May 2006
Volume 47, Issue 13
Free
ARVO Annual Meeting Abstract  |   May 2006
Fluorescein Angiography in Incontinentia Pigmenti
Author Affiliations & Notes
  • M. Laguardia
    Ophthalmology, Catholic University, Rome, Italy
  • D. Lepore
    Ophthalmology, Catholic University, Rome, Italy
  • M.M. Pagliara
    Ophthalmology, Catholic University, Rome, Italy
  • A. Baldascino
    Ophthalmology, Catholic University, Rome, Italy
  • R. De Santis
    Ophthalmology, Catholic University, Rome, Italy
  • G. D'Amico
    Ophthalmology, Catholic University, Rome, Italy
  • C. Angora
    Ophthalmology, Catholic University, Rome, Italy
  • L. Orazi
    Ophthalmology, Catholic University, Rome, Italy
  • F. Molle
    Ophthalmology, Catholic University, Rome, Italy
  • E. Balestrazzi
    Ophthalmology, Catholic University, Rome, Italy
  • Footnotes
    Commercial Relationships  M. Laguardia, None; D. Lepore, None; M.M. Pagliara, None; A. Baldascino, None; R. De Santis, None; G. D'Amico, None; C. Angora, None; L. Orazi, None; F. Molle, None; E. Balestrazzi, None.
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science May 2006, Vol.47, 5804. doi:
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    • Get Citation

      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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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose: : 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.

Methods: : 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.

Results: : 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.

Conclusions: : 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.

Keywords: retinal degenerations: hereditary 
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