PURPOSE: The treatment of retinopathy of prematurity (ROP) is not standardized and can vary significantly between providers. This study aims to determine preferred practices in treating ROP by globally surveying pediatric ophthalmologists. METHODS: Between January and February 2017, an international pediatric ophthalmology interest group was invited to complete an anonymous survey of 18 questions. The main objectives were to determine the preferred first line of treatment for ROP, the preferred dosage of intravitreal bevacizumab (IVB) used, and the outcome and possible complications following bevacizumab injection. RESULTS: Out of 101 pediatric ophthalmologists, 72 (71.8%) stated that they had direct involvement in the treatment of ROP. When presented with type 1 ROP which requires treatment, 69 ophthalmologists (68.3%) stated that they prefer laser treatment over bevacizumab, and 33 ophthalmologists (32.7%) stated they would recommend bevacizumab as a first choice. Ninety-three ophthalmologists (92.1%) reported the success of 1 laser treatment between 75% and 100%, and 35 ophthalmologists (34.7%) perceive bevacizumab to be 75%-100% successful. Half dose of adult-prescribed bevacizumab at 0.625 mg/0.05 mL was preferred by 47 of the ophthalmologists (46.5%). No cases of endophthalmitis were reported with intravitreal injection. CONCLUSION: Laser photoablation remains the preferred mode of treatment for ROP among surveyed ophthalmologists across the world. Though bevacizumab is currently being used, this form of treatment is not as common, primarily due to the unknown safety profile and potential long-term ramifications of the drug.

BACKGROUND: Sjögren-Larsson syndrome (SLS) is a rare genetic neurocutaneous disease caused by mutations in ALDH3A2 that results in deficiency of fatty aldehyde dehydrogenase and accumulation of fatty aldehydes and alcohols. The disease is associated with ichthyosis, spasticity, and intellectual disability. Patients exhibit a characteristic retinopathy with macular crystalline inclusions that first appear in early childhood and increase with age. Once formed, the inclusions are thought to be inert and irreversible. We sought to document how the crystalline inclusions change over time. MATERIALS AND METHODS: Serial retinal photographs of 4 SLS subjects (9-23 years old) were taken over a period of 1-3 years. Images were compared by visual inspection and analyzed using ImageJ/Fiji software to observe changes. RESULTS: Visual inspection of retinal photographs of SLS subjects taken over time demonstrated distinctive changes in crystalline inclusions. New inclusions were formed and some established inclusions regressed. These changes were conveniently demonstrated with software-based photographic image analysis. CONCLUSIONS: We conclude that macular inclusions in SLS are not simply inert deposits, but are dynamic structures that form over time and are subject to remodeling. This conclusion provides new insight into the interplay between the metabolic defect and retinal pathology in SLS, and raises the potential for new therapeutic approaches to reverse some aspects of the maculopathy.