Clinical outcomes of phototherapeutic keratectomy in eyes with Thiel-Behnke corneal dystrophy.

Purpose: To investigate the functional and morphologic midterm outcome of phototherapeutic keratectomy (PTK) for Thiel-Behnke corneal dystrophy diagnosed by gene-mutation analysis.

Design: 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.