Simon R. Bababeygy

Long-term outcomes of Group D eyes in bilateral retinoblastoma patients treated with chemoreduction and low-dose IMRT salvage.

To evaluate outcomes of Group D eyes of bilateral retinoblastoma patients treated with primary chemoreduction and external beam radiation as salvage.

Functional and visual improvement with prosthetic replacement of the ocular surface ecosystem scleral lenses for irregular corneas.

Purpose: To evaluate the Doheny Eye Institute Experience with Prosthetic Replacement of the Ocular Surface Ecosystem (PROSE) scleral lenses for the management of irregular corneas with outcomes based on visual acuity (VA) and visual function. Methods: A retrospective chart review of 58 subjects (90 eyes) with irregular corneal surfaces referred to the Doheny Eye Institute for PROSE treatment between July 2009 and December 2011 was performed. The best-corrected VA before and after PROSE fitting was recorded. A functional assessment before and after PROSE fitting was also performed using the Ocular Surface Disease Index, a 12-item questionnaire that grades the severity of ocular discomfort and vision-related function. Results: Keratoconus (43%) represented the largest group, and post-PK astigmatism (31%) represented the second largest group of patients with irregular corneas who had completed the PROSE treatment. Patients with keratoconus had the greatest improvement in VA after PROSE fitting with an 88% improvement in the logarithm of the minimal angle of resolution vision. Patients with post-PK astigmatism had the greatest improvement in Ocular Surface Disease Index scores with a 79% improvement observed after PROSE fitting. Conclusions: PROSE scleral lenses offer improvements in the VA and function, and they could be an option for patients with irregular corneas who have failed conventional treatments before considering additional surgery.

Distinguishing wet from dry age-related macular degeneration using three-dimensional computer-automated threshold Amsler grid testing

Background/aims With the increased efficacy of current therapy for wet age-related macular degeneration (AMD), better ways to detect wet AMD are needed. This study was designed to test the ability of three-dimensional contrast threshold Amsler grid (3D-CTAG) testing to distinguish wet AMD from dry AMD. Methods Conventional paper Amsler grid and 3D-CTAG tests were performed in 90 eyes: 63 with AMD (34 dry, 29 wet) and 27 controls. Qualitative comparisons were based upon the three-dimensional shapes of central visual field (VF) defects. Quantitative analyses considered the number and volume of the three-dimensional defects. Results 25/34 (74%) dry AMD and 6/29 (21%) wet AMD eyes had no distortions on paper Amsler grid. Of these, 5/25 (20%) dry and 6/6 (100%) wet (p=0.03) AMD eyes exhibited central VF defects with 3D-CTAG. Wet AMD displayed stepped defects in 16/28 (57%) eyes, compared with only 2/34 (6%) of dry AMD eyes (p=0.002). All three volumetric indices of VF defects were two- to four-fold greater in wet than dry AMD (p<0.006). 3D-CTAG had 83.9% positive and 90.6% negative predictive values for wet AMD. Conclusions 3D-CTAG has a higher likelihood of detecting central VF defects than conventional Amsler grid, especially in wet AMD. Wet AMD can be distinguished from dry AMD by qualitative and quantitative 3D-CTAG criteria. Thus, 3D-CTAG may be useful in screening for wet AMD, quantitating disease severity, and providing a quantitative outcome measure of therapy.

The utility of routine tuberculosis screening in county hospital patients with uveitis

Background/aim To evaluate the utility of tuberculosis (TB) screening in diagnosing ocular TB in uveitis patients in a government-funded hospital. Methods The charts of 142 consecutive patients seen during August 2011–July 2012 at the Los Angeles County Hospital uveitis clinic were reviewed for manifestation/laterality of uveitis, purified protein derivative (PPD) test results, interferon γ release assay, chest x-ray, birthplace, treatment history and diagnosis. ‘Presumed TB-uveitis’ was diagnosed when patients had positive TB screening and favourable response to anti-TB therapy, and definite ocular TB when Mycobacterium tuberculosis’ presence was demonstrated. Post-test probabilities were determined. Results TB screening was positive in 21.1%. Six patients were diagnosed with TB-related uveitis: one definite, four presumed and one systemic TB with uveitis. With regard to PPD positivity, being foreign-born was the only statistically significant factor with OR of 2.26 (95% CI 1.01 to 5.13; p<0.01) if born in Mexico and 4.90 (95% CI 1.74 to 13.83; p<0.01) if born in other foreign countries. The post-test probabilities of a positive PPD in a uveitis patient showed a 17.2% (overall) or 30.3% (foreign-born patients) chance of ocular TB. Conclusions PPD skin test plays an important role in the diagnosis of TB-associated uveitis in high-risk groups, such as immigrants from TB endemic regions.

Visual improvement with the use of idebenone in the treatment of Wolfram syndrome.

R ecently in the Journal of Neuro-Ophthalmology, we discussed the potential efficacy of idebenone, a coenzyme Q derivative that acts as a carrier in the mitochondrial electron transport chain, in treating patients with Leber hereditary optic neuropathy (LHON) (1). Within the past 2 decades, Wolfram syndrome (WS) has been mapped to chromosome 4p16.1 (2) and is thought to harbor a mitochondrial genome deletion (3,4) or complex III deficiency (5). The only current treatment for this devastating disease is limited to blood sugar control with lower doses of insulin compared with patients with diabetes mellitus (DM). We had the opportunity to treat a patient with WS with idebenone, hoping for improvement in visual function. A 21-year-old Romanian man was initially evaluated 5 years previously with progressive bilateral visual and hearing loss and a neurogenic bladder. Surgical history was significant for bilateral cataract extractions with intraocular lens implants at 11 years of age. Medications included insulin and effexor. Family history was significant for DM in both parents, without a history of hearing or visual loss. Genetic testing confirmed the diagnosis of WS with the mutation of the WFS1 Wolframin gene. The patient was overweight but appeared younger than his stated age. Visual acuity was light perception to bare hand motion bilaterally. Pupils were sluggishly reactive without a relative afferent pupillary defect. Intraocular pressures were normal and funduscopy revealed bilateral optic disc pallor (Fig. 1). Spectral domain optical coherence tomography (OCT) showed marked thinning of the retinal nerve fiber layer in each eye (Fig. 2). The patient had bilateral sensorineural hearing loss of higher frequency sounds with preservation of hearing lower frequencies. He was prescribed idebenone that was gradually increased from 150 mg daily to 150 mg twice a day at 2 months, and then to 150 mg 3 times a day at 4 months. At 3 months, the patient’s vision improved to hand motions at 1 foot bilaterally. At 6 months, visual acuity was hand motions at 2 feet, right eye, and hand motions at 4 feet, left eye. Ophthalmoscopic and OCT findings remained unchanged. Our patient had difficulty with standard kinetic visual field testing given his poor vision (Fig. 3). To enhance light intensity of the stimulus, we used green and red laser stimuli rather than white. We were able to quantitate the visual field using a kinetic technique with these colored stimuli (Fig. 2). The patient could see the brighter more intense green stimulus, with some false positives with the red stimulus, whereas he previously was unable to detect any such stimulus on visual field testing. Recent publications describe the use of idebenone, a coenzyme Q10 derivative, in the treatment of LHON (1,7,8). Given that WS has features of mitochondrial dysfunction, we decided to initiate idebenone treatment. In our patient, idebenone resulted in progressive but subjective visual recovery at 6 months. Similarly, in reports of response to idebenone in LHON, the effects usually do not begin until after 6 months (1,7). This long-term

Isolated abducens nerve palsy secondary to Lemierre syndrome

Lemierre syndrome is characterized by pharyngotonsillitis that is typically secondary to Fusobacterium necrophorum infection and causes adjacent septic thrombophlebitis and thrombosis with subsequent metastatic abscesses. A 3-year-old boy presented with fever, tonsillar enlargement, and bilateral otomastoiditis with purulent discharge. Physical examination revealed abducens nerve palsy on the left side, with a compensatory left head turn. Otomastoid discharge culture was positive for anaerobic F. necrophorum. Magnetic resonance venography of the head and neck revealed thromboses in left internal jugular vein, left cavernous sinus, left superior ophthalmic vein, and left sigmoid sinus. The patient was treated with anticoagulation and a 10-week course of intravenous antibiotics, including ceftriaxone and metronidazole.

Angioarchitectural Evolution of Clival Dural Arteriovenous Fistulas in Two Patients

Dural arteriovenous fistulas (dAVFs) may present in a variety of ways, including as carotid-cavernous sinus fistulas. The ophthalmologic sequelae of carotid-cavernous sinus fistulas are known and recognizable, but less commonly seen is the rare clival fistula. Clival dAVFs may have a variety of potential anatomical configurations but are defined by the involvement of the venous plexus just overlying the bony clivus. Here we present two cases of clival dAVFs that most likely evolved from carotid-cavernous sinus fistulas.

Are melanopsin cells blocked by filtering IOLs

A recent article by Artigas et al. [1] shed light on the role and potential benefit of wavelength-filtering intraocular lenses (IOLs). Previously, numerous authors expressed concerns that these IOLs may cause adverse effects on scotopic vision performance and possible sleep disturbances related to differences in circadian rhythm [2–8]. Although it may be implicated that ultraviolet (UV) and potentially blue light may cause or speed up progression of age-related macular degeneration [3], it is important to note the potential adverse effects that filtering blue light with IOLs could have on patients. Previously, it was referenced that blocking blue light may ultimately disrupt the human body’s circadian rhythm, predisposing the patient to increased morbidity and decreased longevity [9]. Subsequently, a thorough article published by Davison et al. [10] summarized the results of several clinical and computational studies on photoreception and photoprotection, addressing some of the aforementioned issues with these IOLs. However, although these IOL filters discussed may be targeted to filter around 400 nm [1], or use an action spectra for photoentrainment with a peak of 460 nm [3, 7, 11, 12], some of these filters appear to block visible radiation between 400 and 500 nm. Hence, another caveat to this application is the potential suppression of melanopsin-expressing intrinsically photosensitive retinal ganglion cells (mRGC), a small minority of retinal ganglion cells that have been shown to be functional photoreceptors [13]. These cells exhibit sustained excitatory light responses that are responsible for non-image-forming visual functions such as the papillary light reflex and circadian rhythms. Recent animal work shows that mRGC have peak spectral sensitivity at 478 nm [13], and therefore would be likely suppressed with the use of filtering IOLs. Furthermore, more recent human work shows that mRGC are lost with age, are more represented in the parafoveal region, and are implicated to have an important role for circadian rhythm [14]. Landers et al. [15] previously assessed whether filtering IOLs may cause sleep disturbance related to circadian rhythm in a comparative retrospective sleep study using a subjective questionnaire model and found that there is no significance between patients with blue-light-filtering IOLs and conventional IOLs. However, caveats to the study include a small sample size and the lack of objective polysomnography sleep quality test evidence. It is important to keep in mind that, as we age, there is a decrease in the amount of remaining mRGC. It would behoove us to maintain the maximum potential use of these cells. Are we doing harm by blocking S. R. Bababeygy A. A. Sadun (&) Department of Ophthalmology, Doheny Eye Institute, Keck School of Medicine, University of Southern California, 1450 San Pablo St. 5802, Los Angeles, CA 90033, USA e-mail: asadun@usc.edu