Endothelial cell density in children with posterior polymorphous corneal dystrophy: a longitudinal case-control study

Area of Science:

• Ophthalmology
• Corneal Diseases
• Pediatric Ophthalmology

Background:

• Posterior polymorphous corneal dystrophy (PPCD) is a rare genetic disorder affecting the cornea.
• Endothelial cell density (ECD) is a key indicator of corneal health.
• Understanding longitudinal changes in ECD in pediatric PPCD is crucial for patient management.

Purpose of the Study:

• To longitudinally evaluate endothelial cell characteristics in children diagnosed with posterior polymorphous corneal dystrophy (PPCD).
• To compare endothelial cell density (ECD) and its decline rate in pediatric PPCD patients with age-matched controls.

Main Methods:

• Prospective case-control study involving children with PPCD.
• Utilized slit-lamp photography and non-contact specular microscopy for data collection.
• Eyes were categorized by PPCD subtype (vesicular, band, diffuse) and lesion count for comparison with controls.

Main Results:

• Pediatric PPCD patients exhibited significantly lower endothelial cell densities (ECD) compared to controls at baseline and follow-up.
• Eyes with diffuse PPCD and those with 5 or more posterior corneal lesions showed the lowest ECD.
• Clinically unaffected fellow eyes in PPCD patients maintained normal ECD, but annual ECD decline rates did not differ significantly between PPCD and control groups.

Conclusions:

• Endothelial cell density is significantly reduced in children with PPCD, varying by clinical subtype and lesion burden.
• Despite lower baseline ECD, the rate of endothelial cell loss over time is comparable between eyes with PPCD and healthy eyes.