Juvenile Xanthogranuloma

A baby was referred for a new-onset subconjunctival lesion in the right eye. He was found to have a thickened, yellow corneoscleral lesion and hyphema, presumed to be ocular juvenile xanthogranuloma (JXG). He was started on topical steroids as empiric treatment, with initial improvement for the first 3 months. Despite remaining on topical steroids, the lesion remained persistent and was biopsied at age 8 months. At age 11 months, the patient acutely developed a collapsed anterior chamber with complete keratolenticular and iridocorneal adhesions secondary to a pupillary membrane. Biopsies were consistent with early JXG with CD68-positive histiocytes, CD3-positive T cells, and CD20-positive T cells, with notable absence of Touton giant cells. The patient improved after undergoing placement of an Ahmed drainage implant, synechiolysis, pars plana lensectomy and vitrectomy, with subconjunctival and intravitreal injection of bevacizumab, followed by intravitreal methotrexate.

Presentation Date: 12/07/2023

Issue Date: 12/22/2023