Early-Onset Glaucoma Manifesting as Buphthalmos in an Infant with Phakomatosis Pigmentovascularis Type IIa

Abstract

Abstract Phacomatosis pigmentovascularis (PPV) is a rare type of phakomatosis characterized by co-existence of vascular nevus and pigmentary nevus with or without extracutaneous systemic involvement.Presentation of this rare entity is uncommon among infantile age group and bilateral glaucoma with megalocornea in PPV is exceedingly rare.We present a rare case of bilateral buphthalmos associated with PPV type IIa in a seven-month child who had nonvalved glaucoma drainage device(GDD) in right eye(OD) &combined trabeculotomy+ trabeculectomy in left eye(OS), followed by successful control of intraocular pressure(IOP) for the period of 18 months follow up.We discuss challenges in management with a comprehensive review of literature.