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

Ophthalmology. 2003 Jan;110(1):125-30. doi: 10.1016/s0161-6420(02)01451-3.


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.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aniridia / complications
  • Aniridia / surgery*
  • Child
  • Child, Preschool
  • Corneal Diseases / etiology
  • Corneal Diseases / surgery*
  • Epithelial Cells / transplantation
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Keratoplasty, Penetrating
  • Limbus Corneae / cytology*
  • Male
  • Middle Aged
  • Retrospective Studies
  • Stem Cell Transplantation / methods*
  • Transplantation, Homologous
  • Visual Acuity


  • Immunosuppressive Agents