Two types of childhood glaucoma secondary to familial exudative vitreoretinopathy.

BACKGROUND: Glaucoma secondary to familial exudative vitreoretinopathy presents as angle closure by either neovascular or non-neovascular mechanisms. We analyze the presentation and outcomes of two types of childhood glaucoma secondary to familial exudative vitreoretinopathy (FEVR). METHODS: This retrospective cross-sectional study included all patients <18 years of age diagnosed with glaucoma after or concurrently with a diagnosis of FEVR between 2010 and 2020 from Queen Sirikit National Institute of Child Health in Bangkok, Thailand. Two groups were analyzed: neovascular or non-neovascular angle-closure status. Primary outcome measures were final visual acuity and intraocular pressure (IOP) in both groups. RESULTS: Of 144 FEVR patients, 8 children (5.5%; 11 eyes, 3 bilateral cases) developed childhood glaucoma. Mean time between FEVR presentation and glaucoma was 42.2 ± 40.0 months. In the neovascular group, 3 of 9 eyes presented with glaucoma at FEVR diagnosis; 3 of 9 eyes (33%) required glaucoma surgery. In the non-neovascular group, 2 eyes presented with acute angle closure secondary to a phacomorphic lens. Both were treated with trabeculectomy, with resolution of pupillary block. All eyes had stage 4B FEVR or greater. Six of 8 eyes had stable or better visual acuity, and 10 eyes (91%) had IOP <21 mm Hg at final follow-up. CONCLUSIONS: Childhood glaucoma secondary to FEVR is a rare complication caused by later stages of the disease. It may present as neovascular or non-neovascular angle closure, often requiring complex care. Therefore, awareness and adequate management of FEVR can help prevent additional morbidity from childhood glaucoma.

Treatment Outcomes for Juvenile Open Angle Glaucoma in Thailand.

PRCIS: Juvenile open angle glaucoma (JOAG) patients with thick central corneas and negative family history were more likely to undergo surgery, mainly trabeculectomy with half requiring additional surgery within 10 years. PURPOSE: To assess the characteristics and treatment outcomes of patients with JOAG in Thailand. PATIENTS AND METHODS: This retrospective, multicenter study included all patients diagnosed with JOAG over 12 years from 2 tertiary hospitals in Bangkok, Thailand. RESULTS: A total of 200 eyes from 104 patients were included in this study. The mean age of onset was 24.0±10.1 years (range: 5-40 y), with male predominance (60.5%). Over 90% of patients had bilateral JOAG and 25% had a positive family history. Negative family history (adjusted odds ratio=4.59, P =0.02) and thick central corneal thickness were surgical predictors (every 10 µm adjusted odds ratio=1.29, P =0.01). Over 70% of cases needed glaucoma surgery. Trabeculectomy with Mitomycin-C was performed on 131 eyes (65.5%) with a cumulative probability of complete success of 71.0%, 57.8%, 39.2%, and 26.9% and qualified success of 86.3%, 73.6%, 64.8%, and 45.7% at 1, 3, 5, and 10 years, respectively. The mean follow-up after surgery was 94.9 ± 69.8 months (range: 13-153 mo). There were no serious postoperative complications. Myopia and the number of baseline glaucoma medications were significantly associated with surgical failure. CONCLUSIONS: Trabeculectomy with mitomycin C was the most common primary surgery performed in Thai patients with JOAG, and successfully reduced intraocular pressure without significant complications. Patients with thicker corneas were more likely to undergo surgery. By 10 years, half of the patients required additional surgery and risk factors for failure included myopia and the number of medications.

Evaluation of ocular biometry in primary angle-closure disease with two swept source optical coherence tomography devices.

PURPOSE: To investigate agreement between 2 swept source OCT biometers, IOL Master700 and Anterion, in various ocular biometry and intraocular lens (IOL) calculations of primary angle-closure disease (PACD). SETTING: Rajavithi Hospital, Bangkok, Thailand. DESIGN: Prospective comparative study. METHODS: This study conducted in a tertiary eye care center involving biometric measurements obtained with 2 devices in phakic eye with diagnosis of PACD. Mean difference and intraclass correlation coefficient (ICC) with confidence limits were assessed, and calculations of estimated residual refraction of the IOL were analysed using Barrett's formula. RESULTS: Sixty-nine eyes from 45 PACD patients were enrolled for the study. Excellent agreement of various parameters was revealed, with ICC (confidence limits) of K1 = 0.953 (0.861-0.979), K2 = 0.950 (0.778-0.98), ACD = 0.932 (0.529-0.978), WTW = 0.775 (0.477-0.888), and LT = 0.947 (0.905-0.97). Mean difference of axial length (AL) was -0.01 ± 0.02 mm with ICC of 1.000. IOL calculation was assessed with Barrett's formula, and Bland-Altman plot showed excellent agreement in the results of the 2 devices for the IOL power and estimated post-operative residual refraction (EPR). CONCLUSIONS: Mean differences of biometric parameters, obtained with IOL Master700 and Anterion, were small, and ICC showed excellent concordance. No clinical relevance in calculation of IOL power was found, and the two devices appeared to be comparably effective in clinical practice.

Clinical evaluation of ocular biometry of dual Scheimpflug analyzer, GALILEI G6 and swept source optical coherence tomography, ANTERION.

To evaluate the performance of a new swept source optical coherence tomography optical biometer, ANTERION, in ocular biometry and intraocular lens (IOL) calculation compared with the reference standard of Dual Scheimpflug Analyzer (GALILEI, G6). A prospective comparative study was conducted in a tertiary eye center. Cataract patients were scanned with both devices in a random fashion, and parameters from the devices were analyzed in terms of mean difference and intraclass correlation coefficient (ICC). Bland-Altman plots were performed to compare agreement between the devices. Ninety-six eyes from 96 patients were enrolled for evaluation. With the exception of ACD, all parameters were significantly different, but excellent agreement was revealed for all of them. The mean difference in axial length was 0.03 mm, and ICC was 0.999. Calculated IOL power with Barrett formula revealed that 93.75% were within 1 diopter and the prediction error was 0.03 diopter. Biometry of the devices were arithmetically different. However, the mean difference of the key factors in IOL calculation were small and appeared to be negligible for the purposes of clinical application. The performance of ANTERION was comparable to that of G6 in biometric measurement and IOL calculation; however, the devices cannot be used interchangeably.

Diagnostic performance of anterior segment optical coherence tomography in detecting plateau iris.

Objective: To evaluate the diagnostic performance of manual grading of anterior segment optical coherence tomography (AS-OCT) in detection of plateau iris configuration (PIC) based on the presence of standardised ultrasound biomicroscopy (UBM) criteria in at least two quadrants; namely, clinical diagnosis of PIC (DxPIC). Methods and analysis: In this cross-sectional study, paired AS-OCT and UBM images were evaluated by three glaucoma specialists. AS-OCT was classified into two mechanisms, PIC versus non-PIC, of primary angle closure disease (PACD) and AS-OCT-PIC diagnostic performance was tested with DxPIC. Results: One hundred and seventy-nine eyes of 142 patients were enrolled for analysis, and DxPIC was found in 85 eyes (47.49%). Intraobserver agreement rates of AS-OCT classification by the graders were 0.77, 0.701 and 0.742 (all p<0.001), and interobserver agreement rates, between a senior glaucoma specialist and the other two glaucoma specialists, were 0.68 and 0.702 (all p<0.001). Plateau iris was classified in AS-OCT images by the three graders, rated 32.96%-39.1% and 24.58%-34.08% in the horizontal and vertical axes, respectively. Diagnostic performance was analysed, yielding sensitivity ranging from 56.47% to 77.78%, and specificity of 48.94% to 64.29%. We applied disease prevalence of 30%, revealing positive predictive values varying from 32.16% to 44.44%, and negative predictive values of 72.4% to 85.71%. Accuracy ranged from 51.2% to 65%. Agreement between the two devices was fair, kappa range 0.31-0.351. Conclusion: Performance of manual grading of AS-OCT in detection of DxPIC was relatively poor; therefore, unadjusted AS-OCT does not appear to be good for manual PIC screening in PACD patients and cannot serve as a substitute for UBM in PIC detection.

Ultrasound biomicroscopy measured anterior and posterior chamber diameters--a novel way to evaluate angle-closure glaucoma.

OBJECTIVE: To investigate anterior and posterior chamber diameters in primary angle-closure (PAC) and to correlate them to the angle opening parameters. MATERIAL AND METHOD: Fifty eyes of PAC and 45 age-matched controls underwent ultrasound biomicroscopy (UBM; model P60, Paradigm Medical Industries Inc., Salt Lake City, UT) scanning. Superior, inferior temporal and nasal angle opening parameters were obtained. Anterior chamber diameter, indicated by angle-to-angle diameter (AAD) and posterior chamber diameter indicated by sulcus-to-sulcus diameter (SSD), were analyzed. RESULTS: Mean age was not different between PAC and controls, 59.80 +/- 9.11 versus 56.33 +/- 13.39, respectively (p = 0.140). PAC demonstrated a relatively smaller eye than the controls; e.g., corneal diameter; anterior chamber depth (ACD) and axial length, (all p < 0.05). AAD was shorter in PAC than the controls, 10.16 +/- 0.58 versus 10.88 +/- 0.55 mm, (p < 0.001). AAD was correlated to PAS extent (r = -0.358), ACD (r = 0.659), SSD (r = 0.636) and scleral spur to iris root insertion (r = 0.505), (all p < 0.001). SSD was also smaller in PAC than controls, 10.16 +/- 0.45 versus 10.52 +/- 0.52 mm, (p = 0.001). CONCLUSION: Crowded anterior segment of PAC can be demonstrated by a novel parameter of AAD and SSD, obtained with a newer model of UBM. The shorter AAD indicates the greater PAS extent and smaller angle. Both parameters are independent to scleral spur, and are simple to evaluate PAC.

Optic disc area and diameter of the central retinal vein occlusion fellow eyes, determined by optical coherence tomography.

OBJECTIVE: To investigate the diameters and disc area of central retinal vein occlusion fellow eyes (CRVO fellow eyes), whether there is any small diameter of the particular axis, which predisposing to central retinal vein occlusion (CRVO), or not. MATERIAL AND METHOD: A prospective analytic study between 78 CRVO fellow eye patients and 102 healthy control subjects was evaluated. Fast optic disc scan with Stratus OCT was obtained. RESULTS: Six axes of disc diameters, and disc area were not different between the groups. Analysis of the model excluding glaucoma within the study group (n = 59) revealed the similar outcome. CONCLUSION: Optic disc diameters and size in the CRVO fellow eyes were not different from normal subjects. Optic disc size appears not to be the pathogenesis of CRVO.

24-month outcomes of XEN45 gel implant versus trabeculectomy in primary glaucoma.

PURPOSE: To compare the efficacy and safety profiles of XEN implant versus trabeculectomy as a surgical intervention for primary glaucoma. METHODS: A retrospective cohort study of mild to moderate stage glaucoma patients, who had undergone either XEN implantation or trabeculectomy with adjunctive mitomycin C, was performed in a tertiary eye center. RESULTS: Fifty-seven eyes for XEN implant and 57 eyes for trabeculectomy with medically uncontrolled glaucoma were included. Preoperative IOP was 16-33 mmHg. Visual field mean deviation was -9.11±6.93 dB in XEN group, and -9.67±5.06 dB in trabeculectomy group (p = 0.195). At the 24-month timepoint, mean IOP was reduced from 21.6±4.0 to 14.6±3.5 mmHg (32.4% reduction) in the XEN group (p<0.001), and from 22.5±5.8 to 12.5±4.1 mmHg (44.4% reduction) in the trabeculectomy group (p<0.001). Final IOP in XEN was significantly higher than trabeculectomy (p = 0.008) with lesser mean IOP percentage reduction at month 24 (p = 0.045). Mean number of medications was reduced from 2.2±1.4 to 0.5±0.7 in XEN group (p<0.001), and from 2.4±0.7 to 0.8±1.3 in trabeculectomy group (p<0.001). Final number of medications was not different between the groups (p = 0.225). Surgical success was comparable between XEN and trabeculectomy group. Overall success was 71.4% vs. 73.3% (p = 0.850), and complete success was 62.9% vs. 62.2% (p = 0.954), respectively. XEN had lower rate of numerical hypotony than trabeculectomy. No serious complication occurred in either procedure group. CONCLUSION: At 24 months, XEN showed a rate of success comparable to that of trabeculectomy. Although XEN had a higher final IOP than trabeculectomy, XEN achieved 32% IOP reduction, and achieved final IOP in mid-teen level. No serious complication occurred in either group. XEN can be applied for treatment of mild to moderate stages of glaucoma in Southeast Asian patients.

Real-world Surgical Outcomes of Primary Angle-closure Glaucoma.

PURPOSE: To evaluate surgical outcomes of the four common procedures utilized for primary angle-closure glaucoma (PACG). METHODS: A retrospective study of survival rate in surgical management of PACG was conducted in a referral eye center. One hundred and ninety-nine eyes from 173 PACG patients were collected for chart review. The procedures used were phacoemulsification (PE), combined PE with goniosynechialysis (PE-GSL), combined PE with trabeculectomy (PE-Trab), and trabeculectomy alone. Failure was defined as postoperative IOP >21 mmHg in patients who needed second surgical intervention or those who had IOP <5 mmHg with loss of light perception. Cumulative survival rates, risk of surgical failure, and complications were analyzed. RESULTS: PE, PE-GSL, PE-Trab, and trabeculectomy were performed in 84 eyes (42.2%), 76 eyes (38.2%), 21 eyes (10.6%), and 18 eyes (9%), respectively. Cumulative survival rates at 60 months were 13%, 55%, 42% and 43%, respectively. Cox regression analysis indicated that each mmHg IOP increased, the risk of surgical failure decreased by 13% (adjusted hazard ratio (HR) 0.87; 95%CI: 0.84-0.93, p<0.001). CONCLUSION: Real-world surgical outcomes of PACG showed that PE alone had a low survival rate of 13% in 60-month follow-up whereas PE-GSL achieved the highest rate of 55%. PE-GSL should be initially considered for management of PACG, since it can restore and sustain the physiologic aqueous pathway and preserve the conjunctiva for future filtering surgery if needed.

Comparative Study of Lamina Cribrosa Thickness Between Primary Angle-Closure and Primary Open-Angle Glaucoma.

PURPOSE: To compare lamina cribrosa thickness (LCT) of primary angle-closure glaucoma (PACG) and primary open-angle glaucoma (POAG) using the enhanced depth-imaging mode of the Heidelberg Spectralis spectral-domain optical coherence tomography (EDI-OCT). PATIENTS AND METHODS: A comparative cross-sectional study was conducted. We enrolled 34 patients with PACG, 38 with POAG, and 62 controls, testing only one eye of each participant. Lamina cribrosa thickness was determined at the center of the optic nerve head using EDI-OCT. Nine points of LCT were measured, and LCT averages were analyzed. RESULTS: Mean age, number of glaucoma medications, current intraocular pressure (IOP), cup to disc ratio, and visual field indices, were not significantly different between PACG and POAG eyes. The maximum IOP (SD) was higher in PACG than in POAG, at 32.5 (10.46) vs 25.05 (6.42) mmHg (p = 0.001), and LCTs were significantly different among the PACG, POAG and control groups. Mean (SD) LCTs were 226.99 (31.08), 257.17 (19.46), and 290.75 (28.02) µm, respectively (p < 0.001). Lamina cribrosa thickness was correlated with mean deviation of the visual field (p = 0.001; correlation coefficient, rs = 0.347), while it was inversely correlated with maximum IOP (p < 0.001; correlation coefficient, rs = -0.592). Linear regression analysis revealed that LCT was inversely related to age (p = 0.008), female (p = 0.018), and maximum IOP (p = 0.002). LCT was marginally related to visual field MD (p = 0.053). CONCLUSION: Glaucomatous eyes had thinner LCT than controls, and maximum IOP was inversely correlated to the LCT. PACG eyes had higher maximum IOP and thinner LCT than POAG ones. In living eye, EDI-OCT emphasizes the pressure-dependent mechanism of glaucoma on lamina cribrosa deformation and the higher IOP-loaded stress which leads to a greater lamina cribrosa strain.

Deep Learning for Anterior Segment Optical Coherence Tomography to Predict the Presence of Plateau Iris.

Purpose: The purpose of this study was to evaluate the diagnostic performance of deep learning (DL) anterior segment optical coherence tomography (AS-OCT) as a plateau iris prediction model. Design: We used a cross-sectional study of the development and validation of the DL system. Methods: We conducted a collaboration between a referral eye center and an informative technology department. The study enrolled 179 eyes from 142 patients with primary angle closure disease (PACD). All patients had remaining appositional angle after iridotomy. Each eye was scanned in four quadrants for both AS-OCT and ultrasound biomicroscopy (UBM). A DL algorithm for plateau iris prediction of AS-OCT was developed from training datasets and was validated in test sets. Sensitivity, specificity, and area under the receiver operating characteristics curve (AUC-ROC) of the DL for predicting plateau iris were evaluated, using UBM as a reference standard. Results: Total paired images of AS-OCT and UBM were from 716 quadrants. Plateau iris was observed with UBM in 276 (38.5%) quadrants. Trainings dataset with data augmentation were used to develop an algorithm from 2500 images, and the test set was validated from 160 images. AUC-ROC was 0.95 (95% confidence interval [CI] = 0.91 to 0.99), sensitivity was 87.9%, and specificity was 97.6%. Conclusions: DL revealed a high performance in predicting plateau iris on the noncontact AS-OCT images. Translational Relevance: This work could potentially assist clinicians in more practically detecting this nonpupillary block mechanism of PACD.

Central corneal thickness in the central retinal vein occlusion fellow eyes.

OBJECTIVE: To investigate the central corneal thickness (CCT) in the central retinal vein occlusion fellow eye (CRVO-fellow eye) patients. MATERIAL AND METHOD: A cross-sectional study with 97 CRVO-fellow eye patients and 112 healthy subjects. Three measurements of CCT were obtained with ultrasonic pachymetry. RESULTS: Mean age of CRVO-fellow eye was higher than controls, 59.7 +/- 10.3 vs. 46.4 +/- 5.8 years, (p < 0.001). Males were more prevalent in CRVO-fellow eye than in the controls, 47.4% vs. 25%, (p = 0.001). In CRVO-fellow eye group, systemic diseases were more prevalent. Glaucoma and ocular hypertension were detected in 23 eyes (23.7%) of CRVO-fellow eye. Mean CCT of CRVO-fellow eye was thinner than controls, 529.2 +/- 30.3 vs. 543.3 +/- 31.8 on, (p = 0.001). Multiple linear regression models adjusted for age, sex, diabetes, hypertension, cup-to-disc ratio, IOP, and axial length revealed that mean CCT of CRVO-fellow eye was 16.9 microm thinner than controls. CONCLUSION: CRVO-fellow eye patients have thinner CCT than controls. The pathophysiology of this association is unclear It may be related to less lamina cribosa rigidity in the thin corneal eye. Lamina cribosa displacement may compress central retinal vein, leading to CR VO.

Clinical evaluation of intra-bleb pigmentation after trabeculectomy: a case-control study.

OBJECTIVE: To evaluate the clinical status of a filtering bleb with visible pigment (intra-bleb pigmentation, IBP), in a case-controlled manner. MATERIAL AND METHOD: Forty-one patients with IBP and 40 patients without visible pigment in blebs (controls) were enrolled from the authors' clinic. All patients underwent either trabeculectomy alone or combined cataract extraction with intraocular lens implantation and trabeculectomy. RESULTS: Mean follow-up period was 22.8 months. Complete success (final IOP < 22 mmHg without additional treatment) was achieved more often in IBP patients (82.9%) than in controls (60%, p = 0.022). Median survival time was greater in IBP patients (49.9 +/- 0 months) than in controls (35.5 +/- 4.9 months, p = 0.013). Regression analysis revealed that IBP was associated with thin and cystic blebs (p = 0.023). Sex, age, eye, diagnosis, procedure, and mitomycin C had no effect on IBP development. IBP grading, location, and appearance did not relate to the success rate or bleb characteristics. CONCLUSION: IBP appears to be a favorable sign in filtering blebs, in terms of complete success and survival time, because IBP is associated with thin-walled blebs. Clinicians should carefully observe patients for IBP when evaluating filtering blebs.

Genome-wide association study identifies five new susceptibility loci for primary angle closure glaucoma.

Primary angle closure glaucoma (PACG) is a major cause of blindness worldwide. We conducted a genome-wide association study (GWAS) followed by replication in a combined total of 10,503 PACG cases and 29,567 controls drawn from 24 countries across Asia, Australia, Europe, North America, and South America. We observed significant evidence of disease association at five new genetic loci upon meta-analysis of all patient collections. These loci are at EPDR1 rs3816415 (odds ratio (OR) = 1.24, P = 5.94 × 10(-15)), CHAT rs1258267 (OR = 1.22, P = 2.85 × 10(-16)), GLIS3 rs736893 (OR = 1.18, P = 1.43 × 10(-14)), FERMT2 rs7494379 (OR = 1.14, P = 3.43 × 10(-11)), and DPM2-FAM102A rs3739821 (OR = 1.15, P = 8.32 × 10(-12)). We also confirmed significant association at three previously described loci (P < 5 × 10(-8) for each sentinel SNP at PLEKHA7, COL11A1, and PCMTD1-ST18), providing new insights into the biology of PACG.

Intra-bleb pigmentation after trabeculectomy.

PURPOSE: To report the presence of intra-bleb pigmentation (IBP), appearing after trabeculectomy and combined clear cornea phacoemulsification, intraocular lens (IOL) implantation and trabeculectomy (combined surgery). METHOD: Ten eyes of 8 Asian patients with IBP were studied. Four eyes underwent trabeculectomy for uncontrolled glaucoma, and 6 eyes underwent combined surgery for cataract coexisting with glaucoma. Seven of the 10 eyes had adjunctive mitomycin C for the procedures. RESULTS: Mean follow-up time was 11.6 months (range 3-15 months). Five of the 10 eyes had intra-operative and post-operative complications requiring intraocular manipulation (ruptured posterior capsule and in-the-sulcus IOL, flat anterior chamber, malignant glaucoma, iris prolapse). Blebs were thin (8 eyes), or with moderate thickening of conjunctiva (2 eyes) with IBP either overlying the scleral flap or spread beneath the conjunctiva. IBP initially appeared at 2 to 44 weeks after the procedures. Nine of the 10 eyes (90%) had IOPs < or = 22 mmHg without medication. CONCLUSION: IBP is associated with iris trauma at surgery and dispersal of pigment into the bleb, where it spreads or proliferates. A thin bleb provides a clear view of IBP as well as functional filtration. IBP appears to be a favorable sign of the filtration.

Non-arteritic anterior ischaemic optic neuropathy associated with neovascular glaucoma.

We report a case of non-arteritic anterior ischaemic optic neuropathy (NAION) associated with neovascular glaucoma (NVG). A 63-year-old man who had undergone cataract surgery 3 months previously presented with sudden visual loss in his right eye. Ocular examination revealed a relative afferent pupillary defect, intraocular pressure (IOP) of 27 mm Hg, and 360° neovascularisation. Fundus examination revealed a pale and swollen optic disc with diabetic retinopathy. NAION associated with NVG was diagnosed. NVG, leading to reduced optic nerve perfusion pressure, concurrent with ischaemic processes of diabetic retinopathy, resulted in NAION.