Delayed PK may be optimal for congenital hereditary endothelial dystrophy

Penetrating keratoplasty for congenital hereditary endothelial dystrophy yielded better visual and anatomic outcomes in older patients than younger patients, a study found.

“Congenital hereditary endothelial dystrophy is a rare disease that presents as diffuse corneal edema of varying degree at or soon after birth due to primary degeneration and dysfunction of corneal endothelium,” the study authors said. “The decision for timing of surgical intervention is difficult because the disease occurs in the critical period of visual development, and taking into consideration the potential risks associated with pediatric PK.”

The researchers retrospectively reviewed the records of 65 eyes of 35 patients organized into two groups. The first group comprised 47 eyes of 24 patients with a mean age of 26.4 years; the second group included 18 eyes of 11 patients with a mean age of 7.6 years.

Mean postoperative follow-up was 101 months for the first group and 59.7 months for the second group.

Study results showed that the first group had significantly higher graft clarity rates than the second group (P = .023). The first group also had significantly better visual outcomes than the second group (P < .001).

Thirty-nine eyes in the first group (83%) and five eyes in the second group (28%) had postoperative best corrected visual acuity of 20/80 or better.

In the first group, postoperative BCVA was 20/80 or better in 76% of eyes with nystagmus and 91% of eyes without nystagmus.

Further study of endothelial keratoplasty as an alternative to PK in patients with congenital hereditary endothelial dystrophy is warranted, the authors said.

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Source:

Cornea. 2012;31(4):359-365.