Bilateral Descemet Membrane Endothelial Keratoplasty in an Infant With Congenital Hereditary Endothelial Dystrophy

Cornea. 2021 Sep 1;40(9):1201-1203. doi: 10.1097/ICO.0000000000002616.

Abstract

Purpose: Descemet membrane endothelial keratoplasty (DMEK) has been reported in children but not in infants. We evaluate the outcomes and complications of DMEK for an infant with congenital hereditary endothelial dystrophy.

Methods: A 3-month-old male infant with congenital hereditary endothelial dystrophy underwent unilateral DMEK, which was complicated by incomplete graft unfurling. Bilateral DMEK was then performed successfully. The visual acuity, pachymetry, corneal transparency, and complications were assessed over 3 months of follow-up.

Results: At 3 months postoperatively, the nystagmus had resolved and visual acuity improved from 20/2000 to 20/270 in each eye. Pachymetry, which had been unmeasurable preoperatively, was 695 μm in the right eye and 678 μm in the left eye. On examination, there was improvement in corneal transparency bilaterally. There were no postoperative complications.

Conclusions: We demonstrate that DMEK is feasible in infants and propose that it should be considered in the treatment of corneal endothelial disorders of infancy. In theory, DMEK might confer improved visual potential and lower risk for rejection compared with other corneal transplantation techniques, although further studies in this age group are needed.

Publication types

  • Case Reports

MeSH terms

  • Anion Transport Proteins / genetics
  • Antiporters / genetics
  • Corneal Dystrophies, Hereditary / diagnosis
  • Corneal Dystrophies, Hereditary / genetics
  • Corneal Dystrophies, Hereditary / physiopathology
  • Corneal Dystrophies, Hereditary / surgery*
  • Corneal Pachymetry
  • Descemet Stripping Endothelial Keratoplasty*
  • Follow-Up Studies
  • Humans
  • Infant
  • Intraoperative Complications
  • Male
  • Nystagmus, Pathologic / physiopathology
  • Retinoscopy
  • Treatment Outcome
  • Visual Acuity / physiology

Substances

  • Anion Transport Proteins
  • Antiporters
  • SLC4A11 protein, human