Abstract | BACKGROUND: One of the largest series of patients with incontinentia pigmenti who have undergone detailed ophthalmologic examination is reported here, including previously unrecognized findings with visually disabling consequences. METHODS: RESULTS: Abnormalities of the eye, central nervous system, skeleton, teeth, and hair develop in a majority of patients. The authors describe the evolution of retinal vascular abnormalities, and, for the first time, document foveal hypoplasia (in 4 of their 13 patients). The authors also provide the first report of a child with a normal brain shown on computed tomographic scan at 3 days old, which evolved to devastating cerebral ischemia, edema, and cortical blindness beginning at 6 days old. CONCLUSIONS:
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Authors | M F Goldberg, P H Custis |
Journal | Ophthalmology
(Ophthalmology)
Vol. 100
Issue 11
Pg. 1645-54
(Nov 1993)
ISSN: 0161-6420 [Print] United States |
PMID | 8233390
(Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
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Topics |
- Adolescent
- Adult
- Brain Diseases
(diagnostic imaging)
- Child, Preschool
- Diagnosis, Differential
- Female
- Fluorescein Angiography
- Fovea Centralis
(pathology)
- Fundus Oculi
- Humans
- Hyperplasia
(pathology)
- Incontinentia Pigmenti
(diagnosis)
- Infant
- Infant, Newborn
- Middle Aged
- Retinal Diseases
(diagnosis)
- Retinal Vessels
(pathology)
- Tomography, X-Ray Computed
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