A Congenital Glaucoma Associated with Phakomatosis Pigmentovascularis in Infant Case Report

Sakaorat Petchyim


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.1 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.


Glaucoma; phacomatosis pigmentovascularis; nevus flammeus; Mongolian spots; ocular melanocytosis

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