Name

Résultats de la kératoplastie dans les mucopolysaccharidoses: étude rétrospective de 40 yeux

To describe visual outcomes after penetrating keratoplasty (PK) in patients with significant corneal clouding due to mucopolysaccharidoses (MPS)

Historical cohort of patients with MPS and corneal clouding followed in our tertiary center over a 35-year period. Preoperative and postoperative visual outcomes, ocular comorbidities, and graft complications were reported. A functional success was defined as any improvement in best-corrected visual acuity (BCVA) after keratoplasty

40 eyes from 24 patients with MPS I (Hurler syndrome (n=16) and Hurler-Scheie syndrome (n=5)) or MPS VI (n=3) with a history of keratoplasty were identified. The median age at the time of keratoplasty was 19 years (8 to 33 years). Ocular pathway comorbidities were identified in 50% of patients: glaucoma (n=7), retinal degeneration (n=6), optic neuropathy (n=3).

After a median follow-up duration of 65.8 months (36.1-123.7), visual acuity improved in 27 eyes (77.1%), stabilized in 1 eye (2.9%) and decreased in 7 eyes (20%). Median visual acuity was 20/115 (0.75 logMAR (0.70-1.00)) before keratoplasty and 20/50 (0.40 logMAR (0.30-0.65)) at the last visit after keratoplasty, corresponding to a gain of 0.30 logMAR (95% CI: [-0.40, -0.20], p=0.001). Acute rejection episodes occurred in 7.5% of grafts (n=3). 90% of eyes (n=36) treated with corneal graft did not present a recurrence of MPS corneal clouding.

Studies investigating the effects of keratoplasty on the course of corneal clouding due to MPS are rare. Our study suggests that PK positively influences the course of corneal clouding due to MPS by significantly improving the visual acuity of patients. Keratoplasty in this indication seems to be well tolerated with few rejection and recurrence on corneal button. Although less described in corneal clouding associated with MPS, deep anterior lamellar keratoplasty (DALK) could be a surgical alternative in these patients whose endothelial function is often preserved.

PK is a beneficial intervention in appropriate patients with significant corneal clouding due to MPS I and VI. Clear corneal graft can be obtained for patients, although other ophthalmic manifestations may limit improvement in visual acuity