Journal of Neuro-Ophthalmology最新文献

Background: Patients with craniosynostosis are at high risk of developing elevated intracranial pressure (ICP) causing papilledema and secondary optic atrophy. Diagnosing and monitoring optic neuropathy is challenging because of multiple causes of vision loss including exposure keratopathy, amblyopia, and cognitive delays that limit examination. Peripapillary hyperreflective ovoid mass-like structures (PHOMS) are an optical coherence tomography (OCT) finding reported in association with papilledema and optic neuropathy. We investigated the prevalence of PHOMS in patients with syndromic craniosynostosis and the relationship with known risk factors of optic neuropathy.

Methods: This was a cross-sectional retrospective study was performed at Boston Children's Hospital and included 118 eyes (60 patients) with syndromic craniosynostosis that had at least one good quality optic nerve OCT volumetry scan between January 2010 and December 2023. Testing was reviewed by 2 neuro-ophthalmologists to determine the presence of PHOMS. Information collected included demographics, and clinical course including possible Chiari malformation, obstructive sleep apnea (OSA), history of elevated ICP, best-corrected visual acuity (VA), spherical equivalent (SE), retinal nerve fiber layer thickness, macular ganglion cell layer volume, and funduscopic examination. Our primary outcome measure was presence of PHOMS, and secondary outcome measure was the relationship between clinical characteristics and the presence of PHOMS.

Results: Mean age at imaged OCT was 9.8 ± 5.2 years and 41/60 (68.3%) patients were female. The prevalence of PHOMS was 17/60 patients (28.3%) or 26/118 eyes (22.0%), higher than previously reported in children without craniosynostosis (P < 0.001). PHOMS were significantly associated with a history of elevated ICP (odds ratio [OR] 14.4, 95% CI: 1.9-111.0, P < 0.001) and with concurrent papilledema (OR 40.4, 95% CI: 6.6-248.0, P < 0.001). OSA, Chiari malformation, best-corrected VA, craniosynostosis variant, and SE were not independently associated with PHOMS.

Conclusions: Eyes in children with craniosynostosis had a higher prevalence of PHOMS than reported in children without craniosynostosis. PHOMS were significantly more common with a history of elevated ICP and with concurrent papilledema. PHOMS may serve as a clinically useful indicator of optic neuropathy, and of recurrence of papilledema in patients with craniosynostosis and in other populations characterized by multiple sources of vision loss and cognitive changes that limit evaluation.

In this issue of JNO, Drs. Deborah I. Friedman and Mark L. Moster discuss the following 4 articles: Al Kasab S, Almallouhi E, Shu L, Kicielinski KP, Salehi Omran S, Liebeskind DS, Zubair AS, Vedovati MC, Paciaroni M, Antonenko K, Heldner MR, de Havenon A, Henninger N, Yaghi S. Outcomes and recurrence rates among patients with provoked and cryptogenic cerebral venous thrombosis: analysis of the ACTION CVT. Neurol Clin Pract. 2025;15:e200381 [Epub ahead of print].Kwon YN, Kim B, Kim JS, Park KS, Seo DY, Kim H, Lee EJ, Lim YM, Ju H, Chung YH, Min JH, Nam TS, Kim S, Sohn E, Shin KJ, Seok JM, Kim S, Bae JS, Lee S, Oh SI, Jung YJ, Park J, Kim SH, Kim KH, Kim HJ, Jung JH, Kim SJ, Kim SW, Jang MJ, Sung JJ, Waters P, Shin HY, Kim SM. Time to treat first acute attack of myelin oligodendrocyte glycoprotein antibody-associated disease. JAMA Neurol. 2024;81:1073-1084.Akosman S, Li R, Asahi M, Kwon B, Dossantos J, Tavakoli M, Chen JJ. Trends in plasma exchange use in optic neuritis hospitalizations in the United States. Ophthalmology. 2024;131:1207-1214.Chou CC, Pan SY, Sheen YJ, Lin JF, Lin CH, Lin HJ, Wang IJ, Weng CH. Association between semaglutide and non-arteritic anterior ischemic optic neuropathy: a multinational population-based real-world study. Ophthalmology. 2024:S0161-6420(24)00685-7 [Epub ahead of print].

Background: In the aftermath of an adverse event, the first priority is to provide care for the patient, known as the first victim. However, the experiences of healthcare professionals (HCPs) involved in these events, known as "second victims", have been largely overlooked. This review aims to consolidate existing knowledge on second victim syndrome (SVS), explore its unique implications for neuro-ophthalmologists, and suggest support strategies to increase awareness and meet the needs of affected colleagues.

Evidence acquisition: A comprehensive review of the literature was conducted using PubMed, analyzing peer-reviewed articles and reports on SVS across medical specialties. The review focused on the prevalence, risk factors, outcomes, and strategies for addressing SVS at both the individual and system-wide levels.

Results: SVS is alarmingly prevalent among HCPs, with estimates ranging from 10% to 43%, and nearly half of all HCPs are expected to experience SVS at least once in their careers. Although direct evidence is lacking, neuro-ophthalmologists may be particularly vulnerable due to the complexity of their patients, frequent diagnostic challenges, and the risk of poor outcomes. Effective coping strategies include peer support at the institutional level, with an urgent need for healthcare systems to transition toward a "Just Culture" that fosters openness and collective learning.

Conclusions: Neuro-ophthalmologists are likely among several specialty fields suffering in silence following medical errors. To reduce these errors and optimize patient care, we must focus on enhancing support for the physicians involved. Sustainable improvements in healthcare require both targeted clinician support and comprehensive system-level changes to foster resilience and improve quality of care. Key efforts should include formal peer support programs, targeted educational initiatives, and a deliberate shift toward a "Just Culture." These initiatives are essential for fostering clinician resilience, promoting effective recovery, and ultimately improving the broader healthcare system and quality of care.

Background: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare and poorly understood inflammatory disorder of the central nervous system centered on the pons. It has a characteristic imaging appearance with enhancing and T2-hyperintense punctate and curvilinear lesions in the pons. The lesions lack restricted diffusion and have relatively little perilesional edema. Although patients typically present with gait ataxia and other sequelae of brainstem inflammation including diplopia, there is scant literature focusing on patients who present with primarily neuro-ophthalmic manifestations.

Methods: Case series of 3 patients presenting with diplopia who had a final diagnosis of CLIPPERS.

Results: Case descriptions of a 71-year-old man, 61-year-old woman, and 38-year-old man are reported. Diplopia was the chief presenting complaint, owing to internuclear ophthalmoplegia, sixth nerve palsy, or skew deviation. All patients had nystagmus and gait ataxia. Brain MRI displayed punctate or curvilinear enhancement of pontine/middle cerebellar peduncle lesions without restricted diffusion. All patients achieved rapid improvement after corticosteroid treatment.

Conclusions: In 3 patients with CLIPPERS, the main presenting complaint was diplopia. The distinctive imaging signs led to a strong presumption of CLIPPERS, permitting a truncated evaluation and early corticosteroid treatment, which provided rapid reversal of clinical and imaging manifestations.

Background: The rarity of optic nerve sheath meningiomas (ONSMs) complicates the guidelines surrounding optimal treatment strategies and prognostic factors. There are limited data on the visual outcomes of those treated with radiotherapy versus those observed without treatment. This study aimed to characterize the clinical and radiographic presentations of patients diagnosed with ONSMs and to identify factors predicting improvement in visual function after treatment.

Methods: This is a retrospective case series of 26 patients who presented to 2 tertiary neuro-ophthalmology practices for 10 years with the presumptive diagnosis of ONSM. Demographic, clinical, investigative, radiologic, treatment, and outcome data were collected. Visual improvement was defined as improvement in visual acuity (VA) by ≥2 Snellen lines or visual field (VF) mean deviation (MD) improvement by ≥2 dB. Statistical analyses were performed to compare patients who experienced improvement in visual function with those who did not poststereotactic radiotherapy to identify pretreatment predictors of visual recovery.

Results: Seventeen patients underwent radiotherapy (16 received 54 Gy in 30 fractions and 1 received 50 Gy in 25 fractions) and 4 elected observation. Five were lost to follow-up. Visual function improvement was seen in 10 patients who underwent radiotherapy. Pretreatment VA (logMAR 0.492 vs 1.42, P = 0.025), tumor size (8.50 vs 18.3 mm, P = 0.028), and a decrease in tumor size postradiotherapy (P = 0.009 for VA and 0.035 for VF MD improvement) were significantly associated with objective visual improvement.

Conclusions: Pretreatment VA and tumor size may predict improvement in visual function in patients with ONSMs treated with radiotherapy. Future larger scale studies that include these data may be able to draw more definite conclusions.