Purpose: The outcome of successful penetrating keratoplasty (PK) typic
ally is poor in eyes with end-stage chronic cicatrizing conjunctival d
iseases such as ocular cicatricial pemphigoid (OCP), Stevens-Johnson s
yndrome, and toxic epidermal necrolysis due to immunologically driven
conjunctival inflammation associated with conjunctival cicatrization a
nd lid abnormalities, severe dry eye, and extensive corneal neovascula
rization. The authors report the results of their experience with PK i
n 13 patients with OCP, Stevens-Johnson syndrome, and toxic epidermal
necrolysis. Methods: The authors reviewed the records of patients with
OCP, Stevens-Johnson syndrome, or toxic epidermal necrolysis seen bet
ween 1976 and 1992. Patients who underwent PK were examined for the pu
rpose of this study. Initial and final visual acuity, indications for
PK, surgical procedure, postoperative therapy, complications, total nu
mber of repeat PKs, length of follow-up, and the final outcome were re
corded. Results: Thirty-two PKs were performed in 16 eyes of 13 patien
ts with advanced OCP (6 patients), OCP as a sequels of Stevens-Johnson
syndrome (2 patients), Stevens-Johnson syndrome (3 patients), and tox
ic epidermal necrolysis (2 patients). The indications for the first PK
were corneal perforation in six eyes (37.5%) and extensive corneal sc
arring in ten eyes (62.5%). Preoperative visual acuity was counting fi
ngers in five eyes, hand motions in eight, and light perception in thr
ee. Preoperative therapy included systemic chemotherapy (8 patients),
mucous membrane grafting (9 eyes), lamellar keratoplasty (2 eyes), sup
erficial keratectomy (1 eye), and corneal dye laser photocoagulation (
6 eyes). The mean follow-up period was 4.6 years (3 months-13 years).
Eight eyes (50%) had clear grafts, and three eyes (18.7%) had 20/200 o
r better visual acuity at last visit. The major causes of graft failur
e were epithelial defect formation/persistence, stromal ulceration, pe
rforation, and graft rejection. Conclusions: These results indicate th
at PK may be performed for tectonic reasons, but prospects for restora
tion of sight in patients with advanced cicatrizing conjunctival disea
ses, even after extensive preoperative medical and surgical therapy, a
re limited.