Endothelial keratoplasty for posterior polymorphous corneal dystrophy in a 4-month-old infant

M Hermina Strungaru1,2, Asim Ali1, David Rootman1

  • 1Department of Ophthalmology and Vision Sciences, University of Toronto, Toronto, Canada.

Insights

This study presents the youngest case of endothelial keratoplasty (EKs) in a 4-month-old infant with posterior polymorphous corneal dystrophy. Descemet stripping automated endothelial keratoplasty (DSAEK) proved successful after a failed Descemet membrane endothelial keratoplasty (DMEK) attempt.

Area of Science:

  • Ophthalmology
  • Corneal Surgery
  • Pediatric Ophthalmology

Background:

  • Posterior polymorphous corneal dystrophy (PPCD) is a rare genetic disorder affecting corneal endothelium.
  • Endothelial keratoplasty (EK) is a surgical option for corneal endothelial dysfunction.
  • Infantile EK presents unique surgical challenges due to anatomical and physiological differences.

Observation:

  • A 4-month-old infant with PPCD underwent Descemet membrane endothelial keratoplasty (DMEK).
  • Postoperative graft dislocation occurred on day 5, with unsuccessful attempts to re-position the DMEK graft.
  • The infant was subsequently treated with bilateral Descemet stripping automated endothelial keratoplasty (DSAEK).

Findings:

  • This case represents the youngest patient to undergo EKs at 4 months of age.
  • The initial DMEK procedure highlighted potential challenges in infant corneal transplantation.
  • Successful visual outcomes (20/70 and 20/60) were achieved with DSAEK at 3-year follow-up.

Implications:

  • DSAEK is a viable and effective treatment for infantile endothelial dysfunction secondary to PPCD, even after DMEK failure.
  • This case underscores the importance of considering alternative EK techniques in pediatric patients.
  • Further research into optimizing DMEK techniques for infants may be warranted.
Abstract

Related Concept Videos