Intralesional injection of OK-432 for vision-threatening orbital lymphangioma

BACKGROUND: Surgical excision of orbital lymphangiomas is difficult, and almost always incomplete due to the diffusely infiltrative pattern of these tumors. The present report describes the successful use of intralesional OK-432 administration to treat two patients with intractable hemorrhagic proptosis due to orbital lymphangiomas. METHODS: A 3-year-old girl (case 1) presented with aggressive proptosis and ptosis, and a 1-year-old boy (case 2) presented with massive proptosis and exposure keratopathy, associated with recurrent intracystic bleeding of an orbital lymphangioma. In case 1, 0.02 mg OK-432 was intracystically injected in a volume of 2 ml of physiologic saline. Due to a lack of therapeutic response, a second injection of 0.05 mg OK-432 in 1 ml was administered. In case 2, a single dose of 0.05 mg OK-432 in 1 ml was administered. RESULTS: In both cases, intracystic administration of 0.05 mg of OK-432 in a 1-ml volume resulted in a successful outcome. The adverse effects were minor (mild transient fever and lid swelling), and rebleeding and intraocular pressure elevation did not occur. Proptosis and eyelid swelling gradually improved over 1 month, and completely resolved within 3 months of treatment. CONCLUSIONS: Intralesional administration of 0.05 mg/ml OK-432 (delivered in 1 ml) resulted in the successful treatment of two cases of orbital lymphangioma. Although this drug concentration is higher than in previous reports, there were no major adverse effects.ope