title: Macular vasculopathy and its evolution in incontinentia pigmenti.
abstract: OBJECTIVE
  To describe macular vasculopathy in incontinentia pigmenti.
  
  
  METHODS
  Twelve baby girls with incontinentia pigmenti were examined under general anesthesia by fluorescein angiography of the macula. Nine eyes of 9 patients had sufficient detail to allow evaluation of capillary changes. Angiography was initiated as early as 3 months of age and was repeated in 7 eyes at 3- to 12-month intervals. Changes in capillary patterns were identified.
  
  
  RESULTS
  Irregularly enlarged or distorted foveal avascular zones were noted in all 9 maculas. Sparseness of the perifoveolar capillary bed was a characteristic finding. Sequential macular angiography demonstrated non-progressive (stable) capillary closure in 2 eyes; progressive closure in another macula; progressive closure plus addition or reopening of macular capillaries in 3 eyes; and central retinal artery occlusion, with cherry-red spot formation, in 1 eye at 12 days of age. In addition, progressive tractional detachment of the macula occurred in 2 of these eyes, and progressive macular neovascularization occurred in 1 eye.
  
  
  CONCLUSIONS
  Macular ischemia is characteristic of incontinentia pigmenti and is often progressive. It is the initiating event of a typical vasculopathy, characterized by capillary remodelling and, occasionally, by neovascularization and tractional detachment of the retina.
authors.affiliation: Wilmer Ophthalmological Institute, Johns Hopkins University School of Medicine and Hospital, Baltimore, MD 21287-9278, USA.
authors.forename: M F
authors.initials: MF
authors.lastname: Goldberg
mesh_terms: D000013:Congenital Abnormalities / Q000175:diagnosis; D005260:Female; D005451:Fluorescein Angiography; D005654:Fundus Oculi; D006801:Humans; D007184:Incontinentia Pigmenti / Q000150:complications*; D007223:Infant; D007231:Infant, Newborn; D008266:Macula Lutea / Q000098:blood supply*; D012171:Retinal Vessels / Q000002:abnormalities*