The authors present the case of a 6-month-old female infant with a known diagnosis of incontinentia pigmenti with a normal examination by indirect ophthalmoscopy. However, fluorescein angiography revealed vascular abnormalities that were not detected by indirect ophthalmoscopy. Follow-up examination revealed progressive vascular changes that again were only detectable by fluorescein angiography. Because vision loss can cause significant morbidity in incontinentia pigmenti, the use of fluorescein angiography as an adjunctive tool with exams under anesthesia may provide invaluable information in the detection of early vascular changes in this disease.
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