Descemet membrane endothelial keratoplasty in a child with corneal endothelial dysfunction in Kearns-Sayre syndrome

To evaluate clinical outcomes and complications after Descemet membrane endothelial keratoplasty (DMEK) in a child.

A 12-year-old boy with Kearns-Sayre syndrome (chronic progressive external ophthalmoplegia, cardiac conduction block, and pigmentary retinal degeneration) and corneal endothelial dysfunction was successfully treated with DMEK. Corneal transparency, central corneal thickness (CCT), endothelial cell density (ECD), visual outcomes, and complication rates were measured during the follow-up of 6 months.

Best spectacle-corrected visual acuity (BSCVA) improved from counting fingers at 4 feet preoperatively to 20/100, 1 week after surgery. The ECD of the graft was 2595 cells per square millimeter. The CCT diminished from 837 μm preoperatively to 735 μm 1 week after surgery. Six months postoperatively, the BSCVA was still 20/100, and the cornea remained clear and compact. The ECD was 2341 cells per square millimeter and CCT was almost normal with 583 μm. No postoperative complications were observed. Fundus examination showed atypical pigmentary retinal degeneration with arterial narrowing. Electroretinography with full-field flash stimulation showed bilaterally severe retinal dysfunction with absent photopic and scotopic amplitudes explaining the reduced BSCVA.

Although DMEK has been used in adult populations, we are unaware of previous reports of DMEK in a child. DMEK should be considered as a feasible technique in pediatric patients with endothelial dysfunction.