A multi-centre case series of patients with coexistent intracranial hypertension and malignant arterial hypertension.

OBJECTIVE: To describe the clinical characteristics, outcomes, and management of a large cohort of patients with concomitant malignant arterial hypertension and intracranial hypertension. METHODS: Design: Retrospective case series. SUBJECTS: Patients aged ≥ 18 years with bilateral optic disc oedema (ODE), malignant arterial hypertension and intracranial hypertension at five academic institutions. Patient demographics, clinical characteristics, diagnostic studies, and management were collected. RESULTS: Nineteen patients (58% female, 63% Black) were included. Median age was 35 years; body mass index (BMI) was 30 kg/m2. Fourteen (74%) patients had pre-existing hypertension. The most common presenting symptom was blurred vision (89%). Median blood pressure (BP) was 220 mmHg systolic (IQR 199-231.5 mmHg) and 130 mmHg diastolic (IQR 116-136 mmHg) mmHg), and median lumbar puncture opening pressure was 36.5 cmH2O. All patients received treatment for arterial hypertension. Seventeen (89%) patients received medical treatment for raised intracranial pressure, while six (30%) patients underwent a surgical intervention. There was significant improvement in ODE, peripapillary retinal nerve fibre layer thickness, and visual field in the worst eye (p < 0.05). Considering the worst eye, 9 (47%) presented with acuity ≥ 20/25, while 5 (26%) presented with ≤ 20/200. Overall, 7 patients maintained ≥ 20/25 acuity or better, 6 demonstrated improvement, and 5 demonstrated worsening. CONCLUSIONS: Papilloedema and malignant arterial hypertension can occur simultaneously with potentially greater risk for severe visual loss. Clinicians should consider a workup for papilloedema among patients with significantly elevated blood pressure and bilateral optic disc oedema.

Optometric Practice Patterns for Acute Central and Branch Retinal Artery Occlusion.

BACKGROUND: Optometrists are often the first providers to evaluate patients with acute vision loss and are often the first to diagnose a central retinal artery occlusion (CRAO). How quickly these patients present to the optometrist, are diagnosed, and referred for evaluation are major factors influencing the possibility of acute therapeutic intervention. Our aim was to survey the U.S. optometric community to determine current optometric practice patterns for management of CRAO. METHODS: An anonymous seven-question survey was emailed in 2020 to the 5,101 members of the American Academy of Optometry and the 26,502 members of the American Optometric Association. RESULTS: Of 31,603 optometrists who were sent the survey, 1,926 responded (6.1%). Most respondents (1,392/1,919, 72.5%) worked in an optometry-predominant outpatient clinic and were less than 30 minutes from a certified stroke center (1,481/1,923, 77.0%). Ninety-eight percent (1,884/1,922) of respondents had diagnosed less than 5 CRAOs in the previous year, and 1,000/1,922 (52.0%) had not diagnosed a CRAO in the prior year. Of the optometrists who diagnosed at least one CRAO in the previous year, 661/922 (71.7%) evaluated these patients more than 4 hours after the onset of vision loss. Optometrists who diagnosed a CRAO or branch retinal artery occlusion referred patients to an emergency department (ED) affiliated with a certified stroke center (844/1,917, 44.0%), an outpatient ophthalmology clinic (764/1,917, 39.9%), an ED without a stroke center (250/1,917, 13.0%), an outpatient neurology clinic (20/1,917, 1.0%), or other (39/1,917, 2.0%); most (22/39, 56.4%) who responded "other" would refer to a primary care physician. CONCLUSIONS: Optometrists are likely the first providers to evaluate patients with acute vision loss, including from a retinal artery occlusion. However, only 6.1% of optometrists responded to our survey despite 2 reminder emails, likely reflecting the lack of exposure to acute retinal artery occlusions, and a potential lack of interest of optometrists in participating in research. Of the optometrists who reported evaluating a CRAO in the previous year, less than 29% saw the patient within 4 hours of vision loss. In addition, a large portion of optometrists are referring acute CRAO patients to outpatient ophthalmology clinics, delaying appropriate acute management. Therefore, it is imperative that optometrists and ophthalmologists are educated to view acute retinal arterial ischemia as an acute stroke and urgently refer these patients to an ED affiliated with a stroke center. The delay in patient presentation and these referral patterns make future clinical trials for acute CRAO challenging.

Retinal and optic nerve magnetic resonance diffusion-weighted imaging in acute non-arteritic central retinal artery occlusion.

OBJECTIVES: Diffusion weighted imaging hyperintensity (DWI-H) has been described in the retina and optic nerve during acute central retinal artery occlusion (CRAO). We aimed to determine whether DWI-H can be accurately identified on standard brain magnetic resonance imaging (MRI) in non-arteritic CRAO patients at two tertiary academic centers. MATERIALS AND METHODS: Retrospective cross-sectional study that included all consecutive adult patients with confirmed acute non-arteritic CRAO and brain MRI performed within 14 days of CRAO. At each center, two neuroradiologists masked to patient clinical data reviewed each MRI for DWI-H in the retina and optic nerve, first independently then together. Statistical analysis for inter-rater reliability and correlation with clinical data was performed. RESULTS: We included 204 patients [mean age 67.9±14.6 years; 47.5% females; median time from CRAO to MRI 1 day (IQR 1-4.3); 1.5 T in 127/204 (62.3%) and 3.0 T in 77/204 (37.7%)]. Inter-rater reliability varied between centers (κ = 0.27 vs. κ = 0.65) and was better for retinal DWI-H. Miss and error rates significantly differed between neuroradiologists at each center. After consensus review, DWI-H was identified in 87/204 (42.6%) patients [miss rate 117/204 (57.4%) and error rate 11/87 (12.6%)]. Significantly more patients without DWI-H had good visual acuity at follow-up (p = 0.038). CONCLUSIONS: In this real-world case series, differences in agreement and interpretation accuracy among neuroradiologists limited the role of DWI-H in diagnosing acute CRAO on standard MRI. DWI-H was identified in 42.6% of patients and was more accurately detected in the retina than in the optic nerve. Further studies are needed with standardized novel MRI protocols.

Referral Patterns of Central Retinal Artery Occlusion to an Academic Center Affiliated With a Stroke Center.

BACKGROUND: Central retinal artery occlusion (CRAO) is a medical emergency, and patients who present acutely should be immediately referred to the nearest stroke center. We evaluated practice patterns for CRAO management at one academic center over the last decade. METHODS: This was a retrospective study on all adult patients diagnosed with a CRAO seen at one tertiary hospital and outpatient clinic affiliated with a comprehensive stroke center ("our institution") from 2010 to 2020. Our electronic medical records were searched for CRAO diagnoses, and patient medical records were reviewed. The exclusion criteria were incorrect diagnosis, unclear diagnosis, historical CRAO, or satellite clinic location. Demographics, distance and time to presentation to our institution, number and type of prior providers seen, diagnostic tests performed, and treatments provided were collected. Summary statistics of median, mean, and frequency were calculated and reported with measures of variance (interquartile range [IQR], ranges). F, Tukey, and Fisher exact tests were used for comparisons. RESULTS: We included 181 patients with a diagnosis of CRAO (80 [44.2%] women; median age 69 years [range 20-101]). The median distance from patient's home to our institution was 27.8 miles (IQR 15.5-57.4; range 2.4-930). The median time from visual loss to presentation at our institution was 144 hours (IQR 23-442 hours, range 0.5-2,920) from 2010 to 2013, 72 hours (IQR 10.5-372 hours, range 0-13,140) from 2014 to 2016, and 48 hours (IQR 7-180 hours, range 0-8,030) from 2017 to 2020 (P = 0.07). 91/181 (50%) patients presented to an outpatient provider. 73/181 (40%) presented to an emergency department. Eighty-six percent presented within 1 week of visual loss onset, and rates of comprehensive inpatient evaluation for acute CRAO improved from 44% in 2010-2013 to 82% in 2017-2020 (P < 0.01). CONCLUSIONS: Patients with CRAO often present late and only after evaluation by multiple outpatient providers. Improvement has occurred over the past decade, but delays underscore the barriers to performing clinical trials evaluating very acute treatments for CRAO. Educational interventions for healthcare providers and patients are necessary.

Acute Central Retinal Artery Occlusion Seen within 24 Hours at a Tertiary Institution.

OBJECTIVE: Acute central retinal artery occlusion (CRAO) is an emergency with poor visual outcome. Intravenous thrombolysis within 4.5 h of vision loss is safe and may improve vision, but is rarely administered because of frequent delays in presentation. We describe a subgroup of CRAO patients presenting within 24 h of vision loss to a tertiary care center affiliated with a comprehensive stroke center. MATERIALS AND METHODS: Retrospective review of 181 consecutive CRAO patients seen at our institution from 2010 to 2020. RESULTS: Out of 181 CRAO patients, 62 (34%) presented within 24 h of vision loss and tended to live closer to the hospital. These patients were more likely to be admitted to the hospital and receive comprehensive stroke work-up compared to patients who presented after 24 h of vision loss. Patients presenting after 24 h did not necessarily receive prior appropriate work-up at outside institutions. Conservative treatments for CRAO were administered to 20/181 patients, and only 3 patients received intravenous thrombolysis. CONCLUSIONS: Patients with CRAO do not present to the emergency department fast enough and diagnosis of CRAO is often delayed. Despite having a protocol in place, only 3/181 patients received IV thrombolysis, emphasizing the difficulty in administering very acute treatments for CRAO. Public education regarding CRAO is necessary to improve presentation times, management, and visual outcomes. Hospitals need to develop accelerated diagnostic pathway protocols for patients with acute vision loss so that CRAO patients may be diagnosed and be considered for potential acute treatments as quickly as possible.

Medial Rectus Advancement for Secondary Exotropia.

PURPOSE: To determine the preoperative characteristics and surgical results after medial rectus advancement in patients with secondary exotropia. DESIGN: Retrospective, interventional case series. METHODS: Setting: Tertiary Care University Medical Center. PatientPopulation: 221 patients with a diagnosis of secondary exotropia who underwent medial rectus advancement surgery by a single surgeon. OBSERVATION: Preoperative demographics, exodeviation and motility, intraoperative findings, and postoperative results were recorded. MainOutcomeMeasure: Success of surgery, defined as Esotropia <15 prism diopters (pd) at postoperative week 1, or any deviation of <8 pd at postoperative month 2 (POM2). RESULTS: A total of 98 patients underwent unilateral medial rectus advancement (UMRadv), 89 underwent UMRadv with lateral rectus recession (LRc), and 34 underwent bilateral medial rectus advancement (BMRadv). POM2 success rates were 66.7% in UMRadv patients, 62.1% in UMRadv + LRc, and 56% in BMRadv. A total of 117 patients had preoperative adduction deficits, which were significantly associated with the finding of an intraoperative stretched scar (P < .001). Larger preoperative duction deficits were associated with larger stretched scars (P < .001). At POM2, the mean effect of surgery (pd of correction/mm) was 2.3 ± 1.4 pd/mm for UMRadv, 2.5 ± 0.8 pd/mm for UMRadv + LRc, and 2.8 ± 1.1 pd/mm for BMRadv. Patients with a stretched scar had significantly less correction per millimeter (2.2 ± 1.2 pd/mm) compared with those without (2.6 ± 1.2 pd/mm, P < .001). A total of 38.6% of patients experienced exodrift greater than 10 pd. Exodrift was significantly larger in the BMRadv group (P < .005). DISCUSSION: These results provide guidance for surgical correction based on preoperative deviation and ductions. Adduction deficits indicate a stretched scar, which must be treated with resection and advancement of the medial rectus. A larger amount of surgery is needed in patients with a stretched scar. Exodrift is common, and therefore aiming for approximately 10 pd of overcorrection at postoperative week 1 can improve final outcomes. CONCLUSION: Medial rectus advancement results in successful surgical results at POM2 for secondary exotropia.

Variability Within Optic Nerve Optical Coherence Tomography Measurements Distinguishes Papilledema From Pseudopapilledema.

BACKGROUND: To report a linear risk score obtained using clock-hour optical coherence tomography (OCT) data from papilledema and pseudopapilledema nerves that differentiates between the 2 diagnoses with high sensitivity and specificity. METHODS: Patients presenting to a single neuro-ophthalmologist with papilledema or pseudopapilledema were included for a retrospective review. The absolute consecutive difference in OCT retinal nerve fiber layer (RNFL) thickness between adjacent clock hours and the mean magnitude of thickness for clock hours 1-12 were compared between the 2 groups using mixed-effect models adjusting for age and clock hour with a random intercept for subjects and eyes (nested within subject). The area under the curve (AUC) for the receiver operating characteristics curve and a separate calibration curve was used to evaluate potential clinical usage. RESULTS: Forty-four eyes with papilledema and 72 eyes with pseudopapilledema, 36 of whom had optic nerve drusen met criteria. The papilledema group had a higher mean RNFL thickness (papilledema = 163 ± 68 µm, pseudopapilledema = 82 ± 22 µm, P < 0.001). The papilledema groups also had more variability between consecutive clock hours (papilledema = 57 ± 20 µm, pseudopapilledema = 26 ± 11 µm, P < 0.001). A linear combination of each patient's averaged values separated the 2 groups with an AUC of 98.4% (95% CI 95.5%-100%) with an optimized sensitivity of 88.9% and specificity of 95.5% as well as good calibration (mean absolute error = 0.015). CONCLUSIONS: Patients with papilledema have higher intrinsic variability and magnitude within their OCT, and this finding reliably distinguishes them from those with pseudopapilledema.

Quality and Diagnostic Utility of Mydriatic Smartphone Photography: The Smartphone Ophthalmoscopy Reliability Trial.

BACKGROUND AND OBJECTIVE: Establish quality and diagnostic utility of mydriatic smartphone ophthalmoscopy (SO) fundus images compared to fundus camera (FC) images. PATIENTS AND METHODS: In this prospective, cross-sectional study, 94 consecutive patients in an urban eye emergency department underwent SO and FC fundus imaging via one of three study arms: medical student 1 (MS1), medical student 2 (MS2), and ophthalmology resident (OR). Images of 188 eyes were graded for overall quality by two masked reviewers, and observed critical fundus findings were compared to dilated fundus examination documentation. RESULTS: SO images were higher quality in the OR arm than in the MS1 and MS2 arms (P < .017). There were no differences in FC image quality between photographers (all P > .328). In the OR arm, SO images detected 74.3% of critical fundus findings, whereas FC images detected 77.1%. CONCLUSION: SO produces fundus images approaching the quality and diagnostic utility of traditional FC photographs.