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Clinical Features in Children with Posterior Polymorphous Corneal Dystrophy.

Ye Jin Ahn1, Soon Il Choi, Hae Ri Yum

  • 1*MD †MD, PhD Department of Ophthalmology and Visual Science, Seoul St. Mary's Hospital, College of Medicine, Seoul St. Mary's Hospital, The Catholic University of Korea, Seoul, Republic of Korea (YJA, SIC, SYS, SHP); and Department of Ophthalmology, Konyang University Myunggok Medical Research Institute, Konyang University Hospital, College of Medicine, Konyang University, Daejeon, Republic of Korea (HRY).

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Posterior polymorphous corneal dystrophy (PPCD) in children can cause amblyopia and significant endothelial cell loss. Early refractive error treatment and long-term monitoring are crucial for preserving vision in pediatric PPCD patients.

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Area of Science:

  • Ophthalmology
  • Genetics
  • Pediatric Medicine

Background:

  • Posterior polymorphous corneal dystrophy (PPCD) is a rare genetic eye condition affecting the cornea.
  • Early diagnosis and management are vital for visual development in children.

Purpose of the Study:

  • To detail the clinical characteristics of pediatric patients diagnosed with PPCD.
  • To describe the visual outcomes and corneal endothelial changes in children with PPCD.

Main Methods:

  • Retrospective analysis of seven Korean pediatric patients with PPCD followed for at least 3 years.
  • Comprehensive ocular examinations including visual acuity, intraocular pressure, refraction, and specular microscopy.

Main Results:

  • Four patients had unilateral involvement; eight eyes showed amblyogenic astigmatism (>1.5D).
  • Two of four pre-school children had amblyopia, improving with treatment.
  • PPCD eyes exhibited reduced endothelial cell density (ECD) with significant loss over 3 years and a negative correlation between astigmatism and ECD.

Conclusions:

  • Prompt management of refractive errors is essential in early-onset PPCD to prevent or treat amblyopia.
  • Pediatric patients with PPCD require long-term corneal endothelial monitoring due to progressive cell loss.