Juvenile xanthogranuloma with intraocular involvement: a case report

Juvenile xanthogranuloma (JXG) is a rare and usually benign disease occurri ng in early childhood. It causes skin and deep seated lesions, notably in t he eye. We report a case of JXG in the iris of a 9-month-old infant. Examin ation under general anesthesia revealed megalocornea, iris xanthogranuloma occupying the entire anterior chamber and high intraocular pressure. Skin l esions on the left lid and on the back were also found. Ocular hypertension resisted medical and surgical treatment. Cyclodestruction was necessary. The incidence of JXG is low (0.4%). The iris is the most frequently affecte d ocular tissue. Early diagnosis is necessary to avoid complications. Moreo ver, JXG can be associated in rare cases with neurofibromatosis or leukemia which must be systematically searched for.