Bilateral Phakomatosis Cesiomarmorata With Ocular Melanocytosis and Secondary Glaucoma

Abstract

Phakomatosis pigmentovascularis (PPV) is a family of rare congenital diseases where vascular malformation coexists with melanocytic, dermal, or ocular lesions. The cesiomarmorata type is even rarer, and most such cases are reported with unilateral occurrence. We present an atypical case of a patient with bilateral phakomatosis cesiomarmorata, bilateral ocular melanocytosis, and bilateral glaucoma. No malformation to resist aqueous drainage was identified. Long-term management of intraocular pressure (IOP) using topical antiglaucoma medication was successful. This case report refines the clinical presentation of phakomatosis cesiomarmorata and may help diagnose and treat future cases.