Juvenile Xanthogranuloma Involving the Eye and Ocular Adnexa: Tumor Control, Visual Outcomes, and Globe Salvage in 30  XXXX .

@entity1 @entity1539 @entity6725 @entity5 @entity10633 @entity5891
PURPOSE: To report clinical features and treatment outcomes of @entity6725 ( @entity6725 ). DESIGN: Retrospective case series. @entity1 : There were 32 @entity5 in 31 eyes of 30 @entity1 with @entity6725 . METHODS: Review of medical records. MAIN OUTCOME MEASURES: Tumor control, intraocular pressure (IOP), and visual acuity. RESULTS: The mean @entity1 age at presentation was 51 months (median, 15 months; range, 1-443 months). Eye redness (12/30, 40%) and @entity5891 (4/30, 13%) were the most common presenting symptoms. @entity6725 was concurrently present in 3 @entity1 (3/30, 10%), and spinal @entity6725 was present in 1 @entity1 (1/30, 3%). The ocular tissue affected by @entity6725 included the @entity1539 (21/31, 68%), conjunctiva (6/31, 19%), eyelid (2/31, 6%), choroid (2/31, 6%), and orbit (1/31, 3%). Those with @entity1539 @entity6725 presented at a median age of 13 months compared with 30 months for those with conjunctival @entity6725 . In the @entity1539 @entity6725 group, mean IOP was 19 mmHg (median, 18 mmHg; range, 11-30 mmHg) and @entity5891 was noted in 8 eyes (8/21, 38%). The @entity10633 was nodular (16/21, 76%) or diffuse (5/21, 24%). Fine-needle aspiration biopsy was used in 10 cases and confirmed @entity6725 cytologically in all cases. The @entity1539 lesion was treated with topical (18/21, 86%) and/or periocular (4/21, 19%) corticosteroids. The eyelid, conjunctiva, and orbital @entity6725 were treated with excisional biopsy in 5 @entity1 (5/9, 56%), topical corticosteroids in 2 @entity1 (2/9, 22%), and observation in 2 @entity1 (2/9, 22%). Of 28 @entity1 with a mean follow-up of 15 months (median, 6 months; range, 1-68 months), @entity5 regression was achieved in all cases, without recurrence. Two @entity1 were lost to follow-up. Upon follow-up of the @entity1539 @entity6725 group, visual acuity was stable or improved (18/19 @entity1 , 95%) and IOP was controlled long-term without medication (14/21 @entity1 , 74%). No eyes were managed with enucleation. CONCLUSIONS: Ocular @entity6725 preferentially affects the @entity1539 and is often isolated without cutaneous involvement. Iris @entity6725 responds to topical or periocular corticosteroids, often with stabilization or improvement of vision and IOP.
