June 1986
Volume 27, Issue 6
Free
Articles  |   June 1986
Pathogenesis of Chandler's syndrome, essential iris atrophy and the Cogan-Reese syndrome. II. Estimated age at disease onset.
Investigative Ophthalmology & Visual Science June 1986, Vol.27, 873-882. doi:
  • Views
  • PDF
  • Share
  • Tools
    • Alerts
      ×
      This feature is available to authenticated users only.
      Sign In or Create an Account ×
    • Get Citation

      J A Alvarado, C G Murphy, R P Juster, J Hetherington; Pathogenesis of Chandler's syndrome, essential iris atrophy and the Cogan-Reese syndrome. II. Estimated age at disease onset.. Invest. Ophthalmol. Vis. Sci. 1986;27(6):873-882.

      Download citation file:


      © ARVO (1962-2015); The Authors (2016-present)

      ×
  • Supplements
Abstract

The presence of a layer of abnormal material in Descemet's membrane in eight keratoplasty specimens served as a marker to determine whether Chandler's syndrome, Essential Iris Atrophy, and the Cogan-Reese syndrome are congenital or acquired conditions. In all eight cases of the ICE syndrome, a pattern of membrane deposition was observed, which typifies acquired disorders: a completely normal prenatal layer and both normal and abnormal portions of the postnatal layer. The thickness of the membrane deposited before the onset of abnormal secretion was used to estimate a time span for possible ages at which abnormal secretion began. The estimated age intervals for all diseased specimens had their lower limits in the postnatal period. Thus, these results led us to the conclusion that abnormal Descemet's membrane first appeared in postnatal life, years before clinical recognition of disease. The possibility of a viral etiology for this unilateral endotheliopathy resulting in an altered Descemet's membrane is discussed.

×
×

This PDF is available to Subscribers Only

Sign in or purchase a subscription to access this content. ×

You must be signed into an individual account to use this feature.

×