Presentation
 
Figure 1. A 53-year-old woman was noted to have (A) left hypoglobus, exotropia and proptosis from an orbital mass superomedially. Magnetic resonance imaging revealed a well-circumscribed orbital mass
compressing the globe with (B) low signal on axial T1 imaging,
(C) heterogeneous signal on T1 with gadolinium enhancement and (D) slightly heterogeneous signal with crisp margins on T2 imaging.
A 53-year-old Caucasian female presented to her retina specialist, with whom she had been following for a left choroidal nevus, with new complaints of painless diplopia. She had been having progressive symptoms for the past four months, and noted associated swelling of the left upper lid. (Figure 1). The retina specialist pursued magnetic resonance imaging of the orbit, which revealed a large, well-circumscribed mass in the left superonasal orbit, compressing the globe and measuring 30 mm anteroposteriorly. The mass was low signal on T1 and demonstrated heterogenous signal on the T1 series with fat-suppresion and gadolinium enhancement. (Figure 1). The T2 series revealed a slightly heterogeneous mass with crisp margins. The patient was referred to the Ocular Oncology Service at Wills Eye Hospital for further evaluation and management.
 
Figure 2. Fundus photography of the right eye (A) showing a tilted disc and otherwise normal findings. The left eye (B) revealed a tilted disc with prominent chorioretinal folds, tortuous retinal vessels, blunted foveal reflex and retinal pigment epithelial mottling.


Medical History
The patient had a past medical history of seasonal allergies for which she took loratadine as needed. She did not have any additional ocular history or chronic medications. There was no personal or family history of cancer. She had no history of tobacco smoking or excessive alcohol intake. The review of systems was unremarkable.
 
Examination
On ocular examination, her best corrected visual acuity was 20/20 in the right eye and 20/40 in the left. Intraocular pressure was 14 mmHg in both eyes. There was no afferent pupillary defect, and Ishihara color plates were full (8/8) OU. Hertel exophthalmometry demonstrated 5 mm of relative proptosis on the left.

An external exam revealed a downwardly displaced globe OS with a 3-cm, firm, palpable orbital mass underlying the medial portion of the left upper eyelid. Extraocular motility was full OD and reduced by 50 percent in elevation, adduction and depression OS.

Anterior segment slit lamp examination was normal OU. Funduscopic examination OD was only remarkable for a slightly tilted disc (Figure 2). Funduscopic examination OS revealed prominent chorioretinal folds, a tilted disc, tortuous retinal vessels and a blunted foveal reflex (Figure 2). The cup-to-disc ratio was 0.2 OU.
 


Optical coherence tomography OD was normal (Figure 3). OCT OS revealed prominent chorioretinal folds concentrated in the papillomacular region. There was additional intraretinal cystoid edema and disruption of the outer retinal layers (Figure 3).

Fluorescein angiography was normal OD, and displayed the clinically evident chorioretinal folds OS. Unfortunately, the early phase was not captured, but FA at 23 seconds showed alternating bands of hyper- and hypofluorescence corresponding to the obliquely oriented chorioretinal folds OS. FA at seven minutes showed a similar pattern for the folds with no obvious leakage (Figure 3).

Fundus autofluorescence OS clearly demonstrated a similar configuration of alternating hyper- and hypo-autofluorescence representing shifting of the RPE along the folds, with liopofuscin showing a bright signal (Figure 3).

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