Daniel F. Kiernan

The use of intraocular corticosteroids.

Background: Diabetic macular edema (DME), cystoid macular edema (CME), age-related macular degeneration (AMD), retinal vascular occlusion (RVO) and uveitis are responsible for severe visual impairment worldwide. In some patients with these conditions, treatment with intraocular corticosteroids may be beneficial. Although off-label use of these agents has occurred for many years, novel agents including preservative-free and sustained-release intravitreal implants are currently being studied in clinical trials (CTs). Objective: To review the use of intraocular corticosteroids. Methods: Literature review. Results: Used alone, intravitreal corticosteroids may benefit disorders such as DME, RVO and uveitis compared with standard therapy or observation. Patients with AMD may benefit more from combination treatment with photodynamic therapy, intravitreal corticosteroid and intravitreal anti-VEGF injections. Intraoperative use of these agents may assist in visualization and manipulation of fine retinal structures. Sustained-release intraocular implants have been approved for severe posterior uveitis, and have shown benefits in ongoing CTs. Conclusion: Although intraocular corticosteroid injections have a limited duration of action requiring frequent re-treatment, and significant side effects including cataract and glaucoma development, intraocular injections may be of benefit in certain ocular disorders. Corticosteroid implants are emerging as potential treatments for macular edema due to uveitis, DME or RVO.

Fluorescein Angiography of Recurrent Retinopathy of Prematurity After Initial Intravitreous Bevacizumab Treatment

A vaccine to prevent herpes zoster and postherpetic neuralgia in older adults. N Engl J Med. 2005;352(22):2271-2284. 2. Nagpal A, Vora R, Margolis TP, Acharya NR. Interstitial keratitis following varicella vaccination. Arch Ophthalmol. 2009;127(2):222-223. 3. Naseri A, Good WV, Cunningham ET Jr. Herpes zoster virus sclerokeratitis and anterior uveitis in a child following varicella vaccination. Am J Ophthalmol. 2003;135(3):415-417. 4. Naseri A, Margolis TP. Varicella zoster virus immune recovery stromal keratitis in a patient with AIDS. Br J Ophthalmol. 2001;85(11):1390-1391.

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.

Intraocular corticosteroids for posterior segment disease: 2012 update

Introduction: Diabetic macular edema (DME), cystoid macular edema (CME), age-related macular degeneration (AMD), retinal vascular occlusion (RVO) and uveitis are responsible for severe visual impairment worldwide. In some patients with these conditions, treatment with intraocular corticosteroids may be beneficial. Although off-label use of these agents has occurred for many years, novel agents including preservative-free and sustained-release intravitreal implants are currently being studied in clinical trials (CTs). Areas covered: This paper reviews the use of CTs for vitreoretinal (VR) diseases including choroidal neovascularization, CME, DME, RVO and posterior uveitis. It also discusses the use of corticosteroids for treating VR disease, including dexamethasone, fluocinolone acetonide, intravitreal implants and triamcinolone acetonide. Expert opinion: Used alone, intravitreal corticosteroids may benefit disorders such as DME, RVO and uveitis compared with standard therapy. Cases of exudative AMD non-responsive to standard treatment may benefit from combination therapy, including usage of intravitreal corticosteroid injections. Intraoperative use of these agents may aid visualization of retinal structures. Sustained-release intraocular implants have been approved for posterior uveitis and RVO associated with macular edema. In spite of this, most intraocular corticosteroids have a limited duration of action along with significant side effects, including cataract and glaucoma. Currently, intravitreal corticosteroid usage for DME is considered off-label.

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.

EN FACE SPECTRAL-DOMAIN OPTICAL COHERENCE TOMOGRAPHY OUTER RETINAL ANALYSIS AND RELATION TO VISUAL ACUITY

Purpose: To describe a method of en face visualization and quantification of the photoreceptor inner segment/outer segment junction area, using spectral-domain optical coherence tomography, and association with visual acuity. Methods: Case series of 74 eyes in 53 patients. Central 1-mm and 400-&mgr;m en face areas were analyzed with a computer algorithm. Results: The presence or absence of inner segment/outer segment junction was visible on both spectral-domain optical coherence tomography en face and retinal cross sections. Thirty eyes (40.6%) had no retinal pathology and an average logMAR visual acuity of 0.116. Twenty-five eyes (33.8%) had intraretinal edema, with visual acuity of 0.494. Nineteen eyes had nonneovascular age-related macular degeneration (dry age-related macular degeneration, 25.6%), with visual acuity of 0.392. In all eyes, central 1-mm and 400-&mgr;m en face areas were 58.3 ± 25.0% and 56.4 ± 26.0%, which showed significant correlation with visual acuity (Pearson correlation, r = −0.66 and −0.56, both P < 0.001). This correlation was greater than correlation of visual acuity with central subfield thickness (r = 0.39, P < 0.001), macular volume (r = 0.36, P = 0.002), and average macular thickness (r = 0.37, P = 0.001). However, no variables were significantly correlated with dry age-related macular degeneration eyes. Conclusion: Central en face inner segment/outer segment junction areas are significantly correlated with visual acuity in most eyes. This may correlate better with visual acuity than other spectral-domain optical coherence tomography values, as a reflection of photoreceptor integrity. Dry age-related macular degeneration may disrupt the plane used to formulate the en face display. Advancements in spectral-domain optical coherence tomography may provide routine en face visualization analysis.

Peripheral retinal nonperfusion in septo-optic dysplasia (de Morsier syndrome)

tocoagulation was applied and the worm was successfully killed (Figure 1F). The patient reported significant subjective improvement, and his visual acuity measured 20/40 OS 1 month and 20/25 OS 3 months following the initial visit. Spectraldomain OCT showed disruption of the photoreceptors in the papillomacular region secondary to prior photocoagulation but progressive restoration of the normal inner segment/outer segment junction and photoreceptor architecture in the fovea (Figure 2B and C).

Outcomes of chronic macular hole surgical repair

Purpose: To report visual and anatomic outcomes of chronic macular hole surgery, with analysis of pre-operative OCT-based hole size and post-operative closure type. Settings and Design: An IRB-approved, retrospective case series of 26 eyes of 24 patients who underwent surgery for stage 3 or 4 idiopathic chronic macular holes at a tertiary care referral center. Statistical Analysis: Students t-test. Results: Nineteen of 26 eyes (73%) had visual improvement after surgery on most recent exam. Twenty-one of 26 eyes (81%) achieved anatomic closure; 16 of 26 eyes (62%) achieved type 1, and five of 26 eyes (19%) achieved type 2 closure. Post-operative LogMAR VA for type 1 closure holes (0.49) was significantly greater than for type 2 closure and open holes (1.26, P < 0.003 and 1.10, P < 0.005, respectively), despite similar pre-operative VA (P = 0.51 and 0.68, respectively). Mean pre-operative hole diameter for eyes with type 1 closure, type 2 closure, and holes that remained open were 554, 929, and 1205 microns, respectively. Mean pre-operative hole diameter was significantly larger in eyes that remained open as compared to eyes with type 1 closure (P = 0.015). Conclusion: Vitrectomy to repair chronic macular holes can improve vision and achieve long-term closure. Holes of greater than 3.4 years duration were associated with a greater incidence of remaining open and type 2 closure. Larger holes (mean diameter of 1205 microns) were more likely to remain open after repair.

Peripheral Retinal Nonperfusion Associated With Optic Nerve Hypoplasia and Lissencephaly

patient with West Nile virus infection. Arch Ophthalmol. 2003;121(2):205-207. 2. Garg S, Jampol LM. Systemic and intraocular manifestations of West Nile virus infection. Surv Ophthalmol. 2005;50(1):3-13. 3. Garg S, Jampol LM, Wilson JF, Batlle IR, Buettner H. Ischemic and hemorrhagic retinal vasculitis associated with West Nile virus infection. Retina. 2006; 26(3):365-367. 4. Chan CK, Limstrom SA, Tarasewicz DG, Lin SG. Ocular features of west nile virus infection in North America: a study of 14 eyes. Ophthalmology. 2006; 113(9):1539-1546. 5. Seth RK, Stoessel KM, Adelman RA. Choroidal neovascularization associated with West Nile virus chorioretinitis. Semin Ophthalmol. 2007;22(2):81-84. 6. Nash D, Mostashari F, Fine A, et al; 1999 West Nile Outbreak Response Working Group. The outbreak of West Nile virus infection in the New York City area in 1999. N Engl J Med. 2001;344(24):1807-1814.