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.


METHODS
Retrospective noncomparative interventional case series.


METHODS
Thirty-one eyes of 23 patients with aniridic keratopathy.


METHODS
KLAL.


METHODS
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.
Holland
Edward J
EJ
Cincinnati Eye Institute, University of Cincinnati, Cincinnati, Ohio 45242, USA. eholland@fuse.net
Djalilian
Ali R
AR
Schwartz
Gary S
GS
D000293:Adolescent; D000328:Adult; D000368:Aged; D015783:Aniridia / Q000150:complications / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D003316:Corneal Diseases / Q000209:etiology / Q000601:surgery*; D004847:Epithelial Cells / Q000637:transplantation; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015948:Keratoplasty, Penetrating; D016850:Limbus Corneae / Q000166:cytology*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D033581:Stem Cell Transplantation / Q000379:methods*; D014184:Transplantation, Homologous; D014792:Visual Acuity
D007166:Immunosuppressive Agents