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Cerebro-hepato-renal Syndrome of ZellwegerOcular Histopathologic Findings
Raja Haddad, MD;
Ramon L. Font, MD;
David S. Friendly, MD
Arch Ophthalmol. 1976;94(11):1927-1930.
Abstract
• An infant who was the product of a full-term pregnancy was born with multiple congenital defects, including diffuse bilateral corneal opacification. He failed to thrive and died at the age of 4 days. Autopsy findings were consistent with those seen in the cerebro-hepato-renal syndrome. Histopathologic examination of both eyes, obtained at autopsy, demonstrated similar changes. The main pathologic findings in the anterior segment were bilateral, diffuse corneal edema associated with paracentral iridocorneal adhesions and focal attenuation of Descemet membrane. Both retinas disclosed a selective degeneration of the outer nuclear layers and photoreceptors, confined mainly to the macular region. Atrophy of the optic disc and nerve, with reactive gliosis and demyelination, were consistent with findings in the central nervous system described in this syndrome.
Author Affiliations
From the Registry of Ophthalmic Pathology, Armed Forces Institute of Pathology, and the Children's Hospital National Medical Center, Washington, DC.
Footnotes
Accepted for publication May 24, 1976.
The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense.
Reprint requests to Registry of Ophthalmic Pathology, Armed Forces Institute of Pathology, Washington, DC 20306 (Dr Font).
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