DR. KAVITHA DEVI

DR. A. VIJAYALAKSHMI

Abstract

We report an interesting case of bilateral Phakomatosis Pigmentovascularis(PPV) in a 30-year male with Nevus of Ota,diffuse dermal melanocytosis,developmental glaucoma and optic nerve head pigmentation,in association with varicose veins,a feature of Klipple-Trenanauy-Weber syndrome(KTW) and port wine stain with facial hemi hypertrophy,a feature of Sturge Weber Syndrome(SWS).BCVA and IOP RE-6/6,24mmHg and LE-No PL,32mmHg respectively. CDR RE-0.85 with inferior rim thinning.OCT RNFL RE inferior RNFL thinning and HFA 24-2 RE superior arcuate scotoma.He was treated conservatively with maximal antiglaucoma medications.This case report highlights the overlapping features of congenital conditions like PPV, SWS, KTW in a young male and also emphasizes upon the common origin of these entities from the neural crest cells.Through this case report we aim to emphasize on the early diagnosis and timely intervention to prevent glaucoma related blindness in these patients.