Richner-Hanhart syndrome (Tyrosinemia Type II) Case report and literature review

Richner-Hanhart syndrome (TyrosinemiaType 11) is an autosomal recessive disorder of amino acid metabolism characterized by ocular changes, painful palmoplanar hyperkeratosis, and mental retardation. Serum tyrosine increases due to tyrosine aminotransferase deficiency resulting in the deposition of tyrosine crystals in the cornea and in corneal inflammation. Patients are often misdiagnosed as having herpes simplex keratitis.We report on a child who presented with bilateral keratitis secondary to TyrosinemiaType 11 diagnosed as herpes simplex keratitis.