Penetrating keratoplasty and keratolimbal allograft transplantation for corneal perforations associated with the ectodermal dysplasia syndrome.
OBJECTIVE
To report two patients with ectodermal dysplasia-associated keratopathy treated with keratolimbal allograft transplantation and penetrating keratoplasty.


METHODS
Two patients with ectodermal dysplasia suffered multiple corneal perforations. In both patients, keratolimbal allograft transplantation was performed along with penetrating keratoplasty. Histopathology of the corneal buttons was obtained.


RESULTS
Histopathologic examination showed chronic inflammation with thin, irregular epithelium, loss of stromal tissue, and thin or absent Bowman's membrane. Both patients had multiple contributing factors predisposing them to corneal perforations including an inherent ectodermal defect, trichiasis, poor tear film, and limbal stem cell deficiency.


CONCLUSIONS
Patients with keratopathy and corneal perforations associated with the ectodermal dysplasia syndrome has a poor prognosis. Keratolimbal allograft transplantation may have a role in the treatment of this disease. Longer follow-up and additional case reports will determine whether this technique confers a better prognosis than keratoplasty alone.
Anderson
Nicole J
NJ
Minnesota Eye Consultants, Minneapolis, MN 55404, USA.
Hardten
David R
DR
McCarty
Tina M
TM
D000328:Adult; D003316:Corneal Diseases / Q000209:etiology* / Q000601:surgery*; D016039:Corneal Transplantation; D004476:Ectodermal Dysplasia / Q000150:complications*; D005260:Female; D006801:Humans; D015948:Keratoplasty, Penetrating; D008875:Middle Aged; D011379:Prognosis; D014184:Transplantation, Homologous; D016896:Treatment Outcome