[Skip to Content]
Access to paid content on this site is currently suspended due to excessive activity being detected from your IP address Please contact the publisher to request reinstatement.
[Skip to Content Landing]
July 1976

The Richner-Hanhart Syndrome: Report 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

• 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.