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. 2013 Oct;33(9):1871-6.
doi: 10.1097/IAE.0b013e318296b12f.

Outer retinal tubulation in degenerative retinal disorders

Naomi R Goldberg  1 , Jonathan P Greenberg, Ketan Laud, Stephen Tsang, K Bailey Freund
Affiliations

Outer retinal tubulation in degenerative retinal disorders

Naomi R Goldberg et al. Retina. 2013 Oct.

Abstract

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.

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Figures

Figure 1
Figure 1
Various features of outer retinal tubulation (ORT) in retinal degenerative disorders. A, Color photograph of the right eye of a patient with pattern dystrophy. Spectral-domain optical coherence tomography (SD-OCT) scans corresponding to the solid black lines, showing circular ORT structures with hyper-reflective borders (yellow arrows) within B, a zone of geographic atrophy, and C, at the junction of preserved and disrupted outer retina. D, Color photograph of the left eye of a patient with thioridazine toxicity. E, corresponding SD-OCT scan, and F, scan of the fellow eye, showing both oblong and circular ORT structures. The retina overlying the ORT structures in B and F appears slightly elevated and distorted.
Figure 2
Figure 2
Evolution of outer retinal tubulation (ORT) over time. Eye-tracked spectral-domain optical coherence tomography (SD-OCT) scans for varying conditions at baseline (top row) and follow-up (bottom row). A, B, SD-OCT scans of an eye with choroideremia at baseline and 35 months later, showing the development of ORT from the gradual invagination of outer retinal structures, at the junction of intact and atrophic retina. C,D, SD-OCT scans of an eye with Stargardt disease, showing long and ovoid ORT structures evolving into smaller round structures, over a period of 5 months.
Figure 3
Figure 3
Development of outer retinal tubulation (ORT) with progressive atrophy in an eye with pattern dystrophy. Progressive infrared photographs (left) and corresponding spectral-domain optical coherence tomography (SD-OCT) scans (right) at A, baseline, B, 29 months, and C, 42 months. As the area of outer retinal atrophy enlarges (yellow arrows), there is progressive appearance of the ORT structures.
Figure 4
Figure 4
Outer retinal tubulation (ORT) in near proximity to sites of cystoid macular edema (CME) in various degenerative disorders. A, Fundus autofluorescence (left) and corresponding spectral-domain optical coherence tomography (SD-OCT) (right) of an eye with pattern dystrophy, which highlights the distinguishing features of ORT (arrows), such as hyperreflective borders and positioning within the outer nuclear layer, from the findings of CME (black ovals). B-C, Fundus photographs (left) and SD-OCTs (right) of eyes with B, choroideremia, and C, gyrate atrophy.
Figure 5
Figure 5
Fundus autofluorescence imaging showing ORT-containing hyperautofluorescent material within larger atrophic areas. Autofluorescence (left) with corresponding spectral-domain optical coherence tomography scans (right), of A, left eye of a 51 year-old man with thioridazine toxicity, and B, right eye of a 41 year-old man with pattern dystrophy.
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References

    1. Zweifel SA, Engelbert M, Laud K, et al. Outer retinal tubulation: a novel optical coherence tomography finding. Arch Ophthalmol. 2009;127(12):1596–602. - PubMed
    1. Curcio CA, Medeiros NE, Millican CL. Photoreceptor loss in age-related macular degeneration. Invest Ophthalmol Vis Sci. 1996;37(7):1236–49. - PubMed
    1. Wolff B, Matet A, Vasseur V, et al. En face OCT imaging for the diagnosis of outer retinal tubulations in age-related macular degeneration. J Ophthalmol. 2012;2012:542417. - PMC - PubMed
    1. Ellabban AA, Hangai M, Yamashiro K, et al. Tomographic fundus features in pseudoxanthoma elasticum: comparison with neovascular age-related macular degeneration in Japanese patients. Eye. 2012;26(8):1086–94. - PMC - PubMed
    1. Sergouniotis PI, Davidson AE, Lenassi E, et al. Retinal structure, function, and molecular pathologic features in gyrate atrophy. Ophthalmology. 2012;119(3):596–605. - PubMed

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