Clement C. Chow

Rapid response of retinal pigment epithelial detachments to intravitreal aflibercept in neovascular age-related macular degeneration refractory to bevacizumab and ranibizumab

PurposeThe aim of this study is to report the short-term efficacy of aflibercept in the treatment of neovascular age-related macular degeneration (AMD) with associated retinal pigment epithelial detachment (PED) which is refractory or develops tachyphylaxis to bevacizumab and ranibizumab.MethodsThe method comprised a retrospective review of the medical records of patients with neovascular AMD and associated PEDs recently treated with aflibercept and previously treated with bevacizumab and ranibizumab.ResultsThree eyes of three female patients of ages 49, 55, and 65 years old with large serous PEDs and subretinal fluid (SRF) associated with occult choroidal neovascularization and neovascular AMD were treated with aflibercept after intravitreal bevacizumab and/or ranibizumab failed to resolve the lesions. All had complete resolution of SRF and complete or near-complete resolution of the PEDs after aflibercept injections over a 3-month period. Visual acuity improved in all three eyes.ConclusionIntravitreal aflibercept may be an effective treatment option for serous PED in neovascular AMD patients after bevacizumab and ranibizumab have previously failed. Larger studies with longer follow-up are required to determine the role of aflibercept in treatment of PED in neovascular AMD.

Peripapillary retinal nerve fiber layer thickness in sickle-cell hemoglobinopathies using spectral-domain optical coherence tomography

PURPOSE To determine whether patients with a sickle-cell hemoglobinopathy without glaucoma have peripapillary retinal nerve fiber layer (RNFL) thinning by spectral-domain optical coherence tomography. DESIGN Prospective study. METHODS All patients with a sickle cell hemoglobinopathy (sickle-cell disease, sickle-cell hemoglobin C disease, and sickle-cell thalassemia) and age-similar, race-matched controls underwent a comprehensive eye examination and spectral-domain optical coherence tomography of the macula and optic nerve head using the Heidelberg Spectralis (Heidelberg Engineering, Inc, Carlsbad, California, USA). Participants with prior retinal treatments (laser or surgery), diabetes mellitus, glaucoma, or other ocular diseases were excluded. The sickle-cell disease patients were grouped into those with focal macular thinning and those without. Those with macular thinning were grouped further into mild, moderate, and severe thinning groups based on temporal macular thickness. Analysis of variance testing and post hoc analysis with the Tukey test and Pearson correlation were performed to assess for peripapillary RNFL thickness differences among different groups. RESULTS One hundred fifty-one eyes of 88 sickle-cell patients and 55 eyes of 30 age-similar and race-matched (black) controls were included. Sickle-cell patient eyes with macular thinning (n = 81) had thinner mean peripapillary RNFL thicknesses in the nasal sector (P = .01) compared with non-sickle-cell control eyes and in the superotemporal sector (P = .01) compared with sickle-cell patient eyes without macular thinning (n = 70). In the severe macular thinning subgroup (n = 55), the mean peripapillary RNFL thickness was significantly thinner than that of controls (P < .05) in 6 of 7 sectors. There is a positive linear relationship between temporal macular thickness and global peripapillary RNFL thickness with a Pearson correlation coefficient of 0.60 (P < .0001). CONCLUSIONS Nonglaucomatous, black sickle-cell patients with focal macular thinning on spectral-domain optical coherence tomography have significantly thinner peripapillary RNFL than those without macular thinning or controls. The degree of thinning correlates with severity of temporal macular thinning. These patients may require different peripapillary RNFL thickness thresholds for future glaucoma evaluations.

Use of Infliximab in the Treatment of Peripheral Ulcerative Keratitis in Crohn Disease

PURPOSE To report the effectiveness of infliximab in the treatment of peripheral ulcerative keratitis (PUK) associated with Crohn disease. DESIGN Retrospective, interventional case series. METHODS SETTING Institutional, academic referral setting. patient or study population: Six eyes of 3 patients with PUK associated with Crohn disease that failed or were intolerant to traditional immunosuppression such as oral prednisone and cyclophosphamide. INTERVENTION OR OBSERVATION PROCEDURE(S): Infliximab intravenous infusion 5 mg/kg every 2 to 8 weeks. MAIN OUTCOME MEASURE(S) Subjective outcome such as pain improvement and objective outcomes such as visual acuity, signs of inflammation, and progression of corneal thinning. RESULTS Symptom of pain improved in all patients. Visual acuity remained stable in 4 of 6 eyes, improved in 1 of 6 eyes, and worsened in 1 of 6 eyes (secondary to stromal scar). Rapid resolution of inflammation and arrest of further thinning were observed in all 6 eyes. Similar effects were observed on recurrence treated with repeat dosing of infliximab in 5 of 6 eyes. CONCLUSIONS Infliximab produced a rapid, dramatic, repeatable suppression of corneal inflammation, pain, and keratolysis in PUK associated with Crohn disease. Although the effect of long-term therapy with these agents is unknown, infliximab should be considered in patients with Crohn-associated PUK not amenable to traditional therapy. Larger prospective studies are needed to determine the efficacy of infliximab in this aggressive disease entity.

Effects of Green Diode Laser in the Treatment of Pediatric Coats Disease

PURPOSE To review the effect of green diode laser ablation therapy on retinal structure and functional outcome in patients with advanced Coats disease. DESIGN Retrospective, interventional case series. METHODS Fourteen eyes of 13 patients with Coats disease were included in this study. Medical records, Retcam photographs (Clarity Medical Systems, Pleasanton, CA), and fluorescein angiograms were reviewed. All patients initially were treated with green diode laser (532 nm) ablation therapy to areas of the retinal telangiectasis associated with exudation. Main outcome measures included visual acuity, treatment outcome defined as complete resolution of telangiectatic lesions or exudative detachment, and macular status at the end of follow-up. RESULTS Before treatment, 1 eye was at stage 2 (telangiectasis and exudation), 12 eyes were at stage 3 (exudative retinal detachment), and 1 eye was at stage 4 (total retinal detachment with glaucoma). Five eyes had highly detached retina of more than 4 mm. Median age at diagnosis was 51 months (range, 0.5 to 153 months). Median follow-up was 39.5 months (range, 15 to 70 months). Median number of green diode laser photocoagulation treatments was 2 (range, 1 to 5). After laser photocoagulation, 13 (93%) of 14 eyes had no active exudation. Functionally, 4 (29%) of 14 eyes had 20/50 or better visual acuity, 3 (21%) of 14 eyes had 20/60 to 20/200 visual acuity, 5 (36%) of 14 eyes had 20/400 to light perception visual acuity, and 2 (14%) of 14 eyes had no light perception visual acuity. No eye was phthisical or enucleated. CONCLUSIONS Green diode laser therapy can be an effective treatment for advanced Coats disease, even in the presence of a moderate to severely elevated retinal detachment.

Laser Photocoagulation at Birth Prevents Blindness in Norrie's Disease Diagnosed Using Amniocentesis

OBJECTIVE To report the first case of prophylactic laser treatment to prevent blindness in a patient who was diagnosed with Norries disease by genetic testing with amniocentesis. DESIGN Case report. PARTICIPANTS A 2-year-old white boy with Norries disease. METHODS A 37-week gestational age male with a family history of Norries disease was born via Cesarean section after the mother had undergone prenatal amniocentesis fetal-genetic testing at 23 weeks of gestation. A C520T (nonsense) mutation was found in the Norries disease gene. After examination under anesthesia confirmed the diagnosis on the first day of life, laser photocoagulation was applied to the avascular retina bilaterally. The patient was followed closely by ophthalmology, pediatrics, and occupational therapy departments. MAIN OUTCOME MEASURES Functional outcome, as documented by Teller visual acuity and formal occupational therapy testing, and anatomic outcome, as documented by Retcam photography and fluorescein angiography. RESULTS Complete regression of extraretinal fibrovascular proliferation was observed 1 month after laser treatment. No retinal detachment had occurred to date at 24 months. Teller visual acuity at 23 months of life was 20/100 in both eyes. The patients vision and developmental milestones were age appropriate. CONCLUSIONS Pre-term genetic diagnosis with immediate laser treatment after birth may preserve vision in individuals affected with Norries disease.

Microperimetric sensitivity in patients on hydroxychloroquine (Plaquenil) therapy

PurposeThe purpose of this study was to measure macular sensitivity using microperimetry in patients on Plaquenil therapy without evidence of retinopathy as assessed by recommended screening standards.MethodsSixteen patients from a clinical practice treated with 200 or 400 mg/day of Plaquenil for more than 5 years, without visual complaints (visual acuity 20/25 or better), and without a history of diabetes or macular disease were included. Participants underwent a complete ophthalmic examination with spectral-domain optical coherence tomography (SD-OCT), 10-2 Humphrey visual field (HVF), fundus autofluoresecene (FAF), multifocal electroretinography (mfERG), and microperimetry that covered the central 12° of the visual field. Ten age-similar, visually normal subjects served as controls.ResultsThe average age of the study cohort was of 54.5 years, with an average daily Plaquenil dose of 4.00 mg/kg/day (range, 1.77–6.67 mg/kg/day) and an average cumulative dose of 1485 g (range, 256–3650 g). All patients had normal ocular exams, and no evidence of retinopathy based on 10-2 HVF, FAF, mfERG, and SD-OCT. The mean retinal sensitivity by microperimetry was 15.0 dB (OD) and 14.6 dB (OS). The overall mean microperimetry sensitivity of the patients (14.7±1.9 dB) was significantly lower (P<0.001) than that of the controls (16.5±2.1 dB).ConclusionsPatients on Plaquenil without clinical evidence of retinal toxicity can have reduced retinal sensitivity, as assessed by microperimetry. The mean sensitivity difference between the patients and controls suggests that microperimetry can provide important information regarding visual function in patients on Plaquenil therapy.

Treatment of recalcitrant radiation maculopathy using intravitreal dexamethasone (ozurdex) implant

PURPOSE Radiation maculopathy is the most common cause of severe vision loss after radiotherapy of uveal melanoma. To date, no effective therapy exists. The authors report a novel approach to the treatment of radiation maculopathy using dexamethasone (Ozurdex, Allergan Inc) intravitreal implant. METHODS This is a retrospective case series of two patients who developed radiation maculopathy after radiotherapy for uveal melanoma and was treated with Ozurdex. Clinical outcomes included visual acuity, central foveal thickness by optical coherence tomography, intraocular pressure, and cataract formation. RESULTS Both patients were of Caucasian descent. Patient 1 received charged-particle radiation, whereas Patient 2 received iodine-125 brachytherapy for medium-sized uveal melanoma located in the midperipheral retina. Radiation maculopathy developed 47 months and 18 months after radiation exposure in Patient 1 and 2, respectively. Both patients initially received bevacizumab monotherapy followed by alternating therapy with bevacizumab and intravitreal triamcinolone. Secondary to a limited response, the patients were treated with Ozurdex implants. One patient had visual improvement, and both patients experienced a prolonged time frame of anatomical stability. Adverse effects included a rise in the intraocular pressure, which was controlled by topical hypotensive agents and posterior subcapsular cataract formation in Patient 1. CONCLUSION Ozurdex intravitreal implant provides a prolonged period of anatomical stabilization in recalcitrant cases of radiation maculopathy in patients who have failed multiple intravitreal bevacizumab injections and had only a partial response to intravitreal triamcinolone. Larger prospective studies are required to determine the extent of visual benefit.

Peripheral Nonperfusion and Tractional Retinal Detachment Associated with Congenital Optic Nerve Anomalies

PURPOSE To report an association of congenital optic nerve anomalies with peripheral retina nonperfusion and to describe the clinical manifestations and treatment. DESIGN Retrospective, observational case series. PARTICIPANTS Fifteen patients with congenital optic nerve anomalies referred for pediatric retina consultation were studied. Sixteen eyes of 9 patients with optic nerve hypoplasia and 8 eyes of 6 patients with other congenital optic nerve anomalies, including optic nerve coloboma, morning glory disc, and peripapillary staphyloma, were included. METHODS All patients underwent examinations under anesthesia. Wide-angle retina photographs and fluorescein angiograms were reviewed. The severity of nonperfusion was graded. The presence of fibrovascular proliferation (FP), vitreous hemorrhage (VH), and tractional retinal detachment (TRD) were documented. Anatomic outcome after treatment was recorded. MAIN OUTCOME MEASURES Severity of nonperfusion, occurrence of secondary complications, and the anatomic outcome of patients who underwent laser treatment. RESULTS In patients with optic nerve hypoplasia, 12 of 16 eyes (75%) had severe peripheral nonperfusion, 12 of 16 eyes (75%) had FP, 3 of 16 eyes (19%) had VH, and 10 of 16 eyes (63%) had TRD. Six of these eyes with severe nonperfusion received laser photocoagulation to the nonperfused retina; laser-treated retinas remained attached in all 6 eyes. In patients with the other optic nerve anomalies, 7 of 8 eyes (88%) had mild to moderate nonperfusion, 2 of 8 eyes (25%) had FP, 1 of 8 eyes (12%) had VH, and 2 of 8 eyes (25%) had TRD. Six of 9 patients (67%) with optic nerve hypoplasia and 1 of 6 patients (17%) with other anomalies had a coexisting congenital brain disease. CONCLUSIONS Congenital optic nerve anomalies may be associated with peripheral retina nonperfusion and the secondary complications of FP, VH, and TRD. In this select group of patients, the nonperfusion associated with optic nerve hypoplasia seemed to be more severe and associated more frequently with secondary complications. Peripheral retina examination in eyes with optic nerve anomalies may identify nonperfusion or FP. Laser treatment of the avascular retina may have helped prevent complications from proliferative retinopathy in eyes clinically observed to have progressed or considered at risk for progression to proliferative retinopathy.

Bilateral traumatic expulsive aniridia after phacoemulsification

We report a case of bilateral traumatic expulsive aniridia after uneventful phacoemulsification through small clear corneal incisions. Phacoemulsification was performed 8 and 13 months prior to the trauma in the left and right eyes, respectively. In both eyes, the intraocular lens and capsular bag were undisturbed after trauma. After resolution of hyphema, transient elevated intraocular pressure, and anterior chamber inflammation, best corrected visual acuity returned to 20/25 in each eye 6 months later. Self-sealing clear corneal wounds likely serve as a decompression valve during blunt trauma, thus preventing devastating intraocular damage and globe rupture. The intraocular lens may absorb the external force, therefore preventing damage to the capsular bag and zonules as well as preventing prolapse of posterior structures. A review of previously reported cases of traumatic aniridia is also presented.

Cross-Sectional Analysis of Neurocognitive Function, Retinopathy, and Retinal Thinning by Spectral-Domain Optical Coherence Tomography in Sickle Cell Patients.

Purpose: The purpose was to examine the relationship between neurocognitive function and two distinct forms of retinopathy in sickle cell disease. Materials and Methods: Patients with sickle cell disease (n = 44, age range: 19-56 years, 70% female) were prospectively recruited for this cross-sectional study. Retinopathy was characterized by: (1) Presence of focal retinal thinning on spectral domain optical coherence tomography and (2) determination of the sickle retinopathy stage on funduscopic exam based on Goldberg classification. Neurocognitive function was assessed using the Philadelphia Brief Assessment of Cognition (PBAC), a validated test of cognition. Univariate and multivariate analyses for PBAC score outcomes were performed. Retinal thinning and retinopathy stage were primary variables of interest and age, gender, genotype, education, and history of stroke were covariates. Results: Univariate analysis revealed associations with total PBAC score and age (P = 0.049), history of stroke (P = 0.04), and genotype (P < 0.001). Focal retinal thinning and Goldberg retinopathy stage were not associated with each other in this sample. Neither the presence of focal retinal thinning nor degree of retinopathy was associated with total PBAC score in univariate or multivariate analyses. Conclusions: We find an association between lower cognitive function and older age, history of stroke and sickle cell genotype SS in patients with sickle cell disease. Our data do not provide evidence to support an association between cognitive function and retinopathy in sickle cell patients.