Purpose: To demonstrate outer retinal tubulation (ORT) in various degenerative retinal disorders.
Methods: This was a retrospective review of the multimodal imaging of 29 eyes of 15 patients with various retinal dystrophies and inflammatory maculopathies manifesting ORT. The morphologic features of ORT and its evolution over time were analyzed using spectral-domain optical coherence tomography data.
Results: Outer retinal tubulation was identified as round or ovoid structures with hyperreflective borders in pattern dystrophy (six eyes), acute zonal occult outer retinopathy (five eyes), retinitis pigmentosa (four eyes), Stargardt disease (four eyes), gyrate atrophy (two eyes), choroideremia (two eyes), and various other degenerative conditions. These structures appeared to develop from the invagination of photoreceptors at the junction of intact and atrophic outer retina. During follow-up, the number and distribution of ORT largely remained stable. As zones of atrophy enlarged, the frequency of ORT appeared to increase. The ORT structures were found in <10% of patients with retinitis pigmentosa, Stargardt disease, or pattern dystrophy.
Conclusion: Outer retinal tubulation is found in various degenerative retinal disorders that share in common damage to the outer retina and/or retinal pigment epithelium. The presence of ORT may be an indicator of underlying disease stage and severity.
Outer retinal tubulation is demonstrated in a variety of degenerative retinal disorders without choroidal neovascularization.
*Vitreous, Retina, Macula Consultants of New York, New York, New York;
†Department of Ophthalmology, Mount Sinai School of Medicine, New York, New York;
‡Edward S. Harkness Eye Institute, Columbia University, New York, New York; and
§Department of Ophthalmology, New York University School of Medicine, New York, New York.
Reprint requests: K. Bailey Freund, MD, Vitreous, Retina, Macula Consultants of New York, 460 Park Avenue, 5th Floor, New York, NY 10022; e-mail: firstname.lastname@example.org
Supported in part by the Macula Foundation, Inc. The funding organization had no role in the design or conduct of this research.
K.B. Freund is a consultant (H) to Genentech, Heidelberg Engineering, and Regeneron.