title: Outcome of penetrating keratoplasty for mucopolysaccharidoses.
abstract: OBJECTIVE
  To describe the outcome of penetrating keratoplasty (PK) for corneal opacification in the setting of systemic mucopolysaccharidoses (MPS).
  
  
  METHODS
  A consecutive case series and literature review.
  
  
  RESULTS
  Eight eyes from 5 patients with MPS (MPS I, MPS IV, and MPS VI) and a history of PK met inclusion criteria for our case series at the University of Minnesota Medical Center. The mean age at the time of PK was 40.5 years (range, 11.7-65.3 years). Mean follow-up time after the PK was 4.9 years (range, 1-11 years). Mean (SD) visual acuity before PK was 0.90 (0.38) logMAR. The mean (SD) visual acuity at the last visit for all 8 eyes was 0.32 (0.16) logMAR. Visual acuity improved in 7 of 8 eyes (P = .002). Although early rejection led to repeat PK in 1 eye, no recurrent opacity consistent with MPS was noted in any of the corneal grafts. In a literature review, we found 23 reports documenting 40 initial and 3 repeat cases of PK in the setting of MPS. Of these, 31 initial and 2 repeat corneal grafts were reportedly clear during follow-up, ranging from 0.25 to 13 years.
  
  
  CONCLUSIONS
  Penetrating keratoplasty is often a beneficial intervention in appropriate patients with corneal clouding due to MPS. Improvement in vision can be obtained with stable, clear corneal grafts in this population.
authors.affiliation: Department of Ophthalmology, University of Minnesota, 420 Delaware Street SE, Minneapolis, MN 55455-5501, USA. bothu003@umn.edu
authors.forename: Erick D
authors.initials: ED
authors.lastname: Bothun
authors.forename: Alejandra
authors.initials: A
authors.lastname: Decanini
authors.forename: C Gail
authors.initials: CG
authors.lastname: Summers
authors.forename: Paul J
authors.initials: PJ
authors.lastname: Orchard
authors.forename: Jakub
authors.initials: J
authors.lastname: Tolar
mesh_terms: D000328:Adult; D000368:Aged; D002648:Child; D003318:Corneal Opacity / Q000503:physiopathology / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006084:Graft Rejection / Q000503:physiopathology; D006801:Humans; D015948:Keratoplasty, Penetrating; D008297:Male; D008875:Middle Aged; D009083:Mucopolysaccharidoses / Q000503:physiopathology / Q000601:surgery*; D012086:Reoperation; D016896:Treatment Outcome; D014792:Visual Acuity / Q000502:physiology; D055815:Young Adult