The Prevalence of Retinal Disease and Associated CNS Disease in Young Patients with Incontinentia Pigmenti

Danford I.D.; Scruggs B.A.; Capone A.; Trese M.T.; Drenser K.A.; Thanos A.; Nudleman E.; Amphornphruet A.; Tipsuriyaporn B.; Hubbard G.B.; Ells A.; Harper C.A.; Goldstein J.; Calvo C.; Wallace-Carrete C.; Berry D.; Chang E.; Leishman L.; Shapiro M.; Blair M.; Mikhail M.; Shields C.L.; Schwendeman R.; Yonekawa Y.; Gupta M.P.; Orlin A.; Prakhunhungsit S.; Mukai S.; Berrocal A.; Hartnett M.E.; Campbell J.P.

The Prevalence of Retinal Disease and Associated CNS Disease in Young Patients with Incontinentia Pigmenti

Issued Date

2022-12-01

Resource Type

Article

eISSN

24686530

DOI

10.1016/j.oret.2022.05.032

Scopus ID

2-s2.0-85133884434

Pubmed ID

35691580

Journal Title

Ophthalmology Retina

Volume

6

Issue

12

Start Page

1113

End Page

1121

Rights Holder(s)

SCOPUS

Bibliographic Citation

Ophthalmology Retina Vol.6 No.12 (2022) , 1113-1121

Suggested Citation

Danford I.D., Scruggs B.A., Capone A., Trese M.T., Drenser K.A., Thanos A., Nudleman E., Amphornphruet A., Tipsuriyaporn B., Hubbard G.B., Ells A., Harper C.A., Goldstein J., Calvo C., Wallace-Carrete C., Berry D., Chang E., Leishman L., Shapiro M., Blair M., Mikhail M., Shields C.L., Schwendeman R., Yonekawa Y., Gupta M.P., Orlin A., Prakhunhungsit S., Mukai S., Berrocal A., Hartnett M.E., Campbell J.P. The Prevalence of Retinal Disease and Associated CNS Disease in Young Patients with Incontinentia Pigmenti. Ophthalmology Retina Vol.6 No.12 (2022) , 1113-1121. 1121. doi:10.1016/j.oret.2022.05.032 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/85286

Title

The Prevalence of Retinal Disease and Associated CNS Disease in Young Patients with Incontinentia Pigmenti

Other Contributor(s)

Mahidol University

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 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 = no disease, 1 = vascular abnormalities without leakage, 2 = leakage or neovascularization, and 3 = 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 = 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 = -2.12, P = 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.

Keyword(s)

Medicine

URI

https://repository.li.mahidol.ac.th/handle/20.500.14594/85286

Collections

Scopus 2022

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