Bilateral DSAEK in 4-Month-Old With Presumed Congenital Hereditary Endothelial Dystrophy
Narrative Responses:
Purpose
To report a case of bilateral DSAEK performed on a 4-month-old infant with corneal edema and nystagmus.
Methods
A 4-month-old female presented with bilateral corneal edema, nystagmus, normal IOP, and normal corneal diameters. She responded to light in both eyes (OU), had corneal diameters of 11x11 mm OU and had corneal thicknesses (CCT) of 1088 microns OD and 1067 microns OS. Her clinical picture was consistent with autosomal recessive type II-congenital hereditary endothelial dystrophy (CHED). The patient underwent an uncomplicated DSAEK OD followed by DSAEK OS two weeks later. At post op week (POW) 14 of the DSAEK OS, the patient underwent a repeat procedure due to graft failure.
Results
At POW 1 after DSAEK OD, vision (VA) improved to fixing and following (F&F) and the nystagmus had resolved. The lenticle was attached and the cornea was clear. At POW 2, CCT OD was 568. At this time, the patient underwent a DSAEK OS. At POW 3 OS, lenticles were attached OU, and the cornea was completely clear OD and partially clear OS with a CCT OS of 884. At POW 14 OS, the patient underwent a repeat DSAEK OS for graft failure. By post-op month 3 OS, the corneas remained clear and VA was F&F OU without preference.
Conclusion
To our knowledge, our patient was the youngest in the United States to receive DSAEK. We witnessed a drastic improvement following her procedures. Given previous results in the literature as well as our own, we believe that DSAEK is a viable treatment option for infants with CHED.