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Article
July 1976

The Richner-Hanhart SyndromeReport of a Case With Associated Tyrosinemia

Author Affiliations

From Howe Laboratory of Ophthalmology, Boston (Dr Bienfang), the Laboratory of Vision Research, National Eye Institute, Bethesda, Md (Dr Kuwabara), and the Developmental Evaluation Clinic, Children's Hospital Medical Center, Boston (Dr Pueschel).

Arch Ophthalmol. 1976;94(7):1133-1137. doi:10.1001/archopht.1976.03910040045009
Abstract

• The Richner-Hanhart syndrome with tyrosinemia was recognized in a mentally retarded adolescent boy. The clinical manifestations, including hyperkeratosis of the volar aspects of the hands and feet, thickening of the conjunctival epithelium, and corneal opacities, as well as biochemical aberrations of tyrosine metabolism, responded to specific treatment with a diet low in phenylalanine and tyrosine. Light and electron microscopical studies illustrate the underlying conjunctival pathologic changes.

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