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Outcome of penetrating keratoplasty for mucopolysaccharidoses
Erick D Bothun1, Alejandra Decanini, C Gail Summers
1Department of Ophthalmology, University of Minnesota, 420 Delaware Street SE, Minneapolis, MN 55455-5501, USA. bothu003@umn.edu
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.
Insights
Penetrating keratoplasty (PK) significantly improves vision in patients with corneal clouding from systemic mucopolysaccharidoses (MPS). Clear corneal grafts were achieved in most cases, demonstrating PK
Area of Science:
- Ophthalmology
- Genetics
- Corneal Surgery
Background:
- Systemic mucopolysaccharidoses (MPS) are rare genetic disorders.
- Corneal opacification is a common and visually debilitating complication of MPS.
- Penetrating keratoplasty (PK) is a surgical option for corneal clouding.
Purpose of the Study:
- To evaluate the outcomes of PK in patients with corneal opacification due to MPS.
- To assess graft clarity and visual acuity following PK in this patient population.
Main Methods:
- A consecutive case series of 5 patients (8 eyes) with MPS undergoing PK.
- A comprehensive literature review of PK in the setting of MPS.
Main Results:
- Visual acuity improved in 7 of 8 eyes after PK (mean logMAR from 0.90 to 0.32).
- No recurrent MPS-related corneal opacity was observed in grafts.
- Literature review identified 40 initial and 3 repeat PK cases in MPS patients, with most grafts remaining clear.
Conclusions:
- Penetrating keratoplasty is a beneficial treatment for corneal clouding in MPS patients.
- Stable and clear corneal grafts can be achieved, leading to improved vision.
