Differentiation of compressive from glaucomatous optic neuropathy with spectral-domain optical coherence tomography.

PURPOSE: To compare optic disc topography in eyes with compressive optic neuropathy (CON) and open-angle glaucoma (OAG) using spectral-domain (SD) optical coherence tomography (OCT) and Heidelberg retinal tomograph (HRT) (Heidelberg Engineering GmbH, Heidelberg, Germany). DESIGN: Cross-sectional, observational study. PARTICIPANTS: A total of 200 eyes from 123 patients with CON (69 eyes) or OAG (58 eyes) and controls (73 eyes). METHODS: Univariate and multivariate analyses of HRT parameters, SD-OCT circumpapillary retinal nerve fiber layer (RNFL) thickness, and optic nerve head (ONH) parameters. MAIN OUTCOME MEASURES: Circumpapillary RNFL, OCT ONH parameters, and HRT parameters. RESULTS: The univariate analysis of OCT parameters demonstrated significant differences between the temporal and nasal quadrants; clock hours 3 (55 vs. 73 µm), 4, 8 (93.9 vs. 70.7 µm), 9, and 10; vertical cup-to-disc ratio (C:D) (0.6 vs. 0.8) and cup volume (0.2 vs. 0.5) (P<0.001) between patients with CON and OAG, respectively. The CON discs were significantly different from normal discs for all OCT parameters except cup volume. The CON discs were not significantly different from normal discs for HRT parameters, except for mean RNFL thickness and cup shape measure. The OAG discs were significantly different from normal discs in all HRT and OCT parameters (P<0.001). Multivariate analysis demonstrated that the OCT 3 o'clock temporal sector, average C:D ratio, vertical C:D ratio, and cup volume measurements were able to differentiate OAG from CON. CONCLUSIONS: Compressive optic neuropathy is associated with significantly thinner nasal and temporal sectors compared with OAG, whereas OAG results in larger cups and cup volume with OCT measurements. The Heidelberg retinal tomograph is not able to differentiate CON from normal discs.

Orbital amyloidosis masquerading as metastatic breast carcinoma.

One of the most common causes of enophthalmos is previous orbital fracture. In a patient with a known history of breast carcinoma, enophthalmos is concerning for metastatic disease. This report presents a patient with a history of scirrhous breast carcinoma and enophthalmos who was found to have orbital amyloidosis. This is the first report of enophthalmos as the presenting sign of orbital amyloidosis.

Clinical challenge: Looking beyond blurred margins.

A 60-year-old man presented to an outside ophthalmology clinic with 1 month of progressive vision loss in the right eye (OD). Right optic disc edema was noted. Brain and orbit magnetic resonance imaging revealed right optic nerve and left occipital lobe enhancement. He was seen initially by neurology and neurosurgery and subsequently referred to neuro-ophthalmology for consideration of optic nerve biopsy. He was seen 3 months after his initial symptom onset where vision was light perception OD and a relative afferent pupillary defect with optic nerve edema. OS was unremarkable. A lumbar puncture with flow cytometry was negative for multiple sclerosis and lymphoma. At his oculoplastic evaluation for optic nerve biopsy, his vision was noted to be no light perception OD. Optic nerve biopsy demonstrated non-caseating granulomatous inflammation consistent with neurosarcoidosis. The patient was started on high-dose oral steroids with improvement of disc edema, as well as significant improvement in optic nerve and intracranial parenchymal enhancement, although his vision never improved.

Giant cell arteritis incidence: analysis by season and year in mid-Atlantic United States.

BACKGROUND: Evaluate the incidence of biopsy-proven giant cell arteritis for seasonal or annual variability in the Mid-Atlantic United States. DESIGN: Retrospective chart review of all patients undergoing temporal artery biopsy from 1994 to 2011. PARTICIPANTS: The charts of 744 patients were reviewed, and 215 patients were diagnosed with giant cell arteritis based on positive temporal artery biopsy results. METHODS: All results between 1994 and 2011 were reviewed. Giant cell arteritis incidence data were evaluated by year, season and month for any trends or cyclic patterns. MAIN OUTCOME MEASURE: Incidence of biopsy-proven giant cell arteritis. RESULTS: The majority of patients were female (74%) and over the age of 60 (98.6%). The diagnosis of biopsy-proven giant cell arteritis was found in 215 of 744 (28.9%) patients. The incidence of biopsy-proven giant cell arteritis by year varied, with the peak incidence in 1996. The monthly incidence peaked in July and had a trough in October. However, Poisson regression analysis did not show any statistically significant trend over time or cyclic pattern to the incidence by year, season or month. CONCLUSIONS: The incidence of biopsy-proven giant cell arteritis in the population studied did not have any significant cyclic pattern over the last 17 years. The highest incidence by month was noted in July with a trough in October. However, this was not a significant pattern by month or season to support infectious or periodic environmental factors inciting giant cell arteritis.

Shrinkage revisited: how long is long enough?

PURPOSE: Temporal artery biopsy (TAB) is considered the gold standard in the diagnosis of suspected giant cell arteritis. The most commonly accepted length for an adequate postfixation TAB specimen is 20 mm. There is a reported 2.4-mm mean shrinkage with the fixation process, but to date, there is no data correlating shrinkage after specimen fixation with the biopsy results. METHODS: A prospective, Institutional Review Board-approved study of all patients undergoing TAB over 1 year was performed. The pre- and postfixation measurements were recorded. Comparison of the pre- and postfixation lengths was performed, and potential correlation was sought with biopsy results and patient gender, age, and race/ethnicity. RESULTS: Sixty-two TABs were performed over a 1 year period with 53 (85.5%) negative for giant cell arteritis. The mean shrinkage length was 4.61 mm ± 2.97 overall, and the amount of shrinkage between positive and negative TAB specimens was not significant (p = 0.43). Linear regression analysis did not show any correlation between the amount of shrinkage and the length of the specimen or duration in fixative. There was no significant difference between the amount of shrinkage by surgeon (p = 0.82), patient gender (p = 1.00), or race/ethnicity (p = 0.695). CONCLUSIONS: Surgeons should be aware of the amount of shrinkage of TAB specimens to meet the commonly accepted goal of 20 mm postfixation length. Based on the 4.61-mm mean shrinkage with 2.97-mm standard deviation, a 27.58-mm specimen would have to be obtained to reach the 20-mm goal. Surgeon, patient age, gender, race/ethnicity, and biopsy results did not have a significant impact on the amount of TAB specimen shrinkage.

Case report 68Ga-DOTATATE of optic nerve sheath meningioma.

We report a patient with an optic nerve sheath meningioma whose diagnosis and management were guided by using Gallium-68 DOTA-Tyr3-octreotatate (68Ga-DOTATATE). Positron Emission Tomography-Computed Tomography (PET-CT).

Temporal patterns of visual recovery following pituitary tumor resection: A prospective cohort study.

Significant restoration of visual function can occur following pituitary tumor resection, although the time course of visual recovery remains poorly understood. This single-centre, two-year, prospective cohort study investigated the temporal patterns of visual recovery in consecutive patients undergoing pituitary tumor resection, between 2009 and 2018. Eyes were stratified based on pre-operative optical coherence tomography (OCT) retinal nerve fibre layer (RNFL) thickness measurements, with thin RNFL being defined as those within the fifth-percentile of age-matched normative values, and normal RNFL as those above the fifth-percentile. Visual function and OCT parameters were assessed pre-operatively, and at 6 weeks, 6 months, and 2 years post-operatively. 456 eyes of 228 patients (mean ± SD age, 53 ± 15 years) were included, of which 114 (25%) eyes had thin RNFL pre-operatively. Visual field recovery was observed in both groups during the first 6 weeks post-operatively (all Q ≤ 0.02), although improvements in visual field parameters between 6 weeks to 6 months were limited to eyes with thin RNFL (both Q < 0.05). No further improvements in visual function were detected beyond 6 months in both groups (both Q > 0.50). Similar trends were observed in linear regression analysis according to baseline visual function in both groups. In summary, eyes with normal RNFL thickness at baseline experienced most of their recovery within the first six weeks following surgery, while eyes with thin RNFL exhibited gradual improvements during the first six months. These findings have important implications when providing patient counselling and prognostication in the pre-operative setting.

Prognostic Utility of Optical Coherence Tomography for Long-Term Visual Recovery Following Pituitary Tumor Surgery.

PURPOSE: To investigate the association between optical coherence tomography (OCT) parameters and long-term visual recovery following optic chiasm decompression surgery. DESIGN: Prospective cohort study. METHODS: Consecutive patients who underwent pituitary or parasellar tumor resection between January 2009 to December 2018 were recruited in a single-center, 2-year prospective, longitudinal cohort study. Best-corrected visual acuity, visual fields, and OCT retinal nerve fiber layer (RNFL) thickness, macular thickness and volume were assessed preoperatively, and at 6 weeks, 6 months, and 2 years postoperatively. Long-term visual field recovery and maintenance were defined as a mean deviation of >-3 at 24 months, and visual acuity recovery and maintenance were defined as a logarithm of minimal angle of resolution (logMAR) of 0 (Snellen 20/20) or better at 24 months. RESULTS: A total of 239 patients (129 men, 110 women; mean ± SD age: 52 ± 16 years) were included. Multiple logistic regression analysis demonstrated that increased inferior RNFL thickness (per 10 µm) was associated with higher odds of long-term visual field recovery and maintenance (odds ratio [OR]: 1.26; 95% confidence interval [CI]: 1.12-1.41; Q < 0.001), and greater superior RNFL thickness (per 10 µm) was associated with higher odds of visual acuity recovery and maintenance (OR: 1.13; 95% CI: 1.03-1.27; Q = 0.031). A multivariable risk prediction model developed for long-term visual field recovery and maintenance that incorporated age, preoperative visual function, and RNFL thickness demonstrated C-statistics of 0.83 (95% CI: 0.72-0.94). CONCLUSION: Preoperative RNFL thickness was associated with long-term visual recovery and maintenance following chiasmal decompression. The multivariable risk prediction model developed in the present study may assist with preoperative patient counseling and prognosis.

Evaluation of the retinal nerve fiber layer: descriptive or predictive?

For nearly a century, ophthalmologists have recognized that thinning of the retinal nerve fiber layer (rNFL) could be observed ophthalmoscopically in diseases of the optic nerve. Using high-resolution red-free fundus photography, Hoyt found slit-like rNFL defects that corresponded to visual field defects in glaucoma. Frisén extended these observations to multiple sclerosis, predicting the later discovery that axonal loss occurs in the retina without clinical bouts of optic neuritis. In measurement of the rNFL, red-free fundus photography has been superseded by more widely available, robust, and quantitative retinal imaging techniques, including Heidelberg retinal tomography, scanning laser polarimetry, and optical coherence tomography (OCT). Having emerged as the technique of choice in measuring the rNFL, OCT has shown that the degree of preoperative rNFL thinning reliably predicts whether vision will recover after surgery for pituitary adenoma. Such quantitative studies of the rNFL have the potential, therefore, of providing descriptive and predictive information that will be valuable in clinical care.

Bilateral abducens nerve palsy due to septic thrombophlebitis.

PURPOSE: To report a case of septic thrombophlebitis producing bilateral abducens nerve palsy. OBSERVATION: A 65 year-old woman with recent sinus surgery experienced the onset of horizontal diplopia during treatment for bacteremia. Computer tomography of head and a neck ultrasonography showed right internal jugular vein occlusion. Ophthalmology examination was consistent with bilateral abducens nerve palsy. She was treated with systemic antibiotics and antiplatelet therapy with resolution of the internal jugular vein occlusion. The diplopia improved over a six-months. CONCLUSION AND IMPORTANCE: Our patient had Lemierre syndrome with an unusual presentation. The patient was treated for septic thrombophlebitis with a resolution of her ocular symptoms.

Visual loss and recovery in chiasmal compression.

Compression of the optic chiasm causes an optic neuropathy that may be associated with reversible visual loss often immediately following surgical decompression. While the precise pathogenesis of retinal ganglion cell impairment and eventual death remains poorly understood, a number of putative mechanisms may play a role. In this article we review the evidence supporting various stages of visual loss and recovery in chiasmal compression. These include conduction block, demyelination, ischemic insult, and retrograde and anterograde degeneration. We also describe novel advances in magnetic resonance imaging with specialized modalities such as diffusion tensor imaging have provided further information to explain the underlying mechanism of visual loss. Functional measures including electrophysiology are time-consuming but have shown moderate prognostic ability. Optical coherence tomography has provided novel new biomarkers for predicting outcome following surgical decompression. Both retinal nerve fiber layer thickness and ganglion cell complex thicknesses have shown to have excellent predictive power. Such advances serve to inform patients and clinicians of pre-operative factors that predict the extent of visual recovery following medical or surgical treatment of para-chiasmal lesions.

In vivo retinal nerve fiber layer thickness measured by optical coherence tomography predicts visual recovery after surgery for parachiasmal tumors.

PURPOSE: Restoration of visual function after neurosurgery for parachiasmal tumors is variable and unpredictable. The current study was conducted to determine whether in vivo retinal nerve fiber layer (RNFL) thickness measurements predict the visual recovery of such patients. METHODS: Forty patients undergoing surgical resection of parachiasmal lesions were prospectively assessed before surgery with a neuro-ophthalmic examination, involving standard automated visual field (VF) testing and optical coherence tomography (OCT) measurements of RNFL thickness, which was the prespecified marker for axonal loss. Tests were repeated within 6 weeks after surgery. RESULTS: Thinner preoperative RNFL thickness was associated with worse visual acuity (VA) and VF mean deviation (MD). Patients with normal preoperative RNFL had significant improvement in mean VA after surgery, from 20/40 to 20/25 (P = 0.028), whereas patients with thin RNFL did not improve (20/80 to 20/60, P = 0.177). Eyes with normal RNFL showed improvement in MD (-7.0 dB before surgery, -3.5 dB after surgery, P = 0.0007) unlike eyes with thin RNFLs, which had no significant improvement after surgery (-15.3 dB before and -13.3 dB after surgery, P = 0.191). RNFL thickness increased by 1% after surgery among all eyes (P = 0.04). Eyes with severe VF defects (MD

Brightness sensitivity and color perception as predictors of relative afferent pupillary defect.

PURPOSE: To characterize the relationship between brightness sensitivity and color perception and relative afferent pupillary defect (RAPD) in patients with optic neuropathy. METHODS: The "swinging flashlight test" was used to diagnose RAPD, the degree of which was quantified by neutral density filters, in 325 consecutive patients in a case-control study. A separate examiner, masked to the pupillary findings, then assessed participants for Ishihara color plate reading, brightness sense, and red perception. The latter two were quantified by asking the patient to score (out of 100%) brightness (of a light source) or redness (of an object) of the two eyes relative to each other. Pearson correlation coefficients and receiver operating characteristic (ROC) curves were calculated. RESULTS: Brightness sense (r = -0.79; 95% confidence interval [CI], -0.84 to -0.73; P < 0.0001), red perception (r = -0.73; 95% CI, -0.79 to -0.65; P < 0.0001), and Ishihara color plate reading (r = -0.68; 95% CI, -0.79 to -0.66; P < 0.0001) were each strongly and highly significantly correlated with the diagnosis and degree of RAPD. Brightness sense and red perception were each able to discriminate almost all the area under ROC for the diagnosis of RAPD (area of 0.99; 95% CI, 0.98-1.00; P < 0.0001; area of 0.93; 95% CI, 0.90-0.96; P < 0.0001, respectively). Sensitivity and specificity of brightness sense in detection of RAPD were 99% (95% CI, 0.97-1.00) and 95% (95% CI, 0.91-0.98), respectively. The red perception test was only slightly less accurate. CONCLUSIONS: Rapid, simple assessments of brightness sense and color perception provide accurate methods to facilitate the diagnosis of optic neuropathy and may prove to be valuable in screening for optic neuropathy or alternatives to the swinging flashlight test.

Missing the bull's eye.

We report a patient with a long history of hydroxychloroquine use. Clinical examinations had been performed according to American Academy of Ophthalmology guidelines with no abnormalities. A multifocal electroretinogram (mfERG) was performed to further assess macular function. Multifocal ERG may detect macular dysfunction earlier than the currently recommended screening guidelines in patients with potential for macular toxicity from hydroxychloroquine.

The Utility of Color Duplex Ultrasonography in the Diagnosis of Giant Cell Arteritis: A Prospective, Masked Study. (An American Ophthalmological Society Thesis).

PURPOSE: To evaluate the diagnostic yield and concordance of color duplex ultrasound (CDU) of the superficial temporal artery (STA), temporal artery biopsy (TAB), and American College of Rheumatology (ACR) criteria in the diagnosis of giant cell arteritis (GCA). METHODS: Prospective, masked study of all patients evaluated in one institution suspected of having GCA. All patients with a suspected diagnosis of GCA were admitted for pulsed intravenous corticosteroids. Patients underwent serologic work-up and ACR criteria were documented. All patients had a CDU and TAB performed within 3 days of initiation of systemic corticosteroid therapy. Main outcome measure: Concordance of CDU and TAB. Secondary outcome measures: Concordance between unilateral and bilateral CDU and TAB by side and segment, concordance between TAB and ACR criteria, and statistical analysis of serologic markers for GCA. RESULTS: The diagnosis of biopsy-proven GCA was found in 14 of 71 (19.7%) patients. The sensitivity of CDU compared to the reference standard of TAB ranged between 5.1% and 30.8% depending on the signs studied on CDU and correlation of specific TAB parameters. Of the serologic studies, a platelet count threshold of 400,000µL had the highest positive (18.32) and lowest negative (0.37) likelihood ratios for a diagnosis of GCA. CONCLUSIONS: In this study, CDU showed minimal value in diagnosing GCA compared to TAB. There was poor correlation between CDU results and ACR criteria for GCA. The threshold platelet count had higher positive and negative predictive values for GCA than CDU and is a useful serologic marker for GCA.

Relationship between retinal nerve fiber layer and visual field sensitivity as measured by optical coherence tomography in chiasmal compression.

PURPOSE: To investigate the spatial relationship between retinal nerve fiber layer (RNFL) thickness measured with optical coherence tomography (OCT) and visual field sensitivity (VFS) measured by standard automated perimetry (SAP) in chiasmal compression. METHODS: Twenty-six patients with chiasmal compression were enrolled. RNFL thickness was measured with the StratusOCT and VFS with SAP (Humphrey Field Analyzer; both from Carl Zeiss Meditec, Dublin, CA). Relationships between RNFL thickness (in clock hours, hemifields, and sectors) and VFS (zones were divided into hemifields, quadrants, and sectors based on a validated visual field map) expressed in a decibel scale and 1/lambert (L) were evaluated by linear and nonlinear regression. Coefficients of determination (R(2)) were calculated by using a multivariate model. RESULTS: Average RNFL thickness correlated strongly with pattern standard deviation (PSD; R = 0.622) and mean deviation (MD; R = 0.413). The four strongest correlations were between the 8 o'clock OCT position (temporal disc), with the temporal hemifield (R = -0.813), the superotemporal quadrant (R = -0.847), the inferotemporal quadrant (R = -0.855), and the field sector representing the papillomacular bundle (R = -0.809). Coefficients of determination improved significantly in all sectors when time since surgery was included in the regression model-most notably, average thickness and 1/L (R(2) = 0.35-0.49), the decibels (R(2) = 0.31-0.47), and the temporal sector (R(2) = 0.44-0.57). CONCLUSIONS: This is the first study to compare the structure-function correlation of RNFL measured by OCT with SAP in patients with chiasmal compression. RNFL is topographically related globally and sectorally to decreased SAP, with the temporal sectors showing the strongest correlations. The correlation between RNFL and VFS strengthens as the time from surgical intervention increases.

Occurrence of cerebral venous sinus thrombosis in patients with presumed idiopathic intracranial hypertension.

PURPOSE: To report the rate of occurrence of cerebral venous sinus thrombosis (CVST) in patients with presumed idiopathic intracranial hypertension (IIH). DESIGN: Retrospective chart review. PARTICIPANTS: All patients diagnosed with papilledema from November 1, 2002, through October 31, 2003, at 3 tertiary care neuro-ophthalmology centers. METHODS: Consecutive patients with a diagnosis of papilledema from 3 tertiary care neuro-ophthalmology centers were identified. Patients with space-occupying lesions, hydrocephalus, or meningitis were excluded. The remaining patients were evaluated with lumbar puncture, magnetic resonance imaging (MRI), and magnetic resonance venography (MRV). MAIN OUTCOME MEASURES: The rate of occurrence of CVST in patients with presumed IIH. RESULTS: One hundred thirty-one patients with papilledema were identified. Excluding patients with mass lesions, meningitis, or hydrocephalus, the occurrence of CVST was 10 (9.4%) of 106 patients with presumed IIH. Two additional patients had a diagnosis of suspected CVST. Cerebral venous sinus thrombosis was diagnosed in 1 of the 10 patients with MRI alone, whereas it was evident in all 10 patients with MRV. Underlying risk factors for CVST were identified in 9 of 10 patients. CONCLUSIONS: Cerebral venous sinus thrombosis accounts for 9.4% of patients with presumed IIH in 3 tertiary care neuro-ophthalmology services. Magnetic resonance venography in combination with MRI is recommended to identify this subgroup of patients.

Prevalence of a normal C-reactive protein with an elevated erythrocyte sedimentation rate in biopsy-proven giant cell arteritis.

OBJECTIVE: The erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are laboratory tests that have been said to have a strong correlation with a positive temporal artery biopsy in patients with suspected giant cell arteritis (GCA). Published reports suggest that the CRP is a more sensitive diagnostic indicator of GCA and can be elevated when the ESR is normal. It is also clear that the CRP and ESR can both be normal or both be elevated in patients with biopsy-proven GCA and that the CRP can be elevated when the ESR is normal. The purpose of this study was to ascertain if the CRP can be normal when the ESR is elevated in biopsy-proven GCA. DESIGN: Retrospective, longitudinal, comparative study. PARTICIPANTS: One hundred nineteen patients from 6 major tertiary-care university-affiliated medical centers. METHODS: The charts from 119 patients with temporal artery biopsies positive for GCA were reviewed for age, gender, pretreatment ESR, and pretreatment CRP. MAIN OUTCOME MEASURES: The ESR in millimeters per hour Westergren was graded as normal or abnormal based on 2 validated formulas. The CRP was graded as normal or abnormal based on established criteria set forth in the literature as well as at The Johns Hopkins Hematology laboratory. RESULTS: In this study, the ESR had a sensitivity of 76% to 86%, depending on which of 2 formulas were used, whereas an elevated CRP had a sensitivity of 97.5%. The sensitivity of the ESR and CRP together was 99%. Only 1 of the 119 patients (0.8%) presented with a normal ESR and normal CRP (double false negative); 2 patients (1.7%) had a normal CRP despite an elevated ESR according to both formulas. CONCLUSION: Although most patients with GCA have both an elevated ESR and CRP, there can be nonconcordance of the 2 blood tests. Although such nonconcordance is most often a normal ESR but an elevated CRP, the finding of an elevated ESR and a normal CRP also is consistent with GCA. The use of both tests provides a slightly greater sensitivity for the diagnosis of GCA than the use of either test alone.

Correlation of retinal nerve fiber layer measured by scanning laser polarimeter to visual field in ischemic optic neuropathy.

OBJECTIVE: To evaluate the relationship between retinal nerve fiber layer (RNFL) measurement with scanning laser polarimetry (SLP) and standard automated perimetry (SAP) in nonarteritic anterior ischemic optic neuropathy (NAION). METHODS: In this prospective observational case series, all subjects (28 eyes with NAION) underwent SAP and SLP. The RNFL retardation measurements and visual field test points were grouped into 6 corresponding sectors. The contralateral uninvolved eye was used as control. The relationship between RNFL retardation and SAP was evaluated with the Spearman nonparametric technique and linear regression analysis. The main outcome measure was correlation of SLP RNFL parameters and SAP. RESULTS: Global and sectoral SLP parameters showed a significant difference in affected eyes compared with controls. The strongest correlations were seen between mean deviation and number (r = -0.524; P = .004), ellipse modulation (r = 0.5026; P = .006), and maximum modulation (r = 0.526; P = .004). Superior sectoral visual field indexes showed a strong correlation with inferior RNFL changes (r = 0.522; P<.008). Linear regression confirmed a strong relationship between the superior sectoral visual field indexes and the inferior RNFL. CONCLUSION: Scanning laser polarimetry was able to identify structural changes of the RNFL globally and in the inferior SLP sector with functional loss in NAION.