Descemet stripping automated endothelial keratoplasty for spontaneous descemet membrane detachment in a patient with osteogenesis imperfecta.

PURPOSE

To report the first use of Descemet stripping automated endothelial keratoplasty (DSAEK) for spontaneous Descemet membrane detachment in a patient with osteogenesis imperfecta (OI), keratoconus, and acute bullous keratopathy.

METHODS

A case report of a 25-year-old man with OI and symptomatic unilateral bullous keratopathy secondary to spontaneous Descemet membrane detachment is described. The patient presented with acute loss of vision in his right eye and was noted on slit-lamp examination to have a totally detached and taut Descemet membrane, spanning the anterior chamber. This diagnosis was confirmed with an anterior segment OCT (Visante; Carl Zeiss Meditec, Dublin, CA).

RESULTS

After an attempt to reattach the Descemet membrane with air bubbling was unsuccessful, the patient underwent successful DSAEK. Seven months postoperatively, his best spectacle-corrected visual acuity was 20/50 compared with his other eye, which was 20/40. There were some central folds in the donor noted immediately postoperatively that have persisted.

CONCLUSIONS

To the best of our knowledge, this case is the first documented spontaneous Descemet membrane detachment in a patient with OI and probable keratoconus treated successfully with DSAEK. Unlike in classical keratoconus hydrops, medical management is unlikely to resolve a total Descemet membrane detachment. This unusual Descemet membrane detachment was tightly stretched across the anterior chamber without any obvious tear, making simple air or gas bubble apposition impossible. Attempts to tear the Descemet membrane and then bubble led to significant folds. DSAEK was successful in removing the detached Descemet membrane and replacing it with the donor tissue.