Ocular Juvenile Xanthogranuloma

Abstract

To report the clinical and histopathologic characteristics and prognoses of three ocular juvenile xanthogranuloma (JXG) cases. Three cases were included in this study. The first case involved a 5-year-old girl with an enlarging yellowish mass at the limbus with corneal involvement. Ultrasound biomicroscopy showed a poorly demarcated mass involving the underlying cornea and sclera. The mass was excised in combination with a lamellar keratoplasty procedure. No recurrence was seen at the 2-year follow-up. The second case involved a 2-year-old boy with an enlarging yellowish mass on the conjunctiva, without limbal involvement. The mass was excised with no recurrence noted 1 year later. The third case involved a 7-month-old girl with unilateral eye redness and photophobia combined with multiple orange-red, raised nodular lesions on the skin. Examination under general anesthesia revealed a gray-yellow mass in the inferior and temporal iridocorneal angles. The intraocular pressure and corneal diameter were normal. Examination using ultrasound biomicroscopy showed a high-level echo lump in the inferior and temporal angles. There was no treatment for this case. At the 1-year follow up, the eye symptoms had resolved and the skin lesions were flat. Histopathologic examinations were completed on all three cases. The presence of Touton giant cells in hematoxylin-eosin staining, positive CD68 staining, and negative S-100 and CD1a staining confirmed the diagnosis of JXG. We report three histopathologically confirmed ocular JXG cases involving the corneoscleral limbus, conjunctiva, and iris with angle involvement, respectively. Ultrasound biomicroscopy performed on two cases demonstrated no obvious division between the mass and the surrounding structures. The cases with ocular surface involvement were successfully treated by excision and the case with iris involvement spontaneously regressed without any treatment. Early excision may be the better choice for ocular surface lesions, especially when corneal involvement is a possibility.