title: Penetrating keratoplasty for corneal amyloidosis in familial amyloidosis, Finnish type.
abstract: OBJECTIVE
  To analyze the outcome of penetrating keratoplasty (PK) to the first eye for corneal amyloidosis in familial amyloidosis, Finnish type (FAF).
  
  
  METHODS
  Single-center, retrospective, nonrandomized, interventional, noncomparative case series.
  
  
  METHODS
  Thirty-one eyes of 31 patients with FAF.
  
  
  METHODS
  All patients with FAF who had their first PK in Helsinki University Eye Hospital between January 1, 1990, and August 1, 2011, were identified and a retrospective analysis of the patient charts was performed.
  
  
  METHODS
  Best spectacle-corrected visual acuity (BCVA), intraoperative and postoperative complications, graft survival, reason for graft failure, and frequency of regrafting.
  
  
  RESULTS
  The median follow-up period was 32 months (range, 5-114). After 24 months, the median BCVA was 1.15 on a logarithm of the minimum angle of resolution scale (20/280; mean, 1.1; SD, 0.5) in comparison with the preoperative median BCVA of 1.3 (20/400; mean, 1.3; SD, 0.4). At 24 months, 3 of 18 eyes (17%) had a visual acuity of ≥0.5 (20/63) and 13 of 18 grafts (72%) were clear. Rejection occurred in 6 of 31 primary grafts (19%). Graft failure occurred in 16 of 31 eyes and resulted from surface complications in 11 eyes and additionally from rejection in 5 eyes. Seven eyes needed regrafting (twice in 1 eye). Complications were frequent in the early and late postoperative periods. Presence of preoperative corneal or graft neovascularization was an indicator of a high risk of graft failure and poor visual outcome.
  
  
  CONCLUSIONS
  In a minority of FAF patients, PK improves vision. Owing to the high failure risk and guarded visual prognosis after PK, it is important that both the surgeon and the patient have realistic expectations. It may be reasonable to limit PK to cases with bilateral advanced disease. It seems reasonable to optimize ocular surface health and to delay PK.
authors.affiliation: The Helsinki University Eye Hospital, University of Helsinki, Helsinki, Finland.
authors.forename: Jaakko S
authors.initials: JS
authors.lastname: Mattila
authors.affiliation: The Helsinki University Eye Hospital, University of Helsinki, Helsinki, Finland.
authors.forename: Kari
authors.initials: K
authors.lastname: Krootila
authors.affiliation: The Helsinki University Eye Hospital, University of Helsinki, Helsinki, Finland.
authors.forename: Tero
authors.initials: T
authors.lastname: Kivelä
authors.affiliation: The Helsinki University Eye Hospital, University of Helsinki, Helsinki, Finland. Electronic address: juha.holopainen@hus.fi.
authors.forename: Juha M
authors.initials: JM
authors.lastname: Holopainen
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000686:Amyloidosis / Q000503:physiopathology / Q000601:surgery*; D003315:Cornea / Q000503:physiopathology; D003317:Corneal Dystrophies, Hereditary / Q000503:physiopathology / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006085:Graft Survival / Q000502:physiology; D006801:Humans; D007431:Intraoperative Complications; D015948:Keratoplasty, Penetrating; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications; D012189:Retrospective Studies; D016896:Treatment Outcome; D014792:Visual Acuity / Q000502:physiology