Ota Nevus of Skin, Eye and Orbit: Neglected Potential Risk of Developing an Orbital Melanoma, Even in Young Patients

Purpose: To demonstrate the presence of orbital pigmentation in a congenital nevus of Ota. Every medical practitioner should be aware of the increased risk of orbital melanoma.

Methods: Observational case report and review of the literature.

Results: A Caucasian man of 54 years was referred with blurred vision and skin melanocytosis in the dermatome of the ophthalmic- and maxillary nerve, also involving sclera, conjunctiva, iris and choroid. A large uveal melanoma was detected and an enucleation was indicated. During surgery melanosis of the orbital soft tissues and muscles was found. Only few reports in the literature describe orbital melanocytosis.

Conclusion: In the nevus of Ota, besides ocular melanosis, also orbital tissues may be involved, with a potential risk of developing an orbital melanoma. In patients with Ota not only fundoscopy, but also exophthalmometry and orbital imaging should be performed regularly. 

Keywords: Oculodermal melanocytosis, ocular and orbital melanocytosis, melanosis, nevus of Ota, blue nevus, uveal melanoma, orbital melanoma.