@article {1645452,
	title = {The Prevalence of Retinal Disease and Associated CNS Disease in Young Patients with Incontinentia Pigmenti},
	journal = {Ophthalmol Retina},
	volume = {6},
	number = {12},
	year = {2022},
	month = {2022 Dec},
	pages = {1113-1121},
	abstract = {PURPOSE: To evaluate the prevalence of retinal disease on fluorescein angiography (FA) in patients with incontinentia pigmenti (IP) and to compare the severity of retinal disease in those with and without known central nervous system (CNS) disease. DESIGN: Multi-institutional consecutive retrospective case series. SUBJECTS: New patients with a diagnosis of IP were seen at the Casey Eye Institute at the Oregon Health and Science University (OHSU), Moran Eye Center, University of Utah, or Bascom Palmer Eye Institute, University of Miami from December 2011 to September\&nbsp;2018. METHODS: Detailed ophthalmoscopic examination and FA were recommended for all new patients and performed on every patient who had parental consent. Ophthalmoscopic findings and FA images were graded for severity by 2 masked graders on a 3-point scale: 0\&nbsp;= no disease, 1\&nbsp;= vascular abnormalities without leakage, 2\&nbsp;= leakage or neovascularization, and 3\&nbsp;= retinal detachment. The presence of known CNS disease was documented. Additional cases were obtained from a pediatric retina listserv for examples of phenotypic variation. MAIN OUTCOME MEASURES: The proportion of eyes noted to have disease on ophthalmoscopy compared with FA and the severity of retinal disease in those with and without known CNS disease. RESULTS: Retinal pathology was detected in 18 of 35 patients (51\%) by indirect ophthalmoscopy and 26 of 35 patients (74\%) by FA (P\&nbsp;= 0.048) in a predominantly pediatric population (median age, 9 months). Ten patients (29\%) had known CNS disease at the time of the eye examination. A Wilcoxon rank-sum test indicated that the retinal severity scores for patients with CNS disease (median, 2) were significantly higher than the retinal severity scores for patients without CNS disease (median, 1), z\&nbsp;= -2.12, P\&nbsp;= 0.034. CONCLUSIONS: Retinal disease is present in the majority of patients with IP, and ophthalmoscopic examination is less sensitive than FA for detection of disease. There may be a correlation between the severity of retinal and CNS disease.},
	keywords = {Central Nervous System Diseases, Child, Humans, Incontinentia Pigmenti, Infant, Prevalence, Retina, Retinal Diseases, Retrospective Studies},
	issn = {2468-6530},
	doi = {10.1016/j.oret.2022.05.032},
	author = {Danford, Ian D and Scruggs, Brittni A and Capone, Antonio and Trese, Michael T and Drenser, Kim A and Thanos, Aristomenis and Nudleman, Eric and Amphornphruet, Atchara and Tipsuriyaporn, Boontip and Hubbard, G Baker and Ells, Anna and Harper, C Armitage and Goldstein, Jessica and Calvo, Charles and Wallace-Carrete, Chris and Berry, Duncan and Chang, Emmanuel and Leishman, Lisa and Shapiro, Michael and Blair, Michael and Mikhail, Mikel and Shields, Carol L and Schwendeman, Rachel and Yonekawa, Yoshihiro and Gupta, Mrinali P and Orlin, Anton and Prakhunhungsit, Supalert and Mukai, Shizuo and Berrocal, Audina and Hartnett, M Elizabeth and Campbell, J Peter}
}
