congenital Glaucoma Eyes Treated by Combined Angle and Filtering Surgery.

To report on the changes in the natural crystalline lens in primary congenital glaucoma (PCG) eyes that had undergone previous combined angle-filtering surgery with antimetabolites. The pediatric ophthalmology unit of the ophthalmology department of Alexandria Main University Hospital. A retrospective chart review. A retrospective chart review of patients presenting with and operated (by combined angle-filtering surgery with antimetabolites) for PCG in the period from 2005 to 2018. Any lens pathology was noted as well as the management and the outcome. The records of 422 children (613 eyes) were reviewed. Lens pathology was noted in 54 (8.8%) eyes. Abnormalities in lens clarity (cataract) were detected in 31 (56%) eyes, and abnormalities in lens position (subluxation) were detected in 24 (44%) eyes. Management options included observation (in 28 eyes), lensectomy for aphakia (in 14 eyes), lensectomy with in the bag intraocular lens (IOL) implantation (in 11 eyes), and lensectomy with iris-fixated IOL (in 1 eye). Elevation of intraocular pressure (IOP) occurred in only 4 eyes (of 26 operated eyes, 15.3%) after lens extraction. Changes in the natural crystalline lens clarity and/or position occurred in 8.8% (54 of 613) of PCG eyes operated by combined angle-filtering surgery with antimetabolites. Lens extraction was a relatively safe procedure with only 15% (4 of 26) of eyes suffering an elevation of IOP after lens extraction.

Evaluating target intraocular pressures in primary congenital glaucoma.

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 errorResults: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.