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9 RetCam™ Imaging of Pediatric Intraocular Tumors |
9.2 RetCam™ Imaging for Specific
Pediatric Intraocular Tumors
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9.2.1 Retinal Tumors |
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There are several retinal tumors that can occur in children |
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including retinoblastoma, astrocytic hamartoma, acquired |
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astrocytoma, and the vascular tumors such as retinal |
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hemangioblastoma, cavernous hemangioma, racemose |
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hemangioma, and vasoproliferative tumor [18, 19]. |
9.2.1.1 Retinoblastoma
Retinoblastoma is the most common intraocular malignancy of childhood. It affects approximately 300 children in the USA each year and about 5,000 children worldwide. If detected while the tumor is contained within the eye, survival is excellent (Fig. 9.3). Risks for metastases include optic nerve invasion, choroidal invasion, scleral invasion, anterior chamber invasion, and orbital invasion. Treatment of retinoblastoma included enucleation,
Fig. 9.3 Retinoblastoma imaged with RetCam™. Macular retinoblastoma before (a) and after chemoreduction (b). Multifocal retinoblastoma before (c) and after chemoreduction (d). Iris neovascularization in an eye with retinoblastoma imaged on RetCam™ with anterior segment photography (e) and fluorescein angiography (f)
9.2 RetCam™ Imaging for Specific Pediatric Intraocular Tumors |
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Fig. 9.4 Simulators of retinoblastoma imaged with RetCam™. Macular astrocytic hamartoma (a) showing minimal angiographic fluorescence (b). Retinal hemangioblastoma (c) with marked exudative retinopathy showing multifocal leakage on fluorescein angiography (d). Coats disease with macular exudation (e) showing peripheral light bulb telangiectasia, retinal edema, and nonperfusion (f)
chemoreduction plus thermotherapy or cryotherapy, intraarterial chemotherapy, external beam radiotherapy, plaque radiotherapy, laser photocoagulation, and cryotherapy [20]. Enucleation is often necessary for unilateral retinoblastoma, whereas chemoreduction is used for most bilateral cases.
9.2.1.2 Astrocytic Hamartoma
Retinal astrocytic hamartoma is a tumor of glial origin and can be found in patients with tuberous sclerosis or neurofibromatosis. In some instances, it is sporadic. Astrocytic hamartoma tends to develop in the nerve fiber
layer and can cause slight traction with minimal if any dilation of the retinal vessels (Fig. 9.4).
9.2.1.3 Retinal Capillary Hemangioma
(hemangioblastoma)
Retinal capillary hemangioma (hemangioblastoma) is a reddish orange, vascular tumor that can produce retinal and vitreous exudation, subretinal fluid, and macular edema, often leading to poor visual acuity [21] (Fig. 9.4). New genetic information has found that this tumor is stimulated by vascular endothelial growth factor (VEGF) that is secreted by the stromal tumor cells. This tumor