Progressive anterior segment involvement of an infant with corneoscleral juvenile xanthogranuloma refractory to topical steroids.

Purpose: To report a case of corneoscleral juvenile xanthogranuloma (JXG) with progressive anterior segment involvement refractory to topical steroids. Observations: A 4-month-old male 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 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 persisted and was biopsied at age 8 months. Conjunctival, scleral, and aqueous biopsies ruled out uveitic and malignant etiologies and were consistent with JXG with CD68-positive histiocytes, CD3-positive T cells, and CD20-positive B cells. Over the next few months, axial length increased out of proportion to normal growth, and intraocular pressure was elevated despite multiple pressure-lowering drops. At age 11 months, the patient acutely developed a flat anterior chamber with complete keratolenticular and iridocorneal adhesions secondary to a pupillary membrane. The patient improved after undergoing placement of an Ahmed drainage implant, synechiolysis, pars plana lensectomy and vitrectomy, subconjunctival and intravitreal injection of bevacizumab, and intravitreal methotrexate. Conclusions and importance: This is a rare presentation of ocular JXG presenting with diffuse infiltration of the sclera, cornea, and iris. Given the involvement of multiple ocular structures, progression despite initial response to topical steroids, and increased intraocular pressure, aggressive management was required. Lesions should be closely monitored for progression and may benefit from surgical intervention and treatment with intraocular bevacizumab and/or methotrexate if topical steroids are insufficient.