Deep anterior lamellar keratoplasty for Maroteaux-Lamy syndrome

Cornea. 2010 Dec;29(12):1459-61. doi: 10.1097/ICO.0b013e3181d927d6.


Purpose: To present a case of Maroteaux-Lamy syndrome (MLS), which underwent deep anterior lamellar keratoplasty (DALK) for visual rehabilitation.

Methods: A 15-year-old girl with MLS was admitted with severe corneal opacity. We performed DALK on her left eye for visual rehabilitation. Big-bubble technique was used to perform lamellar separation.

Results: Corneal graft was clear 24 months after surgery. Best spectacle-corrected visual acuity of left eye was 20/25 with the correction of +0.75/-0.50 diopters × 170. At the last follow-up, in vivo confocal microscopy revealed mild haze in the posterior stroma at the graft interface level. Endothelial cell count was 2473.4 cells per square millimeter using a noncontact specular microscope.

Conclusions: Given systemic problems in patients with MLS and less postoperative complications of DALK in comparison with penetrating keratoplasty, it seems DALK is the better choice for these patients.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Cell Count
  • Corneal Diseases / pathology
  • Corneal Diseases / surgery*
  • Corneal Transplantation*
  • Endothelium, Corneal / pathology
  • Female
  • Humans
  • Mucopolysaccharidosis VI / pathology
  • Mucopolysaccharidosis VI / surgery*
  • Vision Disorders / rehabilitation*
  • Visual Acuity / physiology