DDX58(RIG-I)-related disease is associated with tissue-specific interferon pathway activation.

BACKGROUND: Singleton-Merten syndrome (SGMRT) is a rare immunogenetic disorder that variably features juvenile open-angle glaucoma (JOAG), psoriasiform skin rash, aortic calcifications and skeletal and dental dysplasia. Few families have been described and the genotypic and phenotypic spectrum is poorly defined, with variants in DDX58 (DExD/H-box helicase 58) being one of two identified causes, classified as SGMRT2. METHODS: Families underwent deep systemic phenotyping and exome sequencing. Functional characterisation with in vitro luciferase assays and in vivo interferon signature using bulk and single cell RNA sequencing was performed. RESULTS: We have identified a novel DDX58 variant c.1529A>T p.(Glu510Val) that segregates with disease in two families with SGMRT2. Patients in these families have widely variable phenotypic features and different ethnic background, with some being severely affected by systemic features and others solely with glaucoma. JOAG was present in all individuals affected with the syndrome. Furthermore, detailed evaluation of skin rash in one patient revealed sparse inflammatory infiltrates in a unique distribution. Functional analysis showed that the DDX58 variant is a dominant gain-of-function activator of interferon pathways in the absence of exogenous RNA ligands. Single cell RNA sequencing of patient lesional skin revealed a cellular activation of interferon-stimulated gene expression in keratinocytes and fibroblasts but not in neighbouring healthy skin. CONCLUSIONS: These results expand the genotypic spectrum of DDX58-associated disease, provide the first detailed description of ocular and dermatological phenotypes, expand our understanding of the molecular pathogenesis of this condition and provide a platform for testing response to therapy.

Structural abnormalities associated with glaucoma using swept-source optical coherence tomography in patients with systemic sclerosis.

PURPOSE: Vasospasm represents an early event in systemic sclerosis (SSc). Ocular vasospasm may induce optic nerve head (ONH) damage and has been involved in the pathogenesis of glaucoma, especially normal-tension glaucoma (NTG). We aimed to investigate the presence of structural abnormalities associated with NTG using swept-source optical coherence tomography (SS-OCT) and to correlate the OCT parameters with clinical, capillaroscopy and digital blood flow measures in patients with SSc. METHODS: In this cross-sectional study, 40 patients with SSc and 23 age-matched controls were included. The following parameters were measured using SS-OCT: mean and sectoral retinal nerve fibre layer (RNFL) thickness, macular ganglion cell layer complex (GCC) thickness and ONH morphology. Nailfold capillaroscopy (NFC) and digital blood flow measurements using laser Doppler imaging (LDI) were performed in all subjects. RESULTS: Patients with SSc showed a thinner temporal RNFL than the controls (69.23 ± 11.74 versus 83.35 ± 20.19 µm, p = 0.001). The other parameters were similar between the two groups. In SSc patients, there was an inverse correlation between the disease duration and the average, superior and inferior RNFL thickness and the GCC thickness and between Raynaud's phenomenon duration and the average RNFL and GCC thickness (p < 0.05). NFC and LDI measurements did not show correlations with OCT parameters. CONCLUSION: A thinner temporal RNFL and the correlation between Raynaud's phenomenon and disease duration and structural abnormalities on OCT suggest the presence of early ganglion cell damage in patients with SSc. Although mild, these findings indicate the need to monitor ocular abnormalities in SSc.

Using Enhanced Depth Imaging Optical Coherence Tomography-Derived Parameters to Discriminate between Eyes with and without Glaucoma: A Cross-Sectional Comparative Study.

INTRODUCTION: New technologies have been developed in order to decrease interpersonal influence and subjectivity during the glaucoma diagnosis process. Enhanced depth imaging spectral-domain OCT (EDI OCT) has turned up as a favorable tool for deep optic nerve head (ONH) structures assessment. OBJECTIVE: A prospective cross-sectional study was conducted to compare the diagnostic performance of different EDI OCT-derived parameters to discriminate between eyes with and without glaucoma. MATERIAL AND METHODS: The following ONH parameters were measured: lamina cribrosa (LC) thickness and area; prelaminar neural tissue (PLNT) thickness and area; average Bruch's membrane opening - minimum rim width (BMO-MRW), superior BMO-MRW, and inferior BMO-MRW. Peripapillary retinal nerve fiber layer (pRNFL) thickness was also obtained. RESULTS: Seventy-three participants were included. There were no significant differences between AUCs for average BMO-MRW (0.995), PLNT area (0.968), and average pRNFL thickness (0.975; p ≥ 0.089). However, AUCs for each of these 3 parameters were significantly larger than LC area AUC (0.701; p ≤ 0.001). Sensitivities at 80% specificity were: PLNT area = 92.3%, average BMO-MRW = 97.4%, and average pRNFL thickness = 94.9%. CONCLUSIONS: Comparing the diagnostic performance of different EDI OCT ONH parameters to discriminate between eyes with and without glaucoma, we found better results for neural tissue-based indexes (BMO-MRW and PLNT area) compared to laminar parameters. In this specific population, these neural tissue-based parameters (including PLNT area, which was investigated by the first time in the present study) had a diagnostic performance comparable to that of the conventional pRNFL thickness protocol.

Exercise-Induced Changes in Ocular Blood Flow Parameters in Primary Open-Angle Glaucoma Patients.

PURPOSE: To evaluate exercise-induced changes in ocular blood flow (OBF) parameters in primary open-angle glaucoma (POAG) patients. METHODS: A prospective observational study was carried out, in which medically treated patients with POAG were enrolled. Following inclusion, all patients performed a 40-min cycloergometry in a standardized fashion. The following parameters were measured and compared immediately before and 1 and 30 min after the exercise: intraocular pressure (IOP; Goldman applanation tonometry), mean arterial pressure (MAP), ocular pulse amplitude (OPA; assessed by dynamic contour tonometry), and ocular perfusion pressure (OPP; 2/3 MAP - IOP). In addition, we investigated possible factors associated with OBF parameter changes immediately after exercise. RESULTS: A total of 30 eyes (30 patients; mean age was 62.9 ± 1.7 years) were included. Most patients were women (53%), and median visual field mean deviation index was -3.5 dB. Both MAP (mean change, 21%) and IOP (mean change, 17.3%) increased significantly immediately after the workout (p < 0.01), persisting higher than baseline following 30 min (p < 0.01%). Regarding OBF parameters, both OPA (mean change, 58.8%) and OPP (mean change, 21.7%) increased significantly immediately after the workout and persisted higher than baseline 30 min after the workout (p < 0.01). Regression analysis revealed that only age was significantly associated with OPA variation (R2 0.14; p < 0.05). No significant associations were found for OPP (p ≥ 0.19). CONCLUSION: Aerobic exercise leads to a significant short-time increase in OBF parameters in patients with POAG. Even though IOP seems to present a modest elevation, it is accompanied by a significant increase in MAP, leading to higher OBF measurements. Exercise-induced short-term changes and its possible implications for glaucoma prognosis deserve further investigation.

Structure-function relationships in glaucoma using enhanced depth imaging optical coherence tomography-derived parameters: a cross-sectional observational study.

BACKGROUND: To investigate structural and functional correlations in glaucoma patients using enhanced depth imaging spectral-domain optical coherence tomography (EDI OCT)-derived parameters. METHODS: We prospectively enrolled healthy participants and glaucomatous patients with a wide range of disease stages. All participants underwent visual field (VF) testing (SITA - Standard 24-2; Carl Zeiss Meditec, Dublin, CA) and EDI OCT imaging (Spectralis; Heidelberg Engineering Co., Heidelberg, Germany). The following optic nerve head parameters were measured on serial vertical EDI OCT B-scans by two experienced examiners masked to patients clinical data: lamina cribrosa (LC) thickness and area, prelaminar neural tissue thickness and area, anterior LC depth, Bruch's membrane opening (BMO) and average, superior, and inferior BMO-minimum rim width (BMO-MRW). Only good quality images were considered, and whenever both eyes were eligible, one was randomly selected for analysis. Scatter plots were constructed to investigate correlations between each anatomic parameter and patient's VF status (based on VF index [VFI] values). RESULTS: A total of 73 eyes of 73 patients were included. All EDI OCT parameters evaluated differed significantly between glaucomatous and control eyes (P ≤ 0.045). A secondary analysis, in which glaucomatous patients were divided according to VF mean deviation index values into 3 groups (mild [G1; > - 6 dB], moderate [G2; - 6 to - 12 dB] and advanced [G3; <- 12 dB] glaucoma), revealed that average BMO-MRW was the EDI OCT parameter that presented more significant differences between the different stages of glaucoma. Significant structure-function correlations were found between VFI values and prelaminar neural tissue area (R2 = 0.20, P = 0.017), average BMO-MRW (R2 = 0.35, P ≤ 0.001), superior BMO-MRW (R2 = 0.21, P = 0.012), and inferior BMO-MRW (R2 = 0.27, P = 0.002). No significant correlations were found for LC area and anterior LC depth (P ≥ 0.452). CONCLUSIONS: Evaluating the distribution pattern and structure-function correlations of different laminar and prelaminar EDI OCT-derived parameters in glaucomatous patients, we found better results for neural tissue-based indexes (compared to LC-derived parameters). The diagnostic utility of each parameter deserves further investigations.

Intraocular Pressure Spikes within First Postoperative Hours following Standard Trabeculectomy: Incidence and Associated Factors.

PURPOSE: To evaluate the incidence of intraocular pressure (IOP) spikes within the first postoperative hours following trabeculectomy (TRAB) and to determine possible associated factors. METHODS: An observational study was carried out. We enrolled consecutive patients undergoing standard TRAB with mitomycin C. They were examined twice within the first few postoperative hours (hours 1-2 and 4-6) and 3 times after TRAB (on days 1, 7, and 30). Demographic and ocular data were collected. Main outcome measurements were postoperative IOP values at each time point and the frequency of IOP spikes, defined as IOP ≥25 mm Hg. RESULTS: A total of 40 eyes of 40 patients were included (mean age 59.62 ± 13.37 years). Although IOP was significantly reduced to 11.14 ± 7.99 mm Hg at hours 1-2 (p < 0.01) and to 11.52 ± 7.30 mm Hg at hours 4-6 (p < 0.01), IOP spikes were documented in 3 patients (7.5%). In the group of patients with IOP spikes, we noted that there was a high incidence of black patients and that the surgeries had been performed by fellow surgeons. CONCLUSION: Although the majority of the cases (92.5% of the patients) did not present IOP spikes, 7.5% of our patients presented the event. In selected cases, such as those with advanced disease, fixation threat, and of black race, IOP should be monitored during the first few postoperative hours for the identification and adequate management of potential IOP spikes, preventing undesirable outcomes.

DOUBLE-ARC SLOW-COAGULATION TRANSSCLERAL CYCLOPHOTOCOAGULATION LASER PROTOCOL: ONE-YEAR EFFECTIVENESS AND SAFETY OUTCOMES.

PURPOSE: To report the short-term efficacy and safety results of a new continuous laser protocol, double-arc slow-coagulation transscleral cyclophotocoagulation (DA-TSCPC). DESIGN: Multicenter retrospective study. PARTICIPANTS: Refractory glaucoma patients that had undergone DA-TSCPC between April 2019 and July 2022, with at least 12 months of postoperative follow-up, were consecutively enrolled. METHODS: The technique was standardized (energy: 1400mW; duration: 4s; 28 applications). The applications were divided into 2 rows (upper and lower arcs). For each arc, 7 spots were applied over the ciliary body shadow and 7 spots 1.5mm behind, sparing the 3 and 9 o'clock meridians. MAIN OUTCOME MEASURES: Success was defined as postoperative intraocular pressure (IOP) between 6 and 18 mmHg, and an IOP reduction of 30% (without oral acetazolamide). For eyes with no light perception (NLP), in which treatment goal was pain relief, success was defined as a 30% IOP reduction and no pain (without oral acetazolamide). Patients were divided according to visual acuity: ≥20/400 (group 1) and <20/400 (group 2). RESULTS: Ninety eyes of 90 patients (mean age: 61±15 years) were included. Glaucoma diagnosis frequency was: neovascular glaucoma (38%), open-angle glaucoma (28%), silicone oil secondary glaucoma (17%) and others (18%). Overall, the mean IOP was significantly reduced from 35±12 to 22±14 mmHg (p<0.01) at the last follow-up visit. The number of hypotensive eye drops (2.6±1 to 2.3±1; p=0.02) and the use of oral acetazolamide (61% to 11%; p<0.01) were also reduced. Kaplan-Meier survival analysis revealed a global success rate of 65.6% after 12 months. A higher success rate was found for group 1 (78.6%) compared to group 2 (59.6%; p=0.047; logrank test). The main complications observed were corneal ulcer (4.4%), macular edema (1.1%) and hyphema (1.1%). Among the 26 eyes with NLP, 65% achieved success criteria at 12 months and 2 (7.7%) developed phthisis. CONCLUSION: Based on these initial retrospective data, the DA-TSCPC protocol seems to be an alternative for refractory glaucoma management, presenting significant IOP reduction and a good safety profile after one year. Better outcomes were observed in eyes with less severe functional damage.

Peripheral Microvascular Abnormalities Associated with Open-Angle Glaucoma.

PURPOSE: To investigate peripheral microvascular abnormalities associated with patients with open-angle glaucoma (OAG). DESIGN: This was a cross-sectional study. PARTICIPANTS: Patients with OAG and controls. METHODS: All subjects underwent detailed ophthalmic evaluation, including Humphrey visual field (HVF) tests and swept source OCT. To evaluate peripheral microvascular abnormalities, nailfold capillaroscopy (NFC) and laser Doppler imaging (LDI) were performed. The presence of microhemorrhages, tortuous capillaries, dilated capillaries, avascular areas, and the capillary density, among other characteristics, were recorded using NFC; fingertip blood flow (FBF) was measured using LDI at different time points, before and 1, 10, and 20 minutes after exposure to a cold stimulus. In addition, venous blood samples were collected to measure serum endothelin-1 (ET-1) concentrations as well as serum autoantibodies. MAIN OUTCOME MEASURES: Presence of microhemorrhages, tortuous capillaries, and dilated capillaries; FBF; ET-1; and autoantibodies. RESULTS: Sixty-eight subjects (43 patients with OAG and 25 controls) were enrolled in the study. Microhemorrhages were found in the nail bed of 65.1% of the patients with OAG compared with 25.0% of the controls (P = 0.003). There was a significant difference in the mean FBF at the baseline in patients with OAG versus controls (293.6 ± 100.2 vs 388.8 ± 52.0 perfusion units, respectively, P < 0.001), together with a significant decrease in the mean FBF 10 and 20 minutes after cold stimulus in patients with OAG in comparison to controls (P < 0.001 for all comparisons). There was a positive correlation between mean baseline FBF and HVF mean deviation (r = 0.27, P = 0.03) and between mean baseline FBF and average retinal nerve fiber layer thickness (r = 0.44, P = 0.001). Neither the analysis of ET-1 concentrations (P= 0.71) nor the autoantibodies measurements (P > 0.05, for all) showed any difference between the 2 groups. CONCLUSIONS: Significant peripheral microvascular abnormalities were found in patients with OAG compared to controls, suggesting that microvascular changes might play a role in the pathogenesis of the disease. In addition, part of these peripheral microvascular abnormalities seems to be correlated with both functional and structural glaucomatous damage. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Vascular parameters and endothelin-1 measurements in glaucoma patients with low- and high-tension optic disc hemorrhages.

This prospective study aimed to compare vascular parameters (endothelin-1 [ET-1] blood levels, laser Doppler imaging [LDI] of distal phalanxes, and nailfold capillaroscopy) between open-angle glaucoma patients with low- and high-tension optic disc hemorrhages (LTDH and HTDH, respectively). The 33 enrolled patients (mean age, 62.3 ± 13 years) were classified as LTDH or HTDH if they presented at the time of DH detection an intraocular pressure (IOP) < 16 mmHg or ≥ 16 mmHg, respectively. Demographic and ophthalmological data, ET-1 concentrations, LDI (before and 1, 10, and 20 min after cold stimulation), and nailfold capillaroscopy findings were evaluated. The ET-1 blood level was 65% higher in the LTDH (2.27 ± 1.46 pg/ml) than in the HTDH (1.37 ± 0.57 pg/ml; p = 0.03) group. Moreover, there was a statistically significant negative correlation between ET-1 blood concentration and IOP at the time of DH detection (r = -0.45, p = 0.02). Blood flow measurements 10 and 20 min after cold stimulation were lower in the LTDH group than in the HTDH group (p < 0.01). Patients developing DH with lower IOPs have higher ET-1 blood levels and more peripheral vascular dysfunction as estimated by LDI than those with higher IOPs. These findings suggest that distinct underlying mechanisms may be involved in patients developing DH within different IOP ranges.

Subtenon triamcinolone as an adjuvant in mitomycin-C-enhanced trabeculectomy in non-inflammatory glaucomas: A randomized clinical trial.

This unicentric randomized clinical trial was designed to compare the surgical outcomes of mitomycin C-enhanced trabeculectomy (MMC-TRAB) with and without subtenon triamcinolone acetonide (TAAC) injection in patients with non-inflammatory glaucomas. This trial is registered at the Brazilian Registry of Clinical Trials (ReBEC) under the register number RBR-53f8nh. Consecutive non-inflammatory glaucoma patients requiring surgical intervention were randomized into two groups. In the control group, eyes underwent standard MMC-TRAB, while in the intervention group, besides the standard MMC-TRAB, these eyes also received a subtenon TAAC injection (4mg) close to the bleb site at the end of the surgery. The main outcomes of the study were surgical success rates, intraocular pressure (IOP) and number of medications at all timepoints. Success was defined as IOP ≤ 15 mmHg and subdivided in complete or qualified according to the need of medication. A total of 75 eyes of 63 different patients were included (intervention group = 39 eyes; control group = 36 eyes). There was no difference between groups at baseline (p>0.11). Multivariable regression analysis indicated that IOP levels were significantly lower in the intervention group at 18 and 24 months of follow-up when number of medications was considered as a covariate (P<0.001). Complete success rates were higher in the intervention group at 06 (90.9% vs 68.7%; p = 0.03), 12 (87.2% vs 66.7%; p = 0.02) and 18 months (87.2% vs 66.7%; p = 0.02). Additionally, although success rates at 24 months were higher in the intervention group (82.0% vs 66.7%; p = 0.09), this difference did not reach statistical significance. Qualified success rates did not significantly differ between groups at all timepoints. In conclusion, this study found significantly lower IOPs levels at 18 and 24 months of follow-up and higher complete success rates until 18 months of follow-up, with the use of subtenon TAAC as an adjuvant to standard MMC-TRABs in non-inflammatory glaucoma patients.

Clinical Profiles of Glaucomatous Patients With High-tension and Low-tension Optic Disc Hemorrhages: A Comparative Study.

PRCIS: Patients with low-tension optic disc hemorrhages (DHs) are more frequently women, have a diagnosis of normal-tension glaucoma (NTG) diagnosis and greater visual field (VF) loss. Symptoms of vascular dysregulation and Asian race also seem to be more prevalent in this clinical subtype. PURPOSE: Optic DH is an important glaucoma risk factor, and occurs in a wide intraocular pressure (IOP) range. We sought to characterize distinct clinical subtypes of patients with high-tension disc hemorrhage (HTDH) and low-tension disc hemorrhage (LTDH). PATIENTS AND METHODS: In this cross-sectional study, treated glaucomatous patients with DHs from 2 glaucoma services were consecutively enrolled. Disc photographs were evaluated for the presence of DH by 2 glaucoma specialists. After inclusion, patients were classified on HTDH (IOP≥16 mm Hg) and LTDH (IOP<16 mm Hg; median split). Clinical and ocular data from the time of DH detection were compared between groups. RESULTS: One hundred thirty-three DH patients were included (LTDH=66 eyes; HTDH=67 eyes). Patients with LTDH were more often women than those with HTDH (77% vs. 42%; P=0.030). There was also a trend for a higher prevalence of Asian descendants (24% vs. 9%; P=0.058) and symptoms suggestive of vascular dysregulation (34% vs. 14%; P=0.057) in LTDH patients. Eyes with LTDH also had worse VF mean deviation index (P=0.037), higher prevalence of NTG diagnosis (46% vs. 17%; P<0.001), and tended to have thinner central corneas (P=0.066). CONCLUSIONS: Patients developing DHs with treated IOPs in the low teens seem to more frequently fit in a profile represented by women, NTG diagnosis and greater VF loss. The presence of symptoms suggestive of vascular dysregulation and race also seem to differ between these 2 clinical subtypes. A closer optic disc surveillance is recommended for patients with the LTDH subtype, as they may develop DHs despite seemingly well-controlled IOP.

Human intraocular filariasis caused by Dirofilaria sp. nematode, Brazil.

A case of human intraocular dirofilariasis is reported from northern Brazil. The nematode was morphologically and phylogenetically related to Dirofilaria immitis but distinct from reference sequences, including those of D. immitis infesting dogs in the same area. A zoonotic Dirofilaria species infesting wild mammals in Brazil and its implications are discussed.

Human intraocular filariasis caused by Pelecitus sp. nematode, Brazil.

A male nematode was extracted from iris fibers of a man from the Brazilian Amazon region. This nematode belonged to the genus Pelecitus but was distinct from the 16 known species in this genus. Similarities with Pelecitus spp. from neotropical birds suggested an avian origin for this species.

Comparison between provocative test-based and long-term intraocular pressure parameters in patients with stable open-angle glaucoma.

PURPOSE: To compare provocative test-based and long-term intraocular pressure variation parameters in patients with stable open-angle glaucoma. METHODS: A prospective observational study was carried out in which stable open-angle glaucoma patients (⩾3 years of follow-up without any changes on current medical regimen) were consecutively enrolled. All patients were submitted to a water-drinking test. Long-term intraocular pressure parameters were obtained from isolated intraocular pressure measurements from each visit (⩾5 visits). We investigated the correlation and agreement between the following water-drinking test and long-term intraocular pressure parameters: mean, peak, and fluctuation. In addition, the percentage of eyes with significant intraocular pressure peaks during the water-drinking test (defined as a difference ⩾25% between water-drinking test intraocular pressure peak and mean long-term intraocular pressure) was assessed. RESULTS: Sixty-three eyes from 63 open-angle glaucoma patients (mean age, 60.7 ± 11.8 years) were included. Mean and water-drinking test intraocular pressure peak correlated significantly with mean (r = 0.67) and maximum long-term intraocular pressure (r = 0.52), respectively (p < 0.01). The correlation between water-drinking test intraocular pressure fluctuation and long-term intraocular pressure fluctuation was not significant (p = 0.45). Limits of agreement from Bland-Altman plots exceeded the maximum allowed difference between methods (3 mm Hg) for all the intraocular pressure parameters analyzed, suggesting poor agreement between water-drinking test-derived and long-term intraocular pressure values. Overall, significant water-drinking test intraocular pressure peaks were observed in 39.7% of these eyes with stable open-angle glaucoma. CONCLUSION: Although some water-drinking test-based and long-term intraocular pressure parameters correlate significantly, water-drinking test results may not reflect the long-term intraocular pressure profile of stable open-angle glaucoma patients, as their agreement is poor. Many of these patients may experience significant water-drinking test intraocular pressure peaks, despite long-standing stable disease and unchanged medical regimen.

Peak, Fluctuation, or Mean? A Correlation Analysis of Long-term Intraocular Pressure Variation Parameters in Patients with Stable Glaucoma.

AIM: To perform a correlation analysis between long-term intraocular pressure (IOP) variation parameters (mean, peak, and fluctuation) in patients with stable open-angle glaucoma (OAG). MATERIALS AND METHODS: A cross-sectional observational study was carried out, in which patients with stable OAG were consecutively enrolled. All patients had to have glaucomatous optic neuropathy and characteristic visual field (VF) defects. Key inclusion criteria were ≥5 VF tests, ≥3 disc photographs, and ≥3 years of follow-up without any changes in current medical regimen. Stable OAG was defined as nonprogressive VF results and absence of anatomical changes for at least 3 years. Long-term IOP variation parameters were obtained from isolated IOP measurements from each visit (minimum of five IOP measurements). The main outcome measure was the correlation between these IOP variation parameters. RESULTS: Of the 63 patients studied, 37 (59%) were women, and the mean age was 61 ± 12 years. Among all the analyses, IOP mean and peak had the strongest correlation (r = 0.94; 95% CI = 0.92-0.97; p < 0.001). There were also significant correlations between IOP peak and IOP fluctuation (r = 0.84; 95% CI = 0.75-0.90; p < 0.001), and mean IOP and IOP fluctuation (r = 0.62; 95% CI = 0.43-0.75; p < 0.001). CONCLUSION: Most long-term IOP variation parameters evaluated seem to be highly correlated. Notably, the correlation between mean IOP and IOP peak was the strongest one. We believe this fact should be taken into consideration as their inclusion as individual variables in a multiple regression model could lead to misinterpretation of the data. CLINICAL SIGNIFICANCE: Different well-designed studies are conflicting regarding which long-term IOP variation parameter is more clinically relevant. Our findings suggest that collinearity issues could explain in part the discrepant results among these studies evaluating the relationship between long-term IOP variation parameters and glaucoma prognosis. HOW TO CITE THIS ARTICLE: Scoralick ALB, Gracitelli CPB, et al. Peak, Fluctuation, or Mean? A Correlation Analysis of Long-term Intraocular Pressure Variation Parameters in Patients with Stable Glaucoma. J Curr Glaucoma Pract 2019;13(1):28-31.

Lack of association between provocative test-based intraocular pressure parameters and functional loss in treated glaucoma patients.

PURPOSE: To correlate provocative test-based intraocular pressure (IOP) variation parameters (fluctuation and peak) with functional status, and to compare these IOP parameters between treated eyes with asymmetric primary open-angle glaucoma (POAG). METHODS: A prospective observational study including consecutively treated patients with primary open-angle glaucoma was performed. Subjects with ocular diseases other than glaucoma or previous incisional glaucoma surgery were excluded. The primary inclusion criteria were 33 visual field tests and 32 years of follow-up, without any changes on current medical regimen. Long-term intraocular pressure parameters were acquired via isolated intraocular pressure measurements from the patients' last 5 office visits. To evaluate provocative test-based intraocular pressure parameters, all patients were submitted to a water drinking test. Initially, the partial correlation coefficients between each intraocular pressure variation parameter and visual field mean deviation were calculated, adjusting for the baseline intraocular pressure and number of antiglaucoma medications. In addition, each intraocular pressure parameter was compared between eyes with better visual field mean deviation and fellow eyes with worse visual field mean deviation in patients with asymmetric visual field loss (defined as an inter-eye visual field mean deviation difference of at least 3 dB). RESULTS: A total of 87 eyes (87 patients; mean age, 61.9 ± 12.5 years; 59.8% women) with primary open-angle glaucoma were included. The patients underwent a median of 5 visual field tests, with a mean follow-up of 4.3 ± 1.4 years. Neither long-term nor water-drinking test intraocular pressure variation parameters were significantly associated with visual field mean deviation values (p30.117). In the subgroup with asymmetric visual field loss (64 eyes of 32 patients; mean age, 65.0 ± 11.4 years), neither long-term water-drinking test intraocular pressure variation parameters differed significantly between eyes with better and worse visual field mean deviation (p30.400). CONCLUSION: Our results indicate that neither long-term intraocular pressure variation parameters nor stress test-derived intraocular pressure metrics, as assessed by the water-drinking test, appear to correlate with the visual field status or differ significantly between eyes with asymmetric visual field damage, suggesting that other factors may explain such functional asymmetry, and that the water-drinking test does not add significant information to these cases.

Trichothiodystrophy: a case report of childhood glaucoma associated with non-acquired systemic disease.

Trichothiodystrophy belongs to a group of rare genetic diseases characterized by DNA repair anomalies. Ocular manifestations can occur in 50% of cases, including cataract, refractive errors, strabismus, microcornea, microphthalmia, dry eye, and pigmentary macular changes. We report a case of childhood glaucoma in a patient with trichothiodystrophy who underwent trabeculectomy in the left eye. To our knowledge, this is the first clinical report of childhood glaucoma associated with trichothiodystrophy.

Factors associated with the presence of parafoveal scotoma in glaucomatous eyes with optic disc hemorrhages.

PURPOSE: Glaucomatous eyes with disc hemorrhage (DH) have a greater risk of paracentral visual field (VF) loss. However, not every DH eye presents with parafoveal scotoma (PFS), and contributing factors are still to be determined. In the present study, we investigated clinical and ocular factors associated with the presence of PFS in glaucomatous eyes with DH. METHODS: A case-control study was carried out. One hundred thirty glaucomatous patients with DH were enrolled. They were divided into two groups based on two reliable 24-2 VF tests: those with PFS (defined as ≥3 adjacent points with p < 5% within the central 10 degrees of fixation, ≥1 point with p < 1% lying at the innermost paracentral points, in the same hemifield) and those without PFS. Clinical and ocular data from the time of DH detection were compared between groups. Factors associated with the presence of PFS were investigated through logistic regression. RESULTS: The PFS group had a higher prevalence of Caucasian patients (82 vs. 47%; p < 0.01). Eyes with PFS had a more negative spherical equivalent and worse VF mean deviation (MD) index (p ≤ 0.01). There was a marginally significant intraocular pressure (IOP) difference between eyes with (15 mmHg) and without PFS (18 mmHg) at the time of DH detection (p = 0.10). Univariable analysis revealed PFS to be significantly associated with Caucasian race (OR, 3.02; p = 0.004), myopia (<-3 diopters; OR, 3.44; p = 0.039), and lower IOP (≤16 mmHg; OR, 2.10; p ≤ 0.047). Multivariable analysis, controlling for VF MD, revealed that only Caucasian race and myopia (as a continuous or categorical variable) remained significant in this model (p ≤ 0.038). CONCLUSIONS: Caucasian race and the presence and magnitude of myopia were found to be significantly associated with the presence of PFS in glaucomatous eyes with DH. Our results may help clinicians in the identification and surveillance of these eyes at higher risk of central VF loss.

Hypotony Management through Transconjunctival Scleral Flap Resuturing: Analysis of Surgical Outcomes and Success Predictors.

AIM: To investigate surgical outcomes and success predictors of transconjunctival scleral flap resuturing for the management of hypotony due to overfiltration following trabeculectomy with mitomycin C. MATERIALS AND METHODS: Noncomparative, retrospective, interventional case series in which all glaucoma patients from two glaucoma services undergoing transconjunctival scleral flap resuturing between May 2012 and July 2016 were enrolled. Included eyes had to have hypotony [intraocular pressure (IOP) < 6 mm Hg] and/or hypotony maculopathy caused by excessive filtration following trabeculectomy. Key exclusion criteria were wound/bleb leaking and postoperative ocular trauma or infection. Preoperative and postoperative IOP, best-corrected visual acuity (BCVA), fundus imaging, surgical complications, and any subsequent related events or procedures were recorded. Rates of postsurgical hypotony and/or maculopathy resolution and possible success predictors were investigated. RESULTS: A total of 22 patients (22 eyes) with a mean age of 56.4 ± 15.2 years were included. Median follow-up was 245 days [interquartilerange (IR); 120-817 days] and mean IOP was increased from 2.9 ± 1.5 mm Hg (1-6 mm Hg) to 8.5 ± 3.1 mm Hg (2-16 mm Hg) at the last follow-up visit (p < 0.01). Approximately 75% of the cases (16 out of 22) had an IOP between 7 and 18 mm Hg at the end of the follow-up period. Median BCVA (log MAR) at last follow-up visit [0.1 (IR; 0.0- 0.3)] was significantly better than preoperative BCVA [0.4 (IR; 0.11.0); p < 0.01]. Hypotony resolved in 81% of the cases, while maculopathy resolution was found in 85% of the cases. Time interval between trabeculectomy and flap resuturing was the only factor significantly associated with patient's IOP at last follow-up visit (R2 = 0.23; p = 0.036). Success rates (IOP > 6 mm Hg at last follow-up visit) were halved in those left untreated for more than 6 months. No serious adverse event was recorded. CONCLUSION: Our findings support the use of transconjunctival scleral flap resuturing as an effective and safe alternative for hypotony management due to overflitration following trabeculectomy. As time interval seems to influence the odds of hypotony resolution, early intervention is recommended. HOW TO CITE THIS ARTICLE: Scoralick ALB, Almeida I, Ushida M, Dias DT, Dorairaj S, Prata TS, Kanadani FN, Hypotony Management through Transconjunctival Scleral Flap Resuturing: Analysis of Surgical Outcomes and Success Predictors. J Curr Glaucoma Pract 2017;11(2):58-62.