Indications for and outcomes of deep anterior lamellar keratoplasty in mucopolysaccharidoses.

PURPOSE: To describe the outcome of deep anterior lamellar keratoplasty (DALK) for visually significant corneal clouding in patients with mucopolysaccharidoses (MPS).

METHODS

A retrospective consecutive case series of patients with MPS and corneal clouding were analyzed at a tertiary eye hospital. A review of the English literature regarding MPS and DALK was performed. The main outcomes measures of the study were intraoperative surgical complications, change in visual acuity, and postoperative DALK-related complications.

RESULTS

Four eyes from 2 patients with MPS I (Hurler's syndrome and Hurler-Scheie syndrome) and a history of DALK met inclusion criteria for the case series. Using the "big-bubble" technique, DALK was performed successfully in all eyes. Completed Descemet's membrane baring was achieved in 3 or 4 eyes and a pre-Descemet's membrane dissection in 1 eye. The mean age at the time of DALK was 17.3 years (range: 15.4 to 19.5 years). Mean follow-up time after DALK was 16.7 months (range: 6 to 31 months). Mean visual acuity before DALK was 20/80 (0.59 ± 0.12 logMAR). Mean visual acuity at the last visit for all 4 eyes was 20/50 (0.41 ± 0.17 logMAR). Visual acuity improved in all eyes. Recurrence of MPS corneal clouding was not noted in any of the corneal grafts.

CONCLUSIONS

DALK is a beneficial and preferable intervention in appropriate patients with significant corneal clouding due to MPS I. Improvement in vision can be obtained with stable, clear corneal grafts, although other ophthalmic manifestations may limit vision.