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Episclera and sclera 2. Dr. Mohammad Shehadeh. Congenital ocular melanocytosis. Congenital ocular melanocytosis is an uncommon condition characterized by an increase in number, size and pigmentation of melanocytes in the sclera and uvea
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Episclera and sclera 2 Dr. Mohammad Shehadeh
Congenital ocular melanocytosis • Congenital ocular melanocytosis is an uncommon condition • characterized by an increase in number, size and pigmentation of melanocytesin the sclera and uvea • and may also involve the periocular skin, orbit, meninges and soft palate.
Classification • Ocular melanocytosis, the least common, involves only the eye. • Dermal melanocytosis involves only the skin and accounts for about one-third of cases. • Oculodermalmelanocytosis (naevus of Ota) is the most frequent type and involves both skin and eye.
Clinical features • Multifocal slate-grey pigmentation within the sclera and episclera which cannot be moved over the globe • Occasionally the peripheral cornea may be involved.
Naevus of Ota is bilateral in 5% of patients, occurring frequently in Orientals and darker races but rarely in Caucasians. • Deep bluish hyperpigmentation of facial skin, most frequently in the distribution of the 1st and 2nd divisions of the trigeminal nerve
Ipsilateralassociations • Iris hyperchromia is common • Iris mammillations. • Fundushyperpigmentation can occur • Trabecularhyperpigmentation (Fig. 8.20D) which is associated with glaucoma in about 10% of cases. • Uveal melanoma may develop in a small minority of patients, and long-term review is required.