Dr. Mehul Rameshbhai Patel, P18712, Dr. Snehal Rajendra Ganatra
Phakomatosis pigmentovascularis (PPV) is a rare cutaneous disorder characterized by combination of capillary malformation and other pigmented naevi.
A 7-year-old female child presented to us with facial port-wine stains distributed along the 3 branches of trigeminal nerve, which suggested the existence of Sturge Weber Syndrome(SWS). The diagnosis of Ota nevus was made by the bilateral scleral melanocystosis. Increased intraocular pressure, megalocornea, and pathologic optic disc cupping supported the diagnosis of bilateral glaucoma.
The simultaneous coexistence of PPV with SWS, Ota nevus, and congenital glaucoma is rare. Detailed clinical examination to exclude other ocular abnormalities or extraocular involvement are necessary.