Ophthalmic Testing Research Articles

8110 Lymphocytic Hypophysitis secondary to a ruptured Rathke’s Cleft Cyst complicated by post surgical recurrence: A rare phenomenon

Abstract Disclosure: M.S. Maharaul: None. F. Thelmo: None. I. Ahmed: None. Introduction: Lymphocytic hypophysitis secondary to ruptured Rathke’s cleft cyst (RCC) is a rare entity. Rathke’s cleft cysts are benign fluid-filled intra-sellar cysts that originate from the remnants of Rathke’s pouch and contain mucoid material. Most are asymptomatic; some may get symptomatic during cyst expansion and rupture releasing liquid content and trigger local inflammation. Surgical resection followed by glucocorticoids is usually curative. Case: A 42-year-old female presented to the hospital with one week history of right sided frontal headache associated with blurred vision in right eye. MRI Brain showed a 15 mm complex cystic lesion in the sella with suprasellar extension associated with peripheral calcifications. On admission, endocrine laboratory investigations including pregnancy test were normal. Ophthalmic testing did not reveal any acute abnormalities. Transsphenoidal surgery was performed for tumor resection. Pathology examination revealed infiltration of massive lymphocytes, macrophages, plasma cells, and eosinophils that were diagnostic of lymphocytic hypophysitis in the anterior pituitary lobe. She was started on glucocorticoids and discharged home. Rheumatology and Infectious diseases workup were unrevealing. On follow up 3 weeks later, she had self-discontinued steroids. The following week, she presented to the emergency room again with a right sided headache and blurred vision. MRI Brain revealed a 16 mm recurrent inflammatory mass lesion in the sella. Endocrine panel demonstrated an 8 am serum cortisol level of 7.4 mcg/dl, ACTH 20 pg/ml, FSH 6.2 mIU/ml, LH 4.9 mIU/ml, free T4 1.1 ng/dl and prolactin 8 ng/ml. She underwent transsphenoidal resection of the mass again and was restarted on glucocorticoids. This time pathology examination revealed an epithelial lined cyst most compatible with Rathke’s cleft cyst. She continued to remain on a glucocorticoid taper during the follow up period. On re-evaluation, her endocrinology laboratory investigations continued to show a deficiency of gonadotropins for which she is following up with OBGYN, but the remaining pituitary hormones were all within normal limits. Conclusion: Rathke’s Cleft Cysts (RCCs) should be in the differential diagnoses for evaluation of hypophysitis. There have only been a handful of cases in the literature of hypophysitis associated with a ruptured RCC, and only one case of recurrence after surgical debulking. This case provides an insight into the clinical behavior, diagnosis, and timely management of lymphocytic hypophysitis due to a ruptured Rathke’s cleft cyst and management post-recurrence. Presentation: 6/3/2024

Implementation of anterior segment ophthalmic telemedicine.

The growing push to integrate telemedicine into ophthalmic practices requires physicians to have a thorough understanding of ophthalmic telemedicine's applications, limitations, and recent advances in order to provide well tolerated and appropriate clinical care. This review aims to provide an overview of recent advancements in the use of ophthalmic telemedicine for anterior segment eye examinations. Virtual care for anterior segment evaluation relies on appropriate technology, novel workflows, and appropriate clinical case selection. Recent advances, particularly in the wake of the COVID-19 pandemic, have highlighted the utility of home-based assessments for visual acuity, external evaluation, tonometry, and refraction. Additionally, innovative workflows incorporating office-based testing into virtual care, termed 'hybrid telemedicine', enable high-quality ophthalmic testing to inform clinical decision-making. Novel digital tools and workflows enable high-quality anterior segment evaluation and management for select ophthalmic concerns. This review highlights the clinical tools and workflows necessary to enable anterior segment telehealth.

Ocular manifestations of juvenile Sjögren's disease.

This review aims to enhance understanding of juvenile Sjögren's disease (jSjD) by exploring diagnostic criteria, ocular clinical features, ancillary ophthalmic testing, and management strategies specific to this rare pediatric condition. Unlike adults, children with jSjD often present with recurrent parotitis and extra-glandular symptoms before developing sicca symptoms. Adult SjD classification criteria do not consider pediatric-specific symptoms and physiological differences. Underutilization of diagnostic tests such as the ocular staining score (OSS) and Schirmer I may result in an incomplete understanding of the prevalence of keratoconjunctivitis sicca in jSjD. Timely referral to an ophthalmologist can address perceived feasibility issues with respect to ocular features in jSjD. Management of keratoconjunctivitis sicca in jSjD includes improving ocular surface lubrication and decreasing inflammation. Recognition of pediatric-specific clinical features and development of universally accepted jSjD classification criteria will allow for better identification of potential participants for future jSjD studies.

Big data and electronic health records for glaucoma research.

The digitization of health records through electronic health records (EHRs) has transformed the landscape of ophthalmic research, particularly in the study of glaucoma. EHRs offer a wealth of structured and unstructured data, allowing for comprehensive analyses of patient characteristics, treatment histories, and outcomes. This review comprehensively discusses different EHR data sources, their strengths, limitations, and applicability towards glaucoma research. Institutional EHR repositories provide detailed multimodal clinical data, enabling in-depth investigations into conditions such as glaucoma and facilitating the development of artificial intelligence applications. Multicenter initiatives such as the Sight Outcomes Research Collaborative and the Intelligent Research In Sight registry offer larger, more diverse datasets, enhancing the generalizability of findings and supporting large-scale studies on glaucoma epidemiology, treatment outcomes, and practice patterns. The All of Us Research Program, with a special emphasis on diversity and inclusivity, presents a unique opportunity for glaucoma research by including underrepresented populations and offering comprehensive health data even beyond the EHR. Challenges persist, such as data access restrictions and standardization issues, but may be addressed through continued collaborative efforts between researchers, institutions, and regulatory bodies. Standardized data formats and improved data linkage methods, especially for ophthalmic imaging and testing, would further enhance the utility of EHR datasets for ophthalmic research, ultimately advancing our understanding and treatment of glaucoma and other ocular diseases on a global scale.

Highly efficient capture approach for the identification of diverse inherited retinal disorders

Our study presents a 319-gene panel targeting inherited retinal dystrophy (IRD) genes. Through a multi-center retrospective cohort study, we validated the assay’s effectiveness and clinical utility and characterized the mutation spectrum of Taiwanese IRD patients. Between January 2018 and May 2022, 493 patients in 425 unrelated families, all initially suspected of having IRD without prior genetic diagnoses, underwent detailed ophthalmic and physical examinations (with extra-ocular features recorded) and genetic testing with our customized panel. Disease-causing variants were identified by segregation analysis and clinical interpretation, with validation via Sanger sequencing. We achieved a read depth of >200× for 94.2% of the targeted 1.2 Mb region. 68.5% (291/425) of the probands received molecular diagnoses, with 53.9% (229/425) resolved cases. Retinitis pigmentosa (RP) is the most prevalent initial clinical impression (64.2%), and 90.8% of the cohort have the five most prevalent phenotypes (RP, cone-rod syndrome, Usher’s syndrome, Leber’s congenital amaurosis, Bietti crystalline dystrophy). The most commonly mutated genes of probands that received molecular diagnosis are USH2A (13.7% of the cohort), EYS (11.3%), CYP4V2 (4.8%), ABCA4 (4.5%), RPGR (3.4%), and RP1 (3.1%), collectively accounted for 40.8% of diagnoses. We identify 87 unique unreported variants previously not associated with IRD and refine clinical diagnoses for 21 patients (7.22% of positive cases). We developed a customized gene panel and tested it on the largest Taiwanese cohort, showing that it provides excellent coverage for diverse IRD phenotypes.

The importance of precisely phenotyping patients with high myopia

High myopia can be isolated or a phenotypic feature of a Mendelian gene defect. These should be recognized as they may require a specific patient’ management and counselling. They should also be precisely individualized while conducting research on deciphering the risk haplotypes for the development of idiopathic myopia as they would represent confounding factors. The presentation will review the differential diagnosis for syndromic myopia and suggest a set of minimal ophthalmic testing to establish an appropriate diagnosis.

Ophthalmic Parameters of Healthy Captive Blue-Tongued Skinks (Tiliqua scincoides)

Abstract Normal ophthalmic parameters of the blue-tongued skink (Tiliqua scincoides), one of the largest members of the skink family of lizards, have yet to be evaluated. The objective of this study was to establish baseline ophthalmic parameters for a captive population of 12 healthy, one year old, blue-tongued skinks. Ophthalmic examination, corneal touch threshold, tear production including phenol red thread test (PRTT) and modified Schirmer tear test-2 (mSTT-2), horizontal palpebral fissure length (HPFL), rebound tonometry (TONOVET and TONOVET Plus), and bacterial and fungal culture were performed. The blue-tongued skinks were all amenable to ocular diagnostic testing. Corneal touch threshold (mean ± SD) was 5.48 ± 0.81 cm. PRTT (mean ± SD) was 9.17 ± 6.62 mm/15 seconds. mSTT-2 (mean ± SD) was 2.54 ± 2.72 mm/60 seconds. HPFL (mean ± SD) was 6.96 ± 0.62 mm and was positively correlated with body weight. Intraocular pressure (IOP) assessed using TONOVET (mean ± SD) was 8.43± 1.51 mmHg, while IOP assessed using TONOVET Plus (mean ± SD) was 12.39± 1.54 mmHg. Differences in TONOVET and TONOVET Plus tonometry values were statistically significant (P<0.0001) and TONOVET values positively correlated with body weight. The most common bacterial species identified were Staphylococcus xylosus (10/12) and Staphylococcus sciuri (8/12). Fungal cultures were all negative. One animal had superficial corneal ulceration which resolved with treatment. Ophthalmic examination and testing is readily performed in blue-tongued skinks. These parameters may be useful for ocular assessment of this species in a clinical setting.

Prognosis, Risk Factors, and Clinical Features of Intraocular Recurrence in Primary Vitreoretinal Lymphoma

PurposeTo investigate the clinical features, risk factors, and prognosis of the intraocular recurrence in primary vitreoretinal lymphoma (PVRL). DesignRetrospective case-control study. Participants97 eyes of 51 patients diagnosed with PVRL between 2011/12 and 2021/1 were enrolled in this study. 14 patients among them had experienced intraocular recurrence. MethodsDate on demographic and ophthalmic characteristics, results of diagnostic tests, treatments, prognosis of intraocular recurrence and non-recurrence PVRL patients were collected and compared. Multivariate logistic regression was used to identify independent risk factors. And receiver operating characteristic curve was conducted to determine the cut-off values. Main Outcome MeasuresClinical features and risk factors. Results14 (19 eyes) of 51 PVRL patients had intraocular recurrences, resulting in a recurrence rate of 27.5% over a mean follow-up period of 42.5 months. No difference was observed in central nervous system lymphoma (CNSL) relapse rate (54.3% vs. 64.3%, p=0.523) and median time to CNSL (36.5 95%CI [24.6-48.3] vs. 37.3 95%CI [24.8-49.8], p=0.777) between intraocular non-recurrence and intraocular recurrence groups. Furthermore, there were no statistically significant difference in the survival outcomes, such as mortality (28.6% vs. 29.7%, p=1.000) and median overall survival (70.8 95%CI [54.0-87.7] vs. 59.2 95%CI [44.8-73.6], p=0.297), between these two groups. Younger onset age (odds ratio [OR] 0.90, 95% confidence intervals [CI] 0.84-0.98, p=0.010), isolated PVRL (OR 35.3, 95%CI 2.08-600.0, p=0.014), and no history of intravitreal chemotherapy (OR 7.72, 95%CI 1.37-43.6, p=0.021) were identified as independent risk factors for intraocular recurrences. Of the patients with intraocular recurrence, 23.6% were asymptomatic and were diagnosed during routine follow-up. The rate of interleukin-10 (IL-10) /IL-6>1 was significantly lower than that at diagnosis (43.8% vs. 92.3%, p=0.008). However, the rate of IL-10≥50 pg/mL was high (81.3%) and not significantly different from that at diagnosis (92.3%, p=0.606). ConclusionsThis study did not identify an impact of intraocular recurrence on CNS manifestations or survival outcomes in patients with PVRL. Younger patients have a higher risk of intraocular recurrence, and combined systemic and intravitreal chemotherapy may reduce intraocular recurrence. Regular ophthalmic follow-up and IL-10 testing are recommended to detect intraocular recurrence.

In Vitro and Ex Vivo Models for Assessing Drug Permeation across the Cornea.

Drug permeation across the cornea remains a major challenge due to its unique and complex anatomy and physiology. Static barriers such as the different layers of the cornea, as well as dynamic aspects such as the constant renewal of the tear film and the presence of the mucin layer together with efflux pumps, all present unique challenges for effective ophthalmic drug delivery. To overcome some of the current ophthalmic drug limitations, the identification and testing of novel drug formulations such as liposomes, nanoemulsions, and nanoparticles began to be considered and widely explored. In the early stages of corneal drug development reliable in vitro and ex vivo alternatives, are required, to be in line with the principles of the 3Rs (Replacement, Reduction, and Refinement), with such methods being in addition faster and more ethical alternatives to in vivo studies. The ocular field remains limited to a handful of predictive models for ophthalmic drug permeation. In vitro cell culture models are increasingly used when it comes to transcorneal permeation studies. Ex vivo models using excised animal tissue such as porcine eyes are the model of choice to study corneal permeation and promising advancements have been reported over the years. Interspecies characteristics must be considered in detail when using such models. This review updates the current knowledge about in vitro and ex vivo corneal permeability models and evaluates their advantages and limitations.

Ophthalmic Manifestations of Arterial Tortuosity Syndrome: Case Series of Patient and Carriers

The aim of this study was to report the detailed ophthalmic findings in a young patient with genetically confirmed arterial tortuosity syndrome (ATS) and the findings in 8 family members who were carriers. Nine members of the same Saudi family were assessed at King Khaled Eye Specialist Hospital in Riyadh, Saudi Arabia, for ATS-related ocular changes after homozygosity for the pathogenic variant of SLC2A10 gene was confirmed in 1 member. All family members underwent complete ophthalmic examination, genetic testing, and corneal tomography at presentation and at 6-month follow-up. All ophthalmic features were manifested in our patient with ATS including schisis-like splitting of the stromal layer with greater peripheral thinning, pannus, deep posterior stromal opacities, myopia, high astigmatism, and keratoglobus. The ocular phenotype was also expressed in some carriers ranging from mild myopia to the full spectrum of corneal abnormalities associated with ATS. Our study provided further insights into the phenotype in both patients with ATS and carriers. Annual ophthalmic examination is warranted in both types of patients and must undergo from early life onward to detect progressive ectasia which may necessitate corneal crosslinking.

Demographic and Clinical Characteristics of 600 Children With Nystagmus.

To characterize a large, international cohort of children with nystagmus. Data were collected from a single-center, prospectively developed database on patients with nystagmus. Outcome variables for this study included: (1) demographic characteristics, (2) nystagmus type, (3) clinical characteristics, (4) associated ophthalmic conditions, (5) associated non-ophthalmic conditions, (6) special testing findings, and (7) treatments. Six hundred patients from 38 states and 30 countries were included. Age ranged from birth to younger than 18 years (mean: 15.2 years), 58% were female, 55% were race other than White, 75% had infantile nystagmus syndrome, 17% had neurologically significant nystagmus, 7% had fusion maldevelopment nystagmus syndrome, 64% had strabismus, 56% had an anomalous head posture, 94% had a significant refractive error, 64% had an associated ophthalmic abnormality (excluding ametropia), and 45% had an associated systemic condition. Special testing showed abnormalities in 67% and 95% had treatment directed at their nystagmus. The most common diagnosis in this cohort is neurologically non-significant infantile nystagmus syndrome. Eye movement recordings provide a path toward accurate diagnosis and classification. There was a high prevalence of underlying ocular and/or systemic conditions requiring ophthalmic special testing as part of a diagnostic routine. Clinical treatments are available to most patients. [J Pediatr Ophthalmol Strabismus. 2023;60(6):406-410.].

Longitudinal Use of Telehealth During the COVID-19 Pandemic and Utility of Asynchronous Testing for Subspecialty-Level Ophthalmic Care

Telehealth in ophthalmology has traditionally focused on preventive disease screening with limited use in outpatient evaluation. The unique conditions of the COVID-19 pandemic afforded the opportunity to evaluate different implementations of teleophthalmology at scale, providing insight into expanding teleophthalmology care. To compare telehealth use in ophthalmology with other specialties and assess the feasibility of augmenting ophthalmic telehealth encounters with asynchronous testing during the COVID-19 pandemic. This quality improvement study evaluated retrospective, longitudinal, observational data from the first 18 months of the COVID-19 pandemic (January 1, 2020, through July 31, 2021) for 881 080 patients receiving care from outpatient primary care, cardiology, neurology, gastroenterology, surgery, neurosurgery, urology, orthopedic surgery, otolaryngology, obstetrics/gynecology, and ophthalmology clinics of the University of California, San Francisco. Asynchronous testing was evaluated for teleophthalmology encounters. A hybrid care model wherein ophthalmic testing data were acquired asynchronously and used to augment telehealth encounters. Telehealth as a percentage of total volume of ambulatory care and use of asynchronous testing for ophthalmic conditions. The volume of in-person outpatient visits dropped by 83.3% (39 488 of 47 390) across the evaluated specialties at the onset of shelter-in-place orders for the COVID-19 pandemic, and the initial use of telehealth increased for these specialties before stabilizing over the 18-month study period. In ophthalmology, telehealth use peaked at 488 of 1575 encounters (31.0%) early in the pandemic and returned to mostly in-person visits as COVID-19 restrictions lifted. Elective use of telehealth was highest in gastroenterology, urology, neurology, and neurosurgery and lowest in ophthalmology. Asynchronous testing was combined with 126 teleophthalmology encounters, resulting in change of clinical management for 32 patients (25.4%) and no change for 91 (72.2%). Telehealth increased across various specialties during the COVID-19 pandemic. Combining teleophthalmic visits with asynchronous testing suggested that this approach is feasible for subspecialty-level evaluation. Additional study is needed to evaluate whether asynchronous testing outside the same institution could provide an effective and lasting approach for expanding the reach of ophthalmic telehealth.

Demographic and clinical characteristics of 600 children with nystagmus

To characterize a large, international cohort of children with nystagmus.Data were collected from a single-center, prospectively developed database on patients with nystagmus. Outcome variables for this study included: (1) demographic characteristics, (2) nystagmus type, (3) clinical characteristics, (4) associated ophthalmic conditions, (5) associated non-ophthalmic conditions, (6) special testing findings, and (7) treatments.Six hundred patients from 38 states and 30 countries were included. Age ranged from birth to younger than 18 years (mean: 15.2 years), 58% were female, 55% were race other than White, 75% had infantile nystagmus syndrome, 17% had neurologically significant nystagmus, 7% had fusion maldevelopment nystagmus syndrome, 64% had strabismus, 56% had an anomalous head posture, 94% had a significant refractive error, 64% had an associated ophthalmic abnormality (excluding ametropia), and 45% had an associated systemic condition. Special testing showed abnormalities in 67% and 95% had treatment directed at their nystagmus.The most common diagnosis in this cohort is neurologically non-significant infantile nystagmus syndrome. Eye movement recordings provide a path toward accurate diagnosis and classification. There was a high prevalence of underlying ocular and/or systemic conditions requiring ophthalmic special testing as part of a diagnostic routine. Clinical treatments are available to most patients. [J Pediatr Ophthalmol Strabismus. 20XX;X(X):XX-XX.].

Pediatric Retinal Detachment in Homozygous Protein C Deficiency: Genetic and Phenotypic Description of a Single Family.

Homozygous protein C deficiency is a rare hypercoagulability disorder. This study describes the ocular manifestations and the genetic background in a family with two affected children. This is a retrospective review of ophthalmic examinations, investigations, genetic testing, and blood work-up of two children with homozygous protein C deficiency from a single family. A family with a positive history of consanguineous marriage was found to have two affected children with homozygous protein C deficiency. Abnormal visual behavior was the presenting symptom. Both children had bilateral total tractional retinal detachments at presentation. Skin manifestations included episodes of discoloration and bruising. Laboratory work-up revealed absent protein C activity. Genetic testing confirmed the presence of a homozygous pathogenic mutation in protein C gene (NM_000312.3: c.1297G>A: p.Gly433Ser). Homozygous protein C deficiency should be considered in the differential diagnosis of early-onset tractional retinal detachment in infancy. Although rare, the ophthalmologist may be the first to encounter the condition, and treatment with protein C replacement or anticoagulants may be life-saving. Examination under anesthesia with fluorescein angiography and laser treatment early in life may be warranted to preserve vision. [Ophthalmic Surg Lasers Imaging Retina. 2022;53:293-296.].

Diagnostic Evaluation of Visual Snow.

Introduction: To determine which patients with visual snow (VS) and VS syndrome (VSS) require standard ophthalmologic testing including automated visual field and which patients require further testing such as macular spectral domain optical coherence tomography (SD-OCT), electrophysiology, and neuroimaging.Materials and Methods: We retrospectively reviewed 52 consecutive patients at three institutions with VS and VSS including the University of Alabama, Callahan Eye Hospital, the University of Missouri-Kansas City School of Medicine, and the Little Rock Eye Clinic from the years 2015 to 2021. We collected historical information, examination findings, ophthalmic testing, electrophysiology, and neuroimaging.Results: Of the 52 patients with VS and VSS, eight of the 52 cases met the clinical criteria for VSS. The ages ranged from 7 to 79 years, with a mean age of 25 years (SD = 14.0). There were 22 males and 30 females. Color vision was tested in 51 cases and was normal in 47 cases (92%). A funduscopic exam was performed in all 52 cases and was normal in 46 cases (88%). The macular SD-OCT was normal in all of the 19 cases that it was performed (100%). A Humphrey visual field was performed in 50 cases and was normal in 43 (86%). A visually evoked potential (VEP) was normal in 18 of the 19 cases where it was obtained (95%). The full-field electroretinography (ffERG) was obtained in 28 cases and was normal in 25 (89%). The multifocal electroretinography (mfERG) was normal in 11 of 12 cases (92%). Only four patients accounted for all of the abnormal electrophysiological tests. In the 37 cases that had an MRI, 29 were normal (78%). Only one patient revealed a lesion in the visual pathway (right optic nerve enhancement in an optic neuritis patient).Conclusions: Patients with VS and VSS, if typical in presentation and with normal testing, do not require a workup beyond a thorough history, neuro-ophthalmologic examination, and automated perimetry. If this testing is abnormal, then ancillary testing is required.

Factors Associated With Increased Emergency Department Utilization in Patients With Acute Optic Neuritis.

Symptoms of acute vision loss and eye pain may lead patients with optic neuritis to seek care in the emergency department (ED). Given the availability of lower cost alternatives for providing medical care for optic neuritis, this study aimed to identify factors associated with higher ED utilization. Subjects with acute optic neuritis were identified through a chart review of adults with International Classification of Diseases-9 (ICD-9) or ICD-10 codes for optic neuritis with corresponding gadolinium contrast enhancement of the optic nerve on MRI in the medical record research repository of a tertiary care institution. Subjects were grouped based on the number of ED visits (0-1 and 2-3) within 2 months of either ICD code or MRI. Demographics, characteristics of disease presentation, type and location of medical care, testing (chest imaging, lumbar puncture, optical coherence tomography, spine MRI, visual field, and laboratory tests), treatment, provider specialty of follow-up visits, and duration of care were extracted from the medical record. Of 30 acute optic neuritis subjects (age 41 ± 16 years, range 18-76, 53% [16/30] female), 19 had 0-1 ED visit and 11 had 2-3 ED visits. Most subjects were Caucasian, non-Hispanic (47%), followed by Asian (23%), Hispanic/Latino (17%), Black (10%), and others (3%). Subjects had an initial clinical encounter primarily in the outpatient setting (63%) as compared with the ED (37%). The median time from symptom onset to initial clinical encounter was 4 days with a range of 0-13. Subjects were mostly insured through a private insurance (60%), followed by Medicare/Medicaid (23%) and uninsured (17%). Fewer ED visits were associated with an initial clinical encounter in an outpatient setting (P = 0.02, chi-square), but not residential distance from the hospital or insurance type. Subjects with a higher number of ED visits were more likely to be of Hispanic/Latino ethnicity (P = 0.047, Fisher exact). There was no significant difference in the ophthalmic, radiologic, or laboratory testing performed in both groups. Both groups presented in a similar time frame with similar symptoms and clinical signs. Treatment was similar in both groups. Subjects with their first clinical encounter for optic neuritis in the ED had more visits to the ED overall when compared with those first seen in an outpatient setting and thus strategies aimed at facilitating outpatient care may help reduce unnecessary ED visits, although some, such as insurance status, may be difficult to modify. Further study in a larger sample is needed to refine these observations.

P009 Service evaluation to optimise ophthalmological services for hydroxychloroquine retinal screening

Abstract Background/Aims Hydroxychloroquine (HCQ), a frequently-used therapy in rheumatology, can be associated with retinal toxicity. More stringent screening and monitoring guidelines for HCQ-related retinopathy were published by the Royal College of Ophthalmologists (RCOphth) in 2018. Recommendations include: 1) baseline retinal screening within six-twelve months of commencing HCQ; 2) subsequent annual monitoring for at-risk patients, specifically: concurrent tamoxifen-use, estimated glomerular filtration rate (eGFR) <60ml/min/1.73m2, HCQ dose >5mg/kg/day; 3) annual monitoring for all other patients after five years continuous treatment; 4) patient education on HCQ retinopathy. We quantified the rheumatology HCQ exposure and estimated burden on ophthalmology, to inform the development of HCQ retinal screening services. Methods Cross-sectional data were extracted for patients established on HCQ between 1995-2020 including: weight; eGFR; concomitant retino-toxic medication; concomitant retinal pathology; HCQ dose and duration; documentation of patient education on retinal side-effects. Documentation of relevant ophthalmic testing at baseline (i.e. at time of starting HCQ) were recorded, specifically colour fundus photography, spectral domain optical coherence tomography (SD-OCT) and 10-2 Humphrey visual field (HVF) testing as necessary. Subsequent monitoring with 10-2 HVFs, SD-OCT, autofluorescence (AF) and electrodiagnostic tests (EDTs) as appropriate annually from baseline and from 5 years was recorded. Results 150 patients were included, 84% female, with a mean baseline age of 50.7 years (SD 14.8) and mean weight of 76.4kg (SD 17.6). 63% were on HCQ >5 years (mean duration 7.0 years, SD 5.1). At time of auditing, 50% (75/150) patients had permanently ceased HCQ (62% due to treatment >5 years). 60% patients had documented evidence of education regarding HCQ retinopathy. Of the 150 patients, 39% had baseline risks for retinopathy. 6% had a baseline eGFR <60ml/min/1.73m2. 32% were commenced on HCQ dose greater than 5mg/kg/day. No patients were on concomitant tamoxifen; one patient was on a known retino-toxic drug (quinine). 4% of patients had pre-existing retinal pathology. Of the 75 patients still taking HCQ at time of auditing, 27 (36%) had baseline risk factors warranting yearly retinal screening and 44 (58%) had been on treatment >5years. Most ophthalmology reviews were put on hold until a screening service was established. Of the small number (5.3%) that had baseline ophthalmic screening, 12.5% had colour photography and 75% had SD-OCT. No patients required HVF testing or EDTs. Annual screening revealed 50% of patients had 10-2 HVFs; 75% had SD-OCT; 75% had AF. Only one patient developed maculopathy, not attributed to HCQ. Conclusion Our results demonstrate the need for service development to facilitate adherence to RCOphth guidance. Up to 40% of patients started on HCQ have baseline risks for retinopathy, most due to dosing >5mg/kg/day warranting yearly screening. Our findings will inform development of a rheumatology HCQ retinal screening pathway and services, to ensure safe long-term use of HCQ. Disclosure C. Lee*: None. M. Dey*: None. M. Hng: None. S. Peterson: None. I. Rahiman: None. M. Elshafei: None. C. Estrach: None. N.J. Goodson: None.

Telemedicine for neuro-ophthalmology: challenges and opportunities.

Telemedicine for neuro-ophthalmology (tele-neuro-ophthalmology) has the potential to increase access to neuro-ophthalmic care by improving efficiency and decreasing the need for long-distance travel for patients. Requirements for decreased person-to-person contacts during the COVID-19 pandemic accelerated adoption of tele-neuro-ophthalmology. This review highlights the challenges and opportunities with tele-neuro-ophthalmology. Tele-neuro-ophthalmology programs can be used for triage, diagnostic consultation, and long-term treatment monitoring. Formats include telephone appointments, interprofessional collaborations, remote data interpretation, online asynchronous patient communication, and video visits. Barriers to long-term implementation of tele-neuro-ophthalmology arise from data quality, patient engagement, workflow integration, state and federal regulations, and reimbursement. General neurologists may collaborate with local eye care providers for ophthalmic examination, imaging, and testing to facilitate efficient and effective tele-neuro-ophthalmology consultation. Tele-neuro-ophthalmology has tremendous potential to improve patient access to high-quality cost-effective neuro-ophthalmic care. However, many factors may impact its long-term sustainability.

Retinal Diseases that Can Masquerade as Neurological Causes of Vision Loss.

This review aims to discuss retinal diseases that may masquerade as neurological causes of vision loss and highlights modern ophthalmic ancillary testing that can help to establish these diagnoses. Retinal diseases with signs and symptoms overlapping with neurological causes of vision loss include central serous chorioretinopathy, retinal ischemia, acute macular neuroretinopathy, Acute zonal occult outer retinopathy (AZOOR) complex diseases, paraneoplastic retinopathy, retinal dystrophy, and toxic retinopathy. Diagnosis is facilitated by electrophysiologic studies and multimodal ophthalmic imaging including optical coherence tomography and fundus autofluorescence imaging. Looking into the future, translation of adaptive optics ophthalmoscopy into clinical practice may facilitate early detection of microscopic retinal abnormalities that characterize these conditions. With conventional methods of physical examination, diagnosis of retinal diseases that may masquerade as neurological causes of vision loss can be challenging. Current advance in multimodal ophthalmic imaging along with electrophysiologic studies enhances the provider's ability to make early diagnosis and monitor progression of these conditions.

Ten-year review of a shared care approach in the management of ocular chlamydia trachomatis infections.

Since 2007, the ocular 4:1 multiplex PCR assay in NHS Greater Glasgow and Clyde includes Chlamydia trachomatis (ocular chlamydia (OC)) testing. OC can be identified following routine 'viral' ophthalmic testing, including in asymptomatic patients. A published audit from 2008 identified only 25% of our OC patients attended and completed sexual health management, particularly when ophthalmologists initiated treatment. We subsequently created a shared care network between ophthalmology, virology and sexual health (including a designated sexual health advisor) to address these clinical issues. A 10-year retrospective service review audit from January 2010 to December 2019 was performed to evaluate this approach. A total of 86 patients were identified (49 males (57%), median age 23 years (range 16-77)). Ophthalmologists initiated treatment for 37 patients (43%) prior to onward sexual health referral. Of this group, 5 (13.5%) received sub-optimal treatments, and 15 (40.5%) subsequently failed to attend sexual health services for partner notification. Of the 49 (57%) patients who attended sexual health, 25 (51%) had genital chlamydia co-infection, and 98% received adequate systemic treatment. All were offered full sexual health screening and 46 (93.9%) completed partner notification. This shared care approach more than doubled the proportion of OC patients attending sexual health services over this 10-year period (previously 25%, now 57%). Ophthalmologists could defer treatment to sexual health for more effective OC management; however, challenges remain to address real-world issues of non-attendance, inadequate treatment and incomplete contact tracing. We recommend a multi-disciplinary approach to best manage OC cases identified following ophthalmic testing.