Deep anterior lamellar keratoplasty for Maroteaux-Lamy syndrome.
OBJECTIVE
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.
Rahmati-Kamel
Mohsen
M
Labbafinejad Medical Center, Shahid Beheshti University (MC), Tehran, Iran.
Javadi
Mohammadali
M
Shojaei
Ahmad
A
Eslani
Medi
M
Karimian
Farid
F
D000293:Adolescent; D002452:Cell Count; D003316:Corneal Diseases / Q000473:pathology / Q000601:surgery*; D016039:Corneal Transplantation; D004728:Endothelium, Corneal / Q000473:pathology; D005260:Female; D006801:Humans; D009087:Mucopolysaccharidosis VI / Q000473:pathology / Q000601:surgery*; D014786:Vision Disorders / Q000534:rehabilitation*; D014792:Visual Acuity / Q000502:physiology