PMC4748786_01
Patient 1 (61-year-old Caucasian male). a Slit lamp photograph demonstrates abnormal nasal surface vasculature (asterisk). b Higher magnification slit lamp image shows the tumor (black arrowhead) with iris neovascularization (white arrowhead). c Scleral angiography early arterial phase shows laminar flow at 20 s. d Arteriovenous phase shows various areas of pinpoint and linear hyperfluorescence at 35 s. e Diffuse fluorescein signal is seen with leakage at 180 s. f Scleral angiography at 35 s in the left eye demonstrates normal and organized vascular patterns with clear intervening regions. g Upon closer examination of the abnormal nasal region, vascular changes (black arrowheads) are seen intermixed with relatively normal appearing sclera (asterisk). h Scleral angiography focusing on the background of normal appearing sclera (white asterisk; black asterisk depicted in g) shows a deep lacy complex of thick and abnormal vessels. White arrowheads in h correspond with black arrowheads in g. OS = Left eye.
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F1
Slit lamp examination revealed large dilated conjunctival and episcleral vessels nasally (fig. 1a, g) intermixed with regions of grossly normal appearing sclera. The anterior chamber exam showed an amelanotic mass with intralesional dilated vasculature in the superonasal quadrant which appeared to have eroded through the iris root into the anterior chamber focally shallowing the anterior chamber (fig. 1b). There was 360 degrees of iris neovascularization (fig. 1b) and a cataract with posterior synechiae. Early arterial phase (<20 s) led to arteriovenous phase (<35 s) culminating in eventual leakage over the region (fig. 1c-e). The left eye showed normal organized vascular arcades with clear intervening regions (fig. 1f). While large, tortuous, and abnormal vessels in the right eye were apparent clinically in the conjunctiva and sclera, scattered areas still appeared white and reminiscent of normal sclera (fig. 1g; black asterisk). However, when viewed under scleral angiography, these areas were actually abnormal with a deep network of lacy and dilated vessels suggestive of further pathology (fig. 1h; white asterisk).
PMC4748786_02
Patient 2 (57-year-old Hispanic female). a Dilation reveals a superonasally displaced white cataractous lens (arrow), with tumor (white arrowhead) and iris root changes (black arrowhead). b Trace hyperemia of conjunctiva (asterisk). c, d Scleral angiography early arterial phase at 20 s. e, f Arteriovenous phase with few pinpoint leakage at 35 s. g, h Continued scleral angiographic signal with expanding areas of vascular leaks at 90 s. Curved white lines denote the limbus. OD = Right eye; OS = left eye.
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F2
Slit lamp examination revealed trace hyperemia of the conjunctiva and a pigmented lesion inferotemporally behind the iris, involving the iris root, and with anterior bowing of the iris (fig. 2a, b). With dilation, the inferotemporal pigmented lesion (fig. 2a), which was more readily apparent, was associated with a superonasally subluxed white cataractous lens (fig. 2a). Scleral angiography in patient 2 demonstrated early arterial phase that quickly included venous filling with pinpoint leaks that expanded gradually with time (fig. 2c-h).
Histological evaluation of tumors in patients 1 (a-d) and 2 (e, f). a Hematoxylin-eosin (HE) of patient 1's tumor demonstrates spindle cells and pigment deposition from the ciliary body melanoma. b Tumor invasion at the tumor/scleral border into the inner scleral bed (arrow). c An abnormal and large scleral vessel with associated tumor cells. d More subtle vessels (arrows) with associated abnormal cells in otherwise normal appearing sclera. Scale bars in a-d = 30 mum. e HE of patient 2's tumor demonstrates that its location (arrow) is separated from the scleral wall (arrowhead) by the ciliary body (asterisk). f Normal appearing sclera in patient 2's right eye. Scale bars in e, f = 100 mum.
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F3
For patient 1, histopathologic examination confirmed the diagnosis of a ciliary body melanoma showing pigment deposition intermixed with spindle cells (fig. 3a). The tumor bordered the scleral wall and points of tumor and vascular infiltration into the sclera were identified (fig. 3b) leading to scattered regions of abnormal larger (fig. 3c) and smaller (fig. 3d) intrascleral vessels with associated abnormal cells. For patient 2, histopathologic examination revealed a ciliary epithelial adenoma (fig. 3e) with resultant compression of the iris, iridocorneal adhesions, and obliteration of the angle. The sclera demonstrated normal collagen bundles and vasculature throughout (fig. 3f).