Spontaneous nontraumatic hyphema occurring in an infant is unusual. Most frequently it is the result of a disease entity recognized relatively recently, juvenile xanthogranuloma (nevoxanthoendothelioma) of the iris.1-5 Generally a diffuse or localized iris lesion is visible clinically, and severe glaucoma results if the disease is not recognized and treated. Characteristic skin lesions are present in some cases. Juvenile xanthogranuloma of the iris has been treated successfully with radiation,1,3 combined radiation and steroid therapy,2 and excisional biopsy.5 In this report, two cases illustrating proper and improper management of juvenile xanthogranuloma of the iris are presented, and an outline for therapy is discussed.
—A 9-month-old Caucasian girl was well until about 3½ months of age, when her mother noted congestion of the right eye and a lesion on the iris. Examination under general anesthesia revealed a normal left eye. The right eye contained a small
GASS JDM. Management of Juvenile Xanthogranuloma of the Iris. Arch Ophthalmol. 1964;71(3):344-347. doi:10.1001/archopht.1964.00970010360009