title: Clinical outcomes of phototherapeutic keratectomy in eyes with Thiel-Behnke corneal dystrophy.
abstract: OBJECTIVE
  To investigate the functional and morphologic midterm outcome of phototherapeutic keratectomy (PTK) for Thiel-Behnke corneal dystrophy diagnosed by gene-mutation analysis.
  
  
  METHODS
  Retrospective, single-center clinical study.
  
  
  METHODS
  Between July 2001 and May 2010, 10 consecutive PTKs were performed in 10 eyes of 5 patients (2 male, 3 female; mean age: 55 ± 13 years) with superficially accentuated opacities caused by Thiel-Behnke corneal dystrophy and were followed up for at least 12 months (range: 12-108 months). Main outcome measures included (1) best-corrected visual acuity (BCVA), (2) uncorrected visual acuity (UCVA), (3) spherical equivalent, and (4) recurrence rate. The probability of recurrence of Thiel-Behnke corneal dystrophy after PTK was calculated using the Kaplan-Meier method for survival analysis.
  
  
  RESULTS
  The p.Arg555Gln mutation was found within the TGFBI gene in all 5 patients. Average logarithm of minimal angle of resolution (logMAR) BCVA change was -0.55 ± 0.26. Average logarithm UCVA change was -0.54 ± 0.31. In 5 of the 10 eyes, recurrence of central superficial opacification was clinically identified during the follow-up periods, and in 4 of those 5 eyes, the level of the recurrence was so significant that the visual acuity was reduced more than 2 lines. The maximum follow-up period of the 1 eye without significant post-PTK recurrence was 108 months.
  
  
  CONCLUSIONS
  PTK is a successful therapy for Thiel-Behnke corneal dystrophy, and results in midterm stable visual acuity and corneal transparency. Unlike in Reis-Bücklers corneal dystrophy cases, PTK delays the need for more invasive surgical intervention in Thiel-Behnke corneal dystrophy.
authors.affiliation: Department of Ophthalmology, Kyoto Prefectural University of Medicine, Kyoto, Japan. ohieda@koto.kpu-m.ac.jp
authors.forename: Osamu
authors.initials: O
authors.lastname: Hieda
authors.forename: Satoshi
authors.initials: S
authors.lastname: Kawasaki
authors.forename: Kouichi
authors.initials: K
authors.lastname: Wakimasu
authors.forename: Kenta
authors.initials: K
authors.lastname: Yamasaki
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Inatomi
authors.forename: Shigeru
authors.initials: S
authors.lastname: Kinoshita
mesh_terms: D000328:Adult; D000368:Aged; D003317:Corneal Dystrophies, Hereditary / Q000235:genetics / Q000503:physiopathology / Q000601:surgery*; D004252:DNA Mutational Analysis; D016326:Extracellular Matrix Proteins / Q000235:genetics; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D054018:Lasers, Excimer / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D018903:Photorefractive Keratectomy; D017354:Point Mutation; D012008:Recurrence; D012029:Refraction, Ocular / Q000502:physiology; D012189:Retrospective Studies; D016212:Transforming Growth Factor beta / Q000235:genetics; D016896:Treatment Outcome; D014792:Visual Acuity / Q000502:physiology; D000099222:betaIG-H3 Protein
chemical_list: D016326:Extracellular Matrix Proteins; D016212:Transforming Growth Factor beta; D000099222:betaIG-H3 Protein