Purpose: To analyze graft survival and visual outcome after penetrating ker
atoplasty in infants with congenital corneal opacity. Methods: We retrospec
tively reviewed the records of 11 patients with congenital corneal opacity
who underwent penetrating keratoplasty as infants. Six patients had a diagn
osis of Peter's anomaly, 1 of congenital hereditary endothelial dystrophy,
1 of posterior polymorphous dystrophy, and 2 of sclerocornea, and in the ot
her patient, the cause of the opacity was unknown. Results. In total, 26 pe
netrating keratoplasties were performed on 16 eyes of 11 infants. All patie
nts initially underwent surgery before the age of 13 months. Five patients
underwent bilateral penetrating keratoplasty, and 10 of the transplants wer
e repeat operations on eyes that had already had at least one previously fa
iled graft. The age of the infants at the time of first penetrating keratop
lasty ranged from 2 to 56 weeks (median, 13 weeks). The graft survival time
for all transplants ranged from 3 to 137 months (median, 16 months). Overa
ll first graft survival at 12 months was 61% (95% Cl, 33%-81%), with 10 of
16 eyes retaining a clear corneal graft. Peter's anomaly, lensectomy, and r
epeat penetrating keratoplasty were factors most highly associated with poo
r graft survival and a low final visual acuity. Conclusion: Early penetrati
ng keratoplasty for congenital corneal opacity may prevent deprivation ambl
yopia. Although this procedure carries a high risk of failure, particularly
in those patients with Peter's anomaly, careful case selection, optical co
rrection, and management of postoperative amblyopia may result in a success
ful visual outcome.