The ODDS Consortium recommendation for diagnosing ODD using OCT is seen in Figure 5. As described previously, ODD always have a signal-poor, hyporeflective core. Without a signal-poor core other pathologies should be considered. ODD are often but not always seen with hyperreflective margins, most prominent superiorly. Sometimes collections of several smaller ODD coalesce to form ODD conglomerates that present with patchy internal reflectivity within a larger hyporeflective area (Fig. 3). Most likely, fragments of calcified margin cause the internal reflectivity from the many ODD that coalesce.
Hyperreflective horizontal lines are sometimes found in eyes with ODD or in otherwise healthy eyes in patients with unilateral ODD, and might possibly reflect early ODD changes.
The goal of the ODDS Consortium is to establish a reliable and consistent diagnosis of ODD using OCT for both clinicians and researchers. Better detection of ODD using OCT might, in time, improve differentiation between buried ODD and mild papilledema, but currently OCT requires further study to make it as the sole means of differentiation. The distinguishing OCT features between buried ODD and papilledema have been widely investigated (15,16,20,22–25), with some studies focusing on retinal nerve fiber layer (RNFL) thickness differences (15,16,20,23,24) and others on a subretinal hyporeflective space (22–24). The use of RNFL thickness to differentiate between the 2 was found to be clinically unreliable (16). This is supported by studies finding normal or increased RNFL thickness in patients with buried ODD (26). The use of the subretinal hyporeflective space to differentiate between the 2 is based on older OCT technology, which had limited penetrance. With newer OCT modalities such as EDI-OCT, there is no longer a subretinal hyporeflective space, but instead the entire optic nerve head is visible down to lamina cribrosa. This suggests that the previously described subretinal hyporeflective space is likely an imaging artifact, and should not be relied on to differentiate cases of ODD from papilledema.
The ODDS Consortium reached a consensus to define ODD as hyporeflective structures with a full or partial hyperreflective margin. This is in agreement with most studies assessing ODD morphology using OCT (9,27–30). Some studies, however, have described ODD as hyperreflective structures with no hyporeflective core (17,20,31) similar to what we, in the ODDS Consortium label PHOMS. These contradictory descriptions of ODD morphology using OCT have resulted in diagnostic confusion (32). After thoroughly reviewing and discussing the issues, the ODDS Consortium, finds that there is no substantial evidence to diagnose PHOMS as ODD. In fact there is histopathological evidence that PHOMS are lateral bulges of retinal nerve fibers (Fig. 4). However, there is a need to study PHOMS longitudinally to better understand how to classify these structures. Unless other evidence becomes available, we recommend not to diagnose these structures as ODD.
Another point of confusion is the hyperreflective horizontal lines often found in patients with ODD. Merchant et al (9) have suggested that the hyperreflective bands may be a sign of nascent ODD. This is supported in a study by Ghassibi et al (28). Including these isolated bands in the definition of ODD resulted in an ODD prevalence of 14.6% among 130 clinically normal subjects (28). This prevalence is several times higher than ever reported and should be interpreted with caution. As it is still unclear whether isolated hyperreflective bands are ODD, we recommend not diagnosing the hyperreflective horizontal lines as ODD until further evidence from longitudinal OCT studies emerges.
The ODDS Consortium recommendations hopefully will help researchers and clinicians to diagnose ODD. The recommendations are based on the individual experiences of the involved researchers, detailed study of the literature, 4 sets of ODD standardization steps, and discussions by the ODDS Consortium.
There are several limitations to this report. Our recommendations are based on a selection of relevant studies but not on systematic reviews of the literature. These recommendations might change as new evidence becomes available. The recommendations are based on the currently available OCT technology. Although we are able to visualize the optic nerve head down to lamina cribrosa there are still artifacts, “noise”, and shadowing from overlying ODD and protruding optic discs, which provide challenges in interpretation. Even with an agreement on ODD morphology using OCT, buried ODD are still difficult to detect reliably. Differentiating buried ODD from papilledema in some cases can be challenging. The absence of ODD on OCT does not entirely rule out the diagnosis nor does it support the diagnosis of papilledema. In such cases, other modalities are needed. These include fundus autofluorescence, B-scan ultrasonography, and other clinical and OCT signs of pathological disc edema such as peripapillary wrinkles and folds and alteration is the orientation of the peripapillary retinal pigment epithelium/Bruch’s membrane layers (33,34).
Future improvements in OCT technology will likely provide visualization of deeper ocular structures in higher resolution without shadowing and other imaging artifacts. We regard our recommendations as a preliminary, but an important step towards improving the diagnosis of ODD.
STATEMENT OF AUTHORSHIP
Category 1: a. Conception and design: L. Malmqvist, L. Bursztyn, F. Costello, K. Digre, J. A. Fraser, C. Fraser, B. Katz, M. Lawlor, A. Petzold, P. Sibony, J. Warner, M. Wegener, S. Wong, S. Hamann; b. Acquisition of data: L. Malmqvist, S. Hamann; c. Analysis and interpretation of data: L. Malmqvist, L. Bursztyn, F. Costello, K. Digre, J. A. Fraser, C. Fraser, B. Katz, M. Lawlor, A. Petzold, P. Sibony, J. Warner, M. Wegener, S. Wong, S. Hamann. Category 2: a. Drafting the manuscript: L. Malmqvist, S. Hamann; b. Revising it for intellectual content: L. Malmqvist, L. Bursztyn, F. Costello, K. Digre, J. A. Fraser, C. Fraser, B. Katz, M. Lawlor, A. Petzold, P. Sibony, J. Warner, M. Wegener, S. Wong, S. Hamann. Category 3: a. Final approval of the completed manuscript: L. Malmqvist, L. Bursztyn, F. Costello, K. Digre, J. A. Fraser, C. Fraser, B. Katz, M. Lawlor, A. Petzold, P. Sibony, J. Warner, M. Wegener, S. Wong, S. Hamann.
This study was supported by Fight for Sight, Denmark, Synoptik-Fonden, and in part by an unrestricted grant from Research to Prevent Blindness, Inc., New York, NY, to the Department of Ophthalmology & Visual Sciences, University of Utah. Collaborators: Nitza Goldenberg-Cohen, MD, Alison Crum, MD, Ruth Huna-Baron, MD, Hanna Leiba, MD, Hadas Stiebel-Kalish, MD, on behalf of the ODDS Consortium.
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© 2018 by North American Neuro-Ophthalmology Society
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