Purpose: To investigate the alterations in the retinal pigment epithelium (RPE) in the crest and trough portions of chorioretinal folds (CRFs) induced by an orbital vascular tumor. Methods: Review of multimodal imaging in two eyes of two patients with globe compression and CRFs from an orbital vascular tumor. Results: Fundus photography demonstrated obliquely extending CRFs with alternating hyperpigmented and hypopigmented linear alterations in both eyes. Fundus autofluorescence (AF) imaging showed obliquely oriented hypoAF lines, incompletely alternating with hyperAF lines. In Case 1, the hyperAF lines had interspersed hypoAF segments and Case 2 had peripapillary mottling of AF. Fluorescein angiography (FA) showed alternating hyper and hypofluorescent lines in the late phase in Case 1. Optical coherence tomography (OCT) documented relative thinning of RPE at the folded crests in Case 1 and preservation of RPE in Case 2. Swept-source OCT angiography (SS-OCTA) demonstrated oblique hyporeflective lines in the outer retina and choriocapillaris layers in Case 2. These findings suggest that the crest of a CRF represents thinned or rarified RPE with hypoAF, transmission hyperfluorescence (FA), partially attenuated RPE layer (OCT), and isoreflectivity (SS-OCTA) while the trough represents compressed RPE with irregular hyperAF, transmission hypofluorescence (FA), thickened RPE layer (OCT), and hyporeflectivity (SS-OCTA). Conclusion: The anatomic and functional status of the RPE in CRFs based on multimodal imaging reveals normal to attenuated RPE with hypofunctionality at the fold crest and compacted, thickened RPE at the trough with segmental functional impairment on AF imaging. Anatomic information regarding CRFs is evident on OCT, FA, and SS-OCTA while the functional status is depicted on AF.

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A 43-year-old patient presented with painless proptosis, limited upgaze, and vision loss in the right eye. Funduscopic examination revealed right optic disc edema and subtle macular compression. Swept-source optical coherence tomography (SS-OCT) revealed a smooth contoured elevation of the posterior pole without any distortion of retinal structures, an appearance closely simulating dome-shaped maculopathy. Swept-source optical coherence tomography angiography (SS-OCTA) revealed normal retinal and choroidal vasculature. Orbital magnetic resonance imaging demonstrated a well-circumscribed intraconal mass compressing the globe and optic nerve in the right orbit. An anterior orbitotomy was performed, whereby the tumor was totally excised and diagnosed histopathologically as cavernous hemangioma. This case represents an orbital cavernous hemangioma touching the eyeball and producing compression of the posterior pole presenting with a dome-shaped maculopathy-like appearance on SS-OCT. SS-OCT and SS-OCTA are important noninvasive tools for evaluating the retinal and choroidal effects in orbital tumors.

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A 22-year-old woman presented with progressive swelling of the nasal conjunctiva in the left eye. Anterior segment examination revealed a diffuse cystic appearance to the inferonasal bulbar conjunctiva and plica semilunaris. Anterior segment swept-source optical coherence tomography (OCT) revealed clear hyporeflective spaces demarcated by hyperreflective septae in the affected conjunctiva, consistent with the diagnosis of lymphatic malformation (LM). Magnetic resonance imaging revealed a well circumscribed intraconal mass located inferonasally in the left orbit. Systemic examination revealed a lesion similar to LM on the left hard palate. The left conjunctival mass was excised subtotally. Subsequently, a transconjunctival anterior orbitotomy was performed and the left orbital mass was completely removed intact. Histopathologically, the conjunctival mass was diagnosed as LM and the orbital mass as venous malformation (VM). This case represents a rare coexistence of histopathologically proven conjunctival LM and orbital VM as well as a presumed LM of the hard palate, all 3 lesions occurring in the ipsilateral midface area.