Deep Anterior Lamellar Keratoplasty in a Case of Hurler-Scheie Syndrome Undergoing Enzyme Replacement Therapy

To report successful management of corneal clouding associated with Hurler-Scheie syndrome with deep anterior lamellar keratoplasty (DALK) undergoing concurrent enzyme replacement therapy (ERT).

A 13-year-old boy with Hurler-Scheie syndrome (MPS I-HS) presented with corneal clouding in both eyes. He was on ERT with laronidase (Aldurazyme; Genzyme, Cambridge, MA) and had a visual acuity of 20/32 in both eyes. Seven years later, visual acuity in his left eye decreased to counting fingers and DALK was performed for vision recovery. Two years later, visual acuity in his right eye decreased to 20/100 and DALK was performed.

At his last visit, which was 3 years after right eye DALK and 5 years after left eye DALK, the grafts were clear and the corrected visual acuity in his right and left eyes was 20/30 and 20/25, respectively.

The present case highlights the possible effectiveness of DALK in patients with mucopolysaccharidosis (MPS)-related corneal stromal opacification, treated with ERT.