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Purpose To report the 5-year outcome of an outbreak of Bipolaris hawaiiensis fungal endophthalmitis caused by contamination of intravitreal triamcinolone from Franck’s Compounding Pharmacy in Ocala, Florida. Design Retrospective case series. Participants Twenty-three patients (n = 25 eyes). Methods Data was collected from the practice of a single retina specialist in Los Angeles (K.W.S) and a retina practice in New York City. Intravitreal injections of triamcinolone obtained from a single lot were subsequently found to be contaminated with Bipolaris hawaiiensis. Main Outcome Measures Visual acuity; presence of vitreous cells, anterior chamber cells, or both; and detection of fungi or fungal elements in samples obtained by vitreous needle aspiration or vitreous biopsy. Results Fungal endophthalmitis developed in 92% (23/25) of eyes. Despite aggressive local and systemic long-term therapy, severe visual loss resulted in the majority of treated eyes and the enucleated eyes showed evidence of hyphae. Conclusions These reported cases of Bipolaris hawaiiensis endophthalmitis provide important messages for clinicians and regulatory agencies. First, patients treated with high-dose, long-term, orally administered voriconazole appeared to achieve better outcomes in treatment of Bipolaris endophthalmitis. Second, treated eyes may still show signs of deterioration, indicating the potential survival of causative organisms or fibrosis encapsulating the ciliary body, leading to hypotony. Third, several parallels can be drawn between this outbreak and the fungal meningitis outbreak after Exserohilum rostratum contamination of methylprednisolone by the New England Compounding Center.

Purpose: This work aims to determine whether previously defined genotype risk groups interact with Age-Related Eye Disease Study formulation (AREDS-F) use in progression to neovascular age-related macular degeneration (nvAMD). Methods: We conducted a case-only study of 265 nvAMD patients. Patients were anonymously genotyped at the complement factor H and age-related maculopathy susceptibility 2 loci and segregated into genotype groups (GTGs) defined by specific combinations of risk alleles. Physicians, who were blind to patients’ GTGs, obtained patients’ AREDS-F use history. The facility performing genetic analysis was blind to the AREDS-F use history. We used logistic analysis to estimate the interaction coefficient between AREDS-F use and GTG 2 vs GTG 3 in a general-population model. Results: The odds ratio of numbers of patients reporting prior AREDS-F use to nonuse for GTG 2 vs GTG 3 was 4.18 ( P = .001). Logistic regression, correcting for nongenetic risk factors, gave an estimate of the β for interaction of AREDS-F with genotype of 1.57 ( P = .001). This estimates a corrected odds ratio associated with the effect of interaction of 4.81 between those in GTG 2 compared with those in GTG 3. Conclusions: Our data indicate an interaction between GTGs and AREDS-F use that is consistent in size and direction with previously published reports, which had found that using AREDS-F supplements significantly increases the risk of nvAMD for some users and significantly protects other users.

North Carolina macular dystrophy (NCMD) is a congenital, developmental abnormality of the macula. It was first described in families living in western North Carolina by Lefler, Wadsworth, and Sidbury in 1971 and initially called autosomal dominant macular degeneration and aminoaciduria (Lefler et al. 1971). Subsequently this disease became known as the Lefler Wadsworth Sidbury syndrome. However, the aminoaciduria was later found to not consistently manifest in the affected family members, making this a misnomer in that it is not a syndrome at all. J Donald M. Gass, MD named NCMD after the founder effect of this initial family in his textbook Stereoscopic Atlas of Macular Diseases: Diagnosis and Treatment (Gass 1997). However, NCMD has now been described in many unrelated families from the United States, the United Kingdom, France, Germany, Korea, and Belize (Small 1998; Reichel et al. 1998; Pauleikhoff et al. 1997).

To review current literature of the benefits that aspirin provides for patients' cardiovascular health compared with the risk of AMD worsening.We performed a review and critically analyzed six cardiovascular and four ophthalmological trials regarding risks and benefits of aspirin use. The prospective randomized cardiovascular trials had a cumulative 167,580 while the 3 smaller ophthalmological data sets had a cumulative 12,015 subjects.The reviewed meta-analysis literature demonstrated a statistically significant 32% reduction in the risk of nonfatal stroke with regular aspirin users. The study also documented that aspirin users decreased the risk of fatal vascular deaths by 15%. Of the three ophthalmological studies highlighting the adverse affects of aspirin association with AMD, all suggested an exacerbation of AMD without statistical significance and broad confidence bands.Overall, the number, size, and quality of the cardiovascular studies recommending aspirin use are far superior to the fewer, smaller and conflicting studies suggesting a possible adverse effect of aspirin use in relation to AMD. The benefits of aspirin usage include preserving the duration and quality of life by decreasing stroke and heart attack risk. These benefits seem to far outweigh the theoretical risks of possibly exacerbating wet AMD, which can be reasonably controlled with anti-VEGF therapy.

Purpose: To highlight the striking similarities between the lesions of congenital toxoplasmosis (CT) and North Carolina Macular Dystrophy (NCMD) using multimodal imaging including spectral domain optical coherence tomography (SD-OCT). Observations: We are comparing a case report of CT compared to that of NCMD. The case of a 64-year-old man with a lifelong history of decreased vision OD from toxoplasmosis and new onset of central retinal vein occlusion OS. Color fundus photography, spectral domain optical coherence tomography (SD-OCT), and intravenous fluorescein angiography (IVFA) were used as diagnostic imaging tools to demonstrate the similarities and differences between CT and NCMD. In this case, unilateral CT demonstrated a large, excavated, coloboma-like chorioretinal lesion identical to NCMD grade 3. Serology studies were positive for toxoplasmosis. The similarities of CT and NCMD grade 3 using SD-OCT are especially striking. Conclusion and importance: Lesions of CT and NCMD grade 3 can appear identical on clinical exam and are indistinguishable from one another on SD-OCT. Because CT is a phenocopy of NCMD, many cases of the original NCMD family members had been misdiagnosed as CT. North Carolina Macular Dystrophy may be more common than previously realized and bilateral CT cases should be reexamined along with family members and genetic testing performed. Cases of bilateral CT actually may be NCMD cases. Now that the genetic and molecular mechanisms of NCMD are known, these may provide clues into the pathogenesis of CT. Keywords: Congenital toxoplasmosis (CT), North Carolina macular dystrophy (NCMD/MCDR1), Optical coherence tomography (OCT), Phenocopy, PRDM13

Purpose To describe multimodal imaging and corresponding functional studies in a newly found family with North Carolina macular dystrophy (NCMD). To our knowledge, this is an original report using OCT angiography to evaluate persons with NCMD. Design A descriptive, retrospective study of a family with NCMD. Participants A total of 3 participants, representing 3 generations of a single family, each demonstrating a different grade of NCMD, underwent clinical and genetic testing. Methods Diagnostic multimodal imaging and functional testing of the retina included color fundus photography, fundus autofluorescence, intravenous fluorescein angiography, spectral-domain OCT and OCT angiography, multifocal electroretinography, full-field electroretinography, and microperimetry. DNA sequencing was performed using Sanger sequencing techniques. Main Outcome Measures Spectral-domain OCT images, fundus photographs, fundus autofluorescence images, fluorescein angiograms, OCT angiograms, multifocal electroretinography images, full-field electroretinography images, and microperimetry maps. Sanger sequencing was performed for molecular diagnosis. Results Multimodal imaging helped to demonstrate the nature of the retinal and choroidal lesions in each participant and the extent of visual function. Genetic testing demonstrated the variant 2 point mutation (chromosome 6: 99593111) in the deoxyribonuclease 1 hypersensitivity binding site on chromosome 6q16 causing overexpression of the retinal transcription factor PRDM13. Conclusions NCMD has great phenotypic variability, which can be appreciated only by examining multiple family members. To our knowledge, this is an original report that shows a correlation of functional studies with multimodal imaging. These findings are consistent with NCMD being a developmental abnormality of the macula. All layers of the retina and choroid demonstrate maldevelopment and varying degrees of malfunction. Although PRDM13 is expressed in the amacrine cells, we have yet to identify an abnormality specific to this cellular layer. The retinal vasculature appears to be surprisingly well preserved or intact by OCT angiogram compared with that shown in intravenous fluorescein angiograms. OCT angiograms suggest that foveal hypoplasia is a phenocopy of grade 1 NCMD, torpedo maculopathy is a phenocopy of grade 2 NCMD, and in this single family, congenital toxoplasmosis is a phenocopy of grade 3 NCMD.

Rosemary Silva-Garcia, Colleen McLellan, Fadi S Shaya, Kent W Small Macula and Retina Institute, Molecular Insight Research Foundation, Cedars-Sinai Medical Towers, Glendale, CA, USA Purpose: To examine the potential long-term benefit of an anti-VEGF/photodynamic therapy (PDT) combination on patients treated for wet age-related macular degeneration (AMD).Methods: A retrospective chart review was conducted on 29 eyes (subjects) from 26 patients (eight male and 18 female) that showed sustained, positive response to combination therapy for exudative AMD for a minimum of 1 year. Collected data included: visual acuity, central retinal thickness, intraocular pressure and history of glaucoma, wet AMD onset and treatment history, concomitant use of anticoagulants and past history or development of cerebrovascular or cardiovascular disease while receiving combination therapy. Results: Subjects underwent an average of five injections and two PDT treatments in total over 16 months before the choroidal neovascular membrane (CNVM) stabilized and became inactive for at least 1 year. Prior to the effective anti-VEGF/PDT combination therapy the median Snellen visual acuity ranged from 20/200 to 20/250 and presented at no worse than 20/200 at 1 year after treatment. Some subjects were followed for up to 5 years and remained inactive.Conclusion: Combination therapy can cause long-lasting closure of the CNVM, even with advanced disease resistant to anti-VEGF monotherapy. Keywords: anti-vascular endothelial growth factor, photodynamic therapy, age-related macular degeneration, bevacizumab, visual acuity, intraocular pressure

BACKGROUND AND OBJECTIVE: Intravitreal ocriplasmin has recently been used to treat vitreomacular traction, including macular holes. Recent safety concerns have arisen. PATIENTS AND METHODS: A retrospective chart review was conducted of the authors’ first nine patients (eight with macular holes) who were treated with intravitreal ocriplasmin. Standard clinical data were collected before and after injection, including electroretinography. RESULTS: Three of the nine patients had significant electroretinogram (ERG) depression; two of these had successful closure of the macular hole. Significant ERG depression, lasting up to 15 months, occurred in one patient. The third patient had moderate ERG depression but without macular hole closure. The six remaining patients experienced no change in visual acuity or ERG. CONCLUSION: This is one of the first reports of ocriplasmin for the treatment of macular hole including ERGs showing long-term loss of rod and cone function. Ocriplasmin successfully closed the macular hole in two of the eight patients (25%), and it was these same two patients who also experienced marked ERG depression. [ Ophthalmic Surg Lasers Imaging Retina . 2015;46:956–962.]