Assessment of Reversibility of Transverse Venous Sinus Stenosis in Patients With Papilledema.

BACKGROUND: Bilateral transverse venous sinus stenosis (TVSS) or stenosis of a dominant venous sinus has been found to be very sensitive radiological findings in patients with intracranial hypertension (IH), yet there is still an ongoing debate whether they constitute reversible or permanent phenomena. Thus, the purpose of this study was to investigate the reversibility of TVSS in patients with IH, including conservatively treated patients with signs of IH as defined by the presence of papilledema. METHODS: This was a retrospective chart review of all patients diagnosed with IH between 2016 and 2022, assessed from 2 tertiary university-affiliated neuro-ophthalmology practices. Inclusion criteria were the presence of papilledema, as quantified by optical coherence tomography, and bilateral TVSS, which is considered typical of IH on neuroimaging. During follow-up, included patients must have had confirmation of papilledema resolution as well as subsequent neuroimaging after conservative treatment or cerebrospinal fluid flow diversion. Patients with dural sinus vein thrombosis or intrinsic stenosis from sinus trabeculations or significant arachnoid granulations were excluded from the study. Either CT venography or MRI/MR venography was reviewed by a fellowship-trained neuroradiologist, and the degree of stenosis was scored through the combined conduit score (CCS), as described by Farb et al. The primary outcome was to assess TVSS changes after resolution or improvement of papilledema. RESULTS: From 435 patients, we identified a subset of 10 who satisfied all inclusion criteria. Our cohort comprised entirely women with a median age of 29.5 years and a median BMI of 32.5 kg/m2. Treatment consisted of acetazolamide in 7 patients, of which 1 had additional topiramate and 2 underwent cerebrospinal fluid flow diversion. Furthermore, 6 patients demonstrated significant weight loss during follow-up. For the primary outcome, 5 of 10 patients exhibited no appreciable TVSS change, and 5 patients demonstrated significant improvement in TVSS, of which 4 received conservative treatment only. Papilledema resolution or improvement was statistically significantly associated with increasing average CCS, TVSS diameter, and grade. CONCLUSIONS: We were able to demonstrate that TVSS can be both irreversible and reversible in patients with resolved papilledema. The finding of TVSS reversibility from conservative treatment alone is novel and has important implications to optimize patient care. Future studies should work to identify factors associated with irreversible TVSS for subsequent targeted intervention and prevention.

Utility of ChatGPT for Automated Creation of Patient Education Handouts: An Application in Neuro-Ophthalmology.

BACKGROUND: Patient education in ophthalmology poses a challenge for physicians because of time and resource limitations. ChatGPT (OpenAI, San Francisco) may assist with automating production of patient handouts on common neuro-ophthalmic diseases. METHODS: We queried ChatGPT-3.5 to generate 51 patient education handouts across 17 conditions. We devised the "Quality of Generated Language Outputs for Patients" (QGLOP) tool to assess handouts on the domains of accuracy/comprehensiveness, bias, currency, and tone, each scored out of 4 for a total of 16. A fellowship-trained neuro-ophthalmologist scored each passage. Handout readability was assessed using the Simple Measure of Gobbledygook (SMOG), which estimates years of education required to understand a text. RESULTS: The QGLOP scores for accuracy, bias, currency, and tone were found to be 2.43, 3, 3.43, and 3.02 respectively. The mean QGLOP score was 11.9 [95% CI 8.98, 14.8] out of 16 points, indicating a performance of 74.4% [95% CI 56.1%, 92.5%]. The mean SMOG across responses as 10.9 [95% CI 9.36, 12.4] years of education. CONCLUSIONS: The mean QGLOP score suggests that a fellowship-trained ophthalmologist may have at-least a moderate level of satisfaction with the write-up quality conferred by ChatGPT. This still requires a final review and editing before dissemination. Comparatively, the rarer 5% of responses collectively on either extreme would require very mild or extensive revision. Also, the mean SMOG score exceeded the accepted upper limits of grade 8 reading level for health-related patient handouts. In its current iteration, ChatGPT should be used as an efficiency tool to generate an initial draft for the neuro-ophthalmologist, who may then refine the accuracy and readability for a lay readership.

The Causes of Optic Disc Edema in Patients Presenting With Significantly Compromised Vision.

BACKGROUND: To evaluate the most common causes of optic disc edema (ODE) in patients with significantly compromised vision (initial best-corrected visual acuity [BCVA] of 20/400 or worse) at presentation. METHODS: Retrospective chart review over a 5-year period of consecutive patients presenting to tertiary neuro-ophthalmology clinics at the University of Toronto. RESULTS: A total of 656 patients with ODE were included, and 49 patients (7.47%) had an initial BCVA of 20/400 or worse. There were 54 eyes included at baseline and 49 eyes at final follow-up. There were 29 female and 20 male patients. The mean age at first visit across patients was 55.9 years. Female patients (n = 29) were significantly older than male patients (n = 20) (P < 0.05). The causes of ODE were optic neuritis (ON) (n = 22; 40.7%), nonarteritic anterior ischemic optic neuropathy (NAION) (n = 22; 40.7%), arteritic anterior ischemic optic neuropathy (AAION) (n = 5; 9.26%), uveitis-related (n = 3; 5.56%), papilledema from idiopathic intracranial hypertension (IIH) (n = 1; 1.85%), and Vogt-Koyanagi-Harada disease (n = 1; 1.85%). Initial BCVA was not significantly different between ON and NAION groups (P = 0.52); however, final BCVA was significantly better in the ON group (P < 0.0001). The mean initial BCVA was worst in the AAION group (2.62 ± 0.54 logarithm of the minimum angle of resolution). The most common cause of ODE in patients <40 years old was ON (83.3%), whereas the 2 most common causes in patients >80 were NAION (60%) and AAION (40%). In patients between the ages of 60-80, NAION (100%) was the only cause. CONCLUSIONS: Patients with ODE and poor vision at presentation represent a minority of cases seen in neuro-ophthalmology clinics (<10%). Optic neuritis and NAION are the 2 most common causes of ODE with poor vision at presentation. These findings are limited by a small sample size and potential sampling bias.

Diagnostic Yield of Investigations in Symmetric Optic Neuropathy.

BACKGROUND: Symmetric optic neuropathy (SON) is commonly seen in neuro-ophthalmic practice and is often discovered incidentally. Although multiple investigations might be performed to discover the underlying cause, they are not always indicated. The aim of this study was to report a clinically reasonable and cost-effective approach to investigating patients with SON. METHODS: SON was defined as bilateral optic neuropathy with normal and/or symmetrically decreased central visual acuity, absence of relative afferent pupillary defect, presence of symmetric optic disc pallor, symmetric thinning of peripapillary retinal nerve fiber layer on optical coherence tomography, and absence of other identifiable causes of optic neuropathy. Records of all patients diagnosed with SON seen at a tertiary university-affiliated neuro-ophthalmology practice from 2016 to 2022 were reviewed to identify the yield of various investigations. Clinical data from the initial and last follow-up visit were obtained. Subgroup analysis was performed to ascertain whether diagnostic yield is higher in patients with severe visual loss (central acuity worse than 20/40) compared with those with mild visual loss (acuity 20/40 or better). RESULTS: One hundred thirty-six patients met inclusion criteria. Testing for OPA1 and OPA2 mutations had the highest diagnostic yield (16.0%), followed by mitochondrial genome sequencing (13.6%), serum vitamin B12 (6.1%), and serum folate (1.6%). MRI brain was performed in 54.4% of patients and had a diagnostic yield of only 5%. Both patients who had abnormal MRI had symptoms of demyelination at presentation. Patients were followed for a mean of 15.0 (SD 21.3) months. The most frequently identified etiologies of SON were Leber hereditary optic neuropathy (8.1%), alcohol/tobacco amblyopia (7.4%), vitamin B12 deficiency (5.9%), and dominant optic atrophy (2.9%). Patients with severe visual impairment were more likely to have a final diagnosis compared with those with milder visual impairment (63.9% vs 12.0%, P < 0.001). CONCLUSIONS: The diagnostic yield of investigating SON in patients with preserved visual function, normal diet, and absence of other neurological symptoms is very low. It is reasonable to observe patients with SON with mild visual impairment, reserving costly investigations for those with the visual acuity worse than 20/40 or progressive course.

Swelling of Atrophic Optic Discs in Idiopathic Intracranial Hypertension.

BACKGROUND: Monitoring patients with idiopathic intracranial hypertension (IIH) and optic atrophy may be difficult as papilledema may not be appreciable on ophthalmoscopy. This retrospective chart review evaluated whether papilledema recurrence can be detected in this population using optical coherence tomography (OCT). METHODS: Serial clinical assessments, ophthalmoscopy, and peripapillary OCT were reviewed in a cohort of patients with IIH and optic atrophy. Atrophy was defined as moderate if average peripapillary retinal nerve fiber layer (pRNFL) thickness was ≤80 µm and severe if average pRNFL thickness was ≤60 µm on at least 2 consecutive high-quality OCT scans. Based on the upper tolerance limit of test-retest variability, mean pRNFL elevation of ≥6 µm with subsequent decrease to baseline thickness was considered papilledema. RESULTS: In a cohort of 165 patients with IIH, 32 eyes of 20 patients and 22 eyes of 12 patients demonstrated moderate and severe optic atrophy, respectively. Over a median follow-up of 198.5 weeks (range, 14.0-428.9), 63.3% (19 of 30) of patients had at least 1 episode of relapse, and 50.0% (15 of 30) had at least 1 episode of papilledema. There was a total of 36 relapse episodes, of which 7 occurred in patients with clinical signs and symptoms but no OCT evidence of relapse, 12 occurred in patients with OCT changes but no clinical signs and symptoms of relapse, and 17 occurred in patients with both clinical and OCT evidence to support relapse. The median percent pRNFL increase in the latter 2 groups was 13.7% (range, 7.5-111.8), and 7 eyes (13.0%) of 5 patients (16.7%) showed thickening greater than 20.0% from baseline. The rate, magnitude, and concordance of pRNFL swelling were similar between moderately vs severely atrophic eyes. CONCLUSIONS: Papilledema recurrence can be detected in atrophic optic discs using OCT. All patients with atrophic IIH should be longitudinally monitored with pRNFL measurement. Concurrence of other relapse-suggestive features should prompt further evaluation.

Maculopathies Referred to Neuro-Ophthalmology Clinic as Optic Neuropathies: A Case Series.

BACKGROUND: The clinical features of maculopathies and optic neuropathies often overlap: Both present with decreased visual acuity and variable loss of color vision; thus, maculopathy can be misdiagnosed as optic neuropathy, leading to patient harm. We aimed to determine what findings and/or tests were most helpful in differentiating between optic neuropathy and maculopathy. METHODS: A retrospective chart review of consecutive patients over 4.5 years who were referred to neuro-ophthalmology clinics with the diagnosis of optic neuropathy but whose final diagnosis was maculopathy. Patient demographics, mode of presentation, clinical profile, complete ophthalmological examination, results of all ancillary testing, and final diagnosis were recorded. RESULTS: A total of 47 patients (27 women) were included. The median age was 55 years (range, 18-85). Most referrals were by ophthalmologists (72.3%) and optometrists (12.8%). The diagnosis of maculopathy was made in 51.1% of patients at the time of first neuro-ophthalmic consultation. Only 6.4% patients (3) had relative afferent pupillary defect. Benign disc anomalies (tilted, myopic, small, or anomalous discs) were present in 34.0%, and 21.3% had pathologic disc changes unrelated or secondary to maculopathy. Macular ocular coherence tomography (OCT) was abnormal in 84.4% (with outer retinal pathology in 42.2% and inner retina pathology in 17.8%). Retinal nerve fiber layer (RNFL) thickness was normal in 82.6% of patients. CONCLUSIONS: Macular OCT is a high-yield test in differentiating between optic neuropathy and maculopathy and should be obtained in patients with suspected optic neuropathies who have normal RNFL thickness. Macular dystrophies, particularly cone dystrophies, unspecified retinal disorders, and macular degeneration were the most common mimics of optic neuropathy. The diagnosis was often present on OCT of the macula. The presence of coexistent benign and pathological disc anomalies may lead to maculopathy being misdiagnosed as optic neuropathy.

Inappropriate Indexing of Case Reports to the "Papilledema" Subject Heading: A Systematic Review.

BACKGROUND: Papilledema must be managed distinctly from other causes of optic disc edema (ODE) due to its basis in raised intracranial pressure (ICP). However, evidence indicates that the term "papilledema" is widely misused across specialties to describe ODE without raised ICP. Sources of this misconception remain undiscerned. Because all physicians consult medical databases, our objective was to evaluate whether nonspecific "papilledema" subject heading definitions misleadingly associate articles on other conditions with papilledema proper. METHODS: Systematic review of case reports, prospectively registered on PROSPERO (CRD42022363651). MEDLINE and Embase were searched to July 2022 for any full-length case report indexed to the "papilledema" subject heading. Studies were graded for incorrect indexing, defined as cases lacking evidence for raised ICP. Nonpapilledema diagnoses were assigned to a predefined set of diseases and pathophysiological mechanisms for subsequent comparison. RESULTS: Incorrect indexing occurred in 40.67% of 949 included reports. Embase-derived studies were misindexed significantly less than MEDLINE-derived studies ( P < 0.01). There was also significant heterogeneity in incorrect indexing among specific diseases ( P = 0.0015) and mechanisms ( P = 0.0003). The most commonly misindexed diseases were uveitis (21.24% of errors), optic neuritis (13.47%), and instances with no mention of ODE (13.99%). The most commonly misindexed mechanisms were inflammation (34.97%), other mechanism (e.g., genetic; 25.91%), and ischemia (20.47%). CONCLUSIONS: Database subject headings, especially from MEDLINE, do not adequately distinguish between true papilledema and other causes of ODE. Inflammatory diseases were most often incorrectly indexed among other diseases and mechanisms. Current "papilledema" subject headings should be revised to reduce the probability of misinformation.

Antivirals vs Antivirals and Steroids for Treatment of Herpes Zoster-Related Ophthalmoplegia: A Case Series and Systematic Review.

BACKGROUND: Up to 1/3 of patients with herpes zoster ophthalmicus (HZO) may develop ophthalmoplegia. Although zoster-related ophthalmoplegia (ZO) is typically treated with antiviral agents, there is controversy regarding the therapeutic role of systemic steroids. METHODS: This was a retrospective case series and case report-based systematic review. For the case series, participants were recruited from tertiary neuro-ophthalmology clinics. Eligible participants were those who developed cranial nerve palsies (CNP) within 1 month of HZO diagnosis. In the systematic review, all adults with ZO in the literature who were treated with antivirals or steroids only, or combination therapy were included. Main outcomes were initial presentation, investigations, neuroimaging, treatment regimen, and final outcomes of ophthalmoplegia. RESULTS: Eleven immunocompetent patients with ZO were included. The most common CNP was CN III (5/11), followed by CN VI (2/11) and CN IV (2/11). One patient had multiple CNPs. All patients were treated with antivirals, and 4 also treated with a short course of oral steroids. At 6-month follow-up, 75% of patients treated with combination therapy and 85.7% treated with antivirals alone had complete recovery of ZO. The systematic review identified 63 studies consisting of 76 cases of ZO. When comparing patients treated with antivirals with those treated with antivirals and steroids, patients on combination therapy had more severe ocular findings, including complete ophthalmoplegia (P < 0.001). Age was the only significant predictor of complete recovery of ophthalmoplegia on multivariable logistic regression (P = 0.037). CONCLUSIONS: The rate of complete recovery in immunocompetent patients with ZO was similar in patients treated with antivirals alone vs those treated with antivirals and oral steroids. The systematic literature review affirmed these findings. However, age may influence recovery of ophthalmoplegia.

Idiopathic Intracranial Hypertension and Socioeconomic Status in the Greater Toronto Area, Canada.

BACKGROUND: Previous studies have identified an association between obesity and socioeconomic variables such as poverty, minority status, and a low level of education. Because obesity is a major risk factor for the development of idiopathic intracranial hypertension (IIH), this study aims to identify and assess relationships between socioeconomic and geographic variables in patients with IIH in Canada. METHODS: A retrospective chart review was performed to identify female patients with IIH presenting to 2 neuro-ophthalmology clinics in Toronto between 2014 and 2022. Consecutive female patients younger than 50 years who did not have IIH were identified as controls. Patient age, body mass index (BMI), and postal code were obtained from electronic medical records. Patient postal codes were then converted to geographic dissemination areas based on the 2016 Canadian census, and data on socioeconomic outcomes were collected from Statistics Canada. RESULTS: Three hundred twenty-two female patients with IIH (mean age: 32.3 ± 10) and 400 female controls (mean age: 33.9 ± 9) were included. The mean BMI was 35.0 ± 8 for patients with IIH and 26.7 ± 7 for control patients ( P < 0.00001). There was a significant difference between dissemination areas resided by patients with IIH and control patients for median income ($34640 vs $36685 CAD, P = 0.02) and rate of postsecondary degree attainment (57.7% vs 60.5%, P = 0.01). There were no significant differences in the percentage of visible minorities, percentage of immigrants, knowledge of official languages, percentage of married individuals, average household size, or unemployment rate. There was a weak but significant inverse relationship between the rate of postsecondary degree attainment in dissemination areas resided by patients with IIH and their BMI ( P = 0.01, R 2 = 0.02). CONCLUSION: Patients with IIH reside in geographic areas with lower average levels of income and education than control patients. Patients with lower levels of education may be at higher risk of elevated BMI and therefore disease incidence and progression.

Idiopathic Intracranial Hypertension: Incidental Discovery Versus Symptomatic Presentation.

BACKGROUND: We aim to compare the clinical characteristics, disease course and visual outcomes between Canadian patients with idiopathic intracranial hypertension (IIH) who were incidentally discovered and those who sought care due to symptoms of IIH. METHODS: Retrospective chart review of consecutive IIH patients conducted at tertiary neuro-ophthalmology clinics. Patients were divided into Group 1 (incidentally discovered disease) and Group 2 (patients seeking medical care due to symptoms of intracranial hypertension). RESULTS: One hundred eighty-six patients were included in the study; of which, 75 (40.3%; Group-1) were incidentally discovered and 111 presented due to symptoms of IIH (Group-2). There were no differences in proportion of females (P = 0.101), age (P = 0.450), body mass index (P = 0.386), MRI findings of empty or partially empty sella (P = 0.41), and sella grade (P = 0.704). Group-1 patients were less likely to have experienced headache (P = 0.001), transient visual obscurations (P < 0.001), and diplopia (P = 0.026) at presentation. Group-1 patients were less likely to receive medical (P < 0.001) and surgical (P = 0.004) treatment. There was no difference in proportion of patients who lost weight between the groups (P = 0.848). At baseline, Group-1 patients exhibited better visual acuity (P = 0.001), Humphrey mean deviation (P < 0.001) and retinal nerve fiber layer thickness (P < 0.001). Group-1 patients continued to have better visual acuity (P = 0.002) and Humphrey mean deviation (P < 0.001) at final follow-up. CONCLUSIONS: A significant portion of IIH patients were incidentally discovered. This group exhibited a favorable prognosis and only a minority of these patients required treatment. The way in which patients enter the medical system may be a valuable way to risk stratify IIH patients.

Neuro-Behçe Disease Causing Nuclear/Fascicular Forth Nerve Palsy.

ABSTRACT: Neuro-Behçe disease (NBD) has a predilection for affecting the parenchyma of the upper brainstem; however, involvement of the fourth nerve nucleus or fascicle by NBD has not been previously described. We report a case of a young man with acute right fourth nerve palsy and history of Behçet disease with an enhancing lesion in the left caudal midbrain corresponding to the left trochlear nerve nucleus/fascicle. This is the first described case of NBD producing nuclear/fascicular fourth nerve palsy. It also demonstrates an important clinicoanatomical correlate of decussation of fourth nerve fibers to the opposite side after exiting the midbrain.

Can Lumbar Puncture Be Safely Deferred in Patients With Mild Presumed Idiopathic Intracranial Hypertension?

BACKGROUND: Lumbar puncture (LP) is considered an essential component of the diagnosis of idiopathic intracranial hypertension (IIH) and ruling out IIH mimics, such as meningeal inflammation and neoplastic disease. Such mimics are unlikely in patients who are systemically well and fit the clinical demographic of IIH. It is important to take into account the risks of performing a LP as patients commonly experience mild adverse effects and infrequently more serious ones including psychological distress. LP can also be difficult to obtain in some health care settings, requiring inpatient admission. We examined the clinical course of a subset of presumed patients with IIH with mild vision loss and papilledema to determine whether LP can be safely deferred in this group. METHODS: This was a retrospective study looking at the clinical characteristics, final visual outcome, and diagnosis of patients with presumed IIH and papilledema determined by clinical examination who did not undergo LP. The inclusion criteria included i) no symptoms suspicious for systemic infectious/neoplastic/inflammatory processes, ii) no secondary causes of raised intracranial pressure seen on magnetic resonance imaging/magnetic resonance venography, iii) optical coherence tomography (OCT)-RNFL thickness ≤300 µm, and iv) automated mean deviation (MD) ≤ -5.00 dB v) at least one follow-up visit. RESULTS: A total of 132 eyes of 68 patients (66 female and 2 male) were included in the study. The mean ± SD age was 31.4 ± 10.2 years, and body mass index was 35.1 ± 6.8 kg/m 2 . Systemic symptoms included headache (n = 47), pulsatile tinnitus (n = 28), transient visual obscurations (n = 10), and diplopia (n = 2). Presenting logarithm of the minimum angle of resolution visual acuity was 0.020 ± 0.090, automated MD was -2.23 ± 1.38 dB, and OCT RNFL thickness was 150.8 ± 48.4 µm. Patients were followed for a mean number of 63.3 ± 78.3 weeks. No additional cause of intracranial hypertension was discovered, and all patients remained systemically well. Two patients were started on acetazolamide, and 31 patients lost at least some weight. There was a significant improvement in the automated MD (-1.73 ± 1.74 dB; P < 0.001) and OCT RNFL thickness (128.1 ± 38.6 µm; P < 0.001) at final follow-up. Seventy-six eyes of 38 patients were considered to have resolved papilledema at the final follow-up. CONCLUSIONS: Some patients with presumed IIH may not be able to undergo LP because of patient factors such as refusal, failed attempts, or the environment in which neuro-ophthalmologists practice. This study suggests that it may be acceptable to defer LP for patients with suspected IIH who are under the care of a neuro-ophthalmologist with experience in diagnosing and managing IIH. These patients should be systemically well, in a typical demographic for IIH patients, have mild optic disc edema, and preserved visual function. Patients should be informed about the controversial nature of this decision.

Junctional Scotoma and Patterns of Visual Field Defects Produced by Lesions Involving the Optic Chiasm.

BACKGROUND: Lesions of the optic chiasm (OC) typically produce bitemporal hemianopia (BTH) on visual field (VF) testing, whereas lesions located at the nasal optic nerve-chiasmal (ON-OC) junction have been proposed to produce junctional scotoma (JXS), a central defect in the ipsilateral eye with temporal field loss in the contralateral eye. In this study, we investigated whether the pattern of VF loss in patients with chiasmal compression predicted the appearance of the causative lesion on neuroimaging and described the clinical presentation of these patients with different types of VF defect. METHODS: Retrospective chart review of patients seen in tertiary neuro-ophthalmology practice over 6 consecutive years with lesions abutting or displacing the OC was performed. Lesion size and location relative to the OC on neuroimaging was determined and correlated with VF defects as well as optical coherence tomography (OCT) of the peripapillary retinal nerve fiber layer and macular ganglion cell complex (GCC). RESULTS: Fifty-three patients were enrolled. VFs demonstrated JXS (n = 18), BTH (n = 14), monocular VF defect (n = 4), and no VF defect (n = 17); 64.7% of cases with normal VFs had radiologic OC compression. Lesion volume was highest in the JXS group, and these patients also had the poorest presenting visual acuity. All patients with JXS showed involvement of the ON-OC junction; however, not all cases showed compression of the OC from the nasal direction (15 of 18), and 17 of 18 also showed compression of one or both prechiasmatic ONs. Compression of the ON-OC junction was also seen in 79% of BTH, 100% of monocular VF defect, and 59% of no VF defect cases. Fifty percent of patients with normal VFs already had thinning of the GCC on OCT. GCC thinning was most pronounced nasally in the BTH group, but diffuse bilateral thinning was found in 38% of cases compared with 60% of JXS. VFs improved in 6 of 6 patients with BTH but only in 5 of 8 JXS cases after treatment. CONCLUSIONS: JXS is more often seen with larger lesions and when there is compression of both the prechiasmatic ON and ON-OC junction. These patients have worse presenting visual acuity and poorer outcomes. Not all patients with radiologic compression had VF defects, although 50% of patients with normal VFs had evidence of compression on the macular GCC analysis, emphasizing the importance of macular OCT in the evaluation of patients with lesions involving the OC.

Binasal Visual Field Defects Due to Sickle Cell Maculopathy.

ABSTRACT: A 26-year-old African American man with sickle cell disease noticed blurry vision in both eyes after a recent complicated hospital admission for sickle cell crisis. Anterior and posterior segment examination of each eye was normal, but visual field testing revealed binasal scotomas. Optical coherence tomography of the macula demonstrated severe thinning of the temporal inner retina, suggesting previous bilateral occlusions of terminal retinal arterioles involving the temporal macula, an uncommon complication of sickle cell disease. This case is a reminder that retinal pathology should always be considered as a potential cause of unexplained visual field defects and highlights the role of macular OCT in evaluation of these patients.