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ENHANCED DEPTH IMAGING OPTICAL COHERENCE TOMOGRAPHY OF INTRAOCULAR TUMORS: From Placid to Seasick to Rock and Rolling Topography—The 2013 Francesco Orzalesi Lecture

Shields, Carol L. MD*; Pellegrini, Marco MD; Ferenczy, Sandor R. CRA*; Shields, Jerry A. MD*

doi: 10.1097/IAE.0000000000000288
Review

Purpose: To review enhanced depth imaging optical coherence tomography of intraocular tumors.

Methods: Review of tumor surface topography and internal characteristics based on published reports and personal experience.

Results: Using enhanced depth imaging optical coherence tomography, choroidal nevus showed smooth moderate dome-shape and with overlying retinal pigment epithelial alterations, subretinal cleft, and photoreceptor loss. Choroidal melanoma was smooth, moderately dome-shaped, and with overlying “shaggy” photoreceptors. Choroidal metastasis showed “lumpy, bumpy” irregular surface topography, subretinal fluid, and shaggy photoreceptors. Choroidal hemangioma was smooth, acutely dome-shaped and with subretinal fluid and/or cystoid retinal edema. Choroidal lymphoma showed “placid, rippled, or seasick” surface, correlating with increasing tumor thickness. Choroidal osteoma displayed smooth undulating surface with intralesional lamellar lines and tubules, representing bone lamellae or vessels. Choroidal melanocytosis produced flat but uniformly thickened choroid with increased stromal density. Choroidal hemorrhage displayed slightly “scalloped” surface in the outer choroid. All choroidal tumors showed inward compression of the choroidal vasculature, except for hemangioma in which the vessels were expanded. Sclerochoroidal calcification arose within the sclera as a “rocky” or “rolling” topography and solitary idiopathic choroiditis appeared as a domed or “volcanic” focal scleral thickening, each causing intense choroidal compression. Retinal tumors such as small retinoblastoma, astrocytic hamartoma, and hemangioblastoma arose abruptly adjacent to normal retina. Exophytic retinoblastoma and retinal hemangioblastoma depicted a full-thickness disorganized retinal mass with normal retina draped over the margins. Flat astrocytic hamartoma arose within the nerve fiber layer, and thicker tumors involved full-thickness retina with “moth-eaten” or cavitary appearance. Retinal pigment epithelial lesions such as congenital hypertrophy of retinal pigment epithelial showed flat topography with transmission of light through lacunae, occasional subretinal cleft and uniform photoreceptor loss, whereas combined hamartoma of retina/retinal pigment epithelial showed “sawtooth” pattern of vitreoretinal traction leading to mini-peak or maxi-peak retinal folds.

Conclusions: Enhanced depth imaging optical coherence tomography shows characteristic topographical and intralesional patterns that appear to be suggestive for selected intraocular tumors.

Enhanced depth imaging optical coherence tomography depicts tumor topography as dome-shaped for choroidal nevus and small melanoma, slightly “lumpy bumpy” for metastasis, “placid, rippled, or seasick” for lymphoma, “sawtooth” for combined hamartoma, and “rock and rolling” for sclerochoroidal calcification.

*Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania; and

Eye Clinic, Department of Biomedical and Clinical Science “Luigi Sacco,” University of Milan, Luigi Sacco Hospital, Milan, Italy.

Reprint requests: Carol L. Shields, MD, Ocular Oncology Service, Suite 1440, Wills Eye Institute, 840 Walnut Street, Philadelphia, PA 19107; e-mail: carol.shields@shieldsoncology.com

Supported by Eye Tumor Research Foundation, Philadelphia, PA (C.L.S. and J.A.S.), Lift for a Cure, Morrisdale, PA (C.L.S. and J.A.S.), and the Lucille Wiedman fund for Pediatric Eye Cancer, Philadelphia, PA (J.A.S., C.L.S.). The funders had no role in the design and conduct of the study, in the collection, analysis, and interpretation of the data, and in the preparation, review, or approval of the manuscript.

None of the authors have any conflicting interests to disclose.

Presented in part as the 2013 Francesco Orzalesi Lecture, Milan, Italy, December 12, 2013 (C.L.S.).

C. L. Shields, has had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.

© 2014 by Ophthalmic Communications Society, Inc.