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M. Laguardia, D. Lepore, M.M. Pagliara, A. Baldascino, R. De Santis, G. D'Amico, C. Angora, L. Orazi, F. Molle, E. Balestrazzi; Fluorescein Angiography in Incontinentia Pigmenti . Invest. Ophthalmol. Vis. Sci. 2006;47(13):5804.
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Incontinentia pigmenti (IP) is an X–linked dominant disorder with skin, teeth and central nervous system lesions. One third of IP show some retinal abnormalities. Fluorescein angiography permits accurate diagnosis and localization of IP ischemic vascular manifestation not evident on fundus exam.
patient n.1 was a 40 years old mother; her daughters (10 and 4 years old) were patient n.2 and 3. 6 years old was patient n.4 and a 1 year old girl n.5. All of them had IP diagnosed by skin biopsy. After complete eye exam we performed angiography with the support of the RET CAM II (Massie Laboratories, Inc Pleasanton, CA) on all patients.
patient n. 4 and 5 showed partial retinal detachment with avascular periphery in one eye, and some avascular area with sea–fan–like neovascularization and posterior leakage in the extreme peripheral of the controlateral eye. Patient n.1, n. 2 and 3 fluorescein angiograms showed avascular retinal periphery retina with AV shunt and irregular vessels.
our angiographic findings confirm the hypothesis of ischemic pathogenesis of the retinal detachment in IP. Angiography is the most important exam to recognize and eventually treat ischemic peripheral retina.
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