DR. MEENA GOPINATH MENON

DR. ROSHAN COLACO

Abstract

Phakomatosis pigmentovascularis (PPV) is a congenital condition characterized vascular malformation or cutaneous nevus flammeus and pigmentary nevus.We report a 6 year old girl presenting to the hospital with bilateral port wine stains on the face. Her best corrected visual acuity in both eyes was 6/9 .On slit lamp examination, left eye showed conjunctival naevus of ota and a single Lisch nodule. Intraocular pressure was 7mmHg in the right eye and 37mmHg in the left eye. Fundus examination was normal in both eyes.General evaluation showed presence of Mongolian spots on the abdomen and back and legs. A diagnosis of phakomatosis pigmentovascularis(PPV) with early juvenile glaucoma was made. She is being treated with maximal antiglaucoma medications to control the IOP. Management of glaucoma is complex in PPV, and is important to correctly classify the type of glaucoma and enable the clinician to make on the most appropriate tailored medical and/or surgical management.