Corneal Endothelial Features in Patients Operated for Primary Congenital Glaucoma

To compare the corneal endothelial cell morphology using specular microscopy imaging between primary congenital glaucoma (PCG) and age-matched healthy controls, and to determine if the endothelial cell parameters vary among different subtypes of PCG.

Retrospective case-control study.

One hundred forty-five eyes of 145 patients operated for PCG between 1991 and 2018 and who returned for a follow-up visit between June 2021 and May 2022. Three hundred and nineteen age-matched healthy individuals constituted the control group.

Corneal endothelial cell layer analysis was performed with EM-3000 (Tomey) noncontact specular microscope. Patients were categorized according to the clinical subtypes of PCG (neonatal, infantile, and late-onset) based on the Childhood Glaucoma Research Network classification.

Specular microscopic parameters, including endothelial cell density (ECD), average cell size, coefficient of variation (CV), and maximum and minimum cell size, were compared with that of the control group.

Mean ± standard deviation (SD) age of patients at endothelial imaging was 13.5 ± 6.54 years, and there was a male preponderance (n = 88, 61%). The majority of the patients had infantile-onset PCG (n = 67, 46%). Eyes with PCG demonstrated a significantly lower ECD compared with controls (2158.7 ± 636.5 vs. 2840.9 ± 232.5 cells/mm²; P < 0.0001). Other endothelial cell parameters were also significantly worse in PCG compared with controls. The mean ± SD ECD was 2344.3 ± 544.3, 2131.9 ± 626.4, and 2054.2 ± 714.0 cells/mm² in neonatal onset, infantile, and late-onset PCG groups, respectively. Although the ECD was lowest in the late-onset PCG group, there was no significant difference in the ECD between the 3 subgroups. Except for CV, all the endothelial cell parameters were significantly worse in eyes with Haab striae than in eyes without.

Patients with PCG have significantly lower ECD compared with healthy controls. Furthermore, other endothelial cell parameters were also significantly worse in the PCG group. These changes in the corneal endothelium in patients with PCG should be considered in long-term medical and surgical management.

The author(s) have no proprietary or commercial interest in any materials discussed in this article.