Multifocal Juvenile Xanthogranuloma Presenting With a Hand Mass and Bilateral Vitreous Hemorrhage in a Neonate

Abstract

Although rare, JXG should be included in the differential diagnosis of spontaneous vitreous hemorrhage in children under the age of 2 years. Topical, periocular, and oral steroids may improve the ocular signs and symptoms in this condition and should be considered in the management of ophthalmic manifestations of JXG.

Juvenile xanthogranulomatosis with bilateral and multifocal ocular lesions of the iris, cornealscleral limbus, and choroid

“…8 A subretinal mass in a neonate with JXG was treated with oral steroids at a dose of 2 mg/kg/day for 2 weeks tapering over the course of a week. 6 When the choroidal mass was diagnosed, our patient was started on oral prednisolone and continued on oral methotrexate.…”

“…2,4 Posterior manifestations are rare. 2,5,6 Posterior segment involvement by JXG may result in retinal detachment and subsequent blindness. 7 Bilateral involvement has been noted but is uncommon.…”

“…7 Bilateral involvement has been noted but is uncommon. 6 No standard of treatment for ocular manifestations of JXG with extracutaneous involvement exists. SubTenon's or subconjunctival triamcinolone acetonide has been used to treat uveitis and iris mass in JXG.…”

Congenital‐type juvenile xanthogranuloma: A case series and literature review

“…Only one child with a solitary cutaneous JXG was documented to have ocular involvement. Gohari and colleagues reported a newborn with an infiltrative tumor involving the palm that was initially misdiagnosed as an infantile hemangioma and treated with oral prednisone, without improvement . On ophthalmologic examination, the infant was found to have bilateral vitreous hemorrhage from JXG involvement of the iris.…”

“…We excluded non‐English journal articles, cases without congenital onset, cases without histopathologic confirmation, and cases of systemic JXG without cutaneous congenital JXGs. In total, we identified 31 cases of congenital JXG involving the skin only and 16 cases of congenital JXG with systemic involvement …”