Management of aniridic keratopathy with keratolimbal allograft: a limbal stem cell transplantation technique.

OBJECTIVE: Aniridic keratopathy is a major cause of vision loss in patients with aniridia. Penetrating keratoplasty has been proven ineffective for the long-term treatment of this disorder because it does not address the stem cell deficiency that is the primary etiologic factor. We evaluated the role of keratolimbal allograft (KLAL), a stem cell transplantation technique, for the treatment of patients with aniridic keratopathy.

DESIGN: Retrospective noncomparative interventional case series.

PARTICIPANTS: Thirty-one eyes of 23 patients with aniridic keratopathy.

INTERVENTION: KLAL.

MAIN OUTCOME MEASURES: Ocular surface stability, visual acuity, and success of subsequent penetrating or lamellar keratoplasty.

RESULTS: Thirty-one eyes of 23 patients were treated with KLAL and followed up for 12 to 117 months (mean, 35.7 months). Twenty-three eyes (74.2%) achieved a stable ocular surface. Overall, the mean visual acuity improved from 20/1000 to 20/165. Twenty eyes (64.5%) underwent subsequent penetrating keratoplasty. Fourteen corneal transplant grafts (70.0%) were successful, and six (30.0%) failed. Nineteen (90.5%) of 21 eyes receiving systemic immunosuppression obtained a stable ocular surface, whereas only 4 (40.0%) of 10 eyes not receiving systemic immunosuppression achieved ocular surface stability (P < 0.01).

CONCLUSIONS: KLAL is effective in treating aniridic keratopathy. Patients receiving systemic immunosuppression have a greater likelihood of achieving ocular surface stability and improved visual acuity compared with those who receive only topical immunosuppression.