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.