Occurrence of mucopolysaccharide in corneal grafts in the Maroteaux-Lamy syndrome.

We describe the histopathologic and ultrastructural features of corneal buttons from the original and repeat corneal grafts of two patients with the Maroteaux-Lamy syndrome (MPS VI-B). The donor cornea, in place for 10 years in one patient, had recurrence of mucopolysaccharide in the corneal epithelium, fibrocytes in the pannus, corneal stroma, retrocorneal fibrous tissue layer, and endothelium. The donor cornea, in place for 1 year in the second patient, had recurrence of mucopolysaccharide in the epithelium, minor deposits in the superficial stroma, and larger deposits in the stroma near the keratoplasty scar.