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Endothelial cell density in children with posterior polymorphous corneal dystrophy: a longitudinal case-control study
Simon Sheung Man Fung1, Hamza Sami2, Ali El Hamouly2
1Department of Ophthalmology, University of California Los Angeles, Los Angeles, CA, USA. simonfung@mednet.ucla.edu.
Insights
Children with posterior polymorphous corneal dystrophy (PPCD) have significantly lower endothelial cell density, particularly in diffuse types. However, the annual rate of endothelial cell loss in PPCD eyes is similar to normal eyes.
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.
Objective:
To evaluate longitudinal endothelial cell characteristics of children with posterior polymorphous corneal dystrophy (PPCD).
Methods:
In this prospective case-control study, children with PPCD were followed with slit-lamp photography and non-contact specular microscopy. Patient's eyes were subdivided according to the clinical subtypes of PPCD (vesicular, band, diffuse, and unaffected) and the number of lesions present on the posterior corneal surface. Findings were then compared with age-matched controls.
Results:
Thirty eyes of 15 patients with PPCD with a mean age 10.5 ± 3.1 years were analysed. Mean follow-up was 3.0 ± 1.0 years. PPCD morphology was vesicular in 40%, diffuse in 37%, band type in 10% and 13% had no detectable lesions despite contralateral involvement. Fourteen eyes (47%) had ≥5 endothelial lesions. Patients with PPCD had significantly lower endothelial cell densities (ECD) at recruitment (1918.9 ± 666.3 vs. 3340.1 ± 286.5 cells/mm2, p < 0.007) and at final follow-up (1793.1 ± 684.6 vs. 3265.2 ± 304.3 cells/mm2, p < 0.007) compared to age-matched controls. The lowest ECDs were found in eyes with diffuse type PPCD and those with ≥5 posterior corneal lesions, while clinically unaffected eyes in patients with confirmed PPCD in fellow eye had a normal ECD. However, the rates of annual ECD decline were not significantly different between eyes with PPCD in general, between the subgroups of PPCD and the normative groups.
Conclusion:
Endothelial cell density is significantly reduced among children with PPCD and depends on the clinical subtype and the number of posterior corneal lesions present. However, annual ECD loss is similar between normal eyes and those with PPCD.
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