Prevalence and Correlation to Visual Field Loss

[Posted 20/Jul/2022]

AUDIENCE: Ophthalmology, Family Medicine

KEY FINDINGS: Charles Bonnet Syndrome was not a rare condition in patients with glaucoma. Patients with a combination of advanced VFL and low BCVA had the highest risk of CBS; however, 1 of 3 patients with CBS had a BCVA of >=0.5 in both eyes. These findings emphasize the importance of being attentive to symptoms of CBS in patients with glaucomatous VFL even when visual acuity is preserved.

BACKGROUND: Objective of this study is to determine the prevalence and characteristics of Charles Bonnet Syndrome (CBS) and its relation to visual field loss (VFL) in patients with open-angle glaucoma (OAG).

DETAILS: Patients attending the glaucoma outpatient department of the Skane University hospital, Malmo, Sweden, between April 1, 2018, and December 31, 2018, were consecutively evaluated for inclusion. Potentially eligible patients admitting to having complex visual hallucinations were interviewed to explore the characteristics of their hallucinatory experiences. Recent automated visual field examinations were available for all participants, and swept-source OCT was performed in participants with CBS to rule out previously undiagnosed macular pathology. The correlation between potential risk factors and CBS was evaluated with logistic regression analysis. Charles Bonnet Syndrome was not a rare condition in patients with glaucoma. Patients with a combination of advanced VFL and low BCVA had the highest risk of CBS; however, 1 of 3 patients with CBS had a BCVA of >=0.5 in both eyes. These findings emphasize the importance of being attentive to symptoms of CBS in patients with glaucomatous VFL even when visual acuity is preserved.

Copyright © The American Academy of Ophthalmology. All rights reserved.

Source: Peters, D., Molander, S., Lomo, T., et al. (2022). Charles Bonnet Syndrome in Patients with Open-Angle Glaucoma: Prevalence and Correlation to Visual Field Loss. Ophthalmology Glaucoma. 2022; 5(3): 337-344. Published: May 1, 2022. DOI: Charles Bonnet Syndrome in Patients with Open-Angle Glaucoma.

[Posted 12/Dec/2025]

AUDIENCE: Ophthalmology, Internal Medicine

KEY FINDINGS: AAC risk was less than 1 in 40,000 per dilation in a high-volume TDRS program serving a diverse safety net population, supporting the overall safety of dilation in this setting. Further discussion about AAC risk as a contraindication to dilation is warranted.

BACKGROUND: Pharmacologic pupillary dilation is vital for eye disease screening but is often avoided due to concerns about triggering acute angle closure (AAC), a sight-threatening ophthalmic emergency. Aim of this study was to assess AAC incidence after dilation and validate the use of International Classification of Diseases (ICD) codes for identifying AAC cases.

DETAILS: his retrospective cohort study used data from a primary care–based teleretinal diabetic retinopathy screening (TDRS) program. Eligible participants were Los Angeles County Department of Health Services patients who underwent teleretinal screening by dilated fundus photography between August 23, 2013, and March 1, 2024. Potential AAC cases were identified using ICD codes for angle closure, including AAC glaucoma, primary angle-closure glaucoma, and anatomical narrow angle, within 3 months of dilation. All urgent care, emergency department, and eye clinic encounters within the next calendar day after TDRS and encounters with Current Procedural Terminology codes for iridectomy/iridotomy or lens extraction within 14 calendar days of TDRS were also identified. Manual medical record review was conducted to verify AAC cases and extract clinical information. Data were analyzed from July 2024 to June 2025. Of 84,008 included patients, 46,255 (55.1%) were female, and the mean (SD) age was 55.4 (10.7) years. There were a total of 168,796 dilations, with a mean (SD) of 2.01 (1.50) dilations per patient. Manual medical record review confirmed 4 AAC cases after dilation: 3 coded as AAC glaucoma and 1 as anatomical narrow angle. The AAC risk was 2.4 (95% CI, 0.05-4.69) per 100,000 dilations (0.002%) or 4.8 (95% CI, 0.10-9.43) per 100,000 patients (0.005%). All 4 AACs occurred in female patients, had narrow angles in the nonpresenting eye on gonioscopy, and presented within 1 day with AAC symptoms, including eye pain and blurry vision.

Copyright © American Medical Association. All Rights Reserved.

Source: Lang, T. Z., Xu, B. Y., Li, Z., et al. Acute Angle Closure Incidence in a Large Countywide Safety Net Teleretinal Screening Program. JAMA Ophthalmology. 2025; 143(11): 883-890. Published: December, 2025. DOI: 10.1001/jamaophthalmol.2025.3162.

[Posted 8/Sep/2025]

AUDIENCE: Ophthalmology, Internal Medicine

KEY FINDINGS: Larger optic disc size is associated with faster cpRNFL thinning in glaucoma, independent of race. Although previous studies have indicated that Black individuals may be at higher risk for glaucoma development, the present study suggests that race may not be a significant predictor of faster cpRNFL thinning when controlling for optic disc size and other clinical and demographic factors in glaucoma.

BACKGROUND: Aim of this study is to investigate the association between optic disc size and circumpapillary retinal nerve fiber layer (cpRNFL) thinning in eyes with preperimetric glaucoma and glaucoma.

DETAILS: A total of 841 eyes (554 primary open angle glaucoma and 287 preperimetric glaucoma) from 553 patients who had at least 4 visits and 2 years of follow-up using OCT participated in the study. Multivariable linear mixed-effects modeling was used to estimate the effect of optic disc size on cpRNFL thinning while controlling for covariates. To eliminate the floor effect, eyes with baseline visual field mean deviation less than -14 dB were excluded. Of the participants, 189 (34.2%) were Black, 338 (61.1%) were White, 20 (3.6%) were Asian, and 6 (1.1%) were another race or ethnicity. Mean follow-up period was 5.3 (95% confidence interval [CI], 5.2-5.5) years, and the mean rate of cpRNFL change was -0.54 (95% CI, -0.61 to 0.47) µm/year. After adjusting for covariates with the Littmann's formula correction, larger optic disc size was associated with faster cpRNFL thinning (-0.03; 95% CI, -0.05 to 0.00) µm/year faster per 0.1 mm² larger; P = 0.034), while no significant differences were found for race and its interaction with optic disc size.

Copyright © The American Academy of Ophthalmology. All rights reserved.

Source: Nishida, T., Vincent Q., Moghimi, S., et al. (2024). Optic Disc Size and Circumpapillary Retinal Nerve Fiber Layer Thinning in Glaucoma. Ophthalmology Glaucoma. 2025; 8(4): 343-350. Published: July-August, 2025. DOI: 10.1016/j.ogla.2025.02.003.

[Posted 28/Aug/2025]

AUDIENCE: Neurology, Pediatric, Neurosurgery

KEY FINDINGS: Infants up to 6 weeks of age with genetically diagnosed SMA who were treated with risdiplam before the development of clinical signs or symptoms appeared to have better functional and survival outcomes at 12 and 24 months than untreated infants in natural history studies. Larger, controlled studies with longer follow-up are needed to further understand the relative efficacy and safety of presymptomatic treatment of SMA with risdiplam.

BACKGROUND: Risdiplam, an oral pre–messenger RNA splicing modifier, is an efficacious treatment for persons with symptomatic spinal muscular atrophy (SMA). The safety and efficacy of risdiplam in presymptomatic disease are unclear.

DETAILS: Authors conducted an open-label study of daily oral risdiplam (with the dose adjusted to 0.2 mg per kilogram of body weight) in infants 1 day (birth) to 42 days of age with genetically diagnosed SMA but without strongly suggestive clinical signs or symptoms. The primary outcome, assessed in infants with two SMN2 copies and a baseline ulnar compound muscle action potential (CMAP) amplitude of at least 1.5 mV, was the ability to sit without support at month 12. Natural history studies have shown that the majority of infants with two SMN2 copies who are untreated would have a severe SMA phenotype (type 1), would never sit independently, would receive permanent ventilation and feeding support, or would die by 13 months of age. Secondary outcomes that were assessed over a period of 24 months included survival, ventilatory support, motor milestones, the development of clinically manifested SMA, feeding, and growth. A total of 26 infants with two, three, or four or more copies of SMN2 were enrolled. After 12 months of treatment, 21 infants (81%) could sit unsupported for 30 seconds, 14 (54%) could stand alone, and 11 (42%) could walk alone. A total of 4 of 5 infants (80%; 95% confidence interval, 28 to 100) with two SMN2 copies and a baseline ulnar CMAP amplitude of at least 1.5 mV were able to sit without support for at least 5 seconds. Three infants were withdrawn from the study by a parent or caregiver after the month 12 visit. Of 23 infants who completed 24 months of treatment, all were alive without the use of permanent ventilation or feeding support. Over a period of 24 months, nine treatment-related adverse events were reported in 7 infants; none of these events were serious.

Copyright © Massachusetts Medical Society. All rights reserved.

Source: Finkel, R. S., Servais, L., Vlodavets, D., et al. (2024). Risdiplam in Presymptomatic Spinal Muscular Atrophy. N Engl J Med. 2025; 393(7): 671-682. Published: August 13, 2025. DOI: 10.1056/NEJMoa2410120.

[Posted 22/Aug/2025]

AUDIENCE: All Healthcare Professionals

KEY FINDINGS:

BACKGROUND: Recurrent Respiratory Papillomatosis (RRP) is a rare and chronic condition caused by human papillomavirus (HPV) types 6 and 11. The disease leads to the formation of benign tumors in the respiratory tract, most often in the larynx, which can cause significant symptoms like voice changes and difficulty breathing. Historically, the primary treatment for RRP has been repeated surgical removal of the tumors, as there have been no approved medical therapies to address the underlying cause.

DETAILS: The U.S. Food and Drug Administration (FDA) has approved Papzimeos (zopapogene imadenovec-drba), a groundbreaking immunotherapy, for the treatment of adult patients with RRP. This therapy is a non-replicating adenoviral vector that works by stimulating a targeted immune response against the HPV-infected cells. It is administered via a subcutaneous injection and represents the first non-surgical therapeutic option for this rare disease, offering a new approach beyond traditional surgical management.

The approval of Papzimeos was based on data from a single-arm, open-label trial. The study demonstrated that 51.4% of patients who received the treatment achieved a complete response, defined as not needing any further surgical intervention for 12 months following the treatment. The clinical benefits were shown to be durable for most patients over a two-year period and correlated with the development of specific T-cells targeting HPV 6 and 11. The therapy had a favorable safety profile with no serious treatment-related adverse events.

Key information:

• First-of-its-kind Approval: Papzimeos is the first approved medical therapy for RRP.
• Novel Mechanism: It is an immunotherapy that targets the root cause of the disease, HPV-infected cells.
• Approval Pathway: The product received Orphan Drug and Breakthrough Therapy designations and was approved under Priority Review, reflecting the significant unmet medical need for RRP patients.

Source: FDA Approves First Immunotherapy for Recurrent Respiratory Papillomatosis. FDA. 2025; Published: August 14, 2025.

[Posted 14/May/2025]

AUDIENCE: Ophthalmology, Internal Medicines

KEY FINDINGS: Lower eyelid and choroidal angiomas were associated with glaucoma diagnosis, suggesting a spatial relationship with SWS findings. However, leptomeningeal angiomas were not associated, possibly because these are further from the eye.

BACKGROUND: Objective of the study is to identify which features of Sturge-Weber syndrome (SWS) were most associated with glaucoma onset, severity, and treatment failure at a tertiary care center.

DETAILS: Electronic health records were reviewed for all children with SWS presenting between 2014 and 2020. Examination and imaging findings from dermatology, neurology, and ophthalmology were collected. Logistic regression was used to identify factors associated with glaucoma-related outcomes. Primary outcomes included glaucoma development, progression to surgery, and treatment failure. Failure was defined as having a final intraocular pressure >21 mmHg, devastating complication, or <=20/200 vision. Twenty-three of 44 SWS patients (52.3%) developed glaucoma, and 6 of 23 patients (26.1%) had both eyes affected. Sixteen of 29 eyes (55.2%) required surgery, and 29.6% overall met our failure criteria (mean follow-up: 5.1 ± 4.3 years). Glaucoma diagnosis was associated with bilateral port-wine birthmarks (PWBs; odds ratio [OR] 5.9; 95% confidence interval [CI] 1.3-43.2), PWB with any lower eyelid involvement (OR 9.7, 95% CI 2.6-44.5), and choroidal hemangiomas (OR 3.8, 95% CI 1.1-13.8), but was not associated with upper eyelid or leptomeningeal angiomas, seizures, prior hemispherectomy, or pulsed-dye laser. Eyes that progressed to surgery were more likely to have PWB affecting the lower eyelid (OR 33.7, 95% CI 4.5-728.0). No clinical or demographic factors were associated with treatment failure. In most cases, angle surgery failed (72.7%) but was a temporizing measure before subconjunctival filtering surgery.

Copyright © The American Academy of Ophthalmology. All rights reserved.

Source: M. Vu, D., Gjerde, H., Elhusseiny, A. M., et al. (20245). Distribution of c and Glaucoma Outcomes in Sturge-Weber Syndrome. Ophthalmology Glaucoma. 2025; 8(2): 181-187. Published: March-April, 2025. DOI: 10.1016/j.ogla.2024.10.007.