Spontaneous Skull Base Cerebrospinal Fluid Leaks and Their Relationship to Idiopathic Intracranial Hypertension.

BACKGROUND: The association between spontaneous skull base cerebrospinal fluid (CSF) leaks and idiopathic intracranial hypertension (IIH) has been suggested, but its significance remains unclear. OBJECTIVE: To estimate the prevalence of IIH in spontaneous skull base CSF leak patients. METHODS: Systematic collection of demographics, neuro-ophthalmic and magnetic resonance imaging evaluation of spontaneous skull base CSF leak patients seen pre- and post-leak repair in one neuro-ophthalmology service. Patients with preexisting IIH were diagnosed with definite IIH if adequate documentation was provided; otherwise, they were categorized with presumed IIH. Classic radiographic signs of intracranial hypertension and bilateral transverse venous sinus stenosis were recorded. RESULTS: Thirty six patients were included (age [interquartile range]: 50 [45;54] years; 94% women; body mass index: 36.8 [30.5;39.9] kg/m2), among whom six (16.7%, [95% confidence interval, CI]: [6.4;32.8]) had a preexisting diagnosis of definite or presumed IIH. Of the remaining 30 patients, four (13.3%, 95%CI: [3.8;30.7]) had optic nerve head changes suggesting previously undiagnosed IIH, while one was newly diagnosed with definite IIH at initial consultation. One out of 29 patients with normal findings of the optic nerve head at presentation developed new onset papilledema following surgery (3.4%, 95%CI: [0.1;17.8]) and was ultimately diagnosed with definite IIH. Overall, the prevalence of definite IIH was 19.4% (95%CI: [8.2;36.0]). CONCLUSION: Striking demographic overlap exists between IIH patients and those with spontaneous CSF leak. Definite IIH was present in approximately 20% of our patients. However, its true prevalence is likely higher than identified by using classic criteria. We therefore hypothesize that an active CSF leak serves as an auto-diversion for CSF, thereby "treating" the intracranial hypertension and eliminating characteristic signs and symptoms at initial presentation.

20/40 or Better Visual Acuity After Optic Neuritis: Not as Good as We Once Thought?

BACKGROUND: Although patients with acute optic neuritis (ON) recover high-contrast visual acuity (HCVA) to 20/40 or better in 95% of affected eyes, patients with a history of ON continue to note subjective abnormalities of vision. Furthermore, substantial and permanent thinning of the retinal nerve fiber layer (RNFL) and the ganglion cell layer (GCL) is now known to occur early in the course of ON. We measured vision-specific quality of life (QOL) in patients with a history of acute ON and recovery of VA to 20/40 or better in their affected eyes to determine how these QOL scores relate to RNFL and GCL thickness and low-contrast letter acuity (LCLA) across the spectrum of visual recovery. METHODS: Data from an ongoing collaborative study of visual outcomes in multiple sclerosis and ON were analyzed for this cross-sectional observational cohort. Patients and disease-free control participants completed the 25-Item National Eye Institute Visual Functioning Questionnaire (NEI-VFQ-25) and 10-Item Neuro-Ophthalmic Supplement to the NEI-VFQ-25, as well as VA and LCLA testing for each eye separately and binocularly. Optical coherence tomography measures for each eye included peripapillary RNFL thickness and macular GCL + inner plexiform layer (GCL + IPL) thickness. RESULTS: Patients with a history of acute ON and recovery to 20/40 or better VA (n = 113) had significantly reduced scores for the NEI-VFQ-25 (83.7 ± 15.4) and 10-Item Neuro-Ophthalmic Supplement (74.6 ± 17.4) compared with disease-free controls (98.2 ± 2.1 and 96.4 ± 5.2, P < 0.001, linear regression models, accounting for age and within-patient, intereye correlations). Most patients with 20/40 or better visual recovery (98/112, 88%) had monocular HCVA in their affected eye of 20/20 or better. Although patients with 20/50 or worse HCVA recovery demonstrated the worst performance on low-contrast acuity, affected eye RNFL and GCL + IPL thickness, and QOL scales, these measures were also significantly reduced among those with 20/40 or better HCVA recovery compared with controls. CONCLUSIONS: Patients with a history of ON and "good" visual recovery, defined in the literature as 20/40 or better HCVA, are left with clinically meaningful reductions in vision-specific QOL. Such patient-observed deficits reflect the underlying significant degrees of retinal axonal and neuronal loss and visual dysfunction that are now known to characterize ON even in the setting of maximal HCVA recovery. There remains an unmet therapeutic need for patients with ON.

Utility of optical coherence tomography in the evaluation of monocular visual loss related to retinal ischemia.

We report four patients with monocular visual loss for whom optical coherence tomography (OCT) was helpful in distinguishing the sequelae of retinal artery occlusion from those of primary optic neuropathy. Determinations of the peripapillary retinal nerve fiber layer (RNFL) thickness as well as macular retinal layer thicknesses and architecture were used. The major findings in our patients show that changes in the inner retinal layers (including ganglion cell and inner plexiform layer) with disruption of normal macular architecture supports a diagnosis of retinal artery occlusion. Our results support the use of OCT imaging for patients with monocular visual loss of uncertain etiology; macular imaging as well as peripapillary RNFL thickness measurement can be helpful in differentiating primary retinal disease or ischemia from primary disorders of the optic nerve.

Optical Coherence Tomography for the Neurologist.

Optical coherence tomography (OCT) is a relatively new technology that is now routinely and very widely used by ophthalmologists for structural documentation of the optic nerve and retina. In neuro-ophthalmology and neurology, the value of OCT is ever expanding; its role in an increasing number of conditions is being reported in parallel with the advances of the technology. Currently, as a clinical tool, OCT is particularly useful for the structural measurement of peripapillary retinal nerve fiber layer thickness, optic nerve head volumetric analysis, and macular anatomy. Optic neuropathies of varied etiology (particularly from multiple sclerosis) may be the most common clinical indications for neurologists to obtain OCT imaging. Documentation and follow-up of disc edema of varied etiology (papilledema and idiopathic intracranial hypertension), discriminating true disc swelling from pseudopapilledema, and differentiating optic neuropathy from maculopathy are some other examples from clinical practice.

In vivo confocal microscopy of keratic precipitates in infectious versus noninfectious uveitis.

PURPOSE: To test the hypothesis that morphologic patterns of keratic precipitates (KPs) evaluated by in vivo confocal microscopy (IVCM) can differentiate infectious from noninfectious uveitis. DESIGN: Cross-sectional, observational case series. PARTICIPANTS: Sixty-eight eyes of 53 subjects with uveitis. METHODS: A cross-sectional study was performed in patients with infectious and noninfectious uveitis presenting to a tertiary care eye hospital. Detailed ophthalmologic evaluation was performed in all the subjects. Keratic precipitates were studied by IVCM using the HRT II Rostock corneal module (Heidelberg Engineering GmbH, Heidelberg, Germany) and categorized on the basis of morphologic patterns. MAIN OUTCOME MEASURES: Morphology of KPs by slit-lamp biomicroscopy and confocal microscopy. RESULTS: The age of patients ranged from 15 to 87 years (median 40 years). Thirty-two patients were male (60.37%). Thirty-eight subjects had a unilateral presentation (71.69%) of uveitis. Infectious uveitis was seen in 38 cases (71.69%). The characteristics in KPs as seen in infectious uveitis were dendritic, central globular with dendritic, and infiltrative. In noninfectious uveitis (28.3%), stippled, globular, and multiple globular types of KPs were found. The sensitivity, specificity, and positive predictive value for specific combinations of KPs with an infectious cause were 84.21%, 93.33%, and 96.96%, respectively. CONCLUSIONS: In vivo confocal microscopy can act as an adjunct tool for differentiating infectious from noninfectious uveitis. A central globular with dendritic form of KPs is strongly suggestive of infectious uveitis.

Safety and efficacy of Intacs in Indian eyes with keratoconus: an initial report.

CONTEXT: In contact lens-intolerant keratoconus patients, intrastromal placement of Intacs is becoming a promising new modality of treatment. AIMS: To study the safety and efficacy of implantation of microthin corneal inserts (Intacs) in Asian-Indian keratoconus patients. SETTINGS AND DESIGN: Retrospective interventional case series, in the cornea and refractive surgery service, at a tertiary care eye hospital in South India, between May 2006 and July 2007. MATERIALS AND METHODS: Intacs (Addition Technology, USA) were successfully implanted by mechanically creating tunnels, in 12 eyes of 12 keratoconus patients, who were contact lens-intolerant. The patients (mean age 25.58 years, nine male and three female) had a minimum follow-up of six months. Five patients had severe keratoconus (mean K > 53 D) and nine had central cones. RESULTS: All patients improved or retained visual acuity. The uncorrected visual acuity (UCVA) improved from a mean value of 0.06, (SD +/- 0.08) to 0.19 postoperatively (SD+/- 0.15) ( P = 0.01). The change in mean best corrected visual acuity (BCVA) was from 0.51, (SD +/- 0.24), to 0.69, (SD +/-0.00) ( P = 0.01) postoperatively. The average central keratometry reading was 52.55 D. The change in mean K from 52.84 to 49.16 and 49.15 at one and six months respectively, was statistically significant. We did not have any major intra- or early postoperative complications. Eight of 12 eyes became contact lens-tolerant post-surgery. CONCLUSION: The procedure of Intacs implantation appears to be safe and effective in a small group of Indian population at an intermediate follow-up.

Intacs in advanced keratoconus.

PURPOSE: To evaluate the surgical outcomes of microthin intracorneal ring segment (INTACS) implantation in advanced keratoconus. METHODS: INTACS implantation was performed in eyes with advanced keratoconus. The main outcome measures were uncorrected visual acuity, best spectacle-corrected visual acuity, change in mean refractive spherical equivalent (MRSE), and keratometry. RESULTS: Intacs were implanted in 14 eyes with advanced keratoconus. At 6 months, uncorrected visual acuity improved from 0.05 +/- 0.08 to 0.16 +/- 0.11 (P < 0.05), and best spectacle-corrected visual acuity improved from 0.50 +/- 0.23 to 0.67 +/- 0.00 (P = 0.01). The spherical refractive error improved from -6.68 D +/- 6.44 to -3.11 D +/- 3.08 (P = 0.03), whereas the cylindrical refractive error improved from -4.89 D +/- 1.91 to -3.64 D +/- 1.27 (P = 0.04). The MRSE reduced from -9.13 D +/- 5.62 to -4.93 D +/- 3.19 (P = 0.01), and the average keratometry decreased from 53.01 D +/- 3.70 to 49.42 D +/- 3.79 (P < 0.05). The results were stable from 6 months to 1 year. The procedure showed 100% safety, and more than 60% tolerated contact lenses. Younger age, male sex, and minimum central pachymetry of more than 400 microm seemed to be associated with better outcomes. CONCLUSIONS: In this series, Intacs provided good results with respect to visual acuity, corneal topography, and MRSE in eyes with advanced keratoconus without major complications or the need for segment explanation. INTACS is potentially a safe and efficacious treatment option in the management of advanced keratoconus.

Electrophysiological and structural assessment of the central retina following intravitreal injection of bevacizumab for treatment of macular edema.

PURPOSE: To evaluate with electrophysiological responses and Optical Coherence Tomography (OCT), the short term functional and structural effects at the macula following intravitreal injection of bevacizumab for macular edema. METHODS: Prospective, non-randomized, interventional case study. In total, 17 eyes of 17 patients with macular edema due to vein occlusions and diabetic retinopathy received intravitreal bevacizumab. All Patients underwent complete ophthalmic examination including Snellen visual acuity testing, Multifocal Electroretinography (mfERG) and Full Field Electroretinography (FERG), OCT scanning at baseline at 1 week and 2 months after intravitreal bevacizumab. RESULTS: FERG did not show any change in waveform parameters following intravitreal injection of bevacizumab. Average mfERG macular responses within central 20 degrees showed increased P(1) amplitude (P < 0.05) at 2 months after treatment as compared to the baseline recordings in all subjects. No changes were seen in the implicit time. There was 22% improvement in central retinal thickness (CRT) at 2 months compared to the baseline (P < 001). CONCLUSION: Intravitreal injection bevacizumab resulted in reduction in the central retinal thickness and mild to moderate improvement in the mfERG amplitudes in this short-term study. The visual acuity changes did not directly correlate with the reduced central retinal thickness or improvement in mfERG. The short-term results showed no serious ocular adverse effects. Therefore on short-term follow up the off label drug showed improvement of macular edema secondary to vein occlusion and diabetic retinopathy with no demonstrable toxic effects.

Ocular manifestations associated with chikungunya.

PURPOSE: To report ocular manifestations associated with chikungunya. DESIGN: Retrospective, nonrandomized, observational case series. PARTICIPANTS: Nine chikungunya patients with ocular involvement. METHODS: All patients with chikungunya infection presenting with ocular complaints from September 2006 to October 2006 were included in the study. The infection was confirmed by demonstration of chikungunya immunoglobulin M antibody in sera of all patients. All patients underwent an ophthalmic examination including fundus photography, fundus fluorescein angiography, confocal microscopy of keratic precipitates, and optical coherence tomography. Positive ocular changes were recorded and tabulated. MAIN OUTCOME MEASURES: Characteristics, frequency, and locations of ocular lesions found in the participants. RESULTS: There were 9 patients with ocular lesions; 1 had nodular episcleritis, 5 presented with acute iridocyclitis, and 3 had retinitis. Four to 12 weeks before the development of ocular manifestations, all of these patients had fever. Although there were no specific changes of iridocyclitis that were diagnostic of the fever, the retinal changes were consistent with viral retinitis. All patients recovered from the infection with relatively good vision. CONCLUSION: It appears that iridocyclitis and retinitis are the most common ocular manifestations associated with chikungunya, with a typically benign clinical course. Less frequent ocular lesions include episcleritis. All the patients responded well to the treatment with preservation of good vision. To the best of our knowledge, similar ocular manifestations associated with chikungunya infection have not been reported. In the differential diagnosis of iridocyclitis and retinitis with features suggestive of a viral infection, the entity of chikungunya-associated ocular changes should be considered in the regions affected by the epidemic.

Giant sarcoid tumor of the iris and ciliary body.

PURPOSE: To report the occurrence of a giant iridociliary sarcoid tumor. METHODS: The patient was evaluated by medical history, ophthalmoscopic examination (including photography and ultrasonography) as well as systemic, hematologic, and radiographic examinations. Tumor biopsies allowed for cytopathologic, histopathologic, and immunohistochemical analysis. RESULTS: The 39-year-old black male was found to have a right iris and ciliary body tumor. Ultrasonography revealed a 10 x 12-mm base, 5.6-mm height, low internal reflectivity, and vitreous debris. Radiographic imaging revealed mediastinal and bilateral hilar lymphadenopathy. A purified protein derivative (PPD) and a hematologic survey were negative. Pathology evaluations of the surgical specimens revealed features of non-caseating granulomata consistent with sarcoidosis. A combination of topical and systemic steroid therapy was locally curative. CONCLUSIONS: We describe a giant iridociliary sarcoid tumor in a patient with no lacrimal gland enlargement, conjunctival nodules, or skin lesions. A biopsy was required to establish the diagnosis.

Clinical, anatomic, and electrophysiologic evaluation following intravitreal bevacizumab for macular edema in retinal vein occlusion.

PURPOSE: To investigate clinical, anatomic, and electrophysiologic response after single intravitreal injection of bevacizumab for macular edema attributable to retinal vein occlusion. DESIGN: Prospective nonrandomized, interventional case series. METHODS: Twenty-one patients with macular edema attributable to vein occlusion received intravitreal injection of bevacizumab 1.25 mg. Nine patients had central retinal vein occlusion (CRVO), and 12 patients had branch retinal vein occlusion (BRVO). Complete ophthalmic examination including optical coherence tomography (OCT) was done at baseline and follow-up visits. Fifteen patients underwent fluorescein angiography at baseline. Selected patients underwent electroretinography (ERG) and visual evoked potential (VEP) at baseline and follow-up. Follow-up was for 12 weeks. RESULTS: At baseline, mean visual acuity was 20/381 (median, 20/400) and showed improvement to mean 20/135 (median, 20/60) after one month, (P = .001). At 12 weeks, mean visual acuity was 20/178 (median, 20/80) (P = .001). The mean central retinal thickness (CRT) was 647.81 microm (median, 609.00 microm) at baseline and decreased to mean 293.43 microm (median, 222.00 microm) at one month (P = .001). At 12 weeks, mean CRT was 320.90 mum (median, 280.00 microm) (P = .001). ERG and VEP showed no worsening of the waveforms. There was no significant difference in the visual outcome between the BRVO and CRVO groups. CONCLUSION: Intravitreal injection of bevacizumab appears to result in significant short-term improvement of visual acuity and macular edema secondary to vein occlusion. The present report confirms the previous studies. No ocular toxicity or adverse effects were observed. However, prospective, randomized, controlled long-term studies are required with an adequate number of patients.

Fuchs' heterochromic iridocyclitis following bilateral ocular toxoplasmosis.

PURPOSE: To describe the development of Fuchs' heterochromic iridocyclitis (FHI) following bilateral ocular toxoplasmosis in an Asian Indian female. METHODS: Case report of a patient with bilateral ocular toxoplasmosis who developed bilateral Fuchs' heterochromic iridocyclitis. RESULTS: Features characteristic of bilateral Fuchs' heterochromic iridocyclitis developed following several attacks of bilateral toxoplasmic retinochoroiditis in a 26-year-old Indian female patient. CONCLUSION: Fuchs' heterochromic iridocyclitis can develop over a period of time in patients with ocular toxoplasmosis.

Patterns of uveitis in children presenting at a tertiary eye care centre in south India.

PURPOSE: To study the patterns of uveitis in the paediatric age group in a referral eye care centre in south India. MATERIALS AND METHODS: Thirty-one patients 15 years or younger with uveitis, examined in the year 2000, were included in this study. The uveitis was classified according to the anatomical site of ocular involvement and the most probable aetiological factor. The final diagnosis was based on clinical manifestations and results of specific laboratory investigations. RESULTS: A total 31 (6.29%) paediatric uveitis cases were seen among the 493 uveitic cases in the year 2000. The male:female ratio was 17:14. Anterior (9 cases), intermediate (9 cases) and posterior uveitis (9 cases) were seen in equal number. Four patients had panuveitis. Twenty-seven patients had visual acuity of 6/36 or better at presentation. Approximately 25% (8 of 31) patients had cataract secondary to inflammation. Immunosuppressives were administered in 4 patients and one patient required cataract surgery. CONCLUSION: Uveitis in children comprises approximately 6% of uveitis cases in a referral practice in south India. Anterior, intermediate and posterior uveitis are seen in equal numbers. We recommend that intermediate uveitis be ruled out in all cases of anterior uveitis by careful clinical evaluation including examination under anesthesia (EUA) when required.

Peripapillary choroidal atrophy in sympathetic ophthalmia and management with triple-agent immunosuppression.

PURPOSE: To describe the occurrence of peripapillary choroidal atrophy and experience with triple-agent immunosuppression in three cases of sympathetic ophthalmia. MATERIALS AND METHODS: Retrospective chart review of three cases of sympathetic ophthalmia with peripapillary choroidal atrophy. RESULTS: Three patients with sympathetic ophthalmia were managed with steroid pulse therapy and triple-agent immunosuppression. All three patients subsequently developed peripapillary choroidal atrophy. CONCLUSIONS: Peripapillary choroidal atrophy can occur in sympathetic ophthalmia and may indicate a severe form of inflammation which would benefit from triple-agent immunosuppression.