The risk intraocular juvenile xanthogranuloma: survey of current practices and assessment of risk

Juvenile xanthogranuloma (JXG) is an uncommon, usually uncomplicated disease of childhood. Ocular involvement, however, can result in glaucoma and blindness if left untreated. The incidence of ocular complications and how best to screen for their occurrence is unknown.

We attempted to ascertain management and referral practices among ophthalmologists and dermatologists and to characterize the risk for ocular complications in children with JXG.

A total of 431 dermatologists and 438 ophthalmologists were surveyed. In addition, the literature was reviewed.

The response rate were 28% (dermatologists) and 44% (ophthalmologists). Most believed screening was important, but referral and surveillance practices varied widely. The survey incidence of ocular complications in patients with cutaneous JXG was approximately 0.3% (7 of 2371). The literature incidence was 0.4% (1 of 260). Children at maximum risk were 2 years of age or younger, had multiple skin lesions, and had newly diagnosed JXG.

Ophthalmologic screening of patients with JXG should be particularly targeted to patients with risk factors of multiple skin lesions, new diagnosis, and age of 2 years or younger.