Case #15 – Bietti with Outer Retinal Tubulations

Bietti with Outer Retinal Tubulations – Page 13 of 24

Cirrus™ HD-OCT OD Patient The PIL is present in every normal eye of the first 1000 or so imaged and abnormal in all eyes with outer retinal disease imaged to date. Normal Control

Bietti with Outer Retinal Tubulations – Page 13 of 242020-07-20T16:22:01+00:00

Bietti with Outer Retinal Tubulations – Page 11 of 24

Cirrus™ HD-OCT En Face OD We have found Outer Retinal Tubulations (ORTs) only in select eyes with outer retinal degenerations and never to date in a normal eye.

Bietti with Outer Retinal Tubulations – Page 11 of 242020-07-20T16:22:23+00:00

Bietti with Outer Retinal Tubulations – Page 10 of 24

Histological Section as Compared to the OCT Image  Although the photoreceptor integrity line, or the PIL (defined as the junction between the inner and outer segments) is barely visible in most histological sections, it is highly prominent in normal SD OCTs. The PIL, as shown above, should be continuous throughout the entire scan

Bietti with Outer Retinal Tubulations – Page 10 of 242020-07-20T16:22:38+00:00

Bietti with Outer Retinal Tubulations – Page 9 of 24

En face OCT above (akin to a fundus photo limited to a specific layer) suggests the round or oval bodies in cross section are really tubular in shape and are confined to the deep retina anterior to an intact BM.

Bietti with Outer Retinal Tubulations – Page 9 of 242020-07-20T16:22:50+00:00

Bietti with Outer Retinal Tubulations – Page 8 of 24

Topcon 3D OCT OS The fundus appearance is similar OS. In addition to the white dots on BM, multiple hollow circular bodies are found In the SD OCT cross sectional images. Large choroidal vessels are also visualized and are unmasked because of the loss of overlying tissue.

Bietti with Outer Retinal Tubulations – Page 8 of 242020-07-20T16:23:00+00:00

Bietti with Outer Retinal Tubulations – Page 7 of 24

Topcon 3D OCT OD Green arrow depicts a well defined Bruch's Membrane (BM). Note theextreme thinning of the overlying RPE and absence of the PIL. BM ismasked in normal eyes because of the presence of a normal RPE. The choroidal vasculature is observable above because much of the overlying RPE and choriocapillaris

Bietti with Outer Retinal Tubulations – Page 7 of 242020-07-20T16:23:14+00:00

Bietti with Outer Retinal Tubulations – Page 6 of 24

Optos ResMax™ images OD and OS Peripapillary "choroidal sclerosis" is quite prominent OU. The tiny white dots and pigmentary changes appear reduced in zones with the most prominent choroidal vessels.

Bietti with Outer Retinal Tubulations – Page 6 of 242020-07-20T16:23:26+00:00