Orbital melanocytosis and OTA naevus

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Presentation and discussion of a rarely reported location of orbital melanocytosis


Case report


In a 54 year old man with an oculodermal melanocytosis (naevus of OTA), enucleation was performed for a choroidal melanoma. During the surgery spots of hyperpigmention, based on melanocytosis, were found in tendons, muscles and orbital fat. Oculodermal melanocytosis is classically described on the periocular skin, sclera, uvea, orbit, meninges, palate or tympanic membrane and is a well-known risk factor to develop choroidal and orbital melanoma. Few reports describe the particular involvement of the orbital tissue. We show the pictures of orbital tissue and extraocular muscle pigmentation and explain it with embryology.


Even after enucleation for a choroidal melanoma in an OTA naevus, the ophthaist should be alert that extraocular melanocytes can remain and potentially be the cause of an orbital melanoma.

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