Successful Descemet stripping endothelial keratoplasty in congenital hereditary endothelial dystrophy.

PURPOSE

To report successful Descemet stripping endothelial keratoplasty (DSEK) in a patient with congenital hereditary endothelial dystrophy (CHED).

METHODS

A 19-year-old boy presented with complaints of decreased vision, hazy corneas, and nystagmus since birth. Six months prior, the right eye had undergone penetrating keratoplasty. In the left eye, he was planned for DSEK.

RESULTS

Successful Descemet membrane (DM) scoring and stripping was achieved with a reverse Sinskey hook after trypan blue staining. Manually dissected posterior stromal donor tissue was transplanted using Busin glide. Corneal edema resolved completely with a final best-corrected visual acuity of 20/100 at 6 months. Serial anterior segment optical coherence tomography scans showed maximum decrease in stromal thickness in the first month. Despite resolution of corneal edema, mild stromal haze persisted, which decreased with time. Histopathology of removed DM showed thickened DM and absence of endothelial cells which was suggestive of CHED.

CONCLUSIONS

To the best of our knowledge, this is the first reported case of successful DSEK in a patient with CHED.