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Review
, 27 (2), 208-16

Imaging of Retinal and Choroidal Vascular Tumours

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Review

Imaging of Retinal and Choroidal Vascular Tumours

H Heimann et al. Eye (Lond).

Abstract

The most common intraocular vascular tumours are choroidal haemangiomas, vasoproliferative tumours, and retinal haemangioblastomas. Rarer conditions include cavernous retinal angioma and arteriovenous malformations. Options for ablating the tumour include photodynamic therapy, argon laser photocoagulation, trans-scleral diathermy, cryotherapy, anti-angiogenic agents, plaque radiotherapy, and proton beam radiotherapy. Secondary effects are common and include retinal exudates, macular oedema, epiretinal membranes, retinal fibrosis, as well as serous and tractional retinal detachment, which are treated using standard methods (ie, intravitreal anti-angiogenic agents or steroids as well as vitreoretinal procedures, such as epiretinal membrane peeling and release of retinal traction). The detection, diagnosis, and monitoring of vascular tumours and their complications have improved considerably thanks to advances in imaging. These include spectral domain and enhanced depth imaging optical coherence tomography (SD-OCT and EDI-OCT, respectively), wide-angle photography and angiography as well as wide-angle fundus autofluorescence. Such novel imaging has provided new diagnostic clues and has profoundly influenced therapeutic strategies so that vascular tumours and secondary effects are now treated concurrently instead of sequentially, enhancing any opportunities for conserving vision and the eye. In this review, we describe how SD-OCT, EDI-OCT, autofluorescence, wide-angle photography and wide-angle angiography have facilitated the evaluation of eyes with the more common vascular tumours, that is, choroidal haemangioma, retinal vasoproliferative tumours, and retinal haemangioblastoma.

Figures

Figure 1
Figure 1
Colour and wide-angle imaging of vascular tumours. (a) Circumscribed haemangioma recorded with the Optomap system. The tumour appears darker and browner and mimics a choroidal melanoma. (b) Same tumour as in (a) imaged with the Panoret camera. A vivid reddish colour of the haemangioma is noted. (c) Peripheral retinal haemangioblastoma imaged with the Panoret camera. The peripheral angioma, dilated feeder vessels, the posterior pole with macular oedema, the optic disc, and the preretinal fibrosis over the tumour can all be documented in one photograph. (d) Vasoproliferative retinal tumour in the temporal periphery imaged with the Panoret system. Typical associated exudative retinal changes and pigmentary changes of the RPE.
Figure 2
Figure 2
Multimodal imaging of circumscribed choroidal haemangioma. (a) Panoret wide-angle fundus photograph demonstrating the circumscribed choroidal haemangioma. (b) Wide-angle 50° fluorescein angiography demonstrating hyperfluorescence of the haemangioma in the arterio-venous phase. (c) SD-OCT with fibrotic changes in the RPE layer. The tumour is not visualized with standard SD-OCT settings. (d) Fundus autofluorescence showing hyperfluorescence of the clinically visible orange pigment over the haemangioma.
Figure 3
Figure 3
SD-OCT in the management of circumscribed choroidal haemangioma. (a) Pre-treatement Optomap picture of a circumscribed choroidal haemangioma above the disc. (b) SD-OCT over the haemangioma, demonstrating fluid between the photoreceptor and the RPE layer. The RPE layer is interrupted. No details of the haemangioma can be visualized. (c) SD-OCT over the fovea. The exudative detachment extends into the fovea. (d) Same patient 8 months following PDT using standard parameters. On the Optomap picture, no significant differences can be seen. (e) SD-OCT over the tumour following PDT. Regression of subretinal fluid and thinning of the retinal layers over the haemangioma. (f) SD-OCT over the fovea following PDT. Complete regression of the subretinal fluid. No indication for additional treatment.
Figure 4
Figure 4
Patient with multiple retinal haemangioblastoma associated with von Hippel–Lindau syndrome in the left eye. The patient was referred following unsuccessful laser and cryotherapy. (a) At the initial examination in our institution, there was a large and active haemangioblastoma in the temporal periphery (arrow) associated with a vitreous haemorrhage (*) and a near-total exudative retinal detachment (arrowheads). (b) The wide-angle fluorescein angiography highlights more than 20 active haemangioblastoma (visible as leaking spots during the angiography). (c) Same patient 4 months after intitial treatment with brachytherapy and laser. Regression of the large angioma in the temporal periphery. Collection of lipoid exudates in the area of the previous exudative retinal detachment. (d) Same patient 17 months following brachytherapy and multiple sessions of laser photocoagulation. Complete regression of all previously active haemangioblastoma. Regression of the exudative retinal detachment and lipoid exudates.
Figure 5
Figure 5
Retinal haemangiblastoma on the disc not associated with von Hippel–Lindau syndrome. (a) Baseline fundus picture showing an endophytic peripapillary angioma with retinal exudates. (b) SD-OCT over the angioma. No retinal structures can be identified over the tumour. The angioma appears as an optically dense inner retinal mass with posterior shadowing. (c) SD-OCT over the macula. Intraretinal exudative changes and exudative retinal detachment involving the fovea. Visual acuity 6/18. (d, e) One year after PDT. (d) Fundus picture demonstrating a fibrotic reaction in the angioma following PDT. Visual acuity 6/9. (e) SD-OCT over the macula. Regression of intraretinal fluid and subretinal fluid following treatment.

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