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

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/mm, p < 0.007) and at final follow-up (1793.1 ± 684.6 vs. 3265.2 ± 304.3 cells/mm, 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.