Predictive Factors for Biopsy-Negative Giant Cell Arteritis and Alternative Diagnoses in a Neuro-Ophthalmology Context.

Giant cell arteritis is a challenging diagnosis for patients given the high prevalence of negative temporal artery biopsies (TAB). Despite the lack of histopathological evidence of giant cell arteritis in the TAB, patients can still have TAB-negative giant cell arteritis. The purpose of this paper is to analyse the predictors for TAB-negative giant cell arteritis and the alternative diagnosis of biopsy-negative patients without a giant cell arteritis diagnosis. A retrospective electronic database review of all TABs performed at the Royal Victorian Eye and Ear Hospital from February 2015 to May 2020. Logistic regression analysis was performed to determine predictive factors for a diagnosis of TAB-negative giant cell arteritis. In all cases, a clinical diagnosis of TAB-negative giant cell arteritis was determined by a neuro-ophthalmologist. Alternative diagnoses for negative TABs were identified and explored. A total of 368 TABs were analysed with 287 (78%) negative for histopathological evidence of GCA. Twenty-seven (9.4%) patients were diagnosed and treated as TAB-negative giant cell arteritis. The clinical predictors of a TAB-negative giant cell arteritis diagnosis were the presence of jaw claudication (OR 2.77, 95% CI 1.10-6.98) and CRP (OR 1.02, 95% CI 1.00-1.03). Alternative diagnoses included non-specific headache, non-arteritic anterior ischaemic optic neuropathy, retinal vessel occlusions, and ocular nerve palsies. Predictive factors for a diagnosis of TAB-negative giant cell arteritis were jaw claudication and an elevated CRP. Several alternative diagnoses can be considered for patients with a negative TAB in a neuro-ophthalmology context.

Developing a Quality Assurance Framework for Neuro-Ophthalmology Using a Multisite Data Registry.

BACKGROUND: Quality assurance (QA) in neuro-ophthalmology (NOPH) is often lacking. We aimed to assess the quality of referral assessment and time to consult for common neuro-ophthalmological conditions by implementing a quality-assurance registry, NODE (Neuro-ophthalmology Database), in a tertiary neuro-ophthalmology clinic. Australian standardized triage categories, namely, P1 (consult ≤30 days), P2 (consult ≤30-60 days), and P3 (consult ≤60-90 days), were developed and validated for neuro-ophthalmological conditions. METHODS: We collected data from NODE on 676 patients at the Alfred Hospital, Melbourne and developed a consensus on the assignation of NOPH conditions to triage categories using a modified Delphi approach. A panel of 7 experienced neuro-ophthalmologists scored conditions and assignation to triage categories. Consensus was considered when ≥75% of the panel strongly agreed or agreed. We analyzed the mean days from referral to triage and from triage to the initial consultation and compared that with the developed triage category standard. RESULTS: Most patients presenting to the service were female (64%). Common diagnoses were idiopathic intracranial hypertension (IIH) (19%), optic neuropathy (ON) (14%), nonspecific headaches (11%), cranial nerve defects (CND) (8%), and papilledema (7%). Consensus on triage category assignment was reached after 2 rounds of scoring from expert panel members. The mean time from referral to triage was performed in <5 days for all the common diagnosis at the NOPH clinic. The mean days (±SD) from P1 category triage to initial consult for IIH was 15 (±12) days, acute ON 16 (±14) days, CND was 20 (±15) days, and papilledema was 20 (±19) days. The mean days from P2 triage to initial consultant for nonspecific headaches was 22 (±20) days and for EOMD was 48 (±22) days. The mean time (days) from P3 triage to initial consultant for nonocular myasthenia gravis was 38 days (±29) days and for visual snow was 54 (±31) days. CONCLUSIONS: We have established a consensus agreement on triage categories for neuro-ophthalmological conditions, which can be further validated using a larger panel of experts. We established a NOPH registry that will serve as a framework to benchmark quality of care between NOPH services. Data from our NOPH registry demonstrated that most conditions are appropriately triaged and seen.

Thinking Beyond Giant Cell Arteritis in COVID-19 Times.

BACKGROUND: The coronavirus disease 2019 has displayed multi-system manifestations since its first presentation. This article highlights an unusual presentation of COVID-19 that was reviewed by our instituition's otolaryngology and ophthalmology team. METHODS: We present 2 cases of COVID-19 which presented with unilateral otalgia and ipsilateral pulsatile headaches involving the temporal area. They were referred to the otolaryngology team for assessment of otalgia and subsequently referred to the ophthalmology team for possible giant cell arteritis (GCA). Both patients had no jaw claudication, scalp pain, or tenderness. RESULTS: Serology testing showed raised C-reactive protein (CRP) but normal platelets and erythrocyte sedimentation rate. Case 1 was tested for COVID-19 as part of a preoperative workup which returned positive. With a marked similarity in presentation, Case 2 was tested for COVID-19 which also returned positive. CONCLUSIONS: These 2 cases highlight another set of symptoms that COVID-19 patients may present with. In the context of a COVID-19 pandemic, if a patient presents symptoms similar to GCA but with isolated CRP, it should prompt consideration for COVID testing.

The Usefulness of Anti-acetylcholine Receptor Binding Antibody Testing in Diagnosing Ocular Myasthenia Gravis.

BACKGROUND: Anti-acetylcholine receptor antibody (AChR-Abs) testing is a safe and simple ancillary method for confirming the diagnosis of myasthenia gravis. Despite the test's high sensitivity (85%-90%) for generalized myasthenia gravis, AChR-Abs testing has been reported to have a low sensitivity 44%-66% for ocular myasthenia gravis (OMG). The aim of the study is to assess the effectiveness of AChR binding Abs testing for diagnosing OMG by evaluating the test's sensitivity, specificity, positive predictive value, and negative predictive value. METHODS: A retrospective chart review on 114 OMG suspects who presented to the emergency department of a tertiary eye center in Victoria, Australia, was completed. The patients presented with diplopia alone, ptosis alone, or the combination of diplopia and ptosis. All participants were followed up longitudinally in the neuro-ophthalmology outpatient clinics for the average of 2.8 months, where they have received AChR binding testing. The final diagnosis was only given to the patients who either were seropositive for AChR binding Abs and had a high clinical suspicion of OMG, or the patient who was seronegative for AChR binding Abs but was regarded as likely to have OMG clinically and responded to the diagnostic treatments (pyridostigmine bromide and immunosuppressant therapy). RESULTS: The sensitivity of AChR binding Abs testing in diagnosing OMG was higher (80%; 95% confidence interval [CI], 51.91%-95.67%) than previously reported (44%-66%). AChR binding Abs testing also had a high specificity (98.99%; 95% CI, 94.50%-99.97%) and positive predictive value (92.31%; 95% CI, 62.68%-98.85%). CONCLUSION: The study suggests the higher utility of the AChR binding Abs testing in diagnosing OMG due to its high sensitivity, specificity, and positive predictive value.

Parent satisfaction and acceptability of telehealth consultations in pediatric ophthalmology: initial experience during the COVID-19 pandemic.

Telehealth in pediatric ophthalmology has predominantly been utilized and reported in the setting of clinician-to-clinician opinion or store-and-forward of images, particularly in the diagnosis and management of retinopathy of prematurity (ROP). We present our initial experience of using a telehealth model of care to deliver real-time specialist pediatric ophthalmology services during the COVID-19 pandemic. Over a 5-week period, parents were invited to complete an anonymous survey following a telehealth ophthalmology consultation for their child. The survey explored their satisfaction, acceptance, and feedback relating to their experience. With an overall response rate of 49.4%, satisfaction was high (43.8% very satisfied; 38.2% satisfied). Most parents (71.9%) would consider telehealth for future ophthalmology consultations for their child.

Optical coherence tomography in paediatric clinical practice.

Optical coherence tomography is a non-invasive ocular imaging technique that is frequently used in the diagnosis and monitoring of optic nerve or retinal disease. Advances in optical coherence tomography speed and image processing capability allow increased use of the modality in clinical practice, especially in younger children. This review outlines the challenges involved in imaging children, highlights the technological progress, the importance of acquiring normative data and, finally, focuses on the clinical applicability of optical coherence tomography in our paediatric population with various ocular conditions.

Video recording in ophthalmic surgery.

Video recording in ophthalmic surgery has immense potential to drive quality improvement in patient care, ophthalmology training, and research. Not only do surgical videos permit introspective and critical analyses of surgical technique, they also allow for objective assessment, allow for more informative audits, and are an invaluable medium for surgical education. Unfortunately, medical-grade video recording equipment is often costly. Various novel methods of video recording that utilize commercially available products offer adequate alternatives. Certain ethical and legal issues also need to be considered before the commencement of video recording in the operating room to protect both the patient and surgeon. We review the current applications and methods of video recording in ophthalmic surgery described in the literature, as well as the potential ethical and legal issues surrounding video recording.

Discordant retinopathy of prematurity in twin anemia-polycythemia sequence.

We report the case of severe retinopathy of prematurity (ROP) in the donor twin with twin anemia-polycythemia sequence managed with the novel treatment of intrauterine blood transfusions. The fellow polycythemic twin was managed with intrauterine venesection and developed only mild ROP. Thus, despite intrauterine improvement of the twins' hemodynamic conditions, the risk of developing severe ROP remained in the donor twin.

New trends in childhood vision impairment in a developed country.

BACKGROUND: The Education Vision Assessment Clinic (EVAC) is a unique statewide service that reviews school children 4-18 years of age with low vision in Victoria, Australia, to determine their eligibility for educational support. The purpose of this study was to identify causes of vision impairment in students in Victoria using data from the EVAC. METHODS: Data collected from students who attended the EVAC at the Royal Victorian Eye and Ear Hospital, Melbourne, between the years 2003 and 2012 inclusive, were analyzed retrospectively. Participants were identified through the EVAC schedule of medical appointments. Medical records were reviewed for clinical and demographic information, including diagnosis of vision impairment and best-corrected visual acuity and/or visual fields. RESULTS: Of 543 students, 355 (65%) were eligible for educational assistance. Of those, 249 (70%) had best-corrected visual acuity between 20/60 and 20/200 and/or a field of vision of <20° to 10°, and 106 (30%) had a best-corrected visual acuity worse than 20/200 and/or a field of vision of <10°. Common causes of vision impairment were retinal dystrophies (24%), optic nerve pathology (14%), albinism (14%), and infantile motor nystagmus (10%). CONCLUSIONS: Treatable and potentially preventable causes of vision impairment, such as retinopathy of prematurity and cataract, caused <10% of vision impairment cases in Victorian school children. Analysis of demographic trends is essential to supporting efforts to ensure that students with low vision, from any socioeconomic background, receive specialist teaching services.

Ocular features in Alport syndrome: pathogenesis and clinical significance.

Alport syndrome is an inherited disease characterized by progressive renal failure, hearing loss, and ocular abnormalities. Mutations in the COL4A5 (X-linked), or COL4A3 and COL4A4 (autosomal recessive) genes result in absence of the collagen IV α3α4α5 network from the basement membranes of the cornea, lens capsule, and retina and are associated with corneal opacities, anterior lenticonus, fleck retinopathy, and temporal retinal thinning. Typically, these features do not affect vision or, in the case of lenticonus, are correctable. In contrast, the rarer ophthalmic complications of posterior polymorphous corneal dystrophy, giant macular hole, and maculopathy all produce visual loss. Many of the ocular features of Alport syndrome are common, easily recognizable, and thus, helpful diagnostically, and in identifying the likelihood of early-onset renal failure. Lenticonus and central fleck retinopathy strongly suggest the diagnosis of Alport syndrome and are associated with renal failure before the age of 30 years, in males with X-linked disease. Sometimes, ophthalmic features suggest the mode of inheritance. A peripheral retinopathy in the mother of a male with hematuria suggests X-linked inheritance, and central retinopathy or lenticonus in a female means that recessive disease is likely. Ocular examination, retinal photography, and optical coherence tomography are widely available, safe, fast, inexpensive, and acceptable to patients. Ocular examination is particularly helpful in the diagnosis of Alport syndrome when genetic testing is not readily available or the results are inconclusive. It also detects complications, such as macular hole, for which new treatments are emerging.