Abstract | PURPOSE: 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.
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Authors | M F Goldberg |
Journal | Transactions of the American Ophthalmological Society
(Trans Am Ophthalmol Soc)
Vol. 96
Pg. 55-65; discussion 65-72
( 1998)
ISSN: 0065-9533 [Print] United States |
PMID | 10360282
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
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Topics |
- Congenital Abnormalities
(diagnosis)
- Female
- Fluorescein Angiography
- Fundus Oculi
- Humans
- Incontinentia Pigmenti
(complications)
- Infant
- Infant, Newborn
- Macula Lutea
(blood supply)
- Retinal Vessels
(abnormalities)
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