Nevus fusco caeruleus ophthalmomaxillaris of Ota is a rare disorder of pigmentation that exhibits melanosis oculi in approximately 65% of the reported cases. It is characterized clinically by the presence of abnormal pigmentation in the distribution of the ophthalmic and maxillary divisions of the trigeminal nerve. In the majority of cases the pigmentation is present at birth, but it is rarely hereditary.
This nevoid disturbance was first described by Ota1 in 1939. Since then, accounts of several series2-5 and many single cases have appeared, the largest series having been reported by Japanese authors. Recently, Mishima and Mevorah6 published a thorough review of this disorder.
Nevus of Ota is characterized histopathologically by the presence of spindleshaped melanocytes scattered throughout the involved tissues. In patients with orbital involvement, these melanocytes may be found in the conjunctiva, subconjunctival connective tissue, Tenon's capsule, sclera, iris, and fundus, and in