Comparative evaluation of corneal characteristics in eyes of primary congenital glaucoma and Axenfeld‐Rieger syndrome

• Published in: Acta ophthalmologica (Oxford, England) Vol. 100; no. S275
• Main Authors: Mahalingam, Karthikeyan, Nathiya, Venkatesh, Singh, Abhishek, Nag, T. C., Sen, Seema, Titiyal, Jeewan, Gupta, Viney, Gupta, Shikha
• Format: Journal Article
• Language: English
• Published: Malden Wiley Subscription Services, Inc 01-12-2022
• Subjects: Collagen (type IV); Cornea; Corneal transplantation; Endothelial cells; Eye; Glaucoma; Immunohistochemistry
• DOI: 10.1111/j.1755-3768.2022.0287
• EISSN: 1755-3768
• ISSN: 1755-375X

Purpose: To evaluate and compare the primary dysgenesis of cornea in Primary Congenital Glaucoma (PCG) and Axenfeld‐Rieger Syndrome (ARS).
Methods: 26 eyes of PCG and 40 eyes of ARS were included. The following parameters were noted: clinico‐demographic profile; ASOCT of cornea/angle; Ocular biomechanical parameters, intraocular pressure (IOP) and central corneal thickness(CCT) (using Corvis ST); corneal aberration (iTrace, Tracey Technologies); morphological and cellular structure of the cornea (Confoscan HRT‐III:RCM) and histopathology of cornea (after keratoplasty).
Results: Mean corneal diameter of PCG (13.4 ± 1 mm) was higher (p = 0.01) than ARS (11.7 ± 1.6 mm). Haab striae were observed more in the eyes of PCG (63.6%) than ARS (5%). Posterior corneal hyperreflectivity was present in 100% PCG eyes v/s 85.2% ARS (p = 0.006). Angle dysgenesis was comparable: PCG (85.4%) and ARS (90.7%). The presence of a double membrane sign, indicating thickened PDL and DM, was visible exclusively in eyes of PCG (29.2%) v/s singular posterior corneal hyperreflectivity (85.2%), indicating thickened DM complex, visible in eyes of ARS. Corneas of ARS were 52.3 μm thicker than PCG. In eyes of both PCG and ARS, the most common total ocular aberration was astigmatism. The endothelial cell count in PCG {1703 ± 661 cells/mm2} and ARS {1442 ± 397 cells/mm2} was comparable. The PDL thickness in the eyes of PCG (11.8 μm) was greater than ARS (9.1 μm). In immunohistochemistry, all tissues of PCG (n = 9) were collagen IV positive (indicating presence of PDL), two tissues of ARS (22.2%) were collagen IV negative.
Conclusions: The difference in characteristic of PDL may cause difference in corneal enlargement and angle differences between the two groups.