A rare case of phacomatosis pigmento vascularis with glaucoma in an Indian child: Management with review of literature

Abstract

We present a rare case of phacomatosis pigmentovascularis in a 6-year-old child. The child had one functioning eye with severe glaucoma at presentation. We managed the case with primary Ahmed glaucoma valve implantation followed by two needling attempts. After 3 years of follow-up, the intraocular pressure was well maintained with the addition of two antiglaucoma medications.