Macular vasculopathy and its evolution in incontinentia pigmenti.

PURPOSE: To describe macular vasculopathy in incontinentia pigmenti.

METHODS: Twelve baby girls with incontinentia pigmenti were evaluated under general anesthesia by fluorescein angiography of the macula. Nine eyes of nine patients had sufficient detail to allow evaluation of capillary changes. Angiography was initiated as early as three months of age, and was repeated in seven eyes at 3-12 month intervals. Changes in capillary patterns were identified.

RESULTS: Irregularly enlarged or distorted foveal avascular zones were noted in all nine maculas. Sparseness of the perifoveolar capillary bed was a characteristic finding. Sequential macular angiography demonstrated nonprogressive (stable) capillary closure in two eyes; progressive closure was noted in another macula; progressive closure plus addition or reopening of macular capillaries occurred in three eyes; and central retinal artery occlusion, with cherry red spot formation, was observed in one eye at 12 days of age. In addition, progressive tractional detachment of the macula, associated with bleeding pre-retinal neovascularization, occurred in two of these eyes, and progressive macular neovascularization also occurred in one 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.