Main Article Content
Phakomatosis Pigmentovascularis (PPV) is the rare condition which has been classified in the same spectrum with Sturge-Weber syndrome, Klippel-Trenaunay-Weber syndrome and oculodermal melanocytosis. PPV is the combination of widespread vascular lesions and extensive pigmentary lesions. We report a 2-monthold-infant with PPV type IIa associated with congenital glaucoma. She showed extensive Port-wine stain, extensive Mongolian spots and Café au lait spots along with soft tissue hypertrophy on her right face. She had buphthalmos
on her right eye and the very high intraocular pressure, so she was diagnosed as congenital glaucoma.
All articles published in the Siriraj Medical Journal (SMJ) are protected by copyright. No material in this journal may be reproduced on any platform including electronic or in print or transmitted by any means, in whole or in part, without the prior written permission of the Editor of the SMJ. Written permission must also be obtained before any part of the SMJ is stored in any retrieval system of any nature.