Diagnosis and management of optic nerve sheath meningiomas.

Optic nerve sheath meningiomas account for a third of all intrinsic tumours of the optic nerve. Despite their classification as histologically benign tumours they cause progressive visual loss that often leads to blindness if left untreated. Recent therapeutic advances have increased the treatment options available to clinicians but patient management remains controversial. We systematically review the progress made in the diagnosis and management of optic nerve sheath meningiomas, clarify current best practice, and suggest future avenues for research.

Suprasellar meningioma presenting with an altitudinal field defect.

We report a female patient with an unusual suprasellar meningioma presenting with a right-sided inferior altitudinal visual field defect. Two causative factors were identified at surgery: an aberrant ophthalmic artery found lying on the superior aspect of the optic nerve, and marked compression of the nerve superiorly against the falciform ligament.

Diagnosis and management of optic nerve glioma.

Optic nerve gliomas are highly variable tumours with an unpredictable clinical course. Consequently, the diagnosis and management of these tumours remains complex and a standardised management strategy does not exist. In this paper we describe a patient with optic nerve glioma treated at our institution and then review recent advances made in the diagnosis and treatment of these tumours over the past 10 years. Our aim is to clarify current best practice in the management of optic nerve gliomas.

Diagnostic accuracy of confrontation visual field tests.

OBJECTIVE: To determine the diagnostic accuracy of confrontation visual field testing and to compare the accuracy of confrontation tests both individually and in combination. METHODS: Patients were prospectively recruited from ophthalmology clinics over a 6-month period. All patients underwent SITA-standard 24-2 Humphrey visual field analysis. Two examiners, masked to the automated perimetry results and the results of the other examiner, assessed patients using 7 common confrontation visual field tests. The order of testing was randomized to reduce any learning effect. For each individual test and combination of tests, the sensitivity, specificity, positive predictive value, and negative predictive value were calculated. RESULTS: A total of 301 eyes from 163 patients were included in the study. The average mean deviation was -5.91 +/- 7.72 (SD) dB. Most confrontation tests were insensitive to the identification of field loss. The sensitivity and specificity varied depending on the type, density, and cause of the visual field defect. Kinetic testing with a red target provided the highest sensitivity (74.4%) and specificity (93.0%) of any individual test and when combined with static finger wiggle testing achieved a sensitivity of 78.3% while retaining a specificity of 90.1%. CONCLUSIONS: Confrontation visual field tests are insensitive at detecting visual field loss when performed individually and are therefore a poor screening test. Combining confrontation tests is a simple and practical method of improving the sensitivity of confrontation testing.

Giant cell arteritis.

Giant cell arteritis (GCA) is an immune-mediated vasculitis affecting individuals over 50 years of age. It is characterised by granulomatous inflammation that affects medium-sized and large arteries. The wide spectrum of clinical manifestations can be divided into those related to tissue ischemia from vascular lesions and those related to a systemic inflammatory response. The pathogenesis of these groups also appears distinct, with vascular lesion formation thought to be an adaptive immune response, and the systemic inflammatory reaction an innate immune response. Clinical suspicion of GCA must remain especially high in those with neurological or visual symptoms and if warranted, prompt treatment with high-dose corticosteroids is invaluable in halting disease progression.

Reduction of optic nerve fibers in patients with Alzheimer disease identified by laser imaging.

In this case-control study, we compared the optic nerves (ONs) by clinical examination and scanning laser ophthalmoscopy (SLO) of 40 patients with Alzheimer disease (AD) and 50 controls. There was a reduction in the number of ON fibers in patients with AD, with a threefold greater odds ratio for a larger optic cup-to-disc ratio in patients with AD.

Comparison of disc damage likelihood scale, cup to disc ratio, and Heidelberg retina tomograph in the diagnosis of glaucoma.

AIM: To evaluate the relative diagnostic strength of cup to disc (C/D) ratio, clinical disc damage likelihood scale (DDLS), a new clinical method of documenting glaucomatous optic disc changes, and Heidelberg retina tomograph (HRT-II) in patients with glaucoma, glaucoma suspects, and normal controls. METHOD: Consecutive observational case series. 110 eyes from 110 patients categorised as glaucoma, glaucoma suspect, or normal were examined clinically to grade the DDLS score. HRT-II examination was performed by an examiner masked to the clinical examination findings. Optic disc parameters and Moorfields regression analysis findings were recorded. Stereophotographs of the optic disc were examined independently by two glaucoma specialists in masked fashion to determine the C/D ratio. Zeiss SITA Standard 24-2 visual fields were obtained within 3 months of HRT-II and clinical examination. For each patient, the eye with the worse mean deviation of the visual field test was enrolled in the study, and each field was additionally graded by the four level Hodapp-Parrish-II-Anderson staging. Specificity and sensitivity were calculated by receiver operating characteristic (ROC) curves. RESULTS: Mean patient age was 58 years (SD 13.3) with 45 glaucoma patients, 23 glaucoma suspects, and 42 normals. The mean deviation on Humphrey visual field assessment using SITA-Standard was -4.95 D (SD 5 D) Clinical examination using DDLS had the best predictive power with an area under the ROC curve value of 0.95 when glaucoma patients and suspects were separated from borderline or normals. This was followed by clinical examination of C/D ratio (0.84), and HRT-II Moorfields analysis (0.68). The order of diagnostic strength did not change when definite glaucoma was compared to borderline and normals. CONCLUSIONS: The DDLS grading performs well compared to C/D ratio and HRT-II evaluation. Attention to disc diameter and to rim width may increase the value of clinical optic disc examination.

Comparison of intraocular pressure measured by Pascal dynamic contour tonometry and Goldmann applanation tonometry.

AIMS: To compare the intraocular pressure (IOP) measurements obtained using the Pascal dynamic contour tonometer (PDCT) with the standard Goldmann applanation tonometer (GAT) and to correlate these with central corneal thickness (CCT) in patients with normal corneas. METHODS: A prospective, masked, comparative case series of 116 eyes from patients attending a glaucoma clinic. IOP was measured with PDCT by one examiner and with GAT by a masked, independent examiner. A mean of six CCT readings was used for analysis. RESULTS: IOP measured by the two instruments correlated significantly (r=0.77; P<0.0001). IOP measured by GAT correlated strongly with CCT (r=0.37, P=0.0001) whereas the relationship between IOP measured by PDCT and CCT approached significance (r=0.17, P=0.073). The differences between GAT and PDCT measured IOP also correlated strongly with CCT (r=0.37, P<0.0001). The 95% limits of agreement between GAT and PDCT were +/-4.2 mmHg. Dividing the eyes into three groups on the basis of CCT, demonstrated those in the thickest tertile showed a poorer agreement between instruments and the GAT measured significantly higher IOP in this group (P=0.003) while the PDCT showed no significant differences with different CCTs (P=0.37). CONCLUSION: Demonstration of the relative independence of PDCT IOP measurements from CCT supports a potential clinical role for this instrument, particularly for subjects with CCT outside the normal range.

I don't need my glasses, but...

A 24-year old man complained of decreased vision in his right eye for 1 week. Examination revealed a subtle right optic neuropathy. Fundoscopy showed bilateral buried drusen, which were confirmed with B-scan ultrasonography. An MRI was normal.

The prevalence of cupping in end-stage arteritic and nonarteritic anterior ischemic optic neuropathy.

OBJECTIVE: To evaluate the prevalence of cupping in arteritic anterior ischemic optic neuropathy (AAION) and nonarteritic anterior ischemic optic neuropathy (NAION). DESIGN: Retrospective, observational case series. PARTICIPANTS: Three hundred one patients. METHODS: Review of clinical records and color fundus photographs. MAIN OUTCOME MEASURES: Photographic and clinical interpretation of optic nerve appearance. RESULTS: Ninety-two patients with AAION and 102 with NAION were included in the study. Disc photographs of 42 patients (48%) with AAION and 32 patients (31%) with NAION were available for reassessment. These were presented in a masked fashion along with a random sample of 27 disc pairs considered to be 'normal' and 27 disc pairs with 'established glaucoma' according to two examiners. Cupping was present in 92% of eyes with AAION secondary to giant cell arteritis and in 2% of eyes with NAION (kappa = 0.96; P < 0.001). CONCLUSIONS: The end-stage optic disc appearance in AAION secondary to giant cell arteritis is cupping, whereas segmental or diffuse pallor without cupping is the typical disc appearance after NAION.

Intraocular pressure changes after treatment for Graves' orbitopathy.

OBJECTIVE: To evaluate the change in intraocular pressure (IOP) in subjects with Graves' orbitopathy (GO) after orbital decompression, strabismus surgery, and orbital radiation. DESIGN: Retrospective case review. METHODS: The charts of 172 consecutive subjects from the Neuro-ophthalmology Service at Wills Eye Hospital (Philadelphia, PA) with GO who underwent either orbital decompression, strabismus surgery, or orbital radiation between 1994 and 1999 were analyzed. Subject age, gender, diagnosis of glaucoma in either eye, use of systemic steroids or topical glaucoma medications, procedure performed, and the preoperative and postoperative IOP (in primary position and upgaze) were evaluated. RESULTS: Of 116 eyes that underwent orbital decompression, the mean preoperative IOP was 21.6+/-4.6 mmHg (standard deviation) in primary position and 27.9+/-6.8 mmHg in upgaze. The postoperative IOP was 17.5 mmHg +/- 3.0 mmHg in primary position and 20.1+/-4.7 mmHg in upgaze, a decrease in IOP of 18.9% in primary position and 27.9% in upgaze (P<0.001). Subjects taking glaucoma medication or who had IOP greater than 21 mmHg demonstrated a significantly (P<0.001) greater reduction in IOP postoperatively. The mean preoperative IOP in the 32 subjects who had strabismus surgery was 18.5+/-2.8 mmHg (primary position), and 24.7+/-4.3 mmHg (upgaze). Postoperative IOP was 16.1 mmHg (primary position) and 16.9 mmHg (upgaze), a decrease of 2.4 mmHg (13.3%, P<0.01 in primary position) and 7.8 mmHg (31.2%, P<0.01 in upgaze). There was no statistically significant reduction in IOP after orbital radiation. CONCLUSIONS: In the selected subgroup of subjects with GO who required intervention, orbital decompression and strabismus surgery resulted in a significant reduction in IOP in the early postoperative period, especially in subjects with preoperative IOP greater than 21 mmHg.

Gaze-evoked amaurosis produced by intraorbital buckshot pellet.

OBJECTIVE: To report the first case of gaze-evoked amaurosis secondary to an intraocular foreign body and to highlight the characteristic clinical findings of patients with this symptom. DESIGN: Case report and review of the literature. METHODS: Case review, clinical history, electrophysiologic testing, and follow-up. MAIN OUTCOME MEASURES: Visual acuity, automated perimetry, and visual fields. RESULTS: A case of gaze-evoked amaurosis as a result of an intraorbital foreign body is described, and 19 additional cases of gaze-evoked amaurosis are reviewed from the English language literature. These cases share certain characteristics including good vision in primary position with deterioration of vision in eccentric gaze; concurrent objective pupillary abnormalities in eccentric gaze; stereotypic onset and recovery of vision; and funduscopic abnormalities consisting of disc edema and chorioretinal folds. CONCLUSIONS: Gaze-evoked amaurosis is a reliable sign of intraconal mass lesion. We report the first case of gaze-evoked amaurosis secondary to an intraorbital foreign body.

Visual loss in pregnancy.

A 36-year-old preeclamptic woman presented with bilateral visual loss upon recovery from anesthesia following cesarean section. Visual acuity was hand motion OU. Pupillary responses to light were normal, without a relative afferent pupillary defect. Treatment consisted of control of blood pressure and the patient's vision improved to 20/25 OU within 3 days.

The ice test versus the rest test in myasthenia gravis.

OBJECTIVE: To compare the ice test with the rest test in subjects with myasthenic and nonmyasthenic ptosis. DESIGN: Randomized, noninterventional trial. PARTICIPANTS: (1) Ten subjects with ptosis from previously undiagnosed myasthenia gravis. (2) Fifteen subjects with nonmyasthenic ptosis. METHODS: Application of ice compared with rest. MAIN OUTCOME MEASURES: Improvement in eyelid elevation in millimeters after the application of a surgical glove filled with ice or cotton. RESULTS: In myasthenic subjects, the median improvement of ptosis with the rest test was 2 mm and with the ice test was 4.5 mm. The difference between the rest and ice tests is significant (P: < 0.001). There was no improvement in ptosis in nonmyasthenic subjects with either test. CONCLUSION: In myasthenic ptosis, improvement in eyelid elevation after the ice test is in part caused by rest. The ice test significantly improves ptosis more than rest alone does.

Low diagnostic yield with second biopsies in suspected giant cell arteritis.

OBJECTIVES: The clinical diagnosis of giant cell arteritis may be confirmed with a biopsy of the superficial temporal artery. Because of "skip lesions," a histologic diagnosis of giant cell arteritis may be missed with a unilateral biopsy. The authors report a study that investigates whether a biopsy of the contralateral superficial temporal artery provides any additional information for confirmation of a diagnosis of giant cell arteritis. METHODS: Available medical records of 91 consecutive patients who underwent bilateral superficial temporal artery biopsy procedures were reviewed. Information that was abstracted included sequence of biopsy procedures, length specimens, and histologic diagnosis. Microslides from all biopsy specimens were retrieved and reexamined in a masked fashion by the ocular pathologist (RCE) who had made the original diagnoses. RESULTS: Seventy-two bilateral simultaneous superficial temporal artery biopsies and 19 bilateral sequential biopsies were performed. The mean length of biopsy specimens was 23 mm, and the mean length of the total artery removed from each patient was 33 mm. The pathologist's original diagnosis and the diagnosis at reexamination were in 100% agreement. In 90 (99%) of the 91 patients, the histologic diagnoses in the left and right superficial temporal arteries were the same. This is a concordance rate of 98.9% (38 of 39 positive biopsy results) among the positive biopsy results. CONCLUSION: There is a low yield of information from a second temporal artery biopsy in patients with suspected giant cell arteritis. This suggests that patients who present to the ophthalmologist with possible giant cell arteritis will, in most cases, have a similar diagnosis on both temporal artery biopsies if the specimens are adequate.

Withdrawal from a donor insemination programme.

The aim of this study was to examine the factors that influenced the withdrawal of couples from a regional donor insemination programme. Demographic, medical, treatment and outcome data were prospectively recorded on 375 referrals. One hundred and eight women had conceived at least 1 normal pregnancy. Withdrawals from the programme, without pregnancy, accounted for 165 couples, whilst 47 had begun treatment, and a further 29 were on the waiting list. Thirty nine (24%) of those withdrawing did so without notice or reason. Most of these (33) were in couples waiting to begin treatment. Natural conception, adoption, identifiable medical or social reasons (including separation) and moving to another region were the predominant causes for withdrawing. Women who had entered the programme because of their partner's oligospermia had a high natural conception rate. Age, socioeconomic status, geographic location and length of infertility did not affect the withdrawal rate.