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Axenfeld-Rieger Syndrome (ARS) type 1 is a rare autosomal dominant condition characterized by anterior chamber anomalies, umbilical defects, dental hypoplasia, and craniofacial anomalies, with Meckel's diverticulum in some individuals. Here, we describe a clinically ascertained female of childbearing age with ARS for whom clinical targeted sequencing and deletion/duplication analysis followed by clinical exome and genome sequencing resulted in no pathogenic variants or variants of unknown significance in PITX2 or FOXC1. Advanced bioinformatic analysis of the genome data identified a complex, balanced rearrangement disrupting PITX2. This case is the first reported intrachromosomal rearrangement leading to ARS, illustrating that for patients with compelling clinical phenotypes but negative genomic testing, additional bioinformatic analysis are essential to identify subtle genomic abnormalities in target genes.
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Segmento Anterior del Ojo , Anomalías del Ojo , Enfermedades Hereditarias del Ojo , Proteína del Homeodomínio PITX2 , Femenino , Humanos , Segmento Anterior del Ojo/anomalías , Anomalías del Ojo/diagnóstico , Anomalías del Ojo/genética , Anomalías del Ojo/patología , Enfermedades Hereditarias del Ojo/diagnóstico , Enfermedades Hereditarias del Ojo/genética , Enfermedades Hereditarias del Ojo/patología , Factores de Transcripción Forkhead/genética , Proteínas de Homeodominio/genéticaRESUMEN
BACKGROUND: Facial contour naturally decreases the visual field. Peripheral visual field defects caused by facial anatomy and ocular pathology can be missed in a routine standard of care. Mathematically calculating the true angle for turning the head to optimize the peripheral visual field has not been studied to date. The purpose of this study was to explore the utility of turning the head during perimetry to maximize the testable visual field. METHODS: Six healthy study participants aged 18-52 were enrolled, prospectively; the dominant eye of each participant was tested. In total, 60-4 visual fields were obtained from each participant's dominant eye with the head in primary position. Then, the 60-4 tests were repeated with the head turned prescribed degrees toward and away from the tested eye ("manual method"). Based on a photograph of the participant's face, a convolutional neural network (CNN) was used to predict the optimal head turn angle for maximizing the field, and the test was repeated in this position ("automated method"). RESULTS: Maximal visual field exposure was found at a head turn of 15° away from the tested eye using the manual method and was found at an average head turn of 12.6° using the automated method; maximum threshold values were similar between manual and automated methods. The mean of threshold in these subjects at the standard direction and the predicted optimum direction was 1,302, SD = 69.35, and 1,404, SD = 67.37, respectively (P = 0.02). CONCLUSIONS: Turning the head during perimetry maximizes the testable field area by minimizing the influence of prominent facial anatomy. In addition, our CNN can accurately predict each individual's optimal angle of head turn for maximizing the visual field.
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PURPOSE: To determine the degree and rate of ptosis in patients undergoing glaucoma and cataract surgery. METHODS: Patients undergoing cataract extraction (CE), trabeculectomy, or glaucoma drainage device (GDD) placement, or a combination, were consecutively enrolled by a sole surgeon. Eyelid measurements, including margin reflex distance 1 (MRD1) and levator function, were obtained preoperatively and at 1 and 3 months postoperatively. Primary outcome measures were the change in MRD1 pre- vs postoperatively; percentage of patients with ptosis (defined as MRD1 < 2 mm pre- vs postoperatively). Secondary measures were the absolute change in MRD1 between groups, decrease in MRD1 of ≥ 2 mm, and change in levator function. RESULTS: In total, 104 eyes of 73 patients underwent CE, trabeculectomy, or GDD placement and completed at least 1-month follow-up; 93 eyes of 65 patients completed 3-month follow-up. MRD1 decreased significantly in trabeculectomy and GDD groups at 1 and 3 months postoperatively, while it did not change in the CE group. The GDD group had a significant increase in percentage of patients with ptosis at 3 months postoperatively. CONCLUSION: Patients who underwent glaucoma surgery, especially those who underwent GDD placement, were more likely to have postoperative ptosis than patients undergoing CE alone. High ptosis rates in patients undergoing glaucoma surgery may warrant preoperative counseling and post-operative referral to oculoplastic surgeons.
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Segmento Anterior del Ojo/cirugía , Blefaroptosis/epidemiología , Párpados/diagnóstico por imagen , Glaucoma/cirugía , Complicaciones Posoperatorias , Anciano , Blefaroptosis/etiología , China/epidemiología , Párpados/cirugía , Femenino , Estudios de Seguimiento , Humanos , Incidencia , Masculino , Estudios Prospectivos , Factores de TiempoRESUMEN
PURPOSE: To determine the aquaporin-4 and myelin oligodendrocyte glycoprotein (MOG) immunoglobulin G (IgG) serostatus and visual outcomes in patients with recurrent optic neuritis (rON) initially seeking treatment. DESIGN: Cross-sectional cohort study. PARTICIPANTS: The study identified patients by searching the Mayo Clinic computerized central diagnostic index (January 2000-March 2017). The 246 eligible patients fulfilled the following criteria: (1) initially seeking treatment for at least 2 consecutive episodes of optic neuritis (ON) and (2) serum available for testing. METHODS: Serum was tested for aquaporin-4 IgG and MOG IgG1 using an in-house validated flow cytometric assay using live HEK293 cells transfected with M1 aquaporin-4 or full-length MOG. MAIN OUTCOMES MEASURES: Aquaporin-4 IgG and MOG IgG1 serostatus, clinical characteristics, and visual outcomes. RESULTS: Among 246 patients with rON at presentation, glial autoantibodies were detected in 32% (aquaporin-4 IgG, 19%; MOG IgG1, 13%); 186 patients had rON only and 60 patients had rON with subsequent additional inflammatory demyelinating attacks (rON-plus group). The rON-only cohort comprised the following: double seronegative (idiopathic), 110 patients (59%); MOG IgG1 positive, 27 patients (15%; 4 with chronic relapsing inflammatory optic neuropathy); multiple sclerosis (MS), 25 patients (13%); and aquaporin-4 IgG positive, 24 patients (13%). The rON-plus cohort comprised the following: aquaporin-4 IgG positive, 23 patients (38%); MS, 22 patients (37%); double seronegative, 11 patients (18%); and MOG IgG1 positive, 4 patients (7%). The annualized relapse rate for the rON-only group was 1.2 for MOG IgG1-positive patients, 0.7 for double-seronegative patients, 0.6 for aquaporin-4 IgG-positive patients, and 0.4 for MS patients (P = 0.005). The median visual acuity (VA) of patients with the worst rON-only attack at nadir were hand movements in aquaporin-4 IgG-positive patients, between counting fingers and hand movements in MOG IgG1-positive patients, 20/800 in idiopathic patients, and 20/100 in MS patients (P = 0.02). The median VA at last follow-up for affected eyes of the rON-only cohort were counting fingers for aquaporin-4 IgG-positive patients, 20/40 for idiopathic patients, 20/25 for MS patients and MOG IgG1-positive patients (P = 0.006). At 5 years after ON onset, 59% of aquaporin-4 IgG-positive patients, 22% of idiopathic patients, 12% of MOG IgG1-positive patients, and 8% of MS patients were estimated to have severe visual loss. CONCLUSIONS: Glial autoantibodies (MOG IgG1 or aquaporin-4 IgG) are found in one third of all patients with rON. Aquaporin-4 IgG seropositivity predicts a worse visual outcome than MOG IgG1 seropositivity, double seronegativity, or MS diagnosis. Myelin oligodendrocyte glycoprotein IgG1 is associated with a greater relapse rate but better visual outcomes.
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Acuaporina 4/inmunología , Autoanticuerpos/sangre , Glicoproteína Mielina-Oligodendrócito/inmunología , Neuritis Óptica/inmunología , Agudeza Visual , Enfermedad Aguda , Adulto , Anticuerpos Antiidiotipos/sangre , Estudios Transversales , Femenino , Citometría de Flujo , Estudios de Seguimiento , Humanos , Inmunoglobulina G/inmunología , Masculino , Persona de Mediana Edad , Neuritis Óptica/sangre , Neuritis Óptica/diagnóstico , Pronóstico , Recurrencia , Estudios Retrospectivos , Tomografía de Coherencia Óptica , Adulto JovenRESUMEN
PURPOSE: To report the prevalence, type, and cause of diplopia in medically and surgically treated patients with glaucoma. DESIGN: Cohort study. PARTICIPANTS: A total of 195 adult patients with glaucoma treated in a glaucoma referral practice. METHODS: A total of 195 adult patients with glaucoma who had undergone surgical or medical management were prospectively enrolled. Forty-seven patients had undergone glaucoma drainage device (GDD) surgery (Baerveldt 350, Baerveldt 250 [Abbott Medical Optics, Abbott Park, IL], or Ahmed FP7 [New World Medical Inc, Rancho Cucamonga, CA]), 61 patients had undergone trabeculectomy, and 87 patients were medically treated. All patients completed the Diplopia Questionnaire to assess diplopia. We defined the presence of diplopia as "sometimes," "often," or "always" in distance straight ahead or reading positions on the Diplopia Questionnaire. A chart review was performed jointly by a strabismus specialist and a glaucoma subspecialist to characterize the type and cause of the diplopia. MAIN OUTCOME MEASURES: Frequency, type, and cause of diplopia. RESULTS: Diplopia was reported in 41 of 195 medically and surgically treated patients (21%) with glaucoma. Binocular diplopia due to the glaucoma procedure was present in 11 of 47 patients (23%) after GDD (95% confidence interval, 12-38), which was significantly greater than in patients after trabeculectomy (2/61 [3%]; 95% confidence interval, 0.4-11; P = 0.002). The most common type of strabismus associated with binocular diplopia due to glaucoma surgery was hypertropia (10/11 GDD cases, 2/2 trabeculectomy cases). Monocular diplopia was found in a similar proportion of medically treated, post-trabeculectomy, and post-GDD cases (4/87 [5%], 4/61 [7%], and 2/47 [4%], respectively). Binocular diplopia not due to surgery was found in similar proportions of GDD, trabeculectomy, and medically treated cases (3/47 [6%], 5/61 [8%], and 10/87 [11%], respectively). CONCLUSIONS: Diplopia may be under-recognized in medically and surgically treated patients with glaucoma, and standardization of ascertaining patient symptoms using the Diplopia Questionnaire may be useful in these patients. Diplopia was more commonly seen after GDD than trabeculectomy, typically a noncomitant restrictive hypertropia. The prevalence of monocular diplopia and binocular diplopia unrelated to glaucoma surgery was similar among medical and surgical groups. It is important to counsel patients on the higher occurrence of diplopia associated with GDD surgery.
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Diplopía/epidemiología , Implantes de Drenaje de Glaucoma/efectos adversos , Glaucoma/cirugía , Trabeculectomía/efectos adversos , Adulto , Anciano , Anciano de 80 o más Años , Diplopía/diagnóstico , Diplopía/etiología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Prevalencia , Estudios Prospectivos , Estrabismo/diagnósticoRESUMEN
PRCIS: Damage to disposable selective laser trabeculoplasty (SLT) lenses correlated with the amount of total laser energy. Furthermore, greater lens damage was associated with diminished patient treatment response. PURPOSE: Disposable SLT lenses have been observed to be damaged during use by laser, potentially affecting therapeutic response. This study sought to identify factors associated with the magnitude of lens damage and its effect on treatment outcomes. PATIENT AND METHODS: We analyzed 113 eyes from 82 patients who underwent SLT between 2020 and 2021 at an American academic medical center. For each procedure, we recorded baseline patient characteristics, procedure settings, physician age (as a possible factor causing laser defocus due to accommodation), and area of lens damage. Treatment response was calculated as the difference between preoperative and postoperative intraocular pressure (IOP). RESULTS: Area of lens damage was associated with greater total laser energy ( r =0.34, P <0.001) and greater mean energy per application ( r =0.37, P <0.001). IOP reduction decreased with increasing lens damage ( r =-0.19, P =0.022). Lens damage was not associated with patient characteristics indicative of glaucoma severity or user settings (physician age and slit lamp used). Greater IOP reduction was associated with higher preop IOP ( r =0.46, P <0.001) and a smaller cup-to-disc ratio ( r =0.22, P =0.036). IOP reduction was not associated with retinal nerve fiber layer thickness, patient age, or the number of glaucoma medications. CONCLUSIONS: Increased laser damage to disposable SLT lenses was associated with diminished treatment effect. The amount of damage was correlated with total and average laser energy, but not patient or physician factors. Overall, these findings call for reconsideration of using disposable SLT lenses as part of routine practice.
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Presión Intraocular , Terapia por Láser , Trabeculectomía , Humanos , Trabeculectomía/métodos , Trabeculectomía/efectos adversos , Presión Intraocular/fisiología , Femenino , Masculino , Terapia por Láser/métodos , Anciano , Persona de Mediana Edad , Estudios Retrospectivos , Glaucoma de Ángulo Abierto/cirugía , Glaucoma de Ángulo Abierto/fisiopatología , Equipos Desechables , Cristalino/cirugía , Agudeza Visual/fisiología , Glaucoma/cirugía , Glaucoma/fisiopatología , Tonometría OcularRESUMEN
PRCIS: Surgical and clinical success rates were similar among Ahmed FP7, and Baerveldt 250 and 350 glaucoma drainage devices at three years. PURPOSE: To compare rates of surgical and clinical success in patients with Ahmed FP7 (FP7), Baerveldt 250 (B250), or Baerveldt 350 (B350) glaucoma drainage devices (GDDs). DESIGN: A retrospective cohort study. METHODS: A total of 157 eyes of 129 adult patients with FP7, B250, B350 GDDs, and 190 eyes of 99 medically controlled glaucoma patients were enrolled at a tertiary care institution from August 2017 through July 2019. They were followed through April 2020. The main outcome measures included surgical and clinical failure. Surgical failure was defined as intraocular pressure (IOP) outside 5-21 mm Hg, IOP reduced <20% below baseline, additional glaucoma surgery, GDD removal, or no light perception. Eyes that did not meet their goal IOP ranges or required secondary glaucoma interventions were deemed clinical failures. RESULTS: A total of 43 (12.4%) FP7, 36 (10.4%) B250, 78 (22.5%) B350, and 190 (54.8%) medically treated control eyes were enrolled. By the postoperative year 3 visit, 10 (23.2%) FP7, 11 (30.6%) B250, and 32 (41.0%) B350 eyes had met a surgical failure criterion ( P =0.127). There were no significant differences in the numbers of eyes meeting their IOP target ranges ( P =0.510), and rates of secondary glaucoma surgeries ( P =0.270). Overall clinical success was attained among 83.3% FP7, 81.8% B250, and 68.0% B350 eyes ( P =0.447). CONCLUSIONS: The GDD groups were similar in their rates of success, based on both the surgical and clinical success definitions.
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Implantes de Drenaje de Glaucoma , Glaucoma , Adulto , Humanos , Presión Intraocular , Estudios Retrospectivos , Resultado del Tratamiento , Implantación de Prótesis , Complicaciones Posoperatorias/cirugía , Agudeza Visual , Glaucoma/cirugíaRESUMEN
PRCIS: When comparing patients on systemic immunosuppressive therapy to those without, there was no difference in intraocular pressure (IOP) early after SLT; however, at 1 year following SLT, IOP was higher in the immunosuppression group compared with controls. PURPOSE: To determine whether patients taking systemic immunosuppressive medications have a different IOP-lowering response to selective laser trabeculoplasty (SLT) compared with a control group of patients. METHODS: All patients who underwent SLT at Mayo Clinic 2017-2021 were identified. Patients on systemic immunosuppressive medications at the time of SLT were compared with control patients not receiving systemic immunosuppressive medications. The primary endpoints of this study were the percentage IOP reduction at 1 to 2, 3 to 6, and 12 months. Additional analyses included the percentage of patients who did not require additional therapy at each time point. RESULTS: There were 108 eyes of 72 patients that underwent SLT in the immunosuppressed group and 1997 eyes of 1417 patients in the control group. There was no significant difference in age-adjusted change in IOP between groups at the first postoperative visit 1 to 2 months following SLT (-18.8±20.7% vs. -16.0±16.5%, P =0.256) or 3-6 months following SLT (-15.2±21.6% vs. -18.3±23.2%, P =0.062). However, at 12 months following SLT, the IOP reduction in the immunosuppressive therapy group was significantly less compared with the control group (-15.1±21.2% vs. -20.3±22.9%, P =0.045). There was no difference between groups in the number of additional treatments during the study intervals. CONCLUSION: Patients in the systemic immunosuppressive therapy group showed equivalent early IOP-lowering after SLT compared with a control group, but the treatment response was diminished at 1 year. Further studies investigating IOP regulation after SLT in immunosuppressed patients are needed.
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Terapia por Láser , Láseres de Estado Sólido , Hipotensión Ocular , Trabeculectomía , Humanos , Lactante , Presión Intraocular , Antihipertensivos/uso terapéutico , Terapia de Inmunosupresión , Inmunosupresores/uso terapéutico , Hipotensión Ocular/tratamiento farmacológico , Láseres de Estado Sólido/uso terapéutico , Resultado del TratamientoRESUMEN
BACKGROUND: Despite, the potential clinical utility of 60-4 visual fields, they are not frequently used in clinical practice partly, due to the purported impact of facial contour on field defects. The purpose of this study was to design and test an artificial intelligence-driven platform to predict facial structure-dependent visual field defects on 60-4 visual field tests. METHODS: Subjects with no ocular pathology were included. Participants were subject to optical coherence tomography, 60-4 Swedish interactive thresholding algorithm visual field tests and photography. The predicted visual field was compared with observed 60-4 visual field results in subjects. Average and point-specific sensitivity, specificity, precision, negative predictive value, accuracy, and F1-scores were primary outcome measures. RESULTS: 30 healthy were enrolled. Three-dimensional facial reconstruction using a convolution neural network (CNN) was able to predict facial contour-dependent 60-4 visual field defects in 30 subjects without ocular pathology. Overall model accuracy was 97%±3% and 96%±3% and the F1-score, dependent on precision and sensitivity, was 58%±19% and 55%±15% for the right eye and left eye, respectively. Spatial-dependent model performance was observed with increased sensitivity and precision within the far inferior nasal field reflected by an average F1-score of 76%±20% and 70%±29% for the right eye and left eye, respectively. CONCLUSIONS: This pilot study reports the development of a CNN-enhanced platform capable of predicting 60-4 visual field defects in healthy controls based on facial contour. Further study with this platform may enhance understanding of the influence of facial contour on 60-4 visual field testing.
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OBJECTIVE: To compare primary selective laser trabeculoplasty (SLT) response in uveitic, steroid-induced, primary open-angle glaucoma (POAG) and pseudoexfoliative glaucoma (PEX). DESIGN: Single-centre retrospective case-control study. PARTICIPANTS: Patients with uveitic glaucoma, steroid-induced glaucoma, POAG, or PEX who had their first SLT. METHODS: Eyes with POAG or PEX were in control groups. Eyes with steroid-induced or uveitic glaucoma were in experimental groups. Change in intraocular pressure from baseline, treatment failure, complication rates, and medication use were compared using rank-sum and log-rank tests. RESULTS: Six-hundred and eight eyes of 433 patients were enrolled. Steroid-induced glaucoma eyes had higher mean baseline pressure and a decrease in pressure at 3-8 weeks (27.6-17.4 mm Hg) than those with PEX (21.7-16.5 mm Hg; p < 0.001) or POAG (18.6-14.9 mm Hg; p ≤ 0.025). Failure rates after 2 years were lower in steroid-induced glaucoma (54%) than in PEX (84%; pâ¯=â¯0.01) or POAG (84%; pâ¯=â¯0.005). This survival benefit persisted when excluding patients with changes to their steroid dosing (p ≤ 0.03) but showed mixed results when compared with patients with a baseline pressure of 25mm Hg or greater (pâ¯=â¯0.020 vs PEX; pâ¯=â¯0.67 vs POAG). At 18 months, the steroid-induced group decreased ocular hypotensive medication use (3.5-1.9; pâ¯=â¯0.005); the uveitic group increased medication use (2.7-3.5; pâ¯=â¯0.02). CONCLUSIONS: SLT is an effective treatment for steroid-induced glaucoma, with greater response and a lower failure rate than in PEX and primary POAG, although high baseline intraocular pressure may be a confounder. Judicious use of SLT can be considered in uveitic glaucoma.
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Glaucoma de Ángulo Abierto , Glaucoma , Terapia por Láser , Trabeculectomía , Estudios de Casos y Controles , Glaucoma/inducido químicamente , Glaucoma/cirugía , Glaucoma de Ángulo Abierto/inducido químicamente , Glaucoma de Ángulo Abierto/diagnóstico , Glaucoma de Ángulo Abierto/cirugía , Humanos , Presión Intraocular , Terapia por Láser/métodos , Rayos Láser , Estudios Retrospectivos , Esteroides/efectos adversos , Trabeculectomía/métodos , Resultado del TratamientoRESUMEN
PURPOSE: There is an increased risk for development of blepharoptosis after incisional glaucoma surgery. Data on safety and efficacy of ptosis repair in this group of patients in limited. The goal of this study is to evaluate outcomes and identify potential risk factors for failure of ptosis repair in eyes with history of incisional glaucoma surgery. METHODS: A retrospective chart review was performed of all patients who underwent incisional glaucoma surgery, specifically trabeculectomy or implantation of glaucoma drainage device (GDD), and subsequent ptosis repair at a single institution from 2009 to 2019. Ptosis surgery outcomes were compared to a control group who underwent ptosis repair after cataract surgery. RESULTS: Seventy-eight eyes of 64 patients were included in the glaucoma surgery group. The rate of severe ptosis (margin reflex distance 1 ⩽ 0 mm) among glaucoma surgery patients was higher compared to control (35 of 78 (44.9%) vs 23 of 82 (28.6%). Ptosis repair was successful in 59 of 78 eyes (75.6%), which was similar to control. Risk for revision surgery was increased more than five-fold in the GDD group compared to control. There were no cases of early or late bleb-related complications. CONCLUSIONS: Ptosis repair can be performed safely in patients after incisional glaucoma surgery. Müller muscle conjunctival resection and external levator advancement are equally effective. Patients with history of GDD should be advised about the potentially increased risk of need for revision surgery.
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Blefaroptosis , Glaucoma , Trabeculectomía , Blefaroptosis/cirugía , Glaucoma/cirugía , Humanos , Músculos Oculomotores/cirugía , Complicaciones Posoperatorias , Estudios RetrospectivosRESUMEN
PURPOSE: New-onset persistent diplopia has become a common complication after glaucoma drainage device (GDD) placement. Understanding the orbital anatomy of such patients may provide information regarding risk of diplopia, GDD selection, and post-operative management. The purpose of this study was to examine the orbital anatomic differences in diplopic and non-diplopic patients after GDD implantation using high-resolution MRI. METHODS: Seven eyes (N = 4 with diplopia and N = 3 without diplopia after GDD placement) of seven patients that had undergone placement of Baerveldt 250 (B250), Baerveldt 350 (B350), or Ahmed FP7 (FP7) GDD were prospectively enrolled at a single institution. All patients underwent a 3.0T orbital MRI with 3D volumetric T1 and T2 weighted sequence. Images were analyzed for orbital volume, axial length, orbital distances, presence of superior rectus-lateral rectus (SR-LR) band, position of GDD, and SR-LR angles. RESULTS: Patients with diplopia had smaller mean ± SD orbital axial (911.5 ± 111.8 mm3 vs 931.7 ± 79.7 mm3) and coronal volumes (1162.5 ± 145.5 mm3 vs 1180 ± 34.6 mm3) compared to non-diplopic patients. Average orbital rim distances were larger for the diplopic group. The SR-LR displacement angle for diplopic patients was larger (101.6° ± 8.1 vs 94.7° ± 17.6) while the SR-LR quadrantic angle (86.6° ± 4.2 vs 89.1° ± 4.3) was smaller. SR-LR band was present and intact in all patients. GDD malpositioning was not evident in any patient. CONCLUSION: The decreased orbital axial and coronal volumes as well as increased orbital rim distances in diplopic patients suggests the need for further studies to understand the role of orbital anatomy in occurrence of diplopia. Dynamic MRI imaging may be helpful in identifying differences in extraocular muscle function that reveal an etiology of diplopia in patients with GDD implantation.
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Implantes de Drenaje de Glaucoma , Glaucoma , Diplopía/etiología , Humanos , Presión Intraocular , Imagen por Resonancia Magnética , Músculos Oculomotores/diagnóstico por imagen , Músculos Oculomotores/cirugía , Estudios RetrospectivosRESUMEN
PURPOSE: To compare health-related quality of life (HRQoL) in patients with Ahmed FP7 (FP7), Baerveldt 250 (B250) and Baerveldt 350 (B350) glaucoma drainage device (GDDs), and medically treated controls. METHODS: This was a prospective cohort study from August 2017 to July 2019. The NEI 25-Item Visual Function Questionnaire (VFQ-25), the Adult Strabismus-20 questionnaire (AS-20) and the Diplopia Questionnaire were conducted ≥30 days postoperatively in GDD patients, on enrolment for controls. Age, sex, treatment type, visual acuity, mean deviation and diplopia were evaluated for association with HRQoL RESULTS: Of the 128 GDD patients enrolled, 35 (27.3%) had FP7, 32 (25.0%) had B250 and 61 (47.7%) had B350. In univariate analysis, decreased HRQoL was associated with younger age (r2 range 0.042-0.071), diplopia (r2 range 0.039-0.119), GDD treatment (r2 range 0.023-0.103), lower visual acuity (r2 range 0.021-0.215) and worse mean deviation (r2 range 0.029-0.131). All GDD groups had lower HRQoL subscores than the controls. HRQoL scores were lower compared with controls among B350 patients for AS-20 Self-perception subscale, B250 and B350 for Reading and General Function subscales, and FP7 and B350 for VFQ-25 Visual Functioning subscale. There were no significant differences among the GDDs. CONCLUSIONS: Glaucoma patients with a younger age, diplopia, lower visual acuity, worse mean deviation or a GDD had lower HRQoL. Those with B350 had lower self-perception scores, consistent with previous reports in the literature. This subscale was not diminished in FP7 or B250, so the decreased self-perception scores may be due to greater visibility or awareness of the B350.
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Implantes de Drenaje de Glaucoma , Calidad de Vida , Adulto , Diplopía , Humanos , Presión Intraocular , Estudios Prospectivos , Encuestas y CuestionariosRESUMEN
PRCIS: Kaplan-Meier analysis was used to compare the rate of corneal grafting after glaucoma drainage device (GDD) placement in pediatric and adult patients. Adults were at an increased risk of receiving a corneal graft after device placement. PURPOSE: The goal of this study was to compare the rate of corneal graft implantation after GDD placement in pediatric and adult patients. PATIENTS: Patients receiving a GDD between January 1, 1985 and December 31, 2017 were selected from the medical records. Patients receiving their first device while <18 years of age were considered children for the extent of the study. METHODS: We compared the rate of receiving a corneal graft after GDD implantation using Kaplan-Meier analysis. Baseline patient characteristics and surgical characteristics were compared using a generalized estimating equation. RESULTS: Corneal grafting occurred in 8.6% of adults and 4.7% of children from the original cohort. The rate of receiving a corneal graft at 5, 10, and 15 years was 9.4%, 16.8%, 39.4% and 1.6%, 1.6%, 12.5% for adults and children, respectively. However, certain characteristics were different between the 2 groups. CONCLUSION: Adult patients were more likely to receive a corneal graft after GDD placement. However, pediatric and adult patients differed in the prevalence of preexisting corneal disease, glaucoma type, GDD type used, and types of previous surgeries. Elucidation of the impact of these factors on corneal graft rate requires a larger cohort size.
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Trasplante de Córnea , Implantes de Drenaje de Glaucoma , Glaucoma , Adulto , Niño , Glaucoma/cirugía , Humanos , Presión Intraocular , Implantación de Prótesis , Estudios RetrospectivosRESUMEN
PRECIS: Diplopia was present in ~20% of patients with Ahmed FP7 (FP7) or Baerveldt 350 (B350), compared with 5% to 6% in those with Baerveldt 250 (B250) or controls, suggesting risk of diplopia should be included in preoperative counseling. PURPOSE: The purpose of this study was to examine the prevalence of diplopia and strabismus in patients with B250, B350, or FP7 glaucoma drainage devices (GDD). MATERIALS AND METHODS: In this cohort study, glaucoma patients 18 years and above who had received, or would be receiving, a B250, B350, or FP7 GDD, and medically treated controls were consecutively enrolled from August 8, 2017, through July 31, 2019. The Diplopia Questionnaire was administered ≥30 days postoperatively, and upon enrollment to the controls. All diplopic patients underwent orthoptic measurements, which were reviewed by a strabismus specialist. Patients with GDDs in quadrants other than superotemporal, multiple GDDs, or scleral buckles were excluded. Bonferroni correction was applied for pairwise comparisons. RESULTS: Diplopia was reported in 23/129 (17.8%) GDD patients and 5/99 (5.1%) control patients (P=0.003): 8/35 (22.9%) FP7, 2/32 (6.3%) B250, and 13/62 (21.0%) B350, with significant differences between FP7 versus controls (P=0.014) and B350 versus controls (P=0.011). Diplopia was attributable to GDD in 2 FP7 (5.9%, 95% confidence interval: 0.7-19.2), 0 B250, and 4 B350 (6.5%, 95% confidence interval: 1.7-15.2) patients, without significant differences between the GDDs. CONCLUSIONS: Patients with the larger (B350) or the higher profile plate (FP7) GDDs were more likely to experience diplopia than controls, and diplopia was attributable to the GDD in ~6% of patients with either a FP7 or a B350 GDD. Since diplopia can affect patients' quality of life, preoperative counseling for GDD surgery, particularly B350 and FP7, should include a discussion of the risk of diplopia.
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Implantes de Drenaje de Glaucoma , Glaucoma , Estudios de Cohortes , Diplopía/epidemiología , Humanos , Presión Intraocular , Calidad de Vida , Resultado del TratamientoRESUMEN
PRCIS: In this longitudinal study of patients with open-angle (OAG), pseudoexfoliative (PXE), or neovascular glaucoma (NVG) receiving glaucoma drainage devices (GDD), posttube cumulative rates of reoperation, corneal graft, and visually threatening complications (VT-complications) increased beyond 5 years and were not significantly affected by glaucoma type. PURPOSE: To study the need for additional glaucoma surgery and development of complications after first GDD surgery in eyes with primary OAG, PXE, or NVG glaucoma. PATIENTS AND METHODS: There were 306 eyes with OAG (n=185), PXE (n=60), or NVG (n=61) glaucoma who received a first GDD between 1996 and 2017. Outcomes including glaucoma reoperation, corneal graft procedure, and VT-complications after GDD were measured. Kaplan-Meier analysis was used to compare cumulative rate of reaching outcomes over time after GDD placement among the 3 glaucoma groups. RESULTS: When comparing the OAG, PXE, and NVG groups, there were no significant differences in post-GDD cumulative rates of reoperation (P=0.33), corneal graft (P=0.26), or VT-complications (P=0.65) over time. For all eyes, the overall cumulative rates for each outcome measure increased beyond 5 years, and specific Kaplan-Meier rates (5-y, 10-y) included: reoperation (16%, 25%), corneal graft (6%, 12%), VT-complications (9%, 14%). When comparing specific GDDs, the Ahmed FP7 had a higher cumulative reoperation rate over time compared with the Baerveldt 350 (P=0.019). CONCLUSION: Glaucoma type did not significantly affect post-GDD cumulative rates of reoperation, corneal graft, and VT-complication among the OAG, PXE, and NVG groups. For all eyes, cumulative rates of reoperation, corneal graft, and VT-complications increased beyond 5 years. The Ahmed FP7 had a significantly higher cumulative reoperation rate compared with the Baerveldt 350 over time.
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Implantes de Drenaje de Glaucoma , Glaucoma , Glaucoma/cirugía , Implantes de Drenaje de Glaucoma/efectos adversos , Humanos , Presión Intraocular , Estudios Longitudinales , Implantación de Prótesis , Estudios Retrospectivos , Resultado del Tratamiento , Agudeza VisualRESUMEN
Importance: Ocular hypertension is an important risk factor for the development of primary open-angle glaucoma (POAG). Data from long-term follow-up can be used to inform the management of patients with ocular hypertension. Objective: To determine the cumulative incidence and severity of POAG after 20 years of follow-up among participants in the Ocular Hypertension Treatment Study. Design, Setting, and Participants: Participants in the Ocular Hypertension Treatment Study were followed up from February 1994 to December 2008 in 22 clinics. Data were collected after 20 years of follow-up (from January 2016 to April 2019) or within 2 years of death. Analyses were performed from July 2019 to December 2020. Interventions: From February 28, 1994, to June 2, 2002 (phase 1), participants were randomized to receive either topical ocular hypotensive medication (medication group) or close observation (observation group). From June 3, 2002, to December 30, 2008 (phase 2), both randomization groups received medication. Beginning in 2009, treatment was no longer determined by study protocol. From January 7, 2016, to April 15, 2019 (phase 3), participants received ophthalmic examinations and visual function assessments. Main Outcomes and Measures: Twenty-year cumulative incidence and severity of POAG in 1 or both eyes after adjustment for exposure time. Results: A total of 1636 individuals (mean [SD] age, 55.4 [9.6] years; 931 women [56.9%]; 1138 White participants [69.6%]; 407 Black/African American participants [24.9%]) were randomized in phase 1 of the clinical trial. Of those, 483 participants (29.5%) developed POAG in 1 or both eyes (unadjusted incidence). After adjusting for exposure time, the 20-year cumulative incidence of POAG in 1 or both eyes was 45.6% (95% CI, 42.3%-48.8%) among all participants, 49.3% (95% CI, 44.5%-53.8%) among participants in the observation group, and 41.9% (95% CI, 37.2%-46.3%) among participants in the medication group. The 20-year cumulative incidence of POAG was 55.2% (95% CI, 47.9%-61.5%) among Black/African American participants and 42.7% (95% CI, 38.9%-46.3%) among participants of other races. The 20-year cumulative incidence for visual field loss was 25.2% (95% CI, 22.5%-27.8%). Using a 5-factor baseline model, the cumulative incidence of POAG among participants in the low-, medium-, and high-risk tertiles was 31.7% (95% CI, 26.4%-36.6%), 47.6% (95% CI, 41.6%-53.0%), and 59.8% (95% CI, 53.1%-65.5%), respectively. Conclusions and Relevance: In this study, only one-fourth of participants in the Ocular Hypertension Treatment Study developed visual field loss in either eye over long-term follow-up. This information, together with a prediction model, may help clinicians and patients make informed personalized decisions about the management of ocular hypertension. Trial Registration: ClinicalTrials.gov Identifier: NCT00000125.
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Primary care physicians are at the forefront of patient care and often are the first clinicians to triage and diagnose any eye-related complaints. They must be able to quickly identify vision-threatening pathologies, as delay in treatment of an ocular emergency can result in permanent vision loss. This concise review describes the definition, presentation, examination, and management of various ophthalmic emergencies including blunt ocular trauma, chemical ocular injury, orbital cellulitis, endophthalmitis, acute angle closure glaucoma, optic neuritis, giant cell arteritis, central retinal artery occlusion, retinal detachment, and homonymous hemianopia in a succinct manner.
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Urgencias Médicas , Oftalmopatías/diagnóstico , Oftalmopatías/terapia , Lesiones Oculares/diagnóstico , Lesiones Oculares/terapia , Atención Primaria de Salud , HumanosRESUMEN
PURPOSE: Anti-vascular endothelial growth factor (VEGF) injections, while used to effectively treat numerous retinal vascular conditions, can be associated with transient and prolonged ocular hypertension. There is minimal literature detailing the development of normal-tension glaucoma (NTG) following intravitreal anti-VEGF injections. OBSERVATIONS: A 38-year-old Caucasian male with no medical or ocular history was diagnosed with an inferior HRVO with macular edema in the left eye. The patient received a total of eleven monthly intravitreal aflibercept injections over one year, with maintenance of stable vision and intraocular pressure (IOP) throughout the treatment period and during follow-up. Nine months after the last aflibercept injection, cup-to-disc asymmetry, inferior thinning of retinal nerve fiber layer (RNFL), and reduced superior visual field was evident in the left eye. Clinically, the patient was consistent with normal tension glaucoma and thus, the patient was started on daily timolol drops; however, the role of the glaucomatous findings being secondary to repeated injection-related IOP elevations is possible. CONCLUSIONS AND IMPORTANCE: While the clinical features based on visual fields and RNFL thinning with unmedicated normotensive IOP may suggest NTG in a patient, this clinical presentation may be a masquerader of NTG with the etiology of the glaucoma optic neuropathy caused by cumulative impact of transient IOP elevations secondary to intravitreal injections.
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Introduction: This manuscript describes data from an original study, simulating a tele-glaucoma programme in an established clinic practice with an interdisciplinary team. This is a 'real life' trial of a telemedicine approach to see a follow-up patient. The goal is to evaluate the accuracy of such a programme to detect worsening and/or unstable disease. Such a programme is attractive since in-clinic time could be reduced for both the patient and provider. This study evaluates agreement between in-person and remote assessment of glaucoma progression. Methods: A total of 200 adult glaucoma patients were enrolled at a single institution. The in-person assessment by an optometrist or glaucoma specialist at time of enrolment was used as the gold standard for defining progression. Collated clinical data were then reviewed by four masked providers who classified glaucoma as progression or non-progression in each eye by comparing data from enrolment visit to data from the visit immediately prior to enrolment. Agreement of glaucoma progression between the masked observer and the in-person assessment was determined using Kappa statistics. Intra-observer agreement was calculated using Kappa to compare in-person to remote assessment when both assessments were performed by the same provider (n = 279 eyes). Results: A total of 399 eyes in 200 subjects were analysed. Agreement between in-person versus remote assessment for the determination of glaucoma progression was 63%, 62%, 69% and 68% for each reader 14 (kappa values = 0.19, 0.20, 0.35 and 0.33, respectively). For intra-observer agreement, reader 1 agreed with their own in-person assessment for 65% of visits (kappa = 0.18). Discussion: Intra-observer agreement was similar to the agreement for each provider who did not see the patient in person. This similarity suggests that telemedicine may be equally effective at identifying glaucomatous disease progression, regardless of whether the same provider performed both in-clinic and remote assessments. However, fair agreement levels highlight a limitation of using only telemedicine data to determine progression compared with clinical detail available during in-patient assessment.