Unilateral Open-Angle Glaucoma Associated with the Ipsilateral Nevus of Ota

The nevus of Ota also known as “congenital melanosis bulbi” and “oculodermal melanocytosis” is a blue-gray hyperpigmentation that occurs on the face and eyes. The sclera is involved in two-thirds of cases (causing an increased risk of glaucoma). Women are nearly five times more likely to be affected than men. It is rare among Caucasian people. The nevus of Ota is congenital or acquired. Most cases of the nevus of Ota are unilateral (90%), although pigmentation is present bilaterally in 5%–10%. Ocular abnormalities included pigmentation of the sclera, cornea, retina, and optic disc and cavernous hemangiomas of the optic disc, elevated intraocular pressure, glaucoma, and ocular melanoma. We reported an appearance of unilateral glaucoma in a Caucasian female patient with the acquired, ipsilateral nevus of Ota. 1. Introduction The nevus of Ota, also known by its original name “nevus fuscoceruleus ophthalmomaxillaris,” is a hamartoma of dermal melanocytes. Ota, known by the pen name Mokutaro Kinoshita, was a Japanese doctor who first described the condition in 1939 [1]. The nevus of Ota is usually located in the region of ophthalmic and maxillary division of the trigeminal nerve. The conjunctiva, sclera, cornea, retina, and optic disc may also be involved. The nevus of Ota may be associated with oral, uveal, or leptomeningeal melanosis, and malignant transformation has been reported repeatedly [2, 3]. The nevus of Ota is most frequent in Asian populations, with an estimated prevalence of 0.014%–0.034%; it is uncommon in Caucasian patients [4]. Glaucoma has also been associated with the nevus of Ota in 10% of patients [5]. In our work, we report an acquired form of Ota’s nevus associated with open-angle glaucoma which was diagnosed five years after the appearance of typical blue-gray hyperpigmentation. 2. Case Report A 47-year-old Caucasian female presented with a 5-year-history bluish-grey pigmentation of the right conjunctiva, sclera, and eyelids (Figure 1). As observed from her previous annual ophthalmic examination, the intraocular pressure never exceeded 16？mmHg, and the optic nerve head appeared normal in both eyes. Gonioscopy showed wide open and medium pigmented chamber angle with all elements of angle visible. Humphrey visual fields and optical coherence tomography findings did not show glaucoma damage. Her medical history included high blood pressure. Figure 1: Slit-lamp photograph shows bluish-grey pigmentation of the right conjunctiva, episclera, and sclera. The patient came for a regular annual ophthalmic exam. The visual acuity was 20/20 in both