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/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.

Corneal endothelium in paediatric patients with uveitis: a prospective longitudinal study.

PURPOSE: To study the longitudinal effect of anterior chamber inflammation on the corneal endothelium in children. METHODS: In this prospective longitudinal observational study, children (aged <18 years) with anterior chamber inflammation and those at risk of developing uveitis due to juvenile idiopathic arthritis (JIA) were included. Changes in central endothelial cell density (ECD) and morphological variables were determined by non-contact specular microscopy, and their correlations with uveitis activity and surgical interventions were analysed. RESULTS: Ninety-nine eyes of 99 children (mean age (±SD): 10.0±4.1 years) with a history of anterior chamber inflammation were recruited. Mean follow-up was 12.3±3.5 months. Eleven children, who were under surveillance but had not developed JIA-associated uveitis were included as controls. While there were no significant differences in mean ECD between controls and subjects without prior surgery (group 1) at all time points, those who had prior ophthalmic surgery (group 2) displayed significantly lower ECD than the controls at recruitment (p=0.002) and at follow-up (p=0.004). However, longitudinal ECD assessments did not show significant changes in either group (group 1, p=0.07, group 2, p=0.54). On regression analysis, once the patient's age was adjusted for, only the occurrence of intraocular procedures during the study (r=0.43, adjusted p=0.03) was associated with a significant annual rate of ECD loss. CONCLUSION: During the study period, longitudinal ECD changes among children with uveitis were associated with intraocular surgery for uveitis-related complications but not uveitis activity. By reducing the need for surgical intervention, the corneal endothelium in these children may be preserved.

Endothelial Cell Loss in Obstetric Forceps-Related Corneal Injury: A Longitudinal Specular Microscopic Study.

PURPOSE: To assess the impact of ocular trauma associated with obstetric forceps delivery on the corneal endothelium in children. METHODS: Five patients who attended the Hospital for Sick Children, Toronto, between 2013 and 2016 with a diagnosis of unilateral obstetrical forceps-related corneal injury were included. Clinical presentation, best-corrected visual acuity, corneal endothelial cell density (ECD, cells/mm), and measures of cellular morphology were obtained. RESULTS: The mean follow-up duration was 30 ± 10 months. Mean ECD in the affected eye at initial assessment was significantly lower than that of the fellow eye (2576 ± 733 vs. 3481 ± 288 cells/mm, P = 0.02). At final follow-up, mean ECD was 3293 ± 175 and 1907 ± 524 cells/mm in the normal and affected eyes, respectively (P = 0.004). The mean annual rate of the ECD decrease was higher in the affected eyes than in the normal eyes (9.1% ± 4.2% vs. 2.0% ± 2.5%), although this difference was not statistically significant (P = 0.06). CONCLUSIONS: In children with forceps-related corneal injury, lower ECD with a higher annual decrease can be assessed with specular microscopy for risk stratification and parental counseling purposes.