Faculty Bibliography

PURPOSE: To develop an anatomical classification scheme for combined hamartoma of the retina and retinal pigment epithelium (RPE) and specify recommendations for follow-up interval. METHODS: Retrospective review of patients with combined hamartoma of the retina and RPE examined during a 7-year period (2008-2015). The clinical presentation, fundus examination, and optical coherence tomography were analyzed. RESULTS: Lesions were classified based on location, fundus features, and optical coherence tomography findings. Lesion location: macular/peripapillary-Zone 1; mid-periphery-Zone 2; and far periphery-Zone 3. Associated fundus findings: no retinal traction-Stage 1; retinal traction and/or retinoschisis-Stage 2; and retinal detachment-Stage 3. Optical coherence tomography findings: epiretinal component only-A; partial retinal involvement-B; and complete retinal and RPE involvement-C. Complete ophthalmologic evaluation is recommended at least every 6 months for patients younger than 12 years, with more frequent follow-up in patients with: lesions in the macula/peripapillary (Zone 1) or with retinal traction, retinoschisis, or retinal detachment (Stage 2 and 3). Surgical intervention is recommended in patients with vision loss secondary to macular traction or retinal detachment. CONCLUSION: A new clinical classification system is proposed for evaluating and managing patients with combined hamartoma of the retina and RPE. The zone and stage of combined hamartoma of the retina and RPE lesion will assist in determining follow-up interval and surgical intervention. Application of a uniform classification scheme will facilitate assessment and comparison of findings across different studies.

PURPOSE: To report an unusual case of ciliochoroidal effusion and presumed acute macular neuroretinopathy associated with cervical traction therapy for the treatment of cervical spinal stenosis. METHODS: Case report. RESULTS: A 75-year-old man reported sudden onset of a wedge-shaped paracentral scotoma in the right eye. Fundus examination showed a ciliochoroidal effusion in the right eye. Optical coherence tomography revealed intraretinal fluid in both eyes without leakage on fluorescein angiography. B-scan ultrasonography and anterior segment ultrasound biomicroscopy of the right eye showed peripheral ciliochoroidal effusion. He had recently started intensive cervical traction therapy for the treatment of cervical spinal stenosis. There was spontaneous resolution of the choroidal effusion and intraretinal fluid after stopping cervical traction treatments. Optical coherence tomography imaging after resolution of the intraretinal fluid revealed thinning of the outer nuclear layer and attenuation of the ellipsoid and interdigitation zones corresponding to a persistent paracentral scotoma, consistent with acute macular neuroretinopathy. CONCLUSION: This is the first report of adverse ocular effects of cervical traction. We postulate that venous and arterial compromise during cervical traction therapy resulted in both ciliochoroidal effusion and a watershed infarct in the outer retina.

PURPOSE: Kinetic visual field testing is used to monitor disease course in retinal dystrophy clinical care and treatment response in treatment trials, which are increasingly recruiting children. This study investigates Goldmann visual field (GVF) changes in young children with mutation-proven retinal dystrophies as they age and with progression of the retinal degeneration. METHODS: Retrospective review of children /= 20%, the criterion for positive treatment outcome defined in recent published therapeutic trials, in 38% (I4e), 34% (III4e), and 33% (IV4e) of eyes. CONCLUSION: In a substantial proportion of children with mutation-proven retinal dystrophies, there is a significant increase in GVF area with age, particularly those < 12 years, despite progression or stability of disease. These findings suggest that change in GVF area in children with retinal dystrophies can be an unreliable measure of response to treatment and on which to base appropriate counseling about visual impairment.

PURPOSE/OBJECTIVE:To report an unusual case of postcataract endophthalmitis secondary to Bosea thiooxidans. METHODS:Case report. RESULTS:An 86-year-old man presented with postoperative endophthalmitis 3 weeks after uncomplicated cataract surgery. He complained of a headache and blurry vision but denied having eye pain. Slit-lamp examination demonstrated leukocytes with a fibrinous membrane in the anterior segment and vitritis. A diagnosis of acute postoperative endophthalmitis was made, and the patient was treated with intravitreal antibiotics. Culture of vitreous tap revealed Bosea thiooxidans, a gram-negative rod found in soil and water systems. CONCLUSION/CONCLUSIONS:To our knowledge, this report is the first to describe Bosea thiooxidans endophthalmitis and one of a few reports of human infection with this microbial agent.

PURPOSE: To summarize key concepts, as well as early safety and efficacy signals from clinical trials, for stem/progenitor cell-based interventions for retinal disease. DESIGN: Interpretive essay. METHODS: Review and synthesis of selected recent reports of stem/progenitor cell-based approaches for retinal disease, with interpretation and perspective. RESULTS: Stem/progenitor cell-based interventions represent a novel class of potential therapies for retinal diseases, such as age-related macular degeneration and inherited retinal dystrophies, aoong others. Sources include pluripotent stem cells and fetal and postnatal tissues. Two mechanisms of "rescue" have been proposed: regenerative or trophic. Although pluripotent and fetal sourced-cell types have been tested in preclinical animal models of retinal disease, many postnatal stem/progenitor cell populations currently in trial do not have preclinical safety or efficacy data. Some early-phase trials of cell therapies suggest acceptable safety profiles. Other reports, involving some types of autologous, nonocular cell sources, have been linked to severe, blinding complications. Larger trials will be needed to determine short-term and long-term safety and efficacy of these cell-based interventions. CONCLUSIONS: Stem/progenitor cell-based interventions have the potential to address blinding retinal diseases that affect hundreds of millions worldwide. Yet no Food and Drug Administration-approved stem cell therapies for retinal disease exist. Although some early-phase trial data are promising, reports of blinding complications from cell interventions remain troubling. It is paramount to apply a strong level of scientific rigor toward a well-planned, step-wise sequence of preclinical and clinical studies, to determine whether this class of potential therapies will be safe and effective for individuals with retinal diseases.