Courtney L. Kraus

Retinal pigment epithelial tears in ranibizumab-treated eyes.

Purpose: To report the incidence of retinal pigment epithelial (RPE) tears in patients treated with ranibizumab therapy for choroidal neovascularization due to age-related macular degeneration. Design: Interventional case series. Methods: One hundred sixty-four eyes from a large clinical practice were retrospectively reviewed for number of injections and the presence or absence of a fibrovascular retinal pigment epithelial detachment. Main outcome measures were occurrence of RPE tears, and timing of tears following the last injection. Results: Patients were observed for an average of 11 months. A single patient (0.61%) experienced an RPE tear and this occurred after the first injection. In eyes with a fibrovascular retinal pigment epithelial detachment the incidence was 5%. Lesions containing a fibrovascular retinal pigment epithelial detachment tended to be larger (4.5 versus 3.8 Macular Photocoagulation Study Disc Areas), but this was not statistically significant. (P = 0.63). However, these lesions required more injections on average (P = 0.05). Conclusion: The incidence of RPE tears associated with ranibizumab therapy is low and may result from a predisposition rather than an effect of treatment. Even so, patients undergoing ranibizumab therapy should be counseled regarding this possible complication.

N-Acetylcysteine Amide (NACA) Prevents Retinal Degeneration by Up-Regulating Reduced Glutathione Production and Reversing Lipid Peroxidation

Oxidative stress plays a critical role in accelerating retinal pigment epithelial dysfunction and death in degenerative retinal diseases, including age-related macular degeneration. Given the key role of oxidative stress-induced retinal pigment epithelial cell death and secondary photoreceptor loss in the pathogenesis of age-related macular degeneration, we hypothesized that a novel thiol antioxidant, N-acetylcysteine amide (NACA), might ameliorate cellular damage and subsequent loss of vision. Treatment of human retinal pigment epithelial cells with NACA protected against oxidative stress-induced cellular injury and death. NACA acted mechanistically by scavenging existing reactive oxygen species while halting production of reactive oxygen species by reversing lipid peroxidation. Furthermore, NACA functioned by increasing the levels of reduced glutathione and the phase II detoxification enzyme glutathione peroxidase. Treatment of mice exposed to phototoxic doses of light with NACA maintained retinal pigment epithelial cell integrity and prevented outer nuclear layer cell death as examined by histopathologic methods and rescued photoreceptor function as measured by electroretinography. These observations indicate that NACA protects against oxidative stress-induced retinal pigment epithelial and photoreceptor cell death in vitro and in vivo. The data suggest that NACA may be a novel treatment in rescuing retinal function and preventing vision loss secondary to retinal degenerative diseases, including age-related macular degeneration.

Comparison of the effectiveness and safety of transscleral cyclophotocoagulation and endoscopic cyclophotocoagulation in pediatric glaucoma.

PURPOSE Among the options for surgical management of pediatric glaucoma, destruction of the ciliary body reduces aqueous production and, consequently, intraocular pressure (IOP). Compared to more invasive filtering and shunt procedures, cyclodestruction is an attractive option for control of IOP in pediatric glaucomas. METHODS The relative reduction in IOP, duration of effect, and comparable safety and efficacy of transscleral cyclophotocoagulation (TSCP) and endoscopic cyclophotocoagulation (ECP) in pediatric patients with glaucoma was studied in this retrospective chart review. RESULTS A reduction in IOP of 28.6% and 33.2% with TSCP and ECP, respectively, was found. Eyes treated with ECP underwent an average of 3.24 cyclodestructive procedures; eyes treated with TSCP underwent an average of 2.29 cyclodestructive treatments. These differences were not statistically significant. A final success rate of 67.6% after TSCP and 62% after ECP failed to significantly differ between the two groups. Consequently, two-thirds of the patients were able to avoid penetrating surgery and the associated risks after one or more cyclodestructive procedures. CONCLUSIONS TSCP and ECP are safe, effective, and comparable treatments for pediatric glaucomas. The results suggest that TSCP and ECP may be considered first-line therapy to achieve control of IOP in all forms of pediatric glaucoma. [J Pediatr Ophthalmol Strabismus 2014;51(2):120-127.].

Nummular keratopathy in a patient with Hyper-IgD Syndrome

PurposeTo report a case of recurrent nummular keratitis in a pediatric patient with Hyperimmunoglobulinemia D syndrome.MethodsA retrospective chart review.ResultsA 14-year-old boy with Hyperimmunoglobulinemia D syndrome (HIDS) presented with photophobia and ocular irritation concomitant with disease exacerbation. He was found on exam to have significant nummular keratitis, which responded to a short course of topical steroids. Despite acute response to local immunosuppression, the patient had several recurrent attacks and eventually developed a large corneal scar and decreased vision. After initiation of infliximab therapy his ocular sequelae improved dramatically and his vision returned to 20/20.ConclusionOne possible form of end-organ damage associated with HIDS is vision threatening nummular keratopathy.

Use of Biologic Agents in Ocular Manifestations of Rheumatic Disease

Biologic agents have dramatically shifted the treatment paradigm for rheumatic disease. Use of these agents can decrease disease burden, allow the patient to be weaned from corticosteroids, and reduce the likelihood of relapse. Eye disease associated with rheumatic conditions may present with a wide range of signs and symptoms. This coexisting pathology should not be overlooked and should be considered a reason for initiation or continuation of biologic therapy. Additionally, many of the ocular manifestations of rheumatic disease respond preferentially to specific targeting molecules. This paper summarizes the available studies on the use, efficacy, and safety of biologic agents in the treatment of ocular manifestations of rheumatic disease.

Challenging presentations of cavernous sinus thrombophlebitis

AimThe purpose of this study was to describe two challenging cases of septic cavernous sinus thrombosis (CST), which presented with vastly different clinical signs and symptoms.MethodsWe present two cases of CST with markedly differing clinical presentations, medical comorbidities, and degree of impairment. Initial imaging of each patient failed to show thromboembolic disease.ResultsBoth patients required multiple imaging procedures to arrive at the correct diagnosis. Each child did respond to treatment once the correct diagnosis was made.ConclusionCST can have a highly variable clinical presentation, from a subtle sixth nerve palsy to complete ophthalmoplegia and loss of periorbital sensation and corneal reflex. Onset of symptoms may be acute and fulminant or indolent and delayed. The diagnosis is challenging, requiring clinical suspicion and confirmation by imaging. These cases illustrate the importance of retaining clinical suspicion when cranial nerve palsies persist and how valuable rescanning a patient can be.

Clinical characteristics and surgical approach to visually significant persistent pupillary membranes

BACKGROUND Infants with hyperplastic persistent pupillary membranes (PPM) may be at risk for deprivation amblyopia due to obstructions of the visual axis. We describe the long-term visual and anatomic outcomes of a surgical technique for their removal. METHODS The medical records of consecutive patients <3 years of age who underwent surgical removal of PPMs between December 1998 and May 2012 were retrospectively reviewed. Each PPM was judged to be visually significant based on poor visual acuity, poor retinoscopic reflex, or inability to visualize the fundus. The surgical technique included injection of a viscoelastic agent beneath the pupillary strands to bow them anteriorly, careful peeling of residual adherent strands from the anterior lens capsule, and lysis of the strands at the pupillary margin with intraocular scissors. Pre- and postoperative visual and anatomic results were recorded. RESULTS This case series included 10 eyes of 6 patients: PPMs were bilateral in 4 patients and unilateral in 2. The patient age at time of surgery ranged from 2.5 months to 2.5 years (mean, 14 months). Mean postoperative follow-up was 5.3 years (range, 2.5-8 years). All patients had successful clearance of the visual axis and good visual acuity. No intraoperative complications occurred. CONCLUSIONS All patients in this series had excellent visual and structural outcomes, with no significant complications. The technique described here may be considered for patients with visually significant PPMs to improve visual function and pupil appearance.

Successful treatment of choroidal blastomycosis with oral administration of voriconazole

Blastomycosis dermatitidis is a dimorphic fungus endemic to the midwestern and southeastern U.S. and was first described by Gilchrist in 1894. Inhalation of airborne spores arising from the soil may lead to pulmonary, cutaneous, and genitourinary symptoms. Ocular involvement is extremely rare; however, eyelid infection, conjunctivitis, keratitis, iritis, choroiditis, endophthalmitis, panophthalmitis, and orbital cellulitis have all been described. We report a patient with disseminated blastomycosis and choroidal involvement who was effectively treated with oral administration of voriconazole (Vfend, Pfizer Inc, New York, N.Y.). To our knowledge, there have been no prior reports of ocular blastomycosis treated in this way. A 57-year-old man with a history of cardiac transplant for ischemic cardiomyopathy sought treatment for lowgrade fevers, dyspnea, nonproductive cough, weight loss, and myalgias for 1 month’s duration. He also reported a 2-day history of blurry vision in the left eye. Physical examination showed pustular skin lesions on the back, abdomen, and feet; diffuse, fine crackles were appreciated in the lungs. On initial ophthalmic examination, best-corrected visual acuity (BCVA) was 20/20 in the right eye and 20/40 in the left eye. The anterior segment examination was unremarkable. Dilated fundus examination revealed multiple choroidal lesions bilaterally, including a subfoveal lesion in the left eye (Fig. 1). There was no vitritis. Computed tomographic results of the chest showed innumerable small pulmonary nodules in a miliary pattern throughout the lungs. Biopsy of the lung and skin lesions showed multiple broad-based budding yeasts, consistent with blastomycosis. Magnetic resonance imaging of the brain was normal. The patient was treated orally with 200 mg of voriconazole every 12 hours. His acute renal failure precluded him from receiving amphotericin B, which is nephrotoxic. He showed no evidence of central nervous system involvement. The patient’s symptoms subsided, the choroidal and skin lesions resolved, and the patient regained 20/25 BCVA in the left eye. The subfoveal lesion in the left eye, as well as the peripheral lesions in both eyes, involuted after treatment (Fig. 2). Pariseau et al. summarized 41 cases of ophthalmic blastomycosis previously reported in the literature. Eight of these were intraocular cases described within the last 30 years. All 8 cases were treated with amphotericin B. Voriconazole, which has a 96% bioavailability when taken

Retinal vascular occlusions and macular thinning in fibromuscular dysplasia.

PURPOSE To report retinal findings in a patient with fibromuscular dysplasia. METHODS A retrospective case report. RESULTS A 36-year-old female patient with fibromuscular dysplasia and a history of cerebrovascular events presented with painless vision loss in the left eye. Ophthalmologic evaluation demonstrated retinal vascular occlusive disease and secondary macular thinning. CONCLUSION This case demonstrates that fibromuscular dysplasia can affect the retinal vasculature and lead to macular ischemia and vision loss.