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.

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.

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).

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.

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.

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.

Optical coherence tomography predicts visual outcome for pituitary tumors.

We evaluate if the relationship between optical coherence tomography (OCT) of the retinal nerve fibre layer (RNFL) and visual outcome continued over long-term visual recovery in 107 patients undergoing pituitary decompression. Recently, it has been recognized that OCT of the RNFL has prognostic value in predicting visual outcomes after surgery for chiasmal compression caused by pituitary tumours. Patients were followed up at three time points: pre-operative (visit 1), 6-10 weeks post-operative (visit 2) and 9-15 months follow-up (visit 3). We found that patients with thin pre-operative RNFL had more severe visual field defects (mean deviation [MD] -9.22 versus -3.96 decibels [dB]; p = 0.001), but pre-operative visual acuity (VA) was good in both normal and thin RNFL groups (Snellen VA 6/5 and 6/4; p = 0.039). For those with thin RNFL the greatest improvement was between visit 2 and 3 (MD -7.1 dB versus -3.4 dB, respectively; p < 0.001) compared with pre-operative -9.8 dB. Normal RNFL patients showed greatest improvement between visits 1 and 2 (pre-operative -4.8 dB, visit 2 -2.0 dB, visit 3 -0.9 dB; p = 0.001). For long-term follow-up, 81.0% of eyes with normal RNFL, compared to 37.1% with thin RNFL (p < 0.001), achieved an MD of -2.00 dB (final visit). At final follow-up, 97.5% of normal RNFL eyes achieved VA of 6/12 or better compared with 88.2% with thin RNFL (p = 0.034). Our results indicate that long-term visual recovery after surgical decompression of pituitary lesions is predicted by pre-operative OCT RNFL. Patients with normal RNFL thickness show an increased propensity for visual recovery. This effect continues after long-term follow-up, however, most visual recovery occurs within the first 6-10 weeks.

Toxic optic neuropathy: an unusual cause.

A 60-year-old woman with a history of chronic alcoholism and tobacco use presented with the complaint of a painless decrease in vision in both eyes. She lost vision first in the left eye then in the right eye. She admitted consuming at least one 16 ounce bottle of over the counter mouthwash daily and denied consumption of any other alcohols, methanol, or antifreeze. She stated that her vision had been continuing to deteriorate in both eyes. Her best-corrected visual acuity was 4/200 in each eye. Color vision was nil in each eye. Her pupils were sluggish bilaterally, and her optic discs were flat and hyperemic with peripapillary hemorrhages. Her visual fields revealed central scotomas bilaterally. The magnetic resonance imaging of the brain and lumbar puncture were within normal limits. Antinuclear antibody, human leukocyte antigen-B27 genotyping, and B12 were normal; serum thiamine was low. While continuing to ingest mouthwash, her vision decreased to count fingers at 2 feet, and maculopapillary bundle pallor developed. She was started on folate and thiamine supplementation. Once she discontinued mouthwash, her vision improved to 20/400 bilaterally, and her central scotomas improved. This case demonstrates an alcohol-induced toxic optic neuropathy from mouthwash ingestion with some visual recovery after discontinuation of the offending agent.

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.

Visual acuity and pattern of visual field loss at presentation in pituitary adenoma.

Our purpose was to analyse the demographics, prevalence and pattern of visual field defects in patients with pituitary adenoma. We prospectively recruited 103 consecutive patients (206 eyes) presenting to a neurosurgical unit with pituitary adenoma. Ophthalmological examination and standard automated perimetry (Humphrey, 24-2 threshold) was performed. Severity of visual field defects was also assessed. The mean population age was 53.9 years (standard deviation=15). Visual loss was the most common reason for presentation (39%) followed by endocrine abnormality (21%) and headache (15%). Patients with endocrine abnormality on presentation were 10.9 years younger than those presenting with visual loss (p=0.001). Bitemporal defects were the most prevalent pattern (n=22, 41%) followed by homonymous defects (n=7, 13%). Of the patients with visual field loss, 33% had unilateral visual field defects. The mean visual acuity in those with bitemporal defects was 6/7.5 with half of these patients having 6/6 vision in both eyes. In conclusion, the majority of patients with pituitary adenoma have visual acuity better than 6/7.5 despite having visual field defects. While a bitemporal pattern of visual field loss is the most common, a significant proportion of patients had unilateral and altitudinal defects. Assessment of the visual field is essential to rule out chiasmal compression.

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.

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.

Validity of the American College of Rheumatology criteria for the diagnosis of giant cell arteritis.

PURPOSE: To assess the clinical utility of the American College of Rheumatology criteria for the diagnosis of giant cell arteritis (GCA) in patients with positive and negative temporal artery biopsies. DESIGN: Retrospective case series of all patients undergoing temporal artery biopsy. METHODS: Retrospective chart review of all patients seen in the Neuro-ophthalmology Service of the Wills Eye Institute undergoing biopsy. One hundred twelve patients were identified between October 2001 and May 2006. Charts were reviewed for American College of Rheumatology criteria, biopsy results, and progression of visual loss after diagnosis. RESULTS: Nine of 35 patients (25.7%) with positive biopsies would not have been diagnosed with GCA using American College of Rheumatology criteria alone. An additional 16 patients (45.7%) met only 2 criteria and required the positive biopsy to establish the American College of Rheumatology diagnosis of GCA. Eleven of 39 patients (28.2%) with negative biopsies met the criteria and would have been diagnosed with GCA. Diagnostic agreement between the American College of Rheumatology criteria without biopsy results and biopsy results alone was 51.4%; with the addition of biopsy results to the criteria, this increased to 73.0%. CONCLUSIONS: The current American College of Rheumatology criteria should not be used to diagnose GCA and all patients suspected of having GCA should undergo a temporal artery biopsy.

Macular thickness predictive of visual field sensitivity in ischaemic optic neuropathy.

PURPOSE: To investigate the ability of optical coherence tomography (OCT) parameters of macular thickness (MT) and peripapillary retinal nerve fibre layer (RNFL) thickness to differentiate eyes with nonarteritic anterior ischaemic optic neuropathy (NAION) from uninvolved eyes and to identify the relationship between macular and RNFL parameters and visual field sensitivity (VFS). METHODS: Thirty patients with unilateral NAION participated in a prospective observational cross-sectional study. Patients underwent Humphrey visual field (SITA Standard 24-2, HVF) testing and OCT to measure MT and RNFL. The contralateral uninvolved eye was used as controls. Areas under the receiver operating characteristic curves (AUROCs) of MT and RNFL for discriminating NAION from control eyes were also determined. The prespecified outcome measure was the correlation between RNFL, MT and mean deviation (MD). RESULTS: Average RNFL and MT were thinner in NAION eyes: 72.8 µm versus 98.9 µm (p<0.0001) and 231.9 µm (SD, 21.4) vs. 251.1 µm (SD, 14.8; p=0.0001), respectively. The largest AUROCs were for average MT (0.87) and average RNFL thickness (0.88). Overall, macular parameters showed stronger correlation with VFS than RNFL parameters. The highest correlation was average MT (0.71; p<0.0001) followed by RNFL parameter nasal quadrant RNFL (0.40; p=0.030). CONCLUSION: Both MT and RNFL show strong correlations with level of VFS in NAION. Macular thickness showed more robust correlations with VF and provides strong surrogate marker of the level of damage in NAION.

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.

Neuroimaging and acute ocular motor mononeuropathies: a prospective study.

OBJECTIVE: To evaluate the necessity of neuroimaging in patients with acute, isolated ocular motor mononeuropathies. METHODS: A prospective case series evaluating diagnostic technology results in 93 patients older than 50 years with acute isolated mononeuropathies was performed. Patients were included in the study if they had new-onset diplopia with an isolated cranial neuropathy (cranial nerve III, IV, or VI palsy) and no other signs of neurologic dysfunction. All patients had gadolinium-enhanced magnetic resonance imaging (MRI). The number of patients with lesions noted on MRI and the overall cost of imaging the patients were determined. Cost analysis of the MRI was conducted using Current Procedural Terminology codes and Medicare costs in 2010 dollars. Cost utility was estimated using cost data as well as published utility values for adults with diplopia and sex-specific life tables for life expectancy in the United States. RESULTS: Four of 93 patients had lesions on MRI; however, only 1 of the 93 patients had a lesion related to the cranial mononeuropathy. The total modeled cost of imaging for these 93 patients was $131,688 to determine an underlying cause in 1 patient with no change in treatment. The estimated cost utility for the patient with a causative lesion found by MRI was $90.19 for diagnosis alone. CONCLUSIONS: It may not be medically necessary to perform MRI scanning on every patient with an isolated cranial nerve III, IV, or VI palsy. In adults older than 50 years with an isolated mononeuropathy, physicians should carefully review the patients' history and findings to determine which patients to image at the initial evaluation.