Fluorescein angiography findings in phacomatosis pigmentovascularis

Ophthalmic Surg Lasers Imaging Retina. Mar-Apr 2013;44(2):201-3. doi: 10.3928/23258160-20130212-01. Epub 2013 Feb 18.


The authors report the fluorescein angiography findings in a 3-month-old patient with phacomatosis cesioflammea, which revealed venous-venous anastomoses in addition to previously undescribed features of peripheral retinal vascular nonperfusion. The authors encourage physicians to consider phacomatosis pigmentovascularis in the differential diagnosis of patients presenting with facial port-wine stain and to screen these patients for peripheral retinal avascularity in addition to glaucoma and primary uveal melanoma.

Publication types

  • Case Reports

MeSH terms

  • Choroid Neoplasms / pathology*
  • Female
  • Fluorescein Angiography*
  • Hemangioma / pathology*
  • Humans
  • Infant
  • Neurocutaneous Syndromes / diagnosis*
  • Port-Wine Stain / diagnosis*
  • Retinal Vein / abnormalities*
  • Vascular Fistula / diagnosis*
  • Visual Acuity

Supplementary concepts

  • Phacomatosis pigmentovascularis