Juvenile xanthogranuloma can involve the eye — a presentation that carries meaningful risk of vision loss if not managed appropriately. This protocol covers the clinical scenario of ocular JXG and the stepwise approach when complications arise.
Ocular juvenile xanthogranuloma is nearly always unilateral. The iris is the most commonly affected structure, though lesions may also occur in the orbit, optic nerve, choroid, and conjunctiva. Complications such as glaucoma or hyphema can develop and may require prompt intervention.
For rapidly progressive lesions or sight-threatening complications, the protocol involves systemic therapy as part of the management pathway. The complete decision algorithm — covering the full range of options, how they are selected, and under what circumstances each applies — is available in the structured protocol.
DOI: 10.1016/j.adengl.2020.10.007
Ocular JXG is nearly always unilateral and generally affects the iris, although the orbit, optic nerve, choroid, and conjunctiva may also be affected.
Topical and intralesional corticoids are usually used to treat ocular JXG, while systemic corticosteroids or surgery may be needed to treat rapidly progressive lesions or complications, such as glaucoma or hyphema; there have also been isolated reports of good response to radiation therapy and chemotherapy.
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