Faculty Bibliography

PURPOSE: To investigate the spectral-domain optical coherence tomography findings in four cases of acute macular neuroretinopathy and identify features that may differentiate acute macular neuroretinopathy from similar conditions in the acute zonal occult outer retinopathy complex. METHODS: Patients with acute macular neuroretinopathy underwent complete periodic examinations, including ophthalmoscopy, color photography and autofluorescence photography, visual field testing, and spectral-domain optical coherence tomography. Abnormalities as seen by multimodal imaging were documented and evaluated during follow-up. RESULTS: The average age of the 4 patients was 32 years (range, 28-34 years), and 2 had bilateral involvement. The follow-up ranged from 1 month to 5 months. In each patient, dark lobular areas seen by ophthalmoscopy corresponded to the measured visual field defect and what appeared to be a regional loss of outer segments of the photoreceptors. The outer segment defect resolved in two patients and improved in the other two during follow-up. All patients showed a colocalizing regional thinning of the outer nuclear layer, which did not change during the follow-up period. There were no associated abnormalities in the fundus appearance. CONCLUSION: Acute macular neuroretinopathy causes reddish brown patches that are because of outer retinal damage as documented by spectral-domain optical coherence tomography. Unlike other entities in the acute zonal occult outer retinopathy complex, acute macular neuroretinopathy has no other synchronic ophthalmoscopically visible fundus abnormalities. These lesions may resolve over time, with restoration of the outermost retinal architecture, but persistent thinning of the overlying outer nuclear layer remained.

PURPOSE:: The purpose of this study was to report long-term results of intravitreal antivascular endothelial growth factor therapy in the management of choroidal neovascularization in patients with angioid streaks associated with pseudoxanthoma elasticum. METHODS:: A consecutive series of patients with pseudoxanthoma elasticum and choroidal neovascularization were managed with intravitreal antivascular endothelial growth factor injections (bevacizumab 1.25 mg/0.05 mL or ranibizumab 0.5 mg/0.05 mL). The main outcome measures were visual acuity and greatest lesion height as measured by optical coherence tomography. RESULTS:: Nine eyes of nine consecutive patients received intravitreal antivascular endothelial growth factor therapy. During the mean follow-up period of 28.6 months, eyes received an average of 8.4 injections. At baseline, the mean visual acuity was 20/368 (median, 20/60) and improved to 20/281 (median, 20/40) at the last visit (P = 0.14). Visual acuity either improved or stabilized in all 9 eyes (100%). Serial optical coherence tomography measurements showed a mean of 353 mum at baseline and decreased to 146 mum at the last visit (P = 0.005). No complications were noted. CONCLUSION:: These long-term results support the use of intravitreal antivascular endothelial growth factor therapy for the management of choroidal neovascularization in patients with pseudoxanthoma elasticum. Continued experience with intravitreal bevacizumab or ranibizumab in this population will help establish long-term efficacy and better define optimal dosing strategies

PURPOSE: To explore the incidence of complications after bilateral same-day intravitreal injections of antivascular endothelial growth factor pharmacotherapies in this retrospective interventional case series. METHODS: An electronic review of billing records was performed to identify all bilateral same-day intravitreal antivascular endothelial growth factor injections performed within a single group retina practice between January 6, 2006 and June 1, 2009. The charts were reviewed to identify the complications of endophthalmitis, intraocular inflammation, retinal tear, and retinal detachment. RESULTS: A total of 1,534 bilateral intravitreal injections (326 bevacizumab and 1,208 ranibizumab: 3,068 injections total) were performed in 367 patients. Three complications were identified. Two cases of unilateral culture-proven endophthalmitis occurred after bilateral intravitreal ranibizumab, and one case of unilateral acute intraocular inflammation occurred after bilateral intravitreal bevacizumab. In all three of these eyes, visual acuity returned to its preinjection level. No cases of retinal tear or retinal detachment were identified. The incidence of culture-proven endophthalmitis was 0.065%, and the incidence of acute intraocular inflammation was 0.033%. CONCLUSION: The complication rates after bilateral same-day intravitreal antivascular endothelial growth factor injections seem to be similar to those after unilateral injections. Severe acute intraocular inflammation can occur unilaterally after same-day bilateral injections of bevacizumab

PURPOSE: To study patients with neovascular age-related macular degeneration (AMD) who experienced a macular hemorrhage after stabilization with intravitreal antivascular endothelial growth factor (anti-VEGF) agents to improve current treatment regimens and prevent disease progression. METHODS: Retrospective chart review of six patients. The main outcome measures included time between last intravitreal anti-VEGF treatment and date of hemorrhage, time between last office visit and date of hemorrhage, and visual acuity before and after hemorrhage. RESULTS: Three of 6 eyes had a macular hemorrhage within 4 weeks of a stable examination. One eye had optical coherence tomography (OCT) that demonstrated no fluid 1 day before the macular hemorrhage. The average time between the date of the last injection and macular hemorrhage was 16.8 weeks (range, 7.3-28.9 weeks). The average time between the last stable examination and an event was 4.2 weeks (range, 1 day to 7.3 weeks). Three of six patients had a persistent decline in vision after the hemorrhage. Among the 4 patients, who had better than 20/200 vision before the macular hemorrhage, 2 dropped to 20/200 or worse. CONCLUSION: Sight-threatening macular hemorrhages from AMD can occur within days to weeks after a stable examination and absence of fluid on OCT. Regimens that treat 'as needed' based on clinical findings and OCT may not be appropriate for certain patients

OBJECTIVE: To determine whether there is an increased prevalence of systemic autoimmune diseases in both patients with white spot syndromes (WSS) and their family members. METHODS: Patients with WSS at participating institutions were asked to complete a questionnaire reporting their own medical histories as well as any autoimmune diseases among their first- and second-degree relatives. RESULTS: As of January 1, 2008, 114 questionnaires had been collected, providing medical histories of 114 patients with WSS and 1098 family members. The number of patients with WSS with self-reported systemic autoimmune diseases was 26 (23%). Of 1098 relatives, 106 (10%) had at least 1 autoimmune disease. Systemic autoimmunity was more prevalent in female relatives (13%) as compared with male relatives (6%). In addition, the prevalence of autoimmunity was significantly higher among first-degree relatives (13%) than second-degree relatives (8%). Patients who themselves had systemic autoimmune diseases showed a greater prevalence of systemic autoimmunity among their families as compared with the families of patients without systemic autoimmune diseases. CONCLUSIONS: Our data indicate that there is an increased prevalence of systemic autoimmunity in both patients with WSS and their first- and second-degree relatives. This suggests that WSS occur in families with inherited immune dysregulation that predisposes to autoimmunity

PURPOSE: To evaluate the response of eyes treated with photodynamic therapy (PDT) for predominantly hemorrhagic lesions in neovascular age-related macular degeneration (NVAMD). DESIGN: Consecutive, interventional case series. METHODS: A retrospective review was conducted of eyes with NVAMD that were treated with PDT for predominantly hemorrhagic subfoveal lesions. Baseline data recorded were patient age, use of aspirin or warfarin, history of previous PDT treatment, visual acuity (VA), extent of subretinal hemorrhage (SRH), and central foveal thickness (CFT) and total macular volume (TMV) measured by optical coherence tomography (OCT). Follow-up VA, SRH status, and complications were recorded. RESULTS: Twenty-one eyes of 21 patients were identified with a mean posttreatment follow-up of 9.4 months. Mean pretreatment VA was 20/210, and mean SRH size was 16 disk areas. The SRH resolved in 18 eyes (86%) after a mean of six months, with a mean VA of 20/210. Mean VA for all eyes at most recent follow-up was 20/250. Thirteen (62%) of 21 eyes exhibited stable or improved VA at the most recent follow-up. VA one year after PDT inversely correlated with patient age (P = .045) and initial SRH size (P = .04) and positively correlated with pretreatment VA (P = .01). Previous PDT treatment, use of aspirin or warfarin, and OCT data did not significantly correlate with VA or SRH resolution. CONCLUSIONS: PDT may be an effective treatment for minimizing visual loss in eyes with NVAMD and predominantly hemorrhagic lesions. Further study is warranted to detail the risk-benefit ratio and compare PDT with other treatment modalities.

PURPOSE: To determine the incidence of endophthalmitis following intravitreal injection of anti-vascular endothelial growth factor (VEGF) agents. DESIGN: A retrospective interventional case series. METHODS: A total of 10,254 intravitreal anti-VEGF injections (406 pegaptanib, 3,501 bevacizumab, and 6,347 ranibizumab) were performed from January 5, 2005 to October 18, 2007. The number of the injections was determined from the injection log books and billing records. The injections were performed as an office based procedure with use of povidone-iodine as a part of preinjection preparation. Preinjection antibiotics, eye drape, or surgical attire were not used. The main outcome measures were the incidence of suspected and proven endophthalmitis. RESULTS: There were three cases of suspected endophthalmitis, one case following bevacizumab injection and two cases following ranibizumab injection. There was no case of culture-proven endophthalmitis. All three patients regained their preinjection visual acuity. The incidence of suspected endophthalmitis was 0.029% (95% confidence interval, 0.006% to 0.085%). There was no difference in the incidence of endophthalmitis between ranibizumab and bevacizumab injections (P = .6). CONCLUSIONS: Although there is no consensus regarding the intravitreal injection procedure technique, the incidence of suspected endophthalmitis was very low in a large series of injected patients in a community setting and the incidence compares favorably with that reported in clinical trials where much more extensive preinjection preparation was mandated. We found no difference in the endophthalmitis risk of patients receiving bevacizumab as compared with ranibizumab

PURPOSE/OBJECTIVE:To describe a fundus autofluorescent photographic pattern in a case of bilateral diffuse uveal melanocytic proliferation (BDUMP) with occult esophageal carcinoma. METHODS:Observational case report. RESULTS:Color photography captured multiple round areas of discoloration throughout the fundus in a background of orange pigmentation that may have represented a lipofuscin-laden retinal pigment epithelium (RPE). Autofluorescence photography showed extensive nummular areas of hypoautofluorescence interspersed between areas of hyperautofluorescence as a result of extensive deposition of lipofuscin within the RPE. CONCLUSION/CONCLUSIONS:Our case attests to the utility of autofluorescence photography as a sensitive, noninvasive imaging modality for the early detection of BDUMP.

PURPOSE: To describe multifocal choroiditis in two siblings. METHODS: Retrospective case reports. RESULTS: Two sisters presented 10 years apart with multifocal choroiditis. The first sister manifested late findings of multiple punched-out chorioretinal lesions and a quiescent central fibrovascular scar. The second sister presented in the acute phase with multiple creamy yellow lesions near the optic nerve and fovea and with a choroidal neovascular membrane. Both cases were unilateral. DISCUSSION: Multifocal choroiditis is an inflammatory disorder of the inner choroid and retinal pigment epithelium of unknown etiology. There is no gene associated with multifocal choroiditis, and to our knowledge, no cases of relatives with the disease have been reported. CONCLUSION: We describe multifocal choroiditis in two sisters, suggesting a possible genetic or environmental component to this disease. Further study is necessary to better elucidate the etiology of this disease.