Multi-center validation of Catquest-9SF visual function questionnaire in Ontario, Canada.

PURPOSE: To investigate the psychometric performance and responsiveness of Catquest-9SF, a patient-reported questionnaire developed to evaluate visual function as related to daily tasks, in patients referred for cataract surgery in Ontario, Canada. METHODS: This is a pooled analysis on prospective data collected for previous projects. Subjects were recruited from three tertiary care centers in Peel region, Hamilton, and Toronto, Ontario, Canada. Catquest-9SF was administered pre-operative and post-operatively to patients with cataract. Psychometric properties, including category threshold order, infit/outfit, precision, unidimensionality, targeting, and differential item functioning were tested using Rasch analysis with Winsteps software (v.4.4.4) for Catquest-9SF. Responsiveness of questionnaire scores to cataract surgery was assessed. RESULTS: 934 patients (mean age = 71.6, 492[52.7%] female) completed the pre- and post-operative Catquest-9SF questionnaire. Catquest-9SF had ordered response thresholds, adequate precision (person separation index = 2.01, person reliability = 0.80), and confirmed unidimensionality. The infit range was 0.75-1.29 and the outfit range was 0.74-1.51, with one item ('satisfaction with vision') misfitting (outfit value = 1.51). There was mistargeting of -1.07 in pre-operative scores and mistargeting of -2.43 in both pre- and post-operative scores, meaning that tasks were relatively easy for respondent ability. There was no adverse differential item functioning. There was a mean 1.47 logit improvement in Catquest-9SF scores after cataract surgery (p<0.001). CONCLUSION: Catquest-9SF is a psychometrically robust questionnaire for assessment of visual function in patients with cataract in Ontario, Canada. It is also responsive to clinical improvement after cataract surgery.

A prospective study of disease modifying therapy and retinal atrophy in relapsing-remitting multiple sclerosis.

BACKGROUND: To compare the rate of retinal atrophy over time in patients with relapsing-remitting multiple sclerosis (RRMS) treated with various disease-modifying therapies (DMT). METHODS: Patients with RRMS on various DMT and those observed without treatment were prospectively enrolled into the study between September 2015 and June 2018. All subjects with follow-up of 1-4 years were included and categorized into groups as "no drug", "low efficacy drug", "high efficacy drug", or "dimethyl fumarate" (DMF), based on treatment modality used for the longest duration of their follow-up. Ocular coherence tomography (OCT) was used to measure peripapillary retinal nerve fiber layer thickness (RNFL) and ganglion cell/inner plexiform layer (GC-IPL) thickness at baseline and every 6 months. A linear mixed effects regression model was performed to compare rates of retinal atrophy across treatment groups. RESULTS: Out of 67 participants who met inclusion criteria (mean age = 37; 76% female), 13 were untreated, 12 on low efficacy therapy, 18 on DMF, and 24 on high efficacy therapy. History of optic neuritis was associated with lower baseline GC-IPL thickness (p = 0.003). Higher baseline GC-IPL thickness was associated with increased rate of GC-IPL thinning (p = 0.009). Age, disease duration, and ethnicity were not predictors of baseline RNFL or GC-IPL thickness, or rate of atrophy of these layers. CONCLUSIONS: There were no differences in rate of GC-IPL atrophy between patients with RRMS on different treatments in this cohort. Age, disease duration, and ethnicity also did not predict retinal atrophy. History of ON was associated with reduced GC-IPL thickness at baseline, consistent with previous research. Rate of GC-IPL thinning was higher for subjects with higher baseline GC-IPL thickness, suggesting a plateau effect.

Obstructive Sleep Apnea in Men With Idiopathic Intracranial Hypertension: A Prospective Case-Control Study.

BACKGROUND: Idiopathic intracranial hypertension (IIH) is uncommon in men. Previous studies reported on high frequency of obstructive sleep apnea (OSA) in men with IIH, but the pathophysiology of this association remains unclear. One possible culprit for increased intracranial pressure in patients with OSA is hypercapnia. The purpose of this study was to compare the rate of hypercapnia during polysomnography (PSG) study in men with and without IIH and to report on the rate and severity of OSA in men with IIH compared with control subjects of similar age and body mass index (BMI). METHODS: Prospective case-control study of male patients diagnosed with IIH underwent PSG with continuous oxygen and carbon dioxide monitoring overnight. Healthy control subjects with similar age and BMI also underwent PSG. The incidence of OSA diagnosis, rate of hypercapnia and hypoxia, and apnea hypopnea index (AHI) were compared between 2 groups. RESULTS: Eleven subjects with IIH and 10 controls underwent PSG. Both groups were similar regarding age and BMI on the Mann-Whitney U test ( P = 0.072 for age, P = 0.251 for BMI). Subjects for whom carbon dioxide data were not available for more than 50% of total sleep time were excluded from hypercapnia analysis. The mean age was 41.9 years, and the mean BMI was 33.8 kg/m 2 in subjects and controls. OSA was diagnosed in 9 of 11 men with IIH and 4 of 10 controls. There was no statistically significant difference in the rate of hypercapnia and hypoxia between 2 groups for whom the data were available. All patients with BMI over 30 kg/m 2 (7 of 7) and 50% (2 of 4) controls with BMI over 30 kg/m 2 were diagnosed with OSA compared with 50% (2 of 4) of cases and 33% (2 of 6) of controls with BMI less than 30 kg/m 2 . BMI was a significant predictor of total AHI ( P = 0.042) and OSA severity ( P = 0.023), but IIH diagnosis was not ( P > 0.05). CONCLUSIONS: There was no difference in hypercapnia rate between men with IIH and controls; thus, hypercapnia is an unlikely causative factor in pathophysiology of IIH. OSA on PSG was almost 2 times as prevalent in patients with IIH compared with controls; however, BMI was the strongest predictor of OSA diagnosis, and most patients (9 of 11) with BMI over 30 kg/m 2 had OSA on PSG. In men with BMI less than 30, the rate of OSA on PSG study was higher in men with IIH. Based on these data, we recommend that all men with the diagnosis of IIH should undergo PSG study.

Validation of a novel patient satisfaction questionnaire for preoperative fasting in cataract surgery: a discrimination analysis.

OBJECTIVE: Preoperative fasting is routinely performed to prevent anaesthesia-related pulmonary aspiration. To capture patients' experiences with preoperative fasting, a 13-item questionnaire was developed and validated using Rasch analysis and shortened to 6 items. This extension study aims to assess this questionnaire's ability to discriminate between participants with a short versus long duration of fasting and early versus late day surgery. DESIGN: Single-centred cross-sectional study. PARTICIPANTS: Subjects were recruited via consecutive sampling of cataract patients on surgery day at Kensington Eye Institute in Toronto from February to December 2019. METHODS: A validated preoperative fasting questionnaire was administered. Discriminative ability was assessed by comparing responses in patients scheduled for surgery in the morning (8:00 am-12:00 pm) versus afternoon (12:00 pm-3:30 pm) and fasting for short (≤8 hours) versus long (>8 hours) duration. Diagnostic ability of the 6-item questionnaire relative to the 13-item questionnaire was assessed with receiver operating characteristics curve analysis. RESULTS: A total of 164 patients (mean age 70.8 ± 10.0 years; 57% female) were included. Total scores of patients having surgery in the morning were greater (i.e., less fasting-related burden) than in the afternoon (p = 0.04). There was no significant difference in scores between patients fasting for a short versus long duration (p > 0.05). Receiver operating characteristics curve analysis showed excellent diagnostic ability of the 6-item questionnaire relative to the 13-item version (area under the curve = 0.964). CONCLUSION: The 6-item questionnaire for fasting-related burden has excellent discriminative ability between early versus late surgery patients. The time fasting while awake may be a more relevant predictor of fasting-related burden relative to the total duration of fasting.

CALRETICULIN MUTATION ASSOCIATED WITH BILATERAL CENTRAL RETINAL VEIN OCCLUSION IN A YOUNG WOMAN.

BACKGROUND: Bilateral central retinal vein occlusion (CRVO) is rare and is usually associated with an underlying systemic illness such as hypercoagulable state or systemic inflammatory disease. We present a case of bilateral CRVO in a young patent who was found to have a mutation in the calreticulin gene, which was presumed to be the culprit. METHODS: Case report. RESULTS: We report a 24-year-old woman with bilateral CRVO. Hypercoagulability work-up was positive for in-frame deletion in exon 9 of the calreticulin gene. CONCLUSION: We suggest that all young patients presenting with CRVO or any patient with bilateral CRVO have genetic testing for a limited set of known, prothrombotic mutations including the recently identified calreticulin gene.

Neuro-ophthalmological manifestations of Wolfram syndrome: Case series and review of the literature.

Wolfram Syndrome (WS) is a rare progressive hereditary neurodegenerative disease with hallmark features of diabetes mellitus, optic atrophy, and hearing loss. Its other clinical manifestations may include diabetes insipidus, urological, neurological, and psychiatric abnormalities. We review systemic and ocular manifestations of WS as well as its pathophysiology, diagnostic approach, and treatment options. We then describe a case series of 5 patients (ages 15-38, 60% male) with WS. All had significant progressive visual loss. 3/5 patients had type 1 DM and 4/5 had hearing loss. Other neuro-ophthalmological findings included convergence impairment and end-gaze nystagmus. This case series highlights the variability in clinical presentations of patients with WS, reminding clinicians to maintain high suspicion for this diagnosis in order to allow for prompt diagnosis and genetic counselling for patients and their families.

New-onset Oscillopsia in a Patient With a History of Pontine Stroke.

ABSTRACT: A 69-year-old man with a history of pontine hemorrhage 2 years ago noticed binocular vertical diplopia after the stroke. On examination, there was a small-angle incomitant left hyperdeviation that did not fit the 3-step test for fourth nerve palsy and incyclotorsion of the higher eye. On motility testing, there was an obvious pendular nystagmus. Resting tremor of the right hand was noticed on neurological examination. Examination of the oropharynx revealed rhythmic oscillations of the soft palate synchronous with the eye oscillations and hand tremor. These findings established a diagnosis of oculopalatal myoclonus (OPM). Although OPM is a well-described entity, this case is unique because the patient was completely asymptomatic from OPM and did not complain of oscillopsia but was very bothered by vertical diplopia because of skew deviation. It also demonstrates that OPM may coexist with skew deviation because anatomically vestibulo-ocular pathway is close to the triangle of Guillain-Mollaret and patients with lesions in one pathway should be examined for abnormalities in the other. Finally, it reminds us about the importance of monitoring patients with a history of brainstem insults for emergence of synchronous tremors years later and that simple maneuver-like ex/amining oropharynx may provide a clear diagnosis.

Optic Disc Edema in Syphilis.

BACKGROUND: Syphilis is an uncommon cause of optic nerve head edema; however, differentiating syphilis from other etiologies of optic nerve head swelling may be challenging. We describe 4 cases of ocular syphilis presenting with swollen optic nerve head(s) without overt signs of intraocular inflammation to better define the phenotypic presentation of this condition to allow its early recognition and treatment and discuss potential pathophysiological mechanisms of syphilitic optic neuropathy. METHODS: Retrospective case series of patients presenting to a tertiary neuro-ophthalmology practice with a swollen optic nerve head(s) but no overt signs of intraocular inflammation, which was eventually determined to be secondary to syphilis. RESULTS: Four patients were included in the study. The mean age was 43 years, 2 were women and 2 had bilateral involvement. Two patients had a recent history of skin rash, and one patient was investigated for abdominal pain and elevated liver enzymes. Two patients presented with photopsias and preserved visual function, whereas 2 presented with vision loss. Although chorioretinitis was present in all cases, it was very subtle in all and was only appreciated on fundus autofluorescence (FA) in 3 of 4 cases. Three patients demonstrated evidence of optic perineuritis on neuro-imaging. All patients were treated with a course of intravenous penicillin with a variable degree of visual recovery. CONCLUSIONS: Systemic symptoms are common in patients with syphilic optic neuropathy. Optic disc edema as a manifestation of syphilis is usually accompanied by subtle chorioretinitis, which is best appreciated on FA. Optic perineuritis is common in patients with syphilitic optic neuropathy, with its pathophysiology likely similar to meningitis seen in neurosyphilis.

Dural venous sinus stenting in the treatment of idiopathic intracranial hypertension: A systematic review and critique of literature.

Idiopathic intracranial hypertension (IIH) is increased intracranial pressure without a known cause. Dural venous sinus stenting (DVSS) is a relatively new intervention for treatment of IIH refractory to medical therapy and lifestyle modifications. In this review, we outline various hypotheses of IIH pathogenesis and describe the role of venous sinus stenosis and the technical details of DVSS. We also present a summary and critique of the available evidence describing the outcomes of DVSS in IIH and review the evidence-based guidelines for this procedure. We conclude that, although many studies have shown generally favorable outcomes of DVSS in patients with IIH, most have serious limitations, the most common one being paucity of pre- and postprocedure ophthalmological data. Thus, there is not enough available evidence to conclude whether DVSS is an effective procedure for treatment of IIH. We also present the most commonly used indications for DVSS as described in the literature and stress the importance of neuro-ophthalmological assessment before and after the procedure to monitor response and potential complications.

Older woman with abdominal pain and bilateral sequential vision loss due to syphilis.

A 62-year-old woman presented with non-specific abdominal pain, elevated alkaline phosphatase levels and bilateral sequential visual loss. Visual acuity was counting fingers in right eye (RE) and 20/400 in left eye (LE). She was noted to have optic nerve pallor in RE and mildly elevated optic disc with signs of ocular inflammation in LE. After 2 weeks, vision deteriorated to light perception bilaterally with now extensive vitreal inflammation present in both eyes. Positive rapid plasma reagen and Treponema pallidum's antibody tites confirmed syphilis infection. Unfortunately, as the diagnosis was delayed by many months, her visual acuity remained poor (hand motions in RE and 20/50 in LE) despite treatment with intravenous penicillin. This case reminds us of the re-emergence of this 'great masquerader' and highlights the importance of maintaining high suspicion for syphilis in patients with unexplained visual loss and systemic symptoms, even in older adults without identifiable risk factors.

Magnetic resonance or computed tomography venography in the evaluation of young overweight women with papilledema.

BACKGROUND/OBJECTIVES: To compare the yield of magnetic resonance venography (MRV)/computed tomography venography (CTV) in a group of overweight women with incidentally-discovered papilledema and symptomatic intracranial hypertension (IC-HTN) patients without risk factors for dural venous sinus thrombosis (DVST). DESIGN: Retrospective cohort study. METHODS: Retrospective cohort study of female patients between ages 16 and 50 with papilledema, body mass index >25 kg/m2 and MRV/CTV imaging. Patients were excluded if they had risk factors for DVST. The incidence of DVST and clinical features were retrieved. Patients were divided into those with incidentally-discovered papilledema (Group-1) and those who presented due to symptoms of IC-HTN (Group-2). RESULTS: One hundred three patients (45 Group-1, 58 Group-2) were included in the study. Group-2 patients were more likely to have pulsatile tinnitus (p = 0.017), transient visual obscurations, and showed a trend towards increased headache (p = 0.058). Group-2 was also more likely to have been treated with acetazolamide (p < 0.01) and undergo lumbar puncture (p = 0.02). DVST was initially reported in four patients (one in Group-1, three in Group-2), but after further review, two cases (one from Group-1, one from Group-2) were found to be false positives. The final two DVST diagnoses were in Group-2 and presented with significant neurological symptoms. CONCLUSIONS: DVST was not found among a group of 45 young, overweight women with incidentally-discovered papilledema and without DVST risk factors. We therefore believe it is reasonable practice to perform MRI alone, without dedicated MRV/CTV, in the investigation of young, overweight women with incidentally-discovered papilledema without risk factors for DVST.

Transient Monocular Visual Loss: When Is It an Emergency?

BACKGROUND: Patients who experienced transient monocular vision loss (TMVL) commonly present to the emergency department for evaluation. Although multiple etiologies can cause TMVL, it is most important to identify patients with retinal ischemia and those with vasculitis (giant cell arteritis) as the cause of TMVL. Patients with transient retinal ischemia have the same risk of cardiovascular events and death as patients who experienced transient brain ischemia. Patients with giant cell arteritis are at imminent risk of visual loss. CASE REPORT: A 65-year-old man noticed three separate episodes of sudden onset of blurry vision in one eye. Ophthalmologic examination was normal but, as his symptoms were compatible with transient retinal ischemic attack, urgent investigations were initiated. He had normal inflammatory markers but computed tomography angiogram of the brain and neck demonstrated a large plaque in the ipsilateral internal carotid artery. Double anti-platelet therapy was started and stenting of the involved carotid artery was performed. The patient was symptom-free at the last follow-up. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Patients with retinal ischemia as the etiology of TMVL are at high risk of cardiovascular events and death. Their risk of cerebrovascular accidents is highest within 48 h from the episode of TMVL, thus they should have an urgent ophthalmologic examination and, if it is unrevealing, inflammatory markers should be checked and an urgent stroke prevention protocol should be initiated. Appropriate management with medical or surgical interventions significantly reduces morbidity and mortality in these patients.

Development and Validation of a Questionnaire Assessing Patient Distress from Preoperative Fasting in Cataract Surgery.

Purpose: To develop and psychometrically validate a questionnaire to measure patient distress with preoperative fasting related to cataract surgery.Methods: In this single-centered cross-sectional study, consecutive sampling of cataract patients was undertaken immediately preoperatively from February to December 2019. A questionnaire evaluating patient distress with fasting was designed and administered. Questionnaire development occurred in an iterative process and was conducted with consultation from expert investigators and patients. Validation and psychometric evaluation of the questionnaire were performed with Rasch analysis.Results: A preliminary version of the questionnaire was developed by 10 study investigators. Across five iterations of development, the questionnaire was administered to 186 cataract patients. Psychometric evaluation of the 13-item questionnaire demonstrated ordered thresholds, acceptable item calibration and fit, adequate internal consistency, ability to discriminate between three levels of distress from preoperative fasting and no notable differential item functioning. However, issues with mistargeting, clustering of items on the person-item map and multidimensionality remained. Given these concerns, 13 separate re-analyses were conducted via removal of certain items. A 6-item subset was determined to be well targeted, unidimensional, did not display item clustering and was able to discriminate between patients with high and low distress from preoperative fasting.Conclusion: A 6-item questionnaire is a valid, psychometrically robust and reliable measure for the assessment of patient distress with preoperative fasting in cataract surgery. Items include hunger, thirst, hoarseness, weakness, anxiety and nausea. Future studies should seek to validate this questionnaire across a variety of sociodemographic contexts, languages and specialties.

Catquest-9SF questionnaire and eCAPS: Validation in a Canadian population.

BACKGROUND: Visual acuity alone has limitations in assessing a patient's appropriateness and prioritization for cataract surgery. Several tools, including the Catquest-9SF questionnaire and the electronic cataract appropriateness and priority system (eCAPS) have been developed to evaluate patients-reported visual function as related to day-to-day tasks. The aim of this study was to validate Catquest-9SF and eCAPS in a Canadian population and propose a shorter version of each, in an attempt to extend their applicability in clinical practice. METHODS: The English translation of the Swedish Catquest-9SF and eCAPS were self-administered separately in pre-operative patients in tertiary care in Peel region, Ontario. Rasch analysis was used to validate both scales and assess their psychometric properties, such as category threshold order, item fit, unidimensionality, precision, targeting, and differential item functioning. RESULTS: A total of 313 cataract patients (mean age = 69.1, 56.5% female) completed the Catquest-9SF and eCAPS. Catquest-9SF had ordered response thresholds, adequate precision (person separation index = 2.09, person reliability = 0.81), unidimensionality and no misfits (infit range 0.75-1.35, outfit range 0.83-1.36). There mean for patients was equal to -1.43 (lower than the mean for items which is set automatically at zero), meaning that tasks were relatively easy for respondent ability. eCAPS had 3 items that misfit the Rasch model and were excluded (infit range 0.82-1.30, outfit range 0.75-1.36). Precision was inadequate (person separation index = 0.19, person reliability = 0.04). 78.8% of subjects scored≤9 (answered that they had no issues for most questions). CONCLUSIONS: Catquest-9SF demonstrated good psychometric properties and is suitable for assessing visual function of care-seeking patients referred for cataract surgery in Ontario, Canada. There was some mistargeting, suggesting that the tasks were relatively easy to perform, which is consistent with previous research. On the contrary, eCAPS is not sensitive in differentiating patients who had impaired visual functioning.

Patients' complaints involving ophthalmologists in the province of Ontario, Canada: a 5-year review.

OBJECTIVE: To present an overview of complaints against ophthalmologists to the regulatory body in the province of Ontario, Canada, during a 5-year period. DESIGN: Retrospective cross-sectional study. METHODS: All completed complaints to the College of Physicians and Surgeons of Ontario (CPSO) involving ophthalmologists from January 2013 to May 2018 were reviewed. Data regarding the prevalence of complaints, physician characteristics, practice location, reason of complaint, and outcomes as decided by the Inquiries, Complaints and Reports Committee (ICRC) were collected. Identified concerns were classified across 3 domains: clinical care and treatment, professionalism and conduct, and practice management. RESULTS: There were 372 complaints involving 211 ophthalmologists out of 448 practicing ophthalmologists in Ontario. A total of 933 issues were raised in the 372 complaints. Complaints related to clinical care and treatment were most common (76.3%), followed by professionalism and conduct (55.4%) and practice management (24.7%). Within these domains, the 5 largest subcategories in order of occurrence were communication, billing practices, consent, procedural mishap, and documentation. Of the 372 investigations, the ICRC took some form of action in 117 cases (31.4%). The most common actionable decisions issued by the ICRC were advice (19.1%), caution (6.2%), and participation in a specified continuing educational or remediation program (3.5%). Four cases (1.1%) were referred to the Discipline Committee. CONCLUSIONS: Almost half of practicing ophthalmologists in Ontario (47%) received at least one formal CPSO complaint within the 5-year study period. Communication was the most common issue raised in complaints.

Validation and application of Catquest-9SF in various populations: A systematic review.

Cataract is a common cause of reversible blindness. Visual acuity alone is not enough to assess appropriateness for surgery, prioritization, and outcomes. Catquest-9SF questionnaire evaluates patients' self-assessed visual function as related to daily tasks. We summarize and assess the validity of Catquest-9SF as a patient-reported outcome questionnaire to determine its suitability for clinical use. Thirteen studies with sample sizes ranging 102-10,886 (total n = 15,289) undertaken from 2009 to 2018 were included. Catquest-9SF showed unidimensionality, ordered response thresholds, and acceptable precision in all studies. Nine studies had significant mistargeting (range 0.66 to -2.04); the tasks being easy relative to the respondent ability in most studies. Two studies had misfitting items. We conclude Catquest-9SF is a valid and reliable tool to measure visual function in patients with cataract in various populations. Because results can be population specific, it is recommended that Catquest-9SF be validated in a new population before it is incorporated in routine practice.