Alterations of Pigment Epithelium in Cystinosis

Vernon G. Wong, Paul S. Lietman, Jarvis E. Seegmiller

Research output: Contribution to journalArticlepeer-review

52 Scopus citations


A previously undescribed pigmentary disturbance of the peripheral fundus was seen in eleven patients with childhood cystinosis. Histologic observations in two of these children demonstrated that extensive degeneration and loss of retinal pigment epithelium was associated with the retinopathy. The peripheral fundus abnormalities may be of special diagnostic value since it can precede the classical corneal deposits of cystine crystals as suggested by the present study. The existence of this retinopathy appears to be peculiar to childhood cystinosis as it was found to be absent in the adult form of the disease.

Original languageEnglish (US)
Pages (from-to)361-369
Number of pages9
JournalArchives of ophthalmology
Issue number3
StatePublished - Mar 1967
Externally publishedYes

ASJC Scopus subject areas

  • Ophthalmology


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